Before she lost her battle with cystic fibrosis (CF) at the early age of 56, Muriel Lagnese had a wish—to help other patients and families overcome by high costs of care.
We established Muriel’s Breath of Life Foundation in her loving memory. The Foundation supports patients’ access to lifesaving treatments by helping to close the financial gaps in care. Listen to a short video from Muriel’s former doctor detailing ways the foundation supports current patients.
Muriel was a wife and a friend with a generous heart. She would be proud of the work that has been done by the foundation since 2010 and the vision of establishing the endowment to help needy CF patients for years to come.
What is CF?
Cystic Fibrosis is a life-shortening, inherited disorder that currently affects over 30,000 Americans. It is one of the most common inherited life threatening diseases and is present in 1 out of every 3000 live births in the United States. The cause of Cystic Fibrosis is a genetic defect that prevents cells from making healthy digestive juices, sweat and mucus. As a result, these secretions are thick and sticky causing them to clog the ducts and tubes within the pancreas and lungs. Thick mucus is difficult to clear from the lungs and sinuses causing CF patients to be highly susceptible to infections including many resistant to treatment. Decreased absorption due to defective digestive juices makes achieving adequate nutrition a challenge. Adult patients may face fertility issues as well.
Treatment for Cystic Fibrosis is intensive and requires a rigorous daily regimen. While incurable, CF is manageable and treatment is focused on preventing complications such as infections and optimizing nutrition. Medications, including antibiotics and inhalers are used to thin secretions, clear secretions and treat infections. Compression vests and various techniques and exercises aid in clearing the lungs.
For those patients with very diseased lungs who are appropriate candidates, lung transplantation may be an option. Transplanted lungs do not have Cystic Fibrosis, but the patient still has the genetic defect and is therefore not cured. While a lifesaving procedure, transplantation brings with it an additional set of issues including the threat of rejection and the side effects of immunosuppressive therapy.
The High Cost of Treatment
Out of pocket treatment costs, which average over $10,000 per year per patient, represent a great burden for many afflicted with CF. Muriel’s Breath of Life exists to ease that burden.
While an aggressive research agenda has propelled CF clinical trials and produced a number of new interventions, the treatment burden is high and the cost of some medications is prohibitive for many patients. The average out-of-pocket expense for medications and equipment for an individual with CF in the U.S. is $10,300 per year. Although most patients are insured at some level, many must make significant co-pays and bear the burden personally of some healthcare expenses that their insurance does not cover. Even with adequate insurance coverage, it can be a struggle to meet the financial demands of CF care, but those who are uninsured or underinsured suffer the most.
With escalating gas prices, travel to and from the clinic and hospital can become an onerous expense for CF patients. All too frequently, CF patients have to choose between paying for a month of medication or covering basic living expenses, such as groceries or utilities.
Adults with Cystic Fibrosis
Adulthood brings special challenges. It used to be that most children with CF died before reaching school age. Today, with the advances in diagnoses and treatment, individuals are living well into their adult years. The current median age of survival is 37.4 years and this number continues to rise. Presently, nearly 50 percent of the CF patient population is 18 years of age or older. In recent years there has also been a rise in the number of the subset of patients who are diagnosed, like Muriel, in adulthood with delayed onset disease. As today’s CF patients navigate the uncharted territory of adulthood they are facing many challenges. For example, this insidious disease can have devastating vocational, educational and economic effects. The chronic nature of the disease, the intensity of treatments, hospitalizations, and frequent infections can result in many lost work and school days. Despite the challenges, the future looks brighter for CF patients with patients being able to look forward to long productive lives that include fulfilling careers, marriage, families of their own and all the things most of us take for granted. Things that were not possible just a few decades ago. Muriel’s Breath of Life has a special focus of helping adults with Cystic Fibrosis, however, we provide aid to CF patients in need regardless of age.