The first Australian child with hereditary pancreatitis to receive a total pancreatectomy with islet autotransplant back in mid-2015 is growing well, enjoying a normal life and free from the severe pain associated with the rare condition.
Professor Toby Coates, director of renal and islet transplantation at the Royal Adelaide Hospital, told the limbic the procedure was life changing for the child.
“He has strongly detectable C-peptides which means the islets are working remarkably well and his insulin requirements are very low.”
“His biggest issue is remembering to take his pancreatic enzymes,” Professor Coates said.
According to a case report in the journal Pancreas, the child was too ill to attend school regularly, unable to enjoy social and sporting activities and experienced pain that was barely managed with daily opioids before his procedure.
He received a total pancreatectomy, splenectomy, cholecystectomy, and biliary and enteric reconstruction at Adelaide’s Women’s and Children’s Hospital.
The severely fibrosed pancreas was flown to Melbourne for islet isolation at St Vincent’s Institute of Medical Research and the cells returned for infusion into his liver 11 hours later.
“A total pancreatectomy and islet autotransplant is a big procedure to undergo but giving back even a small amount of islet cells makes it easier to manage,” Professor Coates said.
The child’s insulin requirements via continuous subcutaneous insulin infusion have gradually reduced from a peak of 0.76 U/kg per day to 0.35 U/kg per day.
Glycaemic control has been good with HbA1c in the range of 5.6 – 6.3% within months of the procedure.
“Our primary indication is to treat pain, to get patients off opiates and to improve their quality of life. However for patients with the cationic trypsinogen defect we can also remove the risk of developing malignancy in their 30s or 40s.”
Professor Coates said ideally the procedure would be funded through the Nationally Funded Centres program, which already supports the islet allografts for patients with type 1 diabetes.
Meanwhile the team had sourced local funding to repeat the procedure in two teenagers and an adult within the next few months.
He said delays in referral, funding or due to interim procedures risked further damage to the pancreas and reduced the chances of good islet isolation.