Look out for Repeat Offenders
Well done everyone, they’ve all been found!
The 22nd - 29th of April was World Primary Immunodeficiency Week and we were on a huge collective mission to raise awareness of this issue in the hope that children can get a diagnosis of this life threatening disorder much earlier.
Our patients at the Great North Children’s Hospital know that a repeated infection is a big warning sign of a primary immunodeficiency. That’s why they have set out to draw you the repeat offenders that have attacked their very own immune systems to shine a light on PI and help everyone be aware of the warning signs to look out for.
Oscars repeat offender
— FOUND by Natalie in the Tyne & Wear Metro newspaper –
Only by finding the Repeat Offenders early, Oscar’s doctors were able to diagnose him with the kingpin behind all these attacks, Wiskott Aldrich Syndrome.
“Oscar was diagnosed at 14 months with a rare blood and immune condition.
The warning signs that showed things weren’t quite right with Oscar were repeated infections which became more serious requiring frequent hospital stays, blood in his stools and inflamed eczema.”
Alfie’s repeat offender
This repeat offender was drawn by Alfie, it gave him bronchitis, and this warning sign led doctors to find the real criminal, severe combined immunodeficiency.
“Alfie from York became ill when he was only three months old and was in and out of his local hospital. The doctors said he had bronchitis and had to stay on the ward. At one point he was so ill that he was admitted to the high dependency unit. It was only when he continued to get worse, didn’t gain weight and was depended on oxygen 24/7 that tests finally revealed an underlying problem. Alfie was diagnosed at five months with Severe Combined Immunodeficiency (SCID) and it was a race against time to find a bone marrow donor to save his life. It was very fortunate for Alfie that his sister was a match and he very luckily made a full recovery thanks to the great team in Newcastle’s Great North Children’s Hospital Due to SCID being a rare genetic disorder, local doctors in York were not fully aware of the warning signs to look out for. But now, because of Alfie’s case, doctors have diagnosed other children’s immunity problems a lot more efficiently now they are aware of the seriousness of immuno compromised children. Alfie’s family are so grateful that he is still here and in turn has helped others.”
Kieon’s repeat offender
This repeat offender has been located by Lauren Ainsley hiding on the The Newcastle upon Tyne Hospitals NHS Foundation Trust facebook page.
Kieon from Seaham Sunderland Aged 10 Started Ig therapy Aged 3 Diagnosed with X Linked Agammaglobulinaemia Following repeated chest infections
Seth’s repeat offender
This repeat offender gave patient Seth a repeated infection. It led doctors to find the real culprit, severe combined immunodeficiency.
Lily’s repeat offender
Lily Pickering From Middlesbrough Aged 8 Started Ig therapy when 4 diagnosed with hypogammaglobulinaemia, specific antibody disorder and suspected ataxia telangiectasia following repeated chest infections this was confirmed later that year.
Oscar M’s repeat offender
This repeat offender has been found by Sophie in Newcastle in the Tyne & Wear metro. It belongs to Oscar from N.Ireland and it gave him a serious pneumonia.
Oscar was born in 2011 and for the first seven months of his life he was a typical baby. He was a healthy boy with only the usual minor ailments that babies endure from time to time but nothing that would suggest that he had any underlying condition.
Unfortunately things changed dramatically for the worst on 19th December 2011. It was my first day back to work and I got a phone call from Pearse’s mother to say Oscar was sick. I thought nothing of it and said for my mother in law to call back if he got worse. She called again worried but I thought she was overreacting.
To put her mind at ease, I got an appointment with the GP and Oscar was seen that morning. I got a phonecall from my mother in law to say that he was very dehydrated and the GP had recommended to take him to the local hospital (Causeway Hospital, Coleraine).
I left work at that stage unconcerned. (I’ve worked in children’s wards and know that dehydration can be solved within a day or so.)
However, when we arrived into hospital I was met by a paediatrician I knew as she was on her way home. She said she’s have a quick look. When she lifted his vest she advised us he had a serious pneumonia – without laying a hand on him…she could tell by his breathing. Again, I thought a course of antibiotics and he’ll be perkier in the morning.
However, through the night he started to require Oxygen and the antibiotics weren’t working. He seemed to perk up on the Tuesday but deteriorated on the Tuesday evening. On Wednesday morning we got the news that he was being transferred to the regional hospital in Belfast. He was intubated and transferred to Paediatric ICU, Royal Belfast Hospital for Sick Children. He was ventilated and on the main PICU ward for 2 days before they made the diagnosis of SCID.
This came as a huge shock. We were shown to his room and had to wear masks, gloves, hairnets and gowns. We were told to go home and pack a bag as we were to be transferred to Newcastle and would be there for a number of months. This was on Friday 21st December. Oscar spent his first Christmas in PICU RBHSC. He was critically ill and at one point they brought in a nitrogen oxide tank as 100% oxygen was not working for him.
He made some progress over the next few days and was subsequently was stable enough to airlifted to PICU, Great north childrens Hosptial on 28th Dec. He spent a further 10 days in PICU before being admitted the Bubble Foundation UK Unit. We had been advised that had Oscar not been diagnosed so quickly it was likely that he would have died.
As I highlighted above we had no idea that Oscar was suffering from any illness other than being lethargic in the weeks before he was admitted to hospital. You may also be wondering why it took so long for the effects of SCID to take hold. The reason for this was that Oscar was breastfed.
We were advised by the medical staff in Newcastle that as Oscar was being breastfed my breast milk was substituting for his practically non-existent immune system. It wasn’t until I stopped breastfeeding Oscar on Thursday 15th December, in preparation for my return to work that the effects started to materialise.
We had also been advised by the medical staff that as Oscar had virtually no immune system the pneumonia could have been affecting him for months previously. Unable to fight the pneumonia the mucus slowly accumulated adhering rigidly to his lungs. As his natural defence did not exist he never coughed up any of this mucus. It was also so densely compacted that it was extremely hard to hear through a stethoscope. Hence the reason why it took so long to be diagnosed.
Oscar spent a total of 19 days in intensive care but his breathing had to be aided by oxygen for approximately another month. He eventually recovered fully from the pneumonia although it has scarred and damaged his lungs and he continues to be on medication.
We spent five and a half months in the Bubble Unit in Newcastle. We were supported by very experienced staff and everyone we met were extremely helpful. It’s hard to explain but we have fond memories of Newcastle and the families and staff we met. We consider them our family and always enjoy returning to see them.
Oscar went on to have a successful transplant and is now full of the joys of life. He also has Lymphoedema in both legs, and attends St George’s hospital in London. Since he started attending there, there have been 4 more children with SCID and Lymphoedema referred to St George’s so they know there is a link but just can’t seem to find it.
Oscar thankfully has no repeated infections now. To me, his transplant does not cause him any difficulties and it is his lymphoedema which affects him more. Generally agility and fatigue would be the main effects however, we can cope with this.
Pearse and Olivia, Parents of Oscar M, SCID Baby, Bone Marrow Transplant patient at The Great North Children’s Hospital
For more information on PI please visit the Jeffrey Modell Foundation at http://www.info4pi.org