What is Bubble Baby Syndrome? 2 month old girl from Karnataka becomes one of the youngest patients to receive bone marrow transplant

Geetesh had lost his first child with the same disorder.

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So what is bubble baby syndrome?

The 1976 Hollywood movie starring John Travolta, The Boy in the Plastic Bubble depicts the story of a person born with this disorder.

It shows the life of Tod Lubitch, a boy born with a deficient immune system, forcing him to live inside a sterilised environment to avoid exposure to germs that could be fatal. Tod falls in love with his neighbour, Gina, and despite the risks, he decides to venture outside his protected bubble to experience the world and pursue a relationship with her. The movie shows how he encounters the challenges and potential dangers of living beyond the safety of his controlled environment.

This movie popularised the fatal disease in common layman’s words, ‘Bubble Baby Syndrome’.

“Individuals with SCID or Bubble Baby Syndrome lack functioning immune cells, making them highly susceptible to infections from bacteria, viruses, and fungi. This condition is often diagnosed early in infancy. The term bubble baby is used since these children need to be isolated in a sterile environment to protect them from infections,” Hyderabad-based haematologist Ganesh Bollam told South First.

SCID occurs due to genetic mutations that affect the development and function of T cells and sometimes B cells (antibody molecules that can latch on and destroy invading viruses or bacteria), which are crucial for the body’s ability to fight infections.

Without a properly functioning immune system, affected individuals are unable to defend against common pathogens and can become seriously ill from infections that are typically manageable or not severe in people with healthy immune systems.

Treatment for SCID may include bone marrow or stem cell transplants to replace the faulty immune system with healthy cells, gene therapy to correct the genetic defect or enzyme replacement therapy.

“The biggest problem we face for any BMTs is in selecting the most suitable donor — whether from a related or unrelated source — and ensuring compatibility through meticulous matching procedures,” said Dr Bollam.

“Following that, we should on the toe to closely monitor the patient for any signs of complications or adverse reactions. And post the transplantation, we need to manage the patient’s recovery, any complications that may arise, and adjust treatment accordingly,” added the haematologist.

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Anisha’s treatment

Following her diagnosis, Anisha became one of the youngest patients to receive a bone marrow transplant from an unrelated donor at BJ Wadia Children’s Hospital in Mumbai.

“This is one of the youngest transplant patients in the country to receive stem cells from a volunteer donor. It is satisfying to treat patients, including very young babies, at Wadia Hospital using complex therapies such as bone marrow transplantation. Her case underlies the importance of early diagnosis and the collaborative efforts of the medical professionals and bone marrow donor registries,” said Wadia Hospitals CEO Dr Minnie Bodhanwala in a statement.

Upon the diagnosis, Anisha was transported to Wadia Hospital from Mangalore in early November as her parents recognised the urgent need for a bone marrow transplant for her.

“Anisha’s parents were aware that she needed the bone marrow transplant (BMT) at the earliest. From the Mangalore hospital, they were referred to our BMT unit. While primary immunodeficiencies are rare, affecting 1 in 100,000 people, life-threatening immunodeficiencies can be entirely cured with a BMT,” said Dr Prashant Hiwarkar, BMT transplant physician at Wadia Hospital in a statement.

The medical team worked tirelessly to find a fully matched donor within days of Anisha’s admission, collecting and transplanting the stem cells on 11 November.

Following two months of hospitalisation, Anisha was discharged on 26 December.

During the period of awaiting a suitable donor, Anisha stayed in the bone marrow transplant ward, where precautions were diligently observed to prevent contact with pathogens that could cause infections.

An early diagnosis and swift referral to the transplant centre played a pivotal role in facilitating a timely transplant for Anisha, averting any major complications.

“Time is crucial in children with SCID as infections can be fatal. Our team worked 24×7 to check the three Indian bone marrow registries and reach out to the fully matched donors. Luckily, within a few days of her admission with us, we found a fully matched donor for the transplant,” said Dr Hiwarkar.

“We did not want the history to be repeated with Anisha and we are lucky that we got the donor and the transplant done on time before she got any infection,” Geetesh Bandekar, said.

The family is going to stay in Mumbai for the next six months to follow up with the doctors.

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