11 October 2017 Read: 476
Nine-year-old Ciara Kotze’s skin is as fragile as a butterfly wing. Ciara was born with a rare disorder called epidermolysis bullosa, commonly known as EB. Ciara’s EB is the more rare form called junctional EB. The disorder affects one in 50,000 people.
Ciara’s father Divan (30) moved the family from Worcester in the Cape to Tzaneen recently to take up a post as assistant manager in charge of compost on a farm outside town.
Mother Monique was only 17 when she gave home birth to Ciara at her mom’s place in Durban. She says that there were early labour signs at five months. Doctors stopped the labour and Monique went 1½ months later into labour again. Ciara was born with a mass of black hair, skin on her face and none on the rest of her body.
Blisters began appearing on her body at three months. At seven months the blisters started on her little face. Monique now opens those blisters, disinfects, treats and cleans them thoroughly. Otherwise the blisters simply grow bigger and get harder. All Ciara’s hair fell out in the last year and a half. She is bathed daily and Divan is 100% there for his fragile daughter too. A bath takes half an hour but if the parents have to open up blisters, the bath can take an hour.
There is no treatment for junctional EB and the Kotze couple have learnt how to deal with Ciara’s needs themselves. They spend in excess of R10, 000.00 a month for medication and other necessities. The most expensive product is Keragel T. This is the only treatment that strengthens the skin. It is imported from New Zealand and costs R3, 500.00 for 50g. Ciara also needs five bandages a week, Monique needs surgical gloves. Then there’s a host of tablets, including morphine syrup, herbal antibiotics, painkillers, fluoride tablets for her crumbling teeth and vitamin D drops.. Ciara is constipated most of the time and this is due, in part, to the sphincter that can’t stretch. For constipation relief Ciara is administered Movicol.
Ciara was born deaf but can lip read and is highly intelligent. The child gets to eat soft food when her mouth is sore, as this too presents with blisters. Ciara walks oddly as her feet are full of blisters and two of the toes have begun fusing. Ciara also feels cold and heat more than most people and she’s prone to flu which often leads to bronchitis. Already Ciara’s immunity has gone down badly this year.
Divan and Monique also have a healthy four-year-old son, Duhan. When pregnant with Duhan, Monique had genetic testing done and ultra sounds to ensure that EB was not present this time.
Junctional EB is a defect in anchoring the epidermis and dermis. Monique states there is no cure and children with junctional EB do not live beyond the age of 12. She says that from the skin EB moves to the bones and then to the organs. Already Ciara is presenting with stomach and kidney issues. For now it’s a case of managing the problems as they arise and keeping Ciara as pain free, hydrated and as healthy as possible.