Charitable Foundation "Ostrova"

to Breathe and to live!

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Cystic fibrosis - general information

General information

Mucoviscidosis(MV), which is also known as cystic fibrosis (CF), is a hereditary disease caused by the mutation of a gene which affects cell membrane permeability. As a result, the secretions of various organs (such as mucus) become highly viscous and dense, adversely affecting systems throughout the body, beginning with those dealing with respiratory and digestive functions. The word «mucoviscidosis» is based on Latin words «mucus» and viscidus — «viscous».

Viscous sputum accumulates in the lungs, causing an inflammatory process which leads to recurrent bronchitis and pneumonia. An agonizing cough is one of the primary symptoms of the disease. Respiratory failure causes the death of the majority of cystic fibrosis patients.

Imagine living within the confines of a gas mask which is gradually failing. That is, in effect, the life of many cystic fibrosis sufferers, some of whose lungs are only 25% functional.

Because of a lack of pancreatic enzymes and the presence of viscous mucus in the gastrointestinal tract, cystic fibrosis patients cannot digest food normally. Despite an increased appetite they fall behind in growth and weight.

Cystic fibrosis is the most widespread of all hereditary diseases. Out of any 20 people, one will be a carrier of the defective gene. If both parents are carriers, then the probability that their child will have cystic fibrosis is 25%.

Cystic fibrosis patients are not contagious. They are not only mentally sound, but often talented. There are many gifted and intelligent people among their number and they are frequently particularly successful in activities which require concentration; they study foreign languages, read and write, engage in creative efforts, and can become remarkable musicians, artists and writers. Cystic fibrosis patients can marry and have healthy children.

Cystic fibrosis is one of the few genetic diseases which can be corrected. The regular administration of modern medicines, treatment and prophylactic techniques can make a productive life possible, and the average lifespan for cystic fibrosis patients has been steadily growing: now, in developed countries, it approaches 50 years (sadly in Russia, at present, it is only half that). The administration of enzymes can assist in restoring digestive functions, and kinesiotherapy and drugs may be used to evacuate the majority of the sputum from the lungs, and to normalize their work.

Kinesiotherapy is an especially important method of treating the disease. The organs most affected by cystic fibrosis are the lungs, the main reason for this being the constant presence of dense, easily infected sputum in the bronchi. Medicines are capable only of decontamination(antibiotics), reducing viscosity and stickiness (mucolytics), or assisting in the dilatation of the bronchi (bronchial spasmolytics). It is much more effective not merely to disinfect and modify the sputum's consistency, but to completely remove it from the lungs, leaving nothing to decontaminate.

Here is a crude but effective analogy. Imagine a bucket filled with viscous, mouldy waste. In order to halt the rotting process, we can add sterilizing chemicals. The mixture will not be infectious, but it is unusable! But another solution is possible; to remove, pump out or pour out the liquid. After this procedure, and a wash of the insides of the bucket, our vessel is functional again.

You can read about the kinesiotherapy method, including specific examples of its application, elsewhere on this page.

Abroad, in economically developed countries, cystic fibrosis patients live longer also because they have access to better medicines, which are very expensive. The Russian Government allocates funds for free medicines for CF patients. These are currently inadequate to meet domestic demand and are often difficult o obtain, especially at a local level.

It is vitally important for CF patients and their parents or carers to know their rights and to understand how they can assert them. Our foundation can provide you with relevant legal advice.

But this is not enough. Existing rights need to be both defended and extended. Public organizations play a significant role in this struggle. Some have already been established in Moscow and in other Russian cities: We encourage you to use these as an example, and to set up your own. The goal of this foundation is to goal is to help you to do so.

Futher reading about cystic fibrosis:

Interview with professor N.I. Kapranov, Medicforum, 02.03.2012

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