Cystic
Fibrosis
- Is a hereditary disease affecting the
exocrine (mucus) glands of the lungs, liver,
pancreas, and intestines, causing
progressive disability due to multisystem
failure.
- Autosomal recessive disease of the
Caucasian population
- The mucus produced by the exocrine gland
is abnormally thick, causing obstruction of
small passageways of the affected organ
- The most common symptoms are:
Progressive chronic lung dse associated with
infection, pancreatic enzyme deficiency d/t
duct blockage, and sweat gland dysfunction
resulting in increase sodium & chloride
sweat concentration
S & Sx:
- Sx are produced by the stagnation of mucus
in the airway, leading to bacterial
colonization (repeated pneumonia &
bronchitis) & destruction of lung tissue
- Emphysema & atelectasis occur as airways
become increasingly obstructed.
- Chronic hypoxemia causes contraction &
hypertrophy of the muscle fibers in
pulmonary arterioles, leading to pulmonary
HPN, and eventual COR PULMONALE
- Wheezing & dry, non-productive cough
- Dyspnea
- Cyanosis
- Digital clubbing
Other symtoms:
GI:
- Meconium ileus - neonate
- Intestinal obstruction (thick intestinal secretions)
- Steatorrhea (frothy, foul smelling stools)
- Deficient fat soluble vit. - easy bruising , anemia
- Malnutrition; Hypoalbumenemia- from diminished
absorption of CHON
- Rectal prolapse d/t large, bulky stools
Integumentary:
-Infants taste “salty” when kissed
- DHN, Electrolyte imbalance
Reproductive System:
- Delayed puberty in females
- Inhibited fertility dt highly viscous cervical
secretions (acts as plug,blocking sperm entry)
- Sterile males dt blockage in vas deferens
Laboratory tests:
1. Quantitative Sweat chloride test:
a. Prodxn of sweat is stimulated (pilocarpine
iontophoresis), sweat is collected (50 mg),
sweat electrolytes are measured
Normal= < 40 meq/L
Positive test result= > 60 meq/L
Highly suggestive= 40-60 meq/L; require repeat test
2. Chest X-ray: Atelectasis, Obstructive emphysema
3. PFT: abnormal small airway fxn.
Implementation:
1. Prevent & treat pulmonary infections
2. CPT (percussion, postural drainage) on awakening
& evenings= not before or immediately after
meals
3. Meds: Bronchodilators (to give before CPT)
: Dornase Alfa( Pulmozyme)-
Recombinant Human Deoxyribonuclease (DNase),
which ↓ viscosity of mucus
: Antibiotics
4. Do not take Cough suppressants: Inhibit
expectoration
5. Regular Exercise program (good breathing pattern)
6. Oxygen
7. Monitor for Hemoptysis and contol by :
bedrest, cough suppressants, antibiotics, Vit K.
8. Diet: Well balanced, high CHON, high calorie,
with Vit. ADEK
9. For GI: Pancreatic enzyme: given with meals &
snacks/ within 30mins of eating. Do not give if
child is NPO
10.Supplement diet with salt esp. during hot
weather; drinks : Gatorade
Surgical Management: Lung Transplantation
