Cystic Fibrosis (CF) is a genetic disorder which causes production of abnormally thick mucus, gastric gland secretion, bile, etc.
Due to the thickened mucus people with cystic fibrosis develop frequent and severe lung infections, and malfunction of pancreas and liver leads to difficulty absorbing food up to severe malnutrition. Without receiving persistent, continuous and adequate therapy patients with CF gradually develop irreversible changes in their lungs, pancreas and liver, up to complete failure of these organs.
No cure for cystic fibrosis is known, however, provided the patients receive adequate therapy, the average life expectancy is 50 years in the developed world, and, in case of a lung (and sometimes liver) transplantation it can be extended by another 10-15 years. There are about 800 persons with CF in Ukraine and only 17% of this number has crossed the 18-year threshold.
Without receiving adequate treatment (special devices, medical equipment, supplemental nutrition), the majority of patients dies from respiratory failure or the disease-related complications (hepatic cirrhosis, diabetes, severe malnutrition, etc.).
The following items are required in order to provide Ukrainian people with cystic fibrosis with the same life expectancy and quality as the one enjoyed by European CF patients:
- High quality portable pulse oximeters (Nonin 9500 or 9590) – devices which monitor oxygen saturation in a patient’s blood and are a must-have for every CF patient. The cost of one unit in Ukraine is ~ 600 UAH, in the US - ~ $22.
- Oxygen concentrators – devices which separate and concentrate oxygen from an ambient air and deliver the purified oxygen to a patient, required at advanced stage of the disease. The concentrators do not only allow seriously ill patients to stay at home rather than in intensive care units, but significantly increase their life span and improve the quality of life. Availability of an oxygen concentrator at a patient’s home allows him/her to stay active longer, have better nutrition, plus he/she is at a lesser risk of acquiring a life/health threatening nosocomial (hospital-acquir
- High quality pressurized inhalers – every patient needs it from the very first day of being diagnosed with CF. CF patients require 2-5 inhalations per day. Specific types of inhalers, such as PARY BOY nebulizers, are required to optimize using of the drugs (which are often quite costly). Their cost in Ukraine ranges from 4500 to 5,000 UAH.
- Sterillizers - Serious infections’ bacteria settling in the lungs cause chronic lung disease, keeping children away from breathing. It means strict requirements not only for the cleanliness but also to the sterility. It implies that small cups of the inhalation devices, inhalation trainers and sometimes even utensils shall be sterilized after every use. Every day for life. The life itself depends upon it.The cost of one sterilizer is 1974 UAH.
- Pulmozyme – the one-of-a-kind medicine which helps thin and loosen CF patient’s mucus. Its continuous administration significantly slows down lung destruction, reduces the rate of respiratory tract infections and extends the period of active life style for a patient. The cost of 1-month treatment course per patient is ~ 18,000 UAH and its administration cannot be discontinued.
- Antibiotics and various medicines aimed at maintenance of a patient’s health, which help prevent or treat some of the CF complications. Antibiotics are an indispensable part of CF patients’ therapy.
- Special nutrition – required for many patients, especially children during their active growth period, as well as in acute phase of the disease when patients are unable to absorb sufficient amount of nutrients from regular food and sometimes have critically low weight. Unfortunately the supplements cannot be obtained in Ukraine and the cost of a 1-month supplemental nutrition per patient (such as Skandishake, Skandical/ USA or Calshake/ Germany) is ~2,000 UAH.
- Special vitamins (aquADEKs) – cystic fibrosis patients have trouble absorbing some of the vitamins from regular food and require special vitamins. The cost of such multivitamin supplement is ~ $27 or $39 per month.
Yes, there is no known cure for people with cystic fibrosis. However, there is a huge difference between an option to die as a 10-year old child at ICU or an option to reach 40 years of age, while being surrounded with your family and friends and being able to study, work, go for walks…
The difference which is LIFE-long.