Genetic disease clinic asks for help

DUBAI // The UAE's only centre specialising in treating thalassaemia patients is calling for more and better screening to reduce the incidence of the genetic disease. Staff at the Dubai Thalassaemia Centre, which operates at 95 to 100 per cent of capacity, also want to create a satellite network of smaller units to help handle some of the more than 800 patients it sees each year.

Thalassaemia is an inherited disorder in which the body produces less haemoglobin than normal. Haemoglobin allows the red blood cells to carry oxygen around the body. If the levels are low it can cause anaemia, which leads to tiredness and fatigue. According to Dr Khawla Belhoul, director of the centre, from four to eight per cent of the UAE population are carriers of the thalassaemia gene. If two carriers have a child there is a 25 per cent chance the child will have thalassaemia, and a 50 per cent chance the child will be a carrier.

"The major thing about thalassaemia is prevention," she said. "It is the most common [genetic disease] but the easiest to prevent. Prevention is awareness and tests. It is a national problem." The centre, which opened in 1989 and is in the grounds of Al Wasl Hospital, is Government-funded and treats its patients for free. It does, however, raise additional money from private sources. Donations pay for a sanctuary for youngsters and adults. It boasts PlayStation consoles, computers, televisions, newspapers and toys - in other words, something for everyone.

Volunteers spend time with the adults and children making art and craft pieces that are displayed in the centre, and schools send their students to learn about thalassaemia and to meet some of the patients. It can cost up to Dh100,000 (US$27,000) to treat one thalassaemia patient for a year, according to Dr Essam Dohair, the centre's outreach co-ordinator and a genetics expert. "Many patients could not afford private treatment," he said. "It is usually a minimum of Dh2,500 for each visit."

The centre treats 428 patients who require regular blood transfusions and another 300 who require monitoring and regular check-ups. More than 95 per cent of the patients come from the UAE; the others travel from other Gulf countries or further afield. Dr Belhoul said she would like to develop satellite units and outpatient centres across the UAE to provide more assistance. "What I have already suggested is to create a satellite that reports back to here, and then we can transfer the know-how to these satellites," she said. "Then in the future these may grow to become equivalents to this centre."

A high proportion of the expatriate population in the UAE comes from high-risk areas such as South Asia, the Mediterranean and the Middle East, Dr Belhoul said. Patients with thalassaemia can lead relatively normal lives with the right treatment and proper management of the disease. It is an autosomal recessive blood disorder, which means patients need a blood transfusion every three to four weeks throughout their life.

A patient receives about three units of blood for each transfusion and has to take daily medication. If the disease is not managed it can cause bone deformities, an increased risk of infections, slowed growth, and liver and heart problems. It also can affect hormone production. A bone-marrow transplant with an exact match is the only cure, and a costly one. The UAE introduced mandatory premarital screening in 2006. The law does not stipulate how soon couples should be screened before their wedding.

Dr Belhoul said the number of new cases each year among the local population has been steadily declining as a result of the screenings. Although premarital screenings are helping, she said, the process is still not perfect. "People do not get premarital screening early; that's what I don't like about it. It is not always easy to change your mind [about marriage]. After committing, it is difficult."

Despite the common assumption that the disease is caused by familial marriages, this is not strictly true, Dr Belhoul said, adding that the disease can be found in a band of countries from Spain to south Asia, and marriage between people from anywhere in that region could bring a thalassaemia risk. A two-stage screening process would work much better, she said. By introducing testing much earlier people could be counselled about the choices they make.

An important part of the centre's work is providing non-medical support such as career-placement and psychological counselling. Some of the younger patients at the centre said they often experience feelings of loneliness and isolation, and also struggle to fit in. Linette Saldanha, a nurse, said that strong bonds are built between the patients and the staff, as many of them have been getting treatment at the centre from infancy.

"These are not patients for us," she said. "We work like a family. In many ways we go beyond our limitations." @Email:kshaheen@thenational.ae munderwood@thenational.ae

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