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What medications and treatments are available?

What medications and treatments are available to reat Cystic Fibrosis?
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replied January 20th, 2018
Thank you for asking at Ehealth forum!

I read your question and i understand your concern.
The goal of treatment in cystic fibrosis is to promote clearance of secretions,to control infections in the lung,to provide adequate nutrition and prevent intestinal obstruction.
Treatment of choice is Gene therapy that is under randomised controlled trial.
Systemwise therapies are;
Lung disease:
Clearing Pulmonary secretions by time honored techniques (excercise,flutter valves,chest percussion),inhaled hypertonic saline 7% and recombinant human DNAse.
Antibiotics are used for infective exacerbation.
Pnemothorax is treated with chest intubation and life threatening hemoptysis with bronchial artery embolization.
Lung transplantation is the ultimate treatment in type 2 respiratory failure.
Gastrointestinal diseases:
Enzyme and fat soluble vitamins replacement are use in case of exocrine pancreatic insufficiency and insulin in case of endocrine pancreatic insufficiency.
Cholestatic liver disease is treated with urodeoxycholic acid.End stage liver disease is treated with liver transplantation.
Genitounrinary disease:
Sperms are taken early in puberty and put in the sperm bank.
Patients are usually depress, so psychotherapy is mandatory.
I hope it helps. Stay in touch with your healthcare provider for further guidance as our answers are just for education and counselling purposes and cannot be an alternative to actual visit to a doctor.
Take care
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