Why Jack is looking forward to Christmas

By Liz HearnshawLIKE any toddler, little Jack Medler enjoys nothing more than playing with his toys in the sunshine on a bright summer's day.But the brave two-and-a-half-year-old has never celebrated his birthday with a big party surrounded by friends and is unable to use public transplant, join a nursery school or eat out at cafes and restaurants.

By Liz Hearnshaw

LIKE any toddler, little Jack Medler enjoys nothing more than playing with his toys in the sunshine on a bright summer's day.

But the brave two-and-a-half-year-old has never celebrated his birthday with a big party surrounded by friends and is unable to use public transplant, join a nursery school or eat out at cafes and restaurants.

For Jack was born with a rare and life-threatening disorder of the immune system that leaves him prone to infection – and, before penicillin was discovered, would have shortened his life-expectancy to just three years.

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Now, following an anxious search for a bone marrow donor and nine months after undergoing a transplant, the future for Jack and his family is looking much brighter and the Medlers are hoping they will receive the all-clear by Christmas, paving the way for celebrations like none other.

Doctors at the West Suffolk Hospital, Bury St Edmunds, first noticed something was wrong with the youngster when he was just three days old.

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Despite his mother, Jenny, enjoying a problem-free pregnancy and birth, her first child developed blood spots on his forehead and four months later, following a series of tests, he was diagnosed with Wiskott-Aldrich Syndrome.

The genetic illness, which only affects one in every two million boys, is a primary immunodeficiency caused by a missing protein. It affects the blood, while leaving sufferers open to infection, and can only be cured by a bone marrow transplant.

Mrs Medler, who is 37 and lives in Brent Eleigh, near Lavenham, with her 34-year-old husband, David, said: "Nobody really knew what was wrong with Jack at first, and although the diagnosis was not the best news, to some extent there was relief in knowing what was wrong.

"Things were difficult as he was such a tiny baby and, of course, he could not tell us how he was feeling or what was wrong.

"During his first year, Jack was admitted to hospital seven or eight times, mostly with infections or viruses which he just did not have the immune system to fight off."

Jack's condition was monitored at the Great Ormond Street Hospital in London, while his concerned parents met the transplant team to gain all the information possible about the forthcoming procedure.

As neither Mr nor Mrs Medler proved a match for their son, the anxious search for a donor then began, with the family receiving positive news in January 2003.

"It was unfortunate when we were told Jack would need a transplant, but it gave us something to aim for," said Mrs Medler. "We knew it was going to be very difficult, with risks involved, but it was his best chance of having a happy and healthy adult life.

"We were very, very lucky to have got a good match and it did not take long to find someone. We spent an anxious two months waiting for the phone to ring, but were overjoyed when we heard the news. It was very emotional."

The couple then endured a nervous year-long wait until the transplant was carried out shortly after Jack had turned two years old in January this year.

Doctors advised that was the best course of action to take and would give the youngster the optimum chance of the procedure working.

Mrs Medler said: "The waiting was hard as we were living month by month until the hospital had the bed. We were keyed up to go in on a rough date and it just got pushed back and pushed back.

"We wanted Jack to be in the best of health when he had the transplant, but all that time we were worried he may get poorly again."

When the day earmarked for the procedure came, Jack had a Hickman Line inserted to allow the transplant – which is carried out in a similar way to a blood transfusion – to run as smoothly as possible, while doctors were able to administer drugs to his tiny body without him feeling any pain.

The youngster also underwent a course of chemotherapy and was then kept in special sterile conditions for about six-and-a-half weeks to ensure he did not contract any infections, while his parents were able to take turns to stay with him overnight.

"It was not nice for him at all, but the doctors were very skilled at controlling the discomfort and pain as much as possible," said Mrs Medler.

"It was a very intense period of time for us, but Jack was at a good age to undergo the procedure because he was too young to really understand, but was nevertheless quite accepting."

Unfortunately, Jack picked up a stomach bug shortly after he was discharged and then contracted Graft Versus Host Disease, which saw his own bone marrow began fighting that that had been donated.

The levels of donor marrow in his system fell to a dangerously low level and doctors feared they may have to carry out a second transplant.

But intravenous treatment with antibodies bought Jack's body time to adjust and the concentration of donor marrow increased.

Mrs Medler said: "When they were talking about possibly having to carry out a second transplant in another year's time, we felt pretty low. To have to put him through that again, with all the risks involved, was not a nice thought.

"But the antibodies seemed to be working, so he was allowed home this May. We have visited the hospital since, but were told on Wednesday that we do not have to go back for another month."

She added: "This is the longest period of time we have had without a visit to the hospital and a real bonus and a positive sign.

"Jack is not out of the woods yet, but things are looking much better. He is so much happier in himself and has much more energy to play with his toys."

The youngster must still adhere to strict rules and sticks to a special diet that means he can only eat fresh foods, cannot eat out and all the water he drinks must be boiled first.

He cannot go into shops or see his friends often, in case he picks up a cold or infection, but can play in the park where there is fresh air and open space.

"We have not been given the all clear yet, but the restrictions should be lifted when the doctors say Jack's immune system can cope with minor infections, which we hope will be by Christmas," added Mrs Medler.

"We will be able to give him a birthday party for the first time and do all the sorts of things we could not do before. He will love going on buses and trains and doing all the simple things we take for granted."

Jack is still on a cocktail of drugs and must take penicillin for the next two years to fight off illnesses such as pneumonia. However, it is hoped he will go on to make a full recovery and will soon begin to enjoy the same kind of lifestyle as every other toddler.

"Jack has coped very well and a couple of years down the line, hopefully, we will have forgotten about all the ins and outs of this and he not will remember at all," added Mrs Medler.

"The illness has put us under a lot of stress, but the support we have had from family and friends has been brilliant.

"All of the medical staff at both Great Ormond Street Hospital and West Suffolk Hospital have been excellent, and the children's community nursing team have been phenomenal, coming to look after Jack so I can have some time to go off and do a few things.

"And the Primary Immunodeficiency Association have been very, very good and introduced us to other people who had been through the same thing, which allowed us to talk things through."

She continued: "Obviously the person who made this all possible is the anonymous donor who has given Jack such a huge gift of a normal and healthy life. No words can ever convey our gratitude to them.

"We also want to thank all the local people in the village and in Lavenham, who do not know us, but have been saying prayers for Jack in the local church.

"We now know not to take our health for granted. You have to enjoy things while you are well and make the most of life."

Mrs Medler encouraged the public to support Jeans for Genes day on October 1, which raises funds for research into genetic illnesses in children.

n Great Ormond Street Hospital welcomes more than 90,000 patients a year. Any donations made to Great Ormond Street Hospital Children's Charity have a profound effect on the care and treatment it can provide to seriously ill children, topping up the NHS funding.

To make a donation to the charity, contact 0207 916 5678, send a cheque or postal order payable to Great Ormond Street Hospital Children's Charity to GOSHCC, Freepost LON20107, London WCIN 3AJ or log on to www.gosh.org/index.html.

For more information about the Primary Immunodeficiency Association, contact 0207 976 7640.


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