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Thursday, 5 April 2001

Emma is fighting fit – saving young children with immune deficiencies

Emma Prott-Buttel, from Putney was diagnosed with Severe Combined Immuno-Deficiency Syndrome (SCIDS) in 1996 at the age of six weeks. She received a Bone Marrow Transplant when she was three months old, from her mother, Penny.

Emma was closely monitored for the next four years to check her platelet counts and to keep her away from infection. Emma is now living life to the full and developing normally like any other child her age.

Specialists at Sydney Children's Hospital, Randwick have been treating children suffering from SCIDS using Bone Marrow Transplants since 1985. They are now able to observe the long-term benefits of this groundbreaking treatment and are seeing survival rates up to 80%.

SCIDS whilst rare is the most serious form of immune deficiency in young children and is usually diagnosed in early infancy. It is almost always an inherited disorder and occurs when the immune system of a baby fails to produce antibodies. That is, the numbers of T-cells in the baby's blood are very low and the ones that do exist do not work properly.

This means that babies with SCIDS are very prone to infection. If the condition is not corrected children do not usually survive beyond two years of age. A Bone Marrow Transplant is the only way to correct the T-cell disorder.

Genetic analysis has further enabled the Immunology team at the hospital to conduct more accurate diagnoses. This is particularly critical in the more rare forms of the disease simplifying considerably the management choices available.

'We are very pleased with the outcomes of our treatment regimes adopted at Sydney Children's Hospital, particularly when we see a child like Emma grow into a healthy little girl'.

'We have been using Bone Marrow Transplants to treat our young patients with SCID for some time now and are seeing successful outcomes over a prolonged period', said Prof. John Ziegler, Director of the Immunology Department.

For Further Information Contact:

Catharina Boer, Public Affairs Manager Tel: 9382 3571 - Mob:0411 730 842



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