James's Stewardson's Story

Born 29th May 1993 - Passed Away 8:20pm 12th November 1999

James with Dad and Sister Georgia, Xmas 1998
James with Dad and Sister Gerogia, Xmas 1998

When our son James was born, all seemed normal for the first few hours of his life. But 9 hours later he was rushed into intensive care for a full blood transfusion because he was severely jaundiced. A few days later he had recovered, and 7 days after he was born we took James home. Despite various investigations, the reason for James's jaundice was never found.

James seemed to progress normally until he became ill at 10 months of age and he was rushed into hospital. Over the space of the next few days James went from being a normal strong healthy baby, to a very ill, weak and floppy baby, unable to eat or drink. After spending 2 weeks in various hospitals undergoing tests, the Doctors did not know what was wrong with James, but suspected it could be a very rare metabolic disorder and this could only be confirmed by special tests at Kings College Hospital in London. We decided to take James home at this stage and wait for the results.

On April 27th 1994 we were told James suffered from a incredibly rare Metabolic Disorder, Triose Phosphate Isomerase Deficiency (TPI) an enzyme deficiency. James was only the 28th such case recorded worldwide, all of which died before the age of 6 years. We were offered no hope unless a treatment or cure could be found within the next few years. James's condition at this stage was very poorly and over the next few months he was in and out of hospital.

The Registered Charity for James was formed in July 1994 by James's Mum and Dad, and Uncle Peter. We very quickly decided that we could not sit around and watch our son die, and we were determined to find a team somewhere in the world to work on finding a treatment or cure. That team turned out to be in London, at Kings College Hospital and headed by Professor Bellingham, they were already working on TPI and drafted a proposal costing approximately 214,000 for a 3 year project. Their proposal was independently vetted by a eminent colleague, who was provided by the Research Trust for Metabolic Diseases in Children (RTMDC), after which we accepted.

Friends, neighbours, relatives, work colleagues and many many people who heard about James became involved in raising funds and to date almost 350,000 has been raised.

After James became ill, aged 10 months, the following 2 years were very difficult with James spending occasional short stays in hospital and him constantly refusing to eat or drink properly.

More about James's medical history

From the age of about 3 James started to eat and drink better and put on weight for the first time in 2 years. From here he got stronger and bigger and although we would have setbacks every 3 or 4 months James really did start to improve in several areas.

Life became a little better for us all and we were able to go on foreign holidays with James and enjoy his favourite passion of theme parks. No ride was too scary for James, although this was probably helped by his poor eyesight, and he spent his summers at places like Alton Towers and Disney Paris and Florida.

During this time the research teams were working on various treatments for James and we would spend time at Kings College Hospital in London.

The best time of James's life was when sister Georgia was born on October 12th 1996, James was so proud of his little sister, seen below with mum

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and Georgia was truly a miracle baby as she was a perfect bone marrow match for James. It was hoped Georgia would be a match and the umbilical cord was stored to be used at a later date for a cord blood transplant for James.

At this time the medical community was divided on whether a cord or bone marrow transplant would help James. This and the fact James was much improved meant we held back and the next 2 1/2 years were probably the best of his life, with many holidays including Disney in Paris and Florida twice.

When James started to deteriorate in June 1999, we asked about a cord or bone marrow transplant, but by this time he was on oxygen assistance and it was felt he would not be strong enough. We started a programme of red cell transfusions with the aim of getting James off the oxygen and strong enough for the transplant. Tragically this never happened.

By far the best part of James's life was his 2 years at Etchells Mainstream school. We were quite worried about sending James to a mainstream school, but because he was very intelligent we thought he deserved the chance to be with normal children.

This was the best decision we ever made for James and the love we felt for James from all at Etchells School was overwhelming at times.

We will never forget James's friends from Etchells School and be forever in their debt.

We always knew that at some stage James's respiratory systems would start to deteriorate and in June 1999 his breathing got to the point that he needed oxygen assistance during his sleeping hours. This initially made James much better and he had a good few months before things worsened and he started to retain CO2.

At this point we knew James would require a BiPap machine to help blow off the harmful CO2 gasses, carbon monoxide, he was reating in his body. We assumed James would take to this treatment without problems, BiPap had kept Ashley Smith in the USA alive for 3 years.

Devastatingly James could not put up with the Bipap machine and he deteriorated very quickly during the early days of November and passed away aged 6 years and 5 months on November 12th 1999 at home with family and relatives around him.

Considering his physical condition after he became ill at 10 months of age James did incredibly well to live a full life as long as he did. TPI affected his motor system and muscles, which made him very weak, and was never able to walk without support. His mental abilities though were as sharp as any boy of his age, and he could be just as cheeky.

As previously stated, the last 2 years of his life were probably his happiest because he attended Etchells Primary School, a mainstream school which caters for special needs children.

James loved every minute at Etchells where the staff and children absolutely loved him dearly. We his parents are so very grateful to Etchells for some very special memories of James.

He was a wonderful child with a terrific personality and his smile touched thousands of hearts, James will be so sadly missed by all who came into contact with him!

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James in his Etchells uniform.

For the story of James's medical history James Stewardson <==== click here