A rocky start, but Harrison’s off home

A rocky start, but Harrison’s off home

Bellinda Kontominas Medical Reporter
August 4, 2007

A COUGH, a sneeze or the common cold could be enough to kill tiny Harrison Draper.

Signs outside the four-month-old’s hospital room ask for hands to be washed before entry. Gowns and masks must be worn and people are urged to stay away if they feel unwell.

Harrison was born with a rare form of the genetic disorder known as severe combined immunodeficiency (SCID), which affects one in 2 million people.

It is so rare that Harrison is the first person to be placed on an Australian register for adenosine deaminase deficiency, although it is thought there may be up to three cases.

The disorder is also known as the “bubble-boy” disease, after an American boy who lived for 12 years in a plastic, germ-free bubble.

Harrison does not live in a bubble, but his first venture outside this week, since falling ill, required a plastic rain cover over his pram to protect him from germs.

It is not all bad news for the little boy, whose mother, Tracey Brown, was able to take him home yesterday for the first time since he fell seriously ill 10 weeks ago.

” We’ve been waiting a long time for this day,” said Ms Brown, who has been sleeping next to Harrison on a fold-out bed while her husband looks after their three other children at the couple’s Central Coast home.

Harrison’s improvement is in part due to a bone marrow transplant he received three weeks ago from his two-year-old sister Ella.

The next few weeks will reveal whether the transplant has taken. If successful, signs of immunity should start to show within six months.

The family must take precautions at home, including setting up an isolation room. “If any of the other kids start to get sick they won’t be able to come home; they’ll have to stay at their grandparents’ or at a friend’s house,” Ms Brown said. “We won’t be able to have any visitors, especially children, and the house will need to be kept clean and sanitised.”

Harrison was diagnosed with the disorder at the age of two months, after falling ill with meningitis and then pneumonia. He suffered neurological complications and will have hearing and sight problems.

If his immunity improves enough, it is hoped he will be able to go to preschool and live a normal life.

linkback url: http://www.smh.com.au/news/national/a-rocky-start-but-harrisons-off-home/
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What is SCID?

Severe combined immunodeficiency disease is the most serious primary immunodeficiency disorder. The defining characteristic of SCID is the absence of T cells and, as a result, lack of B cell function as well. Unless these defects are corrected the child will die of opportunistic infections before their first or second birthday. SCID can be caused by several different genetic defects, most of which are hereditary. In the past, children with this disorder were kept in strict isolation, sometimes in a plastic isolator or "bubble". Bubbles are no longer used as a standard of care, but the name remains a part of the history of SCID.

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Disclaimer: News articles come and go on the internet. When you find an interesting article and link to it, the link is dead in six months and the article is gone. I've copied many news articles here. I've included the information on the writer and linked back to the original publication. My intent is never to take anyone else's work as my own. My intent is to keep a repository of all the articles I find to be interesting. Many of these articles bring hope and encouragement to a family with a SCID child. Families with this disease are so rare, and they need to see that they are not alone in this struggle to keep their child alive. I hope this page is helpful to anyone with a family member or friend with any form of SCID.

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