Cystic fibrosis
Alternative names:
mucoviscidosis
Treatment:
Early recognition of cystic fibrosis and a comprehensive, multidisciplinary treatment program can lengthen survival time and improve the quality of life. Speciality clinics for cystic fibrosis are present in many communities.
Medications include antibiotics for respiratory infections and pancreatic enzymes to replace the missing enzymes. Mucomyst may be used on occasion to thin secretions.
A recent study showed that the pain reliever ibuprofen can slow lung deterioration in some children with cystic fibrosis. The results were most dramatic in children ages 5 to 13. More research is currently being done.
Other treatments include postural drainage and chest percussion, and other breathing treatments.
Lung transplant may be considered in some cases.
New treatments include replacement of the DNAase enzyme. This is available as a medication called dornase. Genetic research is ongoing in hopes of correcting the disease by artificially inserting a "normal" gene into the person. Using retroviruses to insert operating genes into the lungs has proven effective. Research on possible methods used to correct the disorder before birth also looks promising.
Support groups:
The stress of illness can often be helped by joining a support group where members share common experiences and problems. See cystic fibrosis - support group.
Expectations (prognosis):
About half of the children with cystic fibrosis live beyond age 20. Few live beyond 35. Death occurs from pulmonary (lung) complications.
Complications:
Calling your health care provider:
Call your health care provider if symptoms develop that suggest an infant or child may have cystic fibrosis.
Call your health care provider if a person with cystic fibrosis develops new symptoms, particularly severe breathing difficulty.
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