Obituaries

James Edmond Wraith

BMJ 2013; 346 doi: http://dx.doi.org/10.1136/bmj.f3238 (Published 19 June 2013) Cite this as: BMJ 2013;346:f3238
  1. Anne Gulland, London
  1. annecgulland{at}yahoo.co.uk

Paediatrician whose research crucially contributed to treatments for lysosomal storage diseases

Ed Wraith chose a specialty that, at the time, some doctors might have seen as a career cul de sac. He trained as a general paediatrician but his career focus was on lysosomal storage diseases (LSDs), a group of progressive neurological conditions. At the start of his career these conditions were untreatable, with symptom control being the only option. His interest was prompted by wanting to improve the care of these patients with rare, life limiting, and disabling conditions. It was certainly not seen as an area where a young doctor could make their mark.

Wraith worked with a paediatrician at the Royal Manchester Children’s Hospital. He had an interest in metabolic diseases and started to investigate what could be done to help patients with these disorders. During his career he identified many major genetic mutations in storage diseases, including mucopolysaccharidosis (MPS) types. He undertook several natural history studies on the diseases, which led to better treatments and clinical classifications. He also wrote the NHS guidelines for management of MPSI, MPSII, and MPSVI; Niemann-Pick type C disease; infantile Pompe disease; and paediatric Gaucher disease.

Wraith became the UK expert on these diseases, and Brian Bigger, lead scientist for the stem cell and neurotherapies laboratory in Manchester, says that Wraith saw roughly half of the 1500 patients in the country. “Manchester does a huge amount of diagnostics, and that was largely driven by Ed,” says Bigger. Patients came to Manchester from all over the world for diagnosis, and Wraith travelled widely, lecturing and running clinics. From the beginning he developed close links with patient groups, and the MPS Society funded his first consultant post for two years.

Because of the large numbers of patients coming to the unit it was an obvious base for conducting clinical research, and Wraith led or was the coinvestigator on more than 20 clinical trials—notable as drug trials are so rare in paediatrics. These trials resulted in the development of enzyme replacement therapy (ERT) for many of the LSD disease types and a substrate reduction therapy drug for Niemann-Pick type C disease. Six licensed drugs came out of these clinical trials and new products were developed to treat MPSI, MPSII, and MPSIV; Fabry disease; and Pompe disease.

Bigger says Wraith had a real compassion for patients, and his work was driven by a desire to help children deemed untreatable. “First there was a treatment for Gaucher disease in 1994, and then along came ERT, and then more therapies. It must have made a huge difference to him, being able to see patients improve,” he says.

Wraith also had an interest in stem cell transfer as a treatment for LSDs, which led to a collaboration with the bone marrow transplantation group at Royal Manchester Children’s Hospital. Rob Wynn, consultant paediatric haematologist on the group, says his own interest in transplantation for metabolic diseases was driven entirely by Wraith.

He adds: “He [Wraith] was among the first to recognise the utility and role of transplants in these rare disorders, and through referral to transplant teams, support of those teams, and his pivotal role in fostering important collaborations between centres he helped to improve outcomes. Ed did not directly make the decisions in the unit and always left that to us, but he was always absolutely aware of where his patients were up to during the transplant process.”

Wraith was born in South Shields in Tyne and Wear to working class parents and won a place at the local grammar school. He trained at Sheffield University, worked for a few years in hospitals in the city, and then moved to Manchester. He took up a clinical research fellow post at the Murdoch Institute for Research into Birth Defects in Melbourne between 1986 and 1988.

He became the clinical director of the Willink Biochemical Genetics Unit at Royal Manchester Children’s Hospital in 1993 and insisted that it follow the Australian model with the merger of clinical and metabolic genetics. Bronwyn Kerr, a consultant clinical geneticist at the unit and close colleague, says this was important. “He believed firmly that this was the way forward as the work becomes synergistic. It gave people the chance to collaborate on understanding these diseases,” she says.

He pushed for LSDs to get specialist commissioning status and was the lead clinician on the national commissioning group from 2007.

Wraith had a long ponytail, rarely wore a suit, and was seen as something of a maverick. He was a lifelong Newcastle United supporter but was also very private and outside work was happy at home with his family and two lurchers.

He was also devoted to his patients, giving them his phone number and making himself available to them—perhaps too available sometimes, says Wynn. He even moved within walking distance of the hospital so he could be on hand in emergencies.

Patient group representatives speak of a doctor who spent his career giving bad news but to whom the families were also incredibly grateful. Toni Mathieson, executive director of the Niemann-Pick Disease Group, says: “He recognised that parents were very knowledgeable about the disease and that they knew what was best for their children. He always allowed you to ask questions.”

He travelled widely, and his expertise and knowledge were recognised around the world. When he was diagnosed with a rare degenerative disorder in 2009 he cut back on his travelling but retained his clinics and saw patients on the day he died.

Kerr says that he was a true advocate for people with storage diseases. “Not only did he pioneer new drug treatments he gave a voice to people with these diseases,” she says.

He leaves his wife, Sue, and three children.

Notes

Cite this as: BMJ 2013;346:f3238

Footnotes

  • James Edmond Wraith, consultant paediatrician (b 1953; q University of Sheffield 1977), died from a neurodegenerative disorder on 10 April 2013.

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