Yesterday a family came to seek help for their child who is detected with thalassemia. We meet parents only on Sundays but we made an exception for this family as they had come all the way from a place 600 km away. The 8 month old baby girl had already received 3 transfusions and the family wanted to discuss the prospects of transplants. They had already visited 3 of the very popular BMT centres in the country and taken opinion. Unfortunately though the child had a sibling, his HLA type did not match.
I realised that the family had been sufficiently and appropriately advised. They had been told by multiple doctors that there is no need to rush for a transplant. Still, they were desperate to find an immediate cure. Though had learnt the nitty-gritty of transplantation, they were extremely poorly informed about management of thalassemia. I emphasised that their child can lead a near normal life with or without the transplant. I emphasised the need for focus on systematic management of the disorder. At this point that the family opened up and remarked – “but our doctor told us that these children have no future and they won’t survive.” I was shocked!
When I enquired about the doctor who advised them, they told me that he was a local physician - someone who had no prior experience at managing kids with thalassemia. Without proper information, experience or training, the person decided to inform the family that there is no future for the child. At this point I understood why the family was desperately seeking a transplant – ignoring the involved risks and options. In the next few minutes I shared with the family stories of thalassemia patients who have done so well in life that they are an inspiration for the so called “normal children”.
Once told that the child has no future with chronic thalassemia management, the family rushed to seek the option of cure. The least risk option for transplant – stem cells sourced from sibling donors, was not an option for the family because the HLA did not match. When the family enquired about other options, they were advised transplants which have much higher risks involved. What was shocking is that the family was not made aware of the comparative risks between the procedures leave alone considering the option for management at all.
Thalassemia is a manageable disorder. If sufficient resources are available – with proper management and care – the patients can lead a near normal life. True, the option of transplant is there and a permanent cure is the desirable solution. However, for each child the choice between lifetime of management and transplants has to be made weighing the actual risks and benefits involved.
With the option of oral chelation therapy, advances made in the field of complication prevention and management, increased blood safety and strengthening of the care and management services – the mortality and morbidity associated with thalassemia has reduced drastically. Today, it is not surprising to find thalassemiacs well into their late twenties who are doing well in their professional and personal life. Of course the access to systematic care and management in itself is a big challenge. However the financial status of this family was good and they are likely to secure systematic treatment for their child.
Transplants within themselves range in success from about 60% to 95% chances of survival depending on the condition of patient, the source of stem cells and the centre where the transplant is being done. The family needs to know as accurately as possible what are their chances and then they can compare it against the option of regular thalassemia management and make a choice.
I found myself sitting against the parents who were looking at their child’s future with the view that if there was no transplant, their child may not survive at all. Surprisingly, the same family had little idea about what exactly is required for their child’s management in the coming month. The following minutes were spent demystifying the options available to the parents, the need for regular management and care, and most importantly – reinforcing the confidence of the parents that their child is most likely to have a near normal future, regular employment and personal life with or without the transplant.
When a family is told that their child is born with thalassemia major, it is a very delicate situation. Unfortunately we see this situation being mishandled every day. This is one story. We come across such families every week who have recently been informed about their child’s condition. They complain of doctors giving them very little time for consultation. They complain of little support from the extended family. The local physicians who they trust make the situation worse with half baked knowledge. If the family has financial resources, they become desperate to seek a solution and there starts a mad race from centre to centre looking for that someone who can offer the magic solution and make their lives normal once again. This is a very vulnerable situation to be in which opens these families up for exploitation.
In the entire pyramid of society this is the story of the top 10% - who have the resources. Darker truths confront those families who get the news of a child with thalassemia when they barely make a living.
The least that needs to be done is properly counsel and inform the families about their options taking a wholesome view of all three aspects of thalassemia – prevention, management and cure. Gently the shock of getting to know about the child’s genetic disorder needing lifetime of management needs to be replaced with hope and promise of a bright future.
The thought that even the educated and the resourceful parents are not able to escape unnecessary trauma was a troubling one. A few hours later I received an sms from the parent of one of the girl who comes to our centre– “Sugandha(name changed) scored 88% in first semester of first year B. Com. in-spite of suffering from measles during exam time”. That’s when I smiled once again.
तमसो मा ज्योतिर्गमय । Let us all move from darkness towards light!