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Please help the HI fund at GOSH 
13th-Jun-2011 03:56 pm
A six month old baby wearing a red shirt and blue dungarees, sitting and holding a toy pancreas. He is looking at the camera and smiling a happy, open smile. There is a tube coming out of his left nostril, taped to his cheek.

Charlie Loves His Pancreas!

Three weeks after I moved to New York, my sister gave birth to my first nephew – Charlie. Anyone who knows me knows how much I dote on his sister Abby, so my apparent silence on the subject for the months following Charlie’s birth hit a few alarm bells among my friends. Charlie was born with a rare form of Hyperinsulinism - HI – as his pancreas was producing dangerous levels of insulin, which not only kept his blood sugar levels through the floor, but left his with no hunger feeling to engage him to feed.  Within hours of his birth he was put on a glucose drip, that was the only thing keeping him alive. Within days, he was removed to Great Ormond Street Hospital for children in London, one of the few places in the world that includes specialisation for HI, and very fortunately within two hours travel from Charlie’s parents and grandparents. His condition is also known as hyperinsulinaemic hypoglycaemia  – HH.

A baby of about two months old, big for his age. He is lying cradled by a woman's arms and is looking off camera. He is wearing a nappy, but is otherwise naked except for a tangle of tubes that work around his feet and torso. He has a hospital name tag on his foot and bandages on his left hand and right side. There is a dummy in his mouth

Sugar and drugs. Just hook them up to my veins.

Over the course of the next few months, it was an urgent scramble to find the cause of his symptom, so that he could be treated as soon as possible; the first months are a critical stage in development of a number of functions, within which window a child could suffer serious long term effects, including hypoglycaemic brain damage. The sooner he could come out of hospital, the better chance Charlie stood of  becoming a normal child.

There are two predominant types of congenital hyperinsulinism: focal, which means that one specific part of the pancreas is playing up, and diffuse, which indicates the entire organ. If you have the misfortune to be born with HI, you want to hope it’s the focal type, as this can be treated  – even cured – by a partial pancreatectomy (going in and cutting bits off the pancreas). Diffuse HI, if it doesn’t respond to medical treatments, requires an entire pancreatectomy, and – well, anyone with diabetes could tell me how much it sucks not to produce enough insulin. Fortunately, the two types can usually be differentiated using a blood test.


Charlie’s blood tests were inconclusive.

A PET image of a cross section of a child's torso. It is mostly grey and purple, with an intense area of red and orange in the centre.

A PET image showing focal Hyperinsulism (the yellow area in the centre; the head of the pancreas) Taken from Kapoor et al, 2008

Fortunately, there are other ways to determine HI variety, and that’s by injecting the baby with a radio-isotope and running him through a PET scanner. The isotope in question is [18F]DOPA: an isotope of dihydroxyphenylalanine (DOPA), which the pancreas takes up and converts to dopamine. A PET scanner detects the presence of this isotope in the pancreas, and can identify localised areas of intensity, indicating focal HI.

It’s not a risky procedure, but the isotope itself has a pretty short half life, and the unit at GOSH did not have the isotope to hand. Charlie, still hooked up to a glucose drip as well as lined of countless drugs that were treating his condition, had to be flown out to Berlin, where they had both isotope and scanning facility. The news was good; it was a focal occurence caused by a lesion on his pancreas. This should be fixable using a pancreatectomy. So back he was flown and he was prepared for surgery.

You guys know that I have a phobia of people I love having surgery, right? 2010 was a hellish year for me. On the day of Charlies first (yes, first) operation, 5,000 miles away, I had to walk out of my class in order to have a good cry.

The surgery was  – well, not a failure, as it didn’t make anything worse, but it didn’t make anything better, despite a good lesion-y chunk being taken out. It looked like Charlie would need another scan. At this point, however, the uncle of my brother-in-law had heard of this scan and the trip to Germany. And “Hang on,” said he, “I use that isotope [or an equivalent] at my lab all the time.”

He didn’t say that; I’m paraphrasing. The point is, that GOSH were able to talk to the physics department of the university up the road and aquire a new source of [18F]DOPA, which meant the Charlie, and every British child that follows him, was able to have his second scan in London. That showed that the lesion the surgeon had previously removed was only a fraction of the size of Charlie’s actual lesion. A second surgery was required, and this one had outstanding results.

Charlie was born on 12 September, 2010. He came home on 17th December – two days before his sister’s second birthday, having spent the first three months of his life hooked up to a glucose drip. A week later, I met my nephew for the first time.

Two women - one a generation younger than the other, sit on a couch. The older woman has a white towel draped over one shoulder and a red towl on her lap, on which is seated a baby, wearing a red shirt under an orange bib. He has a tube coming out of his right nostril, taped to his cheek. The younger woman is wearing a cream sweater, black skirt and black and purple striped socks, and is holding a book open. Both women and the baby are looking at the open book, engaged in the act of reading it.

The second best thing to being bounced and walked is to be held and read to. The towels covering my mum are a precaution against vomiting.

Of course Charlie’s journey didn’t end there; having never been breastfed, he had none of the musculature and techniques learned from suckling, and suddenly he was going to have to learn to take food in through his mouth. This is a difficult thing to learn if you miss the first window, and he was fitted with a gastro-nasal tube, through which milk could be directed into his stomach from a syringe. He hated taking anything in through his mouth, and even with the syringe, he would repeatedly throw up everything he took in. Sure this was just a result of being drip-fed so long, it wasn’t until he was six months old that he was identified as lactose intolerance, along with other allergies. With those things cut out of his diet, he’s made leaps of improvement, and is due to have his tube out next week.

Charlie is a beautiful, cheerful, bouncing baby boy. He was extremely lucky to be close to a hospital that could deal with his condition, that he had a treatable version of HI, and that his parents and secondary caregivers have handle the stress so amazingly well that he’s on the way to a full recovery. He has no intention of crawling any time soon, but he’s not in a hurry; he likes sitting and cuddling and he loves sausages – his mum reckons he was just waiting until he could get meat, because milk is girl food. I know a few people who wouldn’t argue on that front.

But not all kids who are born with HI can be as lucky as Charlie. Some of the children he shared a ward with haven’t made it this far. Others have had to have a full pancreatectomy and will spend their entire lives dependent on external insulin sources. Congenital Hyperinsulin is a rare condition, but severe, and there is only one centre of research and treatment in the UK – Great Ormond Street Hospital.


GOSH literally saved my nephew’s life, and without the amazing staff at that hospital, I never would have been able to meet him.

To that end, his family are organising a Charity Toddle the day before his first birthday, in order to raise money for the Hyperinsulinism fund at GOSH. The chances are, that unless you live in the Windsor and Maidenhead area, you will be unable to attend, but please please please consider donating to his fund:

Sponsor Charlie on Just Giving.com

Every thing you can give is welcomed. HI is an under researched condition, but research is helping; the scan that saved Charlie is a recent procedure, and the quicker children can get treatments, the more we can reduce long term damage.

Thank you all so much for reading this far.

Bibliography for this Post

Hussain, Khalid. “Congenital Hyperinsulinism and Neonatal Diabetes Mellitus.” Reviews in Endocrine and Metabolic Disorders 11.3 (2010): 155-56. Print.
Kapoor, R. R., C. Gilbert, K. Mohnike, O. Blankenstein, F. Fuechtner, and K. Hussain. “Congenital Hyperinsulinism: [18F]DOPA PET/CT Scan of a Focal Lesion in the Head of the Pancreas.” Archives of Disease in Childhood – Fetal and Neonatal Edition 93.2 (2008): F166. Print.

This post can also be found at Thagomizer.net. Feel free to join in the conversation wherever you feel most comfortable.

13th-Jun-2011 08:09 pm (UTC)
What's the current exchange rate, more or less?
13th-Jun-2011 08:17 pm (UTC)
It's been floating around $1.63 to the pound for the past three months.
13th-Jun-2011 08:30 pm (UTC)

And we are making the donation in memory to Batya's mom, who was an early education teacher. She never met a kid she did not like. And she and we came to appreciate hospitals that go that extra mile for its patients and their families.
14th-Jun-2011 02:13 am (UTC)
Thank you so much, both of you.

I've been working alongside child life specialists recently, and the work that people do with kids in hospitals amazes me.
13th-Jun-2011 08:58 pm (UTC)
FYI: something is borked in the Thagomizer template, the post itself doesn't start until after the sidebar has finished, but the title is in the right place.

It's not just my tiny netbook either which was my first guess, it happens in Jennie's laptop too (both Windows & Fx)
14th-Jun-2011 02:17 am (UTC)
That's weird - I don't see it on my end. Lemme tweak for a while and I'll see what editing does to it.
14th-Jun-2011 10:27 am (UTC)
I see it both on my laptop (OSX, Firefox 3.6) and my iPhone (iOS 4.whatever, Safari).

13th-Jun-2011 08:59 pm (UTC)
That was quite hard reading - and I can only express my relief that little Charlie is now well and happy. I'm really glad that GOSH and your brother-in-law's uncle came through for him.
14th-Jun-2011 02:33 am (UTC)
Thank you.
13th-Jun-2011 09:03 pm (UTC)
I'm so glad that Charlie is now doing so well. I can't even begin to imagine how hard it must have been for you and your family.

I will be donating ASAP, Debi ♥
14th-Jun-2011 02:40 am (UTC)
Thank you so much, Lauren.

You were so supportive when he was sick, seriously. I don't think I could have got through that time without you.

Have I toild you recently how much you mean to me?
14th-Jun-2011 07:45 am (UTC)
*hugs* I'm glad I could be there for you :)
13th-Jun-2011 09:16 pm (UTC)
As a recent father, I'm amazed at Charlie's strength - and indeed, that smiling picture. He's an amazing baby, and he'll grow up to be an amazing kid.

14th-Jun-2011 02:48 am (UTC)
Thank you!

He IS amazing. And he has a terrific smile, as well :)
14th-Jun-2011 10:28 am (UTC)
Baby nephews: they are great. Aunts: same.
14th-Jun-2011 02:27 pm (UTC)
14th-Jun-2011 02:39 pm (UTC)
It's never easy seeing a young baby so ill - the sense of helplessness is near-overwhelming at times. It sounds like Charlie is fighting back hard and doing well.
14th-Jun-2011 02:42 pm (UTC)
He is the Bruce Wayne of babies.
15th-Jun-2011 03:17 pm (UTC)
<3 to you all - I'm so glad he's doing better.


And on a shallow note, what a gorgeous little boy! Those cheeks are ones you just want to squish...
15th-Jun-2011 04:22 pm (UTC)
Thank you! He has the aesthetic advantage of being an enormously heavy baby who is... not losing weight exactly, but losing fat faster than he's growing, which makes him extra cuddly.
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