PREPARED BY:

CLASS PRESENTATION ON:CYSTIC FIBROSIS (CF)

DEFINITION:

Cystic Fibrosis (CF) is a disorder of the endocrine glands that affects primarily the lungs, gastrointestinal (GI) tract and sweat glands.

CF is a genetic disorder and was thought to be just a childhood disease, because most affected

children did not survive past puberty. However, with new treatments, Patients with CF are lining longer and more productive lives. Some CF patients now marry, have careers and live well into their thirties.

CF is the most common fatal genetic disease of children of European-American heritage.

PATHOPHYSIOLOGY:

CF, affects primarily the lungs, GI tract and sweat glands. The disease varies in severity; some patients have no GI involvement.

Abnormal sodium and chloride transport across cell membranes, causing thick, tenacious secretions, is responsible for many of the characteristic symptoms.

Thick, sticky respiratory secretions that are difficult to remove cause airway obstruction, resulting in frequent respiratory infections.

CONT…..

Similar abnormalities in the pancreas cause blocked ducts and retained digestive enzymes.

These retained enzymes digest and destroy the exocrine pancreas.

The absence of digestive enzymes in the

intestines causes:

CONT….

a) Malabsorption of essential nutrientsb) Frequent foul smelling Fatty

stoolsc) Excess flatus Patients with CF secrete sweat that is

high in sodium and chloride because these electrolytes are not reabsorbed as they pass through the sweat ducts.

ETIOLOGY:

Hereditary Both parents must be carriers of the

defective gene for CF to be present in a child.

Pancreatic insufficiency

CLINICAL MANIFESTATION:

Presentation usually occurs younger than age 6 months but may occur at any age. Signs and symptoms and severity of the disease vary and change over time as the disease progresses.

RESPIRATORY MANIFSTATION:1. Recurrent pulmonary infections-H. influenza, S.

aureus, P. aeruginosa.2. Cough, dry to productive. Chronic clearing of

throat may indicate increased mucus production.

CONT….

3. Wheezing, crackles on auscultation are indicative of respiratory exacerbation.

4. Dyspnea5. Barrel-shaped chest (increased

anteroposterior chest diameter).6. Cyanosis7. Clubbing of fingers and toes8. Nasal polyps and pansinusitis

CONT…..

GI MANIFESTATION:1. Meconium ileus found in neonates.2. Failure to thrive and failure to gain weight in

the presence of a good appetite3. Abdominal distention4. Vomiting, dehydration and electrolyte

imbalance5. Maldigestion, steatorrhea (fatty stools, loss of

fat soluble vitamins).6. Rectal polapse

CONT…

7. Distal intentional obstructive syndrome8. Biliary cirrhosis, obstructive jaundice9. Pancreatitis.

CONT…

OTHER MANIFESTATIONS:1. Thin extremities, sallow skin, wasted buttocks.2. Hyperglycemia, glucosuria, polyuria, weight

loss.3. Salty taste when parents kiss the skin. 4. Sterility in males5. Hypoproteinemia and anemia6. Bleeding

diathesis7. Hyponatremia and heat prostration8.Kyphosis

COMPLICATIONS:

Patients with CF are at high risk for a variety of complications including:

-bronchiectasis-pneumothorax-corpulmonale-respiratory failure-bowel obstruction can occur as a result of thick

mucus binding with poorly digested fecal matter.-Diabetes from pancreatic islet cell involvement

may be present late in the disease.

DIAGNOSTIC TESTS:

Quantitative sweat chloride test; pilocarpine iontophoresis, performed at a CF foundation accredited center by skilled personnel. Measures sodium and chloride content in sweat.

a) Chloride level greater than 60mEq/L is diagnostic.

b) Chloride of 4o to 60mEq/L is borderline and should be repeated, followed by genotype for the most frequent CFTR mutations.

c) Sodium level greater than 60mEq/L is diagnostic.

CONT…..

Measurement of trypsin concentration in duodenal secretions; absence of normal concentration virtually diagnostic.

Analysis of digestive enzymes (trypsin and chymotrypsin ) in stool- reduced, used for initial screening for CF.

Chest x-ray may be normal initially; later shows areas of infection, overinflation, bronchial thickening and plugging, atelectasis, fibrosis and emphysema.

CONT…..

Sinus radiograph or computed tomography shows mucus plugging.

Analysis of stool for steatorrhea. Sputum or throat cultures to rule out

infection. Pulmonary function studies (after age 4) a) Decreased vital capacity and flow rates. b) Increased residual volume or increased

total lung capacity.

CONT….

Present diagnostic tests- prenatal genetic screening for families affected with CF.

Neonatal screening: immunoreactive trypsinogen ; if elevated, DNA assay for single and multiple CFTR mutations.

MEDICAL TREATMENT:

No cure for CF, Treatment is aimed at relieving symptoms.

Hydration, flutter mucous clearance device and chest (CPT) physiotherapy up to 4 times/day.

Regular exercise. Hot shower. Nebulize mist treatment using saline or

mucolytic medications before CPT.

CONT….

High doses of ibuprofen. Breathing exercise, incentive spirometry and

effective coughing techniques. Lung transplant is potentially promising

treatment. Antibiotics / prophylatic antibiotic therapy. Pancreatic enzyme replacement An increase in calories requirements

necessitates a high calorie, nutrient-dense diet.

ASSESSMENT

1. Check for family history of CF ,failure to thrive ;check for clients history and physical condition. Carefully listen for subtle information that may suggest CF.

2. Assess respiratory status; respiratory rate, presence of tachypnea, wheeze, cough, character of sputum, and oxygen saturation saturation level.

- Increased work of breathing.- Quality of breath sounds by auscultation.- Ability to participate in activities of daily living ,

exercise tolerance, quality of sleep.

- Assess for oxygen desaturation with sleep.

3. Assess nutritional status and characteristics of stool.

NURSING DIAGNOSIS:

Ineffective airway clearance related to thick pulmonary secretion.

Risk for infection related to thick , tenacious secretions.

Imbalanced nutrition: less than body requirements related to decreased appetite .

Disturbed body image related to chronic disease process.

Interrupted family processes related to child with a chronic disease.

NURSING INTERVENTIONS

Promoting Airway clearance:1. Use intermittent nebulizer therapy three to four

times per day when client is symptomatic. Use pretreatment postural drainage. Administer bronchodilators and other medications,

diluted in normal saline, in aerosal form to penetrate respiratory tract.

2. Perform CPT 3-4 times after nebulization if infection is present.

- Perform prior to meals or 1hour after eating to prevent vomiting or discomfort

- Place the client in a position that gives the greatest access to affected lobes of lung and facilitate gravity drainage of mucus from the lung area.

- Client with gastro- esophageal reflux should not use head down position.

3. Help client to relax to cough more easily.4. Suction the cough if necessary and monitor oxygen

saturation levels during procedures.5. Monitor for haemoptysis and provide treatment for

it .6. Maintain cautious oxygen therapy due to chronic CO2

retention.

Preventing infection:1. Provide frequent mouth care to reduce chance of

infection because mucus is present.2. Administer antibiotics as prescribed if client is

symptomatic.3. Monitor closely for deteriorating respiratory status.4. Provide good skin care and position changes to

prevent skin breakdown.Promoting adequate nutrition:1. Encourage diet composed of high calories and

protein foods and moderate to high fat because

absorption of food is incomplete.2. Administer fat soluble vitamin in water miscible

solution three to four times as prescribed.3. Administer pancreatic enzymes with each meals

and snacks .dose is based on client’s weight, weight gain, growth , food intake and character of bowel movements.

4. Increase salt intake in hot weather, fever, or excessive exercise to prevent sodium depletion.

5. Check weights at least weekly to assess nutritional interventions.

Enhancing self esteem and Body image1. Explain each procedure, medication and treatment to

client as appropriate to age.2. Allow client to show frustration , fears and feelings by

talking , complaining or crying.3. Provide diversional activities according to client’s

interest.4. Help the client to indentify the strengths and

limitations and to feel good about self.5. Encourage regular exercise and activity to foster a

sense of accomplishment and independence and to improve pulmonary function.

Family education and Health maintenance:1. Teach parents or family members to have through

understanding of the dietary regimen and special need for calorie , fat and vitamins.

2. Help the family members to schedule care for client within framework of family life.

3. Provide emotional support to client and family members.

4. Stress the importance of regular medical care.5. Stress the importance of follow up and regular

health care .

Discuss with family members about limitation and expectation for client.

7. Investigate homecare facilities for families and especially respite services for caregivers.

EXPECTED OUTCOMES

Tolerates CPT four times per day for 30 minutes with stable oxygen saturation.

No signs of respiratory infection. Eats well with no vomiting ; weight

stable. Family caretakers take part in care of

their client.