I lay by my baby boy as he died – this is how we keep his twin sister alive

Parents Paul and Jeanette Mercer have lost count of the number of times they’ve had to resuscitate their young daughter.

Haunted by the memory of her twin brother’s death at just five months old, they vowed never to leave her side.

The seven-year-old’s bright smile and playful nature hide the pain she’s faced on a daily basis – but her family say she is still alive because of a wonder drug denied to thousands of children in the UK.

Doctors initially said neither Kennedy nor twin brother Karter would live beyond eight months after being born with Type 1 Spinal Muscular Atrophy (SMA).

But Paul, 39, and Jeanette, 40, believe a treatment called Spinraza – which is not available to new patients because of its high cost – has both saved her life and allowed her to do things she would have missed out on otherwise.

Paul said that if Spinraza was available to all babies with SMA from birth, Karter would still be alive.

The dad fights back tears as he recalls the night Karter stopped breathing, but says he wants his son’s legacy to make a difference to others affected by SMA.

The cost of the drug, also known as nusinersen, meant the National Institute for Health and Care Excellence (NICE) has not recommended it for use in England – despite it being available in 24 other European countries.

It is the first and only treatment for SMA – which affects 1,300 children and adults in the UK – and costs £450,000 per patient in the first year, and £225,000 each year after that.

Denying it, said Paul, means young lives are being lost every day.

Last week Mirror Online reported how seriously-ill tot Maryam Malji, aged five months, is not allowed the treatment, even though her older brother Abdullah helped trial it.

Paul told Mirror Online: "Another Karter will die tomorrow because Spinraza isn’t available, as devastating as that sounds in this day and age.

"It’s amazing that there’s a drug right there and it isn’t available to families.

"It’s absolutely shameful. Lives will be lost.

"In the UK it’s always been a case of being told to take your child home and wait for them to die, but we weren’t willing to let that happen."

And he said the drug which has changed Kennedy’s life around could have made all the difference to his son.

He said: "If Spinraza was given to Karter at birth, we’d now be talking about my son going to school, I genuinely believe that.

"We wouldn’t be having this conversation now."

Kennedy first received the treatment through Great Ormond Street Hospital in London, which made the decision not to withdraw it after NICE chose not to recommend it.

Now no new patients with SMA – a deadly condition which means they struggle to breathe, swallow or even sit up without support – are being given Spinraza.

Paul said Kennedy’s health has improved so much that she has been able to go on family holidays and play outside with her friends – something her parents never thought would happen.

And since she was first given the drug at the age of five, Paul and Jeanette haven’t had to call an ambulance once – having been forced to do this "hundreds" of times before.

Jeanette said: "Since Kennedy started on Spinraza all our lives have changed.

"The bravest, strongest, funniest , loving child who anybody knows now has a real chance at a quality life.

"Now she always has a large a smile on her face. The independence that Spinraza offers, there are no words to describe!

"Every child with SMA deserves a chance at life, Spinraza gives them that and should be given now."

And Paul said: "After Karter died me and Jeanette promised she’d never be without a parent, we never leave her.

"I’ve lost count of the number of times I’ve been working in London when I’ve had a call saying Kennedy isn’t breathing, and I’ve broken the speed limit to get home while an ambulance is on its way.

"It’s hard to articulate the emotions you go through in that situation, especially after losing a child.

"But since she’s had Spinraza, we haven’t called an ambulance once."

He first noticed something was wrong one night while looking after the twins, who were born prematurely at 32 weeks after the couple underwent IVF treatment.

Karter, he observed, had not moved his legs for several hours, and was having difficulty swallowing.

After a series of tests for conditions including meningitis, he was sent to Great Ormond Street Hospital in London, where doctors confirmed Paul and Jeanette’s worst fears.

"Never at any point do you think your child’s life is at risk, you think it’s a severe viral infection.

"As soon as they tell you your child is going to die, you begin the grieving process. You think about all the things that you’ll never get to do."

Tragically just seven weeks later, Karter died.

Paul was in the bed next to the twins when he realised his son had stopped breathing.

"I went to give him his food and he had gone cold," he recalled. "We’d been trained to give CPR and immediately I tried to save my boy, but we couldn’t get him breathing again."

By that stage Kennedy had also been diagnosed, after her parents pushed to find out if she had SMA as well.

"We did the tests, but asked for them not to tell us until after Christmas. We wanted that Christmas without knowing," Paul said.

"Two days after Christmas a consultant turned up at our home with the health visitor.

"As soon as I saw them at the door I knew what the results were, and started grieving straight away.

"The children are still looking at you smiling, but you’re already grieving."

Paul and Jeanette initially attempted to get Kennedy enrolled on a drug trial in New York without success.

But after a lengthy battle they were able to secure Spinraza treatment for her, commencing in late 2017.

She has been described as a miracle baby by medics after she was able to adapt her diaphragm to breathe.

Paul said: "A doctor told me that her trying to breathe every day was the equivalent of Whitney Houston doing a concert every day – that’s how much effort it took.

"Before she started taking Spinraza she would probably make five noises a day, but now she’s talking and singing, it’s like comparing night and day.

"As a parent you have to feel that you’ve tried your best.

"I can see how happy she is, she’s a beautiful person with a big heart, and she deserves someone to be fighting for her.

"To her she lives a perfect life, it’s different but it’s all she knows.

"She has above average intelligence, she wants to do everything, it’s just her body that’s holding her back.

"Last year I saw my little girl playing in the street with her friends, and I never thought that would happen.

"It’s life-changing."

Last year the family visited DisneyLand in Paris, and went on a holiday to Spain, where Kennedy was able to swim with dolphins.

And in March she will receive a power chair which will allow her to move around independently.

Paul said: "I’m going to cry for about three days, tears of joy, when that happens."

And he continued: "I feel blessed seeing the change that it’s made for Kennedy. It was a fight to get it, but all these parents in a similar position shouldn’t give up.

"I think it’s just shameful that there are drugs there, but parents are still having to have that conversations where doctors tell them their child isn’t going to make it to two years old.

"It’s not right."

Campaigners have called on health service chiefs to make the drug available to all.

Catherine Woodhead, Chief Executive of Muscular Dystrophy UK, said: “Spinraza offers families hope, and stories like Kennedy’s show how effective it can be in treating people with spinal muscular atrophy.

"It is unthinkable that a child who is now diagnosed with the most severe form of the condition cannot access this treatment in England, yet could if they lived in Scotland or in another European country.

"Continued delays in approving the treatment for use on the NHS are unacceptable and it is families who suffer as a result.

"Time is of the essence, and without access to Spinraza babies with the most serious form of the condition are likely to die, while children with other types of spinal muscular atrophy will irreversibly lose the ability to walk, crawl and swallow.

"Now more than ever, we need to see urgent action from NICE, Biogen and NHS England, who must sit down together to find a solution before any more lives are cut short and so children like Kennedy can benefit from this life-changing treatment.”

Meindert Boysen, director of the centre for health technology evaluation at NICE, said last year: “The committee accepted that SMA is both rare and a very serious condition that can have a severe impact on people with it, as well as on family members and carers, and that there is an unmet need for effective treatments.

"The committee was willing to be flexible because of the nature of the condition and the paucity of the evidence, but the very high cost of nusinersen meant it could not recommend the drug as a cost effective use of NHS resources."

In a statement, drug company Biogen said: "The NHS is responsible for treating people living with spinal muscular atrophy (SMA) in England, and we continue to collaborate with NICE and NHS England to work towards a positive outcome for patients.

"Biogen recognises that the delay in accessing Spinraza (nusinersen) is deeply frustrating and concerning for SMA patients and their families, and we share that concern.

"England is already significantly behind many parts of the world in making nusinersen available."

It said it remains in discussions with NICE and NHS England about the drug.

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