Treatment Of Cystic Fibrosis

Treatment is directed at 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 tissue	medical 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 women	gynecologist may need to be consulted
liver tissue	end stage liver cirrhosis may require liver transplant
protruding veins in the lower esophagus that lead to vomiting of blood	esophageal varices as a result of "portal hypertension" from liver cirrhosis may require an emergency endoscopic sclerotherapy
pulmonary hypertension	initially 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 depletion	loss of salt needs to be replaced with supplements in diet
nasal polyps	nasal polyps and nasal infections need ENT specialist attention; chronic recurrent sinus problems may require surgical drainage procedure by ENT specialist
gall stones	gall 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:

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

6. Merck Manual: Cystic Fibrosis

Last Modified: Feb. 1, 2008