pediatrics
Cystic Fibrosis
Prognosis for Cystic Fibrosis
The course is largely determined by the degree of pulmonary involvement. Deterioration of pulmonary function over time, generally characterized by progressive bronchiectasis, leads to debilitation and eventually increases the risk for death, usually due to a combination of Overview of Respiratory Failure and Prognosis has improved steadily over the past 5 decades, mainly because of early diagnosis and aggressive treatment before the onset of irreversible pulmonary changes. Median age at death in 2021 was 33.9 years. However, median predicted survival in the United States for children born in 2021 is age 65.6 years. Long-term survival is significantly better in patients without pancreatic insufficiency ( Prognosis reference ). Outcomes are also affected by Patients and their families deserve sensitive discussions of prognosis and preferences for care throughout the course of illness, especially if pulmonary function progressively declines. One mark of respect for patients living with CF is to ensure that they are given the information and opportunity to make life choices, including having a substantial hand in determining how and when to accept dying. When appropriate, Palliative care , including sufficient symptom management, should be offered to ensure peaceful end-of-life care. A useful strategy for the patient to consider is to accept a time-limited trial of fully aggressive treatment when needed, but to agree in advance to parameters that indicate when to stop aggressive measures (see 1.Cystic Fibrosis Foundation Patient Registry2021 Annual Data Report Bethesda, Maryland 2022 Cystic Fibrosis Foundation. Accessed October 20, 2023. 