eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Cystic Fibrosis: Follow-up

Author: Girish D Sharma, MD, Associate Professor, Department of Pediatrics, Rush University Medical Center, Rush Children's Hospital; Director of Pediatric Pulmonary Section and Rush Cystic Fibrosis Center
Contributor Information and Disclosures

Updated: Oct 17, 2008

Follow-up

Further Outpatient Care

  • Patients are monitored in the cystic fibrosis (CF) clinic every 2-3 months to achieve the following goals:
    • Maintenance of growth and development
    • Maintenance of as nearly normal lung function as possible using clinical assessment, pulmonary function testing, and oxyhemoglobin saturation
    • Intervention and retardation of the progression of lung disease via appropriate use of antibiotics, bronchodilators, and airway clearance techniques
    • Clinical assessment to monitor gastrointestinal tract involvement and presence of malabsorption and to provide enzyme and nutrition supplementation
    • Monitoring for complications and their treatment
    • Addressing psychosocial issues
  • Airway clearance techniques: Various techniques used to clear airways may include chest physical therapy by hands, forced expiratory technique and autogenic drainage, positive expiratory pressure (PEP) using a PEP mask, high-frequency oscillation using a Flutter device, and high-frequency chest compression using a ThAIRapy Vest. These can be combined with bronchodilator therapy via a nebulizer.

Inpatient & Outpatient Medications

  • Pancreatic enzyme supplements
  • Multivitamins (including fat-soluble vitamins)
  • Mucolytics
  • Nebulized, inhaled, oral, or intravenous antibiotics
  • Bronchodilators
  • Anti-inflammatory agents
  • Agents to treat associated conditions or complications (eg, insulin)

Complications

  • Nasal polyps
  • Chronic and persistent sinusitis with complications such as mucopyocele formation
  • Bronchiectasis
  • Atelectasis
  • Pneumothorax
  • Hemoptysis
  • Hypertrophic pulmonary osteoarthropathy
  • Allergic bronchopulmonary aspergillosis (ABPA)
  • Gastroesophageal reflux
  • Pulmonary hypertension
  • Cor pulmonale
  • End-stage lung disease
  • Pancreatitis
  • CF-related diabetes mellitus
  • Meconium ileus
  • Distal intestinal obstruction syndrome
  • Rectal prolapse
  • Vitamin deficiency (especially fat-soluble vitamins)
  • Fatty liver
  • Focal biliary cirrhosis
  • Portal hypertension
  • Liver failure
  • Cholecystitis and cholelithiasis
  • Rickets
  • Osteoporosis

Prognosis

  • Prognosis in patients with CF has improved over the last few decades, but CF remains a life-limiting disease, and a cure for the disease remains elusive.
  • Median survival age is 36.8 years.
  • The median survival age in males is slightly higher than that in females.
  • Marked heterogeneity is observed in disease severity and progression.
  • Severity of pulmonary disease determines prognosis and ultimate outcome.
  • With current treatment strategies, 80% of patients should reach adulthood.

Patient Education

  • Provide counseling at the time of initial diagnosis, including information regarding inheritance and risk for recurrence in subsequent pregnancies.
  • Instruct patients and parents regarding appropriate airway clearance technique and the need for chest physical therapy.
  • Instruct patients and parents regarding the use of various drug delivery devices, such as spacers and nebulizers.
  • Instruct patients and parents regarding methods for modifying the pancreatic enzyme dosage.
  • Discuss when to contact CF center personnel (eg, for acute pulmonary exacerbation or complications) with patients and parents.
  • Be prepared to counsel families regarding the impact of the diagnosis on the emotional life of parents, siblings, and members of the extended family.

Miscellaneous

Medicolegal Pitfalls

  • Failure to suspect and diagnose cystic fibrosis (CF) in a child presenting with atypical symptoms (A child can never look too well to have CF.)
  • Failure to counsel families on inheritance and recurrence risk
  • Failure to avoid fibrosing colonopathy caused by excessive dosages of pancreatic enzymes

Special Concerns

  • Women with CF can have successful pregnancies provided that special care is taken with their nutrition (vitamin and energy supplements), airway clearance, and treatment of respiratory infections. Patients and their partners should attend counseling, including genetic counseling, when planning for pregnancy.
 


More on Cystic Fibrosis

Overview: Cystic Fibrosis
Differential Diagnoses & Workup: Cystic Fibrosis
Treatment & Medication: Cystic Fibrosis
Follow-up: Cystic Fibrosis
Multimedia: Cystic Fibrosis
References
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References

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  2. Collaco JM, Vanscoy L, Bremer L, et al. Interactions between secondhand smoke and genes that affect cystic fibrosis lung disease. JAMA. Jan 30 2008;299(4):417-24. [Medline].

  3. Sharma GD, Doershuk CF, Stern RC. Erosion of the wall of the frontal sinus caused by mucopyocele in cystic fibrosis. J Pediatr. May 1994;124(5 Pt 1):745-7. [Medline].

  4. Cystic Fibrosis Genetic Analysis Consortium. Cystic fibrosis mutation database-. CFMDB Statistics. Available at http://www.genet.sickkids.on.ca/cftr/StatisticsPage.html. Accessed September 25, 2008.

  5. LeGrys VA, Yankaskas JR, Quittell LM, Marshall BC, Mogayzel PJ Jr. Diagnostic sweat testing: the Cystic Fibrosis Foundation guidelines. J Pediatr. Jul 2007;151(1):85-9. [Medline].

  6. Comeau AM, Accurso FJ, White TB, et al. Guidelines for implementation of cystic fibrosis newborn screening programs: Cystic Fibrosis Foundation workshop report. Pediatrics. Feb 2007;119(2):e495-518. [Medline]. [Full Text].

  7. Hale JE, Parad RB, Comeau AM. Newborn screening showing decreasing incidence of cystic fibrosis. N Engl J Med. Feb 28 2008;358(9):973-4. [Medline].

  8. Ren CL, Brucker JL, Rovitelli AK, Bordeaux KA. Changes in lung function measured by spirometry and the forced oscillation technique in cystic fibrosis patients undergoing treatment for respiratory tract exacerbation. Pediatr Pulmonol. Apr 2006;41(4):345-9. [Medline].

  9. Yankaskas JR, Mallory GB Jr. Lung transplantation in cystic fibrosis: consensus conference statement. Chest. Jan 1998;113(1):217-26. [Medline].

  10. Liou TG, Adler FR, Cox DR, Cahill BC. Lung transplantation and survival in children with cystic fibrosis. N Engl J Med. Nov 22 2007;357(21):2143-52. [Medline].

  11. Allen J, Visner G. Lung transplantation in cystic fibrosis--primum non nocere?. N Engl J Med. Nov 22 2007;357(21):2186-8. [Medline].

  12. [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].

  13. [Best Evidence] Elkins MR, Robinson M, Rose BR, et al. A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med. Jan 19 2006;354(3):229-40. [Medline].

  14. [Best Evidence] Flume PA, O'Sullivan BP, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med. Nov 15 2007;176(10):957-69. [Medline].

  15. 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].

  16. Saiman L, Marshall BC, Mayer-Hamblett N, et al. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. Oct 1 2003;290(13):1749-56. [Medline].

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Further Reading

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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

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Contributor Information and Disclosures

Author

Girish D Sharma, MD, Associate Professor, Department of Pediatrics, Rush University Medical Center, Rush Children's Hospital; Director of Pediatric Pulmonary Section and Rush Cystic Fibrosis Center
Girish D Sharma, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, and Royal College of Physicians of Ireland
Disclosure: Nothing to disclose.

Medical Editor

Susanna A McColley, MD, Director of Cystic Fibrosis Center; Head, Division of Pulmonary Medicine; Associate Professor, Department of Pediatrics, Children's Memorial Medical Center of Chicago, Northwestern University
Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, and American Thoracic Society
Disclosure: Genentech Honoraria Speaking and teaching; Genentech Consulting fee Consulting; Novartis Consulting fee Consulting; Altus Consulting fee Consulting; Axcan Scandi Consulting fee Consulting; Boston Scientific Consulting fee Consulting

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Charles Callahan, DO, Professor, Deputy Chief of Clinical Services, Walter Reed Army Medical Center
Charles Callahan, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American College of Osteopathic Pediatricians, American Thoracic Society, Association of Military Surgeons of the US, and Christian Medical & Dental Society
Disclosure: Nothing to disclose.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians
Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD, Attending Physician, Pediatric Pulmonary Medicine, Columbia University Medical Center; Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons
Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society
Disclosure: Merck Honoraria Speaking and teaching

 
 
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