Cystic Fibrosis Symptoms in Adults

Cystic Fibrosis Symptoms in Adults
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Cystic fibrosis, the most common autosomal recessive inherited disease among Caucasians, was once considered a disease of children, simply because children with the disorder didn't survive to adulthood. In 2010, due to improved treatments, around 45 percent of those with CF in the United States are adults, the Merck Manual states, with a median survival age of 37. CF affects the lungs and intestines because thick secretions develop in sweat, mucus and digestive cells. Adults with CF develop additional problems with age.

Diabetes

Between 15 to 45 percent of adults with CF develop diabetes, John Heintz, M.D. of Ohio State University reports on NetWellness. CF related diabetes, known as CFRD, results from the decreased ability of the pancreas to produce insulin over time. The pancreas frequently becomes affected by mucus blockage from CF. Long term steroid use to decrease inflammation also increases the chance of developing diabetes

Fertility Issues

Up to 98 percent of men with CF are infertile, due to malformations or blockages within the reproductive system, the Merck Manual states. Men who do make sperm can sometimes become fathers by undergoing sperm aspiration, removal of the sperm directly from the testes with a needle, in conjunction with advanced reproductive technologies (ART). Female fertility issues involve thick secretions in the cervix and can be overcome with ART, but have an increased risk of pregnancy loss and maternal complications in pregnancy.

Intestinal Symptoms

Inability to absorb nutrients remains a common problem in adults with CF. Bowel obstruction from mucus in the lower end of the intestine affects 10 to 20 percent of adolescents and adults with CF, according to Merck.

Liver Symptoms

Blockages in the bile ducts leading to the liver causes liver disease in around 25 percent of adults and children with CF, according to Merck. In rare cases, liver disease progresses to cirrhosis and requires liver transplantation. Liver and lung transplants are done in some cases.

Lung Symptoms

Respiratory problems remain the leading cause of death in CF. Chronic infections, recurrent sinusitis, pneumonia, collapsed lung and right heart failure related to pulmonary hypertension, an increase in the pressure inside the pulmonary arteries due to blockages and tissue destruction. Severe lung disease can lead to a need for lung transplant. Up to 60 percent of CF patients who undergo double lung transplant survive for at least 5 years, Merck reports. Lung transplants improve quality of life in addition to extending life in people with CF.

Osteoporosis

People with CF often have malabsorption due to digestive blockages. A decrease in vitamin D absorption, along with chronic corticosteroid use, causes osteoporosis, low bone density, in nearly all adults with CF, Dr. Heintz reports.

References

Article reviewed by Rachel Mattison Last updated on: Apr 19, 2010

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