Thursday, February 12, 2009

Status of Thalassemia in the Maldives


Thalassemia, at present, is one of the most challenging hematological disorders. Patients with ├čthalassemia major need regular blood transfusions in order to live and the resulting iron overload requires chelation therapy. The problems facing them and their parents are immense, challenging them physically, emotionally and socially.

In the Maldives thalassemia affects about 0.16% of the population, and is found throughout the country, with the highest rate found in Kaafu atoll, most likely because an address in Male╩╝ is given at the time of registration at the National Thalassemia Center (NTC). Apart from Kaafu atoll, the highest rates are found in Noonu, HDh and Laamu atolls.

To date a total of 670 cases have been registered at NTC. The number of new cases registered has not declined over the last five years, with about 28 new cases on average being registered over the last 7 years. Last year saw an increase to 39 in fact.

Considering that the country has two centers dedicated to thalassemia (Society for Health Education and NTC), and the government spends roughly $5000 per year per child for the treatment of a child with thalassemia above 12 years, this current trend raises questions whether these centers are doing enough for the prevention of thalassemia.

The current preventive program focuses on discouraging the marriage of carriers to one another, thus increasing the number of carriers throughout the country. And in Maldives where the social circles are small, the chance of intermarriage of careers is high. And their career status will not be a deterrence to getting married or having children. They will continue to take the risk and hope for that 75% chance of having a normal baby.

The approach has not helped in reducing the number of new cases as most of the children with thalassemia are being born sadly, to those who know their career status at the time of marriage, and to those who already have a child with thalassemia. This is an important observation that needs to be addressed in the prevention program as just the mere knowledge of the risk has not prevented couples from having children.

The goal of the program should be to lessen the burden of thalassemia in at-risk families, accomplished not only by providing information about the risk but also providing options for dealing with it by helping at-risk couples obtain prenatal diagnosis and selective abortion, or by helping them to cope with the birth of an affected child.

The current management protocol too needs revision to include oral iron chelators in the treatment. At present only a select few are being provided oral iron chelators at NTC as the treatment requires regular monitoring for side effects. Recommendations have already been made to the Ministry of Health and Family, in view of the considerable improvement in the serum ferritin levels in patients on combination therapy, to start the treatment at the regional levels, but its implementation is yet to materialize.

Adherence to the current management needs to be enforced more rigidly as reports of mismanagement are quite common at the regional and island level. Most doctors employed at the island level are not well versed with the disease and have different opinions regarding when to transfuse, how much to transfuse and how to give chelation therapy.

And sadly the psychosocial impact of the disease is completely ignored in the management. Compliance levels are lowest at the adolescent age group and it is essential that they get the proper psychosocial support including a clear understanding of the disease. In a survey conducted amongst adolescents at NTC, 90% did not believe that they had complications despite not complying with treatment and having their serum ferritin levels well above the target range. The extent of complications has yet to be documented.

A total of 140 of those registered have died so far, and from 1997 to 2007 each year about 8 children have died at the average age of 8 years. This young average age at death should be a matter of concern in a country where all aspects of treatment are free of cost and where treatment is readily available. No other single disease enjoys this level of commitment from the government and the statistics should be in favor of this support.

As it is the status of thalassemia in Maldives is not encouraging and revisions need to be made in both the preventive and treatment aspects. Genetic screening should be accompanied by improved counseling. Efforts should be directed towards prenatal diagnosis programs. Combination therapy for iron chelation needs to be started across the country at regional levels and psychosocial support for both the affected child and family should be given. A comprehensive program encompassing public education, screening for carriers, genetic counseling and prenatal diagnosis has markedly reduced the incidence of B-thalassemia major in several countries such as Cyprus and replication of such a program in the Maldives is necessary to lessen the burden of this preventable disease.

[Article contributed by Dr. Faisal Saeed]

22 comments:

Abdullah Waheed's Blog said...

Some people do things because they have something to do; others do things because they have to do something. It was the latter case with Society for Health Education, when it had to choose something to do. It was unfortunate for Maldives that it chose thalassemia screening. The increasing incidence of thalassemia we see today is the long term effect of that misguided strategy.

Anonymous said...

From the figures you provide more than $3 million is spent each year on treating thalassemia. We have to do something to decrease thalassemia inidence urgently. if abortion works it needs to be started.

Mustafa.I said...

I saw this trend in 2002, which thalassemia major cases were declining, while I was diagnosing thalassemia in this center, I wrote a small article on thalassemia week supplement in Baqai Medical University, where i wrote thalassemia in Maldives is a success story.

But before I left in 2004 I have noticed this is not working in Maldives, I personally tried to change the protocols, but I didnt get the support.

Lot of obstacles because of bureaucracy and red tape, every small thing has to go to MOH and so many committees and meetings, nothing works.

As Faisal said we need to revise this and need urgent action rather than talk.

Anonymous said...

Effective preventive approaches to genetic diseases have been demonstrated in countries where an inherited condition is common and carriers of abnormal genes can be reliably identified. For example, in Cyprus, Greece and Italy, screening for thalassaemia is standard practice and national audit data are available; most at-risk couples are identified in time to be offered early diagnosis in the first pregnancy, of whom the majority use this service and produce healthy offspring. Screening programmes need to be supported by public education and regulatory structures to empower individuals to make informed decisions and to ensure that people are protected against discrimination as a result of their test results." paragraph from WHO report"

Anonymous said...

Children should be tested to determine if they are thal carriers. If they are it should be a legal requirement to abort the baby. When Islamic affairs was under Sheikh Rasheed and Maumoon it was declared abortion before a certain period was not un-Islamic and not murder.

Anonymous said...

with proper managment most thalassemia kids aught to grow up into adulthood. So in the Maldives when so much public money is spent for this one disease, why are so many dying?

Anonymous said...

OK. may be SHE took up the wrong method. But they worked in good intention. So give them some credit.

Anonymous said...

According Sheikh Al-Qaradawi, euthanasia is forbidden in Islam (http://www.islamonline.net/servlet/Satellite?pagename=IslamOnline-English-Ask_Scholar/FatwaE/FatwaE&cid=1119503544774). So prenatal diagnosis and abortion are haraam.

Anonymous said...

first clot.then flesh .bones etc. life is put several weeks later. you abort before the unborn becomes human with life.We should be more believing in forward thinking sheks like our own Rasheed etc . Not this Qaradawi chap.

Anonymous said...

We dont need to screen for thalassemia, human involvment in any biodiversity there will be consequences. Thats what is happening thalassemia in Maldives. Naturally for a reason may be thalassemia existed in Maldives population, scientific evidence suggests thalassemics has natural immunity against malaria. Maldives been in a region where malaria is highly prevalent, it may be a good thing to survive to have thalassemia.

The current situation of thalassemia is a political issue, even last campaign some people said if they vote for their party, they can get help to go for a bone marrow transplantation.

Anonymous said...

if we really need a change to thalasemics in the country, we need to change the people involved in the process. there are people like Malaria Gafoor in the circle who become master of thalasemia by chlorinating drinking water wells years back when he was young. we need people like him to be out and replace with educated people in the field. Guys like gafoor they are day light silent robbers born in the Maumoon era. This is 21st century, we need people of high caliber, not silent robbers.

Anonymous said...

Test and abort should be the policy to eradicate thalssemia. This policy has worked in many countries.

Hilath said...

The former government lacked any vision and proper long-term planning to tackle any important issues facing the country. This is just one of the many results of neglect our country has inherited from the previous government.

Anonymous said...

Thalassemia is a disease invented by Gayoom's wife.because she was bored at home. Now that SHE is no longer so powerful the disease will find a natural death.

hussainfaiz said...

i believe SHE is no longer capable of doing the job..They failed to decrease the number of major cases over the past years. Government should not depend on them as a part of this programme. I call on MOH to promote awareness at a large scale using all the medias available.
I worked at NTC until late 2007, but as Mr.Mustafa has mentioned there are no authorities for the admin from top to bottom to improve the case.
If you have analyzed the no. of major cases due to wrong diagnostic report from SHE you would have come across many cases. This is due to the mishandling and unprofessional staff who stands at the very important point of the organisation. The report issuing is the main concern here..The technique is not well and so fragile that anytime you may receive a wrong result..
SHE has worked more prominently for their beneficiaries rather than the community. The funds they get from MOH and other International corporations can be utilised to improve the community more efficiently rather than just spending a double work and money.
NTC and SHE both are doing the same work for the same community. SHE must work under MOH not under the former first lady. The out come will be much brighter and better ..

Anonymous said...

I dont think, we need to blame SHE for any of these thing, SHE is an NGO, they chose create thalassemia awareness in Maldives. They have got substantial amount of data.

But where is MOH and DPH who has to be responsible on public health issues. NTC is technically the regulatory body to look after thalassemics in the country, other than just giving regular transfusion , what else they have done past 15 years.

In my opinion, thalassemia issue in Maldives is not matter of how its diagnosed or treated, its matter of how the message is passed and there is lack of communication gap to pass this in to society. Currently is been like a fear among community, where 20% of the country carries this genetic genes. First thing we need to do is abolish this card system, red card and white card to label people in their genetics. Of course we can screen people , but we can issue reports like any other laboratory investigation, with privacy and respect.

Anonymous said...

why should SHE work under MOH? SHE is a non government organisation registerd under Home Affairs. I guess in that case all NGO's work on health issues should be under MOH. and Journey & SWAD should be under NNCB since they are working on drugs.Moreover for your information if you really look SHE is not a property of the Govt. Its an independent NGO providing free services to the community. people need to look into works of others before commenting. SHE works towards awareness and people know Thalassaemia because of SHE. why did you not look at what SHE has ever done in treatment by providing Desferol pumps, Filters and BMT.

mustho said...

I disagree, with some people who says errors in the diagnosis from SHE or some where else, in any experiment the possibility of errors are there.

The notion that SHE is responsible for thalassemia in Maldives is not the right information. SHE is an NGO they are doing what they have chosen to be right for health awareness including thalassemia. In my view they are doing commendable job.

As Dr.Faisal has expressed in his data, if the current system does not work to reduce registered transfusion dependent patients. In my opinion MOH and NTC has to take the responsibility and they need to find a solution for this.

According to published data 18.1% carries heterozygous for beta thalassemia genes in Maldives. If this is a public health issue ,I think its a responsibility of health policy makers in the government.

Zawid Naseem said...

Oh my God!! Please stop this blame game. Let me clarify something. The blame for having this high percentage of Thalassemics in Maldives can not be attributed to any organization. It is an established Principle of Genetics called Hardy-Weinberg Principle, which says that in an inbreeding population the frequency of any gene will remain constant. So let’s talk logically. The only way of reducing this gene’s frequency is to go for selective breeding. That means identify the population as carriers and non-carriers (which have been done to a great extent already) and prevent marriages between normal and carrier. Allow only carriers to marry each other and go for in vitro fertilization (IVF) to implanted selected embryo's which are free from this gene only. This will not involve abortion so no haraam act. Mullahs will be happy and so will be the parents. Invest money in this. This could one day lead us to a Thalassemia free Maldives

Zawid Naseem said...

Oh my God!! Please stop this blame game. Let me clarify something. The blame for having this high percentage of Thalassemics in Maldives can not be attributed to any organization. It is an established Principle of Genetics called Hardy-Weinberg Principle, which says that in an inbreeding population the frequency of any gene will remain constant. So let’s talk logically. The only way of reducing this gene’s frequency is to go for selective breeding. That means identify the population as carriers and non-carriers (which have been done to a great extent already) and prevent marriages between normal and carrier. Allow only carriers to marry each other and go for in vitro fertilization (IVF) to implanted selected embryo's which are free from this gene only. This will not involve abortion so no haraam act. Mullahs will be happy and so will be the parents. Invest money in this. This could one day lead us to a Thalassemia free Maldives

Anonymous said...

The idiot who wrote this can't even spell CARRIER!

For his sake,

CARRIER
Genetics.
a. an individual possessing an unexpressed, recessive trait.
b. the bearer of a defective gene.

CAREER
an occupation or profession, esp. one requiring special training, followed as one's lifework. Eg; He sought a career as a lawyer.


It is not an easy thing trying to find an eperts in a subject. As far as I am concerned, someone who do not possess the basic knowledge (proof to our eyes: inability to spell the basic term "carrier") of genetics or the disease in particular, is far from being an expert. Watch out for any wrong information that may be passed by such "experts"!

Anonymous said...

Wow.... Before I thought Maldives had kind,brave educated people..... But after Reading these Comments I must Say Maldivians just went to school while they already had they mouth to spread rumors and to blame others and politicians... Why Should we legal Abortions,TO say If we had this Thalassemia HBA2 test Done(IF). Why to Blame Government.... After MDP Government falls all will Say that Was in Mohamed Nasheed era... Blame Our Self First..Its Our Country After All... IS THERE ANY ONE HERE DONATE ANYTHING TO THALASSEMIA CARRIERS...