Abu Dhabi family's plight puts spotlight on 'bubble boy' disorder

Aisha has been in ICU for almost a year – and there is a 25 per cent chance her sister has the same disorder

Aisha Rizwan in the intensive care unit at Burjeel Medical City in Abu Dhabi. The 15-month-old has not left the hospital since April 2022. Photo: Rizwan family

Aisha with her brother Ahmed, who donated bone marrow to help his sister. Photo: Rizwan family

Aisha and Ahmed before she was taken to hospital. Photo: Rizwan family

Aisha's sister Anaya, who was born last month, has a one-in-four chance of developing SCIDs. Pawan Singh / The National

Rizwan, Ammara and Anaya in the isolation unit at Burjeel Medical City in Abu Dhabi. Pawan Singh / The National

A couple whose infant daughter has spent nearly a year in intensive care say they hope that she can return home one day soon.

Aisha Rizwan, who is 15 months old, was taken to hospital in Abu Dhabi last April after contracting a viral infection.

Doctors fought for eight hours to save her life after her heart and kidneys stopped functioning.

Aisha survived, but doctors fear she suffered brain damage from the oxygen shortage.

“To see your child’s heart stop and then be on a ventilator and in a coma ... I wouldn’t want any parent to ever experience that,” her mother, Ammara Javed, 30, told The National.

Aisha was born healthy, so Ms Javed and father Rizwan Farid, 39, from Pakistan, were unaware that their daughter had severe combined immunodeficiency (SCID) until her hospital admission.

SCID is a group of rare disorders caused by mutations in genes involved in the development and function of infection-fighting immune cells.

Children with with the disorder lack the ability to produce an immune system.

The condition is often called “bubble boy disease,” after the 1976 film The Boy in the Plastic Bubble, starring John Travolta, who cannot have direct contact with the outside world.

Both Aisha's parents carry a gene that makes the condition, which occurs in one in every 100,000 births, far more likely. There is a one in four chance that a child born to Ms Javed will have SCID.

If detected early, a bone-marrow or blood stem cell transplant could cure the disorder.

But the child is often born healthy, which means it is often detected only after they have caught a potentially fatal infection.

Aisha had a bone-marrow transplant, in July 2022, from her brother Ahmed, 6.

While her immune system has strengthened, the damage caused by the virus has left her gravely ill.

“They are weaning her off the ventilator and we hope that she will be home with us soon, but it is all in God’s hands right now,” Ms Javed said.

Sister Anaya kept in isolation

Last year, she became pregnant again and last month gave birth to Anaya, which means gift.

Doctors carried out tests on Anaya within a few days of her birth, and as a precaution she is being kept in isolation at the same hospital.

If positive, she will immediately undergo a bone-marrow transplant to give her the best chance of developing a strong immune system.

Heartbreakingly, the family cannot be in the same room together given the need for isolation, but they hope to return home together, whatever Aisha's condition.

“Outcomes for severe combine immunodeficiency are greatly improved by early diagnosis and treatment,” said Zainul Aabideen, head of pediatric hematology oncology and bone marrow transplant at Burjeel.

'Early diagnosis is essential as patients who receive a transplant before three and a half months of age have the best outcomes," Dr Aabideen said.

“As we speak, there will be few babies in UAE with this problem — they are at home and no one suspects that they have SCID. ”

These babies will be repeatedly admitted to hospital for various infections for months on end until a diagnosis is made.

Screening for SCID is standard in many western countries, but rare in the Middle East.

“We recommend that it be standard in the UAE and the region,” said Mansi Sachdev, a transplant consultant at Burjeel Medical City.

“Not all newborns get this test done — it is more when you know there is a suspicion that a child is getting frequent recurrent multiple infections," Dr Sachdev said.

"That is when this test is done. What we are recommending and saying is that there is a need to get this screening test done for newborns in the UAE and region as well.

"This has already been happening in western countries on a routine basis but not here."