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Cystic fibrosis

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  • 1. PRESENTED BY: A.PRIYADHARSHINI M.Sc(N), LECTURER,JAI INSTITUTE OF NURSING AND RESEARCH, GWALIOR.
  • 2. It is an autosomal recessive geneticdisorder that affects most criticallythe lungs, and also the pancreas, liver,and intestine. It is characterized byabnormal transportof chloride and sodium across an epithelium,leading to thick, viscous secretions
  • 3.  It is most common in causcasians with an incidence of 1 in 2500 children born in the U.K. It is extremely rare in India. The incidence in migrant Indian populations in the UK and USA has been estimated to be 1 in 1000 population. Cystic fibrosis is diagnosed in males and females equally. 
  • 4.  The basic defect in CF is a mutation in the gene for chloride conductance channel i.e. cystic fibrosis transmembrane conductance regulator (CFTR). The failure of chloride conductance by epithelial cells leads to dehydration of secretions that are too viscid and difficult to clear.
  • 5.  Salty tasting skin Poor growth Poor weight gain despite a normal food intake Accumulation of thick, sticky mucus Frequent chest infections Coughing Shortness of breath  Males can be infertile due to congenital absence of the vas deferens 
  • 6.  Symptoms often appear in infancy and childhood, such as bowel obstruction due to meconium ileus in newborn babies. In rare cases, cystic fibrosis can manifest itself as a coagulation disorder.Young children are especially sensitive to vitamin K malabsorptive disorders because only a very small amount of vitamin K crosses the placenta, leaving the child with very low reserves.
  • 7.   Newborn screening Sweat testing Genetic testing
  • 8.  The newborn screen initially measures for raised blood concentration of immunoreactive trypsinogen.
  • 9.  Sweat-testing involves application of a medication that stimulates sweating (pilocarpine). The resultant sweat is then collected on filter paper or in a capillary tube and analyzed for abnormal amounts of sodium and chloride. People with CF have increased amounts of sodium and chloride in their sweat.
  • 10.  It is the direct examination of DNA molecule of the affected child..
  • 11.  No cures for cystic fibrosis there are several treatment methods. Supportive treatment has to be given based on the signs and symptoms.
  • 12.  Many CF patients are on one or more antibiotics at all times, even when healthy, to prophylactically suppress infection. Inhaled therapy with antibiotics such as tobramycin, colistin, and aztreonam is often given for months at a time to improve lung function by impeding the growth of colonized bacteria. Oral antibiotics such as ciprofloxacin or azithromycin are given to help prevent infection or to control ongoing infection.
  • 13.  Chest physiotherapy (CPT) The "Vest Airway Clearance System", a form of chest wall oscillation, is a device and system for clearing excess mucus from lung airways. (ThAIRapy Vest)  Intrapulmonary percussive ventilator (IPV) Aerosolized medications that help loosen secretions include dornase alfa and hypertonic saline.[  Bilevel positive airway pressure (BiPAP) Lung transplantation
  • 14.  Halotherapy is the use of salt vapor to treat respiratory ailments, skin irritations, and combat mental lethargy.
  • 15.  Diabetes is the most common non-pulmonary complication of CF. It mixes features of type 1 and type 2 diabetes. cystic fibrosis-related diabetes (CFRD). While oral anti-diabetic drugs are sometimes used, the only recommended treatment is the use of insulin injections or an insulin pump. Dietary restrictions are not recommended.
  • 16.  Treatment of pancreatic insufficiency by replacement of missing digestive enzymes allows the duodenum to properly absorb nutrients and vitamins that would otherwise be lost in the feces.
  • 17.  Development of osteoporosis can be prevented by increased intake of vitamin D and calcium, and can be treated by bisphosphonates.
  • 18.  Poor growth may be avoided by insertion of a feeding tube for increasing calories through supplemental feeds or by administration of injected growth hormone.
  • 19.  Female infertility may be overcome by assisted reproduction technology, particularly embryo transfer techniques. Male infertility caused by absence of the vas deferens may be overcome with testicular sperm extraction(TEST), collecting sperm cells directly from the testicles. Third party reproduction is also a possibility for women with CF.
  • 20.  The prognosis for cystic fibrosis has improved due to earlier diagnosis through screening, better treatment and access to health care.
  • 21.  Bronchiectasis Chronic infections Nasal polyps Pneumothorax Atelectasis Respiratory failure Nutritional defeciencies Diabetes Intussusception Rectal prolapse
  • 22.  Pancreatic insufficiency Infertility Osteoporosis Electrolyte imbalance
  • 23. THANK YOU