Amálka had a difficult start in life. Harlequin ichthyosis is a very rare disease and it is no wonder that at first not even the doctors had any idea what had brought little Amálka into the world. “At first it was not clear what was wrong with her skin, the doctors told us that her fingers were fused together, that she was in pain and there was only a small chance that she would survive,” recalls Kristýna Ježková, Amálča’s mother.
The first months in hospital
After the birth they spent a month with the baby in the Hradec Králové hospital and then were transferred to Brno. “I was in the mothers’ department, Amálka at the ARO. I went to see her every three hours and the only thing I could espresso milk was her thing,” Kristýna recalls.
Amálka spent the first two months of her life in hospital, doctors trying to find her a suitable treatment. “But they just didn’t know. Maybe there are more harlequins in the country, but even if they were, it’s a very rare and rare disease. It was a challenge, as a family we needed direction, something to hold on to. When I and my husband remember that time, even after almost 10 years, our eyes fill with tears,” says the now mother of five.
Photo: rodiny archive
Amálka plays the violin even though she was born with fused fingers. Photo: Family archive
Ichthyosis, when the skin grows too quickly
The disease has several types, from mild to moderate to severe. But all of them are characterized by the fact that the patient’s skin grows several times faster than normal and is manifested by peeling scales. These skin deposits must be smoothed out and then the skin must be extremely lubricated with a large amount of cream. It dries out quickly, can crack to the point of bleeding, and inflammation often forms. Some patients consume a kilo of body cosmetics in a month, others easily up to ten. “It is the opposite of butterfly wing disease. With them the skin must be cared for as much as possible, while with ichthyosis it must be removed as much as possible, so that the patients feel comfortable, because in simple terms they are covered of skin,” explains Hanka Kadlecová, founder of the Ichthyosis Association.
A demanding lubrication routine
A key part of ichthyosis skin care is frequent, daily lubrication and smoothing. Today it’s easier as time goes by, because they have it as a fixed routine, plus Amálka is already at an age where she helps and can clean herself. “We get up at five in the morning and take intensive care of our skin until seven. We take care of his eyes, his nose and his ears so he can hear, and we fix his face to make him look nice. Then at seven we start cleaning and continue until half past eight”, Kristýna describes their daily care. Then the body needs to be lubricated at school at midday, with the help of Amálka’s assistant, and of course in the evening, which lasts about half an hour.
“On Sunday she takes a big bath, from morning until lunch, or if Amálka wants to sleep, she does it after lunch and it can last until five or six in the evening. We do a head bandage once every 14 days, and this adds another two hours,” Kristýna describes life with the disease. In Amálka, one kilo of body cream is enough for two days and the costs for cosmetics thus rise to tens of thousands per month.
Due to the large layer of skin, correct thermoregulation does not work in patients with ichthyosis and therefore there is a frequent risk of overheating. “When Amálka waits for me even for a moment and forgets to unbutton his jacket in winter, he immediately falls into a state of collapse. You have to keep an eye on him,” describes Amálča’s mother. Summer is the busiest time and Amálka can only go out in the morning or evening, during the day he has to stay at home in an air-conditioned room.
Photo: rodiny archive
Amálka at Christmas with her sister Photo: Family archive
Amálka succeeds in the impossible
When Amálka was born, her fingers were fused together and it was not known whether she would be able to move them. Kristýn and her husband Amálka bandaged their hands and with the help of bandages and ointments they gradually managed to lengthen their fingers. With further practice, the hand began to function normally. “This was a great victory for us, because many other possibilities opened up for us and Amálka is able to handle ordinary things. We continue to say that she is a prodigy. She skis, plays the violin and is not afraid to go out in front of the people and sing in a choir. It’s amazing how much I can do,” says Kristýna.
And how do they deal with their husband? “There are worse days and better days, but we are doing well. In these 9 years we have experience and if something doesn’t feel right on our skin we are already able to react before the disease turns into a bigger infection. Continue to get better and better. At the same time, children are our driving force and we are grateful that our dream of a large family has not faded. We live for each other, this is our strength. Furthermore, we have we are lucky to have fantastic people around us who support us, which we are very grateful for. And one of them is Hanka Kadlecová from the Ichthyosis Association”, concludes Kristýna Ježková.
The Ichthyosis Association unites families affected by this skin disease and also works for greater awareness. A transparent account for a public collection for the purchase of creams and medical supplies for ichthyosis sufferers:
2024-01-16 04:01:33
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