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.”

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Before you say ‘qubool hai’

August 23, 2010

Courtesy: tribune.com.pk

When five-year-old Affifa goes to school she carries more than the burden of books on her shoulders. “My daughter was diagnosed with Thalassaemia, a blood disorder, at the age of three,” says Wajahat Ayaz, who works as an engineer at a leading power company in Karachi.

Affifa’s problems are compounded not only by the fact that her body generates an abnormal form of haemoglobin cells, but also by her rare blood group, known as the Bombay blood type. According to Dr Saqib H Ansari, chief of the Thalassaemia programme at the National Institute of Blood Diseases, there are only seven known donors in the country who share this blood group. “Patients like Affifa survive on blood transfusions, but it becomes a huge problem when one has a rare blood group because one can’t find matching donors,” Ansari says.

However, the little girl is lucky for now. Nuzhat, a woman of 40, is providing crucial life support for Affifa. “I will donate my blood to her as long as I live,” says Nuzhat. But what will happen after that?

Ayaz, who earns around Rs30,000 a month and spends close to Rs10,000 of that on monthly treatments for his daughter, says he can only pray that a miracle like Nuzhat will be around. However, that’s not the issue which torments him each day.

“If only I knew that we, the parents, would be the cause of her illness,” laments Ayaz, without saying anything about what he would have done had he known this fact beforehand.

Thalassaemia is a genetic blood disorder which a child inherits from their parents. “If both parents are Thalassaemia minor — that is, they carry the disease but it is not active in their system — there is a 25 per cent chance that their child will be a Thalassaemia major — an active patient –a 50 per cent chance that she will be a Thalassaemia minor and a 25 per cent chance that everything will be normal,” says Ayesha Mehmood, the spokesperson for the Fight Against Thalassaemia (FAiTh). Also, if a Thalassaemia minor’s partner is normal, their children, in all likelihood, will be born free of the disorder.

initiatives

Ayaz supports recent initiatives taken in certain provincial assemblies regarding the nikahnama law that advocates testing for blood disorders like Thalassaemia, Hepatitis C and HIV/AIDS in couples before they marry. He suggests the government should make it mandatory to mention blood types on national identity cards so that potential donors can be identified.

But so far the Khyber Pakhtunkhwa provincial assembly is the only legislative body which has passed a bill making it mandatory for couples to carry out Thalassaemia and Hepatitis C tests before the marriage takes place. The law does not ban couples from getting married if they find out they are both, for example, Thalassaemia minor carriers, but it gives them the advantage of knowing what they’re getting into. Meanwhile, the Sindh Assembly has to date only passed a resolution urging the federal government to consider making such tests compulsory.

On July 08, 2010, the Punjab government’s Local Government and Community Development Department proposed eight amendments to the nikahnama law; these included complete medical check-up reports prior to marriage. However, the amendments were criticised because of the caveat that the nikahnama document must also be signed by the parents of the consenting adults and were consequently withdrawn.

Member of the National Assembly Sherry Rehman has strongly criticised the Punjab government’s move. Rehman fears the measure might increase the number of forced marriages in the country. “Having parents sign the nikahnama would have meant the return of the wali system for women. The Lahore High Court has a ruling against it,” she says. She added, however, that she was all for testing couples for disorders before marriage and would strongly advocate such a bill.

Meanwhile, Masood Alam, who is about to get married this year, voices another concern. He says that even if blood testing before marriage becomes federal law, it would be hard to convince families that it is a healthy measure. “I and my fiancée may agree, but our families might not want us to get tested because of social pressure and superstition,” Alam says.

Even Nuzhat, Affifa’s donor, says that girls in Pakistan already face a lot of problems getting married and the proposed changes to the nikahnama law may become an added hurdle.

Dr Ansari proposes a solution. “We can follow the Iran model, where the man is asked to get tested first,” he says, adding that the woman only need be tested if the man is found to be suffering from, for example, Thalassaemia minor.

Also, families can be convinced about the benefits of testing with a little persuasion, says Shahzad Shah, who married in 2009 and got himself and his fiancée tested before marriage. “After we told our families about the advantages, they themselves took us to get our blood tests done,” Shah says. He adds that today he is a proud father and his daughter has no genetic blood disorder.

Religious scholars say that while conducting blood tests is not a necessity before marriage, according to Shariah law there is no harm in doing so. Darul Uloom Karachi’s Mufti Asghar Rabbani and Jafferia Alliance’s Maulana Sheikh Hasan Salahuddin say tests can help identify problems that an unborn child might face, because in the end, the child has to suffer the consequences.


Thalassemia: Learn something, will you?

July 11, 2010

Courtesy: tribune.com.pk

For over four years, Fight Against Thalassemia (FAiTh) has been trying to convey a simple message across to the government: to pass and implement a Pre-Marital Mandatory Tests Bill in the country. This is a simple request for a simple bill and a simple solution to highly complex problems that our society faces today.

Even though right now there are no government-based Thalassemia treatment centers, this situation will improve soon as there will soon be as many as seven Thalassemia treatment centers run by the Government of Pakistan. The exact date of when these centers will be setup however, only two G’s know: God, and Government.

FAiTh has attempted to utilize a myriad of media: from print to web-based, from television to spreading awareness on its own through the help of media owners. While FAiTh was busy with awareness campaigns, the Khyber-Pakhtunkhwa assembly sent us a shocking, yet pleasant surprise: they passed the Pre-Marital Mandatory Tests Bill.

This was a cause for celebration! However, this overwhelming joy was quickly swept over by another question: When a conservative province like the Khyber-Pakhtunkhwa is progressive enough to pass the bill, why are the other provinces so reluctant?

The North West is a place where the men do not want anyone to see their female counter-parts. I salute them for passing the bill and giving other provinces what we call, “ghairat ka dose.”

And, just after a few short days, the Sindh Assembly gathered all of its courage, and passed the resolution as well! What a brilliant milestone!

This is all great! However, what the Punjab Assembly did comes as a big slap across the metaphorical faces of all the other Provincial Assemblies. I understand that I am going to be opposed with many objections after this post. Even as I wrote this, I came up with several queries myself, such as it will be an invasion of privacy or that more problems will be caused for girls and aged women who are still awaiting their match.

I am supporting this bill because of the medical check-up made necessary. This can surely help to put a full stop to the birth of 5,000 Thalassemic children every year. It is not the children, that shouldn’t be born. It is Thalassemia. I have mentioned in my earlier posts as well, that I am representing Thalassemia patients of Pakistan. I am their ambassador, and I speak for them when I assert that they will be happy with this new law.

Now, the National Assembly needs to get some ‘ghairat‘ and do their part: implement the law countrywide!


Bill to control thalassemia presented to parliament

June 13, 2010

Courtesy: Minivannews.com

A bill to control the recessive disease ‘thalasemia ‘ has been presented to the parliament.

The bill was presented to parliament by Dhivehi Rayyithunge Party (DRP) MP Visaam Ali.

Thalassemia is a recessive blood disease that can cause anemia, and the Maldives has the highest incidence of it in the world with 18 percent of the population thought to be carriers.

As a result, a large number number of families suffer from the consequences of the disease

DRP MP Ahmed Nihan said there were two main purposes of the bill.

”One is that the Maldives, relative to its small population, has a large number of thalassemia patients,” Nihan said. ”The the current government has been careless with thalassemia patients, so we need a law for this.”

Nihan said that the increasing number of thalassmia patients in the Maldives was “a serious social issue, which should be prevented for the future of the country.”

”The disease was first discovered in 1921, and Maldivians became aware of it after Madam Nasreena [wife of former president Maumoon Abdul Gayum] formed the ‘Society for Health Education’ and conducted awareness programs,” Nihan explained.

For a long time people were unaware of the disease, he said.

”Many lives have been lost due to the disease through a lack of awareness,” Nihan said, ” and yet there was no laws about it.”

He said that all the DRP MPs supported the bill, and congratulated MP Visaam for his work.

”I would like to take this opportunity would like to thank the police, NGOs and individuals who work really hard for the thalassemia patients,” he said.

Maldivian Democratic Party (MDP) MP Eva Abdulla said the bill should be more broader and comprehensive rather than focusing solely on for thalassemia.

”As thalassemia is a blood disorder, the bill could be broadened by making it a bill for other blood disorders,” Eva said. ”There are many blood disorders that are very common in the Maldives.”

Eva noted that 38 percent of females of reproductive age were affected by anemia while 50 percent had child anemia.

”We want the bill to be a bill for other blood disorders,” Eva said. ”The treatment policy in the bill was just the same policy used previously – awareness programs and screening.”

Eva suggested that prenatal diagnosis would be more effective to prevent the disease.

”The third thing we highlighted was to establish a hematology (blood) centre instead of a thalassemia centre,” she said, adding that all the suggestions would be incorporated in the committee stage and discussed.

”We want the bill to be broader,” she said.

Correction: This article formerly stated that 38% of women suffered from anemia, when it should have read 38% of women of reproductive age. Several translation errors have also been corrected.


Official calls for premarital medical test

March 4, 2010

Courtesy by: arabnews.com

It called for the criminalization of marriages in which premarital medical checks are not carried out and the Ministry of Justice’s endorsement is not obtained.

The Health Affairs Department made the statement after discovering a number of potential couples carry genetic or communicable diseases, Al-Madinah newspaper reported.

“Health officials discovered that 1,801 persons were unfit for marriage out of a total of 92,149 people who underwent medical checkups over the past five years,” said Saeed Al-Zahrani, spokesman for the Health Affairs Department in Taif.

Of those tested, four were HIV-positive, 931 carried sickle cell anemia genes, 17 carried the actual sickle cell disease, 307 were either carriers or ill with thalassemia, and 542 were carriers of hepatitis B or C.

Al-Zahrani called on would-be couples not to neglect the checks, adding that 34 people went ahead with their marriages even though checkups showed they suffered from genetic diseases.

He said such people are jeopardizing the health of their future children and subsequent descendants.

Sheikh Abdul Mohsen Al-Obeikan, adviser at the Royal Court, also called on punishing marriage registrars who ignore premarital checkups when conducting marriages.

He added that there is a committee at the Justice Department to monitor marriage officials who register marriages without completing necessary formalities.

“An official who conducts a marriage without fulfilling regulations is responsible for the damages his act might cause to society,” said Al-Obeikan.


Family Laws Amendment Bill presented to NA

January 12, 2010

Courtesy by: app.com.pk

National Assembly Tuesday granted leave to move ‘The Muslim Family Laws Amendment Bill 2009’ further to amend the Muslim Family Laws Ordinance 1961.

MNA Justice ® Fakhar-un-Nisa Khokhar moved the bill to the House that was sent to Standing Committee concerned by Acting Speaker Faisal Karim Kundi after the government did not oppose it and the House granted leave to move the bill.

Presenting the bill to the House, the member said, it provides for presentation of a medical certificate about blood test by the bride and bride groom that they are not suffering from Thalessemia.

Referring to specific amendment in the Ordinance, she said, the Bill provides ‘before registration of a marriage, the Nikah Registrar shall ensure that pre-marital blood screening of the bride and the bride groom have been undertaken and certificate to this effect have been produced by each party before the Nikah os solemnized or registered.’

She said in Pakistan 12.5 million children and individuals are affected by Thalassemia, which can be controlled through appropriate tests before entering into wedlock. In this way, we can prevent the birth of children suffering from Thalassemia.

Advisor to Prime Minister Shahnaz Wazir Ali described this bill as of utmost importance and said, the rate of under nourished children and those suffering from blood diseases was high and blood test must be a requirement before marriage.

She urged to regularize functioning of medical laboratories in private sector and also upgrade the testing system at the public sector laboratories.

Shahnaz said, the medical standards regulation system also needed to be strengthened both at provincial as well as federal level.


Pre-nuptial tests outlined in handbook

October 30, 2009

Courtesy by: thenational.ae

A handbook listing the pre-nuptial medical tests and check-ups that people have to take before entering into a marriage has been jointly released by the Ministry of Health and the Marriage Fund Corporation.

The booklet is part of a series of projects put together by the two government institutions after Dr Hanif Hassan Ali, Minister of Health, and Dr Maitha al Shamsi, Minister of State and chairwoman of the Marriage Fund, signed a memorandum of understanding in June. Sixty-thousand copies of the book will be distributed through both ministries and also made available in clinics and medical centres, the government news agency WAM reported yesterday.

It will also be given to new couples applying for a Marriage Fund.

Dr Mahmoud Fekri, the executive director for health policy affairs, said the handbook was in line with a personal affairs law of 2005, Article 27, in which it is stated that medical tests are compulsory before marriage for anyone wanting to wed in the UAE.

The medical report must be presented before a marriage contract can be finalised and both parties have to be clear of all the diseases listed in the handbook.

These include inherited blood disorders such as beta thalassemia and sickle-cell anaemia; contagious diseases; and sexually transmitted diseases such as HIV and hepatitis B and C.

In March 2008, the MoH began providing services for pre-marriage tests through nine medical centres across the UAE.


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