Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses and sex organs. CF causes your mucus to be thick and sticky. The mucus clogs the lungs, causing breathing problems and making it easy for bacteria to grow. This can lead to problems such as repeated lung infections and lung damage.

The symptoms and severity of CF vary widely. Some people have serious problems from birth. Others have a milder version of the disease that doesn't show up until they are teens or young adults.

Although there is no cure for CF, treatments have improved greatly in recent years. Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, people with CF live, on average, to be more than 35 years old.

National Heart, Lung, and Blood Institute

Cystic Fibrosis on Wikipedia

'''Cystic fibrosis''' ('''CF'''), also called '''mucoviscidosis''', is an autosome recessive hereditary disease that affects the lungs, sweat glands and the digestive system.

Symptoms

The symptoms of CF usually develop during early childhood. Both lungs and pancreas produce abnormally viscous mucus. This mucus begins to build up and starts to clog the opening to the pancreas and the lungs. The mucus in the lungs can become a growth medium for bacteria, resulting in chronic respiratory infections and eventual permanent damage to the lung tissue. As lung function deteriorates, CF patients develop pulmonary hypertension and eventually cor pulmonale. Death usually occurs from severe infection or heart failure. These thick secretions also obstruct the pancreas, preventing digestive enzymes from reaching the intestines to help break down and absorb food. The disease can be diagnosed by symptoms such as a high salt concentration in a baby's sweat or by genetic testing. Males are also frequently sterile because of the obstruction or absence of the vas deferens.

History and statistics

Cystic fibrosis (CF) was first described as a disease in the late 1930s. It is the most common genetic disease among people with European ancestry. Approximately one in every 25 people of European descent is a carrier for the cystic fibrosis gene, having one normal gene and one CF gene. Since cystic fibrosis is recessive, both copies of the gene have to be CF genes to cause the symptoms that occur in about 1 in every 2500 children. The high incidence of this lethal gene can be explained by the fact that CF carriers, who don't show any symptoms, enjoy some protection against cholera, since the extreme water loss in the intestines is prevented. People from areas where cholera is not a problem show a much lower incidence of CF. Genetic counseling and genetic testing is recommended for families who may be carriers ... [ Read More ]

External Resources

Alice Martineau - Official site. Photos, forum, her music, and information about cystic fibrosis, from which she died.

The Telegraph: Alice Martineau - 'Pop singer dies after valiant battle with cystic fibrosis'. Includes details of her daily routine of oxygen, chest physiotherapy, intravenous antibiotics and taking 40 pills a day.

Hanson Music House - Supports fans with Cystic Fibrosis. Lyrics, fun, links and campaigns.

Cff.org: Special People - Salutes Cochran for her support of the Cystic Fibrosis Foundation. (April 2001)

HHCS Health Group - A full-service pharmacy, disease management and medical management company encompassing the cystic fibrosis pharmacy, medi-paws pet pharmacy, and a vitamin and supplements pharmacy program for homeopathic medicine.

Howard Hughes Medical Institute - HHMI investigators have been involved in many important recent advances, from the discovery of genes related to cystic fibrosis, obesity, and other diseases, to new insights about how human cells develop or communicate.

Genentech - Uses human genetic information to develop and manufacture pharmaceuticals such as growth hormones, tissue-plasminogen activators to dissolve blood clots, and cystic fibrosis therapeutics.

Thorpe, P. H. - Studies of Cystic Fibrosis gene therapy. From the University of Edinburgh in Scotland.

Proteome Systems Ltd. - Specializes in interpretation of 2-dimensional gels, and developing point-of-care diagnostics in the areas of cystic fibrosis, cancer, infectious disease and aging. Includes alpha-amylase test, and research programs, with contacts in USA, Japan and headquarters in Sydney, Australia.

Howard Hughes Medical Institute - Biomedical research, grants. HHMI investigators contributed to the discovery of genes related to diseases such as cystic fibrosis and obesity, and to basic understanding of cell development and communication. Scientists are employed by HHMI and conduct their work at HHMI facilities at leading research institutions nationwide. Web site has extensive news and information on recent research findings.

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