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NKH Families

Meet Joesph

By 11th June 2020No Comments

Joseph Michael Kendrick, was born on May 9 2009 after an unremarkable pregnancy at 42 weeks in Greater Manchester, UK. At birth he was noted to be very quiet, but thought to be otherwise healthy.

Over the next couple of days, however, he rapidly deteriorated, stopped waking for nappy changes and feeding, and fell into a coma. Joseph was immediately taken to PICU (Pediatric Intensive Care Unit), where they began testing him to find the cause of his lethargy and low muscle tone.

After six frantic days, his parents, Emma + Paul found out that Joseph had a severe form of a very rare genetic, metabolic disorder known as NKH. Emma + Paul were absolutely devastated.

As if they didn’t have enough to worry about, they were then told that Joseph was unlikely to live past day 10. And, if he did, he almost certainly wouldn’t reach his first birthday.

Thankfully, Emma + Paul had their families around them. Their unwavering support got them through those dark first few days. Every day we would stand around our little boy’s cot praying that he would wake up, open his eyes or cry. They were told of a special medicine – sodium benzoate – which may help to wake Joseph up and remove some of the glycine from his brain, but not from his cerebral fluid.

Eventually, on day 13, Joseph finally ‘woke up’, he started crying, and he seemed to act more like a ‘typical’ little baby. They were able to change his nappies, feed him via his ng tube and cuddle him. To them, that was everything. Emma and Paul were told that Joseph was very jerky due to a kind of seizure called a myclonic jerk and, if he lived, these would turn into other types of seizures.

Joseph did get through those horrible early days in hospital, and they were able to take him home after about a month. They had a very worrying year waiting for him to become as poorly as we had been told and to stop breathing – but thankfully he never did.

Joe has now reached eleven years old, and is doing remarkably well despite his condition. He still has frequent seizures, but takes medicine to help control them. Joe ate orally till he was 4 years old but now has a gastrostomy for milk and medicines which is much easier to feed him through. Joe still manages tasters of food when he’s well and particularly enjoys yoghurt and ice cream in the Summer.

As parents, Emma and Paul are very proud of their strong and resilient little boy. He has done so well and so far has exceeded all expectations. Joesph has shown he wants to fight all the way – and his parents are determined to fight on his behalf to help create awareness of this devastating condition, find a better treatment for him, and one day find a cure for NKH.

As such, Emma and Paul founded Joseph’s Goal in October 2012, which has grown into one of the leading NKH charities.

Joseph’s Goal funds research at UCL, under Prof. Nick Greene. As such, Prof. Nick is making great strides in gene replacement therapy – the defective gene in the brain that causes NKH has been identified, and the task now is to find a route to replace it with a healthy gene. Prof. Nick has acknowledged that funds received from Joseph’s Goal have significantly accelerated his NKH research.

There is, at last, some light at the end of a very dark tunnel, with real grounds for optimism that better treatments for NKH are not far away.

Joseph’s Goal is still the only NKH charity in the UK and is now the biggest fundraiser in the world for NKH research. We are hopeful that this year will be the year that breakthroughs in NKH research are made, and Joseph’s Goal is determined to maintain our level of fundraising and support to make this happen.

www.josephsgoal.org
Read about Prof. Nick’s research
Follow Joseph’s Goal on Facebook
Donate to Joseph’s Goal on justgiving