I WOULD love to see a sickle cell free Zambia, says bone marrow transplant specialist Vikas Dua.
The Head and Additional Director in the Department of Paediatric Haematology, Oncology and Bone Marrow Transplant at Fortis Memorial Research Institute in Gurugram, India was recently in the country to conduct workshops on Sickle Cell Anaemia.
In an interview last month, Dr Dua said as research advances in the field of Sickle Cell Disease, a new range of treatments and medicines are being introduced.
“In the past Sickle Cell Disease was incurable and millions of lives were lost worldwide. But today science has advanced and so has treatment for SCD,” he says.
Sickle Cell Anaemia is an inherited form of anaemia – a condition where there are not enough healthy red blood cells to take oxygen throughout the body. Normally red blood cells are healthy and round, moving easily through blood vessels. In SCA, however, the red blood cells become rigid and sticky and are shaped like sickles or crescent moons.
These irregularly shaped cells can get stuck in small blood vessels, which can slow or block blood flow and oxygen to parts of the body.
Dr Dua says people living with sickle cell should consider bone marrow transplant as an option for treating the disease.
He adds that in the absence of centres in Africa where the BMT can be conducted, India, compared to countries such as the United States and Australia, is the most cost-effective destination for receiving the treatment.
He says BMT is the only curative treatment available for this disorder where one preferably finds a full match donor.
Last year, Dr Dua led a team in treating Lukumo Chibilu, an eleven-year-old Zambian boy who was diagnosed with sickle cell at age 5.
Lukumo received BMT from his younger
He returned to Lusaka in May and is recuperating at home.
He is the second-born of four children born to Robert and Mwanza Chibilu.
Lukumo’s immediate younger sister also has sickle cell.
What has been your main challenge in getting people to consider Bone Marrow Transplant as a treatment for Sickle Cell Disease?
Whenever I discuss with people in the whole of Africa and say transplant is a cure for sickle cell, they say that, ‘oh, but people don’t have money’. I say I greatly understand; it’s an expensive thing but at least give them the option. At least tell the families and let them decide. How can I decide just by seeing somebody’s face that he cannot afford? It’s my duty as a doctor to tell the family that this is the cost and then we leave it to them to do what they want.
How prevalent is Sickle Cell Anaemia in Zambia?
Sickle cell is very prevalent. Let’s start with the whole of Africa. Nigeria is called the ‘sickle capital’ of the world because the prevalence rate of carriers is high in Nigeria. But Zambia is not far behind. In Nigeria also there are a lot of carriers. So what you need to understand is that if two carriers are getting married, then there is a chance that they may produce a sickle baby, which is a problem. It’s a problem for the family, a problem for the baby. So that’s how we need to create awareness about preventing the birth of sickle cell anaemia in a baby, and once a [sickle cell] baby is born, how to treat that.
So sickle cell can only be passed on to a child if both parents have the trait?
Correct. If the two carriers – you can call it trait, you can call it carrier – get married, there is a 25 per cent chance that they may produce a sickle cell anaemia baby. That doesn’t mean if they produce a sickle cell anaemia baby now, then the next three pregnancies are safe. That’s not the thing. In every pregnancy, there is a 25 per cent risk. Like in the Chibilus’ case, two children are sicklers and the other two are not.
What were you addressing in the workshops you recently held?
First we went to Ndola, then Kitwe and then we had a workshop at the University Teaching Hospital in Lusaka. Every year we discuss the same things; we discussed about the prevalence [and] how to prevent sickle cell. You cannot stop two people getting married but at least if they know their sickle status, if they do a simple test called HPLC, or you can call it Haemoglobin Electrophoresis – that’s the two names. By that you can know whether you are a carrier or not. So if one is a carrier and the other is not, no problem. [But] two carriers get married, still inside the womb, between ten to twelve weeks of pregnancy – between two and three months of pregnancy, you can check inside whether the baby has sickle cell anaemia or not. Then if it’s a normal pregnancy, if it’s not sickle cell, you can continue with the pregnancy. But if it’s a sickle baby – sickle foetus inside you, then it is your choice whether you want to continue with the pregnancy or to terminate. That’s one thing.
And then we talked about Bone Marrow Transplant, how BMT has advanced in the last couple of years; what are the newer ways by which we are doing transplants, how nowadays the transplant is getting less risky and easier and how the success rate of transplants has proven in the past couple of years, and what is our experience in our hospital with the transplant [procedure] because we do one hundred to one hundred and twenty transplants in a year, that means roughly ten transplants in a month. And there are a lot of people from the whole of Africa who travel to our hospital for Bone Marrow Transplant. So these were the things that we discussed.
Does this mean there are no facilities in Africa where the BMT can be done?
Unfortunately, at this present moment in time, there is no facility available in Africa where transplant can be done. But we are really keen and interested in starting this facility in Africa, in Zambia. In the near few days, we are going to discuss with the Ministry of Health if we can open a transplant centre in Lusaka so that people don’t have to leave the country for transplant. You don’t have to spend so much money. If it is done in-house, in your own country, then the costs can go down and the logistics can be easily managed.
How did the connection between you and Lukumo come about?
This is not my first time to Lusaka. I came here twice before. So we conducted similar workshops in UTH, though it was my first time to go to Ndola and Kitwe. But in the previous two visits, when we came, we met a couple of families who were quite keen to learn about how transplant could be a permanent cure for sickle cell anaemia. So Lukumo’s family met me in one of those workshops and at that time we discussed these transplants. The family was quite keen and we did a test called HLA (Human Leukocyte Antigen) typing to see whether the donors – or siblings – are matching or not. There is a 25 to 30 per cent chance that your sibling will be a complete match to you on HLA. So we did the test. Fortunately the younger sister [Luusa], who I think was about 3 years old at the time, was fully matching and so once we came to know that she can become the donor, we invited them and that’s how they came to India.
Please shed some light on the BMT procedure itself
After the HLA typing, we do some blood tests on the patient and the donor to be sure that the patient is okay to accept and the donor is clear to donate. Then the patient is admitted in the hospital to get some chemotherapy so that his bone marrow is suppressed. He can accept the donor’s cells. It’s a change in the immune system, so once your immunity goes down, then only can you accept the donor cells. Simultaneously, the donor gets some injections under the skin for four days. On the fifth day, you collect the stem cells in the blood. Once the stem cells are collected, you infuse the stem cells and it takes around two to three weeks for the stem cells to start working in the patient’s body. So that means one week of chemotherapy and two to three weeks after transplant the patient will be in the hospital. That means three to four weeks in the hospital. Then weekly coming to the hospital for some blood tests and staying in there for at least the first hundred days till the time we are sure that the patient is okay to fly back. That’s what we did for Lukumo and that’s our general policy for all the patients who come to us for transplants.
How would you describe how Lukumo is doing?
When I met him the day before yesterday, it’s been six months post-transplant – 174 days to be exact – and he is doing very well. He is living a normal life and the donor cells are just jumping in his body and fully active donor. So we advised him to continue some of the medicines for two months and then he will be off these medicines and have a normal immunity and go back to school. We have asked his family that he stays away from school for the next four to five months because there is still some chances of infection. Once his immunity is recovered completely, he can go back.
How long have you been conducting the BMT procedure?
It’s been nine years. In this point of time we have done five hundred transplants all over the world. India has become a medical hub because there are a lot of people from Africa, the Middle East, people from different parts of the world travelling to India for Bone Marrow Transplant. The reason is simple: the expertise is there, qualified people are there, and the cost. Cost is much less as compared to the US, UK or Australia. So if somebody thinks of going for a transplant in the US, he will be spending, I would say 50 times more than the cost of what they would pay in India. Though it is still expensive for people to get the transplant treatment in India, but you cannot compare that with the United States. It is huge.
What’s your advice to governments and other stakeholders regarding SCD?
My advice is first and foremost we need to do prevention. Prevention is better than cure. So we need to have some policies in which we do the HPLC test free of cost before conception so that we know the carrier status and we can then only counsel the families if there is a carrier. Then the CVS [Chronic Villus Sampling] test which can be done between ten to twelve weeks of pregnancy is always advisable in case two carriers are getting married. … I think 10,000 sickle cell anaemia patients are born every year in this country and there are a lot of mortalities, lots of deaths happening because of strokes, chest syndromes and back complications. So we need to have our own transplant centre. We need to think of our own transplant centre. Until that time, there should be collaboration with people like us who can help you in treating these sickle cell ‘warriors’.
Your final thoughts?
For the people of Zambia, I would love to see a sickle free Zambia. I would love to see a sickle free Africa because it’s not that we are gaining financially by doing the transplant but we want to prevent the disease because these kids are really suffering. We are seeing the pain and agonies of the families who have these sickle cell warriors. They have to rush to the hospital in the middle of the night because of a pain crisis, or suddenly having a stroke or weakness in the bodies. So these young babies, these warriors have not done harm to anybody and they are unfortunately suffering. So we need to have some policies in the government that will prevent the birth of a sickle baby and we should target that by 2030, we should have a sickle free Zambia.