'Marriage with strings attached' Article published in The News International

The News on Sunday, 22 November, 2009
http://www.jang.com.pk/thenews/nov2009-weekly/nos-22-11-2009/dia.htm#4

awareness




Marriage with strings attached

Interestingly, the NWFP PHB 2009, which makes it mandatory for a prospective couple to get their blood tested for hepatitis C and thalassemia, envisages no mechanism for its implementation

By Ammara Farooq Malik

Six and a half years ago, if someone had asked me what a blood disorder was, I would definitely have shown my ignorance. My family had no history of blood disorders and so I could not have imagined in a million years what was going to happen to my own child.

It is high time that someone who has experienced the misery of having a child with a blood disorder spoke out. Every single person who is affected by a blood disorder, thalassemia or leukemia, wishes that no one should have to go through so much pain. It's just that very few people who are directly affected by this disease have the courage to put their depression and misery aside to speak up for others.

One such person was 24 year old Salman Mahmood, a thalessemic, who died this October trying to raise awareness about the difficulties faced by such families. Salman died advocating that at least premarital blood screening should be made compulsory in Pakistan.

Facts about thalassemia

- Pakistan has more than 1,50,000 thalassemia major cases.

- At least 10 per cent of Pakistan's population or one in every ten persons is a thalassemia minor carrier.

- There are 5,000 to 6,000 thalassemia babies born every year.

- The estimated birth rate of thalassemia-affected children is 1.3 per 1,000 live births.

- At this estimated rate, the number of thalassemia cases will be doubled to over 200,000 in the next 10 years.

- The national exchequer incurs an expenditure of approximately Rs 130,000 per thalassemic child annually.

- These are only estimated numbers, the actual numbers (including the unreported cases, the deaths following birth and the cases involving other blood disorders such as enzyme deficiencies) are much higher.


A cry of joy ran through the families of children who heard this November that the NWFP government had finally passed the NWFP Preventive Health Bill (PHB) 2009. The Sindh Government had also passed a similar resolution in October to make blood testing compulsory before a couple decides to get married.

According to Health Secretary Khushnood Akhtar Lashari, the government is working on legislation for compulsory blood screening before marriage to protect newborn babies from thalassaemia and added that the bill in question had already been sent to the National Assembly Standing Committee. However, the matter has not been raised for further debate in the Parliamant yet and one hopes that further amendments to the proposal can still be considered before such a bill becomes a law.

Interestingly, the NWFP PHB 2009, which makes it mandatory for a prospective couple to get their blood tested for hepatitis C and thalassemia, envisages no mechanism for its implementation. There is a sanction imposed upon the Nikkah registrar that in the event that he is unable to register the test reports of the couple before the solemnisation of marriage, he will be liable to have his licence as registrar revoked. If, however, the couple decides to by-pass the registrar and get another individual to solemnise their marriage without such blood test results, that person shall be fined Rs 10,000 only.

The premarital blood screening can save not just one life but the lives of all the members of such a family which has to deal with the financial and emotional difficulties of hereditary blood disorders.

Former Dean of Children's Hospital, Dr. Sajjid Maqbool, is of the view that making blood tests compulsory before marriage would be very useful. He also suggested that a media campaign should be launched to create awareness about such a proposal.

However, where there are supporters of the cause there are critics too. A famous hematologist in Lahore, on condition of anonymity, gave a half hearted response to such a proposal, saying: "How can such a law be made? It would be impossible for everyone to get their blood tested. It's so expensive. Such laws suit other countries more than Pakistan."

Saudi Arabian case

Granted that Pakistan has a host of problems, but one of them does happen to be the rise in the number of thalassemia and other blood related disorders. Many other Islamic states have similar laws on the point. In Saudi Arabia, blood screening for thalassemia before marriage became a law in 2004.

Though initial research findings of the premarital screening programme under the 2004 legislation in Saudi Arabia, which has included tests for AIDS and hepatitis since 2008, are rather sobering, one has to give this programme more time to show positive results.

According to a study published in 2007, of 488,315 individuals tested, 2,375 couples were rated 'high-risk'. A total of 89.6 per cent of these couples got married despite the risk involved while only 11 per cent of these couples decided to call off their weddings.But the point is: at least 11% did.

A Pakistani couple that decided to settle in the UAE and had a thalassemic child eventually decided to undergo a risky bone marrow transplant to cure their daughter. Now after almost six years of good health, the parents are keen to help others to fight this disease as well. When contacted to express their views about such a legislation in Pakistan which can help control thalassemia, the mother remarked, "It would be wonderful if such a law can be passed in Pakistan. Thank God, it's compulsory in the UAE to have oneself tested before marriage…so by the time it's my daughter's turn to get married, we can make an informed decision."

In Pakistan, it would be difficult to lay down one uniform law for all provinces on this point, considering we have a very low percentage of high literacy rate and access to healthcare facilities. But according to Barrister Ahmad Farooq Malik, who is an active volunteer at a health and social awareness think tank: "What can be done here is that at the time of making the national identity cards, an evaluation of blood type and testing for thalassemia traits should be done. This will ensure that people can know their status of hereditary conditions before they get married."

The NWFP law does not force a high risk couple to not get married. However, it focuses more on letting the couple be informed about the consequences of their decision. The law further requires the registrar to keep the blood test reports for a period of two years. The problem with this requirement is that medically a report for identifying thalassemia traits may remain valid even after two years but a test to identify hepatitis may not be valid even after a few months. Saudi Arabia requires men to get themselves tested for thalassemia and HIV every time they are about to enter into wedlock and acknowledges such blood test reports to be valid for a period of six months only. According to health officials, "The disease spreads because of intermarriages. As a result, the disease is carried from generation to generation,"

Mufti Kafayatullah, a JUI member, validly opposed the NWFP Bill on the grounds that it would be very difficult to screen people in places such as Kohistan and Chitral. One can argue logically here that it is the responsibility of the government to allocate funds within the provincial budgets to make allowance for such compulsory screening. There is no point in making a law where there can be no possibility of its implementation. Naturally the person who will have to bear the brunt of such a legal violation under the PHB 2009 will be the nikah registrar or any other such person who will solemnize the marriage without having the requisite test results. When the poor and possibly uneducated residents of Kohistan and Chitral will not be able to afford the tests themselves then they may work their way around the law and get married anyway, giving rise to corruption and legal violations.

The government allocated a 20 per cent increase in the Fiscal Budget 2009-2010 for the health sector. However, sources said that the programme for the prevention and control of thalassemia had been discarded due to the financial crunch. Philanthropists and NGOs must step forward at this stage after the passage of the PHB 2009 to urge the government to allocate funds for the implementation of this law because without it, the spirit of this law and the morales of 150,000 thalassemia major patients and their families will indeed be crushed.

Though this law may not be the only solution for protecting people from HIV/AIDS and hepatitis, it certainly is a way to avoid a hereditary disease which cannot be checked by safe intercourse practices, women's empowerment or accessibility of quality services.

There may be doubts about the law's implementation and effectiveness, but this bill is a first step in the right direction. A parent whose child has thalassemia remarked, "This is a very positive step. This can save so many people the agony of having a child who has to have a life of transfusions and pain…and who may possibly die just by the age of 10."

What such a health policy gives a couple is a CHOICE…a choice many couples were not able to have. Couples may feel that they still want to go ahead and get married despite knowing that they are thalassemia or other blood disorder carriers, but then they will become solely responsible for the health concerns of their off springs. If the effects of thalassemia and other blood disorders can be fully explained then hopefully with more awareness, not many couples will be willing to live with this guilt of knowingly bringing a child in pain into this world.

The writer is an academic lawyer and founder of SEPLAA , a health and social Think Tank. She is currently writing her memoir on dealing with bone marrow transplants and can be reached at ammarafm@gmail.com

How can transfusion dependent children get used to needles?

Do transfusion dependent children ever get used to the scary needles?

It's heartening to see little children born with blood disorders or those who unfortunately are diagnosed with leukemia to be tested repeatedly through blood tests.

The blood tests have to be conducted through blood drawn through the veins in the arms or through the veins on the hands.

When babies have to be tested, the worse part is that their veins are too tiny to be easily visible and so finding the right 'spot' to prick the child with the needle becomes a tricky part. Often the needle is pricked and the nurse or doctor has no choice but to move the needle left, right and centre so that the blood starts coming into the vile or tube.

The needle can become such a traumatic sight for the child that some children develop a fear of needles for a very long time, some times even when the prick does not hurt that much. On the other hand there are children who become so used to the prick of the needle that they hardly even wince when they are pricked. This later case is a relief for parents who have to see little children aged less than 6 years of age, go through repeated blood transfusions or phlebotomies for blood samples being drawn for tests etc.

The best thing for parents to do would be to let the child feel safe in their arms while the needle is being pricked. Hold the child tight so that the child does not move. Any untoward movement will very likely mean that the child might need to be pricked twice.

A local anesthetic cream for children called EMLA is very effective too. It helps to numb the area where the child is to be pricked. However studies have shown that long term and prolonged use of EMLA is harmful for the veins of the child, so use it only when you know that the spot that's going to be pricked is an especially sensitive spot (such as the inner wrist, foot, scalp etc. which can be very painful spots for children to have needles inserted in).

Getting used to needles is something that happens so gradually that even the child does not notice that he or she is getting accustomed to the pain. But this happens only with thalassemia or blood disorder patients. Leukemia child patients who may develop the disease after the age of 6 remember the pricking sensation of the needle very vividly and usually find it hardest to adjust to the prick of the needle.

At this point, doctors usually suggest a Central Venus Line to be inserted in the chest of the child patient. A CVL is a tube dangling from the chest of the patient which has to be inserted into the body normally through an incision in the neck and it is inserted into a main vein close to the heart. This vein then comes out of the chest and hangs there with a clip attached at it's end to open and close the valve of the tube.

Once inserted a CVL is an extremely useful device to give the child iv medicines, transfusions of packed cells (Red Blood Cells), platelets and even a bone marrow transplant.

A child becomes much more relaxed and the best part is that the CVL can be used daily for drawing blood samples for testing too.

In cases where a child becomes very difficult or scared of being pricked, doctors sometimes suggest (if they are convinced that the child will be pricked repeatedy in the future too) to get the child a CVL. A CVL can function without any major problems for ypto 6 months. After which time a fresh CVL will need to be srgically inserted under general anesthesia.

One of the biggest risks of having a CVL inserted apart from the risks involved in surgery, is that the tube can be a HUGE source of infections. So though the CVL will help the child through a lot of pain, an infected tube, especially where blood counts are (probably) already not normal, can become a cause of death.

I've written a small explanation of some key terms that I feel parents who have blood transfusion dependent children should know. If any one would like to add to the thoughts expressed above then I would be very happy to hear. My daughter has had around 100 transfusions, several before and some after her bone marrow transplant.

My daughter did become used to the needles at one point but when she had a break from the pain for a few months, it was a whole new dilemma. I'm writing about my experiences with my daughter in my memoir. hope it helps other parents and many doctors who have to deal with child patients in such predicaments.

Until we meet again!

Stay positive for the children!

A Warm Welcome To My Support & Guidance Group

Hello and welcome to my support blog for patients and their families who are going through chemotherapy, blood transfusions or a bone marrow transplant.

I'd like to begin with a background as to why I've started this blog.

I'm sure there are a lot of support groups out there that are run by psychiatrists or doctors but I felt that I had learnt so much in my twelve years of first hand experience in handling near and dear ones fight cancer and then see my own daughter struggle through over six years (to date) of transfusions and bone marrow transplants...that I know it is now my responsibility as a surviving human being to share all that I have learnt. If all that I write helps anyone in pain out there in any way at all then it would give me a deep sense of relief, contentment and humility.

There are so many little things the doctors don't explain and the psychiatrists sometimes overlook. Things only a person going through the pain can understand or a parent seeing his or her own child can feel.

I hope to explain many misconceptions of blood disorder related practical problems and the pitfalls related with bone marrow transplants in my posts.

I came back from Italy to Pakistan two months ago after my daughter got her medical treatment there. We experienced the most extraordinary incidents, some related with medical issues, some with pain, some with losing friends to death, some with language and communication issues and some even with clashes of faith, culture and racism.

I am deeply grateful to the doctors at the Policlinico San Matteo, Pavia, Italy, who did a wonderful job but....there are many 'BUTS'!

When we were living in Italy, it was something like living in 'un altra mondo' ( a different world) and I want to capture all the friendships made with people from different countries and faiths, the pain and suffering of chemo, the successes and the tragedies at the hospital, the politics and how it affected the patients... all in the form of a memoir. I know that this account will be most invaluable to patients, doctors, psychiatrists, social workers and ordinary people who will learn to live and enjoy the most precious gift they've got: LIFE.

I also want to write it because I want my 6 year old daughter's courage and struggle to not be erased and forgotten from memory...but to live on and give others the same courage and support that she gave to me. My daughter has really shown me how to live and I pray to God that she has a beautiful and fulfilling life ahead of her.

Since this blog is meant as a support group, please feel more than welcome to ask any questions which you might feel are 'too silly to ask the doctor'. It is my hope that I will be able to help but my promise that I will do my best to guide you.