A New Era in Cystic Fibrosis: Australian Surfers, and Diabetes Care
By Cindy Eshelman, RN
Cystic Fibrosis (CF), a disease of the exocrine gland system, is one of the most common fatal genetic diseases. In CF (sometimes called Mucoviscidosis), some body secretions are thicker than normal, thus interfering with the physiological processes that involve them.
One product of the exocrine glands is mucous. In CF, mucous is thick like sludge. In the gastrointestinal tract, this buildup of sludge interferes with the secretion and transport of digestive enzymes, making absorption of needed nutrients almost impossible. In the lungs, thick mucous clogs structures, leading to chronic infections and damage to lung tissue
In 1966 the median age of a patient with CF was 7.5 years; by 1990 it had increased to 27.6 years. Use of dietary enzymes, dedicated lung cares, and aggressive treatment of infections have helped CF patients live longer. As a result, new complications and new opportunities are appearing
Salt Spray on the Beach Becomes Hypertonic Saline Nebulization
Some Australian surfers with CF recently told their doctors that their airways felt clearer after exposure to the salt spray. The Australian CF Team noted that there was evidence that in CF, salt is depleted from the liquid that lines the airways. This led them to the idea that sterile salt water might be a useful therapy. A study of therapeutic saline inhalation (the largest CF clinical trail ever conducted in Australia) showed some very beneficial results.
With the use of 7% hypertonic saline nebulizations twice a day, patients had fewer pulmonary “flare-ups” and when “flare-ups” occurred, they recovered swiftly. This treatment has now been approved by the CF Foundation for use in patients who are 6 years of age or older, whose pulmonary function test (PFT) results meet prescribed parameters.
At Children’s our protocol for use includes a physician order and initial therapy in the PFT lab using before-and-after pulmonary function tests to assess airway reactivity. This is a prerequisite because hypertonic saline can irritate the airways. Some people have experienced more coughing, sore throat and chest tightness from the therapy.
Hypertonic saline is a ripe breeding ground for bacteria if not prepared under sterile conditions. Patients should only use saline prepared by a pharmacy.
Just exactly how hypertonic saline works is currently unknown. Further study needs to be done to determine if it is safe to use on sicker patients and those under 6 years of age.
Cystic Fibrosis-Related Diabetes: A Consequence of Good Care
As a person with CF gets older, more and more areas of the pancreas become clogged until in some cases the pancreas functions only intermittently or not at all. The plugged ducts form cysts, and the blood supply is lost to those areas of the pancreas, compromising its ability to produce insulin. Pancreatic insufficiency can vary from impaired glucose tolerance during stress to diabetes with elevated fasting blood sugars. CF-Related Diabetes (CFRD) now occurs in 10-20% of patients with CF.
Young CF patients need to grow; controlling their blood sugars by limiting their diets does not work. Exogenous insulin is needed to maintain weight and keep blood sugars within normal limits. Keeping their diabetes in good control enhances their ability to maintain lung function.
Helping adolescents and young adults add diabetes management to their already complicated treatment plan is a real challenge. It can be a very hard sell to adolescents who value their independence and sense of infallibility, especially young people with CF whose lives have always included daily medical treatment. The challenge foe the nurse caring for patients with CFRD is to educate and help them incorporate diabetes care into their lives.
Cindy Eshelman, RN, is clinical nurse educator for 6th Floor Medical/Surgical.
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