Monday, July 17, 2017

Cytochrome C oxidase deficiency

The life-or-death battle over baby Charlie Gard has captured hearts and sparked debate from the White House to the Vatican.

But for one mother, the case is an inescapable reminder of her own decision to allow her child – who is also battling a mitochondrial disease – to die. 

Two years ago, Chloe Harris made the agonising decision to sign a “Do Not Resuscitate” notice for her daughter Bailey-Rose Bickerton, who has cytochrome oxidase deficiency.

Like Charlie, whose future is being decided by the High Court, five-year-old Bailey-Rose’s disease has left her brain damaged, unable to walk or talk and her life is punctuated by violent seizures.
She was not expected to live past three and experts say she will never get better.

And while Chloe, 22, has come to accept the reality of her daughter’s prognosis, she says Charlie’s high-profile case means some friends wrongly think Bailey-Rose can be cured.

The mum has been inundated with suggestions that her daughter could benefit from new treatment in the US that parents Connie Yates and Chris Gard want Charlie to have.

The misguided comments are painful for Chloe, but the mum of three refuses to justify her decision to allow Bailey-Rose to slip away when the time comes.

Speaking exclusively to the Sunday Mirror, Chloe said: “I won’t apologise for doing what’s best for my daughter.
“It was an extremely hard decision to sign the DNR notice and not one I took lightly.

“The general public have no idea how complex mitochondrial disease is. There is a lack of awareness and now people seem to think there is a miracle cure.

“I want my daughter to be comfortable, pain-free and, most importantly, as happy as she can be. We feel she wouldn’t want to pass away horribly, in pain with foreign objects in her body, so while I respect Charlie’s parents’ position, my own decision still makes perfect sense for our daughter.”

Bailey-Rose was born with a severe infection and medics at Liverpool Women’s Hospital battled to help her survive the night.

At three months, she was diagnosed with cytochrome oxidase deficiency, having inherited the genetic disease from parents Chloe and Lee Bickerton, 33. The illness is a form of mitochondrial disease and affects around one in 65,000 people. It means Bailey-Rose has abnormalities in her heart and kidneys, high levels of lactic acid in her blood and weak muscles.

Chloe, who also has Arabella, two, and 11-month-old Auden with Lee, explained: “Medics told us our girl was unlikely to live past three and she’d never be able to walk, talk or hear.

“She was severely brain damaged and would need round-the-clock care. To say we were heartbroken doesn’t begin to describe it.”

Lee and Chloe had a one-in-four chance of passing the condition to their two younger children, but tests during both pregnancies came back clear.

Chloe became a full-time carer for her wheelchair-bound daughter and the pair shared a bed so the mum could keep an eye on her. Increasingly violent seizures saw the tot come close to death several times in her first two years. In 2015, when Bailey-Rose was three, a build-up of acid in her bloodstream meant her kidneys were shutting down.

Chloe recalled: “She was extremely distressed, thrashing around and trying to bite herself. Doctors were trying to find a place to cannulate her but struggling because she’s had so many IV drips. The whole thing was very traumatic for her.”

Bailey-Rose pulled through, but it was after this that Chloe and Lee, of Tameside, Greater Manchester, decided to sign the DNR notice.

Chloe said: “Doctors made it perfectly clear Bailey-Rose would not live much longer.

“She has been through so much and she is a human being. Lee and I decided we did not want doctors pummelling her chest or stabbing at her just to keep her alive so we can have her that bit longer.

“Bailey-Rose can’t speak for herself. As a mother, I had the ability to make sure that she was comfortable.”

The notice means doctors will not give Bailey-Rose CPR or advanced cardiac life support, allowing her to die naturally should the event arise.

Both parents’ families fully supported their decision. Since signing the paperwork, Bailey-Rose’s increasingly regular and violent seizures mean she and her family spend much of their time in and out of Tameside Hospital. On two occasions, at three and five years old, Bailey-Rose came close to passing away.

Chloe recalled: “Watching her, I had no regrets about the DNR notice.

“I just wanted her to know everything was okay. I had conversations with her in my head, telling her: ‘If you need to, you can go now’.”…

The mum tries to avoid reading stories about other “mito-warriors” – children battling mitochondrial diseases – as she finds them “harrowing”.

But the worldwide attention on Charlie Gard’s case – Donald Trump and The Pope have offered support to the tot’s family – is impossible for Chloe to ignore.

She said: “I feel Charlie’s case is misinforming the public about mitochondrial disease. They do not understand that it’s an umbrella term for dozens of diseases and each case is different.

“MD didn’t get enough coverage in the first place and now, suddenly, people say ‘Oh there’s a cure for it now’. They don’t know what I have been through and assume that something can be done for Bailey-Rose and it can’t…

Despite her own heartache Chloe has “nothing but respect” for the decision of Charlie’s parents to seek treatment – and has offered her support on Facebook. She added: “All I can give them is empathy. I know how they’re feeling, it’s hard and it’s scary. Your heart breaks. I have to go through a process of grieving while my daughter is still here and they are probably doing that for their son.” 

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