Mikołaj

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 12 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, 15-year old Olek and 25-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

Request for help

Mikolaj weighs only 34 kg – and taking into account his age (12 and a half) and his birth weight he should weigh at least 37 kg. For us this extra 3000 grams is a dream weight. In summer our son weighed more but he lost weight in autumn after another disease – he loses weight every time he is ill, which is very often.

Mikolaj has to gain weight in order to develop, grow, have strength and immunity to fight subsequent infections. The nutritional supplements (Protifar, Fantomalt and Nutridrink) prescribed at hospital are not refunded and cost respectively 25€, 31€ and 57€ and suffice for one month, though we soon are supposed to give him more. It makes 113€ a month for the nutritional supplements only (8 cans and 60 bottles).

Unfortunately we can only afford medication and vitamins (extra 55€ a month) and high calorie diet, i.e. premium products – full fat diary products, high-quality meat, etc. Besides that we have everyday expenses on clothes, shoes, toys.

Because of Mikolaj’s disease and intensive care he requires I have to stay at home. I have given up permanent work and I work as a freelancer. Medications, vitamins, nutritional supplements and the rehabilitation equipment are only partly refunded. We have to pay for journey to the clinic and medical appointments at various specialists. My husband’s earnings must be divided into five uneven parts (most is spent on Mikolaj, but our oldest son who suffers from diabetes also needs our care and attention).

We, Mikolaj’s parents, know that nowadays there is no hope for our son but we believe with our whole hearts that the cure for his disease will be invented in his lifetime. The only thing we can do is to make our beloved Mikolaj live up to this moment but the proper care of him is beyond our financial capabilities. We ask you for support in our fight for the least painful and the longest life for Mikolaj.

Mikolaj’s history

His birth

Mikolaj, our third son, was born on 5th August 2004 unaided, on the day of his mum’s birthday what was her greatest present. He weighed 3150 grams and he got 10 points in the Apgar scale.

Problems

In the third month of his life Mikolaj was diagnosised with severe anaemia treated with iron preparations and lack of weigh increase – he wasn’t strong enough to suck at the breast longer, had diarrhea, was always hungry and slept badly. Mikolaj had to be fed with supplements mixed with pumped breast milk.

Hospital

In the fifth month of his life Mikolaj went down with viral bronchitis, which transformed into pneumonia to which came stomach flu caused by adenoviruses. He spent a month at hospital – in the first week doctors were fighting for his life, then he was recovering slowly. As he could neither eat nor drink and kept vomiting, his anaemia deepened and he had blood transfusion.

Development

Mikolaj was three-month retarded in physical development (he got a medical certificate of disability). As he was recommended rehabilitation, we went with him to massages and exercises with him every week and we practiced with him at home. He had his heart examined and ultrasound of the head to reveal heart defect (fortunately cardiologists diagnosed it as not severe) and muscle tone impairment which manifests itself as deviation of feet and lumbar hyperlordose. Mikolaj had regular medical appointments with a neurologist.

Diagnosis

His low weight (he looked as if he was malnourished), big appetite and constant diarrhea worried us and we went to a gastroenterologist. The check-ups results were not bad, but additional digestive test showed fat in stool. After first sweat test we heard the diagnosis first time – CF. As there are no specialist in this field in Bielsko, we were sent to Katowice to The Centre of Mother’s and Child’s Health (Centrum Zdrowia Matki i Dziecka). The check-ups showed abnormal twofold bigger than the norm amounts of sodium and chloride in our son’s sweat. From Katowice we were sent to Rabka where repeated tests, also genetic, proved the diagnosis.

Acknowledgements

Mikolaj is fortunate in his hard fate to have met many sympathetic people at every stage of his life. We would like to deeply thank the various people who provided us with helpful assistance and care, among others the senior registrar of the infant ward of a paediatric hospital in Bielsko-Biała who fought for his life and made the only right decisions to save him, his doctor from the Clinic for Pneumonology and Cystic Fibrosis (Klinika Pneumonologii i Mukowiscydozy Instytutu Chorób) in Rabka, who explained us calmly and to the point how we could help our son in the unfair fight with his illness, the ladies from Polish Association of Cystic Fibrosis (Polskie Towarzystwo Walki z Mukowiscydozą), who welcomed us warmly to the premises of the Association, gave us advice and spiritual support; and our friends who helped us to prepare this website. Thank you very much! With such a support Mikolaj will not feel his illness so acutely and we will help him with all our strength.

We would like to thank Polish Association of Cystic Fibrosis for a one-time financial help of 560 € which we spent on supplements for Mikolaj.

We would like to thank employees of the firm ARGOS from Cracov for a financial help for Mikolaj.