Cystic Fibrosis

• Cystic fibrosis

Affects mucus producing (exocrine glands) Inherited autonomic recessive trait. On chromosome #7. The mucus is thick (increased viscosity) causes obstruction in small passages in organs. Sodium and Chloride is increased 2-5 times in these children.

• Resp. Changes=Reduced gas exchange from the mucus leads to hypoxia, hypercapnia and acidosis. Lots of infections, (s. aureus, h. influenzae, pseudomonas aeruginosa). Become resistant to drugs. Fibrotic areas. Pneumothorax and hemostasis. Symptoms--wheezing, dry cough, barrel shaped chest, dyspnea, cyanosis, clubbing of fingers/toes, repeated bronchitis, bronchopneumonia. Manage= prevent. Remove secretions. Antibiotics. Chest PT. Play…hang upside down!! Use O2 carefully…remember chronic hypercapnia and breathing stimulus. Hemoptysis greater than 300cc/24 hours is life threatening.
• GI changes= Secretions block the ducts lead to fibrosis. Pancreatic enzymes can not reach the duodenum. Results in: steatorrhea, azotorrhea. DM may develop. Biliary obstruction--leads to biliary cirrhosis--leads to portal hypertension. Dry mouth. Infection. Symptoms= in infants--meconium ileus. Signs of intestinal obstuction= abdominal distention, vomit, failure to pass stools, dehydration. Increased amount of frothy stools, failure to thrive related to malabsorption, thin extremities. Easy bruising, anemia. Deficiency of fat soluble vitamins(A,D,E,K). Manage= sprinkle capsules of pancreatic enzymes with meals. Diet= high calories. Vit K. TPN for failure to thrive. Salt supplements in hot weather. Reproductive changes= females--fertility inhibited. Male--mostly sterile.
• Integument changes= salty. Risk for hyperthermic conditions
  Sweat Test Stimulate sweat production and measure sweat electrolytes. Normal sweat chloride=less than 40mEq/L. Greater than 60mEq/L diagnosis of CF. Test is done twice, separately.
• Tune Up --q 6 months--chest PT--antibiotics (inhaled and IV)--prophylactic prevention of serious infections. Done at Dupont Hospital for Children. Antibiotics= The order will appear as an unsafe dose. This is anticipated with CF…but the nurse still needs to call MD to clarify. CF children metabolize the antibiotics quickly.
• Give Psychological support--refer to CF Foundation.