Unmet medical need faced by people with rare, progressive and serious diseases
In preparation for a special hearing on Oct. 26, 2023 the Senate Committee on Aging requested stories from patients with rare diseases. Of the 350 submissions received, PF Warriors is one of the relatively few patient organizations providing a story that will be published in the Congressional Record. Here is PF Warriors response.
October 25, 2023
Senate Special Committee on Aging
Senator Mike Braun, Ranking Member
Dear Honorable Senator Braun and the Senate Special Committee on Aging:
We are the PF Warriors patient and caregiver support organization representing thousands of Americans suffering from debilitating and life-threatening respiratory scarring, also known as pulmonary fibrosis (PF). Thank you for your invitation to share our story and the plight of our patients who have no disease halting or life-saving therapy.
Pulmonary Fibrosis is considered a rare disease that afflicts 200,000 people in the U.S. By its definition, the disease is scarring of the lungs. The scarring inhibits normal breathing and ultimately destroys the lungs, a process that is progressive, irreversible, and almost invariably deadly. The median survival
ranges between 2-5 years. The average age of our patients is 65 for sporadic cases and 10 years younger for genetic ones.
Drugs, devices and innovative options are desperately needed and yet research dollars are sorely lacking given the increasing incidence and prevalence and the desperation and the excruciating nature of the
disease that ultimately causes suffocation and death.
Our patients are from all walks of American life. And at least one of our patients was a member of Congress. Rep. Charlie Norwood from Augusta, GA introduced the first legislation for PF before he died. Our patients are veterans of our military who were exposed to Agent Orange, burn pits and particulate matter in various parts of the world. They are 9/11 first responders, coal miners, construction workers, pilots, and firefighters among other professions. The largest increases in cases appear to be in severe
COVID and long COVID patients. Most of our patients are forced to give up their careers, livelihoods, favorite activities, family time, pastimes and dreams. Breathing is difficult and it worsens with activity. Over the past 15 years, not only have more people been diagnosed than ever, the amount of benefits our patients receive from Medicare continues to decline including lack of access to oxygen and related equipment as well as pulmonary rehabilitation. So, not only can our patients not catch their breath, they can’t catch a break, either.
Shelly Mathis is the director of educational programming for the PF Warriors and the wife of PF patient, Scott Calhoun. Shelly discovered the PF Warriors organization when her husband was diagnosed and soon joined the team of the organization. Both volunteers, Scott also helps the organization that provides support services for patients and their caregivers.
Traveling is difficult for Shelly and Scott because Scott needs a significant amount of oxygen 24-hours-a-day. Like most respiratory patients in America, he struggles to get the oxygen supplies he needs to be able to remain active since the more he moves, the more oxygen his body needs and the more
supplemental oxygen supply he requires. They noticed on a trip outside of the country that gaining access to oxygen supplies was significantly easier, more accessible and the tools for oxygen use were much improved.
Scott participated in a pulmonary rehabilitation program not long after being diagnosed. He was able to learn how to live and breathe better and more efficiently. Because of Medicare cuts to pulmonary rehab for PF and some other respiratory patients, though Scott has a doctor’s order for more rehab, he will have a difficult time accessing it because of lack of reimbursement for providers along with vast increases in patients requiring oxygen support and suffering from respiratory issues since the pandemic driving increased demand.
For a lucky approximately 2,000 patients in the U.S. suffering from life-threatening respiratory disease and damage, including PF, lung transplantation is an option. For Scott, who is 74, the odds are stacked
against him and even for younger PF patients. Recent changes to the lung transplant organ distribution system appear to seemingly discriminate against people over 65 who now may have decreased access to the only life-saving option since the new system went into effect in March of this year. Arbitrary estimations of how long a patient will live post-transplant are applied to individual patients – to their detriment.
One additional example of issues regarding Medicare support for respiratory patients is the not-so-recent letter issued by FDA warning about inconsistent pulse oximeter readings from non-FDA approved devices. And yet Medicare has taken no action to provide these higher-priced and hard-to-find approved
devices to patients requiring oxygen, thus subjecting them to damage in other organ systems as well as further damage in the lungs due to hypoxemia.
PF patients are not just struggling to breathe, they are struggling against a broken system. The tools they need are difficult to get and harder to maintain due to unreliable oxygen equipment systems that are damaged because of deep cuts by Medicare. They are struggling because there are no therapies
available that can improve their lungs or save them from this nightmare of a disease. The only two drugs that have ever been approved for the disease were approved almost a decade ago. Though modestly effective at stalling progression, most PF patients are not on them – due to lack of tolerability
of side effects and the six-figure price tags. There have been more than 25 failed drug trials for PF here in the U.S. alone and still we have no life-improving therapies.
PF Warriors appreciates the opportunity to share Shelly and Scott’s stories that reflect a dimming reality for our patients.
Please Congress, help move legislation that can increase innovation in the respiratory space, especially in pulmonary fibrosis. Please ask Medicare to stop the cuts and improve the access for our patients to life sustaining oxygen and oxygen equipment. And please consider a funding option such as a common fund request to ARPA-H / NIH for a desperately needed focus on fibrosis and respiratory failure.
Sincerely,
Teresa Barnes, Chief Executive Warrior, PF Warriors
Dolly Kervitsky, RCP, CCRC, CNP, President, PF Warriors
Shelly Mathis, Interim Director, PF Warriors and Scott Calhoun
CC: PF Warriors Staff, Patients and Caregivers
United States Senate Special Commission on Aging website
My Life with Pulmonary Fibrosis – Scott and Shelly 5 min. video
