Cystic Fibrosis Or Respiratory Failure?

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Cystic fibrosis is an inherited autosomal recessive disorder in children that affects the lungs and digestive system of children. “Cystic fibrosis is the most common cause of chronic lung disease in children and young adults, and the most common fatal hereditary disorder affecting Caucasians in the US” (MedlinePlus, 2006, Para 1). In the United States some 30,000 children and adults have CF. In the US, there is approximately 1,000 new cases of cystic fibrosis diagnosed each year and 70% of patients are diagnosed with CF by the age of two, 40% of patients with CF are 18 or older and in 2006 the median age of survival was 37 years (Cystic Fibrosis Foundation, 2007). Respiratory failure is a consequence of CF and is usually the cause of death.…show more content…
The procedure involves placing a small amount of a sweat producing chemical to the arm or leg. An electrode stimulates a weak and painless impulse, which causes a tingling sensation. Sweat is collected after a few minutes and analyzed by lab. To ensure that a false-positive or false-negative result has not happened two separate tests are taken on the same day. If the individual has an unusually high level of salt on the test that is indicative of CF. The sweat test is not useful in newborns as babies do not produce enough sweat to confirm the diagnosis. Physicians will wait until the newborn is a few months old to perform…show more content…
Medications are administered orally, intravenous and inhalation. Children with CF have increased clearance of many antibiotics therefore they need higher doses and longer treatments. Problems with digestion are eased with the use of pancreatic enzymes, which is individualized based on weight and any digestive problems. The goal is that the child will have almost normal appearing stools and adequate weight gain. Fat soluble vitamins (A, D, E, K) are taken to prevent deficiency. A diet high in calories calorie and well-balanced is preferred for CF children. Psychosocial support for parents and children with CF is essential as CF causes anxiety and fear in both the child and parents. Issues parents and children deal with include body image from stools and odor, frequent hospitalizations and the fatal nature of the disease process. Parents may have feelings of guilt and blame themselves for the child’s condition. Caring for a child with CF can be very expensive and draining on the family’s budget. Referring the family to social services for financial support may be necessary. Teaching on chest physiotherapy techniques and the child’s nutritional status is important. Teaching should be age appropriate and include activities to help improve lung function, endurance and airway

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