Teen with rare blood disorder undergoes pioneering brain tumour surgery at Sheffield Children’s

“I wouldn’t be here today without the skill and dedication of the surgeons and the team at Sheffield Children’s.”

A teenager with an extremely rare blood disorder has undergone groundbreaking brain tumour surgery at Sheffield Children’s Hospital, in a case doctors say could help open the door for similar life-saving operations in the future.

The complex 13-hour operation required highly specialised planning to manage the risk of catastrophic bleeding caused by the condition Bernard-Soulier syndrome, a rare inherited disorder affecting platelet function.

The surgery, carried out by specialists at Sheffield Children’s NHS Foundation Trust, successfully removed the tumour and Ahmed, 17, from Sheffield, is now recovering well.

As Sheffield Children’s marks 150 years of caring for children and young people, the case highlights the role of specialist children’s hospitals in treating some of the most complex and rare conditions.

For more than a century, the organisation has pioneered care while bringing together national expertise to care for children and young people in often challenging circumstances.

Ahmed was diagnosed with Bernard-Soulier syndrome shortly after birth, when blood tests revealed dangerously low platelet levels.

His mum Maryam, a nurse, said: “We had never heard of it before. We were told the chances of having it were more than a million to one.

“We have always had to be careful. From the start we were told that surgery could carry a risk of death because his blood might not clot properly.”

In May 2023, Ahmed came home from school complaining of double vision. After being examined in hospital, doctors diagnosed a Pineal brain tumour.

Maryam said: “You never think you are going to be told that your teenage son has cancer. You always think it happens to other people, not you. The whole world just came crashing down around us.”

Despite chemotherapy, the tumour continued to grow. Doctors warned surgery would be extremely high risk due to Ahmed’s bleeding disorder.

Complicating matters further, Ahmed had developed antibodies from previous platelet transfusions, meaning standard transfusions would no longer work effectively.

Consultant Neurosurgeon Mr Veejay Bagga, who led the surgery, said: “If we did nothing, the tumour would have been fatal. Surgery carried enormous risks because of Ahmed’s blood condition, but it was the only option that could give him a chance.”

The surgery required a whole-team approach from Sheffield Children’s. Mr Bagga added: “It required incredibly careful planning between multiple specialist teams to make sure we could manage the bleeding risk safely.”

Consultant Haematologist Dr Jeanette Payne, who coordinated the complex blood management plan at Sheffield Children’s, said: “I have looked after Ahmed since diagnosing his condition when he was a baby. When he developed a brain tumour as a teenager it was incredibly worrying because treating cancer in someone with a severe bleeding disorder is extremely challenging.”

The surgery was successfully completed thanks to an efficient and effective multidisciplinary collaboration between specialists at Sheffield Children’s, alongside professionals from NHS Blood and Transplant (NHSBT). Throughout Ahmed’s time at Sheffield Children’s, colleagues across multiple teams worked harmoniously to deliver holistic and bespoke care, making sure Ahmed had the best possible outcome.  

Dr Jeanette added: “I am so pleased that he has done so well.”

In the weeks before surgery, Ahmed underwent plasma exchange treatment with NHSBT’s regional Therapeutic Apheresis Services team to reduce the antibodies affecting transfusions.

During the operation he received carefully selected clotting factors and specially matched platelets while his blood levels were closely monitored

Mr Bagga added: “Ahmed is brilliant now, he’s running again. We knew instantly that it was successful – it was a really good feeling.” 

The operation also relied on specialist support from NHS Blood and Transplant to identify closely matched platelet donations, so that his blood could clot. NHSBT provided 113 units personally matched for Ahmed, including 10 units for the operation and 24 for the recovery. More than 100 donors helped keep him alive.

Kirti Mepani, Clinical Scientist Section Head at NHS Blood and Transplant Colindale, said: “Platelets are essential for blood clotting and are especially important in people with Bernard-Soulier syndrome, where the body cannot form clots properly.

“But Ahmed could not receive just any platelet donation. They had to be carefully matched to his tissue type so his immune system would accept them.

“He is from a minority ethnic background, which can make it more difficult to find closely matched donors because some groups are under-represented in the donor base.

“This was a very high-risk operation that required close coordination between the clinical team, the hospital blood bank and NHS Blood and Transplant to ensure the right platelets were available.

“I am incredibly proud that our team was able to supply the carefully matched platelets needed to make the surgery possible.”

Ahmed is now in his first year of college doing T-levels in mechanical engineering. His walking is much better and he is starting to run.

Mum Maryam said: “The team at Sheffield Children’s gave Ahmed a chance we never thought was possible. Their expertise, care and planning made all the difference.”

Ahmed said: “I wouldn’t be here today without the skill and dedication of the surgeons and the team at Sheffield Children’s. They gave me a chance to continue my life and look forward to the future.”

Ahmed’s brain scan without tumour

Ahmed’s scan with tumour

It is hoped that the case will be published as a medical case report, which clinicians hope could help other patients with rare bleeding disorders access similar life-saving surgery in the future.