Bronchiectasis develops early in the course of cystic fibrosis, being detectable in infants as young as 10 weeks of age, and is persistent and progressive. Recent data reveal that neutrophil elastase activity in BAL fluid in early life is associated with early bronchiectasis in children with cystic fibrosis.
When we look up at the sky on a fine cloudless night, we see hundreds upon hundreds of twinkling points of light, some faint, others much brighter. We can see them best standing on a hill or high ground in the country; in towns, because of the smoke and the bright lights, we cannot see so many. Though most of these starts seem to flicker and twinkle, there are a few which are steady. These are not really stars but planets, quite near when compared with the stars, which are far away in space.
Systic fibrosis is an inherited autosomal recessive disease in which abnormally thick mucus affects the reproductive, gastrointestinal, and respiratory tracts. Cystic fibrosis causes respiratory disease, characterized by chronic infection, obstructive airway disease, and progressive decline in lung function. Death is most commonly the result of respiratory failure. The life expectancy of patients with cystic fibrosis has increased to the mid-30s as a result of advances in treatment. Adult palliative care providers will be caring for more patient with cystic fibrosis as life expectancy of these patients continues to increase.
Hospice is an all-encompassing service available for patients with a prognosis under 6 months. Under the Medicare hospice benefit, patients eligible for hospice are greater than 65 years or receiving Medicare disability payments. At the start of care, two physicians must sign a statement certifying that the patient’s life expectancy is six months or less based on their best estimate of the patient’s medical prognosis. While hospice benefit was originally designed for Medicare recipients, most insurance providers cover hospice care for patients not eligible for Medicare. But hospice eligibility depends on more than only a physician determining a prognosis of six months or less until death.