Thalassemia in Sindh: Children suffer the most with the blood disease

January 16, 2011

Courtesy: tribune.com.pk

“I have seen broken marriages, deserted children and family feuds just because of this one disease,” says Dr Haroon Memon. He has been treating thalassemia patients for years in interior Sindh and is thoroughly aware of the devastation the disease has and can cause.

Working at the Thalassemia Care Centre in Badin, Dr Memon heads the facility where patients flock from eight districts of the province for a cure. The centre was built under the US Agency for International Development’s (USAID) District Grants Programme in 2005 and now functions with the help of Badin’s district government.

Victims of the inherited blood disorder — that results in severe blood shortage — suffer from an increase of iron in their bodies, diabetes and other major side effects. In the rural parts of the country where poverty, lack of resources and illiteracy result in the late or no diagnosis of the disorder, the social implications become more prominent than the physical ones.   Pakistan is counted among those less-developed countries where thalassemia patients are multiplying at a very fast rate. Although no proper research has been done in the country, experts say almost seven per 100 people are carriers, known as thalassemia minors. The carriers are normal themselves but have the potential to transmit it to the next generation causing thalassemia major.

When two carriers reproduce, there are 25 per cent chances that the child will be normal, 25 per cent chances that he or she will have thalassemia major and 50 per cent chances that the child will also be a carrier, says Dr Memon. Therefore a large proportion of thalassemia patients are children.

There is growing concern that thalassemia may become a very serious problem in the next 50 years – one that will burden the world’s blood bank supplies and the health system in general.

With the requirement of a bottle of blood almost every few days, the life of a thalassemia patient is like that of a car, one whose engine runs on fuel, explains Dr Memon. In such a scenario, prevention  not cure is what needs to be done to fight the genetically transmitted disease.

Beta thalassemia

In Sindh, most people suffer from beta thalassemia, which is one of the most familiar types. It involves decreased production of normal adult haemoglobin (Hb A), which is the predominant type of haemoglobin.

(All haemoglobin consists of two parts: heme and globin). The globin part of Hb A has four protein sections called polypeptide chains.

Two of these chains are identical and are designated as the alpha chains. The other two chains are also identical to one another but differ from the alpha chains and are termed the beta chains. In people with beta thalassemia, there is a reduced or absent production of beta globin chains.

Iron concentration

Dr Memon says that there are patients at his centre who, because of continuous blood transfusions, have developed a concentration of iron in different parts of the body and have to be treated for that. The iron concentrates in the heart, pancreas, liver, spleen and the endocrine glands, he adds.

Such children show signs of a ballooned stomach, broader forehead and jaw bones and prominently bad teeth.

Our job is to maintain the haemoglobin level of the thalassemia patients at 10.5 grams, to control the iron concentration in different parts of the body and to counsel the parents, says Dr Memon.

Law in the making

Concerned doctors are lobbying for a law to be passed for the identification of carriers in the province, informs Dr Saqib Ansari, a haematologist at the National Institute of Blood Diseases (NIBD). “In the year 2010, close to the World Thalassemia Day (May 8), the Sindh Assembly adopted a resolution to make the thalassemia test a pre-condition for nikah, but it could never be formed into a bill,” he says. Dr Ansari claims that this will successfully reduce the danger of thalassemia transmission without stigmatising women.

Meanwhile, Dr Ansari also says that a fatwa has been taken that allows a child with thalassemia major can be aborted before the 16th week.

The government needs to realise that apart from human suffering, supporting thalassemia patients is a huge financial cost, he says. “The disease needs to be controlled before the numbers shoot up.”


No test, no nikkah (Part 2)

June 20, 2010

Courtesy: tribune.com.pk

After my post “No test, no nikkah”, I have got some appreciation and at the same time some objections as well.

Here is the reply to those objections:

Several people asked me how they can ask their partners to get tested. “It doesn’t look good,” they said. A friend of mine said “The in-laws might start beating me Ayesha!” and I simply replied, “Just for once think of your generation – that beating won’t be anything then.”

Then, there are people who ask me who am I or the government to stop them marrying their love if both are Thalassemia minor.

Who said you can’t marry them?

No test, no nikkah doesn’t mean that you can’t marry your love if both of you are Thalassemia minor, it just means that for a precaution get yourself tested. If both are found minor then in every pregnancy you will have to get a test called “Prenatal diagnosis or prenatal screening”

What is prenatal test is for?

Just to make sure that your child does not have Thalassemia major (disease state), and God forbid, if the baby does have the disease then you can abort the baby within 120 days of the pregnancy.

Is it too difficult getting tested?

Then, for once imagine this: will you be able to bear your child pointing his/her finger at you and saying “Mom/Dad, you are responsible for my illness”? Will you be able to bear seeing your child getting pricked by needles, and all the other painful procedures that come with being a Thalasemmia patient? No…you won’t be…

It’s so difficult to get your partner tested?

Okay, don’t ask them for it, go and get YOURSELF tested, and if God forbid you get some irregular result then you can ask your partner to get tested. If the result is normal then it doesn’t matter whether or not your partner carries Thalassemia Minor gene, because it won’t do any harm. In that case just a kid or two will carry a minor gene, that’s it.

Is it still difficult?

You might think I must have pointed fingers at my parents too. Or maybe I feel bad for having Thalassemia and that is why I am writing such posts? Let me assure you that is not the case. I have seen two-year-old children at the transfusion center – even kids younger than 2.

I have seen how they get pricked, how they cry, how the nurses get their veins. If you see it yourself, you will cry… I bet you will.

And since I have been seeing this for so long, I really want to save our next generation. Wont you step ahead and help me? I don’t want another child to get pricked with needles because of lack of awareness, please?

Just one blood test can save your generation, what else do you want now? If you still can’t get it … then it’s simply useless to tell you anything…

And sorry people, but your objections are overruled and now you are left with no choice but to get yourself tested.

FAiTh’s dream : Thalassemia free Pakistan!


No test, no nikkah!

June 20, 2010

Courtesy: tribune.com.pk

Almost every third girl gets married to a guy she doesn’t know, has never met or never talked to before. The guy’s family just saw the girl somewhere, they sent the proposal and if the girl’s family didn’t find anything objectionable then everything is final – the date is fixed, venue decided…and they get married to each other.

Now, the happily married couple is expecting a new family member, and when the new family member is born, they get to know that the baby is having a disease called Thalassemia. They go blank. They run to doctors. They try to do what ever they can do. In the end, they get to know that both of them were Thalassemia Minor which is why their baby is Thalassemia Major.

Minor + Minor = ?
25% chances are of Thalassemia Major
50% chances are of Thalassemia Minor
25% chances are of Normal

Sadness takes the place of happiness soon…. And the baby becomes used to hospital visits, transfusions, needles and medicines.

Now the question is: Where did it all begin? What was the mistake they made?

Till a few years ago, people used to ask parents of Thalassemia major that if they were cousins? Did they get married within the family? Yes, Thalassemia genes do get inherited, but it is not only in cousin or interfamily marriages, it can even happen when a guy and girl do not know each other and get married and when they never get tested…

Asking the couple to get the test done before marriage is like asking one to commit the biggest SIN!

But why can’t you think, just for once, for your next generation? Why don’t you see the bigger picture?

When are we going to stop being so narrow-minded?

A few months ago there was a comment on my blog which said:

“No Test, No Nikkah!”

I feel the government should pass a bill about this issue soon. There are many countries that have already started this, why can’t we? And when will we?

We all know our government so why don’t we start something on our own too?

What you can do as an individual?
* You can get yourself and your partner tested before marriage
* Ask your friends to do the same
* If someone you knowis getting married ask them if they have gone for the test
* Spread awareness about this to family, friends, colleagues, neighbours and everywhere…TO SAVE THE NEXT GENERATION!

The test is called HB electrophoresis and it is not very expensive…

So, when are you going to get yourself tested?


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