A special Christmas at home for ‘Queen of Robin Ward’ Isla

Affectionately known as ‘Queen of Robin Ward’ by her nursing team, Isla has recently learnt to walk again after being bed bound for so long with the help of the Physiotherapy team.

Isla is affected by a rare immunodeficiency called Activated phosphoinositide 3-kinase delta syndrome or APDS1 – an inherited condition which means she has a compromised immune system and will get very sick easily. Currently only a BMT can give patients a potential cure.

Isla, her older brother, Lewis, aged 11, and Mum, Chloe and other relatives all share the same immunodeficiency.

Isla first came to GOSH in early 2021 for her first BMT, but unfortunately she rejected her donor cells and had to have a second transplant in October 2022. She returned to GOSH again in early 2023 after further complications and remained on Robin Ward until September 2024.

Isla’s journey at GOSH in 2023 was incredibly tough. She continued to experience several life-threatening complications during her time here, including a severe gut bleed and almost 30 episodes of  sepsis. She received so many courses of antibiotics, that left her infected and unwell with highly resistant bacteria. During this time, her prognosis was so poor that she had been referred to and then discharged from the palliative care team, twice.

Throughout this time, her mum, Chloe, was also unwell and spent many months in their local London hospital recovering from brain surgery to treat listeria meningitis.

Isla’s clinical team led by Dr Rob Chiesa and Dr Persis Amrolia, Paediatric BMT consultants, worked together with many other teams at GOSH including Robin Ward, Specialist Neonatal and Paediatric Surgery (SNAPS),  gastroenterology, physiotherapy, occupational therapy, home parenteral nutrition and others to support Isla and her family through this time. Robin Ward specialises in the management of complex children with immunological disorders and infectious diseases and it is one of only two paediatric BMT units for primary immunodeficiencies in the UK.

The team also sought advice from the ethics committee to see if non-typical medications could be used to help Isla.

In April of this year, Isla became again very unwell with sepsis and gut bleed and after several treatments with no clinical improvement, the clinical team took the hard decision to perform surgery that would see Isla’s small bowel removed and a stoma bag put in place. It was a risky and complex surgery that is not routinely performed but as Isla’s prognosis was so poor, the decision was taken. In Isla’s case the procedure was a success enabling her to get better, and it is now hoped that it can be offered as a possible solution for the future.

Speaking about Isla’s return home Chloe said: “Our family has been through so much over the last year and to see her start walking again and spend time with her brothers playing outside of her hospital bed has been so special. Isla has been brave and resilient through it all and we are so grateful to all the team that helped us to get here”.

The family are excited to have Isla home after a difficult time.

Chloe says: “This time last year, Isla was so fragile with numerous infections, and we didn’t think we’d ever be able to celebrate with her again. So, to have her settled at home and involved in the magic of Christmas is everything we could’ve ever dreamt of."

Looking ahead to the big day, Chloe and Mitch will be hosting at their place as Isla is still vulnerable to infections. Chloe will be cooking her first Christmas dinner and says it will be a day surrounded by gifts, chocolate and untold amounts of pigs in blankets.

Isla continues to return to GOSH every month for follow-up clinic appointments as the team hopes to see her T-cells to increase. This is important so Isla can remain well and fight any future infections.

Dr Rob Chiesa said: “Isla and her family are very special to us and will always be part of the GOSH family.

“I am very happy and proud of Isla, of her endurance. Her story is one not only of resilience but also shows how highly specialised teams across specialties and directorates come together to support our families on their medical journey at GOSH."