Girl, 8, gets kidney transplant without need for life-long drugs in UK-first

A girl’s immune system was ‘reprogrammed’ by her mother before receiving one of her kidneys, a first in Britain.

Aditi Shankar received a stem cell transplant using her mother Divya’s bone marrow six months ahead of a planned kidney transplant.

The procedure, carried out in Great Ormond Street (Gosh) in London, means the eight-year-old’s body accepted the organ as its own, meaning she had to take a lifetime of anti-rejection drugs.

A year later, she can now behave like a typical girl of her age: she enjoys swimming, dancing and bouncing on her trampoline.

Aditi was diagnosed at the age of five with a rare one-in-three million genetic condition called Schimke’s immuno-osseous dysplasia, which affected her immune system and kidneys.

Aditi, 8, pictured with father Uday and mother Divya at Great Ormond Street Hospital, London, a year after a life-changing kidney transplant

Professor Stephen Marks, pediatric kidney specialist, pictured with Aditi during a post-transplant appointment.  Experts from the kidney, immunology and stem cell transplant teams at Gosh worked with international colleagues to develop a treatment plan, which involved a double transplant of bone marrow and kidneys.

Professor Stephen Marks, pediatric kidney specialist, pictured with Aditi during a post-transplant appointment. Experts from the kidney, immunology and stem cell transplant teams at Gosh worked with international colleagues to develop a treatment plan, which involved a double transplant of bone marrow and kidneys.

It meant she had to travel from her family home in Greenford, north-west London, to the children’s hospital for treatment at least three times a week.

In March 2021, her kidney function dropped dramatically, but her immune system was so weak that doctors feared a traditional kidney transplant would not work.

Although they serve a vital function after a transplant, immunosuppressants work by dampening the body’s immune system, meaning anyone who takes them is at greater risk of infection and other complications.

Experts from the kidney, immunology and stem cell transplant teams at Gosh worked with international colleagues to develop a treatment plan, which included a double transplant of bone marrow and kidneys.

After the bone marrow transplant, she spent four weeks in intensive care while continuously undergoing kidney dialysis.

Six months later, in March 2023, Aditi, an avid Scrabble player, was healthy enough to undergo the kidney transplant.

She took anti-rejection drugs for a month, but because the bone marrow transplant and the kidney came from the same donor, her new kidney works without them.

Divya, 38, said she was 'happy and proud' to donate both bone marrow and one of her kidneys to her daughter

Divya, 38, said she was ‘happy and proud’ to donate both bone marrow and one of her kidneys to her daughter

Aditi can now behave like a typical girl of her age: she enjoys swimming, dancing and bouncing on her trampoline

Aditi can now behave like a typical girl of her age: she enjoys swimming, dancing and bouncing on her trampoline

Divya, 38, said she was “happy and proud” to donate bone marrow as well as one of her kidneys to her daughter.

The shopkeeper said: ‘I was so happy that I could give her blood cells and a kidney. I’m just so proud.’

Professor Stephen Marks, a pediatric kidney specialist at Gosh, said she is the first patient in Britain to have a kidney transplant where the immunosuppressive medication is not needed.

“Because of her underlying immune disease, this meant she would not be able to receive a kidney transplant,” he explained.

‘Her immune deficiency had to be corrected by first receiving her mother’s bone marrow, and because Aditi was able to accept her mother’s bone marrow, it meant her body could see her mother’s kidney as part of her.

‘A month after the transplant we were able to take her off all her immunosuppression, so she no longer suffers from the side effects of the medications.

‘And now, a year after a bone marrow transplant and six months after a kidney transplant, it is so heartwarming to see that she has a good quality of life: going to the beach, singing, dancing and also going to school and being able to do things that normal children can do. doing.’

He said it was ‘exciting’ that Aditi was the first person in the NHS to undergo the procedure.

But he warned that the risks of a double transplant are much greater, so it is likely to only be considered for people with similar conditions, such as a compromised immune system.

Her father Uday, a 48-year-old chef, said, “Most of the support for the family comes from Aditi.

“She had to be on dialysis for six to eight hours a day and she would come home and still light up the whole house.”

WHAT IS SIOD?

Schimke immuno-osseous dysplasia (SIOD) is the rarest form of dwarfism in the world.

It affects every 1 million to 3 million people worldwide.

SIOD is passed on genetically through a recessive gene called SMARCAL1.

In people with this condition, short stature is caused by flattened spinal bones, resulting in a shortened neck and torso.

The disease leads to kidney failure, possible bone marrow transplants, hip transplants and death at age 11.

Signs of SIOD:

  • Inflammation of the kidneys with nephrotic syndrome (a kidney disease that causes your body to excrete too much protein in your urine)
  • Disproportionately small stature
  • Low numbers of T cells – a type of white blood cell

Common features:

  • Exaggerated curvature of the lower back
  • Dark spots on the skin (usually on the chest and back)
  • A wide nose bridge with a rounded nose tip.

Those with SIOD are at risk for kidney failure, kidney failure, heart failure, and lung disease.