Medicare Coverage For Cystic Fibrosis: Testing, Management, And Treatments
Each part of Medicare offers different coverage that can help you get the treatments and care you need for cystic fibrosis. Items and services covered under each part include:
• Medicare Part A. Part A is hospital insurance. It covers any inpatient care you need. This includes stays in the hospital and rehabilitation facilities, as well as limited home healthcare services.
• Medicare Part B. Part B is medical insurance. It covers outpatient care such as the treatments you receive from your doctor. Part B also covers: ambulance rides, urgent care visits, diagnostic tests, medical equipment used at home
• Medicare Part C. Part C is also called Medicare Advantage. It covers everything that parts A and B do and often includes coverage for additional services and prescription drug coverage. Medicare Advantage plans use networks to provide coverage, so you’ll often need to stay in network to get care when you use a Medicare Advantage plan.
• Medicare Part D. Part D is prescription drug coverage. Medicare parts A and B, together known as original Medicare, don’t offer any prescription drug coverage unless you receive the medication during a hospital stay or doctor’s office visit. Stand-alone Medicare Part D plans can be added to original Medicare so that the prescriptions you take at home are covered. All Part D plans have a list, called a formulary, that tells you which prescription drugs are covered and how much they will cost.
• Medigap. Medigap plans, also called Medicare supplement plans, cover some of the out-of-pocket costs of original Medicare. They don’t offer additional coverage, but they can allow you to receive medical care without worrying about out-of-pocket costs like deductibles or copayments.
Some of the services Medicare covers are listed below:
Testing and diagnosis. Some cystic fibrosis patients don’t know they have the condition until adulthood. You’ll have coverage for the testing you need under Medicare Part B or a Medicare Advantage plan. Doctor’s and specialist’s visits. Medicare Part B or a Medicare Advantage plan will cover your doctor’s office visits and your visits to a specialist. You don’t need a referral to see a specialist if you’re using Medicare Part B, but you will need a referral with many Medicare Advantage plans. Physical therapy. You can get physical therapy coverage through Part B or a Medicare Advantage plan. Medicare doesn’t limit the amount of physical therapy you can receive as long the therapy is deemed medically necessary by your doctor. Respiratory care. Respiratory care, including the services of a respiratory therapist, is generally covered under Part B or a Medicare Advantage plan. However, if you receive respiratory care in a hospital, skilled nursing facility, or as part of home healthcare service, it will be covered under Part A. Medicare Part B and Medicare Advantage cover outpatient pulmonary rehabilitation. However, you may need preauthorization before you can use this service. Medicare will also provide coverage if you need a lung transplant. Your doctor’s visits and preparation will be covered under Part B, while your actual transplant and hospital stay will be covered under Part A. Medicare will cover prescription drugs that have been FDA approved. Coverage will fall under a Part D plan or Medicare Advantage plan that includes prescription drug coverage.
Commonly covered medications include: medications to open your airways. Medications in this category include albuterol, ipratropium, and theophylline. Medications to help you cough up sputum. Medications in this group include guaifenesin, hypertonic saline, and N-acetylcysteine. Medications to help you absorb nutrients. Medications in this group include pancrelipase and pancreatin. Medications to decrease lung inflammation. Medications in this group include beclomethasone, flunisolide, fluticasone, ibuprofen, methylprednisolone, and prednisone.
Medicare will also cover medications you need temporarily—such as, antibiotics or antiviral medications you need to treat an infection. Keep in mind that not all Medicare Part D or Medicare Advantage plans cover every prescription you might take for cystic fibrosis. Plans cover only prescriptions that are part of their formulary. Medicare will also cover the at-home medical equipment you need. This is known as durable medical equipment and is covered under Medicare Part B. Some common covered equipment includes: at-home oxygen therapy, including, oxygen tanks, tubing, and other supplies, nebulizer machines, vests for chest physical therapy, positive expiratory pressure devices. To ensure full coverage, you must get your equipment from a supplier that participates in Medicare and accepts assignment. Most of the services you need will be covered by Medicare. Exceptions include: Experimental treatments. Medicare won’t cover any treatments or procedures that haven’t been approved by the FDA to treat cystic fibrosis. Long-term skilled nursing care. Medicare will cover only 100 days of skilled nursing care. Those 100 days are covered only if you meet set conditions, including a recent hospital stay of at least 3 days. Plus, you’ll owe daily coinsurance fees starting on day 21. Long-term home healthcare. Medicare covers only home healthcare that’s medically necessary for a condition expected to improve. For example, Medicare would cover home healthcare if you’re homebound and need a nurse to treat you for an infection. Medicare doesn’t cover extended home healthcare. Any other type of long-term care. Medicare never covers stays at assisted living, custodial care, personal care homes, or nursing homes. Medicare considers all these services to be nonmedical and doesn’t cover them.
https://tinyurl.com/yaue9arg

CF Patients, Carriers Of European Ancestry At Higher Pneumonia Risk
Cystic fibrosis (CF) patients and carriers of European ancestry are at a higher risk of developing pneumonia. CF patients were also found to be at an increased risk of more severe pneumonia. The findings may help in identifying patients more susceptible to pneumonia caused by a number of triggers, including COVID-19 infection, and in starting early approaches that might prevent more severe disease. Researchers conducted a genome-wide association study. Genome-wide association studies look for genetic variants that might serve as predictive markers of the presence of a trait or disease. Study results showed that the genetic region most strongly associated with both pneumonia susceptibility and severity among those of European ancestry was located in the CF-associated CTFR gene. CF carriers were also more susceptible to pneumonia, but to a lesser extent than people with the full-blown disease. This is consistent with results from a previous study showing that these carriers are at higher risk of several CF-related conditions, when compared with people without CFTR mutations.
https://tinyurl.com/ydhpxot8

Minorities In US Ineligible For Key CF Therapies Due To Unknown Mutations
Individuals with cystic fibrosis (CF) from racial and ethnic minority groups in the U.S. are twice as likely to be ineligible for disease-modifying therapies than white patients, because their mutations are not known to be suited to approved treatments. The resulting lack of treatment access exacerbates these groups’ already severe disease course and earlier mortality. CFTR modulators are compounds that help to restore healthy CFTR function by targeting specific mutations. The specificity of these mutations means that each modulator only works for patients with those specified mutations. Approximately 90% of patients with CF have CFTR mutations that are currently FDA-approved for CFTR modulators. Patients of a minority group were over twice as likely to have CFTR mutations that were not classified compared to non-Hispanic white patients. Patients without two known CFTR mutations cannot qualify for CFTR modulators. Eligibility for CFTR modulators also affected lung function, as measured by forced expiratory volume in one second (FEV1). Patients across groups who were ineligible for these CFTR modulators had the lowest FEV1 measures, meaning worse lung function. The researchers went on to note that besides being less eligible for CFTR modulator therapy, minorities tend to be under-represented in pharmaceutical trials. This issue will grow as the CF community’s demographics continue to change.
https://tinyurl.com/yar7o628

Bronchoscopy Plus BAL Safe Way Of Checking Lung Health Of Patients
Using bronchoscopy to examine the airways and the surrounding tissues of people with cystic fibrosis, coupled with the collecting of lung fluid through bronchoalveolar lavage (BAL), is safe and a reasonable alternative when sputum samples are not possible, a study reports. In bronchoscopy, a thin tube (bronchoscope) through is inserted through the nose or mouth, then down the throat and into the lungs. It is used in CF research to identify immune cells and microorganisms in alveoli (air sacs of the lungs) to study airway inflammation. BAL consists of injecting a sterile saline solution through the bronchoscope into alveoli, and then collecting the fluid, along with cells, for analysis. It has been shown to be useful in helping predict pulmonary exacerbations in children with CF, and in microbiologic sampling of patients whose sputum is scarce due to the use of newer CF modulator therapies. Chronic lung inflammation remains a problem in CF. To identify novel therapeutic targets to combat CF lung inflammation, the use of primary immune cells is critical. Therefore, research bronchoscopy with BAL is a highly useful tool to facilitate the development of improved therapies to target inflammation. The study noted that throat swaps that might be used when a sputum sample cannot be collected lack sensitivity in detecting Pseudomonas aeruginosa and other potentially harmful bacteria. Sore throat and cough were the most common side effect among CF patients. Fever and headaches were also reported. Most patients with a fever and headaches also had poorer lung function, with predicted FEV1 values lower than 65%.
https://tinyurl.com/y8gm89j2

Sinus Surgery Can Be Helpful For Those With Poorer Lung Function
Endoscopic sinus surgery led to better lung function in people with moderate or severe cystic fibrosis, and some with mild disease, with benefits sustained one year after surgery, a medical records review found. These findings support the role of sinus surgery in improving lung health, especially for patients with the greatest need. Sinus disease affects about one-fifth of those under the age of 18 and over half of adult patients. Studies indicate nearly all people with CF have clinical findings consistent with chronic rhinosinusitis (CRS), or inflammation of the nasal cavity and sinuses. Nasal polyps can also develop. Endoscopic sinus surgery (ESS) is a procedure for treating sinus disease. The main goals of ESS are to remove polyps and irrigate out excess mucus, and to reduce inflammation and the number of bacteria. It is also done as a preventative measure for lung transplant candidates, as the sinuses are a likely source of infectious bacteria. Prior studies examining the impact of ESS on lung function tests report conflicting results. The majority compared pre- and post-operative lung test outcomes without accounting for factors that may influence the results, such as different disease severity levels or a person’s use of disease-modifying therapies. Improvements in percent predicted forced expiratory volume in one second was the primary goal. Mild lung disease was defined as a %FEV1 higher than 70%, 40% to 70% as moderate disease, and less than 40% as severe lung disease. Lung function was assessed 90 days before surgery, and for one year after ESS. Results of the adjusted model showed the effect of ESS varied by the severity of lung disease. The %FEV1 significantly increased by 8.1% among patients with severe lung disease, and by 3.0% in those with moderate lung disease. A 7.3% increase in lung function was also seen in a subset of mild lung disease patients. Among patients with better lung function after ESS, the mean improvement was sustained one year after surgery. The ESS effect did not vary by CFTR mutation, age, whether it was the first or second surgery, or whether the surgery was performed alone or with bronchoalveolar lavage. These results support the role of ESS in improving pulmonary status in the CF population.
https://tinyurl.com/yj7no9s6

CF Antibiotic Gentamicin Does Not Need To Cause Deafness, Study Finds
Scientists discovered the subtypes of gentamicin that are more likely to cause deafness and created a new formulation that is as effective at eliminating bacteria but much safer for patients. The team is planning to write to the U.S. Food and Drug Administration (FDA), recommending the agency to change its requirements on the way gentamicin is manufactured, as current instructions are making people go deaf. Statistics indicate that up to 20% of gentamicin-treated patients will have some degree of irreversible hearing loss. Nowadays, the gentamicin given in hospitals is a mixture of five different antibiotic subtypes containing up to 10% of impurities. Investigators used a series of techniques to separate the different compounds in gentamicin’s composition and figure out which were responsible for the antibiotic’s ototoxic properties (properties toxic to ear tissues). After establishing a protocol that enabled them to separate each component of the mixture, they proceeded to test how each gentamicin subtype affected the inner-ear tissues of animals. Researchers discovered that gentamicin C2 (sisomicin) was the most toxic of the five antibiotic subtypes, while gentamicin C2b was the least ototoxic. Although both antibiotic subtypes had different levels of toxicity, their antimicrobial properties were identical and were found to be comparable to that of gentamicin’s original mixture. Ototoxicity could be further reduced by eliminating other impurities from gentamicin’s mixture. Since all gentamicin subtypes are approved for use by the FDA, it is likely that less toxic formulations would not have to be retested, allowing them to be quickly brought into clinical practice. Both individual and reformulated mixtures of C-subtypes demonstrated decreased ototoxicity while maintaining antimicrobial activity, thereby serving as a proof-of-concept of drug reformulation to minimizing ototoxicity of gentamicin in patients. The team also noted that the ototoxic properties of each gentamicin subtype seemed to be associated with the way each of them interacted with specific channels within the inner ear. More specifically, they discovered that the antibiotic subtypes that bound most strongly to these channels tended to be the least toxic. Researchers are now planning to create a new aminoglycoside that could further lower the risk of deafness.
https://tinyurl.com/y7edasqz

Posaconazole Vs Voriconazole For Primary Treatment Of Invasive Aspergillosis: A Phase 3, Randomised, Controlled, Non-Inferiority Trial
Researchers undertook this randomised, prospective, double-blind, double-dummy, controlled trial to evaluate non-inferiority of posaconazole to voriconazole for the primary treatment of invasive aspergillosis. Posaconazole (intravenous or oral posaconazole 300 mg twice on day 1, followed by 300 mg once a day for days 2–84) was compared with voriconazole (6 mg/kg intravenous or 300 mg oral twice on day 1 followed by 4 mg/kg intravenously or 200 mg orally twice a day for days 2–84) for 12 weeks or less. Findings demonstrated the non-inferiority of posaconazole to voriconazole with respect to all-cause mortality up until day 42 in patients suffering from invasive aspergillosis. Good tolerability of posaconazole was reported, and fewer treatment-related adverse events were experienced by patients vs in the voriconazole group. Thus, posaconazole use as a first-line treatment for the condition is supported.
https://tinyurl.com/yb75zy55

Newer CF Bacteria Species Shows Antibiotic Resistance, Ability To Spread
The Achromobacter bacteria species that is an increasing source of chronic lung infections in people with cystic fibrosis (CF) shows evidence of patient-to-patient transmission and emerging antibiotic resistance. Recent studies are reporting the bacteria species Achromobacter is increasingly being detected in CF patients, and associated with antibiotic resistance and more severe disease. Little is known about the transmission of this bacteria and its antibiotic resistance among patients. So a team of researchers conducted DNA analysis on Achromobacter bacteria isolated from 51 CF patients. Their goal was to further understand how these pathogens spread and how resistant they are to treatment.  All patients received early antibiotic treatment for Achromobacter after first testing positive, with follow-up treatments based on antibiotic susceptibility testing to find the most effective therapy. In total, 182 genomes were analyzed from 101 clinical isolates of Achromobacter from the 51 patients. Before this study, all isolates included were identified specifically as the Achromobacter xylosoxidans using routine microbiology techniques. But following DNA analysis, the researchers found their collection was composed of five different Achromobacter species. These included 15 patients infected with A. ruhlandii, 12 with A. insuavis, 31 with A. xylosoxidans, and one with A. aegrifacies. Another patient was infected with a yet unknown Achromobacter species. To investigate patient-to-patient transmission, genome results were compared to identify specific bacteria with a related common ancestor (same clonal type), which was defined as bacteria that differ by less than 5,000 single-nucleotide variants (SNV) — single changes in the building blocks of DNA. More closely related bacteria within a species, which suggests close transmission, will have less genetic variation than more distantly related bacteria within the same species. The analysis found 16 suspected patient-to-patient transmission cases that were investigated further. In 12 of these cases, multiple clonal relatives were isolated from at least one of two patient pairs, and in nine of these cases, isolates from different patients were more closely related than those from the same patient. Next, a phylogenetic tree (similar to a family tree) was constructed based on the genetic relationship of the four suspected patient-to-patient transmitted clonal types — one clonal type of the A. ruhlandii species, one A. insuavis, and two from the A. xylosoxidans species. Of 14 suspected transmission cases with full phylogenetic information, 12 cases supported patient-to-patient transmission as bacteria from one patient was found to be descended from bacteria from another patient. To support this genetic evidence of transmission, researchers looked for overlap in patient visits to the clinic. Of the 16 patient pairs with suspected transmission events, all but one patient pair with microbial sampling were in the same hospital ward on the same day. The A. ruhlandii clone type AX01DK01 showed the most transmissions, being found in 27 isolates across 13 patients. To investigate antibiotic resistance, routine diagnostic measurements of antibiotic susceptibility profiles were assessed against 21 antibiotics for 92 isolates. For the 21 patients with single isolated samples, these bacteria were resistant or intermediately resistant to a median of 14 antibiotics. For 30 patients with multiple samples over time, those isolated early were resistant to a median of 14 antibiotics, while those collected later were resistant to 18 antibiotics. Statistical analysis found bacteria isolated later to be significantly less susceptible to antibiotics than early and single isolates. Nearly all isolates showed resistance or intermediate resistance to nine antibiotics; five showed some efficacy. No antibiotic was effective against all the bacteria tested.
https://tinyurl.com/yaetn6dl

Defects In Mitochondria May Sustain Burkholderia Cenocepacia Infections In CF
Specific immune cells called macrophages in cystic fibrosis are unable to kill the bacteria Burkholderia cenocepacia due to impairments in mitochondria, the cell’s powerhouses. The finding suggests that boosting mitochondria function could help resolve chronic infection in CF. Macrophages, immune cells specialized in the detection and destruction of bacteria and other harmful agents, have been shown to contribute to CF. Macrophages had an impairment in their ability to undergo autophagy, a natural cleaning system used by cells to get rid of damaged components and pathogens. This made the cells unable to clear infection by the Burkholderia cenocepacia (B. cenocepacia). Mitochondria, cells’ energy source, are particularly important for the proper function of macrophages. For an infection, healthy macrophages use their mitochondria to produce mitochondrial reactive oxygen species (mROS) that trigger inflammation and destroy bacteria. Researchers observed that mitochondria from CF macrophages worked poorly compared to mitochondria in healthy macrophages. Two indicators of mitochondrial working capacity, called maximal respiration and spare respiratory capacity, were significantly impaired in CF macrophages. This meant that when called into action, their mitochondria would have more difficulty trying to respond to an increased demand for energy. The oxygen consumption rate, another measure of mitochondria’s health, also was reduced in CF macrophages compared to healthy cells. Next, researchers looked at how B. cenocepacia affected the mitochondria of CF macrophages. Six hours after infection, the team observed that the mitochondria were smaller, less elongated and had lower interconnectivity scores, all signs suggesting that infection triggered the fragmentation of CF mitochondria. At a metabolic level researchers saw that, when infected, CF macrophages had a higher mitochondrial membrane potential compared to healthy macrophages. This meant that the metabolism in the CF macrophages changed and was more glycolysis-dependent, a more inefficient way for mitochondria to produce energy. Before infection, no significant differences in mitochondrial ROS production were found between healthy and CF macrophages. After infection, both macrophages increased their ROS levels. Yet, previous work had demonstrated that CF macrophages accumulated more bacteria due to a defective autophagy mechanism, which could mask defects in mitochondrial ROS production. Two hours after infection, CF macrophages slowed down mitochondrial ROS production and by eight hours there was a significant decrease in ROS compared to healthy macrophages. These findings suggested that mitochondria impairments affect the ability of CF macrophages to respond to infections.
https://tinyurl.com/yalnsvy2

Phage Therapy Clears Resistant Infection In CF Lung Transplant Patient
For the first time, bacteriophage therapy treated a boy with chronic and antibiotic-resistant Achromobacter bacteria infection following a double lung transplant due to cystic fibrosis. Bacteriophages (or phages) are viruses that selectively infect and kill bacteria, while being otherwise harmless. The article describes the case of a young CF patient, successfully given phage therapy for a post-transplant, antibiotic-resistant A. xylosoxidans infection.
https://tinyurl.com/ycct2hnl

Research Reveals How Bacteria Defeat Drugs That Fight Cystic Fibrosis
Researchers have discovered a slimy strategy used by bacteria to defeat antibiotics and other drugs used to combat infections in people with cystic fibrosis. A common strain of bacteria, Pseudomonas aeruginosa, often thrives in the lungs of people with cystic fibrosis. Once a P. aeruginosa infection is established, it can be incredibly difficult to cure. The research showed that the stubborn germs living in the lungs of cystic fibrosis patients create a self-produced carbohydrate slime. And this slime makes the bacteria more resistant to the antibiotics prescribed as well as drugs that reduce the thickness of mucus. They found the first direct evidence that these carbohydrates are produced at the sites of infection and showed that one of the carbohydrates, called Pel, sticks to extracellular DNA, which is abundant in the thick mucus secretions prominent in cystic fibrosis lungs. This interaction makes a slimy protective layer around the bacteria, making them harder to kill and it reduces the pathogen’s susceptibility to antibiotics and drugs aimed at reducing the thickness of airway mucus by digesting DNA. The work supports a hypothesis that it’s the carbohydrates that group, or aggregate, the bacteria in cystic fibrosis lungs. Understanding the mechanisms that promote bacterial aggregation may facilitate new therapeutic approaches aimed at digesting the carbohydrates holding bacterial cells together. The research also suggests that the carbohydrate Pel likely diminishes the efficacy of the most commonly used therapeutics for cystic fibrosis, which are inhaled antibiotics and a drug that breaks down the thickness of the airway mucus, making it easier to cough up.
https://tinyurl.com/ygkxob67

Laura is 72 and has CF. She is a former director and President of USACFA. She and her husband, Lew, live in Northville, MI.