Cystic fibrosis (CF) is an autosomal recessive disorder of the cystic fibrosis transmembrane regulator that affects the respiratory tract, pancreas, intestines, male genital tract, hepatobiliary system, and exocrine sweat glands resulting in multisystem disease.
In 1959, the median age at death was 6 months, in 2008 it increased to 27 years, and for those born in the year 2010 it is predicted to increase to more than 50 years. This improved survival is attributed to improvement in the supportive care of patients including early diagnosis, family support, adequate nutrition, infection control, and the use of nebulized mucolytics and antimicrobials.
Pulmonary involvement in CF is the most common cause of morbidity and mortality in this group of patients; dyspnea, anxiety, cough, and pain commonly interfere with the patients’ quality-of-life. For some patients, lung transplantation offers hope of improved survival and quality-of-life. However, most will die of their disease in the hospital.
The natural history of CF without transplantation is well-established and patients and families should be prepared for the physical and emotional challenges of end-stage CF, including end-of-life decision-making, even without a precise estimation of survival time.
See reference for more information about morbidity, mortality and end-stage issues.
Adapted from Estrada-Veras J, Groninger H. Palliative care for patients with cystic fibrosis. Palliative Care Network of Wisconsin. Fast facts and concepts #265. Internet. Accessed on January 8, 2019