Seven year old Shaemus has a rare condition called Jeune’s syndrome, also known as asphyxiating thoracic dystrophy. Children born with Jeune’s syndrome have small limbs and a small chest and ribcage and can have difficulty breathing as a result. It can also affect other parts of the body including the kidney.
Shaemus has stage 5 chronic kidney disease Chronic kidney disease (CKD ) has five stages. In the earlier stages (Stages 1–3), your kidneys provide between 30-100% of normal function and you may not experience any clinical symptoms. In the later stages (Stages 4–5), you will have more clinical symproms such as tiredness, poor appetite, nausea ,bone pain and anaemia and you are likely to need some form of kidney replacement therapy such as dialysis or a kidney transplant.
Shaemus developed stage 5 kidney disease and needed regular dialysis. There are two kinds of dialysis. In hemodialysis, you are connected to a machine through tubes and your blood is pumped out of your body to an artificial kidney machine and returned to your body once filtered. However, in peritoneal dialysis, a thin tube is used to fill your belly with a cleaning fluid called dialysate. The fluid stays in your body for a few hours while it cleans your blood before it is drained.
For the first four years, the family were able to do peritoneal dialysis ovenight at home which could take up to 13 hours. However, Shaemus then had to move to haemodialysis and has been travelling to Alder Hey from his home in Lancashire, up to seven times a week for 10 months.
“At each stage of CKD our goal is to slow down the damage to your kidneys and keep your kidneys working as long as possible. Although dialysis is an incredible life-saving method it can only perform up to 10 % of kidney function and therefore children may often feel unwell and have restrictions to what they can eat and how much they can drink. It is also not a permanent solution and can be very time consuming for children and their families. Although a kidney transplant needs to be well looked after with regular medical check ups and medication children feel much better, can attend school more regularly and eat and drink more freely. . We’ve been busy preparing Shaemus for a new kidney, we had to wait until he was big enough and strong enough.”
Consultant Paediatric Nephrologist Dr Caroline Jones
Mum Sarah and dad Kieran were tested to see if their kidney’s were compatible with Shaemus and thankfully Kieran was a perfect match. The day of the operation came on 11th January 2024 and Shaemus and family travelled to Manchester Children’s Hospital for the transplant. Since then, Shaemus has come back to Alder Hey for his after care and him and dad have been recovering well.
“It’s so peculiar not to be at Alder Hey every day. We’re hoping Shaemus can go back to school soon. The renal team have been amazing, without them and Caroline, we don’t think he’d be with us today. Shaemus’ favourite person is his little sister Josephine who has just turned 1, they’re best friends. We can’t wait to spend more time together at home as a family. We’re so grateful Kieran was a match, this isn’t always the case for a lot of families and this is why registering for organ donation is so important. ”
Sarah, Shaemus’ mum
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