Cystic fibrosis

Cystic fibrosis Nurses CME Nursing Management Diagnosis patho-physiology treatment manifestations

1. Cystic Fibrosis - For Nurses!! Prepared by Dr. Rajesh T Eapen Burjeel Hospital Muscat

2. Outline What is cystic fibrosis (CF)? What causes CF? What are the manifestations? How do you diagnose CF? How do you treat CF?

3. Cystic Fibrosis – Overview of Disease Cystic fibrosis (CF) - inherited autosomal recessive disorder in children Most common cause of chronic lung disease in children – 30,000 children & adults in US Incidence Annually 1,000 children diagnosed Most common lethal, hereditary disorder among Caucasians 1 in 1,000 live births 70 % of patients are diagnosed by age 2 40 % of CF population is age 18 and older

4. Etiology Genetically transmitted disease Genes (DNA ) from parents Gene located on 7 th chromosome 2 copies of genes needed to inherit disease Carrier states 1 copy (National Institutes of Health)

5. About the Disease Chronic genetic disorder of the endocrine system that affects all organ systems • Respiratory • Digestive • Pancreas • Kidney • Liver • Reproductive Glands secrete thick, sticky mucus

6. Pathophysiology Defective gene Related to protein involved in chloride ion transport Body produces thick, sticky mucus Clogs the lungs Stagnant mucus Obstructs the pancreas Malabsorption & malnutrition (MedlinePlus, 2006)

7. Pathophysiology Lung High rate of sodium absorption and low rate of chloride secretion reduces salt and water content in mucus, depletes peri-ciliary liquid Mucus adheres to airway surface, leads to decreased mucus clearing Predisposition to Staph and Pseudomonas infections

8. Pathophysiology Gastrointestinal Biliary tree Retention of biliary secretion Focal biliary cirrhosis Bile duct proliferation Chronic cholecystitis , cholelithiasis Sweat Normal volume of sweat Inability to reabsorb NaCl from sweat as it passes through sweat duct

9. Symptoms Symptoms may include Meconium ileus Appears at birth Salty-tasting skin When newborn is kissed Steatorrhea Greasy, bulky and foul smelling Poor growth/weight gain in spite of good appetite Chronic coughing, at times with phlegm Frequent lung infections

10. Pulmonary System Mucus accumulates in the airways Causes obstruction and infection Over time: permanent damage to lungs Primary cause of death: respiratory insufficiency

11. Digestive System Pancreatic insufficiency: Missing enzymes that break down certain proteins Occurs in 85-90% of CF patients Causes malabsorption of proteins, fats, fat-soluble vitamins Underweight, poor growth, delayed onset of puberty

12. Manifestations Common presentations Chronic cough Recurrent pulmonary infiltrates Failure to thrive Meconium ileus

13. Manifestations Respiratory tract Chronic sinusitis Nasal obstruction Rhinorrhea Nasal polyps in 25%; often requires surgery Chronic cough Persistent Viscous, purulent, green sputum

14. Manifestations Respiratory tract Chronic cough Exacerbations require aggressive therapy Postural drainage Antibiotics Become more frequent with age Progressive loss of lung function Infection Intially with H. influenzae and S. aureus Subsequently P. aeruginosa Occassionally, Xanthomonas xylosoxidans , Burkholderia gladioli , Proteus , E. coli , Klebsiella

15. Manifestations Respiratory tract Lung function Small airway disease is first functional lung abnormality Progresses to reversible as well as irreversible changes in FEV1 Chest x-ray may show hyperinflation, mucus impaction, bronchial cuffing, bronchiectasis

16. Manifestations Respiratory tract Complications Pneumothorax ~10% of CF patients Hemoptysis Digital clubbing Cor pulmonale Respiratory failure

17. Manifestations Gastrointestinal Meconium ileus Abdominal distention Failure to pass stool Emesis Abdominal flat plate Air-fluid levels Granular appearance meconium Small colon

18. Manifestations Gastrointestinal Meconium ileus equivalent or distal intestinal obstruction syndrome RLQ pain Loss of appetite Emesis Palpable mass May be confused with appendicitis

19. Manifestations Gastrointestinal Exocrine pancreatic insufficiency Found in >90% of CF patients Protein and fat malabsorption Frequent bulky, foul-smelling stools Vitamin A, D, E, K malabsorption Sparing of pancreatic beta cells Beta cell function decreases with age Increased incidence of GI malignancy

20. Manifestations Genitourinary Late onset puberty Due to chronic lung disease and inadequate nutrition >95% of male patients with CF have azospermia due to obliteration of the vas deferens 20% of female patients with CF are infertile > 90 % of completed pregnancies produce viable infants

21. Mucous in the airways cannot be easily cleared from the lungs. Presentation of Disease

22. Presentation of Disease Colon Pancreas Sticky mucus secretion Ducts are filled with sticky mucus. Scarring of tissue.

23. IN SUMMARY

24. Diagnostic Tests Sweat Test Measures sodium or chloride in person’s sweat Two samples Ensure false-positive does not occur Not reliable on newborns Genetic Analysis Newborn with signs and symptoms may confirm diagnosis with blood test. Inherited disease Recommend checking family members and first cousins

25. Diagnosis Sweat chloride test >70 mEq /L 1-2% of patients with clinical manifestations of CF have a normal sweat chloride test

26. Treatments Aimed at relieving symptoms and complications Antibiotics Aerosolized Mucus-thinning drugs Thins secretions Easier to cough up Bronchodilators Relaxes smooth muscles in the airways

27. Treatments Bronchial airway drainage Postural drainage Oral enzymes and better nutrition High calorie diet Special vitamins & pancreatic enzymes Lung transplant Pain relievers Ibuprofen

28. Treatment Major objectives Promote clearance of secretions Control lung infection Provide adequate nutrition Prevent intestinal obstruction Investigation into therapies to restore the processing of misfolded CFTR protein

29. Treatment Lung >95% of CF patients die from complications of lung infection Breathing exercises Flutter valves Chest percussion ? Hypertonic saline aerosols

30. Treatment Lung Antibiotics Early intervention, long course, high dose Staphylococcus- Penicillin or cephalosporin Oral cipro for pseudomonas Rapid emergence of resistance Intermittent treatment (2-3 weeks), not chronic IV antibiotics for severe infections or infections resistant to orals

31. Treatment Lung Antibiotics Pseudomonas treated with two drugs with different mechanisms to prevent resistance e.g. cephalosporin + aminoglycoside Use of aerosolized antibiotics Increasing mucus clearance N -acetylcysteine not clinically helpful Long-term DNAse treatment increases time between pulmonary exacerbations

32. Treatment Lung Inhaled b -adrenergic agonists to control airway constriction No evidence of long-term benefit Oral glucocoticoids for allergic bronchopulmonary aspergillosis Studying benefits of high dose NSAID therapy for chronic inflammatory changes

33. Treatment Lung Atelectasis Chest PT + antibiotics Respiratory failure and cor pulmonale Vigorous medical management Oxygen supplementation Only effective treatment for respiratory failure is lung transplantation 2 year survival >60% with lung transplatation

34. Treatment Gastrointestinal Pancreatic enzyme replacement Replacement of fat-soluble vitamins- especially vitamin E & K Insulin for hyperglycemia Intestinal obstruction Pancreatic enzymes + osmotically active agents Distal- hypertonic radiocontrast material via enema

35. Treatment Gastrointestinal End-stage liver disease- transplantation 2 year survival rate >50% Hepatic and gallbladder complications treated as in patient without CF

36. Life Expectancy Chronic illness that ultimately results in premature death Severity: varies, affects life expectancy Mean life expectancy: 1950: 4 years 1990: 28 years 2006: 37 years Cause of death: lung disease

37. Cystic Fibrosis Prognosis improving >38% of CF patients are older than 18 13% of CF patients are older than 30 Median survival Males: 32 years Females: 29 years

38. Expected Outcomes The expected outcomes of nursing care include Family and/or child demonstrate proficiency in Providing pulmonary care Reducing pulmonary infections Developing a schedule for pulmonary cares that fits into family needs Adequate calories and pancreatic enzymes are consumed to support growth of the child to stay within developmental weight ranges

39. High calorie diet Recommendations: 120-150% RDA 35-40% calories from fat Enzymes with every meal and snack Vitamins, supplements Tube feedings at night Dietary Regimen

40. Parent and Child Education Respiratory infections Avoid exposure Chest percussion & postural drainage Diet Community resources Genetic counseling Written information Home care Support services

41. Common Nursing Diagnoses Ineffective airway clearance r/t tracheobronchial secretions and obstruction Imbalanced nutrition: less than body requirements r/t inability to digest food or absorb nutrients Risk for infection r/t chronic pulmonary disease Interrupted family processes r/t chronic illness

42. Planning & Interventions Provide respiratory therapy Administer medications Meet nutritional needs Provide psychosocial support Discharge planning and home teaching

43. NURSING CARE PLAN 1 Nursing diagnosis : Ineffective airway clearance related to excess tenacious mucus. Expected outcome : To improve pulmonary, GI and pancreatic status.

44. Nursing Intervention Rational Encourage regular exercise Exercise helps maintain physical wellness and supplements the patients airways clearance strategies by helping to loosen pulmonary secretion. Administer oxygen therapy and receive digoxin and/or diuretics To decrease the lungs work. Encourage coughing, deep breathing exercise and frequent position changes Promote lungs expansion, mobilization, and drainage of secretions. Monitor respiratory rate, depth and work of breathing Respiratory may remain somewhat rapid because hyperthyroid state, but development of respiratory distress is indicative of tracheal compression from edema or hemorrhage. Assist with repositioning, deep breathing exercises, and coughing as indicated Maintains clear airway and ventilation.

45. NURSING CARE PLAN 2 Nursing diagnosis : Imbalanced nutrition related to poor appetite and increased calorie expenditure. Expected outcome : The patient’s weight will be stable at desired weight for height

46. Nursing Intervention Rational Monitor food intake and weekly weight. Regular monitoring can help identify nutriton problem that are severe. If the patient is too dyspneic to eat, schedule rest period s and bronchodilator treatments before meals. Eating takes a lot of energy and resting can help conserve energy before meal. Create a pleasant eating environment. Unpleasant views or odors can spoil an appetite. Provide smaller and more frequent meals of the patient’s favourite food. Eating a lot at one time can fill up the stomach and reduce the room for lung. Encourage family members to bring favourite foods from home for hospitalized patient. A large tray of unappetizing food may be more than a patient can handle and may spoil the appetite.

47. NURSING CARE PLAN 3 Nursing diagnosis : A nxiety related to dyspnea . Expected outcome : The patient will state anxiety is controlled. The patient will be able to use technique to control dyspnea and anxiety when they occur .

48. Nursing Intervention Rational Remain with the patient who is acutely dyspneic and anxious. Feeling alone during episodes of dyspnea can increased anxiety. Calmly remind the patient to breath slowly in through the nose and out through pursed lips. During acute episodes of dyspnea , the patient may forget that breathing exercises can help. Teach deep breathing exercise s during times when anxiety is minimal and remind the patient to use them during acute anxiety. Relaxation can help reduce muscle tension and distract the patient. Administer anti anxiety medication. Medication can reduce anxiety but can also depress respiration, so should be use with caution.

49. Summary CF is an inherited monogenic disorder presenting as a multisystem disease Pathophysiology is related to abnormal ion transportation across epithelia Respiratory, GI and GU manifestations Treatment is currently preventative and supportive

50. Thank You