LYCOS RETRIEVER 
Cystic Fibrosis: Patients
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Cystic fibrosis occurs in about 1 in 2,500 births in Victoria. All patients are seen at the Royal Children's Hospital at the time of diagnosis, which is usually in the first year of life. Some patients living in the south-east of the city or eastern Victoria are cared for jointly by the department of Thoracic Medicine at the RCH and the Department of Paediatric Respiratory Medicine, MMC.
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Cystic fibrosis occurs in about 1 in 2,000 live births among white children and at a far lower rate in black and Asian children. Boys and girls get cystic fibrosis equally. At present, only 50% of those diagnosed with CF as infants or young children live beyond their late twenties. However, that percentage is rising as an increasing number of patients are only mildly affected and survive into adult life.
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RESULTS: Age and sex adjusted standard deviation scores (SDS) for height and weight were consistently higher in children screened for cystic fibrosis than in those born before screening. At 10 years of age, average differences in SDS between groups were 0.4 (95% CI -0.1, 0.8) for weight and 0.3 (95% CI -0.1, 0.7) for height. This translates to an average difference of about 2.7 cm in height and 1.7 kg in weight. Mean FEV1 and FVC (as percentage predicted) were significantly higher in the screened cohort at 5 and 10 years of age, with an average difference of 9.4% FEV1 (95% CI 0.8, 17.9) and 8.4% FVC (95% CI 1.8, 15.0) at 10 years. Chest x-ray scores were not different between the groups at any age, but by 10 years screened patients scored an average 5.3 (95% CI 1.2, 9.4) points higher on the Shwachman score.
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Due to advances in medical treatment, the median life expectancy of a newborn with cystic fibrosis increased from 4 years (in the 1960s) to 32 years today. It is not unheard of to have patients, or PWCF (Person with Cystic Fibrosis) reaching ages of 40 or 50+. Modern treatment now includes the intake of digestionenzymes, nutritional supplements, percussion and postural drainage of the lungs, improved antibiotics and inhalation of aerosols containing medication. A few attempts at gene therapywere initially successful, but failed to produce acceptable long-term results.
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Cystic fibrosis is a genetic disorder that affects the digestive system and lungs. It causes children to be more likely to have infections in their lungs repeatedly. This happens because there is a disruption in the normal function of the body's epithelial cells. These cells line passageways in the liver, lungs, pancreas, reproductive, and digestive systems. The skin's sweat glands are ... made up of epithelial cells. Patients with cystic fibrosis continuously have an excessive buildup of mucus in the lungs and passageways.
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Cystic fibrosis patients with diabetes produce some insulin on their own, but they require daily injections to boost their levels when eating so they can properly use sugar and other food nutrients for energy. At times they ... become very resistant to the insulin they do make, similar to people with type 2 diabetes.
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