London-based hospital gives new lease of life to Bahraini girl suffering from rare condition
A young girl from Bahrain has been given a new lease of life after receiving treatment from Great Ormond Street Hospital in London (GOSH) for hyperinsulinaemic hypoglycaemia (HH).
Sarah was born with a congenital complex overgrowth syndrome called Beckwith-Wiedemann syndrome, which occurs in about 1 in 15,000 births. The most common symptoms are overgrowth, asymmetry of growth, large tongue, low blood glucose and abdominal wall defects. Sarah had severe HH, which is one of the known associations of this syndrome.
HH is a very complex condition that causes severe hypoglycaemia (low blood sugar) in the neonatal, infancy and childhood period. To treat her condition, Sarah was transferred from Bahrain to GOSH in London, which treats 1,500 children from the Middle East every year.
Dr Pratik Shah, Consultant Paediatric Endocrinologist at GOSH, explains, “Glucose (sugar) levels in your body need to be steady so that the brain can function well, as it needs sugar for it to run smoothly, just like a car needing petrol. The pancreas is a small organ in the abdomen that produces various hormones like insulin, glucagon, somatostatin which keeps your blood glucose level steady. In some children, the pancreas produces too much insulin that causes low blood glucose levels. This is called hyperinsulinaemic hypoglycaemia.”
When HH was confirmed by doctors at GOSH, Sarah was initially given treatment to stop insulin production. However, Sarah was not responding to medical therapy. After undergoing a scan, doctors noticed a very unusual large pancreatic cyst on her abdomen and pancreas, initially thought to be a tumour on Sarah’s pancreas.
Sarah then went on to have more detailed scan of the pancreas providing very detailed, three-dimensional images of the body.
Explaining the results, Dr Shah said, “Sarah’s scan confirmed she had a large cystic lesion in the head of the pancreas. Sarah had a severe form of hyperinsulinism and after having multidisciplinary team meeting involving a surgeon, radiologist, oncologist, and myself, we recommended undergoing surgery to remove the lesion in the head of the pancreas. Her hypoglycaemia became much milder after the major reconstructive pancreatic surgery, and can now be managed with feeds and medications.”
Professor De Coppi who led the surgery adds, “Sarah had a very rare lesion in a potentially very dangerous area of the pancreas, where the intestine, the liver, the pancreas and their vascular supplies all connect. It was a very challenging surgery but we are very pleased she has recovered well from the procedures. It is thanks to all the expertise present at GOSH that we can embark in such challenging procedures”.
Sarah stayed at GOSH for one year and three months to treat issues resulting from the severity of having hypoglycaemia during her first years. She required a multidisciplinary team at GOSH, who collaborated with specialists at other hospitals.
“Sarah has been discharged and is currently stable from a blood glucose point of view. She is now home in Bahrain and is doing well with regular oral/ gastrostomy feeds. Sarah’s progress will continue to be monitored by GOSH in order to screen other issues stemming from her overgrowth syndrome; her next review is in 6 months’ time. There is still a long way to go but the future is now looking brighter for Sarah and her family,” concludes Dr Shah.
Dr Pratik Shah
With over 15 years of experience in Paediatrics, Dr Shah has published numerous articles in the field of Congenital Hyperinsulinism and presented in international and national meetings. He also works very closely with parent support groups (both in the UK and international) to promote awareness and education other professionals about this condition. Dr Shah also has a research team in UCL Great Ormond Street Institute of Child Health who aim to deliver high quality research in the area.