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A Sea of Pain

Sickle cell anaemia is recognised as the most serious of all genetic disorders in the Kingdom. Is Bahrain doing enough to promote understanding and management of this condition?

Picture this:
Thirteen year old Ahmed has handled pain for as long as he can remember. Forbidden from the playground since he was a toddler, the boy was equally stoical when he was forced to drop out of school owing to his fragile health. For all his poise, Ahmed eventually broke down after his father was killed in a road accident. He had rushed out of the house to purchase medicines to address his son’s crisis.

And this:
At 26, Maryam lives with her step-mother, but not because she isn’t married. Battling sickle cell anaemia since infancy, she tried to start a family and suffered a miscarriage. She was then deserted by her husband in view of her failing health. Maryam has now developed acute heart complications. Friends have to rush her to Salmaniya Medical Complex (SMC) almost every other day as she struggles for breath and battles severe
chest pain.

In Bahrain:
As countries worldwide observe World Sickle Cell day on June 19 for the third year, it’s perhaps time for a reality check on this condition and the scenario in Bahrain.

Sickle cell anaemia (SCA) is caused by an abnormal kind of haemoglobin, the Haemoglobin S, which turns the normal disk-shaped red blood cells (RBCs) into a deformed crescent shape. Following the shape change, RBCs become fragile and deliver less oxygen to the tissues. They can get blocked easily in the blood vessels, interrupting normal blood flow and triggering a severe attack known as “sickle cell crisis”, which involves intense body pain. Over time, sufferers become more susceptible to infections as the damaged cells eventually clog the spleen, followed by the liver. Sufferers commonly die of stroke or multiple organ failure.

All in the family
Bahrain is home to around 18,000 people with SCA, while approximately 65,000 among the population carry the trait (gene from only one parent).

Former molecular researcher, Hussain Al Jamry, has traced the prevalence of sickle cell disease in the region as far back as BC 1800. In Bahrain, there’s evidence to suggest that the condition has been around since BC 500, he says.

The disease is passed down through the generations, primarily through marriages within the family. “If both parents are carriers of the gene, the offspring has a 25 per cent chance of developing this condition. In the event that only one parent carries this gene, the offspring will be a carrier but won’t be affected by it,” says Jamry, whose research paper was published in 1999 in American Journal of Haematology.

While Bahrain is close to wiping out incidences of sickle cell disease in newborns, challenges abound in the management of this condition in existing patients. The average life expectancy of males with SCA in Bahrain is 42, compared to the average age of 62 in the US.

“In the absence of any real cure, except doses of morphine to relieve the pain, patients here are commonly perceived by health professionals as drug addicts,” observes Zakareya Al Kadhem, chairman of Bahrain Society for Sickle Cell Disease Patient Care. “The Kingdom is yet to roll out adequate health infrastructure for these people,” he says.

According to Zakareya, there were 31 sickle cell deaths in Bahrain in 2011, while 35 sufferers succumbed to the condition in 2010. This year, there have been seven deaths so far. A proposed BD 2.5 million haematology centre at SMC is expected to be launched next year following a two year delay.

Treatment options
In Bahrain, doctors commonly administer the drug hydroxyurea to patients suffering crisis attacks. “The drug reduces the severity of sickle cell disease by stimulating production of foetal haemoglobin, which can block the sickling action of RBCs,” says consultant paediatrician and haematologist at SMC, Dr Hussain Al Muharraq. Hydroxyurea, however, is not a cure and can only make the condition milder. Regular blood transfusions are also critical in managing severe episodes of the condition.

While there is still no cure for sickle cell disease, experimental bone marrow or stem cell transplants in countries abroad have proven successful, especially for younger patients.

“We have referred several cases overseas for transplants, in the event that the patient has found a matching donor,” says Dr Muharraq.

Watch out for addiction
With only painkillers with which to battle their condition, sufferers are in a real danger of slipping into addiction. “It all depends on the person’s threshold of pain. Many will need daily doses of morphine to keep away the pain. Sometimes, one may take an overdose as a precaution against an oncoming attack,” notes Zakareya.

Sufferers may also resort to drugs to seek an escape from the reality of their condition. “Family members should keep an eye out for sufferers who start spending more money than usual, or those seen seeking morphine at multiple health centres in a single day and those buying medicines other than the painkillers prescribed by a physician,” he says.

What can help?
In the long term, sickle cell sufferers need to take the utmost care of themselves, to minimise attacks and for an improved quality of life. People need to keep a tab on their haemoglobin count, with blood tests every month and seek transfusions when it drops. Those who smoke should kick the habit at the earliest. A relaxed life with a good work atmosphere is desirable, as stress in any form can trigger a crisis episode.
 
“Sufferers should avoid heavy exertion and take precautions against extreme weather conditions, whether heat or cold. The focus should be on good nutrition to keep the body free from infections,” says Dr Muharraq.

Experts believe research could be the key to dealing with crisis episodes, especially on part of health personnel. “What we need is a centre for sickle cell patients in Bahrain, with specialised doctors and trained medical staff handling them. Every patient has the same crisis periodically and if we were to maintain follow up files for every sufferer and study their pattern, we’d be able to predict when a person will have the next attack. That way, doctors at SMC and other health centres can keep space reserved for a certain number of patients, when they need them,” suggests Jamry.

Make a difference
Sufferers can themselves monitor their diet and lifestyle, to understand their individual triggers.

For his part, Zakareya retired from his teaching job at Bahrain Training Institute to raise awareness about this condition and to demonstrate, through his own example, that “it is possible to live with SCA and not let the pain destroy you or your family.”

In many ways, the disease has become a blessing for him. “As a sickle cell patient, I’m aware that I have only half the lifespan of an ordinary person. I have to accomplish something before I leave. Every day is important and there’s no time to be wasted,” he says.

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