By Kamal Tayo Oropo, News Agency of Nigeria (NAN)
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.
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.
Ectodermal Dysplasia (ED) are a group of disorders in which two or more of
the ectodermally 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.
Courtesy en.wikipedia.org
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. 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 challenge 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, 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. 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 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 always boost their immune system. (NANFeatures)
