kamal Tayo Oropo

News Agency Of Nigeria

<h1>They are just about a year and three months apart in age, while Shawn clocked 10 last December 10, Alvin will be nine by next March 24.</h1>

Ectodermal Dysplasia (ED) are a group of disorders in which two or more of the ectoderm derived structures — the skin, sweat glands, hair, nails, teeth and mucous membranes — develop abnormally. Each person with an ectodermal dysplasia may have a different combination of defects.

Their mother, Idara Udoh, said that coping with a child with the disorder is tasking, but having to cope with the challenges of two is almost impossible.

“Trust me, you don’t want to know; the horror is better left alone,’’ she told the News Agency of Nigeria (NAN).

NAN reports that both Shawn and Alvin do not sweat, always hot from the inside and are always pouring water on themselves to keep cool; even in the class.

At nine and 10, the two children do not have teeth. They do not have hair; completely bald and they have to cope with significant learning disabilities.

“There is presently no cure for the condition. It’s like Sickle Cell Anaemia; you can only manage it and the downside of it is convulsions.

“Once they overheat, they convulse, especially children under five.

“They pour water on themselves 24 hours, walk around half naked and always drink water,’’ Udoh said.

Medical experts, including Dr Temiye Edamisan, a Dermatologist, and Dr Babafemi Oyekunle, a Pediatric Dermatologist and Endocrinologist, told NAN that, while the disorder may not be fatal on its own, the kids and their family face serious challenges.

Also, Dr Salaudeen Akinsanmi, a family medical practitioner (General Practice) with James Cook University Hospital, Middleborough, United Kingdom, expressed similar concerns.

He added that the challenges could only be more bearable with intervention of the society; both at governmental and non-governmental levels.

According to Akinsanmi, the ailment is a genetic disorder that affects the outer layer of the tissue of the developing embryo — Ectoderm — which makes up the skin, sweat gland, hair, teeth and nails.

“As such, all these structures can be affected. In general, it consists of about 180 disorders depending on the areas affected.&nbsp;It usually does not have significant effect on life expectancy.

“Although, one must pay attention to the problems associated with the disease, including high fever, which can cause seizures, and increased body temperature, which can cause brain damage,’’ he said.

Akinsanmi said there was no specific symptom for the disorder, as symptoms they present with are treated as needed.

He, however, said that in a country like Nigeria, where the healthcare system is relatively disadvantaged, it will be advisable for the parent to pay very close attention to the kids’ health problems.

Edamisan, who is the Chairman, Nigeria Medical Association (NMA), Lagos Chapter, also pointed out that the disorder is inherited, adding that people can live with it but with the hair and teeth growing poorly.

He said the skin and the nails could be affected to various degrees.

“The challenges in the environment is heat.

“We sweat to lose heat, but the kids can’t sweat well. So, they have very high temperature. With little fever,&nbsp;they may convulse and have stroke easily.

“They need to be cooled down most of the time. The environment is very moist, the humidity is high and the skin is one of the first defence against germs their skin can’t do that effectively, so they can have recurrent infections.

“Stigmatisation is also a problem, people in the society tend to look down on them. They have to keep their skin moist most times,’’ he said.

Edamisan said that while Shawn and Alvin can survive to adulthood, their sperm production, as males, may be compromised leading to infertility, as they will not be able to produce fertile sperm.

He explained that “if the heat does not affect them, they may live long, their primary doctors must be dermatologists that will attend to them all their lives and be available at all times.’’

Edamisan also advised that their parents should not be tired of taking them to hospitals.

“The society today is almost lackadaisical about it. That is one of the problems we have.&nbsp;Societal support in Nigeria is very poor and it’s very challenging for this rare condition.

“The country should be able to protect these people but we don’t have such help except a particular group of people decide to take it up and form an awareness association to discuss the problem. But it is very rare,’’ he said.

In this regard, Mrs Udoh, their mother, told NAN of her effort in creating an awareness platform, the African Foundation for Ectodermal Dysplasia (AFED).

“Many people don’t know about the disorder. So, I started an advocacy to create awareness about it across Africa.

“We started this just recently and, so far, we have people from Zambia and Kenya&nbsp;with the condition in the group.

“I am not one to sit down and question the universe when faced with challenges, I face them headlong till I get results. And I think that’s the approach I use for my kids too.

“What has happened has happened. So, what I look for are ways to make life easier for all of us in the family unit.

“I try to build my children’s self-esteem in every step of the way and try to enlighten people when they stare at them strangely that they just have a rare genetic disorder; they are not different from them.

“That’s how we have been coping, some days, it’s hard, other days we get by,’’ she said.

Udoh, a civil servant, however, recalled a day one of the children came home from school crying.

She said that upon enquiry, the child said his teacher brought him out in front of the class and asked the other kids to boo him because at seven there was no teeth in his mouth.

“As a mother, that was painful, I felt like shielding him from the world but I know I can’t.

“Or when the brother said kids laugh at him and sing vampire every time he plays with them because he looks differently.

“It’s not easy to live through such humiliation and agony and as a mum, it’s worse because there’s nothing you can do about it, but tell them not everyone will be as mean.

“I teach them that they should not harbour bitterness or hate in their hearts,’’ she said.

Udoh, fondly called Mma by friends, however, said it is unfortunate that the society mostly haven’t even heard of Ectodermal Dysplasia, wondering, “how does one help a situation like that?’’

She said most health practitioners met or heard of the condition first from her case.

“It’s that bad; and they’ll be using my kids as lab rats on appointment days.

“Family has been supportive, at least, no one taunts them with it and we make it a duty to explain what they have.

“Even the children will tell you straight up and with cute smiles on their faces, ‘I have ED, I’m not like you’,’’ she said.

She noted that the kids need attention regularly, but it’s expensive, as the family could not afford it and so they manage at home, most of the time.

“They have few or no teeth with the condition and fixing of dental implants is in millions of Naira.

“As a civil servant, how can I afford almost twenty million Naira for teeth implants for the two boys?

“I just don’t visit the hospital anymore. The bills were too enormous to continue. We were asked to pay N10 million for dental implants for one son.

“The kids need constant attention, but we manage it as best as we can. And God has been helping us,’’ she said.

In the meantime, and before help comes, the mother said the children make do with only antibiotics; adding that they did this on a regular basis plus loads of multivitamins to&nbsp;always boost their immune system.&nbsp;

Ectodermal dysplasia: The agonies of 2 children with rare skin disorder/Photo used for the purpose of illustration.. [semanticscholar]

Shawn and Alvin Udoh are not twins, but are more identical in the challenges nature has thrown at them than most Siamese twins, as they both battle with Ectodermal Dysplasia, a rare skin disorder.

Ectodermal dysplasia: The agonies of 2 children with rare skin disorder

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kamal Tayo Oropo

Articles written by the author

Ectodermal dysplasia: The agonies of 2 children with rare skin disorder