Cystic fibrosis (CF) is the most common severe genetic disease among people of European descent, affecting about one in three thousand newborns.
CF is a multisystem disease, affecting primarily respiratory and digestive systems. In people affected by CF, mucus and other secretions are thick and sticky. In the lungs, a build-up of thick mucus makes it difficult to remove bacteria and leads to repeated life-threatening infections and diminished lung capacity. Only regular administration of mycolytics (medications that loosen secretions) and antibiotics helps patients with CF maintain comparatively normal lung function.
In the digestive tract, thick mucus blocks the ducts of the pancreas, preventing enzymes from reaching the intestines and making it extremely difficult to digest and absorb adequate nutrients from food. Therefore, persons with CF need to supplement every meal and snack with artificial enzymes to avoid digestive disorders and malnutrition and maintain normal growth. Often they require specialized high-calorie nutritional supplements to reach and maintain a healthy weight.
There is no known cure for CF yet, and in most patients the disease eventually leads to lung failure. Previously most patients affected by the disease died in early infancy; however, great strides have been made in recent decades to improve the situation. The life expectancy of a person with CF in Western countries is now as high as 40 or even 50 years, and, with the existing medications and therapies, patients lead a fairly normal life . They study, work, participate in sports and have their own families (their children will be CF-free if the spouse is not a CF carrier).
With CF, it is crucial that the therapy is lifelong and administered daily, without interruptions, just as an average person with diabetes needs lifelong daily insulin. Every day, people with CF need mucolytics, antibiotics, and pancreatic enzymes. In addition, they need certain medical devices (inhalers etc.) and equipment for regular exercise therapy.
Unfortunately , in Russia and other former Soviet countries very few children born with CF survive into their twenties. The reasons are shortage of financing and interruptions in supply of medications: from mucolytics and antibiotics to specialized nutritional supplements.
The total cost of treatment for a child with CF is estimated to be close to $10,000 per year. In many countries these costs are covered by state-funded or other insurance programs, at least in part. In Russia such programs have not yet been implemented to sufficient extent, and many children, especially from poor provincial regions, get little or no help. The families struggle to raise money for their child's treatment. Many end up losing their children at a very young age. The situation is even more complex with adults. The Russian medical authorities still cannot fully recognize the fact that children with CF can grow into adulthood, and are therefore unprepared to address the treatment needs of adults with CF.
In other countries of the former Soviet Union, the lack of financing is even more severe, making it extremely difficult to provide adequate treatment for children with CF.
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