GENERAL MEASURES :
β’ Care by experienced physician and team (respiratory therapist, nurse, nutritionist, physical therapist,
counselor, social worker)
β’ Goals are to prevent and treat respiratory failure and pulmonary complications
β’ Postural drainage and chest physiotherapy - adjuncts include flutter valve and CPT vest (expensive)
β’ Pancreatic enzyme replacement; H2 receptor blockers can increase utilization of enteric coated enzymes
β’ Regular exercises for fi tness
β’ Press for adequate growth through good nutrition - supplements may be needed
β’ Aerosol B2 agonists; consider ipratropium
β’ Antibiotics - especially to target Pseudomonas , Staphylococcus
β’ DNase: aerosolized mucolytic, 2.5 mg neb q day. Use pari-plus nebulizer cup.
β’ Oxygen: when needed
β’ Monitor sleeping O2 saturation
β’ Hypaque or diatrizoate (Gastrografin) enemas or surgery for unrelieved meconium ileus (newborn)
β’ For fecal accumulation and intussusception in older children or adults - enemas of diatrizoate (Gastrografin)
or polyethylene glycol (Golytely) per NG
β’ Early identifi cation of diabetes, and treatment with insulin
β’ Assisted ventilation with BiPAP is acceptable and temporary. May be used nightly as a bridge to lung
transplant.
SURGICAL MEASURES:
β’ Surgery may be indicated for some complications
β’ Organ transplants possible for lung, liver, pancreas
ACTIVITY : Physical conditioning to the extent possible for cardiorespiratory fi tness (does not improve
pulmonary function)
DIET :
β’ Allow liberal salting of foods per patient preference
β’ High protein
β’ High calories (1.5 x recommended for general population)
β’ High fat (previously not recommended)
β’ Vitamin supplements (double RDA)
β’ May need supplemental feeds, oral or by gastrostomy
DRUG(S) OF CHOICE :
. Oral antibiotics
. Ciprofloxacin for exacerbations
. Cephalexin (Keflex) for Staphylococcus aureus
. Aerosolized antibiotics
. Tobramycin 300 mg bid. 28 day on/28 day off rotating schedule for patients colonized with Pseudomonas
. IV antibiotics
. According to culture and sensitivity studies when ill with respiratory infections
. For Pseudomonas infections - IV tobramycin, start with 10 mg/kg/24 hrs; peak 8-12 g/mL (17-26 mol/
L); trough 1-2 g/mL (2-4 mol/L) plus IV ceftazidime or antipseudomonal penicillin.
. For staphylococcal infections - IV oxacillin or ticarcillin-clavulanate (Timentin). Vancomycin if resistant.
. Other therapies
. Pancreatic enzyme replacements - dose 500-2500 units lipase/kg/meal. Distal colonic strictures possible
with high doses.
. Bronchodilators, if response demonstrated
. Dornase alfa (DNase) nebulized daily 2.5 mg
. Azithromycin, by anti-infl ammatory protocol; if < 40 kg, 250 mg po q MWF; if > 40 kg, 500 mg po q MWF.
Patient should not be colonized with atypical TB (could suppress).
. Ibuprofen, regular use, may retard lung disease, but levels must be monitored
. Oxygen therapy for severe pulmonary insuffi ciency or hypoxemia, pulmonary hypertension
. Night-time mask BiPap effective with significant respiratory insufficiency
. Occasional assisted ventilation, controversial
. IPPB contraindicated
. Routine administration of annual influenza vaccine
ALTERNATIVE DRUGS :
β’ Other antipseudomonal antibiotic
β’ Cephalexin for staphylococcal prophylaxis in infants not indicated
PATIENT MONITORING : At least 3 times a year; Cystic Fibrosis Center recommended
PREVENTION/AVOIDANCE :
. In prenatal situations :
. Genetic counseling
. Prenatal diagnosis for future pregnancies
. For complications
. For respiratory infections - maintain pertussis and measles immunity; annual influenza immunization
. Avoid general anesthetics. Consider epidural, spinal, or local.
. Good medical teamwork in the management of the multifaceted problems of the disease
POSSIBLE COMPLICATIONS :
. Diabetes: affects growth, lung function
. Atelectasis
. Pneumothorax
. Hemoptysis
. Right heart failure
. Pulmonary hypertension
. Pulmonary emphysema
. Digital clubbing
. Hypertrophic pulmonary osteoarthropathy
. Metabolic alkalosis
. Volume depletion
. Bleeding esophageal varices
. Symptomatic biliary cirrhosis
. Intestinal obstruction
. Female fertility rate about 80% of normal
. Numerous psychosocial aspects
. Malnutrition
. Retarded growth
. Hypovitaminosis A (increased ICP)
. Hypovitaminosis E (hemolytic anemia)
. Hypovitaminosis K (bleeding tendency)
EXPECTED COURSE/PROGNOSIS :
. Largely dependent on pulmonary involvement
. Prognosis improving due to early detection and aggressive treatment
. Median survival is to age 33
Pediatric: Diagnosis usually confi rmed in infancy or early childhood but some go undetected until Adolescence