This combination of previously developed medicines is able to treat up to 90% of people with cystic fibrosis. This medications restores some effectiveness of the CFTR protein so that it can work as an ion channel on the cells surface. Ecological therapy
It has previously been shown that inter-species interactions are an important contributor to the pathology of CF lung infections. Examples include the production of antibiotic degrading enzymes such as β-lactamases and the production of metabolic by-products such as short-chain fatty acids (SCFAs) by anaerobic species, which can enhance the pathogenicity of traditional pathogens such as Pseudomonas aeruginosa. Due to this, it has been suggested that the direct alteration of CF microbial community composition and metabolic function would provide an alternative to traditional antibiotic therapies. Society and culture
Sick: The Life and Death of Bob Flanagan, Supermasochist, a 1997 documentary film
65 Redroses, a 2009 documentary film
Breathing for a Living, a memoir by Laura Rothenberg
Every Breath I Take, Surviving and Thriving With Cystic Fibrosis, book by Claire Wineland
Five Feet Apart, a 2019 romantic drama film starring Cole Sprouse and Haley Lu Richardson
Orla Tinsley: Warrior, a 2018 documentary film about CF campaigner Orla Tinsley
The performance art of Martin OBrien
Continent Chasers, traveller and CF patient documenting travel and CF blogs, continentchasers.com

Notes
References
Further reading
External links

Search GeneCards for genes involved in cystic fibrosis
Cystic Fibrosis Mutation Database
"Cystic Fibrosis". MedlinePlus. U.S. National Library of Medicine.
Imiglucerase is a medication used in the treatment of Gauchers disease.It is a recombinant DNA-produced analogue of the human enzyme β-glucocerebrosidase. Cerezyme is a freeze-dried medicine containing imiglucerase, manufactured by Genzyme Corporation. It is given intravenously after reconstitution as a treatment for Type 1 and Type 3 Gauchers disease. It is available in formulations containing 200 or 400 units per vial. The specific activity of highly purified human enzyme is 890,000 units/mg, meanwhile the enzyme activity produced by recombinant DNA technology is approximately 40 units/mg. A typical dose is 2.5U/kg every two weeks, up to a maximum of 60 U/kg once every two weeks, and safety has been established from ages 2 and up. It is one of more expensive medications, with an annual cost of $200,000 per person in the United States. Imiglucerase has been granted orphan drug status in the United States, Australia, and Japan.Cerezyme was one of the drugs manufactured at Genzymes Allston, Massachusetts plant, for which production was disrupted in 2009 after contamination with Vesivirus 2017. Side effects
The most common side effect is hypersensitivity, which occurs in about 3% of patients. It is associated with symptoms such as cough, shortness of breath, rashes, itching, and angiooedema. Less common side effects include dizziness, headache, nausea, diarrhoea, and reactions at the injection site; they are found in less than 1% of patients. Interactions
No clinical interaction studies have been conducted. Miglustat appears to increase the clearance of imiglucerase by 70%, resulting in decreased enzyme activity.