ATI template Cystic Fibrosis PDF

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Summary

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Description

ACTIVE LEARNING TEMPLATE:

System Disorder

Cruz ST UDENT NAME Delani _____________________________________ Cystic fibrosis DISORDER/DISEASE PROCESS __________________________________________________________

Alterations in Health (Diagnosis)

Pathophysiology Related to Client Problem pt inherits a defective copy of the CF gene from each parent resulting in decreased secretion of chloride and increased reabsorption of sodium and water across epithelial cells, resulting in thick, viscous secretions

ineffective airway clearance, impaired gas exchange, imbalanced nutrition

REVIEW MODULE CHAPTER ___________

Health Promotion and Disease Prevention Genetic testing should be offered to adults with a positive family history of CF and to partners of people with CF who are planning a pregnancy.

ASSESSMENT

SAFETY CONSIDERATIONS

Risk Factors

Expected Findings

If both parents are carriers, their risk of having a child with CF is one in four (25%) with each pregnancy. Most common in Caucasians.

Laboratory Tests Sweat test Fecal lipid level test Deoxyribonucleic acid testing Liver enzyme test Sputum culture Albumin level test Serum electrolyte levels Arterial blood gas analysis Fecal elastase-1 level

productive cough, wheezing, hyperinflation of the lung fields, and obstruction of the airways. pancreatic insufficiency, recurrent abdominal pain, biliary cirrhosis, vitamin deficiencies, recurrent pancreatitis, and weight loss, CF-related diabetes, and genitourinary problems (male and female infertility)

Diagnostic Procedures Genetic counseling is an important part of health care for couples at risk. The diagnosis of CF requires a clinical picture consistent with the CF phenotype and laboratory evidence of CFTR dysfunction

PATIENT-CENTERED CARE

Nursing Care Strategies that promote removal of pulmonary secretions, chest physiotherapy, and breathing exercises.

Therapeutic Procedures Chest physiotherapy, nebulization, and breathing exercises, Noninvasive positive-pressure ventilation (bilevel positive airway pressure) High-frequency chest compression via vest.

ACTIVE LEARNING TEMPLATES

Check for allergies Emphasize the need of up-to-date immunizations and an annual influenza vaccines

Complications Medications IV fluids,Antibiotics, Oxygen therapy, as needed Beta-adrenergic agonists such as albuterol sulfate for inhalation therapy and with chest physiotherapy, Inhaled hypertonic saline Mucolytics, Anti-inflammatories, Corticosteroids if pt experiences asthma or allergic bronchopulmonary aspergillosis, pancreatic enzymes such as pancrelipase, Vitamin A, D, E, and K supplements, Cystic fibrosis transmembrane regulator modulators, such as ivacaftor or tezacaftor/ivacaftor (for patients with identified G551D mutation of the CFTR gene) to target the underlying protein defect that causes the disease

Client Education Breathing exercises, Reduce risk factors associated with respiratory infections, Early signs and symptoms of respiratory infection and disease progression that indicate the need to notify a primary provider

Interprofessional Care Surgery: Gastrostomy tube placement and Lung or liver transplantation Respiratory therapist, Nutritionist, Social Services, Psychiatrist

Bronchiectasis, Pneumonia, Atelectasis, Pulmonary hypertension, Pneumothorax, Dehydration, Malnutrition, GERD, Hepatic disease, Diabetes, Arthritis, Biliary disease, Liver failure, Clotting problems, Osteoporosis, Delayed sexual development, Infertility, Electrolyte imbalances, Bowel obstruction, Cardiac arrhythmias Shoc, death

THERAPEUTIC PROCEDURE

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