A blood line for sufferers of genetic illness

It looks like a nursery, except for the bandages wrapped around the children's hands.

The Thalassaemia Centre in Dubai, just off Al Wasl hospital, serves about 700 people with thalassemia and other blood-related illnesses.

The bandages are from the once-monthly blood infusions most patients here receive.

Children take it well. Twelve-year-old Abdul Rahman from Al Ain sits in the recreation room with friends, blood gushing through a small tube into his skinny body.

"Sometimes it takes eight hours for the blood to come in," Abdul Rahman explains, "but it's better than another hospital I used to go to. They are quick here."

His father moved to Ajman to be closer to the Thalassaemia Centre, one of the best places, patients said, to treat the illness.

Abdul Rahman does not know why he and his brother have thalassaemia.

Walid, 15, sits close to Abdul Rahman. He does not know either.

He waits for his young niece to get a blood transfusion.

"It's a dangerous illness, and she must change her blood. I'm afraid for her."

Thalassaemia is a genetic illness. It causes severe anaemia by reducing the body's ability to produce haemoglobin, a protein that carries oxygen.

Like other genetic illness, chances of being born with the illness increases in families with intermarriage.

It was once known as a disease of the Mediterranean, but it impacts people of ethnicities but some more than others. Cypriots, for instance, have a one in seven chance of being carriers of the illness.

According to random surveys, eight per cent of UAE nationals carry the illness, about double the international rate.

If untreated, the condition kills its sufferers early on. One staff member, a UAE national, described how in previous generations it was called the "yellowing," where seemingly-normal babies would turn yellow, inactive, and then die.

"We know better now. It was thalassaemia they were dying of."

Sufferers have to take medication and undergo regular blood transfusions. The transfusions can lead to iron accumulating in the body, which can be deadly.

Mohammad Salah, senior physician at the centre believes awareness about this genetic illness "is increasing".

In part, the media has done a good job, and the Marriage Fund, which demands blood tests from prospective brides and grooms before funds are given, is also working, he says.

But more awareness raising is needed, Salah says.

Others call for compulsory blood tests for the illness before marriage.

Even so, it is quite easy to check for thalassaemia. Salah says a blood test at any Department of Health and Medical Services (DOHMS) health centre will do. "It's a simple check. They'll send the blood to us."

For more queries, he said people could visit the centre.

Najah, 38, a UAE national, carries her feisty daughter, Fatima.

Najah said she wasn't aware of thalassaemia's seriousness before she became pregnant.

But then Fatima was diagnosed after she "began to turn a waxy yellow. She used to be extremely lazy and cry a lot."

Now, Najah says she will insist her children's future husbands be tested for the illness. "It should be compulsory."

Salah agrees, while emphasising treatments have broadened. "There's a lot of hope now.

"Options for thalassaemic patients is greater. Life spans are longer. Success of bone marrow transplants is now 95 per cent. Gene therapy is reaching final stages of research."

The doctor is also big on prevention through education, genetic screening by selecting through an IVF process ova and sperm free of thalassaemia and implanting those in affected patients who want to have children, and finally, in extreme cases, abortion.

Salah emphasises the positives, in part because he fears women will feel compelled to abort thalassaemic fetuses. "It's more complex than that and there should be carefully considered, individual diagnosis."

But the genetic disease does have its side-affects, from accumulated iron in thalassaemic patients undergoing blood infusions every three to four weeks.

Heart attacks, liver failure, retardation and dysfunctional hormones leading in part to delayed puberty can affect thalassemic patients.

There is a successful cure albeit long and expensive through bone marrow transplants, if a match can be made to the donor. The centre can also help women have babies who can also act as potential donors.

"There are two mothers here who we've helped get pregnant with healthy babies, who are also perfect donor matches for bone marrow transplants."

The stem cells from blood in the umbilical cord can also be used.

About six months ago, the government stopped funding its services for residents. Treatment costs rise to Dh3,000 a month. Salah said the Shaikh Mohammad Bin Rashid Charitable and Humanitarian Foundation stepped in to cover costs.

Sometimes, the centre looks like a permanent party, staff said. An acting social worker (the permanent social worker is in Italy, where her thalassemic son is undergoing a bone marrow transplant, soon to be available in the UAE) organises parties and visits.

"There's always a birthday cake, somebody gets good exam results, somebody marries it's always busy here," Salah says.

Last week, Modhesh (Dubai Summer Surprises mascot), gave presents to the kids.

"Some of the children look forward to coming here for their blood transfusions. They get to meet all their friends. There's a family attitude here."

The centre, Salah says, "is not just a place to get a blood transfusion and go."

It's a home with a family, he says, and a place where people are given hope and "community care". The recreation area is the place of choice to get blood transfusions.