Abu Dhabi, UAEWednesday 16 October 2019

UAE records new blood disorder cases after hopes of eradicating condition

Medics at government-run Thalassaemia Centre in Dubai say more vigilance needed after 15 new cases treated

Alya Ali with her son Abdulrahman at her home in Abu Dhabi. Reem Mohammed / The National
Alya Ali with her son Abdulrahman at her home in Abu Dhabi. Reem Mohammed / The National

Doctors recently recorded new cases of a serious blood disorder after hopes its spread had been halted.

Medics at the government-run Thalassaemia Centre in Dubai dealt with new cases this year and said that more vigilance and awareness is needed.

There were 15 new cases reported last year after more than two years when virtually no cases were reported. This led medics to believe they had turned a corner in the fight against the condition.

Thalassaemia is a group of inherited conditions that affect haemoglobin. Those with the condition produce either no or too little haemoglobin, which is used by red blood cells to carry oxygen around the body. This makes the patient anaemic, causing tiredness and shortness of breath.

It is not shameful to have thalassaemia. The problem is when they don’t do the test and ignore our advice not to marry if they are both found to be carriers

Dr Essam Dohair, Thalassaemia Centre

“It is a disaster when you have new cases, even if it is a single case,” said Dr Essam Dohair, senior haematologist at the Dubai Health Authority-run centre.

“It means we have failed,” he said.

There are about 2,000 people with thalassaemia in the UAE and nearly half of them are Emirati. The centre in Dubai has about 850 patients and the most serious 450 must receive regular blood transfusions.

A larger number – at least 8.5 per cent of Emiratis, according to official estimates – have the less-harmful thalassaemia beta. These people carry the gene without any effect on their own health, but can pass it on to their children, particularly if the mother and father are both carriers.

Dr Dohair blames the new cases on the reduction of public awareness campaigns, which ran in the past.

“We need to focus again on thalassaemia. We need to invest in education rather than treatment,” he said. “Voluntarily, no one is doing [the test].

“When we started our awareness programmes 30 years ago, people started doing the test. Once we reduced our awareness programmes, that is when we started noticing, for the first time, new thalassaemia cases. The number of new cases has started going up. We’ve seen newborn thalassaemia cases in the past year – it’s alarmed us.”

Dr Salem El Shawarby, an IVF specialist in Abu Dhabi. Victor Besa / The National
Dr Salem El Shawarby urges couples to get tested as early as possible. Victor Besa / The National

The inherited condition is most common in people of Mediterranean, South Asian, South-East Asian and Middle Eastern origin.

Consanguineous marriages – when the wife and husband are cousins, for example – are relatively common in the Arab world. If both parents are carriers then their child could develop the most serious type of the condition, which can be fatal.

Dr Dohair said past campaigns were effective at identifying carriers who did not know they had the condition, thereby limiting its spread.

In 2006, a law was introduced making it mandatory for Emirati couples to undergo genetic screening before marrying to determine if they are at high risk. But it is ultimately their decision whether they want to go through with the marriage or not.

“It is not shameful to have thalassaemia. I have patients who are married and have families and lead a normal life,” Dr Dohair said. “The problem is when they don’t do the test and ignore our advice not to marry if they are both found to be carriers of the gene.”

He said tests should be done “much earlier – as soon as a girl reaches maturity”.

He said all residents, not only Emiratis, should be tested to prevent the condition being spread. If not, the fear is numbers will continue to grow.

“We are always at maximum capacity because our patients are always being replaced with new ones,” Dr Dohair said.

Dr Salem El Shawarby, IVF medical director at Fakih Fertility Centre Abu Dhabi, has hundreds of couples among his patients.

He urges those at high risk to undergo preimplantation genetic diagnosis, a procedure used to help identify genetic defects in embryos. Costs range between Dh8,000 and Dh18,000, but are covered by the Thiqa insurance card given to Emiratis.

“There is a social awareness that in general it is best to avoid getting married to a close relative, but here in the Middle East it is part of the inherent culture,” he said.

“So we need to raise public awareness of the availability of these tests, as well as the message to avoid consanguineous marriage if possible and for couples to seek medical assistance from IVF centres before planning to have children.”

My whole life is hospitals and living in worry that they might die at any moment

Alya Ali

Dr Arun Sharma, a neurologist and medical director of Emirates Hospital Clinic, sees many children of consanguineous parents who are born with genetic disorders.

“I see it quite often – mental disorders, autism, ADHD and epilepsy are all quite common in consanguineous marriages,” he said.

Researchers have made progress in developing better treatments using gene therapy. Last year, scientists published the results of a trial in which 22 patients had blood stem cells extracted, treated and reintroduced to stimulate red blood cell production.

Fifteen patients were able to stop transfusions altogether while others needed fewer, the BBC reported. Lead researcher Alexis Thompson, professor of paediatrics at Northwestern University in the US, said the results were exciting and “very promising”.

And, as The National reported in May, Dubai teenager Hussein Al Blooshi, 17, was cured after a successful haploidentical bone-marrow transplant in 2016 at Cleveland Clinic in Ohio.

Other patients are less fortunate and face a life of long-term care.

Alya Ali with her son Abdulrahman, 18, at her home in Abu Dhabi. Reem Mohammed / The National
Alya with her son Abdulrahman, 18, at her home in Abu Dhabi. Reem Mohammed / The National

Emirati Alya Ali’s two sons, Abdulrahman, 18, and Mansour, 21, have a severe form of thalassaemia. Both have frequent seizures, need feeding tubes, and cannot speak, walk or perform the most basic tasks. She married before genetic screening was made mandatory.

“I was 19 when I married and unaware of the complications,” she said. “My whole life is hospitals and living in worry that they might die at any moment.”

She urged couples to have the checks, to consider the risks if both parents are carriers and to listen to doctors.

“I wouldn’t wish this life on anyone,” she said.

Updated: September 16, 2019 06:23 PM

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