In The Shadow of Virtual Absence of Health Care ‘Butterfly Children’ Living with their flayed skin
Um Samir Cannot forget the terror she felt when she saw her son,
merely 8 years old, fall off his bicycle when his skin was flayed
‘simply as if a glove coming off a hand’ she describes. Despite
knowing the nature of her son’s disease ‘Epidermolysis Bullosa
Acquisita’, which he has suffered from since his birth. It was the
doctors’ and one public hospital’s refusal of treatment due to
lack of knowledge and ways to treat this disease, that doubled her
feelings of shock and pain, which ultimately led her to go back
home empowered by an unknown source as she says, and managed to
restore her son’s flayed skin after sanitizing it.
‘Epidermolysis Bullosa Acquisita’ is a rare genetic disease that
inflicts blisters or water bubbles in skin layers, and causes
severe itching that leads to skin lacerations or molting. In some
cases, the blisters might be internal which damages the body’s
internal organs. It is estimated that around 50 in every one
million people are affected by this disease around the world
[Wikipedia].
Victims of Epidermolysis Bullosa (also known as Butterfly Children
as newborn victims’ skin is as fragile as butterfly wings) suffer
from several issues starting from birth and before discovery of
the disease, which requires testing before diagnosis. Most
affected children suffer painful and dangerous wounds due to
medical mistakes, which comes from the lack of medical expertise
in handling the disease. When we reached out to a few
dermatologists to discuss the disease many of them asked for its
name in English, as they have not heard about it in Arabic before,
and have no information regarding it nor have they encountered any
patients suffering from it.
Rare Genetic Disease
Dr.Nour Al Ma’ani, Dermatologist in the Jordanian University
Hospital, explains the nature of this rare disease. Saying that it
is caused by a defect in some of the skin’s components that bond
it together. The skin is naturally made from an external layer
(epidermis) and another layer below it (dermis), with the two
layers meeting at a point called the basilar membrane. With the
different types of Epidermolysis Bullosa being largely determined
by the layer of skin where the blisters are formed, as some might
form in the external layer while others in the deeper one.
She adds, “The skin’s fragility leads to the formation of bubbles
that are caused by friction, and in some cases pressure on the
skin tears these bubbles open. The disease appears more frequently
in areas where close family intermarriages are incest is marriages
between brother and sisters common. The affected gene can be
transmitted by one of the parents (autosomal dominant
inheritance), or from both parents (autosomal recessive
inheritance), or it can develop as a new genetic mutation in the
victim. Symptoms of this he disease does not appear before birth
as the fetus is usually protected from shocks inside the womb. The
disease is usually discovered either at birth or during the
following days or weeks when bubbles or blisters form on the skin,
and in some cases the disease manifests at an older age.”
Um Samir is married to her cousin and has three sons, two of them
are suffering from the disease. Due to the hardships she is
enduring with her two sons, she has decided not to have any more
children, in fear that they might be born with the disease as
well.
Rawan is also a mother of a child suffering from the disease. She
resorted to the internet to gather information, and determine ways
to handle her child’s illness, and started treating him herself.
She made contact with Um Samir through a group on social media,
and compared their experiences. She says, “When I gave birth to my
child, as soon as the doctors held him his foot was injured. And
after a few tests it was determined that the newborn was suffering
from this rare disease.”
No Statistics, No Studies
Dr. Adel Al Shawabke, head of dermatology at the ministry of
health, says that the ministry deals with Epidermolysis Bullosa,
which is caused by a genetic defect, as it does with any other
disease. Since there isn’t a known cure for the disease, the
patient is given creams and anti-inflammatory medicine. He also
clarifies that doctors don’t have any special guidelines to help
instruct patient’s parents how to better care for their children;
and confirms the absence of statistics at the ministry regarding
the number of people suffering from this disease.
The Association of Private Hospitals in Jordan also confirms,
through its president Dr.Fawzi Al Hammouri, the absence of any
sectors in private hospitals capable of treating patients
suffering from Epidermolysis Bullosa, and those hospitals also
don’t keep any data regarding patients suffering from this
disease.
According to Dr.Al Ma’ani, some children are born with flayed skin
caused by the friction of feet rubbing together, while others’
skin is torn off due to medical bandages. Which is what happened
to Um Samir’s son after a medical operation on his esophagus. The
doctor goes on to say that some minor cases cause small bubbles
due to friction, while others form bigger bubbles that might occur
internally in the intestines and might cause internal bleeding;
and finally there might be extreme cases where the Epidermolysis
is permanent which might sometimes cause cancer.
In Jordan, until this day, there have not been any medical studies
or experiments using stem cells to treat Epidermolysis Bullosa,
confirms Dr.Mohammed Al Tarawineh, acting head of the physicians
syndicate. He says highlights brings attention to the fact that
our investigation is the first to shed light on the ‘Butterfly
Children’ and their suffering, and shows interest in cooperating
in the creation of an association dedicated to their issue after
getting permission from the Ministry of Health.
Um Samir painfully recounts, “After my son’s operation on his
esophagus, I could not recognize him. The doctors fixed the tube
connected to his mouth with medical tape, and when they tried to
remove it, the skin on his face completely went off and his
features disappeared”. As for Rawan, she says, “My son remained in
prematurity for a month, and the gauze on his stomach was fixed
with medical tape! When I went to take him from the hospital there
was medical tape on his head, and when the nurse tried to remove
it his scalp came off along with hair follicles and some of the
skin off his face. When I asked the doctor about how much his
disease affects his movement, speech, or even his mind, he replied
that he did not know!”
She goes on to say, “When the water bubbles formed on his body the
doctors asked to cover them with medical gauze, but it was clear
afterwards that the bubbles needed to be removed and not covered.
Moreover, when his anal region was injured the doctors refused to
operate on it due to its sensitivity, so I decided to treat it
myself at home. One day his fingers fused together and the doctors
refused to treat them, which forced me to cut the fused skin by
myself and treat for months until his hand almost went back to its
normal shape.
Specialized Clinic Needed
Patients suffering Epidermolysis Bullosa require a special kind of
clothing made of thin fabric that does not cause injuries or
molting. The patient might suffer from injuries around the mouth,
esophagus, and anal areas. In some cases these injuries can cause
scars and cysts that affect the patient’s movement, according to
the American scientific research association ‘Debra’
.
Dr.Al Ma’ani clarifies, “Patients require an inclusive treatment
by a cadre of specialists including Dermatologists,
Gastroenterologists – especially for those suffering from
esophagus contractions, cosmetic experts, in addition to natural
treatments. However, no cure for the disease currently exists, as
the treatments available are merely preventative aiming to ease
the burden on the skin and its dissolution, and are overall quite
expensive”. She goes on to note that patients in Jordan are in
need of a specialized clinic that saves them the effort of going
to several clinics, not to mention that such a clinic will provide
a suitable environment to conduct studies around the disease, and
will increase doctors’ experience in dealing with it”.
Prices of some medications used as remedies:
Prices in Jordanian Dinar
3,5
Dinars
6,5
Dinars
14,5
Dinars
4
Dinars
6,5
Dinars
3,00
Dinars
17,20
Dinars
