1. Long Term Respiratory Dysfunction
II Cystic fibrosis

2. Cystic fibrosis CF CF is inherited as an autosomal recessive trait.
The affected child inherits the defective gene from both parents on chromosome 7, the incidence 1:4
Cystic fibrosis transmembrane regulator.

3. Pathophysiology CF is ch-ch by several feature:
Increased viscosity of mucous gland secretion.
Elevated of sweat chloride & salt.
Increase in several organic and enzymatic (constituents of saliva and abnormalities in autonomic nervous system).
Decrease pancreatic secretion of bicarbonate & chloride.
The primary factor, and the one that is responsible for many of the clinical manifestation of the disease is mechanical obstruction caused by the increased viscosity of mucous gland secretion. Fig 23-5

4. Pathophysiology
Basic defect__ exocrine gland dysfunction__ abnormal mucus secretion (high viscosity) and obstruction to small ducts
1.__ bile ducts __ focal billary fibrosis with concretions__ billiary cirrhosis__ portal hypertension.
2. __ pancreatic ducts__ secondary degeneration of pancreas__ pancreatic achylia (fibrosis)__ malabsorption syndrome__ bulky stools, Steatorrhea.

5. Pathophysiology
3. __ small intestine __ inspissated meconium__ intestinal obstruction of newborn__ rectal prolepses.
4.__ bronchi__ bronchial obstruction:
a.__ generalized obstructive emphysema+ patchy atelectasis.
b.__ thick secretion good media for bacterial growth__ chronic bronchial pneumonia.

6. Diagnostic Evaluation
Diagnostic of CF is established on the basic of:
History of the disease in the family.
Absence of pancreatic enzymes.\
Increase in electrolyte concentration at sweat.
Chronic pulmonary involvement.

7. Clinical manifestation of CF
Meconium ileus: (in about 12% of cases.:
Abdominal distention, vomiting, failure to pass stools, rapid development of dehydration.
Gastrointestinal manifestations::
Large ,bulky, loose, frothy, extremely foul-smiling stools.
Voracious appetite (early in disease).
Loss of appetite (later in disease).
Weight loss.
Marked tissue wasting.
Failure to growth.
Distended abdomen.
Thin extremities.
Sallow skin.
Evidenced of deficiency of fat-soluble vitamins A, D, E, and K.
anemia

8. Clinical manifestation of CF
Pulmonary manifestations:
Initial signs: wheezing respirations, dry, nonproductive cough.
Eventually: increased dyspnea, paroxysmal cough, evidence of obstructive emphysema and patchy areas of atelectasis.
Progressive involvement: over inflated, barrel-shaped chest, cyanosis, clubbing of fingers and toes, repeated episodes of bronchitis and bronchopneumonia.

9. Diagnostic Evaluation
Diagnostic tests:
Sweat chloride test: abnormal high sodium & chloride concentration in the sweat is a unique ch-ch of CF. normal sweat chloride less than 40 meq /L, in CF become more than 60 meq/L.
Chest radiography: patchy atelectasis & obstructive emphysema.
Pulmonary function test (PFTs).
Stool analysis (stool fat).
Barium enema __obstruction.

10. Therapeutic management
Management of pulmonary problem:
Infection__ bacterial infection, the seriousness with bacterial colonized.
Treatment:
Antibiotic __with initial symptoms of infection as prophylactic, IV at hospital and even at home by CV catheter.
Chest Physiotherapy (CPT) and flatter mucus clearance device.
Bronchodilator medication (aerosol) before CPT.

11. Therapeutic management
4. Recombinant human deoxyribonuclease: decrease viscosity of mucus( side effects: altered voice + laryngitis).
5. Exercise :
Adequate breathing pattern.
Stimulate mucus secretion.
High self-esteem.
6. O2 therapy with caution ___increase CO2 __O2 narcosis pneumothorax with advanced disease.

12. Therapeutic management
Management of gastrointestinal problems:
The principle treatment for pancreatic insufficiency is: Replacement of pancreatic enzymes which administer with meal or before it 1-5 caps or les with snack (creon) to achieve normal growth and decrease in the number of stool.
High protein-high caloric diet (because there is impaired intestinal absorption.
Fat-soluble vitamins is needed (A,D,E and K).

13. Prognosis & Screening Prognosis:
Pulmonary involvement mainly affected.
Pancreatic enzyme deficiency is less of a problem if adequate nutrition is ensured.
Screening:
Fetus: based on detection CF mutations.
Newborn: detecting of abnormal chloride secretions in sweat and (DNA study)
Sibling and family member: Carrier: DNA study.

14. Nursing consideration
Pulmonary assessment : disciple the same in Asthma, lung sound &cough& degree of finger clubbing.
GI assessment: observe frequent &natural of stools & abdominal distention.
Assess sign of FTT (wt loss, wasting, pallor, fatigue).
Hospital care:
Hand washing to prevent nosocomial infection to other patients .
Sputum & soiled tissue should be discarded in covered no-touch receptacle.
Isolation room for colonized infection's CF patients.

15. Nursing consideration
CPT will be done by nurse if no therapest.
O2 therapy: cautiously administered (O2 narcosis).
Diet:
High calorie intake, frequent meal, high fluid intake.
Pancreatic enzymes are supplies for each meal or snack.
Adequate salt especially for febrile child
Skin care: especially on bony prominences.
Careful selection of injection site.
Provide support for child & family (sympathetic attention).

16. Nursing consideration
Home care:
learn family how to use the equipments.
Lear family about preferred diet( tolerated fat, high protein and CHO , administration pancreatic enzymes at beginning of all meals and snacks.
Fluid intake and caution with salt administration especially with hot weather.
Oral hygiene.
Teaching parents CPT & exercises.
Postural drainage.
Recommended immunization of influenza vaccine start at 6 month age then yearly booster.