eMedicine Specialties > Pediatrics: General Medicine > Pulmonology
Cystic Fibrosis: Treatment & Medication
Updated: Oct 17, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
As a result of the complex and multisystemic involvement of cystic fibrosis (CF) and the need for care by specialists, treatment and follow-up care at specialty centers with multidisciplinary care teams (ie, CF centers) is recommended. At the time of initial confirmation of the diagnosis, baseline assessment, investigations, and initiation of therapy are recommended. In addition, patient/parent education, including counseling and instructions regarding airway clearance techniques and the use of equipment (eg, nebulizer, spacer for metered-dose inhaler), is recommended. When a patient presents with complications necessitating hospital admission, these objectives can be obtained during hospitalization. Follow-up outpatient visits are scheduled at 2-3 monthly intervals. Hospital admission is required for treatment of acute pulmonary exacerbation and severe complications.
Surgical Care
Surgical therapy may be required for the treatment of respiratory complications such as pneumothorax, massive recurrent or persistent hemoptysis, nasal polyps, or persistent and chronic sinusitis. GI tract complications, such as meconium ileus, intussusception, gastrostomy tube placement for supplemental feeding, and rectal prolapse, also require surgical therapy. Lung transplant is indicated for the treatment of end-stage lung disease.9 Studies suggest that, although lung transplant may improve quality of life, it may not improve survival.10,11
Consultations
In addition to the specialists available at CF centers (usually pulmonologists and/or gastroenterologists), other specialists may need to be consulted when other systems are involved or complications involve other organs, including the following:
- Surgeon
- Otolaryngologist
- Endocrinologist
- Cardiologist
- Transplant surgeon
Diet
In general, a normal diet with additional energy and unrestricted fat intake is recommended. A high-energy and high-fat diet, in addition to vitamin (especially fat soluble) and mineral supplementation, is recommended to compensate for malabsorption and the increased energy demand of chronic inflammation. In children, because of various physical activities and eating habits, base assessment and modification of energy requirements on growth and weight gain. Special consideration is given to female patients with a potential for delayed puberty because of malnutrition, patients with diabetes mellitus, and patients with liver disease. Nutritional supplements in the form of either high-energy oral preparations (eg, Scandishake) or enteral feeds (eg, elemental formulas, high-fat mixtures) via nasogastric tube or gastrostomy may be indicated in some patients.
Activity
Regular exercise increases physical fitness in patients with CF. Upper body exercises, such as canoe paddling, may increase respiratory muscle endurance.
Medication
The primary goals of cystic fibrosis (CF) treatment include maintaining lung function as near to normal as possible by controlling respiratory infection, clearing airways of mucous, administering nutritional therapy (ie, enzyme supplements, multivitamin and mineral supplements) to maintain adequate growth, and managing complications.
Mild acute pulmonary exacerbations of CF can be treated successfully at home by increasing the frequency of airway clearance; by inhaled bronchodilator treatment, chest physical therapy, and postural drainage; by increasing the dose of dornase alfa (Pulmozyme); and by use of oral antibiotics such as oral fluoroquinolones.
Enzymes, pancreatic
These agents aid digestion when the pancreas is malfunctioning. Current pancreatic enzyme preparations are derived from porcine extracts and contain various proportions of lipase, amylase, and protease. Most of the preparations are available in multiple strengths. A particular dose is prescribed based on clinical symptoms and age and weight and then modified according to the clinical response. Usually, the dose of pancreatic enzymes should not exceed 2000 U/kg/meal of lipase. The novel preparation TheraCLEC-Total, a highly purified microbiologically-derived enzyme preparation, is under investigation in clinical trials.
Pancrelipase (Creon, Pancrease, Ultrase, Viokase)
Enteric-coated pancreatic enzyme microspheres containing various amounts of lipase, protease, and amylase. Assists in digestion of protein, starch, and fat.
Adult
1-3 PO cap or tab with meals; titrate dose to desired clinical effect
Pediatric
500-2000 U of lipase/kg/meal PO; individualize dose to patient; patient's response guides dose; dose of 1-3 cap per meal is sufficient for most patients
Adjust dose according to stool fat and nitrogen content
Drugs that increase gastric pH (eg, H2 antagonists, proton pump inhibitors) may increase effect of pancreatic enzymes by inhibiting destruction of ingested enzymes
Documented hypersensitivity, history of pork protein allergy
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Monitor weight gain or loss, abdominal cramps, frequency and nature of stools, and bloating; patient can take more enzymes with large fatty meals; caps should be swallowed whole or sprinkled on food immediately prior to ingestion and should not be chewed, crushed, or taken with hot liquids; coadministration with H2 blockers or proton pump inhibitors to reduce gastric acid production (ie, optimizes pH [5-6.5] for enzyme dissolution) helps reduce daily dose of pancreatic enzymes; intake of higher dose of pancreatic enzymes (more than recommended dose) has been reported to cause fibrosing colonopathy; enzyme products widely vary (do not interchange brand once stabilized)
Vitamins
Vitamins are organic substances required by the body in small amounts for various metabolic processes. They may be synthesized in small or insufficient amounts in the body or not synthesized at all, thus requiring supplementation. They are classified as fat or water soluble. Vitamins A, D, E, and K are fat soluble while biotin, folic acid, niacin, pantothenic acid, B vitamins (ie, B-1, B-2, B-6, B-12), and vitamin C are generally water soluble.
Vitamin deficiency may result from an inadequate diet, increased requirements (eg, pregnancy, lactation), or secondary to disease or drug use. They are clinically used for the prevention and treatment of specific vitamin deficiency states.
Supplementation of fat-soluble vitamins is routine in CF because of chronic malabsorption.
Vitamins, fat soluble
Vitamins A, D, E, and K are fat-soluble vitamins and are essential for antioxidant effects and function as coenzymes for biological pathways, neurodevelopment, bone development, and coagulation. Typical multivitamin preparations formulated especially for patients with CF are referred to as ADEKs. The following doses are typically are prescribed for diseases of malabsorption.
Adult
Vitamin A: 5000 IU/d or more PO
Vitamin D: 400 IU/d or more PO (modified according to vitamin level)
Vitamin E: 200-400 IU/d PO
Vitamin K: 5 mg PO twice per wk (if patient has liver disease or is on antibiotics)
Pediatric
Vitamin A
<2 years: 1 mL/d PO of multivitamin liquid (eg, Poly-Vi-Sol provides 1500 IU/mL)
2-8 years: 5000 IU/d PO
Older children: Administer as in adults
Vitamin D
<2 years: 1 mL/d PO of multivitamin liquid (eg, Poly-Vi-Sol provides 400 IU/mL)
2-8 years: 400 IU/d PO
Older children: Administer as in adults
Vitamin E
0-6 months: 25 IU/d PO
6-12 months: 50 IU/d PO
1-4 years: 100 IU/d PO
4-10 years: 100-200 IU/d PO
>10 years: Administer as in adults
Note: Small amount of vitamin E typically is provided in multivitamin preparations; may require separate supplementation with vitamin E preparation (Aqua-E) to provide adequate dose and bioavailability
Vitamin K (ie, phytonadione)
<1 year: 2.5 mg PO qwk
>1 year: Administer as in adults
Vitamin K is included in ADEKs
Vitamin K counteracts effects of oral anticoagulants; prolonged use of phenytoin or phenobarbital may exacerbate vitamin D deficiency; bile acid–binding resins (eg, cholestyramine) may decrease absorption of fat-soluble vitamins; large doses of vitamin E may prolong PT, requiring dose adjustment of warfarin
Documented hypersensitivity, pregnancy (ie, vitamin A and D doses exceeding RDA)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Vitamin A may cause irritability, drowsiness, vertigo, headache, increased intracranial pressure, erythema, and cheilosis with doses exceeding physiologic replacement
Vitamin D may cause hypercalcemia
Vitamin E may induce vitamin K deficiency; necrotizing enterocolitis may occur with large doses of vitamin E
Vitamin K should be used with caution in patients with impaired renal function or renal stones
Bronchodilators
Albuterol provides selective agonistic action on beta2-adrenoceptors. Stimulate adenyl cyclase resulting in smooth muscle relaxation of the bronchi, uterus, and skeletal muscle. Inhaled beta2-agonists are often administered before chest physical therapy for airway clearance. They also are indicated when clinical evidence of bronchial hyperresponsiveness exists. In children with CF, the use of bronchodilators must be evaluated. Children with bronchiectasis may have a paradoxic bronchodilatation in response to beta-adrenergic agents. Pulmonary function testing before and after bronchodilators is suggested to avoid these counterproductive effects.
Albuterol (AccuNeb, Proventil)
Most commonly used bronchodilating agent available in multiple dosage forms (eg, solution for nebulization, metered-dose inhaler, PO solution). Typically, 2.5 mg of albuterol nebulizer solution is used either in premix solution with isotonic sodium chloride solution or 0.5 mL of albuterol solution is mixed with 3 mL of 0.9% NaCl and administered before chest physical therapy.
Adult
2.5-5 mg via nebulizer q4-6h in 2- to 5-mL sterile 0.9% NaCl or water
To make solution, dilute 0.5 mL (2.5 mg) of 0.5% inhalation solution in 1-2.5 mL of 0.9% NaCl
Pediatric
0.5 mL of 0.083% (0.83 mg/mL) solution mixed with 2-3 mL of 0.9% NaCl via inhaled nebulizer tid/qid before chest physical therapy or for bronchospastic symptoms
Also available in ready-to-use solution 0.021% (0.63 mg/3mL)
Beta-adrenergic blockers antagonize effects; inhaled ipratropium may increase duration of bronchodilatation by albuterol; cardiovascular effects may increase with MAOIs, inhaled anesthetics, tricyclic antidepressants, and sympathomimetic agents
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Beta2-agonists may decrease PaO2 by increasing ventilation-perfusion mismatch and decreasing airway wall tone, resulting in enhanced airway collapse during expiration; caution with conditions associated with tachycardia
Mucolytic Agents
Large amounts of neutrophil-derived DNA released from the dead neutrophils increase sputum viscosity. Mucolytics, such as dornase alfa, an enzyme that hydrolyses the DNA, are used in patients with CF to improve airway clearance.
Hypertonic saline inhalation has been proposed as a therapy to increase hydration of airway surface liquid in patients with cystic fibrosis.12 Patients receiving 7% hypertonic NaCl (4 mL via nebulizer bid) were compared with patients receiving normal saline in similar fashion; those receiving hypertonic NaCl had improved lung function and fewer pulmonary exacerbations.13 Hypertonic saline was not associated with worsening bacterial infections or inflammation. The Pulmonary Therapies Committee of Cystic Fibrosis Foundation recommends chronic use of hypertonic saline for patients with CF aged 6 years or older to improve lung function and to reduce the number of exacerbations.14
Dornase alfa (Pulmozyme)
Recombinant human DNase (rhDNase). Cleaves and depolymerizes extracellular DNA and separates DNA from proteins. This allows endogenous proteolytic enzymes to break down the proteins, thus decreasing viscoelasticity and surface tension of purulent sputum.
Adult
2.5 mg/d via nebulizer; some patients (especially patients >21 y or with FVC >85%) may benefit from administering bid
Pediatric
Administer as in adults
None reported
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
May cause hoarseness, pharyngitis, rash, or chest pain
Antibiotics
Antibiotic treatment may vary from a short course of one antibiotic agent to a continuous course with multiple antibiotics administered via various routes, including oral, intravenous, or inhalation. Because patients with CF have a larger lean body mass, they often have a higher clearance rate for many antibiotics. Achieving effective levels in respiratory secretions is difficult; higher doses of antibiotics and monitoring of aminoglycoside levels are required.
Administer aerosolized antibiotics when the airway pathogens are resistant to oral antibiotics or when the infection is difficult to control at home. Aerosolized antibiotics may reduce symptoms by reducing the organism density in the airways. Other advantages include prevention of infection or delay of chronic colonization, treatment of acute infection, and treatment of bacterial colonization in patients following transplantation to prevent infection in the transplanted lungs.
Of all the agents used in the aerosolized form (eg, gentamicin, colistin, tobramycin), preservative-free high-dose tobramycin is especially formulated for inhalation (ie, TOBI) and has been used in 2 large controlled studies and been reported to be safe and effective in patients older than 6 months.15 The usual dose is 300 mg twice daily administered during alternate months. Currently, clinical trials using a powder form of tobramycin and colistin are underway. These preparations use novel delivery devices and shorten the time required for dosage administration.
Cephalosporins are effective against staphylococci and H influenzae. A small subset of third-generation cephalosporins is effective against P aeruginosa. Generally speaking, moving from first-generation to third-generation cephalosporins gives increasing gram-negative coverage and less gram-positive coverage.
Fluoroquinolones are effective against most gram-positive and gram-negative organisms. They are the only class of oral antibiotics effective against P aeruginosa. The most commonly used medication in this class is ciprofloxacin. None are approved for children because of concern regarding their effects on deposition in the cartilage. However, studies from Europe have reported substantial evidence of their safety in patients with CF.
In patients with colonization with P aeruginosa, azithromycin administered orally 3 times per week on a long-term basis has been shown to improve lung function and nutritional status and to reduce acute pulmonary exacerbations.16
Tobramycin, inhaled (TOBI)
Formulated specifically for inhalation. Chronic intermittent administration in patients with P aeruginosa infection improves pulmonary function and nutritional status and reduces symptomatic pulmonary exacerbation.
Adult
300 mg inhaled via jet nebulizer q12h; used episodically (28 d of active treatment followed by 28 d without drug)
Pediatric
<6 years: Not established
>6 years: Administer as in adults
Concurrent use with systemic aminoglycosides has caused ototoxicity
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May cause bronchospasm, voice alterations, or tinnitus
Gentamicin (Garamycin)
Usually combined with one of the penicillins used to treat pseudomonad infections in patients with CF.
Adult
3 mg/kg/dose IV q8h
Pediatric
Administer as in adults
Coadministration with other aminoglycosides, cephalosporins, penicillins, and amphotericin B may increase nephrotoxicity; because aminoglycosides enhance effects of neuromuscular blocking agents, prolonged respiratory depression may occur; coadministration with loop diuretics may increase auditory toxicity of aminoglycosides; possible irreversible hearing loss of varying degrees may occur (monitor regularly)
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Renal toxicity and ototoxicity are possible adverse effects; drug-level monitoring is required to ensure therapeutic levels and minimize adverse effects; high-frequency hearing loss and tinnitus may occur; adjust dose in renal dysfunction
Tobramycin (Nebcin)
Usually combined with one of the penicillins used to treat pseudomonad infections in patients with CF.
Adult
3 mg/kg/dose IV q8h
Pediatric
Administer as in adults
Aim for peak levels of 10-12 mcg/mL, with trough levels of <2 μ g/mL
Increases effects of neuromuscular blockers and potentiates effects of extended spectrum penicillins; concurrent administration with amphotericin B, cephalosporins, and loop diuretics increases risk of nephrotoxicity
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Renal toxicity and ototoxicity are possible adverse effects; drug-level monitoring is required to ensure therapeutic levels and minimize adverse effects; high-frequency hearing loss and tinnitus can occur; adjust dose in renal dysfunction
Piperacillin (Pipracil)
Effective against most strains of P aeruginosa and H influenzae. Usually not effective against staphylococci.
Adult
2-4 g IV q6h; not to exceed 24 g/d
Pediatric
300 mg/kg/d IV divided q6h; not to exceed 24 g/d
Tetracyclines may decrease effects; piperacillin at high concentrations may physically inactivate aminoglycosides; probenecid may increase levels; coadministration with aminoglycosides has synergistic effects
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in renal impairment and history of seizures; caution in conditions requiring salt restriction (contains 6.5 mEq NaCl/g)
Cephalexin (Keflex)
First-generation cephalosporin that arrests bacterial growth by inhibiting bacterial cell wall synthesis. Bactericidal activity against rapidly growing organisms. Primary activity against skin flora.
Adult
250-1000 mg PO q6h for 10-14 d; not to exceed 4 g/d
Pediatric
50-100 mg/kg/d PO divided q6h
Coadministration with aminoglycosides increases nephrotoxic potential
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adverse effects may include rash, thrombophlebitis, nausea, vomiting, and diarrhea; approximately one third of patients who are allergic to penicillin are sensitive to cephalosporins; adjust dose in severe renal insufficiency (high doses may cause CNS toxicity); superinfections and promotion of nonsusceptible organisms may occur with prolonged use or repeated therapy
Ceftazidime (Ceptaz, Fortaz, Tazidime, Tazicef)
Third-generation cephalosporin with broad-spectrum gram-negative activity; lower efficacy against gram-positive organisms; higher efficacy against resistant organisms. Arrests bacterial growth by binding to one or more penicillin-binding proteins.
Adult
250-500 mg to 2 g IV q8-12h
Pediatric
200 mg/kg/d IV divided q6h; not to exceed 6 g/d
Nephrotoxicity may increase with aminoglycosides, furosemide, and ethacrynic acid; probenecid may increase levels
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adverse effects may include rash, thrombophlebitis, nausea, vomiting, and diarrhea; approximately one third of patients who are allergic to penicillin are sensitive to cephalosporins; adjust dose in severe renal insufficiency (high doses may cause CNS toxicity); superinfections and promotion of nonsusceptible organisms may occur with prolonged use or repeated therapy
Ciprofloxacin (Cipro)
Fluoroquinolone with activity against Pseudomonas organisms, streptococci, MRSA, Staphylococcus epidermidis, and most gram-negative organisms but with no activity against anaerobes. Inhibits bacterial DNA synthesis and, consequently, growth. PO bioavailability is lower in younger patients with CF (65%) than in those older than 13 years (95%).
Adult
250-750 mg PO q12h
Pediatric
500 mg PO q8h or 20-30 mg/kg/d PO divided q8h (see precautions)
Antacids, iron salts, and zinc salts may reduce serum levels; administer antacids 2-4 h before or after taking fluoroquinolones; cimetidine may interfere with metabolism of fluoroquinolones; ciprofloxacin reduces therapeutic effects of phenytoin; probenecid may increase ciprofloxacin serum concentrations; may increase toxicity of theophylline, caffeine, cyclosporine, and digoxin (monitor digoxin levels); may increase effects of anticoagulants (monitor PT)
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Due to possible deposition in growing cartilage, use is not routinely preferred in children, and if used, preferred use is for short periods; perform periodic evaluations of organ system functions (eg, renal, hepatic, hematopoietic) during prolonged therapy; adjust dose in renal function impairment; superinfections may occur with prolonged or repeated antibiotic therapy
Trimethoprim and sulfamethoxazole (Bactrim, Septra)
The broad spectrum and action of trimethoprim (TMP) and sulfamethoxazole (SMZ) against organisms found in patients with CF and the convenience of PO administration is useful for milder infections on an outpatient basis.
Adult
160 mg TMP/800 mg SMZ PO/IV q12h
Pediatric
8 mg/kg/d (based on TMP component) or 40 mg/kg/d (based on SMZ component) PO divided q6-12h
5-10 mg/kg/d (based on TMP component) or 25-50 mg/kg/d (based on SMZ component) IV q6-12h
May increase PT when used with warfarin (perform coagulation tests and adjust dose accordingly); coadministration with dapsone may increase blood levels of both drugs; coadministration of diuretics increases prevalence of thrombocytopenia purpura in elderly persons; phenytoin levels may increase with coadministration; may potentiate effects of methotrexate in bone marrow depression; hypoglycemic response to sulfonylureas may increase with coadministration; may increase levels of zidovudine
Documented hypersensitivity, megaloblastic anemia due to folate deficiency
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Contraindicated in pregnancy near term; discontinue at first appearance of skin rash or sign of adverse reaction; obtain CBC counts frequently; discontinue if significant hematologic changes occur; goiter, diuresis, and hypoglycemia may occur with sulfonamides; prolonged IV infusions or high doses may cause bone marrow depression (if signs occur, administer 5-15 mg/d leucovorin); caution in folate deficiency (eg, chronic alcoholism or patients who are elderly, receiving anticonvulsant therapy, or have malabsorption syndrome); hemolysis may occur in patients with G-6-PD deficiency; patients with AIDS may not tolerate or respond to TMP-SMZ; caution in renal or hepatic impairment (perform urinalyses and renal function tests during therapy); administer fluids to prevent crystalluria and stone formation
Chloramphenicol (Chloromycetin)
Effective against H influenzae and staphylococcal species. May help treat P aeruginosa infection for unclear reasons. PO preparation no longer available in the United States.
Adult
50-100 mg/kg/d IV divided q6h; not to exceed 4 g/d
Pediatric
Administer as in adults
Administered concurrently with barbiturates, serum levels may decrease while barbiturate levels may increase, causing toxicity; manifestations of hypoglycemia may occur with sulfonylureas; rifampin may reduce serum levels, presumably through hepatic enzyme induction; may increase effects of anticoagulants; may increase serum hydantoin levels, possibly resulting in toxicity; levels may be increased or decreased
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Contraindicated in pregnancy near term because of potential toxic effects on fetus (gray syndrome); can cause dose-related bone marrow suppression or idiosyncratic reaction, causing more serious aplastic anemia; patient should have hemoglobin, hematocrit, and white blood cell count at frequent intervals during therapy
Use only for indicated infections or as prophylaxis for bacterial infections; serious and fatal blood dyscrasias (aplastic anemia, hypoplastic anemia, thrombocytopenia, granulocytopenia) can occur; evaluate baseline and perform periodic blood studies approximately every 2 d while in therapy; discontinue upon appearance of reticulocytopenia, leukopenia, thrombocytopenia, anemia, or findings attributable to chloramphenicol; adjust dose in liver or kidney dysfunction
More on Cystic Fibrosis |
| Overview: Cystic Fibrosis |
| Differential Diagnoses & Workup: Cystic Fibrosis |
 Treatment & Medication: Cystic Fibrosis |
| Follow-up: Cystic Fibrosis |
| Multimedia: Cystic Fibrosis |
| References |
| « Previous Page | Next Page » |
References
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[Best Evidence] Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC. Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med. Jan 19 2006;354(3):241-50. [Medline].
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Gibson RL, Emerson J, McNamara S, Burns JL, Rosenfeld M, Yunker A. Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis. Am J Respir Crit Care Med. Mar 15 2003;167(6):841-9. [Medline]. [Full Text].
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Further Reading
Keywords
cystic fibrosis, CF, CFTR, mucoviscidosis, cystic disease of the pancreas, chronic respiratory infection, pancreatic enzyme insufficiency, airway obstruction, lung infection, lung inflammation, exocrine gland function, progressive lung disease, end-stage lung disease, meconium ileus, distal intestinal obstruction syndrome, DIOS, fecal impaction, intussusception, obstructive cirrhosis, splenomegaly, hypersplenism, malnutrition, hepatic steatosis, right heart failure, gallstones, bronchitis, bronchiolitis, bronchiectasis, cor pulmonale, hemoptysis, pneumothorax, mucocele, mucopyocele, sinusitis, nasal polyps, intestinal obstruction syndrome, rectal prolapse, peptic ulcer, gastroesophageal reflux, volvulus, intestinal atresia, perforation, meconium peritonitis, amenorrhea, delayed sexual development, sterility, hydrocele, rhinitis, scoliosis, kyphosis
