There  is a rapidly growing group of people who now believe a concept of  infectious disease that is reminiscent of the model of the flat earth.  It is called the “terrain model” and its proponents are germ theory  denialists, a/k/a people who don’t believe that microbes such as viruses  and bacteria cause disease. The terrain folks actually believe that  disease spreads from one person to another not due to an external  agent—a pathogenic bug—but because the internal environment of the  receiving person is “weak” or “toxic” and therefore they cannot fend off  disease. Yes, this is a “victim is at fault” theory that can be  infuriating to those of us who understand science and psychology,  especially those of us who live with CF. Certainly, it is true that in  order to stay healthy it helps to have a healthy/strong internal  environment. Another phrase for this internal environment is “immune  system,” so the theory isn’t completely off base. It is a bit like the  old adage: the rich get richer. In this case, the idea is that the  healthy stay healthy. The corollary is that the sick tend to get sicker,  which is sort of true. But to blame the sick for this is heartless. And  to actually deny that pathogens exist and cause disease is hard to  swallow.

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Now I’m  sure that readers of this particular publication are well versed in the  scientific theory/evidence that bacteria and viruses do, in fact, cause  illness, so I don’t need to dispel terrain theory here. I’m simply  mentioning it because there are people out there who likely have no  significant scientific background, nor personal history of the effects  of bacteria and viruses quite like we do. It is easy to get angry at  these people and add to the polarity that we see every time we watch the  news or walk into a Walmart. But anger doesn’t help. It just entrenches  everyone further into their stance of “I am right, and you are an  idiot.”

Before I  knew anything about science or immune systems or pathogens, I got a flu  shot every year. I did this because I was forced to do so. My parents  gave me no choice. It was my least favorite day of the year, but they  indoctrinated me into the wisdom of doing this by telling me over and  over why it was important and by doing it themselves. They did such a  good job that, by the time I was old enough to do so, I rode my bike  voluntarily to Dr. Brugh’s office myself, without a hint of objection,  to take this unwelcome poke in my arm.

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Most  people aren’t raised like this. Most people in our world haven’t learned  from a very young age that they routinely have to tolerate  uncomfortable procedures to stay alive. Most people also haven’t been  conditioned to learn the names of the bacteria that colonize their lungs  when they are sick. They don’t have the direct lived experience of  noticing that feeling like crap correlates perfectly with the green  color of their sputum, which indicates that pseudomonas is on another  rampage inside their body. To a person with CF, terrain theory is  absolutely, mind-numbingly crazy.

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But we  are a minority. Fortunately, most people who don’t have CF also believe  in science, specifically in microbiology and virology. As I type this,  there is a slim majority of people in the U.S. who are fully vaccinated  against COVID-19. Today, the rate is 53.6%. Hopefully, by the time you  read this, it will be higher, but reading about the terrain theory  people has wiped out my hope that the vaccination rate will be the thing  that ends this pandemic. Since access to the vaccine isn’t actually a  problem now, I fear the 46.4% who are not vaccinated have made a  conscious decision not to do so.

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Decisions  like this are very personal, of course. The mRNA vaccinations are newer  technology, and many people are very wary about subjecting their bodies  to what they consider “unproven” science. They also likely have no idea  how clinical trials are run, and therefore don’t trust that adequate  scrutiny of this newer technology was conducted at such a fast pace.  Even I was amazed at how fast the vaccines were rolled out, being very  aware from a young age at how frustratingly slow it can be for a new  drug to be approved.

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For me,  this lightning speed was evidence of how much science has progressed.  For the anti-vax people, I suspect this speed was evidence that the  books were cooked, that we are all being deceived, and that this is all a  ploy to take away our freedom and allow the rich to get richer. We all  come from different backgrounds, with more or less exposure to science,  and with more or less trust in corporate America. Hence, the divide.

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When I  think about my decision process regarding the vaccine, it didn’t take  very long. The decision was automatic. Of course, I would get the  vaccine. In fact, my main concern was how fast could I get it? What led  to this automaticity of the decision? Well, it started with those  mandatory flu shots. That conditioned me to understand by direct  experience that vaccinations work. I rarely got the flu. Then I learned  the science. Even before medical school, I loved science. I loved the  scientific method and I trusted that using the scientific method got us  as close to “the truth” as humans can get. Then I learned, through my  own life with CF, as well as from professors of microbiology, about  bacteria and viruses and how they do damage. I viewed them under the  microscope (bacteria) and electron microscope (viruses). I learned that  they exist. At some point, I did autopsies on people who had died from  infections with bacteria and viruses. Talk about real-life experience!

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So I had  no doubts and the vaccination decision was automatic, like a reflex  hammer causing a leg to kick. But I’m not your average bear. It helps  me, when I read about terrain theory or see anti-vax or anti-mask people  asserting their rights, to realize that people only act the way they do  because of what they have experienced in life and what they have been  told and believe. After all, when we enter into this world, we have no  beliefs about anything. They are all acquired at some point. People who  don’t believe vaccinations work have clearly lived lives filled with  very different experiences from mine. To call them crazy or idiotic,  while it may feel good in the moment, is not seeing the big picture.

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I have  good friends who are in this category. They won’t get the vaccine. Some  don’t trust it, and others don’t even believe that COVID-19 is real. A  man I look up to in many ways believes in terrain theory. He is a good  man with a good heart (he also happens to live in a very healthy body).  At the end of the day, as I lie on my deathbed (hopefully not with  COVID-19), I wonder what decision would sit best with me? Calling these  other people stupid and shutting them completely out of my life or  trying to understand their decision? And by “understanding” I don’t mean  “agreeing with” their decision. After all, we all want to be healthy.  We all have an inborn instinct to do whatever we can to stay alive. We  just have, at this moment in history, very different ideas of how that  actually works.

Dr.  Julie Desch is 60 years old and has CF. She lives in San Rafael, CA,  with her partner and their three dogs. She enjoys biking, meditating,  and filling her days with joy.

Many  employers have ended remote working arrangements, requiring all  employees to return to in-person work. However, the COVID-19 pandemic  continues. Many people with cystic fibrosis continue to be concerned  about exposure to and infection with COVID-19 given the prevalence of  the highly transmissible Delta variant. Many people with CF have  successfully worked remotely during the past 18 months and want to  remain working remotely (often called tele-work in federal guidance).

Citations to published guidance from the United States EEOC, OSHA, and the CDC can be found at the end of this article.

Nothing  in this article is meant to be legal advice and is only meant to be  information. If you have questions about employment-related issues,  Social Security benefits, education, or health insurance, you can  contact the CF Legal Information Hotline and set up a time for a no-cost  confidential call by emailing CFLegal@sufianpassamano.com.

Question  No. 1: My employer says that a person with CF is not at risk of a bad  outcome from a COVID-19 infection and therefore I cannot continue to  work remotely.

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The CDC  has acknowledged in its COVID-19 guidance that people with CF (either  with or without solid organ transplant) are more likely to become  seriously ill if they contract COVID-19.(1)

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By  “seriously ill,” the CDC means that a person with CF (or one of the  other underlying conditions it identifies) has a greater likelihood of  needing hospitalization, intensive care, requiring ventilator-supported  breathing, or dying with COVID-19 compared to a person without health  issues.(1)

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Exposure  to COVID-19 in the workplace is also a serious concern. The CDC and  public health authorities have found that COVID-19 is highly contagious,  potentially fatal, and spreads readily in the community. Based on these  findings by the CDC, the U.S. Equal Employment Opportunity Commission  (EEOC) concluded that COVID-19 meets the definition of “direct threat”  because it presents a significant risk of substantial harm to the health  or safety of others in the workplace.(2)

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The U.S.  Department of Labor Occupational Safety and Health Administration  (OSHA) has also recognized that the SARS-CoV-2 virus (which causes  COVID-19) is a significant health and safety risk in the workplace to  workers with underlying conditions regardless of their vaccination  status.(3) OSHA affirms that workers with disabilities may be legally  entitled to reasonable accommodations that protect them from the risk of  contracting COVID-19.(3)

Question  No. 2: When does an employer have to provide a reasonable accommodation  under the Americans with Disabilities Act (ADA)?

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Under  the ADA, a covered employer must provide a reasonable accommodation to a  qualified individual with a disability when an accommodation is  necessary for the disabled employee to perform the essential functions  of the job, or otherwise receive equal terms and conditions of  employment. Reasonable accommodations are simply adjustments or  modifications in the manner or circumstances in which a job is usually  performed.(4)

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Remote  work is within the scope of potential reasonable accommodations, but  whether remote work should be permitted as a reasonable accommodation  depends on the circumstances of the individual employment situation. The  duty to provide the accommodation arises when it is requested, or when  the need is clearly apparent.(4)

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However,  employers covered by the ADA and Rehabilitation Act are not obligated  to provide an accommodation that is unduly burdensome.(5) “Undue burden”  is an objective standard based on the actual costs or administrative  burdens of providing the accommodation.

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Not all  employers are covered by the ADA or the Rehabilitation Act. The ADA  covers employers with 15 or more employees. The Rehabilitation Act has  similar protections for disabled employees, and the Rehabilitation Act  applies to employees who work for the federal government or who work for  an employer who receives financial assistance from the federal  government, often known as a federal contractor. There is no employee  minimum number.(4)

Question  No. 3: All employees at my workplace performed remote work for the past  12 months or more. My employer now says everyone must work in-person.  Can I remain working remotely?

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A person  with CF is a person with a disability under the ADA if they have a  physical impairment that substantially limits a major life activity.  Typically, a person with CF has a limitation in breathing or digestion.  As an individual with a disability, an employee with CF can request to  continue remote work as a reasonable accommodation even if an employer  has said that all employees must work in-person. Whether an employee can  work remotely as a reasonable accommodation depends on several factors.  The increased risk of serious illness from COVID-19 for people with CF  is a disability-related limitation that weighs in favor of allowing  remote work.

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However,  the need for the reasonable accommodation must be communicated to the  employer and every accommodation must be individually assessed. If an  employer recalls employees from remote work to in-person work, a worker  with CF will need to request remote work as an accommodation. The  employer must then engage in discussions with the employee to evaluate  whether a disability-related reason requires remote work. If there is a  disability-related reason, then remote work can be provided as a  reasonable accommodation.(4)

Question  No. 4: I asked my employer for a reasonable accommodation, but my  employer says that the ADA does not apply during a pandemic. Is my  employer correct?

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The  employer is not correct. Laws protecting the rights of workers including  the ADA have not been suspended and continue to apply during the  COVID-19 pandemic.

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The EEOC  specifically stated that the United States equal employment opportunity  laws, including the ADA and Rehabilitation Act, continue to apply  during the time of the COVID-19 pandemic, and they do not interfere with  or prevent employers from following the guidelines and suggestions made  by the CDC or state and local public health authorities about health  and safety precautions.(5)

Question  No. 5: My employer says that the COVID-19 pandemic is over, so I cannot  have a reasonable accommodation. Is my employer correct?

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The  pandemic has not ended. A covered employer’s obligation to provide a  reasonable accommodation to a qualified individual with a disability  exists without regard to whether a national emergency is declared. In  addition, the COVID-19 national emergency was extended on February 24,  2021, and remains in effect nationwide.

Question  No. 6: I do not have CF or another disability, but my adult child has  CF and lives with me. I work in a place that has many people from the  public visit each day. Is my employer obligated to provide me with the  reasonable accommodation of remote work to protect my adult child?

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An ADA  or Rehabilitation Act covered employer’s obligation to provide a  reasonable accommodation is owed to an employee with a disability. An  employer is not obligated to provide a reasonable accommodation to a  non-disabled employee, even if the accommodation is needed to protect  the health and safety of a disabled child or spouse.(5) However, an  employer may always voluntarily provide remote work to an employee even  without the legal obligation to do so.

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The ADA  and Rehabilitation Act protect non-disabled employees from  discrimination based on their association with a disabled person. If an  employer took an adverse employment action against a non-disabled  employee because that employee had a disabled child, spouse, or other  disabled associate, then the non-disabled employee may have a claim for  discrimination under the ADA or the Rehabilitation Act based on the  association with a disabled person.

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Question  No. 7: I have not disclosed my CF diagnosis to my employer. Is it true  that when I request a reasonable accommodation, I do not have to  disclose my condition to my employer and they cannot ask what health  condition causes my disability?

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It is  correct that an employer cannot make disability-based inquiries of a job  applicant if the employer is covered by the ADA or the Rehabilitation  Act of 1973. However, an employer may ask disability-related questions  and conduct medical exams after an offer of employment is made and  before work begins, as long all entering employees in the same job  category are subjected to the same questions or examination.(5)

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After an  employee begins work, an employer may make disability-related inquiries  and require medical examinations only if such questions and  examinations are job related and consistent with business necessity. Any  medical or disability-related information collected by an employer,  regardless of when it is obtained, must be treated confidentially and,  if retained, must be kept separately from other employment  information.(5)

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However,  when an employee requests a reasonable accommodation, an employer may  ask questions or request medical documentation to determine whether the  employee’s disability necessitates an accommodation. Obtaining this  information is permitted because it is necessary for the employer to  know the nature of the disability, how it affects performance of job  functions, and how the disability can be accommodated.(5)

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Typically,  a letter from the treating physician is good evidence. Sometimes  physician offices use a template letter that they use when a patient is  filing an application for Social Security Disability benefits, which  talks about all the complications CF may cause. This type of letter is  not effective when requesting an accommodation under the ADA. If a  letter makes it sound like the employee is too sick to do their job, the  employer may conclude that the employee is not an employee who is  protected by the ADA. The employee with CF should make sure the treating  physician’s letter provides information about why the person CAN  perform the essential function of the job with a reasonable  accommodation.

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The ADA  allows the employer to ask: (1) how the disability creates a limitation;  (2) how the requested accommodation will effectively address the  limitation; (3) whether another form of accommodation could be  effective; and (4) how a proposed accommodation will enable the employee  to continue performing the essential functions of the job.

If an  employer fails to provide a reasonable accommodation, the employee can  file a Charge of Discrimination with the EEOC within 300 days of the  date the discrimination occurred. The EEOC now allows employees to file a  Charge of Discrimination online at www.eeoc.gov.  Most states have state disability laws that provide a similar right to a  reasonable accommodation. A person can also file a charge of  discrimination with a state agency that will also investigate the charge  of discrimination but often the time limit to file a charge is 180  days. A person should check their state law to determine the time limit  for filing a complaint of disability related to disability  discrimination.

Endnotes:

(1). People with Certain Medical Conditions (CDC Update August 20, 2021). Https://www.cdc.gov/coronavirus/2019-ncov/need-extra-precautions/people-with-medical-conditions.html

(2). Pandemic Preparedness in the Workplace and the Americans with Disabilities Act, ¶ II.B.; 29 C.F.R. § 1630. https://www.eeoc.gov/laws/guidance/pandemic-preparedness-workplace-and-americans-disabilities-act

(3). Protecting Workers: Guidance on Mitigating and Preventing the Spread of COVID-19 in the Workplace. https://www.osha.gov/coronavirus/safework.

(4). Pandemic Preparedness in the Workplace and the Americans with Disabilities Act, § C. https://www.eeoc.gov/laws/guidance/pandemic-preparedness-workplace-and-americans-disabilities-act#23.

(5). What You Should Know About COVID-19 and the ADA, the Rehabilitation Act, and Other EEO Laws § D.13. https://www.eeoc.gov/wysk/what-you-should-know-about-covid-19-and-ada-rehabilitation-act-and-other-eeo-laws; and 29 C.F.R. pt. 1630 app. § 1630.2(o). s

Beth  Sufian is 55 years old and has CF. She is an attorney who focuses her  law practice on disability law and is the Treasurer of USACFA. Her  contact information is on page 2. You may contact her with your legal  questions about CF-related issues at CFLegal@sufianpassamano.com.

In  the corner of my house is a new prized possession. It is an imposing,  life-sized Japanese samurai with my mother’s Arima family crest on the  chest. The metal armor is intricately put together with leather and  fabric ties. The helmet and facemask are daunting and will likely ward  off an intruder who mistakenly thinks it is a person standing there.  This was a gift from my Uncle Juichi, who boasts of coming from samurai  blood.

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Armor  like this was worn for centuries when men went to battle. After several  trips to European museums, I was always in awe of the heavy, bulky steel  armor. I couldn’t imagine anyone moving in these outfits. And yet,  going into battle without armor meant almost certain death.

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Regardless  of whether we are into samurai or medieval armor, we all carry our own  armor to protect ourselves in this world. We wear this armor in our  battles and we choose what kind of armor we need for particular fights.  There is so much hurt and pain in life that we can be weighed down by it  all. Armor provides a barrier between us and that hurt. We would be too  naked and vulnerable without it. What types of spiritual and emotional  armor do you use? How much does your armor weigh you down?

When you  have cystic fibrosis, character armor “forms in response to chronically  stressed and traumatic events,” according to alternative medicine  practitioner, Ben Oofana. Along with our innate temperaments, how we  cope with CF and the stresses it imposes creates character defenses and  habitual patterns and attitudes. This armor can serve us and help us  adapt or it can become problematic.

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I’m  pondering common types of armor we might use to deal with cystic  fibrosis. Achievement can help us feel worthy and accomplished despite  being sick. Self-confidence helps us feel good about ourselves in spite  of, or because of, CF. Passivity, in the form of non-adherence or  resignation, protects us from the energy of fighting or the fear of  failure or self-blame if we try to face a challenge and lose. Hedonism  protects us from the harshness of life and helps us enjoy being alive no  matter how much time we have. Hypervigilance gives us safety to help us  feel we can catch illnesses or problems early, but it can also make us  stuck in fear and what-ifs. Boundaries protect us from dysfunctional  relationships that can deplete our energy and hurt us. Even caregiving  protects us from feelings of abandonment and gives us purpose and worth.  Stoicism can protect us from feeling. Sometimes we have to suppress and  inhibit our feelings to just get things done or keep our cool during a  medical procedure. Regulating our emotions is the heaviest armor we have  to carry. Numbing or avoidance protect us from pain and give us an  escape from unpleasant feelings. Vulnerability protects us from the  exhausting energy of stoicism; we release our sorrow and pain when we  cry alone or to a loved one, and we can acknowledge our suffering.  Sometimes unprocessed emotion can lead to tightness and tension,  creating a hardening around the body, much like the exoskeleton around a  lobster. This kind of emotional armor is not good for the body. Just  pause right now and take note of your physical sensations. Do you notice  any armor around your body?

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Real  armor is made of metal, the strongest material. Emotional and spiritual  armor has to be made of a strong material: control. Control helps us  with fear and anxiety, gives us some predictability, and gives us power.  It builds our confidence and stretches our potential. Yet this kind of  armor can backfire if it turns into rigidity, micromanaging, and  criticism. In fact, judgment is a type of control armor that protects  the ego by making us seem better than the other. With CF, controlling  our healthcare demands can be empowering, yet it can lead to  over-identifying with illness. Control is maladaptive when it is fueled  by excessive fear (or power). I think the pandemic gives us ample  evidence of that.

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Faith is  a significant type of armor as well; one where we put trust in the  general of the army fighting the battle we are in. Faith means believing  we are protected by a higher force, and that that force has our best  interest and survival in mind. Reciting scripture that inspires and  uplifts us serves as protection from pain for some. Spiritual armor  helps us feel we are on the right side of the perceived battle between  good and evil and life and death. In some cases, spiritual armor can  backfire if extreme views make it harder to empathize with others’  perspectives.

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In CF,  faith as armor can be life sustaining. It might manifest itself in a  belief that you can become a parent or will reach old age or that a  modifier drug will come out for your mutation. Believing in life after  death is armor to reduce our fear of death and diminish our grief over  separation from our departed loved ones. Spiritual armor also means  wearing things that will bolster your wellbeing, like self-compassion,  self-care behaviors, mindfulness, or affirmations. No matter what  happens to our physical bodies, our spiritual armor can still lead to a  happy life. I think that’s what Claire Wineland, CF advocate, meant by,  “We teach sick people that when they are sick, somehow, someway, they  cannot be as happy as normal, healthy people. We teach them that their  happiness, their contentment in life, their joy in life, is tied to how  healthy they are. We can be happy.”

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As I  said before, armor can be heavy and bulky. In life we need to be able to  move freely and nimbly. Can we be stealthy in armor? We have to learn  how to wear it right and how to move in a certain way. We have to shed  the pieces that weigh us down and impede us. Then we can face our  battles with the greatest chance of winning. This is our great lesson—to  be able to carry all the types of character armor we possess that  promote our freedom, inner peace, and harmony with others. This is our  true protection, and our most prized possession.

Isa Stenzel Byrnes is 49 years old and has CF. She lives in Redwood City, California. She is 17 years post-lung transplant.

My life’s most important lesson about caregiving began with a spoon—or more accurately, with the lack of one.

Many  years ago, before my wife and I were married, we commuted back and forth  between our respective apartments in Tampa and Tallahassee. When I  would come back to Tampa after being away for a few weeks, I would often  find small things changed in our home: new art on the walls, new  records or books on the shelves, new food in the cabinets and  refrigerator. That weekend though, I felt more surprised by what I did  not find: a single spoon in the entire place. I was making tea in the  small kitchen space, looking for something to stir in the sweetener I  had added.

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I felt  baffled by this. I also felt certain that the complete absence of spoons  in the apartment had a story behind it. So, I walked over to J where  she sat on the couch and said, “You know, I could have sworn we had some  spoons last time I was here.” My wife immediately looked sheepish. She  cast a sidelong look at the floor and explained, “I had to get rid of  them. They were mocking me.”

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I should  back up a bit. Just as I live with chronic physical health issues  because of my CF, my wife lives with chronic mental health conditions.  One of the specific things J experiences is depersonalization, a process  in which people simultaneously become alienated from their own sense of  self and project that sense of self onto inanimate objects.  Depersonalization often stems from early life trauma, such as abuse  experienced in childhood. It can become less profound over time as  people experience healing and feelings of safety with those they love.  This has happened for my wife, who these days will say with a grin that  it has been a while since any cutlery spoke to her. But at the time, the  spoons were a threat and had to be eliminated.

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I could  have said many things in that moment. Only one seemed appropriate,  though. After all, it did not matter whether I thought the talking  spoons were “real.” All that mattered at the time was the fact that J  was authentically experiencing hostile communication from the spoons and  took action to improve her own quality of life by removing them from  the premises. My wife discarding the spoons seemed quite reasonable to  me given how they were tormenting her. So I looked thoughtfully at J,  then down at my cup, and then back at her. I nodded and said “okay.” And  then I went back to the kitchen and stirred my tea with a fork.

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That  day, as on so many others, I was in the Matrix. I find the Wachowski  sisters’ iconic film as good an allegory as any for the process of  understanding and negotiating the socially (and sometimes  technologically) constructed nature of reality. On this occasion, there  quite literally was no spoon. But in a sense, all things in our worlds  are spoons—because our ability to interact meaningfully with them hinges  on us not only constructing reality, but also sharing that reality with  others. It thus seemed unsurprising to me as I progressed in my own  graduate studies, and later in my work as a faculty member, that  research clearly demonstrates acceptance of another person’s reality as  one of the most powerful forms of caregiving.

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And  paradoxically—to reference another theory of “spoons” coined by fellow  chronic illness patient Christine Miserandino—allowing ourselves to  embrace with open arms the lack of spoons in a concrete sense can often  increase the “spoons” we have in an abstract one. In Miserandino’s Spoon  Theory model, “spoons” represent the internal resources we bring to  each day in living with our respective chronic conditions. These  resources can be cognitive, emotional, social, etc. The amount and  diversity of “spoons” with which we start each day shape how that day  will go—and how exhausted we will become in trying to get through it.

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My  entire philosophy of caregiving thus begins and ends with the fact that I  do not ever want to take spoons away from my wife in an abstract sense.  And if that means she must sometimes take spoons away from me in a  literal one, so be it.

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I have  been thinking a lot about spoons lately. I am just now recovering from a  positively nasty bacterial sinus infection in which some 100+ teaspoons  of neon orange muck emerged from my face. And yes, I measured! If I am  to produce such ghastly secretions, I can at least find solace in  quantifying them for later discussion with my clinic team. Collecting  data increases my own “spoons” by distracting me from the physical pain  of an acute infection. I have had people tell me that I am too  data-driven, too clinical, too analytical in how I cope with my CF from  day to day. I have told most of those people to stop talking and start  listening, because caregiving looks different from person to person.

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The care  I give to myself reflects the same things I value in the care I receive  from others—and the care I give to them in kind. I value that complete  openness to my own unique personality as someone navigating lifelong  chronic illness, and to the unique things I experience along the way. I  value observation and affirmation over inference and judgment. And being  a scientist, I also value the robust evidence basis for these  practices.

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Reality  sharing has long been uplifted in the dementia care literature as a  transformational practice for helping people with memory loss and their  loved ones enjoy time together and experience mutual joy. Rachael  Wonderlin, one of my favorite authors in this field, describes how  abandoning the paternalistic practice of attempting to convert others to  our own experiences of reality liberates ample time and energy for  sharing in the reality of our loved ones and making the most of quality  time.

I have  seen the value of this approach consistently in my own work as a medical  sociologist and public health program evaluator focused on health  equity in aging with chronic disease. For example, I have served for  several years as the qualitative methodologist for the ACTS 2 skill  building and support project centering Black dementia caregivers. The  model for all the services and resources we provide to family caregivers  focuses centrally on finding community and serenity with loved ones  with dementia. This includes sharing in loved ones’ realities from  moment to moment as caregivers also navigate their own realities with  appropriate support. I have also served for several years as the  evaluator for the REACH geriatrics workforce enhancement program at FSU.  We offer several different trainings and resources to help family  caregivers of all backgrounds and experiences live their best lives  while providing impactful support for their loved ones. Our courses and  materials consistently nurture skills and practices of reality sharing.

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And I  have likewise seen the value of reality sharing in how my wife cares for  me as someone living and aging with CF. Unlike many of the people I  have been close with in the past, J never expects me to perform health  or hope in particular ways. Rather, she wants to share in whatever I am  authentically experiencing on a given day—be it wellness or sickness,  hope or despair. Her ability to be present with me emotionally in a  struggle without either centering or sidelining herself has taught me  endless lessons about the meaning of love and caring. I often reflect  that love is a verb more so than a noun; our actions are ultimately what  communicate our affection for others.

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Living  with CF means spending ample time negotiating others’ expectations. We  are bombarded with copious and often conflicting expectations from the  people who care for us both formally and informally. We face complex  expectations about how we will care for ourselves on a daily basis—what  treatments we will choose, how we will monitor our health for signs of  change, what interventions we will open ourselves to in times of crisis,  how we will feel about all of it, and how we will behave as a result.  We often spend so much time trying to manage these expectations and the  feelings of others surrounding them that we can become lost to ourselves  in the process. CF is an all-consuming disease socially as well as  physically and mentally.

So,  reality sharing can be absolutely transformational in how we care for  ourselves and each other. Within ourselves, this can take the form of  intentionally cultivating acceptance about changes in our health and our  bodies. I often think about how my friend Dominic, who published some  of his art in the last issue of Roundtable, nurtures this acceptance by  exploring the full artistic potential of his post-transplant body. With  others, reality sharing likewise starts with acceptance. It means being  fully present in the moment with another person, accepting how the world  looks and feels to them. I did this with my wife when the spoons  disappeared, to give one example from a decade of shared history. And  she does this with me every single day, whether I am coughing out bloody  discharge and struggling to breathe or jumping up on her back and  laughing so hard my sides hurt.

It has  been years since the spoons disappeared from our Tampa apartment. Yet  just as we both enjoy revisiting that story with people who are new in  our lives, I continue to learn more from J every single day about what  effective caregiving looks like. I try to reflect that spirit of  openness and acceptance back to her in full, and to practice these  approaches in kind with others in my life, both within and beyond the CF  community. We often learn at young ages that “sharing is caring” but  leave behind these important lessons as we age. Indeed, sharing in the  reality of those we love rather than trying to impose our own is the  very essence of caring.

Dr.  Alexandra “Xan” Nowakowski is 37 years old and has CF. Xan is a  director of CF Roundtable, in addition to being a medical sociologist  and public health program evaluator. They currently serve as an  Assistant Professor in the Geriatrics and Behavioral Sciences and Social  Medicine departments at Florida State University College of Medicine.  They also founded the Write Where It Hurts project (www.writewhereithurts.net)  on scholarship engaging lessons from lived experience of illness and  trauma with their spouse, Dr. J Sumerau. You can find their contact  information on page 2.

Admittedly,  I do not have a lot of experience as a caregiver. I am not yet a parent  and my parents are not yet at the point of needing care. Like many  individuals with CF, most of my experience with caregiving has been on  the receiving end. I will not pretend as if I have anything to offer in  the way of advice to anyone else who is currently in a caregiving role.  God bless you if you are. What I will do, however, is take this  opportunity to share some insights into my own shortcomings and lessons  learned from my experience in the trenches of severe lung disease and  end-stage CF pre-Trikafta.

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The  first thing you should know is that it wasn’t pretty. In fact, most of  the time it was downright ugly. My health declined fast and furiously,  and none of us was ready for what was to come. My mom became my fulltime  caregiver when I was no longer capable of taking care of myself. There  was an odd tension between us before I acquiesced to the fact that, yes,  I needed help. I’m sure pride and stubbornness played a role, along  with a healthy dose of denial. My mother hesitated taking on certain  responsibilities of mine after so much of my independence had already  been ceded as a result of my ailing health.

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The  following months, during which I found myself in need of a significant  amount of caregiving, were some of the most challenging times I have  ever been through—I had never mentally prepared for finding myself in a  place where I was utterly reliant on others in order to fulfill many of  my most basic needs.

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If I could go back and give myself some advice, it would be this:

• Take  it easy. Take it easy on yourself—what you are going through is hard.  Take it easy on your mom, too—what she is going through is even harder.

• Your  suffering affects everyone else around you, even though you may not  realize it at the time. It is easy to take the myopic view that you are  the only one going through this. Don’t allow yourself to be deceived by  this false narrative.

• You  are fortunate to have someone who loves you unconditionally and is  willing to put up with you on your worst days. Not everyone is so  fortunate. Be grateful, and don’t take this for granted.

• No one  expects to lose their independence and functional ability at such a  young age, and, when you do, you won’t be prepared. Don’t be afraid to  ask for help.

• This  process is full of difficult emotions and experiences, many of which  will remain unspoken or unacknowledged. Don’t keep them all to yourself.  Life is better and richer when shared.

• There will be days when you feel like giving up. Don’t.

• Be  adaptable. Just as your caregiver will learn to adapt to your changing  needs, you must be cognizant of the needs of your caregiver. We are all  human.

• Find  some meaning and purpose if you can. Reaching a point in life where you  are no longer able to act of your own accord and volition requires that  something fill this void. Try not to let it be bitterness and anger.  Living for something outside of yourself is the best solution.

• Try  not to live in your head so much. Living from the heart is the best way  forward. A rigid intellect is an unwelcome companion when trying to  rationalize and justify questions of the existential variety.

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You hear  the word dignity thrown around a lot when it comes to end-of-life care  or caregiving. What I can tell you, having watched people reach the end  of their lives, is there is absolutely no dignity to be found in facing  the stark realization that everything they once identified with or  prided themselves in their ability to do is about to be lost. The best  you can hope for is grace. The type of quiet and humble strength that is  required to surrender totally and fully to love. Love in the form of  someone else showing up and being there for you when your body no longer  shows up for you. When the pain of daily living is too heavy a burden  to bear and you question why it is that you are still here, grace is the  answer.

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My  mother and other family members who took care of me when I fell to my  lowest state are the epitome of grace. Although I did not arrive at this  point in my life with any preparation for how to handle it, my mother  sure did. She was born for it. I made a lot of mistakes my first go  round, and I hope that I don’t next time. Caregiving is hard on  everyone. I can only hope that one day I will have the opportunity to  return the favor in a meaningful way.

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For me,  being on the receiving end of caregiving has opened my eyes—we are all  just doing the best we can. I am not sure anyone is ever quite ready for  a truly demanding caregiving relationship, but if we can approach the  experience with open minds and open hearts, maybe in some small way we  can alleviate the collective weight of human suffering.

After all, the truth of the matter is that, in the words of Ram Dass, “[w]e’re all just walking each other home.”

Dave  Tarnow is 32 years old and has CF. He lives in Erie, Colorado. Dave is  the founder of “Dave’s Cycle for a Cure,” which inspired the national  Cystic Fibrosis Foundation event now known as “CF Cycle for Life.” You  may contact him at dtarnow@usacfa.org.

Life  is so hard as an adult. I miss the days when I got lollipops and  stickers when I went to the doctor or I got a prize if I didn’t (and  even if I did) cry during a blood draw. What happened to celebrating our  life with CF like the good ole days? That may be the pick-me-up I’m  craving.

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I think  we could all benefit from going back to easier days. In reality, those  days were never easy, really, but snacks make all the things seem  easier. It’s less daunting going to multiple doctor appointments,  suffering through procedures, and having tests done when there’s a  reward at the end.

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Maybe it  does mirror Pavlov’s dog a bit; I don’t see anything wrong with that,  though. We could all benefit from a little classical conditioning—after  all, our paycheck is the reward that gets us out of bed in the morning  to go to work, and breathing clearly is the impetus for doing our  breathing treatments. And the absolute bliss of being on a beach or a  mountaintop is what gets us out of bed at 4:00 a.m. to leave for a trip,  whether via car or airplane.

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As a  therapist, I believe we all need to find our individual motivator.  Without it, it can be challenging to show up for the things we have  to—the things we don’t really have a choice in doing. And why wouldn’t  we make difficult matters more fun? It only makes sense given the amount  of mutilation our bodies endure.

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I am  sure I am not alone within the CF or transplant communities in thinking  of food as the only motivator for going through the many bodily  injustices we have to endure. We should have something to look forward  to that will get us through the next thing we have to do. Since the  majority of us have rocking metabolisms (or just terrible digestion), we  have the glorious reward of being able to eat just about anything we  want. In this instance, it’s the epitome of Pavlov’s dog.

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At my  pediatric hospital, they gave out bags to “get me through” the hospital  stays. I miss that, too. I lived for those bags. I got my first (and  only) iPod mini, beach towel (that I still use), and tons of games,  candy, books, and more. It was like hitting the hospital lottery every  inpatient stay (thank you Donna Crandall Foundation)!

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I can’t  deny that growing up has been hard. Granted, it’s a privilege to be in  my 30s writing this as a person with CF who has also survived a  double-lung transplant. I’m not negating that fact. But why did  everything change once we got older? It feels like it no longer matters  that we need to be seen and validated just as much as when we were  younger. We probably would benefit from that level of validation now  more than ever.

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We CFers  are living longer now, so let’s change the adult culture! We don’t  become numb to life’s assaults just because we grow up. Why, therefore,  can’t we have rewards just as we did when we were children? In fact, I  would suggest we need more comfort, more awards, and more high-fives for  still being alive! It’s a huge feat.

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As we  know, clinic isn’t always a happy place to attend. For me, this was  especially true prior to my transplant. Having that pit of dread in my  belly, otherwise known as anxiety, would hit me as I rode up the  elevator. Usually it was a “you should really come into the hospital”  talk that left a sour taste in my mouth. And mostly the disruption of my  life that threw me into the cycle of anger, tears, and acceptance. But  none of it was accompanied by a lollipop, which was drastically  detrimental to my well-being.

Nowadays,  clinic is a breeze with my new lungs. Gosh I never thought I’d say  that. And, although I don’t get rewarded, the cafeteria’s coffee is  definitely my motivator… as well as the homemade chicken salad  sandwiches on gluten-free rolls.

Who knew  I’d be living so fully with little inhibition? Cruising into clinic  devoid of oxygen tanks and heavy breathing is a miracle just as much as  it is to leave clinic with a smile on my face. Maybe that’s the sweet  difference.

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Don’t  underestimate the gravity of a sweet treat (unless you’re salty) in  stressful situations. It can be our greatest motivator, no matter how  temporary it is. But it’s worth it every time. And, hey, you only live  once (or twice, for us transplantees). s

Lara  Govendo, M.S.Ed. is 34 years old and has CF. She lives in Vermont as a  wild adventure enthusiast who holds a Master’s Degree in Mental Health  Counseling. She currently works as a mental health counselor for middle  schoolers. She also writes about living life beyond chronic illness and  develops educational programs to restore hope to those in need. Thanks  to her double-lung transplant in 2017, you can now find Lara traveling  on the regular, exploring the glorious outdoors, and belly laughing with  her loves. You can find her online at www.laragovendo.com (and on Facebook and Instagram) at “Lungs4Lovey.” You can email her at lgovendo@usacfa.org.

Reader,  you are in for a treat. I (Jeanie) had the pleasure of interviewing  Sonya Haggett, who is 46 years young and five years post-double-lung  transplant. She lives in Oakland, California, and is a licensed clinical  social worker. I first met Sonya six years ago at the CFRI Adult  Retreat, which she attended while requiring oxygen 24/7 and awaiting new  lungs. She was well loved and appreciated by others and her insightful  nature, openness, and wit during the group rap sessions led to more  profound discussions. When I saw her again a few years later, she was  free of the oxygen tether, having received her transplant, and was  radiant. Since then, Sonya has contributed to many organizations, like  CFReSHC and CFRI, with her newfound energy, passing along her  experiences of dealing with the issues surrounding transplant, women’s  health and CF overall. Please welcome, Sonya, our newest star.  Spotlight, please!

What was your CF like prior to your lung transplant?

Being  extremely out of breath and fatigued. I asked my doctor why I had  recently started coughing so much in the shower, and she explained it  was because I was desaturating. In retrospect, I had been living without  getting enough oxygen for about a year before starting supplemental  oxygen, but only while sleeping, and about one and a half years before  requiring supplemental oxygen 24/7. A lot of time was spent in the  hospital on IV antibiotics between 2012 and 2016. On December 10, 2014, I  was admitted for three weeks for an exacerbation—I celebrated my 40th  birthday, Christmas, and the New Year in the hospital.

What influenced your decision to go ahead with the transplant?

Transplant  was my best option to stay alive. Watching peers with CF on the path to  transplant and getting to know them and their stories over the years at  the CF retreat influenced me. I also cannot overstate the importance of  the long and trusted relationship I had with my CF center and hospital.  I cannot imagine going through the process without that level of trust  and feeling the medical professionals were invested and working hard to  keep me healthy and alive. Through hospitalizations I got to spend time  with and know the pulmonologist on the transplant team and see the  crossover between the transplant and CF teams at my hospital.

How long did you wait for new lungs and what was most challenging?

I was on  the transplant list for two years and one month. It was challenging:  although emotionally healthy, I had to energetically let go of so much  because I needed all of my mental and physical energy focused on staying  alive with peace of mind. I had to have faith things were going to work  out for the best and let go of fear and doubt as much as I possibly  could. It has been an incredible lesson I have tried to continue living.

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I also  learned the importance of leaning on others. For years, my coping  mechanism was striving to live an independent life and working to meet  life goals similar to peers without chronic illness. End-stage CF  brought me to my knees, teaching me I cannot cling to delusions of  independence and normalcy; it is not a healthy way to live. My family  and friends and larger community showed up for me in ways I could never  have imagined. Now that I am healthier and not so dependent on others, I  find myself getting back into the independent mindset and have to work  on keeping interdependence in mind.

What could you do after lung transplant that you couldn’t do before?

Travel,  day trips, going to the beach, dancing, and enjoying exercise and yoga  again. Travel highlights include going to New Mexico to meet a fellow  individual with CF who also was a lung transplant recipient; visiting  Italy and Thailand; as well as seeing Ireland with two of my nieces.  Spending good, quality time with my nieces and nephews is a tremendously  important part of my second chance at life. It is also important to  mention I can now complete life’s mundane tasks, such as showering and  taking out trash, independently. I oddly feel a small joy taking trash  out of my apartment and down the stairs to the outside garbage. My body  feels light and at relative ease as this task always takes my mind to a  state of gratitude.

Is there anything you could do before that you can’t do now?

Healthcare  decisions feel like a collaboration with my health professionals more  so than life before transplant. They orchestrated and directly connected  to me to my second chance at life and I feel accountable. In the past, I  knew my body extremely well and felt like the expert; however,  post-transplant I am no longer the expert.

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I miss eating raw oysters.

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Perhaps  it is age, but I really feel I cannot push my body as much as I have in  the past to meet a deadline, finish a project, or hang out enjoying  myself. My body requires sleep and rest.

Can you take CFTR modulators?

I took  Trikafta for six months. My ENT proposed it to treat my sinus disease.  My eyes teared up instantly. I had so many strong emotions and was  hopeful that the latest modulator would help my sinus disease—clearing  them of the Pseudomonas infection they have cultured for years—and  ultimately keep my transplanted lungs as healthy as possible. Both my  transplant and CF teams were in agreement that Trikafta was safe and  held promise. Within the first week of taking Trikafta, I felt air in my  sinuses like I never had. Not to mention all of the Pseudomonas  infection I saw swirl down the drain with my daily sinus rinses. Graphic  and unpleasant, I know, but such is life with CF. Then came the  disappearance of the ubiquitous CF throat clearing.

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I also  noticed a pattern of waking earlier in the morning. I was averaging five  to six hours of sleep a night despite feeling exhausted. Anxiety and  extreme negative thinking were at a feverish pitch during this first  month. I was trying to “tough it out” in hopes the difficult side  effects would subside as my body acclimated to the profound changes  spurred by Trikafta. At the end of the first month, I started  experiencing worsening constipation which proved to be too much to  manage. At day 30, already on a full dose of Trikafta, I was desperate  for relief and followed my doctor’s recommendation to lower the dose to  one orange pill daily, rather than taking both the orange and blue  pills.

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Sleep  deprivation, extreme negative thinking, and anxiety decreased  significantly once the dose was lowered. My sinuses felt less clear,  though, and the throat clearing returned. I felt disappointed and sad to  have lost all the improvements to my sinuses I had gained over the  first month. Over four months ago, I stopped taking Trikafta and I still  have GI issues, which are worse than before I started.

What benefits have you had from counseling?

I am  fortunate enough to have weekly, long-term therapy. It has served as a  place to process my hopes and fears, formulate goals for my life and my  health, and peel back the layers of the onion to focus in on what is  truly important and energetically worthy. There is no way I could have  done this work with my friends and family. I need an unbiased third  party.

Why did you become a social worker?

My  decision to go into social work was influenced by my time spent in  support groups at CF Adult Retreat and how helpful that process was to  me in my CF journey. So many ideas of how to take care of one’s health  and pursue life goals were sparked in these settings.

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It also  stemmed from recognizing the importance of self-advocacy in getting  medical care. Some people need support in developing self-advocacy  skills or an empathetic party advocating on their behalf.

How did retreats with other CF adults impact your life?

My peers  with CF are the largest influence on my life and decision to get a  transplant. I watched friends in varying stages of the disease  process—before and after lung transplant—over the years at the CF Adult  Retreat. Seeing and getting to know others who were going through the  same pre-transplant struggles was scary. At a certain point I knew I was  headed toward the transplant journey. Some seemed to live and soar  post-transplant as they recounted the year since being together in  person. People also talked about their imminent death related to  complications post-lung transplant and deeply understood I would not see  them next retreat. These are opposite ends of the post-transplant  experience spectrum. So many nuances to the new purpose of life  post-transplant along with the changed definition of good health and  general feeling of satisfaction lie in-between. Time spent in support  groups and informal settings at CF Adult Retreat—the empathizing and  learning from my peers—is precious, sacred, invaluable, and helped me to  feel more prepared as I became a patient with my lung transplant team.

What have you been doing to cope during the COVID-19 pandemic?

The CF  community has been more of a support than ever amidst the pandemic  shutdown. The majority of my time spent on Zoom has been with my  community participating in educational, emotionally supportive, and  exercise programming provided by CF-related organizations.

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There is  an inherent connection among my peers through the deep understanding of  our need to follow guidelines set forth for people with CF and/or  transplants to stay healthy. Developments in medical science have  allowed me to live this long and now is not the time for me to stop  following the guidance of medical professionals who care for me.

Peers  who continue to believe in medical science validate the measures I take  to decrease COVID-19 risk. The counter messages—those who don’t believe  in the science behind vaccines, masking, and taking contact  precautions—wear on me. It has been difficult to stay the path of best  practices to not catch COVID-19 as healthy family members, peers, and  the community around me return to something resembling life before March  2020.

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Friends with transplants who are immunocompromised like me have been supportive in a special way, making me feel less isolated.

What is your motto or favorite saying?

Make a plan and God laughs. Nothing is perfect but everything is ok. s

Jeanie  Hanley is 59 and has CF. She is a Director and the past President of  USACFA. Andrea Eisenman is 56 and has CF. She is a Director of USACFA  and is both the Webmaster and Executive Editor of CF Roundtable. Their  contact information is on page 2.

If you would like to be interviewed for “In The Spotlight,” please contact either Andrea or Jeanie.

When  I saw two lines on the pregnancy test, I knew I was starting a journey  that was going to change my life. I always imagined that moment to be  one filled with unbridled joy. However, in reality, I was as scared as I  was happy. I deeply wanted to raise children and have a family to call  my own. I also didn’t know how exactly I was going to do that well while  also managing my CF.

I’m  grateful to say that, since that day six years ago, I’ve figured some of  that out. My son Simon, who is five, helped me learn many lessons along  the way. He, along with my ten-month-old daughter Eloise and husband,  Christian, together with our dog, Teddy, and cat, Rosa, make up my  village of supporters. Among them are also my CF care team, our family,  and our friends. The many recent medical advances in CF care have made  this possible for me, too.

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We have  an active, hopefully intentional household and one in which I’m the  manager and primary caregiver. I also work part time as a Community  Support Specialist for the Cystic Fibrosis Foundation. Two kids, two  animals, my husband, my job, and CF are a lot to balance. And as hard as  I work to maintain a healthy balance of all those things, I don’t  always get it right.

But I do  know I have come a long way from where I started and I am happy to  share a few lessons I’ve learned about caregiving and balancing CF.

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• Self-care plan:

Being a  caregiver for others means I first need to care for myself. When I was  in graduate school for my master’s degree in social work, every semester  we had to develop a self-care plan. At first, these exercises felt  redundant. However, as the semesters went on, I began routinely checking  in with myself so much that it became a habit. I would review what I  was doing to take care of myself and maintain my boundaries.

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These  plans had to include specific actions, a timeline for completing them,  and a person to hold us accountable. Through that experience, I learned  to hold myself accountable and to routinely check in with myself. When I  am feeling off, I know there is an unmet need. These self-care plans  have also been useful to me as a caregiver. When my child is having a  rough day, it’s not because they want to make my life miserable, it’s  because they have an unmet need.

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• Therapy:

My  therapist is at the foundation of my self-care. She supports me through  living with CF and it is something we talk about often. I’m able to  bring my thoughts and worries to her in a safe space where I know I am  supported and can work toward healing. I think it’s important to  normalize this as an aspect of our CF care. Parts of living with CF can  be traumatic. It has been wonderful to have more tools in my toolkit to  care for my mental health with therapy.

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• My people:

Another  key piece of balancing caregiving with CF is knowing the people whom I  can rely on in my life. This includes my close friends and family, as  well as my CF friends and coworkers, with whom I communicate in a  virtual capacity. Some days my spouse knows it’s just a tough day for me  and comes home for lunch or takes an item off my ever-growing to-do  list. Some days it means going for a walk with a friend when our kids  are restless. We live about six hours from our family so I don’t have as  much physical help as I’d like to, but it makes the other pieces that  much more important. It means communicating with my spouse about what I  need—accepting and humbly asking for help and/or saying no when I need  to. I cannot and should not do it all, even if I didn’t have CF.  Empowering others is a gift and a learning experience for everyone.

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Particularly  important in this group of people are my CF friends. Their presence in  my life is critical because they understand what it’s like to wake up  and not feel great, to calculate risks in the pandemic, to be working  hard to take care of your body, and the daily ins and outs of CF. Having  their support as I am a caregiver helps normalize my particular needs  and unique situation. They help remind me to take care of my  needs—physically, mentally, and emotionally.

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• Exercise:

Exercise  has been critical for me this past year and a half, during which I have  been living through the pandemic, working remotely, and experiencing  pregnancy and postpartum. Until I used Beam, which is an exercise  platform for people with CF, I never worked out as consistently. I  especially like the live classes because accountability is important to  me. Most days, Simon gets his tin cans and joins in on the classes with  me. He, too, has made fast friends with the people who attend the  classes and shares his love for dinosaurs with them willingly. I tried  out Beam for physical and mental wellness, but it was the community of  people that was a gift (to both me and my kids!) and kept us coming  back.

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• Routine:

On a  very practical level, routines are very helpful and they ground us in  our daily life. Routine does not need to be soul sucking. I find it  gives me freedom to just have those anchors in my day to care for my  needs (like sleep, meals, exercise, meds, treatments, insulin, etc.) and  the needs of others in my life. My days now revolve around meals,  Simon’s school drop-off and pick-up times, and Eloise’s nap times. I fit  what I can for myself around those anchors in the day.

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• Involving my kids:

Another  important piece of my caregiving experience is involving my kids in my  care. They will always know I have CF, and I strive to be open and  honest (as is appropriate for their age and understanding.) Simon is  quite skilled at giving me insulin shots, helping me organize my  medicine, and he is sensitive and compassionate with my needs, just as I  hope he will be with others throughout his life.

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• Practicing gratitude:

The act  of caregiving is not something I knew I would get the chance to do. So  many people have cared for me in my life and now I have the chance to  show up for my family tenderly and consistently. That means a lot to me,  and it fills me up with joy and gratitude. It is tempting to be afraid  because CF could take that away.

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I  remember when Simon was born a little over five years ago, when we came  home from the hospital I remember very distinctly wondering “how long  will I be here? What will I get to see?” and I decided then to make the  best of the days I do get.

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This  quote by Mary Oliver from a poem called “Don’t Hesitate” speaks to my  heart in this season of life that requires more intense caregiving for  my children. Mary Oliver says at the end of this poem that “joy is not  meant to be a crumb.” This speaks to me as a person who has CF and is  also a caregiver. It reminds me to be present in the moments of joy I  get to experience and not let these moments be diminished by my worries  for the future.

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My kids  show me best about the very present and tangible joy that can be found  in any moment. That joy is meant to be immense! Caring for them gives me  a front-row seat to their contagious wonder and awe.

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Some  days when my body is feeling more CF symptoms, it is harder to see the  joy. I try to accept my body as it is that day and remember it is still  good. Other days, CF is a lens that amplifies the joy in my life. Each  night, I tuck in Simon and Eloise and tell them that I am so glad to be  their mom. Every day, I wake up to the fact that it’s a rare gift to be  alive and care for my family. s

Gillian  Mocek is 30 years old and has CF. She lives in Evansville, IN. Gillian  enjoys trying new recipes in the kitchen, hiking with her family, and  fueling her caregiving tasks with walks to the local coffee shop. You  can email her at gillian.mocek@gmail.com.

One  of my dad’s mottos, and there were many (eyeroll emoji), was: take care  of number one first! Which he had no problem doing. I always felt this  was very selfish of him, and, if I were to adopt this behavior, I would  also be selfish. I never wanted to be like my dad. But, in some ways, he  was right. I cannot take care of others if I am not well or on top of  my own treatments. But I think there are gradations in this motto—it’s  not all black or white. I eventually came to see that making sure I am  well before I offer to care for or help others has been useful. I  learned the hard way—as is mostly the case—by burning myself out for  others or for work and then winding up in the hospital with pneumonia  (pre-transplant). I am now better at this balancing act.

When I  was younger, in my 20s, and my health was starting to decline with  frequent IV antibiotics and hospitalizations, my dad constantly accused  me of “not taking care of number one!” I later realized this was born  out of frustration about my disease rather than about what I was doing,  or supposedly not doing. That said, he was not wrong. I was mainly  prioritizing fun or socializing over treatments as I wanted to be like  my peers.

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If I  used a scale to balance my care with the care of others, I would say I  usually lost that bargain. But things have changed in my favor in the  last couple of decades. While in the past I tried to prioritize my  health first, there were times where I sacrificed it in order to take  care of my mom or my dog or my job (when I still worked). In retrospect,  it was good I didn’t have children because I would have chosen their  care over mine and probably would not have lived long enough to see them  grow to adulthood.

One of  the reasons I felt so strong about putting myself last might have had to  do with low self-esteem—everyone was more important than me. And most  likely, because my mom did so much for me, sacrificing so much time and  most of life to keep me alive, I felt this is what everyone did for  everyone else, right?

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But  learning how to balance my resources was important. I tried to balance  my care with that of others around me. I sought to be mindful of my  needs, which were many, and that of my friends, spouse, and elderly  parents. My parents are now all deceased. My mom and stepmom died about a  year apart and then my dad died eight months later. The losses were sad  and shocking, but it was also clear to me that my parents’ care was no  longer my obligation. Now I didn’t have an excuse to not fully take care  of my health. Sometimes what kept me so compliant in my own care was  worrying about who would care for my mom if I died. Toward the end of  her life, there were times when I needed to stay with her to ensure she  was being taken care of—going to doctor appointments with her, cooking  her food, etc. After she died, I no longer wanted to live. I had lost my  reason to care for myself (forget that I am married and had a Boston  terrier who was forever needing vet visits). It was a friend of mine who  rightly made me see that I had to keep on living for my mom’s sake. She  pointed out how upset my mom would be if I gave up and stopped caring  for myself. The real reality check for me was being diagnosed with  post-transplant lymphoproliferative disease (PTLD), a lymphoma that can  happen to people who are immune suppressed, post-transplant. It just  made me angry and want to keep on fighting to live.

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I’ve  realized I am more fatigued and my energy is limited. I am currently in a  clinical trial for the recurrence of PTLD. It has been extremely  tiring. Instead of expending my energy on shopping for food and cooking,  I realized I have a neighborhood friend who cooks for people for a  reasonable fee. She is a chef, so we both benefit! I enjoy and take  pride in my ability to transform my groceries into yummy delights, but I  knew I needed a break to save my energy for other things. I would never  have considered this option years ago. I might have ordered takeout  more frequently, but I tend to get sick to my stomach if I eat too much  restaurant food. I felt this was a good solution while I’m undergoing  treatment for my PTLD. I have also prioritized my exercise regimen over  that of cooking. I realized cooking took a lot of my time away from  things I needed to do each day like napping and exercising. Usually, I  had to choose one over the other. I still do cook a few things on the  weekends, which I can freeze for future meals. It is lovely to have  fresh food that just needs minimal heating up.

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While I  feel it has taken me all of my life to get to a place where I am uber  compliant in all of my treatments, I see people respect me for it. They  may think I am simply and sensibly spending hours a day on my health;  however, the real reason I follow all my doctors’ instructions is  because I do not want to second-guess myself when I am dying!

Did I do  enough? Could I have done more? And, at end-stage disease, it is too  late to change anything. I try to take responsibility for my care and  get creative with optimizing my health. I seek out helpful modalities  like acupuncture and Reiki or other forms of healing to complement my  western medicine regimen and to help maintain a healthy balance.

All this  prioritizing of my health has not stopped me from wanting to help  others. I still want to, and, when I feel I can, I put myself out there.  People in my life have been so supportive to me in my many times of  need, I do want to give back, just more mindfully this time, with my  father in mind, after I take care of number one.

Andrea  is 56 and has CF. She lives in New York, NY, with her husband Steve and  dogs, Willie and Roscoe. Andrea is the Executive Editor for USACFA. She  enjoys cooking new recipes, playing pickle ball, biking, tennis when  possible, and staying active as her health allows. Her contact  information is on page 2.

Throughout  my life, I’ve been a caregiver for both patients and families. When my  own family member has a serious and/or prolonged illness that requires  extended support, it can be the most daunting situation, emotionally and  physically. When one of my sisters with CF was hospitalized and  subsequently intubated for months, two different times, due to the flu,  and when my aging parents could no longer manage for themselves, I  learned quickly that, in order to advocate for them, I had to properly  care for myself.

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I needed  to prioritize and maximize my health to have the ability to medically  support others. In order to do that, it was important to keep other  family and/or friends (a/k/a the “village”) abreast of the medical needs  and enlist their support. This opened up extra time for my own CF  maintenance.

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I  realize I’m preaching to the choir here, but I found that our routine CF  therapies are easy to throw by the wayside when advocating daily for  someone else. For instance: missing one or more of what’s optimal on a  daily basis such as inhaled treatments, not getting enough rest, staying  hydrated, and eating! It’s also easy to skip on exercise, but I’ve  found that regular exercise, at least three days per week, is necessary.  I must also regularly keep appointments with my CF center and other  doctors. And I make sure to get my annual flu vaccine and other vaccines  as they are due—pneumococcal, shingles, etc. To have time for these  visits and vaccines, the “village” of caregivers helps considerably.

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Getting  vaccinated protects me and those whom I treasure from preventable  illnesses. Immunizations include the COVID-19 vaccine, which I received  as soon as I possibly could. Why did I get the COVID-19 vaccine series?  First, because it’s effective. (See Dr. Moss’s article on page 30 of  this issue for the science behind the vaccine.) Second, to protect  myself, you, anyone I look after (like my three-month-old granddaughter,  whose immune system is not fully developed), and for everyone I know  and don’t know with whom I may come in contact.

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The last  year without the COVID-19 vaccine was a tragic time for many families  who were unable to visit their loved ones in the hospital, in hospice,  or nursing homes. It was no different for me. Adding the risk of  COVID-19 infection to the mix caused such heartbreak during my father’s  last five months of life. I was unable to visit him face to face or give  one last hug. Today, it is only because of effective COVID-19 vaccines  that visiting restrictions can be relaxed, enabling a more peaceful end  of life with loved ones.

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I’ve  already received my yearly flu vaccine, which is part of my usual autumn  routine. Why? To boost my annual protection for myself, for you, for  those under my care, for those who are immunocompromised, and for  everyone I know and don’t know. Very importantly, it’s well known to be  effective at reducing symptoms. Even when the flu vaccine is mismatched  to the seasonal strain, there is significant protection against other  influenza strains contained within the vaccine (Belongia, Expert Review  of Vaccines 20171 ) which, similar to COVID-19 vaccines, prevents severe  disease and hospitalization.

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My  aforementioned sister skirted death two years in a row after catching  the flu during winter. She was unvaccinated both times. Each  hospitalization necessitated months-long intubation, tracheostomy, and  pulmonary rehabilitation. It’s been three and a half years since her  last ICU hospitalization and her memory loss has never recovered. It  took two near-fatal bouts of flu for her to be on board with all  immunizations including influenza and COVID-19. Don’t let that happen to  you.

After  maximizing my COVID-19 and seasonal flu protection, I will continue to  mask up as much as possible. Why? To protect myself, especially my  bronchiectatic lungs, and others by not bringing home the multitude of  other viruses out there that can wreak havoc. I used to get such strange  looks pre-pandemic whenever I wore a mask in public. I love how much  more commonplace masking has become recently. When I see others masked, I  feel a kinship with them and quietly give gratitude for their  considerate behavior of others while protecting themselves, too.

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Caregiving  is challenging for those of us with CF. It’s difficult to stay on top  of your health while urgent and emergent situations arise when  advocating for a loved one. But it’s even harder when priority isn’t  given to your health on a daily and long-term basis. As is announced on  planes: “please place the mask over your own mouth and nose before  assisting others.” In that same vein, ultimately, to be an effective  caregiver to others, you must first be a good caregiver to yourself.

Dr.  Jeanie Hanley is 59 years old and has CF. She is a director and former  President of USACFA. She is the founder of Planning Health, a patient  advocacy non-profit. Her contact information is on page 2.

The  past 18 months have been a journey. I turned 40 this year! It is such  an achievement being both a person with CF and a lung transplant  recipient of almost 11 years. I am so grateful to be alive even during a  worldwide pandemic. For the past four and a half years I have lived in  Melbourne, Australia. I immigrated here from the U.S. after meeting my  Aussie husband at the Transplant Games of America. I am waiting for my  dual citizenship to be processed as I write this. My life during the  pandemic has been so different than my life prior to the pandemic. The  biggest change is that I became a mother to my daughter, Zoe. Zoe was  born through surrogacy in August 2020, at the height of our most  restrictive lockdown.

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Life in  Melbourne, Australia, has been a rollercoaster for the past 18 months.  We have been locked down with the longest and harshest lockdown in the  world, for a total of 227 days and counting. We have national healthcare  and, to protect our system, we must protect the community health.  Australia has taken an elimination strategy to the virus. This means we  have kept the lid on the virus for a good part of the pandemic, until  now. We are once again in lockdown—our sixth. We have strict rules, with  only essential retail, such as pharmacies, groceries, and gas stations  staying open. Food is only for takeaway; no restaurants are open. We are  not allowed to visit with others at home or outside. Masks are required  inside and outside. We cannot go further than three miles from our  homes and we have a nightly curfew of 9 p.m. to 5 a.m. It is strict  here. If you break these rules you can get as much as a $5,000 fine.  Yet, even with these restrictions, our numbers of COVID-19 cases of the  Delta variant are continuing to grow. We have also been stuck in a very  slow vaccination rollout wrapped up in a bad PR disaster with the  AstraZeneca vaccine. We only had 4% of the population vaccinated when I  was able to receive my second dose on June 20, 2021. My experience with  both my first and second jab were virtually free of side effects—just a  sore arm and a little fatigue. As I write this, we have finally gotten  more people vaccinated and have 34% fully vaccinated. We finally have  more supply of the Pfizer vaccine and will soon be receiving Moderna as  an additional choice. Right now, we are finally having a big campaign  push for people 12 and up to be vaccinated. You see, we will continue to  stay in our extreme lockdowns until we have reached an 80% vaccination  rate. It is also important to note that, since the pandemic started, we  have had our international borders shut to the entire world. With the  travel ban we are not allowed to come and go. For myself, I am an  American who married an Aussie and my whole family is back home in the  States. I miss them so very much. I would do anything to see them again,  and for them to meet my daughter, Zoe. We hope that once we have 80% of  the population vaccinated, we will have some normalcy again and I will  finally reunite with my family.

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Living  through these lockdowns has been a bizarre introduction to the world for  our Zoe, and for us as parents. She doesn’t know a world without masks  and has never played with other children. She has kept me going through  all this. She is the reason we work so hard to stay healthy and well.  She is one of my reasons for being vaccinated. I hope that this world  will return to a normal place for her to grow up in and I hope that I am  able to survive this pandemic despite being immune suppressed. The  vaccine is my hope; it is all of our hopes. Minimizing the disease that  SARS-CoV-2 causes is the most important thing. Making this illness less  lethal and less contagious is so important.

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I look  forward to one day finally having my booster shot. As we are so slow in  our countrywide rollout, the authorities have not yet considered  approving the booster vaccination. I am just holding on for that third  shot and however many after that to keep me alive so I can live and  thrive alongside my darling daughter. As a one-year-old, she doesn’t  even have the option to be vaccinated, so we are even more careful to  make sure she stays well. The first year of a child’s life includes a  plethora of vaccinations. We just finished her 12-month shots, and we  feel proud as parents to be protecting her against all those crazy  viruses out there that we are so fortunate not to experience as a  society anymore. Until I became a parent, I didn’t even realize that  chicken pox in childhood had been virtually eradicated here*!

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I have  read and discussed the pros and cons about vaccinations with my family  and my healthcare providers. I have always been someone who happily will  take a vaccination to prevent illness. I think a quick needle over a  serious illness is a no-brainer. I love getting my flu shots. In fact,  here in Australia, we get two flu vaccinations, six weeks apart from  each other, for extra protection. To say that I was excited to get my  COVID-19 vaccine is an understatement. I was in the second group to be  allowed to get vaccinated here. I made my appointment as soon as I found  a place I could get the vaccine. We have struggled with shortages of  supply. I was scheduled back in April for an AstraZeneca jab (jab is the  word they love to use here for a shot). The day before my appointment  we were given disappointing news—the AstraZeneca vaccine was being  recalled for anyone under 60 because of the risk of blood clots. Until  further studies surfaced, it was all on hold. I was beyond disappointed.  Even with the approval of my transplant team, I had to wait. It was  then determined that if you were under 60 you could only receive the  Pfizer vaccination. Again, more waiting, as we didn’t have the supply.  Finally, on the day they had supply available, I was scheduled two weeks  out to get my first Pfizer jab. The day I got the jab, May 28, was also  my fourth wedding anniversary and the day we went back into lockdown  for the fourth time! On May 29, they finally opened up the rollout for  individuals 40+ to get vaccinated, so my husband went on the first day  and waited in line starting at 7 a.m. to get his jab. After about four  and half hours of waiting, he came home with his first shot completed.  Similar to me, he experienced just a sore arm and a little fatigue after  the jab. He did it for me and he did it for our baby Zoe. Our family is  vaccinated to keep us together as long as possible. The minute that a  COVID-19 vaccine is approved for our little girl, we will be signing her  up!

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I have  confidence in the vaccinations being given to the world right now. Why?  Because I trust medicine. I have put my life in the hands of doctors so  many times. I have seen the benefits of all the vaccines I have taken  and the ones I have watched my daughter receive. Prior to SARS-CoV-2,  the mRNA vaccine has been studied for outbreaks such as flu and rabies.  Once scientists had more information about the virus behind COVID-19,  they worked to develop an mRNA vaccine specific to SARS-CoV-2. The  science is there and I trust the science.

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Since  the pandemic began, I have received monthly updates from my transplant  team at the Alfred Hospital in Melbourne. I want to note that, in  Australia, we have only had one or two lung transplant patients who have  caught COVID-19, so we are relying on data from other countries. One of  the most reassuring things I have heard from my medical team is their  support of the COVID-19 vaccines. They reported to us that in a  government report from the U.K. using AstraZeneca and Pfizer with about  50% of the included patients receiving each vaccine, 40% of the  unvaccinated transplant patients died from COVID-19. Mortality with one  COVID-19 vaccination decreased to 10% and the death rate decreased to 8%  with two vaccinations. For me, this was a huge reassurance. I really  don’t want to die from COVID-19. I have much better things to do. So,  until we reach an 80% vaccination rate here and until I can get my  booster, I will just hang tight. I hope to see my family within the next  year. I hope to swim in a pool again. I hope to be able to go shopping  again. I hope to breathe fresh ocean air without a mask again. Until  then I will consider I have a jab well done!

*Editorial  Comment: It is only due to vaccinations that childhood chickenpox has  been dramatically reduced (nearly eliminated) as have been  hospitalizations and death (99.99% eradicated—only 1 in 100,000 reported  deaths in children last year) in the U.S. and other countries like  Australia. However, the same chickenpox virus still attacks 20% of  adults in the form of shingles; therefore, it has not been eliminated in  adults, nor will it be. Similarly, chickenpox has not been eliminated  in many developing countries where vaccination or infections have not  achieved herd immunity. s

Anna  Modlin is 40 years old and has CF. She is a double lung transplant  recipient of almost 11 years. Anna is an American who lives in  Melbourne, Australia, with her husband, Terry and one-year-old daughter,  Zoe. Pre-pandemic, Anna and Terry competed in ballroom and Latin  dancing together. Anna has always had a passion for swimming and  bringing Zoe to the pool is a highlight in life for her. Anna is  passionate about volunteering and helping where she can within the CF  and transplant communities.

What  a year and a half this has been! When the COVID-19 pandemic hit in  March 2020, I truly had the fear that this insidious virus might wipe  out a great percentage of our patient population. For those of us living  with cystic fibrosis, and those who have also had bilateral double lung  transplants, the future seemed very uncertain.

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As I  reflect back on those months of shelter-in-place, Instacart, lack of  toilet paper, and flour and yeast shortages, of all things, I must  congratulate our CF community on doing an excellent of job of caring for  ourselves. We have trained our entire lives in excellent hand hygiene,  social distancing (our six-feet-apart rule in CF group functions) and  mask wearing. Gratefully, these practices literally saved our extended  CF family. We have pivoted from in-person gatherings to virtual  platforms for CF meetings, retreats, and conferences with such  excellence. I am not sure if we will ever return to in-person functions.

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We have  been fortunate to have access to life-saving vaccines almost 18 months  into this pandemic. My transplant team encouraged patients to receive  the COVID-19 vaccine. I knew that being on immunosuppressant medications  to prevent the rejection of my transplanted organs might also impact my  immune system’s ability to create antibodies against COVID-19.  Interestingly, this most likely applies to other necessary routine  health maintenance vaccines such as the flu shot, shingles vaccine, etc.  There are limited studies with regards to these other vaccines that  have been done on solid organ recipients1.

I was  eager to get the vaccine to avoid experiencing the very serious  pulmonary complications and/or possible death as an outcome of COVID-19.  I have received the standard protocol of two Moderna shots and followed  the recommendation of my transplant team to get a third booster  vaccine. In an effort to get a better understanding of how the vaccine  is working in solid organ participants, I volunteered to participate in  the Johns Hopkins COVID-19 Vaccine Transplant Research Study. After  strictly following their post-vaccine lab draw schedule for the  SARS-CoV-2 antibodies, I am seeing firsthand that being on  immunosuppressant medications is inhibiting my immune system from  creating the necessary level of antibodies to be protected from  COVID-19. Further studies are underway studying solid organ recipients’  T-cells and memory cells, which, beyond just antibodies, are also  important defense mechanisms in our complex, multifaceted immune system.  With this research, scientists may find that solid organ recipients  have more protection in order to fight COVID-19 than the initial  antibody tests reveal.

Upon  receiving a negative antibodies result following my third vaccine, I  realized that I need to rethink this “new normal” and figure out a way  to live a full life with healthy and safe boundaries. In the CF and  transplant community, we all know how precious time is. There was no way  I was going to let a year or more pass by just existing. I was  optimistic for a miracle vaccine and to be able to return to life as  normal like everyone else. For now, this doesn’t seem to be the case. I  have found meaningful ways to stay engaged and connected with family,  friends, and my CF/post-transplant community through virtual  volunteering, Zoom meetups, developing new interests and hobbies, and  exercising! From the beginning of the COVID-19 pandemic, I made the  decision to mask up and continue to walk/hike outdoors while social  distancing with friends. The human connection is so important for our  overall wellbeing. Caring for our mental health is just as important as  caring for our physical health!

While we  wait for more information on how the COVID-19 vaccines ultimately  perform in solid organ recipients, I encourage others to get vaccinated.  The immune response system is exceedingly complex. Research is ongoing  and I am optimistic that there is a commitment, by the medical  establishment, to find a solution to protect our transplant community.

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In honor  of my donor and their gift of life, I feel a responsibility to take  care of these precious lungs. As my daughter so beautifully said to me,  “Mom, it is better to miss a few holidays, social gatherings, and travel  experiences now than to miss them all in the future.”

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(1) Hum Vaccin Immunother. 2018; 14(6): 1311–1322. Published online 2018 Mar 21. doi: 10.1080/21645515.2018.1445446

Elyse  Elconin Goldberg is 62 years old and has CF. She lives in Los Gatos,  California with her husband, Craig, and their puppy Teddy. She is the  mother of two grown children and one of her greatest post-transplant  joys has been becoming a grandmother! You can email her at eeglilac@msn.com.

As  of September 2, 2021, CF Centers reporting to the CFF Patient Registry  find 8,183 people with CF [PWCF] have received at least one COVID-19  vaccination. As about 30,000 PWCF are included in the Registry, this  would suggest less than 30% of PWCF have been vaccinated. But when one  considers that the PWCF Registry total also includes children <12  years not eligible for any COVID-19 vaccine, as well as underreporting  of actual vaccinations (almost all of which are not given at a CF  Center, but rather outside the CF care network, so Registry reporting is  gathered later and at least secondhand), it seems vaccination, as well  as public health measures of masks, social distancing, avoiding indoor  gatherings, and hand hygiene, have substantially contributed to the CF  community in the USA doing relatively well during the pandemic, with  only 14 deaths and 268 hospitalizations.

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How safe  and effective are COVID-19 vaccines in PWCF? Studies specific to CF are  not available, so we rely on data obtained in much larger populations.  Two of these are relevant—first, the general population (which  demographically skews older and therefore more susceptible to COVID-19  compared to CF) and second, the solid organ transplant population. Note  that studies in immunocompromised populations are probably not relevant  to non-transplant CF, as PWCF do not have an impaired adaptive (B & T  cell) immune system (i.e., ability to make functional antibodies and  develop cellular immunity), which is the body’s defense system activated  by vaccination.

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The  safety of COVID-19 vaccines has been established by intensive monitoring  starting with large clinical trials in spring 2020 and continuing in  real-world settings internationally, making these vaccines by far the  most extensively studied vaccines in history. As of this writing, over  5.4 billion doses have been given, with 2.16 billion people fully  vaccinated—nearly 30% of the entire human race. Perhaps the best study  of safety has come from Israel (Barda et al, NEJM 8/25/21), a nationwide  study of nearly 900,000 Pfizer vaccine recipients, who were compared  about six weeks after completing vaccination to matched unvaccinated  controls. Vaccine safety data were presented along with medical records  of roughly 200,000 cases of diagnosed COVID-19 compared to healthy  controls. Probably the most worrisome vaccine-adverse event, mild and  transient inflammation of the heart (myocarditis), occurred at 2.7 more  events per 100,000 than unvaccinated; importantly, this compared to  myocarditis occurring from COVID-19 itself in 11 more events per 100,000  than controls. So even the most concerning reported vaccine-adverse  event—which occurs very rarely—occurred far less after vaccination than  from catching COVID-19. Additionally, COVID-19 was associated with a  wide range of other serious complications not seen with vaccination.  This is but one of a large number of studies showing excellent vaccine  safety. The vaccines have also been found to be very safe in the very  old, in pregnant and breast-feeding women, and in people with many  chronic conditions, including immunocompromised individuals and  transplant recipients. Studies in children under 12 are ongoing with  results expected soon.

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When  evaluating efficacy, the worldwide sweep of the Delta variant has made a  difference. The Delta variant of SARS-CoV-2 is one of the most  infectious, easily transmitted viruses ever discovered. It is also, we  now know from a study in the UK, more virulent and inherently dangerous  than prior COVID-19 strains (Twohig et al, Lancet Infect Dis 8/27/21).  The three vaccines licensed under Emergency Use Authorization in the  USA—Pfizer, Moderna and J&J, with Pfizer now fully approved after  extensive safety analysis by the FDA—all continue to show superb  efficacy in protection against serious illness, hospitalization and  death, with only a slight fall from >95% to 85-90% in the new Delta  era.

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What  confuses many people is the more dramatic falling efficacy against any  infection as Delta has taken over. This endpoint includes asymptomatic  carriage of the virus as well as mild, cold-like symptoms and more  serious potential manifestations of infection. With this endpoint,  studies such as a recent large UK study comparing Delta to Alpha vaccine  protection (Lopez Bernal et al, NEJM 8/12/21) have shown that while  protection by an mRNA vaccine against any type of infection, including  asymptomatic carriage (positive PCR nasal test), has fallen from >90%  with earlier variants to perhaps ~50% with Delta, retention of  protection against any symptomatic infection remains very good (88%).  There is also some evidence that the mRNA vaccines may be more  protective against Delta infection than other types of COVID-19 vaccines  (Lopez Bernal et al, NEJM 8/12/21). The drop in protection against any  infection, including asymptomatic nasal carriage, is what has led to the  recommendations to resume public health measures to prevent  transmission. Given the ongoing low numbers of hospitalizations and  deaths in PWCF, there is no reason or evidence to believe that  vaccination is losing its major protective effect. Concerns about waning  immunity as time passes are being addressed by ongoing studies, but  currently there is little evidence this is happening independently of  variant effects.

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The drop  off in protection against any infection, including asymptomatic or very  mild infection as opposed to the ongoing great protection against  serious illness and death, has driven huge interest in giving a third,  booster shot to further augment immunity and hopefully reduce any  infection, and thereby cut transmission and thus help end the  pandemic—here again, new data from Israel strongly suggests a booster  can do so, reducing any infection by >70% in the short term (Patalon  et al, medRxiv 8/31/21). Other research has shown that boosters work  better when given after at least several months’ interval following the  initial jabs. The current trend suggests boosters will become widely  available and recommended, but there is a countervailing worry about the  inequity of boosters in developed countries like the USA when the great  majority of the world’s population has yet to see a first dose.

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What  about PWCF who are transplant recipients? Studies in CF have clearly, if  unsurprisingly, shown this is a very vulnerable population with  disproportionate rates of hospitalization, ICU care, and mortality.  Immuno-suppressive drugs needed to maintain organ grafts target the same  immune system components that vaccines stimulate. Here, studies show  that the COVID-19 vaccines are much less effective. Less than half of  solid organ transplant recipients had an antibody response after the  standard two-dose mRNA vaccination (Boyarsky et al, JAMA 5/5/21); it may  be even lower specifically in lung transplant recipients (Havlin et al,  J Heart Lung Transplant 5/21/21). Breakthrough COVID-19 infections  occurred 82 times more often (serious illness 485 times more often) than  in nontransplant controls (Qin et al, Transplantation 7/23/21). Again,  no studies focus solely on CF, but several include CF lung or liver  transplant recipients along with those suffering many other diseases  resulting in transplantation. There is some partially good news even  here, though: a booster shot can result in a much better immune  response, resulting in antibodies in 70% of solid organ transplant  recipients (Kamar et al, NEJM 8/12/21), so transplant recipients join a  list of those with other causes of immunocompromise currently encouraged  to get a booster at least a month after the second mRNA vaccine shot.  The CDC has an excellent website on this topic, which I encourage anyone  who has received, or is listed to receive, a transplant, to review (https://www.cdc.gov/coronavirus/2019-ncov/vaccines/recommendations/immuno.html).

Finally,  a few words on influenza. It hasn’t gone away, it’s here to stay,  mutating constantly so that annual flu shots to keep current (and  occasionally additional shots for worrisome variants like 2009’s H1N1  pandemic strain) have long been part of routine CF care. The 2020-2021  winter flu season was practically nonexistent, courtesy, it is thought,  of COVID-19 public health measures. That doesn’t mean it will stay  subdued as the country has opened up and flu season approaches. People  with CF have good antibody responses to flu shots (Dharmaraj &  Smyth, Cochrane Database of Systemic Reviews 2014). Over 90% of PWCF get  them (Ortiz et al, Chest 2010), and flu vaccines significantly reduce  likelihood of PWCF getting the flu (Wat et al, JCF 2008). You should  continue to get your annual flu vaccine.

Dr.  Richard B. Moss is former chief of the Pediatric Pulmonary and Allergy  Divisions, and allergyimmunology and pulmonary fellowship training  programs director, at Stanford University. He was educated at Columbia  (BA), SUNY Downstate (MD), Northwestern/Children’s Memorial Hospital  (pediatrics residency) and Stanford (allergy-immunology, pulmonology  fellowships). He was Director of the Cystic Fibrosis Center at Stanford  (1991-2009) and site principal investigator for the Cystic Fibrosis  Therapeutics Development Network (1999-2009), where he was also  inaugural Chair of the Protocol Review Committee. Dr. Moss has reviewed  and consulted for the NIH, CFF, national and international foundations,  peer-review journals and biopharmaceutical companies. His research  interests include immunopathogenesis, outcome measures, and treatment of  chronic airway diseases of childhood. Recent work has focused on  allergic fungal lung disease. If you have questions for Dr. Moss, you  can email CF Roundtable at cfroundtable@usacfa.org.

In  January 2021, the first COVID-19 vaccines were beginning to arrive here  in the U.K. The government announced that both Pfizer and AstraZeneca  vaccines would be available and would initially be offered to those most  in need. They did this by assigning every person in the country to one  of ten priority groups and I discovered that I was in priority group  four—“all those 70 years of age and over and clinically extremely  vulnerable individuals (not including pregnant women and those under 16  years of age).”

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In 2020,  shortly after the pandemic began, I was designated as a “clinically  extremely vulnerable individual” on account of having received a double  lung transplant and having a suppressed immune system. I was asked to  spend much of 2020 sheltering at home and was unable to leave for my own  protection. At first, the government arranged for food supplies to be  delivered to our homes because the supermarkets didn’t have enough  delivery slots. It was fantastic having a friendly person arrive at your  door with canned goods, bread, pasta, cookies, jams, tea, instant  coffee, and shelf-stable milk. Later on in the pandemic, the  supermarkets started to offer priority delivery slots to people based on  the same priority groups.

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I was  delighted that I would be receiving my vaccine soon and recall jumping  up and down around my apartment yelling, “I’m getting the vaccine!” I  was six years post-transplant and knew all too well the fragility of my  immune system. I heard horror stories from both the U.S. and the U.K.  about how serious COVID-19 was proving to be for transplant patients and  how many of them were unfortunately dying. This vaccine would also be a  ticket out of my apartment where, frankly, I had been stuck for far too  long.

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As the  weeks went by and I patiently waited for an appointment, I spent a lot  of time watching and reading the news. I gathered all the information I  could on the vaccines. My transplant hospital was brilliant with getting  information to us and I attended several of their virtual meetings and  Q&A sessions. They explained the studies and calmed any fears I had  that these vaccines were safe enough for both the cystic fibrosis and  transplant communities.

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It was  late in February 2021 when the phone rang from my doctor’s office,  “Margaret, it is your turn for the jab. Would you like me to schedule  that for you?” The poor woman on the phone must have thought I was  insane because I was so giddy with excitement and my voice reflected  that sentiment. The English already think Americans are too much and I  was proving them right on this phone call! Thankfully, she seemed  unfazed by my enthusiasm and the appointment was booked for a few days  later.

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I  arrived at my appointment and was definitely the youngest person in the  waiting area. I waited patiently to be called into the room. I was  greeted by a very nice male nurse who asked a few questions, gave me a  leaflet on the side effects of the vaccine and then proceeded to draw up  the liquid from a vial. I was getting the AstraZeneca vaccine, which  was what I wanted as I had heard of potential issues with the Pfizer  vaccine if you’ve ever had severe allergies, as I have had in the past.  It was a quick poke to the arm and I hardly felt a thing, far less than  my flu shot every year! As I left, I felt like a weight had been lifted  off my shoulders. I wasn’t out of the woods but had taken the first step  to regaining some normality. I didn’t feel great for a few days after  my first vaccine, but it was nothing too major—just a little tired,  dizzy, and generally lethargic.

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At the  time, the U.K. government’s strategy was to give the first dose of the  vaccine to as many people as possible before giving out second doses of  the vaccines. This meant I would have to wait 12 weeks for my second  vaccine, which I wasn’t too happy about as I watched some of my fellow  Americans, who had gotten the Pfizer vaccine, receive their second shot  after only three weeks. I waited 12 weeks for my second shot and just  repeated the process. Since having my second shot, the advice has  changed to only wait eight weeks between shots in order to receive the  most immunity with the AstraZeneca vaccine.

Why did I  get my vaccines? I wanted to protect myself given that I’ve had a  transplant and knowing I have a compromised immune system. I’d also  spent a year stuck at home in my apartment and frankly, I wanted to be  able to go out again.

How do I  feel about two doses of vaccine causing a weak immune response in some  post-transplant? I’m sad and angry that the vaccines have not produced  the antibodies so many of us needed to feel safe. I’m hoping that the  third booster vaccine that I’m getting in the coming weeks will give me  some added protection.

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What do I  think about all the misinformation surrounding vaccines? It is  fascinating to be living in another country outside of the U.S. and to  see the differences between the U.K. and the U.S. The U.K. doesn’t seem  to have as much misinformation surrounding the vaccine. It never became  political and most people wanted the vaccines to protect themselves and  their loved ones. We still have people who are opposed to the vaccines,  but we are already at a 90% vaccination rate in England. I was also  fortunate to receive information from my transplant team, which helped  me feel safer in my choice to get the vaccines. s

Maggie  Williamson is 33 years old and has CF. Maggie received a double lung  transplant in 2014 at Stanford Hospital in California. She now lives in  the U.K. with her British husband. She is passionate about cooking and  food in general and has built a small catering business called Just A  Sprig. She is a peer connect volunteer with the Cystic Fibrosis  Foundation, which involves helping those with cystic fibrosis who need  support in coping with their disease. She is also a committee member for  her transplant hospital club, The Harefield Hamster Club. Maggie is  looking forward to traveling the world once it is safe and is  particularly looking forward to seeing her family in Chicago. You can  connect with Maggie through her @justasprig page on Instagram.

I  have CF. I am a lung and kidney transplant recipient. I have diabetes  and I have recurring bouts of skin cancer. I have a multitude of other  health issues that are too long to list here. Do any of these things  qualify me to be a doctor? Of course not. That is why I talked to my  primary care provider (PCP), my CF doctor, and my lung transplant doctor  about getting COVID-19 vaccinations. They all agreed that I should get  them, so I did. I also got the third dose, as recommended by the Centers  for Disease Control and Prevention (CDC) and my doctors. Because I am  at high risk for getting all sorts of things due to my compromised  immune system, I also get my yearly flu shot in addition to vaccinations  for pneumonia, shingles, and TDaP (Tetanus, Diphtheria, and Pertussis),  all with approval and endorsement from my doctors. I have had no health  issues from any of these vaccines, and I am protected from these  illnesses as much as I can be. That is what vaccines are for.

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I think  of getting the COVID-19 vaccines as me protecting myself and protecting  everyone around me. This is not just about one person. This is not about  personal liberty. This is about putting you, your family, and your  community first. This is about caring for other people. This is a group  effort, which means everyone needs to participate if we are ever going  to get past this pandemic. There are breakthrough cases of COVID-19  (people getting COVID-19 even though they are fully vaccinated).  Scientists tell us that the vaccines are not 100% effective, which means  it is no surprise that there are some breakthrough cases. This does not  mean that people should not get vaccinated; COVID-19 vaccines are  effective at preventing infection, serious illness, and death. We need  the COVID-19 vaccines for protection, especially with the variants being  more contagious and deadly than the original strains.

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Since  writing this, I have received good news: I still have COVID-19  antibodies! I am so thankful about this because I was told by my lung  transplant clinic nurses that only a few of their patients developed  COVID-19 antibodies after being vaccinated.

Yes,  people with CF are probably smarter about medical stuff than the general  population, but please do not be lulled into a false sense of security  because you think you know what you are doing in this realm. COVID-19 is  a force to be reckoned with, even for us.

Colleen  Adamson is 52 years old and has CF. She lives in Alexandria, VA, with  her husband, Scott, and their dog Penny. She can be reached at scott.adamson@cox.net.

Constant  anxiety runs through my mind. I try to fill my head with peaceful  thoughts so that I can be present in the moments that make pleasurable  memories. Some days it is easy to focus on the good, but some days are  consumed by fighting through the aches in my body and in my soul. I was  born with cystic fibrosis, and the life I have had so far is the only  one I have known. My appetite is filled with handfuls of pills and my  hours are consumed with breathing treatments and therapies. These  aggressive attempts are to ease the difficulties that come from the  mucus that has cemented itself inside my organs. No matter how hard I  will the poison to exit from my body, it becomes something that I must  adapt to and accept. The ability to do so many things has been stripped  from my existence. My inability to breathe well has limited my  involvement in activities. My stomach pains have brought on emergency  procedures. My fears of being too far from a medical facility that can  handle my health has kept me from traveling. My life came with  boundaries and a strict routine, and I did not dare to deviate too far  from my safety net.

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Those  struggling with the disease were given the possibility of relief, as  scientists had developed a medication that helps the defective CFTR  protein work more effectively inside our bodies. The first magic pill  was only available for a small portion of those afflicted, and I was not  a part of that group. To feel some hope that our disease was treatable,  I hovered around those lucky enough to take the new modulators, asking  for updates, hoping they were experiencing a new reality that the rest  of us could only see in our dreams. After a few modifications of the  drug, and several phases of research later, a miracle drug was available  for most of those suffering from CF. I had tried some of the earlier  pills without much benefit, so I was looking forward to trying the  latest medication, which was considered a serious breakthrough in the  medical world. Our community of sick and dying was celebrating across  the globe.

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I never  thought twice about taking the medication, as anything that might ease  my tired, heaving lungs was worth it. Since most people with CF perish  from respiratory failure, even the idea of wiping that fate from my  future was almost unfathomable. However, within hours of taking my first  dose, the vile mucus began fleeing from me. My sinuses began to open,  and I was realizing everything had a smell. I was coughing intensely  like I had been a drowning victim saved from the lake. My obstructed  bowels were clearing, and the pains were flowing away. I could feel  things in my body that I didn’t even know could be felt. By the time I  went to bed that night, I was able to fill my lungs with air beyond  anything they had before. I could not process the quietness of my  breathing, so I silently slipped into a deep slumber.

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Our  community of desperately sick people was diminishing and our frequent  pep talks were disappearing. The need for support from those that  understood wasn’t needed anymore because everyone was living. People  were doing things they didn’t think would ever be possible. Even though I  was also experiencing amazing things, I longed for the relationships  that used to keep me grounded. I used to walk up and down the hallways  of the CF ward and chit chat with my fellow fighters from a distance,  always learning a new way to deal with the disease. But instead of  seeing each other in the hospital, we were seeing each other on social  media living life to the fullest—images of fellow fighters having  babies, traveling to extreme destinations, and being in all sorts of  places other than a hospital bed. I even began to do things that I  didn’t think I’d have enough energy or years to accomplish—writing a  book, starting a business, and traveling abroad.

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I was  also starting to see things from a whole different perspective. I felt  like I didn’t have to rush to fit everything in. I didn’t have to  maneuver my activities around how I was feeling. I didn’t have to fit my  hospitalizations into my work schedule. I wasn’t tethered down to tubes  and lines for hours each day. My life no longer had an early expiration  date. I had a hard time figuring out what to do with all my spare time.  The feelings of suffocation and impending doom that used to overtake  most of my thoughts had been replaced with emptiness, and I had to find  new thoughts to fill the void. I had to restructure my day and fill it  with things that made me happy and filled my heart.

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But being able to fill up your life where there used to be tedious
requirements was almost a chore. I had to find a new way to live with my  body. I didn’t have to rush to fit it all in. I didn’t have to stop and  smell the roses. My passion for photography and nature dwindled. The  gorgeous sunsets didn’t look so amazing anymore. I felt like I had so  much time and so little to do. What was my purpose? I now had the  ability to do amazing things and I was not sure how to apply it to my  new life. I missed my people. The medical team who used to lift me up  and delicately take care of me was no longer part of my family. The  excitement of being able to breathe soon spiraled into a pit of  depression.

I tried  to reach out to people whom I had previously been neglecting because of  my health and tried to organize get-togethers and reunions. I joined  softball leagues and put passion into my gardens. But just as my mind  started to blossom into accepting this newfound freedom, my body started  to feel worse. I developed a rash on 50 percent of my body that  resembled an allergic response. I itched and scratched throughout the  day and night, turning my inflamed skin to scabs. My elbows, knees, and  shoulders began to give out, causing me pain with every movement. I had  all this energy and breath but couldn’t use it because of the crippling  pains and rashes. I tried treatment after treatment, even considering  surgeries, trying to sort out these new challenges.

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One  morning after I took my miracle drug, my airway started to tighten. Even  though I was used to the feeling of suffocation, this sensation was  different. I could feel my body rejecting the medication. I decided to  stop taking it immediately for fear that it would do more harm than  good. My doctor agreed that it was too risky to continue. I was just  beginning to figure out how to manage my new existence, when I was spun  back into my old life. Almost immediately, my cough returned, my  intestines plugged, and my weight dropped. My body was finding its way  back to the way it was. Mentally though, it was almost exciting to be  back to my old self; a reality that was familiar.

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As I was  returning to a state of health that was dire, I was finding that others  were experiencing some of the same disappointments with our life-saving  medicine. The side effects so severe that they also had to make the  gut-wrenching choice to stop taking the medication. How can something so  good make us feel so bad? Our freedom to live with health was being  stripped from us. I noticed that even though my joints were feeling  better, my lungs were filling at a quick rate. Breathing became tiresome  again. I found myself in the emergency room on two occasions for lung  issues, the latter ending in an admission for IV antibiotics. It was  like my previous life came flying in at full force like it never left.  Even though it was disheartening to be sick, I felt a sense of comfort  as I was being treated by my old pals at the hospital.

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It  became clear that I cannot live without the drug, but I am not sure I  can live with it either. Under medical supervision, I made the choice to  try taking the drug again. At least I would be able to make an informed  decision as to whether taking the drug was even an option. As the  nurses set up my hospital room with life-saving equipment in the event  of me having an allergic response, I mentally prepared myself for the  test. I needed to be okay if I was able to start the drug again, and I  needed to be okay if I was not able to. The memories of the past year  were swirling through my head as I lay the pill on my lips. I opened my  mouth quickly and swallowed the pill before I could change my mind.  Anxieties overran my thoughts as I intently focused on my airway. Was I  feeling an adverse reaction? I calmed myself with a few deep breaths and  found that my worries were causing me more difficulties than anything  else. As the time passed, my vitals remained stable, and I was cleared  to take the drug again. A new sense of relief filled my soul.

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The tug  of war with my self is currently at ease. I now feel that I have a  second chance to fill my heart and soul with the things that make me  happy. My life’s time is not ticking so fast. Although I do not know  what the future holds, I need to realize that my life-shortening disease  is still a part of me and will always be. Whether my days are filled  with medications and therapies, or whether they’re filled with  experiences, I must be able to find peace in that reality. I must  continue to grow and learn about my body and my mind, finding a balance  between the familiar and the unfamiliar. s

Leah  Sands is 40 years old and has CF. She lives in Detroit, Michigan. She  loves photography, team sports, and traveling. She can be contacted by  email at leah.sands@gmail.com.

The U.S.  Adult CF Association (USACFA) is pleased to announce the recipients of  the Higher Education (formerly the Lauren Melissa Kelly Scholarship).

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In our  evaluation, we look for students who demonstrate tremendous academic  achievement, community involvement, and a powerful understanding of how  their CF—matched with these achievements—places them in a unique  situation to gain leadership roles within the community. Our scholarship  is open to all pursuing any degree, from associates to Ph.Ds. We  believe that any higher education is a strong foundation for advocacy  and involvement in the CF community.

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Nancy  Wech established this scholarship in honor of her daughter, Lauren  Melissa Kelly. This semester’s winners demonstrated outstanding  potential, just like Lauren years ago. Lauren was an inspiration to all  who knew her. An incredible leader and scholar, her drive and success  are the foundation of her memory. She was transformative in every aspect  of her life. She had distinguished herself as a member of the Golden  Key Honor Society, Mortar Board, Phi Upsilon Omicron, Gamma Beta Phi,  Delta Gamma sorority, and was chosen as one of ten Senior Leads at the  University of Georgia. She acted as one of the re-founding members of  the Phi Kappa Literary Society and was significant in the metamorphosis  of the Z Club into the William Tate Society. Although Lauren lost her  battle with cystic fibrosis late in her senior year, her hard work and  memory continue to live on through her inspiring involvement.

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We are  pleased to announce Emily Schutz and Julia Kennedy as the recipients of  the scholarships for this calendar year. They will each be awarded  $2,500. Congratulations to both!

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Emily  Schutz is an accomplished young woman pursuing her bachelor’s degree in  English and Media Com-munication Studies at Florida State University.  Her writing has been featured in several publications in the last two  years. Additionally, Emily was the Editorial Shoot Director for the DWF  Magazine in Tallahassee for one semester in addition to serving as  Graphic Designer for Her Campus FSU. Currently, she serves as both  Secretary and Social Media Coordinator for Sigma Tau Delta in  Tallahassee. Outside of her schoolwork, Emily is busy volunteering her  time both within the CF community and within her wider campus community.  Emily dreams of sharing stories with the world, especially the stories  from those who need a voice.

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Julia  Kennedy is a motivated young woman who holds both a Bachelor of Arts in  Social Studies and a Masters of Art in Educational Leadership from  Michigan State University. She is currently completing the Special  Education Supervisor Program offered through Wayne State University.  Julia has worked as a resource room teacher in several elementary  schools in Michigan for the past several years. In addition to her  educational accomplishments, Julia has advocated tirelessly for persons  with CF in her community. In April of 2019, she and her husband met with  Michigan representatives and senators to advocate for the continuation  of medical benefits under the Children’s Special Health Care Services of  Michigan Medicaid programs.

Both  Emily and Julia demonstrated the leadership, intelligence, and drive of  Lauren Melissa Kelly. All of us at USACFA look forward to seeing them  further develop their leadership and advocacy in the cystic fibrosis  community.

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Scholarships  are awarded each year. More information, including the application and  relevant deadlines, can be found on our website. For questions about  future scholarships or anything related to the application process,  please contact us at scholarships@usacfa.org.

Partnership Aims To Lower Out-Of-Pocket Costs For Rare Disease Meds

AllianceRx  Walgreens Prime, a specialty and home delivery pharmacy, is partnering  with TailorMed, a healthcare technology company, to help lower  out-of-pocket prescription costs for specialty pharmacy patients.  Medications attained through specialty pharmacies are those used to  treat rare and chronic conditions in the U.S., and are often extremely  costly. For this reason, Alliance Rx noted that it has staff dedicated  to helping link eligible rare and chronic disease patients with  financial aid programs. There was a 5% rate of non-adherence to  prescribed treatment when out-of-pocket costs were zero, and a 45%  non-adherence rate when these costs surpassed $125. A majority of  patients, 60%, stopped using treatments as prescribed when  out-of-pockets costs exceeded $500. TailorMed’s platform identifies  people at risk of being unable to afford their specialty prescriptions,  and automatically connects them to financial assistance programs. Its  comprehensive database of financial assistance programs is updated in  real-time. AllianceRx Walgreens Prime will have access to more than  5,000 aid programs via this platform. They include co-pay assistance,  manufacturer voucher and bridge programs, government subsidies,  community and state resources, and assistance from disease-specific  foundations.

https://tinyurl.com/yhjpvvpe

Relizorb Will Be Covered By Medicare

The  Centers for Medicare and Medicaid Services (CMS) has published a local  coverage determination confirming that Relizorb—an enzyme cartridge to  help in digestion for cystic fibrosis (CF) patients receiving their  nutrition through enteral (tube) feeding—will be covered by Medicare.  Relizorb is a first-of-its-kind digestive enzyme cartridge that can be  attached to the end of a feeding tube. The cartridge mimics the function  of pancreatic fat-digesting enzymes (lipase), so that as feeding  formula passes the cartridge, fat molecules are broken down into  components that are easier for the body to absorb. Relizorb can break  down up to 90% of fat molecules found in most enteral feeding tube  formulas, including molecules that are comparatively difficult to digest  but are critical for growth and development, such as certain omega-3  fatty acids. With the newly issued LCD, the CMS has basically stated  that Relizorb is both reasonable and necessary as a treatment for  eligible patients. As such, Relizorb will be covered by Medicare.

https://tinyurl.com/yfjm8o9h

Functional Respiratory Imaging Offers Value In Cystic Fibrosis

Air  trapping and pulmonary blood distribution were found to be clinically  relevant functional respiratory imaging (FRI) parameters associated with  spirometry and the 6-minute walk test (6MWT) in patients with CF,  suggesting these FRI variables could offer value in the assessment of  functional characteristics of the CF respiratory system. The FRI  modality comprises a combination of high-resolution computed tomography  (CT) images and computational fluid dynamics to gain objective and  quantitative insights into lung structure and function. Investigators  conducted a cross-sectional analysis in which the FRI outcomes were  regional airway volume, airway wall volume, airway resistance, lobar  volume, air trapping, and pulmonary blood distribution. The CF-CT score  was used by 2 investigators to independently evaluate structural  abnormalities on the CT scans. Patients also underwent spirometry and  the 6MWT. A potential limitation of this study included the lack of  controls without CF. Additionally, the researchers noted that additional  investigation is needed to evaluate the association between  longitudinal changes of FRI parameters and other relevant clinical  outcomes. However, the investigators concluded that the set of  structural components of FRI providing quantitative, objective and  regional information have the potential to complement results derived  from conventional outcome measures in future CF research as an  alternative to visual CT scores.

https://tinyurl.com/yjduljk8

Joint Disease More Common With P. aeruginosa Infection, Female Sex

Chronic  Pseudomonas aeruginosa infection in adults with cystic fibrosis  increases their likelihood of developing CF arthropathy. A significant  risk for CF arthropathy was found with increasing age, a higher number  of hospitalizations, CF-related diabetes, and the presence of  comorbidities. Women were more than twice as likely to have CF  arthropathy, as were patients with pancreatic insufficiency. Those with  sinusitis or nasal polyps were also at higher risk for CF arthropathy.  Treatment with anti-fungal medications linked with an almost three times  greater risk of CF arthropathy in adults without P. aeruginosa.  Arthropathy, a collective term for joint diseases, is associated with CF  when patients have pain in the joints with signs of inflammation, but  without evidence of periostitis (inflammation of the tissue that  surrounds the bone) and with no other causes for joint inflammation.

https://tinyurl.com/4nzxjuas

Cystic Fibrosis-Related Diabetes (CFRD) And Cognitive Function In Adults With Cystic Fibrosis

This  study was carried out to assess cystic fibrosis-related diabetes (CFRD)  as a mechanism for cognitive impairment in people with cystic fibrosis  (CF). It was hypothesised that cognition would be poorer in adults with  CFRD than in those with CF without diabetes (CFND) or in healthy  controls. Researchers evaluated cognitive performance using the  Cambridge Neuropsychological Test Automated Battery which provides a  comprehensive cognitive assessment with tests mapping onto specific  brain regions. They recorded CF specific clinical variables for the two  CF groups. The results showed that managing CF requires higher order  executive function. It has been considered that impairments may be  sufficient to interfere with self-care and the ability to conduct  everyday tasks efficiently. Moreover, at which point in the CF disease  trajectory these difficulties begin, and what may attenuate them, has  yet to be ascertained.

https://tinyurl.com/4x6ayw53

Study: Use Lung Clearance Index To Measure Function

An  assessment called the lung clearance index could be used to help  identify people with cystic fibrosis (CF) who are at high risk for  worsening lung function. The study reported on factors associated with a  worsening lung clearance index (LCI) over time in CF patients, which  included initial LCI measurements and bacterial infections. LCI is a  measurement of how well the lungs are able to take gas in and out. LCI  represents the number of lung volume “turnovers” needed for clearance of  a tracer gas; a higher value is indicative of worse lung function.  LCI  may provide useful clinical information, it is not used routinely in  many places, in part because the assessment can be complex to  administer. Another challenge is that questions remain about how this  measurement tends to change over time. In this study, a research team  reported data from a prospective study in which LCI was incorporated  into routine clinical practice at three specialty centers. Because they  were interested in how measurements might change over time—in the  absence of noteworthy clinical changes—the researchers specifically  looked at patients with relatively stable CF. The majority of patients  with predominantly mild CF fell into a group with stable LCI throughout  the course of the study.

Still,  other trends were seen in a minority of patients. One in 10 started with  LCI measurements about normal, and then the values increased over time  (indicating worsening lung function). A similar proportion started with  higher-than-normal measurements, which then worsened over time. In  statistical models, the factor most powerfully associated with changes  over time was LCI at baseline (at the start of the study). Other  significantly associated factors included age, infection with the  bacteria Pseudomonas aeruginosa, use of antibiotics, and baseline forced  expiratory volume in one second—a measure of lung function based on the  amount of air a person can exhale in one second. Overall, the procedure  was well-tolerated, with most patients saying it was easy to complete.  The most common suggested improvement was shortening the time it took to  complete the measurement (median of about 20 minutes). The findings  support the use of LCI in clinical practice in identifying patients at  risk of lung function decline. The measurement, however, is challenging  to deliver in routine practice so may be better suited to annual  assessments.

https://tinyurl.com/5265hdkd

Low VIP Hormone May Contribute To Early CF-Related Diabetes

Reduced  levels of the VIP hormone in the pancreas due to low nerve supply may  contribute to the development of early diabetes in people with cystic  fibrosis. Cystic fibrosis-related diabetes (CFRD) is characterized by  decreased production of insulin, a hormone secreted by the pancreas that  lowers glucose in the bloodstream. VIP (vasoactive intestinal peptide)  is a hormone known to modulate insulin secretion in the presence of  glucose. VIP is produced in the nervous system and acts as a signaling  molecule between nerve cells (neurotransmitter). It also is found in the  respiratory, digestive, reproductive, and cardiovascular systems. VIP  also has anti-inflammatory and immunomodulatory properties, and plays a  crucial role in maintaining clean airways. Researchers demonstrated VIP  was 50% lower in the lungs, sweat glands, and small intestines of mice  that carry the F508del-CFTR mutation. The study found VIP deficiency  originated from a reduction in nerve supply (innervation) starting at a  young age before signs of CF-like disease were seen. The team also  examined whether there were VIP changes in the pancreas, affected by  nerve supply, at different stages of CF in the same mouse model to  determine whether changes in VIP levels would contribute to CFRD  development. Based on their previous work, these mice showed minimal  disease by eight weeks of age, whereas 17-week-old mice display  well-developed CF-like disease.  The data also showed that the amount of  VIP is strongly reduced in the pancreas of CF mice at both early and  late stages of disease progression. Finally, assessing the effect of  altered insulin and glucagon production, the team found significantly  higher levels of glucose in the bloodstream of CF mice. The results show  a reduction in insulin and an upregulation of glucagon in the pancreas  of CF mice, indicating that a CFRD phenotype can develop at an early  stage (as early as 8-week-old) of disease progression.

https://tinyurl.com/5x3bycu3

Hormonal Fluctuations Linked To Cystic Fibrosis Exacerbations In Women: Study

Epidemiologic  studies demonstrate worse outcomes in women with cystic fibrosis (CF)  than men. Women are colonized earlier with respiratory pathogens and  have increased rates of pulmonary exacerbations after puberty and near  ovulation. There are potentially important fluctuations in inflammatory  biomarkers in the lungs that correlate with changes in lung function in  women with CF. Researchers sought to explore whether natural hormonal  fluctuations and hormonal contraception associate with changes in lung  function, respiratory symptoms, or inflammatory markers. The researchers  prospectively followed women with CF who were not on hormonal  contraceptives and reported regular menstrual cycles. A subset of  subjects were subsequently placed on a standard oral  estrogen/progesterone combination contraceptive pill, ethinyl  estradiol/norethindrone (loestrin), and reevaluated. Measurements  included lung function, symptom questionnaires, sweat tests, blood for  hormone concentrations, and sputum for inflammatory markers, bacterial  density, and cytology. Hormone concentrations were as expected on and  off hormonal contraception. At times of peak estrogen (ovulation), there  was a significant increase in sputum proinflammatory cytokines  (neutrophil-free elastase) and a corresponding pattern of decrease in  lung function. Furthermore, proinflammatory cytokines (IL-8, TNF-α, and  neutrophil-free elastase) improved when placed on hormone contraception.  As a result, the authors concluded that there are potentially important  fluctuations in inflammatory biomarkers in the lungs that correlate  with changes in lung function in women with CF.

https://tinyurl.com/228svm6h

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Pregnancy For Women With Cystic Fibrosis

The  Mayflowers study, which will begin in July and end in December 2025,  will enroll 285 women with cystic fibrosis at 40 sites across the United  States. The women will be followed through pregnancy and 2 years after  giving birth to document their health changes. The study team expects  that about 25% of the women will stop taking their cystic fibrosis  transmembrane conductance regulator (CFTR) modulator during part or all  of their pregnancy. There is no published data in human women whether  all 3 components of the newest CFTR modulator are transferred to the  baby through the placenta or breast milk. Additionally, only small  retrospective surveys have reported use of CFTR modulators in pregnancy,  limiting understanding of the impact of modulators on outcomes for  infants exposed in utero and during lactation. A substudy will evaluate  the pharmacokinetics of modulators in pregnant women and in the infants  exposed to modulators for women who choose to continue them during  pregnancy and breastfeeding.

The  study expects to show that it is safe for a woman to stay on her  medications, especially one of the new modulators, and not experience a  significant health decline during pregnancy, particularly in the first  year after her child is born. The study will evaluate diabetic women  with cystic fibrosis as well. A substudy of diabetic women will use  continuous glucose monitoring. This substudy will provide evidence-based  data for managing diabetes during pregnancy that is specific to the  unique needs of women with cystic fibrosis. The Mayflowers study will  provide definitive data to guide women and their partners in their  decision-making.

https://tinyurl.com/ve4u74e8

Fertility And Pregnancy In Cystic Fibrosis

Sexual  and reproductive health is increasingly important for pwCF as many are  considering parenthood. Most men and some women with CF (wwCF) will have  reduced fertility, which in both sexes is multifactorial. However,  unplanned pregnancies in women are not rare, and contraception and its  interaction with CF complications need to be addressed by the CF team.  Reduced fertility may be overcome in most pwCF through use of assisted  reproductive technologies. Most wwCF will have normal pregnancies, but  premature birth is common especially in the setting of reduced lung  function and CF related diabetes (CFRD); optimization of treatment is  recommended during pregnancy planning. Parenting imposes an increased  burden on pwCF, with the challenges of caring for the newborn,  postpartum physiologic changes and maintaining CF treatments. Most drugs  used to treat CF are considered safe in pregnancy and lactation, but  exceptions need to be acknowledged, including the limited data regarding  safety of CF transmembrane conductance regulator (CFTR) modulators  during conception, pregnancy, and lactation. Prospective studies  regarding these issues in people treated with CFTR modulators are  paramount to provide evidence-based guidance for management in the  current era of CF care.

https://tinyurl.com/4xsxft2u

What To Know About Cystic Fibrosis And Pregnancy

This is  an in-depth article that covers the following areas: Planning Your  Pregnancy, Cystic Fibrosis Drugs and Pregnancy, Post-Transplant  Considerations, Cystic Fibrosis Doctor Discussion Guide, Cystic Fibrosis  and Fertility, Genetic Counseling, Cystic Fibrosis and Gestation,  Pulmonary Exacerbations, Gestational Diabetes, Nutritional Deficiency,  Constipation, Hypertension, Cystic Fibrosis and Postpartum, Impact on  Recovery, and Breastfeeding. If you’re planning on starting a family,  you should definitely read this article.

https://tinyurl.com/yecm2sy3

Depression, Anxiety Common Among CF Patients

Depression  and anxiety are common among people with cystic fibrosis. Evidence  suggests that adults with CF, as well as parents of children with CF,  have an increased risk to experience depression compared to the general  population. Depression and anxiety are known contributors to poorer  quality of life, and linked with increased healthcare costs. An  investigative team conducted a systematic review to gather and summarize  evidence from studies regarding rates of the conditions among CF  patients worldwide. The results obtained after specific data analysis  revealed that the overall global prevalence of anxiety was 24.91%, and  depression 14.13% across CF patients. Researchers then conducted  subgroup analyses and compared both anxiety and depression prevalence  according to the geographical location. Anxiety prevalence showed a  marked variation across continents: the lowest prevalence was seen in  North America, and the highest seen in Europe. The opposite was seen for  depression, with the highest prevalence seen in North America and the  lowest in Europe. Overall, these results support previous evidence and  show that both anxiety and depression are common among CF patients.  These findings highlight the need for close monitoring of the patient,  regular screening for anxiety and depression and appropriate prevention  techniques.

https://tinyurl.com/ytmnfc2d

Relatives With CF Can Be Source Of MRSA Transmission, Study Finds

Among  people with cystic fibrosis (CF), antibiotic-resistant Staphylococcus  aureus is sometimes transmitted among relatives, but rarely in  healthcare settings. Findings also indicate that distinct subsets of S.  aureus may be associated with differences in disease progression. A team  of researchers reported an increase in MRSA infections among CF  patients at their institution. They suspected that rising MRSA  prevalence over the past decade may have been hastened by either  person-to-person or healthcare worker-to-patient transmission.

To test  this idea, the scientists sequenced the genomes (genetic code) of 97  MRSA isolates collected from 74 patients. By comparing genetic sequences  among the different isolates, the researchers could look for clues as  to how the bacteria were transmitted. In general, bacteria with  relatively similar sequences are more closely related to each other. As  such, if two people have MRSA isolates with very similar sequences, it  is probable that one person acquired the infection from the other.  Analysis showed evidence of MRSA transmission between relatives.  However, instances of related individuals with distinctly different MRSA  strains were also noted. In contrast, results showed minimal evidence  suggesting transmission within hospitals, which indicates that these  infections probably are not being acquired in a healthcare setting.  Understanding the origins of MRSA in patients with CF could help limit  acquisition of these resistant bacteria and improve patient outcomes.

https://tinyurl.com/4nsbahkr

Enterprise Therapeutics Doses First Subjects In Phase I Trial For Novel Cystic Fibrosis Therapy ETD001

Enterprise  Therapeutics Ltd announced it has successfully dosed the first subjects  in a Phase 1 trial for its novel inhaled cystic fibrosis therapy,  ETD001. The first-in-man safety study is being conducted in healthy  participants. ETD001 is an ENaC ion channel inhibitor with best-in-class  potential aimed at treating all people with CF. ETD001 has previously  been shown to have long duration of action in the lung and is therefore  expected to provide a superior efficacy and safety profile compared to  other ENaC drug candidates. ETD001 targets the epithelial sodium ion  channel ENaC, which controls fluid volume and mucus clearance from the  airways. By increasing the amount of airway fluid available to hydrate  mucus, ETD001 addresses the underlying mechanisms of mucus congestion,  and is expected to restore lung function, reduce the frequency of lung  infections and improve patient quality of life. As ENaC inhibition is  independent of the mutational status of CFTR, this makes the approach  applicable to all people with CF. Additionally, ETD001 is expected to  deliver benefit as a monotherapy and in combination with other  therapies, including CFTR repair.

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RNA-Based Therapies Show Promise In Early Studies, ReCode Reports

RCT223  and RTX0001, ReCode Therapeutics’ experimental RNA-based therapies for  cystic fibrosis (CF), safely restored function to CFTR in  patient-derived lung cells. Delivered through the company’s non-viral  platform—called the selective organ targeting (SORT) lipid nanoparticle  (LNP) platform—the therapies were also found to be well-tolerated in  lung cells from patients and in mice. ReCode plans to apply to the U.S.  Food and Drug Administration in 2022, seeking the agency’s clearance to  start clinical trials of these therapies in the U.S. ReCode’s CF program  is focused on treating the 10–13% of CF patients who have nonsense  mutations and limited therapeutic options. The CF program currently  comprises two RNA-based therapies delivered through the company’s SORT  LNP platform, which uses tiny non-viral fat (lipid) particles to  transport and deliver their cargo to targeted organs. The company’s lead  candidate, RCT223, uses a transfer RNA (tRNA) molecule—which plays a  role in the process of protein production—to replace the premature stop  signal with a “go” sequence in the CFTR gene, so that the full CFTR  protein can be produced. Data showed that both single and repeated  dosing of RCT223 restored CFTR function in lung cells derived from CF  patients and grown in the lab. This effect lasted for at least 72 hours  (three days) after a single administration, and CFTR functional levels  continued to rise with twice-weekly doses. ReCode’s mRNA replacement  agent for CF, RTX0001, is designed to deliver to cells a working version  of CFTR’s messenger RNA molecule (mRNA), the intermediate molecule  derived from DNA that guides protein production. As such, RTX0001 is  expected to increase the production of a working CFTR protein.  Preclinical data showed that RTX0001, delivered through a commercially  available mesh nebulizer, successfully delivered CFTR’s healthy mRNA  into patient-derived lung cells and into the lungs of mice. The  delivered mRNA was found to effectively and significantly increase the  production of a working CFTR after a single, low dose, and CFTR’s  activity was sustained for at least 72 hours after twice-weekly  administration. Both RNA-based therapies were generally well-tolerated  in the evaluated preclinical models.

https://tinyurl.com/uf6z54e3

Study Describes New Compound For Nonsense Mutations

A novel  compound that may hold promise for treating the roughly 11% of cases of  cystic fibrosis (CF) that are caused by nonsense mutations has been  described in a new study.

A  nonsense mutation results in a premature stop codon introduced in the  DNA sequence. This causes the cell to produce a truncated form of the  CFTR protein, which is quickly degraded within the cell. In the new  study, researchers set out to identify novel readthrough compounds that  might be useful for treating CF and other diseases caused by nonsense  mutations. As the name suggests, the goal of a readthrough compound is  to let ribosomes “read through” the erroneous stop codon to produce a  full-length protein. The team used a cell model that basically consisted  of cells engineered with a gene encoding a detectable protein that had a  nonsense mutation in it. The researchers used this model to screen  771,345 small molecules, and found that 180 of them had readthrough  activity. Among these molecules, the most active was called SRI-37240.  Further testing using cellular models of CF with nonsense mutations  demonstrated that treatment with this small molecule could allow the  cells to produce functional, full-length CFTR protein. The researchers  next synthesized dozens of derivatives of SRI-37240, and they found  one—called SRI-41315—that had even more potent readthrough activity. The  increased readthrough efficacy of SRI-41315 was confirmed in lung cells  from people with CF, particularly when used with G418, a type of  antibiotic known to aid readthrough of premature codon mutations.  Further investigation revealed these readthrough small molecules work by  reducing levels of eRF1, a protein used to recognize stop codons. The  team suggested that targeting eRF1 may be a useful strategy in CF.

https://tinyurl.com/4rwawphe

Scientists Demonstrate Promising New Approach For Treating Cystic Fibrosis

Researchers  demonstrated a potentially powerful new strategy for treating cystic  fibrosis (CF). It involves small, nucleic acid molecules called  oligonucleotides that can correct some of the gene defects that underlie  CF but are not addressed by existing modulator therapies. The  researchers used a new delivery method that overcomes traditional  obstacles of getting oligonucleotides into lung cells. The scientists  reported that they were able to restore the activity of the protein that  does not work normally in CF and saw a prolonged effect with just one  modest dose. Treatments for CF now include CFTR modulator drugs, which  effectively restore partial CFTR function in many cases. However, CFTR  modulators cannot help roughly ten percent of CF patients, often because  the underlying gene defect is of the type known as a splicing defect.  In principle, properly designed oligonucleotides can correct some kinds  of splicing defects. In practice, getting oligonucleotides into cells,  and to the locations within cells where they can correct RNA splicing  defects, has been extremely challenging, especially into the lungs.  Therapeutic oligonucleotides, when injected into the blood, have to run a  long gauntlet of biological systems that are designed to keep the body  safe from viruses and other unwanted molecules. Even when  oligonucleotides get into cells, the most usually are trapped within  vesicles called endosomes, and are sent back outside the cell or  degraded by enzymes before they can ever do their work. This new  strategy overcomes these obstacles by adding two new features to splice  switching oligonucleotides: Firstly, the oligonucleotides are connected  to short, protein-like molecules called peptides that are designed to  help them to distribute in the body and get into cells. Secondly, there  is a separate treatment with small molecules called OECs, which help the  therapeutic oligonucleotides escape their entrapment within endosomes.  The researchers demonstrated this combined approach in cultured airway  cells from a human CF patient with a common splicing-defect mutation.

https://tinyurl.com/ygutw7hf

Inhaled Drug Could Treat Rare Cystic Fibrosis Mutations

Trikafta  treatment combining three drugs – elexacaftor, tezacaftor and ivacaftor  –reduces symptoms for those with cystic fibrosis. The new medication,  although not a cure, works wonders for the 80 percent of cystic fibrosis  patients with the predominant mutation causing the disease - F508del.

But what  about the 20% of CF patients who have a different genetic mutation?  SpliSense manipulates and “fixes” defective messenger RNA that generates  a non-functioning cystic fibrosis transmembrane conductance regulator  (CFTR) protein. Rather than trying to repair defective proteins,  SpliSense’s technology generates a new fully functioning protein from  RNA. SpliSense has demonstrated in cells derived from patients that it  can completely restore CFTR function. The company is now moving on to  animal models and the first human clinical trials are planned for 2022.  Since CF primarily affects the trachea, bronchi, bronchioles and  alveoli, SpliSense’s treatment is meant to be inhaled so that it reaches  the lungs quickly without any uptake by other organs or the  bloodstream. If approved by regulators, the treatment would be  administered weekly for 10 minutes at home throughout the patient’s  life. Ideally, the cost of this expensive treatment would be picked up  by health insurance providers. SpliSense’s lead product is based on  “Anti Sense Oligonucleotide” (ASO), a synthetic nucleic acid molecule  that can bind to specific sequences within target RNA molecules.

The ASO  sequences are specific to the target mutation region in the RNA, so the  treatment won’t affect (or damage) nearby organs and tissues. That  should reduce potential side effects.

https://tinyurl.com/yhf7awht

CF  Foundation Launches New Collaboration With Deep Science Ventures To  Overcome Challenges To Developing Genetic Therapies For CF

The CF  Foundation announced a new collaboration with venture creator Deep  Science Ventures, focused on uncovering and designing new technologies  with the potential to overcome challenges to developing genetic  therapies for cystic fibrosis. Deep Science Ventures and the CF  Foundation will work together to assess pressing barriers to genetic  therapies in CF, explore the feasibility of potential solutions, and  design proof-of-concept studies. By aligning the Foundation’s CF  expertise and scientific capabilities with Deep Science Ventures’ track  record of uncovering innovative technologies, this agreement has the  potential to push the CF field forward and significantly accelerate  progress toward future therapies for CF.

https://tinyurl.com/4ufcf6

Vertex To Initiate Phase 3 Program For Once-Daily Triple Combination Regimen In Cystic Fibrosis

Vertex  Pharmaceuticals Inc. will initiate a phase 3 development program for the  new once-daily investigational triple combination of  VX-121/tezacaftor/VX-561 (deutivacaftor) in people with Cystic Fibrosis.  Phase 2 data suggested the triple combination holds the potential to  restore cystic fibrosis transmembrane conductance regulator (CFTR)  function in people with cystic fibrosis to even higher levels than seen  with other Vertex CFTR modulators and thereby provide enhanced clinical  benefit.  The combination of VX-121/tezacaftor/VX-561 was first  identified as having potential for increased efficacy based on its  ability to drive higher levels of chloride transport compared to  TRIKAFTA in human bronchial epithelial cells in vitro. The Phase 3  program consists of two randomized, double-blind, active-controlled  48-week trials, which will evaluate the safety and efficacy of VX-121  (20 mg)/tezacaftor (100 mg)/VX-561 (250 mg) in comparison to TRIKAFTA.

https://tinyurl.com/32t3m398

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Kaftrio Treated Severe CF Without Confirmed Secondary CFTR Mutation

Kaftrio  successfully improved lung function and quality of life in three cystic  fibrosis patients who have had severe lung disease with an unknown  mutation in the CFTR gene in addition to the common F508del mutation, a  case series reported. Results showed that over 24 weeks of Kaftrio  treatment (almost six months), the sweat chloride levels in all  participants progressively lowered, eventually reaching the normal  range. The treatment also led to clinically relevant improvements in  lung function in all patients. CF Questionnaire-Revised (CFQ-R)  respiratory domain scores significantly increased for all participants  by eight weeks and were sustained for the six months of treatment. Other  CFQ-R domains showed an enhanced quality of life. No pulmonary  exacerbations were reported during the treatment. No abnormal adverse  events were reported in terms of vital signs, clinical laboratory tests,  or physical examinations. Kaftrio combines three CF medicines:  elexacaftor and tezacaftor, designed to help faulty CFTR get to the cell  membrane, and ivacaftor, a so-called CFTR potentiator that aims to open  blocked channels allowing water and ion transport. Kaftrio, sold as  Trikafta in the U.S. is available only for CF patients with at least one  CFTR F508del mutation or another known secondary mutation. Patients  whose DNA analysis is inconclusive typically are excluded from Kaftrio  clinical trials and treatment. These findings support expanding Kaftrio  treatment in CF patients without a confirmed secondary CFTR mutation,  especially in those with advanced lung disease.

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Laura  Tillman is 73 years old and has CF. She is a former director and  President of USACFA. She and her husband, Lew, live in Northville, MI.