DRUKUJ
Mikolaj
Welcome to Mikołaj Malcew's website. Mikolaj suffers from a genetic disease called Cystic Fibrosis (pancreas fibrocystic disease) of the severest type, caused by mutations in the gene F508del/F508del. Prognosis of the disease is bad, though not as bad as 30 years ago, when children had a lifespan of not more than 3 years. Mikolaj can live even to 40 (on average individuals with CF in Poland have a lifespan of approximately 20 years).
At present the disease is affecting digestive system the most severely – though our son is hungry all the time and eats a lot, he assimilates food very badly and very often excretes it in the form of undigested, thin and fatty faeces. Malabsorption leads to malnutrition and poor growth and Mikolaj doesn't gain weight or height at the same rate as their peers, he is shorter and thinner than they are. Mikolaj has been taking medication supporting digestion and food assimilation (pancreatic enzyme). We also give him high calorie diet and nutritional supplements to provide a bigger dose of energy for him and enhance his growth.
As sticky mucus accumulating in his lugs makes breathing hard to him, Mikolaj inhalates Pulmozyme. To dislodge sputum from his organism we utilize physical therapy – drainage (pounding) and aerobic exercises, among others on a springboard. When our son is cold or ill, which is very often, we have to intensify both the number and duration of inhalation and drainage.
Majority of children with cystic fibrosis has staph injection in the throat, so does Mikolaj. He regularly undergoes antibiotics therapy to decrease the number of staph strains (it can't be eliminated entirely). Furthermore he has been diagnosed as having muscle tone impairment which causes curvature of the spine, so we regularly go to rehabilitation with him.
We are very proud of Mikolaj, who though only 7 and a half years old, has been enduring his disease and all inconveniences related very bravely. He enjoys his life, he loves playing with his brothers, 10-year old Olek and 20-year old Jarek, and he is a warm, intelligent and cheerful boy.
What is cystic fibrosis?
Cystic fibrosis (CF) is a hereditary disease that affects mainly the lungs and digestive system, causing progressive disability. It is a life-threatening disease that causes mucus to build up and clog some of the organs in the body, particularly in the lungs and pancreas. It affects the entire body and impacts breathing, digestion, and sexual reproduction. Currently, there is no cure for cystic fibrosis. The treatment for cystic fibrosis continues throughout a patient's life, is very expensive and is aimed at maximising organ function, and therefore quality of life. At best, current treatments delay the decline in organ function.
Links
en.wikipedia.org/wiki/Cystic fibrosis
www.cff.org
www.cysticfibrosis.com