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Birth

Scarlett and DaddyScarlett was born in July 2012, 7 weeks early by emergency c-section as a result of complications with the pregnancy weighing 5lb 4oz – a good size for being premature. As a result of this she was immediately put into the Special Care Baby Unit (SCBU) at the hospital. One of the basic tests carried out in SCBU is the testing of blood glucose levels and it became apparent Scarlett’s bloods were not staying within the normal range. As a result she was given a combination of high calorie milk and IV dextrose utilising the umbilical cord.

After approximately a week or so of this it became apparent that there was a persistent problem with Scarlett having low blood sugars and as a result we had some diazoxide couriered to the hospital to begin treatment on it. The diazoxide had no effect at all and seemed to make Scarlett more unwell if anything and after 16 days in SCBU it was decided she required more specialist care and she was transferred to Birmingham Children’s Hospital.

Scarlett was put in the High Dependency Unit and it was immediately apparent that Scarlett was actually getting worse, and that the amounts of dextrose required now to keep her within a safe range were becoming higher. She was put on an emergency theatre list to have a central line inserted so that they could use higher strengths of dextrose. After 2 days and nights there we were told that Scarlett required more specialist treatment than could be provided in Birmingham and we were told that we would be moving to Great Ormond Street Hospital – one of two of the national specialist centres.

Great Ormond Street Hospital

Scarlett arrived on a Saturday lunchtime, the day of the Men’s Olympic Football Final at Wembley. We were put in a bed on Rainforest ward and couldn’t wait to meet the specialist HI team on Monday. Scarlett again began treatment on diaxoxide with no improvement. We had bloods taken for genetic testing and treatment was started using octreotide infusion and glucagon.

Scarlett was very frustrating in that she would show signs of improving whilst trying to wean her down off these medicines and dextrose but right at the end of the weaning cycle she would react and we would have to start all over again. This would be a cycle that would that repeat itself lots of times.

After 6 weeks the genetics came back inconclusive and so Scarlett was sent for a PET scan at UCLH which confirmed Scarlett had diffuse disease. Different combinations of medicine were tried again and the PET scans were repeated until we were told in early November that they had really exhausted all options and we should begin the process of ‘fattening up’ Scarlett for surgery to have her pancreas removed. Scarlett was given a date in mid-November for her surgery and we were left with the prospect of Scarlett losing her pancreas and a life of Type 1 diabetes and enzyme treatment.

The next time we saw the team we were asked totally out of the blue, if we would like to try a new drug called sirolimus – a drug they thought might help Scarlett. It was an opportunity we weren’t expecting and had no real idea what it was about or what it might do. Sirolimus is an oral medicine used principally in the use of kidney transplant patients and is an immuno-suppressant. Sirolimus takes a little while to build up in the system and after 2 weeks of this treatment Scarlett’s blood sugars began to both improve and stabilise – a miracle.

Scarlett was weaned off her dextrose and for the first time we were allowed to take Scarlett outside. The following week, her operation was cancelled and we were allowed to go home.

Home – present

Scarlett is doing well at home, she is nearly 3 years old and attends nurture nursery. She doesn’t seem to have suffered any developmental setbacks, due to her early diagnosis and excellent treatment. She is small for her age and unfortunately even though she retains her orality, suffers with food adversity so has had to have a gastrostomy fitted when she was about 17 months old. This has proven invaluable when she been unwell, for giving medication and also when she is refusing to eat, or in reality topping up what little food she eats. Occasionally she we will eat a reasonable amount which is reflected in higher blood sugar levels.

Because Scarlett takes sirolimus, an immuno-suppressant medicine, it is essential that she is regularly and closely monitored to ensure that she has the right level of medicine in her blood and also that her immunity is in good order. This requires regular blood tests by our community nurse team around every 4 weeks, or more frequently as necessary.

Scarlett has suffered infections like all other children which have resulted in hospital admissions but clearly can be worse as a result of having her immune system supressed by her treatment. She also takes a very low dose of octreotide which is injected 4 times a day which we have been trying to wean her off over a long period of time. Hopefully, as a result of keeping her pancreas we will have a better long term outcome for Scarlett and for other children who no longer need to have major surgery who have diffuse disease.

 

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The Children's Hyperinsulinism Charity
Registered Charity Number: 1165562

The CHC is set up by parents for parents

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