Cystic fibrosis (CF) is a serious heritable disease caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene. This gene encodes for a protein involved in transporting salt across cell membranes. Mutations cause the CFTR protein to be made incorrectly, or not at all. Without proper salt transport, thick, sticky mucus builds up in organs. In the lungs, this mucus can make breathing difficult, as well as making patients more susceptible to respiratory infections.
One treatment that can help some patients with breathing problems is oxygen therapy.
What is oxygen therapy?
Oxygen therapy is a treatment in which a patient breathes pure oxygen through a mask or nose tube. A patient using oxygen therapy receives more oxygen in the lungs with each breath than he would breathing normal. Many CF patients wear an oxygen mask at night to ensure they are receiving enough oxygen while they are sleeping. Some patients use oxygen while they are exercising so they will not become oxygen-deprived. Some patients may need to have a machine in their home or take a portable oxygen tank with them when they travel.
Research about oxygen therapy for CF
Although oxygen therapy has long been prescribed to patients with CF, there have been relatively few clinical trials to determine how oxygen therapy can be used most effectively in these patients, especially over the long-term.
A summary of these clinical trial results were published as a review in the Cochrane Library. The authors compiled the results of 11 studies that included 172 CF patients. All of the studies included in the review compared the effect of low flow oxygen (such as from an oxygen tank) to that of room air. Ten of the 11 studies examined the short-term effects of oxygen therapy.
The authors concluded these studies have shown that oxygen therapy improved the ability of CF patients to exercise, fall asleep, and attend school and work regularly. However, they said more studies were needed to establish a standard of care for oxygen therapy in CF patients.
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