A five-month-old female infant, weighing less than 5 kg, who was diagnosed with a rare congenital immune disorder — Leukocyte Adhesion Deficiency (LAD) — got a new lease of life through a complex bone marrow transplantation (BMT) procedure at a private hospital in Bengaluru.
The infant’s early days were marred by recurrent severe infections reminiscent of the tragic loss of her elder brother to a similar infection during infancy. Her parents, Bandesha and Balamma, daily wage workers from Raichur district, noticed that her condition started deteriorating when she was two months old.
Malnourishment and low immunity made matters worse. A comprehensive immunological and genetic testing at Aster CMI Hospital in Bengaluru confirmed LAD, a rare condition with an incidence of one per million live births.
Immunodeficiency
LAD is a rare genetic disease that causes severe immunodeficiency, which prevents the body’s immune system from normal functioning. This makes children with LAD very susceptible to infections, which can be fatal. This life-threatening disorder often goes undetected, which emphasises the importance of early detection and intervention. The only curative treatment for LAD is bone marrow transplantation from a healthy donor, doctors said.
Fortunately, the mother emerged as a beacon of hope as she was human leukocyte antigen (HLA) compatible with the infant. Despite the inherent complications, doctors at Aster decided to go ahead with the BMT. Thus, the infant’s mother actually gave a new lease of life to her child twice.
Although the treatment cost went up to ₹15 lakh, the family paid only ₹50,000 due to their poor financial background. Upon understanding the family’s difficulty in arranging funds for the treatment, the hospital extended support through the Aster Sick Kids Foundation as well as CSR funds. The infant was admitted for the procedure on September 26 and was discharged on November 8.
Early intervention
Addressing presspersons here on Thursday, Anoop P, Senior Consultant, Haematology, Paediatric Haemato-Oncology and BMT, at the hospital, said, “Due to the complications involved, most hospitals, even in affluent countries, may prefer to perform BMT after the baby weighs at least 10 kg and is completely free of active infection. In this case, it was clearly not an option, as she would have succumbed to the disease. Our team chose to proceed with transplantation regardless of the baby’s age or weight as it was the only hope for her survival.”
Pointing out that the procedure was challenging, the doctor said post surgery the infant is recovering well. “Her immune system is healthy, and she is expected to lead a normal life,” he said.
A team of experts comprising Paediatric Haemato-Oncology and BMT, Senior Consultants - Stalin Ramprakash and Raghuram CP; and Consultant - Pediatric Immunology and Rheumatology- Sagar Bhattad conducted the challenging procedure.
