Ellie and Tate's Story
I am Julia, mother of Ellie and Tate; we are from Essex, England.
Ellie was born in 1996 and has had two pancreatectomies, she is now an insulin-dependant diabetic and on pancreatic enzymes. Tate was born in 1999 and has had one pancreatectomy, he also is an insulin-dependant diabetic and on pancreatic enzymes.
Here is their story.....
Ellie was born full term weighing 8lbs 7oz, she seemed perfectly healthy. At home I was breast feeding, but I found I was unable to keep up with Ellie's demands - every two hours, day and night - so I started bottle-feeding as well as breast in an attempt to satisfy her appetite.
This went on for a few weeks and my husband, Darren, and I were starting to feel the strain of constant feeding, but whenever we spoke to a Health Visitor about the two-hourly feeds, they just advised us to try spacing the feeds out. Darren and I would leave the clinic laughing - space Ellie out, no chance! On two occasions, we found Ellie twitching in her cot and her eyes rolling, we thought this was wind and gave her colic drops, the twitching stopped.
Then one day I went shopping, Ellie screamed out from her pram, when I took her out she had gone floppy and was not responding. Thankfully a restaurant telephoned for an ambulance. In the hospital Ellie had several more fits (seizures) over a period of 2 days, before they realised it was her blood sugars.
It took another two weeks before they told us she had Nesidioblastosis and we were transferred to Great Ormond Street Hospital, Ellie was 10 weeks old. During the two-week wait at our local hospital, we were also told that Ellie had brain damage. We were absolutely devastated. On further investigation it was found that she had a subdural haemorrhage, they were unsure how she had got this and not sure how this will affect her, only time will tell i.e. reaching her milestones.
While in Great Ormond Street Hospital (GOSH), Ellie was put on diazoxide, this made little impact and they were unable to reduce the glucose IV or the continuous feeds of milk and maxijul. The decision had been made by all of us to have a 95% pancreatectomy (the only option left). We finally went home with Ellie, medication free. This only lasted for a month, steadily Ellie's blood sugars got lower and lower.
After several stays in GOSH to do true bloods and fasting studies, Ellie went in again to try diazoxide and this time with Nifidepine. It started off well but over 2 weeks the dose went up and the blood sugars went down. Ellie had to have another pancreatectomy; this time the whole lot was coming out. She finally came home at 8 months old an insulin-dependant diabetic on pancreatic enzyme supplements.
While in GOSH, we were told the likeliness of having another PHHI child, was thousands to one; they felt that Ellie was just sporadic. So 2 years later we went ahead and I became pregnant with our second child. During my pregnancy I started to have low blood sugars and got quite concerned. I contacted several people and all of them informed me it was probably nothing and my midwife told me to put the machine away, I was neurotic.
Towards the end of the pregnancy, I had to have regular scans (not for PHHI, but for Anti-D problems). At every scan the radiographer would comment on how big the baby was. I did not want to hear this, but still kept optimistic. Tate was induced 3 weeks early (because of Anti-D problems) and weighed 9lbs 13ozs. I knew straight away. Darren insisted on a blood sugar being done. Tate was 1.6 so I then fed him. 15 mins later his blood sugar was 0.6. He went straight to intensive care. He stayed there for 5 days before he was transferred to GOSH, until then our local hospital still did not believe he had PHHI.
While in GOSH, they put Tate on diazoxide, to no avail. They then added Nifidepine, nothing changed and finally they added Octreotide (Somatostatin) and glucagon, his blood sugars rose slightly but nothing significant. After three weeks, the only option left was a 95% pancreatectomy. When Tate came out of surgery we were told that his bile duct had been pierced and so he had to have this section cut out and his bile duct replumbed (Prof Spitz's wording). It was horrendous, watching Tate recover, he went very jaundice and it seemed they might have to go back in as the bile duct was obstructed. Thankfully over a couple of days, the swelling went down and he recovered well. He went home at five weeks old an insulin-dependant diabetic and on pancreatic enzymes.
Ellie and Tate are the first recorded case in the UK, of two PHHI children in a family.
Latest update: Latest update: Ellie is now 16 years old; she is on an insulin pump and takes Creon 25000 capsules with every meal. Her subdural haemorrhage dispersed and she reached all her milestones. She is now at college studying Business and looking forward to starting a career at Stansted Airport.
Tate is now 14 years old, and is also on an insulin pump and takes Creon 25000 with every meal. Tate was diagnosed with Aspergers Syndrome, when he was 7 years old. Although, we are now unsure of this diagnosis as his symptoms have lessened considerably. He is now studying for his GCSE’s.
Both are thriving and living life to the full.