Harrison – a story about SCID

HarrisonSevere Combined Immunodeficiency Disease (SCID) is a life-threatening condition which is present from birth. If undetected or unsuccessfully treated, SCID is fatal in 100% of cases, usually before a baby’s first birthday. I wasn’t aware of this condition until the loss of my beautiful nephew Harrison in 2010. Harrison’s mother, my sister Bianca Noble has bravely shared his story:

“Harrison was a happy, healthy little baby who loved his milk and gave out smiles to everyone that smiled at him. He had been born seven weeks premature, but was very strong and thrived as any healthy full term baby would.

At four and a half months of age, Harrison became sick with a cold. A couple of trips to the doctor resulted in antibiotics to clear a suspected infection. For a few days Harrison’s condition began to improve, then suddenly he deteriorated and was admitted into North Shore Hospital (in Sydney, Australia) with pneumonia and hepatitis.

Four days of treatment and Harrison’s condition failed to improve, his liver function was worsening. He was transferred to The Children’s Hospital at Westmead for specialist care and diagnosis.

After five days of continuing tests and consultation by a number of specialists, our family was informed that Harrison had Severe Combined Immune Deficiency, a lack of immune system resulting in him being defenceless against viruses and infections.

We were advised that the only hope for his survival was a bone marrow transplant and there was a chance that Harrison was too sick to make it to the transplant. He was found not only to have pneumonia caused by a virus and a fungus, but also widespread infection caused by a virus called EBV. This is the same virus that can cause glandular fever, but was causing Harrison to be very ill with hepatitis.

Harrison’s condition continued to deteriorate, and two days later he was transferred to Intensive Care where upon arrival he had a massive lung haemorrhage and stopped breathing. He was placed on a ventilator and our family were told that due to the extensive damage to multiple organs caused by the infections, we had to say goodbye to our darling baby. Only two weeks after being admitted to Hospital, and only two days shy of being 6 months old, little Harrison died.

The grief from this loss is heartbreaking, particularly in such preventable circumstances. We have to live with the knowledge that a Newborn Screening test could have detected Harrison’s condition at birth and allowed him to access treatment that could have saved his life”.

PLEASE SIGN THE LETTER OF SUPPORT asking for the inclusion of SCID in Newborn Screening programs in Australia.

Support newborn screening for Severe Combined Immune Deficiency (SCID)

We ask that you share this post in honour of Harrison, to raise awareness of SCID and most importantly – to get as many people as possible to sign the Letter of Support for SCID Newborn Screening in Australia.

Many states in America are now universally screening their babies following birth. Please get on board so we can do better for our Australian babies and their families.

Want to know more?
More commonly known as the disease of the “Bubble Boy”, SCID is the result of a genetic condition which sees sufferers born with an undeveloped immune system. Babies with SCID are left defenseless against the broad spectrum of viruses, fungi and bacteria that would be virtually harmless to people with normal immunity. Many babies with SCID have no family history of the condition.

EVERY child with SCID will die unless the condition is successfully treated; successful treatment depends on early detection. Given that babies with SCID appear healthy at birth due to a brief window of coverage from their mother’s antibodies (as well as the common delay in diagnosis) many children succumb to infection before treatment can be provided. Newborn screening provides an opportunity to detect SCID early in a baby’s life while the child still has protective antibodies and prior to irreversible, major exposure to infections.
As babies with SCID usually present with common pediatric conditions requiring diagnostic workup, Newborn Screening for SCID would ensure treatment is initiated earlier, minimising risk of exposure as well as limiting unnecessary and invasive testing.

Medical developments mean it is possible to effectively treat SCID, which may provide the sufferer with the opportunity to lead a full life. A Bone Marrow Transplant (BMT) has the potential to provide positive long-term outcomes. If early diagnosis is achieved, BMT within the first 3.5 months of life results in a 95% survival rate! Positive outcomes however are dependent on early detection and successful uptake of treatment before babies are harmed and overcome by opportunistic infections. Hence, the imperative for Newborn Screening.

Medical professionals can do a free online training package to learn more, at:
http://www.allergy.org.au/health-professionals/health-professionals-e-training

Harrison

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