Focus on Cystic Fibrosis. Cystic Fibrosis. Cystic Fibrosis

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1 Focus on (Relates to Chapter 29, Nursing Management: Obstructive Pulmonary Diseases, in the textbook) Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. Autosomal recessive, multisystem disease with altered function in exocrine glands of Lungs Pancreas Sweat glands Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 2 Abnormally thick mucus can lead to chronic diffusive, obstructive pulmonary disorder. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 3 1

2 Occurs primarily in whites First signs and symptoms usually occur in children Median life span is more than 37 years. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 4 CF results from mutations in a gene located on chromosome 7. Alters ionic transport of sodium and chloride in the sweat Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 5 Obstruction of ducts of the exocrine system is caused by thick, viscous secretions. Adheres to lumen of the ducts, which eventually undergo fibrosis Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 6 2

3 Upper respiratory tract infections may present and include Chronic sinusitis Nasal polyposis Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 7 Thick secretions obstruct bronchioles, leading to air trapping and hyperinflation. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 8 Most common organisms cultured are Staphylococcus aureus Haemophilus influenzae Burkholderia cepacia Pseudomonas aeruginosa Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 9 3

4 Progressive loss of lung tissue from inflammation and scarring Resultant chronic hypoxia leads to pulmonary hypertension and cor pulmonale. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 10 Blebs and large cysts in lungs are severe manifestations of destruction. Complications include Hemoptysis (which can be fatal) Pneumothorax Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 11 Death usually results from loss of pulmonary function. Exocrine function of pancreas is altered or may be lost completely. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 12 4

5 Enzymes trypsinogen, lipase, and amylase do not reach the intestine to digest ingested nutrients. Endocrine function may also be affected by advanced pancreatic insufficiency. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 13 Fat, protein, and fat soluble vitamins are malabsorbed. Results in steatorrhea Protein malabsorption results in failure to grow and gain weight. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc % to 43% of adult CF patients have related diabetes mellitus. Osteopenia and osteoporosis are common. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 15 5

6 Many also have GI problems. GERD DIOS (distal intestinal obstructive syndrome) Gallstones Constipation Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 16 Biliary cirrhosis may not be detected until late. Chronic cholestasis, inflammation, fibrosis, and portal hypotension can occur. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 17 Clinical Manifestations Early manifestations Failure to grow Clubbing Persistent cough with mucous production Tachypnea Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 18 6

7 Clinical Manifestations Early manifestations Large, frequent bowel movements Large, protuberant abdomen with emaciated appearance of extremities Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 19 Clinical Manifestations First symptom in adult is usually frequent cough. Becomes viscous and purulent Produces greenish colored sputum Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 20 Clinical Manifestations Recurrent respiratory problems may be indicative of CF. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 21 7

8 Clinical Manifestations As disease progresses Exacerbations of cough Weight loss Sputum In pulmonary function Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 22 Clinical Manifestations Exacerbations become more frequent, and recovery of lost lung is less complete. Ultimately results in respiratory failure Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 23 Clinical Manifestations Distal intestinal obstruction causes Right lower quadrant pain Loss of appetite Emesis Palpable mass Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 24 8

9 Clinical Manifestations Reproductive system altered Males usually sterile Females usually Have delayed menarche Report menstrual irregularities May be unable to become pregnant Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 25 Complications Pneumothorax is common (>10% of patients). Hemoptysis (can be life threatening) Digital clubbing CFRD Bone disease Liver disease Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 26 Complications Late complications Respiratory failure Cor pulmonale Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 27 9

10 Diagnostic Studies Sweat chloride test with pilocarpine iontophoresis method Pilocarpine carried by electric current is used to stimulate sweat production. Values >60 meq/l for both sodium and chloride suggest CF. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 28 Diagnostic Studies Genetic test Chest x ray Pulmonary function tests Fecal analysis for fat Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 29 Diagnostic Studies Duodenoscopy for quantitative determination of pancreatic enzymes Fetal diagnosis by amniocentesis or chorionic villus sampling Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc

11 Collaborative Care Promote clearance of secretions. Control infection in the lungs. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 31 Collaborative Care Provide adequate nutrition. Manage pulmonary problems (e.g., relieving airway obstruction). Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 32 Collaborative Care Aerosol and nebulization treatments of medications to liquefy mucus and to facilitate coughing Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc

12 Collaborative Care Airway clearance techniques CPT Postural drainage PEP devices Breathing exercises High frequency chest wall oscillation systems Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 34 Flutter Device Fig Flutter mucus clearance device is a small hand-held device that provides positive expiratory pressure (PEP) therapy. It is used to facilitate removal of mucus from the lungs. A, It consists of a hard -plastic mouthpiece, a plastic perforated cover, and a high-density stainless steel ball resting in a circular cone. B, The Flutter effects occur during expiration. Before exhalation, the ball blocks the conical canal of the Flutter. During exhalation, the position of the ball is the result of an equilibrium between the pressure of the exhaled air, the force of gravity on the ball, and the angle of the cone where contact with the ball occurs. As the steel ball rolls and moves up and down, it creates an opening and closing cycle that repeats itself many times throughout each exhalation. The net result is that vibrations occur in the airways resulting in the fluttering sensation. C, These vibrations loosen mucus from the airway walls and facilitate their movement up the airways. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 35 Acapella Fig Acapella. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc

13 Collaborative Care Aerobic exercise can be effective in clearing airways. Frequent rest periods are needed. Increased nutritional demands of exercise (including liquids) must be met. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 37 Collaborative Care Early intervention with antibiotics when lungs become infected Long courses of antibiotics are usual treatment. Drugs are abnormally metabolized and quickly excreted. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 38 Collaborative Care Sclerosing indicated for recurrent pneumothorax Lung transplants Replacement of pancreatic enzymes and supplements Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc

14 Nursing Management Nursing Assessment Health history Respiratory infections, cough with sputum Family history Dietary intolerances, weight loss Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 40 Nursing Management Nursing Assessment Health history Large, frequent bowel movements Decreased activity tolerance, dyspnea Abdominal pain Anxiety, depression Cyanosis, salty skin Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 41 Nursing Management Nursing Assessment Health history Runny nose Diminished breath sounds Hemoptysis Use of accessory muscles Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc

15 Nursing Management Nursing Assessment Health history Tachycardia Foul, fatty stools Abdominal distention Abnormal ABGs X ray Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 43 Nursing Management Nursing Diagnoses Ineffective airway clearance Ineffective breathing pattern Impaired gas exchange Imbalanced nutrition: Less than body requirements Ineffective coping Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 44 Nursing Management Planning Adequate airway clearance Reduced risk factors associated with respiratory infection Adequate nutritional support Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc

16 Nursing Management Planning Ability to perform ADLs No complications related to CF Active participation in planning and implementing a therapeutic regimen Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 46 Nursing Management Nursing Implementation Help patient assume responsibility for career and goals. Discuss altered sexuality. Provide relief of bronchoconstriction, airway obstruction, and airflow limitation. Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc. 47 Nursing Management Nursing Implementation Aggressive CPT, antibiotics, O 2 therapy, and corticosteroids Adequate nutrition Postural drainage with percussion and vibration Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc

17 Nursing Management Nursing Implementation Aerosol nebulization therapy Controlled coughing techniques Deep breathing exercises Progressive exercise conditioning Copyright 2011, 2007 by Mosby, Inc., an affiliate of Elsevier Inc

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