Mum pleas for Organ Donor Register sign ups to help people like daughter Poppy who needs second transplant

A mum has made an emotional plea for more people to consider organ donation as her daughter awaits a life-saving transplant operation for a second time.

Poppy Hughes, 17, from Cleveleys, suffers from the rare condition Chronic intestinal pseudo-obstruction (CPI) and received a liver, small bowel, stomach, pancreas and large bowel in September 2014.

But after what her mum Kim terms “an amazing nine years”, Poppy and her family are again facing an agonising wait for a multiple transplant after the donated organs started to fail, now leaving the teen attached to Intravenous machines 21 hours a day, unable to eat or drink, in pain and vomiting, unable to walk and even unable to breathe for herself at night.

Kim is pleading for more people to consider organ donation to help those in need such as her daughter, as even with consent given by bereaved family, just a tiny fraction of transplant irgans are viable, particularly for children.

"All I'm asking is people talk about it, to think about it” said Kim. “Not just for us, we are just one. There are over 7,000 currently on the transplant list and almost 250 of those are children.

“Without that transplant nine years ago, Poppy wouldn't be alive. Her call came right at the last moment - we were lucky that a family of a paediatric donor thought of another family at the worst possible moment of their lives.”

Poppy was diagnosed with Ehler Danlos Syndrome, which caused intestinal failure, meaning her bowel and stomach didn’t work properly, when she was 20-months-old after being born with the condition and up to the transplant at almost eight years old, she had never eaten or drank, and was connected to intravenous machines 24 hours a day.

"Huge nursing care was required daily to keep her alive, in and out of intensive care, ventilated regularly,” said Kim.

"Not only did the transplant save her life but it gave her life too, gave her a chance to have a childhood, go to school and make friends, to be a normal little girl. She has had an amazing nine years that she never would have had, if it wasn't for organ donation.

"At a time of sorrow a family chose to give life – and I can honestly say that our donor family is never far from our thoughts. With every amazing thing she does we give a thought to our donor, because they are our heroes who made it happen.

“Who knows if we will be as lucky as we were nine years ago. We are again relying on strangers to give hope at a time of sorrow, to think of others at a time they are broken.

"It's a huge ask, but if people gave it thought and discussed it, should the worst happen they have had that conversation already, making it easier to discuss it at that terrible time of sheer grief. “We were lucky the first time, but I'm not ready to give up yet – no-one can ever be ready. We have an amazing team at Blackpool Victoria hospital and her transplant team in Kings College Hospital, they give her the best care – we are already very lucky.”

Organs failing

Kim says Poppy’s transplanted organs failing dates back originally to just a year after the initial operation, when she picked up a bug in her bowel.

"For years after that, it was fine and she was able to enjoy life much more but it started getting worse about three years ago and in the last year, it has got so much worse that all of the organs now need replacing again.

“We find ourselves right back at square one, on the transplant list again for the same transplant, attached to Intravenous machines 21 hours a day, unable to eat or drink, in pain and vomiting and so weak she can't walk. She can't even breathe for herself at night and needs a BIPAP Ventilator – life is really tough for her.”

‘Very limited matches’

Kim points out that adults are presumed consent for organ donation now thanks to Max and Keira’s Laws which she regards as “amazing”, but many families still say no, and withdraw that consent.

"With children it’s very different – it has to be a choice, a gift, and without people talking about it, that just doesn't happen,” she said.

"Children’s organs often need to be size matched and only one per cent of deaths a year are viable to be donors, so lives being saved are very limited.

“Talking about organ donation more is the first step to making changes. Our country needs to be more like others, we need to be kinder and think of others.

"Anyone who has ever received organs, or have donated loved ones’ organs knows that the donor really does live on.

"With every single thing Poppy does that's amazing, we always give thanks and a thought to our donor who made it happen, and the family who lost that loved one. They are never ever far from our thoughts, they truly are a huge part of ours lives – always.”

Details about organ donation at www.organdonation.nhs.uk/register-your-decision/donate/

Seven and a half hour operation

Back in 2014, brave Poppy, then just seven years old, spent three months recovering from the seven-and-a-half-hour transplant operation in which she had her small and large bowel, liver, pancreas, and stomach replaced,

It was the culmination of an 11-month wait for a suitable donor and took place at Kings College Hospital, London.

Poppy was able to return home just in time for Christmas, with the ambition to enjoy her first-ever Christmas dinner with her family.Mum Kim told The Gazette at the time: "Poppy wouldn’t be here without her donor. We are very lucky."She got sicker and sicker last year - we had no choice."The call for the transplants came at the right time."It has given her a complete new life - she says she loves her new life.

"We are not planning anything for Christmas Day - all I want is for her to feel well."She has never eaten Christmas dinner before so this will be the first year."Hopefully she’ll be able to have a bit of a taste."She has been working hard with her eating - learning how to chew and swallow."She’ll have a spoonful of mashed potato or mushy peas. She’ll eat a bag of Skips, she loves them!"Top of Poppy’s wish list for Christmas as she returned home following the operation were a couple of Leap Pad games.She said: "I’d like two more Leap Pad games to fill my case for Christmas."And I want to watch Home Alone on Christmas Day."

What is CIP?

Chronic intestinal pseudo-obstruction (CIP) is a rare disorder of gastrointestinal motility where coordinated contractions (peristalsis) in the intestinal tract become altered and inefficient. When this happens, nutritional requirements cannot be adequately met.To facilitate normal digestion and the absorption of nutrients, food content is propelled through the GI tract by synchronized or coordinated contractions in a process called "peristalsis." Peristalsis is dependent upon the coordination between the muscles, nerves, and hormones in the digestive tract.

In CIP the intestines react as if there is a true mechanical obstruction or blockage. However, when tests are performed, no physical evidence of blockage is found. There is no unique sign or symptom that allows a positive diagnosis of the pseudo-obstruction.