How is our ‘Bubble Baby’ doing? Many are the calls to the Sunday Times from anxious readers wanting to know how baby Sanjana Praveen Shivanka who is now at the Apollo Speciality Hospital in Chennai, India, for the treatment of the rare genetic disease, Severe Combined Immunodeficiency Syndrome (SCID), is faring.
Shivanka has had the transplant, says Clinical Haemotologist Dr. Lallindra Gooneratne who is in touch with Consultant Haemotologist Dr. Revathie Raj who is looking after Shivanka at Apollo, explaining that a stem cell transplant is best performed with donor stem cells which are compatible (closely resemble the patient’s stem cells but are not diseased). The source of these stem cells can be from a compatible close relative, a compatible unrelated person (can be difficult to find) or a compatible umbilical cord from a ‘cord blood bank’.
For good engraftment (donor’s stem cells to replace the baby’s diseased cells) to take place, chemotherapy and drugs which suppress the baby’s immune system even further have to be given before and after the transplant. This is associated with a significant mortality (risk of death) and side effects, he says.
Therefore, the transplant team from Apollo Hospital in consultation with experts from Great Ormond Street Hospital (GOSH), London, decided on a procedure called ‘CD34 selected haplo infusion’ of the father’s stem cells without any conditioning chemotherapy. This way the transplant related mortality is close to 0% and the baby will not be exposed to chemotherapy with its short and long term complications, explains Dr. Gooneratne.
Has the transplant been a success? Says Dr. Gooneratne, “I think it would be best to quote the words of Dr. Revathie who says, ‘Baby is doing very well. CMV PCR has been consistently negative and he is thriving beautifully. You have to see the baby to believe how good he looks’. CMV is a viral infection which can infect patients with the baby’s immune deficiency condition and patients who have undergone a transplant. She has also been sending photographs which indeed substantiate her claim. Baby Sanjana does look very well!”
However, there is still a long way to go, he explains, adding that although the baby’s bone marrow is now producing some amount of cells of the immune system (lymphocytes) they are not adequate. The experts from GOSH say that it may take up to about four months for adequate numbers of lymphocytes to be produced.
On the question whether baby Shivanka will have to remain in India until then, Dr. Gooneratne says it is not necessary. As he is doing quite well and free of infections, the plan is to send him back to Sri Lanka in a week or two. Of course, he will require close monitoring and injections of immunoglobulin to prevent infections.
It was the overwhelming response from Sunday Times readers who sent in numerous donations, both large and small, to collect Rs. 5.2 million that enabled Shivanka’s parents K.B.N. Damayanthi and K.W.N. Neil Shantha of Dippitigala, Lellopitiya in Ratnapura to take their baby to India for this life-saving procedure.