Treatment Of Cystic Fibrosis

There is now a new treatment that the FDA has approved for cystic fibrosis, Kalydeco. It is one pill that is taken twice per day. It seems to be well tolerated, but is expensive. It targets a defective protein that is the cause of cystic fibrosis.

Otherwise treatment is directed at the symptoms of the acute clinical situation first, but also at the overall CF condition in general. When pneumonia is present, this is treated with the appropriate antibiotics and with chest physiotherapy. However, all of the other factors have to be treated also, such as the dietary insufficiency knowing that a certain degree of malabsorption has to be taken into account and certain dietary supplements such as vitamins (particularly D and E) may be required.

This is why many centers offer a comprehensive multidisciplinary team approach to the treatment of cystic fibrosis (CF). In the following table the various organ systems affected by CF that have been mentioned at the beginning of this overview (see the table above entitled "Organ changes in Cystic Fibrosis") has been repeated.

This time, however, the treatment options are listed complete with links to more detailed discusssions in various useful medical sites.

Treatment of Cystic fibrosis
Organ system affected: Description of therapy:
lung tissuemedical treatment with antibiotics for pneumonia or flareup of chronic bronchitis or bronchiectasis; lung surgery for specific problems
pancreas

1. digestive enzymes and other supplementations

2. 10% of CF patients with diabetes mellitus need insulin therapy

98% infertility in males urologist to do investigation, if needed; often counselling required regarding acceptance
decreased fertility rate in womengynecologist may need to be consulted
liver tissueend stage liver cirrhosis may require liver transplant
protruding veins in the lower esophagus that lead to vomiting of bloodesophageal varices as a result of "portal hypertension" from liver cirrhosis may require an emergency endoscopic sclerotherapy
pulmonary hypertensioninitially needs therapy with diuretic therapy; later on needs careful monitoring by cardiologist and a medical team; end stage disease may require heart and double lung transplant
salt depletionloss of salt needs to be replaced with supplements in diet
nasal polypsnasal polyps and nasal infections need ENT specialist attention; chronic recurrent sinus problems may require surgical drainage procedure by ENT specialist
gall stonesgall stones may require surgery (cholecystectomy)

 

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Disclaimer:

This outline is only a teaching aid to patients and should stimulate you to ask the right questions when seeing your doctor. However, the responsibility of treatment stays in the hands of your doctor and you.

References:

1. Noble: Textbook of Primary Care Medicine, 3rd ed., Copyright © 2001 Mosby, Inc.

2. National Asthma Education and Prevention Program. Expert Panel Report II. National Heart, Lung and Blood Institute, 1997.

3. Rakel: Conn's Current Therapy 2002, 54th ed., Copyright © 2002 W. B. Saunders Company

4. Murray & Nadel: Textbook of Respiratory Medicine, 3rd ed., Copyright © 2000 W. B. Saunders Company

5. Behrman: Nelson Textbook of Pediatrics, 16th ed., Copyright © 2000 W. B. Saunders Company

6. Merck Manual: Cystic Fibrosis

7. Goldman: Cecil Textbook of Medicine, 21st ed., Copyright © 2000 W. B. Saunders Company

8. Ferri: Ferri's Clinical Advisor: Instant Diagnosis and Treatment, 2004 ed., Copyright © 2004 Mosby, Inc.

9. Rakel: Conn's Current Therapy 2004, 56th ed., Copyright © 2004 Elsevier

10. Suzanne Somers: "Breakthrough" Eight Steps to Wellness-- Life-altering Secrets from Today's Cutting-edge Doctors", Crown Publishers, 2008

Last Modified: Jan.31, 2012