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- Screening Matters, Issue 60, December 2017
- Severe combined immune deficiency disorder now included in newborn screening
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Screening Matters
The National Screening Unit newsletter
In this issue:
- Benefits of the Universal Newborn Hearing Screening and Early Intervention Programme (UNHSEIP)
- Severe combined immune deficiency disorder now included in newborn screening
- Positive start to bowel screening in Hutt Valley and Wairarapa
- Sharing data to improve BreastScreen Aotearoa participation and attendance
- Profile of Mana Wāhine Day in Lower Hutt
Severe combined immune deficiency disorder now included in newborn screening
Severe combined immune deficiency is a rare inherited disorder caused by a deficiency or absence of T cells, a type of lymphocyte that plays a central role in immunity. This results in babies having little or no immune response. Babies born with SCID appear healthy at first, but are extremely vulnerable to recurrent infections.
The symptoms of SCID include recurrent life-threatening infections, failure to thrive, diarrhoea and thrush. The average age of diagnosis is approximately six months. If not treated, most babies with SCID do not survive past one year of age.
The incidence of SCID is estimated to be 1 in 50,000 to 1 in 60,000 live births. So only about one baby a year in New Zealand is expected to be identified with the classic form of SCID. However, the severity of the condition and the impact on families and health services is considerable.
When the disorder is found through screening, rather than after infections have occurred, babies are significantly more likely to survive and have better health outcomes.
The National Screening Unit has been working with Auckland District Health Board LabPlus and Lead Maternity Carers (LMC) in the lead-up to the new test and has developed some fact sheets about SCID to support health professionals in their conversations with patients.
If a baby is suspected of having SCID, the LMC will be contacted by the immunology specialists at Starship Children’s Hospital to discuss the test results as soon as they are back. The baby should not be given the rotavirus vaccine until their immune system has been further tested.
For more information about SCID, visit this page on the NSU website.
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