A dad has told of how his gravely-ill little girl must be fed for THIRTEEN hours a day – or she could slip into a coma and die.
Three-year-old Tessa-Mai Deeprose has to be fed even when she is not hungry.
She was born with a condition called glycogen storage disease type 1b, meaning she is missing an enzyme that helps the body maintain normal blood sugar levels .
As such, her parents James, 39 and Julia, 30 must feed her every 90 minutes.
She is also machine fed for 13 hours each night to stop her from falling into a potentially fatal coma.
“We’d never heard of the condition before Tessa-Mai was diagnosed. It’s incredibly rare. She’s only the sixth child in the UK to have her type since the 1970s,” said hospice maintenance worker James of Hastings in East Sussex.
“We have to feed her even when she’s not hungry. It’s difficult for us to explain to her that we’re doing this for her own good.
“She could end up in a coma or even die if her blood sugar dropped too low.”
Throughout her pregnancy, Julia said she didn’t “feel right.”
But, as she had suffered with pre-eclampsia whilst expecting her first child Ella-Marie, now eight, doctors believed it was simply that happening again.
Then, when Tessa-Mai was born via emergency caesarean at Conquest Hospital in St Leonards in May 2013, routine tests revealed that her blood sugar levels were dangerously low.
“The reading showed her blood sugar was 0.1. A normal level is around 4.0,” said James.
“It was so low that doctors thought the machine was broken. They tried again but it was the same reading.
“From there, she was taken straight to the special care baby unit.”
After around two weeks in the unit, where doctors checked her blood pressure every hour, Tessa-Mai was transferred to King’s College Hospital in south London.
Despite running a string of tests, medics there could still not work out why the newborn’s blood sugar levels kept plummeting.
The official diagnosis did not come until a few weeks later, when Evelina Children’s Hospital in central London ran a genetic test and realised she had glycogen storage disease type 1b.
With no known cure, the condition interferes with the way the body turns food into energy.
Sufferers are at risk of seizures and hypoglycaemic shock if they do not eat regularly.
It can also weaken the immune system and lead to the development of chronic inflammatory bowel disease and Crohn’s disease.
In Tessa-Mai’s case, it has almost taken her life three times.
James told: “We’ve nearly lost her on three occasions. Once, she had a reaction to some drugs and her temperature soared to 42 degrees.
“She wasn’t moving or responding. She was just lying there – it was really terrifying.
“Another time we took her swimming, and she just flopped over in the pool. We had to quickly get her to the changing rooms and give her a sugar boost.
“After that we had to go into an emergency regime of feeding her every half hour instead of every 90 minutes until she picked up a little.
“That’s what our days are like – we never know what’s going to happen so we just take everything as it comes.”
Now, Tessa-Mai is still under the care of Evelina Children’s Hospital, and she also has regular check ups at Conquest Hospital.
As her immune system is so poor, she needs booster injections three times a week.
Her dad continued: “She’s been pricked by so many needles now that most of her veins are collapsed.
“Once, she had 17 cannulas in just three days. They even had to put one in her neck, which was very distressing for her. She’s built up a fear around needles now.”
It’s impossible to say what Tessa-Mai’s prognosis is but her parents are determined to fill her life with as many happy memories as possible, should the unthinkable happen.
They have set up a GoFundMe page called ‘A better life for Tessa-Mai,’ where almost £500 has been raised to date.
Her parents plan to take her on some fun family adventures with the money, including a visit to Peppa Pig world.
“We would love to see more funding go into researching a cure for this awful condition,” said James.
“At the moment, we can’t even go on holiday. There’s too much equipment to take and running out of the powder she’s fed with is too risky.
“Even if we just pop to town, we take a whole day’s worth of feed just in case of any delays.
“If managed properly, she can live to adulthood, but when she was first born, we were told that a lot of children with the condition don’t live past three.
“We want to give Tessa-Mai some positive memories instead of just this horrible disease.”