Parents enlist expert support to win heart surgery for baby Willow

THE parents of a baby battling a rare and life-limiting condition have won the support of international experts as they fight for crucial heart surgery.

David and Gina Scanlan’s 13-week-old daughter Willow was born with the incurable chromosomal condition Edwards’ syndrome, which causes disabilities and serious heart defects.

But the Aberdeenshire couple believe medical teams are delaying a decision to carry out surgery that could save her.

They also fear that offers of help from experts in the US and details of ground-breaking research there – which it’s thought could double Willow’s chances of survival – are not being heeded at the Aberdeen and Glasgow hospitals where she is a patient.

The couple have also appealed to Scottish Health Secretary Shona Robison to intervene.

The Sunday Post last month told how the couple had begged doctors to give them as long as possible with their baby after learning that some children with her condition can live into their 30s.

Gina, 43, who lives near Fyvie with David and their son Jude, three, said: “We fear our baby is being discriminated against because of her disabilities. We want Willow to be given the same right to life as any other ‘normal’ child with a life-limiting condition.

“Surgeons operate on children with late stage and terminal cancer to extend their lives, as they rightly should. Why not on Willow? Is her life any less valuable because she has disabilities? Would we mourn her less?”

David added: “We are seeking legal advice to determine what our next steps might be.”

Willow needs surgery to close a hole in her heart (a VSD) and to correct defects in two of its valves. But because she has Edwards’ – also known as Trisomy 18 – her parents have been told by her cardiac consultant that it is unlikely she will be given the life-saving procedure.

Her parents claimed that for several weeks they have been seeking a definitive answer from the Royal Aberdeen Children’s Hospital and the Royal Children’s Hospital Glasgow, so that in the event of a refusal they can appeal for help elsewhere before time runs out.

David also claimed that he had asked Willow’s consultant in Aberdeen to forward her medical records as a matter of urgency to experts in the US who had offered to help.

But, he claims, he was told he would have to submit a standard ‘access to medical records’ application form which could take 40 days to process and cost up to £50.

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David said today: “We believe our daughter is being discriminated against due to her disability caused by her genetic condition. And we fear that she is effectively being refused surgery despite US research that I have put to both hospitals that shows it can double her chances of survival.”

The Scanlans said that Thomas Collins, clinical associate professor of paediatric cardiology at the Stanford University School of Medicine has agreed to look at Willow’s case notes.

Research he conducted with colleagues at the University of Arkansas for Medical Sciences found that heart surgery increased survival and hospital discharge on average from 33 per cent to approximately 67 per cent for Trisomy 13 (Patau syndrome) and Trisomy 18 patients (Edwards’ syndrome). He was reported to have said that further analysis showed the number of Edwards’ babies that survive more than doubles after surgery.

Professor of Paediatrics at the University of North Carolina Martin J McCaffrey – who has written papers on the condition – has also offered his support.

David said: “We were told by our cardiac consultant that getting a second opinion would be no problem. Yet here we are with some of the best minds in the field of Edwards’ prepared to look at Willow’s case but we fear both hospitals are putting obstacles in the way.

“We are frightened that even though we can get Willow’s heart scans independently assessed and that that assessment shows surgery would significantly increase her life span, the lack of appetite in the UK to treat children with her disability will work against her.

“We have asked to be told as a matter of urgency if surgeons will operate on Willow and when we might find this out, so that we can find an alternative team if the answer is ‘no’.

“If Willow’s heart begins to fail in the meantime – which would render any surgery impossible in any case – Willow dies. The doctors may be able to afford to wait but Willow can’t.”

NHS doctors have said that heart surgery is not routinely offered to children with Edwards’ because of their short life expectancy and because they are unable to justify the heavy impact of the surgery on the patient and the risks involved.

But Professor McCaffrey, who contacted the Sunday Post after being alerted to Willow’s case, said today: “Willow has a VSD. As for trisomy infants, the most common congenital heart defect for all babies is a VSD. The incidence may be as high as 4 cases per 1000 of all live births.

“This is the most common paediatric cardiothoracic surgical procedure in the US and the world. The mortality is 0.6% in the US. There is no hesitation in operating on children with medically unmanageable VSDs in the developed world.”

And he claimed: “To deny such surgery would be to defy the standard of care and sentence a child to a tortuous cardiac death.”

He said: “It is time for medical providers worldwide to confront biases and the fallacy of ‘lethality’ which continues to plague the care we offer mothers, families and infants with trisomy 18 and 13.

“This is not a demand that every trisomy child should undergo challenging procedures for life limiting conditions which include the reality of severe motor and cognitive impairments.

“This is a plea, however, for us as a medical community to honestly review the data, inform parents about the potential for a life for their child with trisomy 18 or 13, speak to the likelihood of severe disability and its challenges, but also speak to the potential for a longer life, perhaps of years with medical interventions, and the real value such a life may have for families who elect to pursue medical care.

“The Scanlans clearly are determined to give Willow access to surgical interventions which would be offered to a child with normal chromosomes without hesitation.

“I pray that physicians in Scotland will muster the humility to review current trisomy literature and accept that the choice of some families for interventions to support their trisomy child’s life, including cardiac surgery, can be life-saving. Some physicians may not see these as lives worth living, but they are to the children and their families who seek them.

“Willow is now 13 weeks old. That places her well within the category of survival at one year of 36%. The Scanlan’s appear to fully appreciate the challenges they and their daughter will face. They know her life may only be months or years or decades, but they want her to have a chance to survive.”

NHS Grampian said: “Strict rules on patient confidentiality prevent us from commenting on individual cases. Speaking generally, patients (or their parents/guardians) can seek a second opinion if they are unhappy with the course of treatment set out by our clinicians. Access to medical records – if requested – will always be supported in line with current legislation.”

NHS Greater Glasgow and Clyde said: “This patient is under the ongoing care of her local paediatricians, as is standard practice throughout the UK, in this instance in Aberdeen.

“It is usual clinical practice in the United Kingdom to not offer cardiac surgery to children with Trisomy 18. As a result, we have advised the patient’s local paediatric team of this and they continue to provide her care.

“There is an existing well-defined process for further review where indicated, under which we can arrange for patients to have further assessments carried out in their local centre by members of our specialist cardiac team from the Royal Hospital for Children in Glasgow. Such reviews are initiated and co-ordinated by the local paediatric team. We have not received any request for the release of medical records.”

 

 

THE RESEARCH

According to American research published in October, heart surgery significantly reduces in-hospital deaths among infants with Trisomy 13, known as Patau syndrome,  and Trisomy 18, or Edwards’ syndrome.

The study by Thomas Collins, MD, clinical associate professor of paediatric cardiology at the Stanford University School of Medicine and his co-authors at the University of Arkansas for Medical Sciences used data from 44 children’s hospitals across the United States between 2004 and 2015.

The researchers reported outcomes for nearly 1,600 patients, the largest study ever of infants with Patau or Edwards syndromes, according to lead author Collins.

The study found that heart surgery increased survival and hospital discharge on average from 33 percent to approximately 67 percent for these patients, and that this benefit lasted through two years of follow-up.

Collins is reported in ScienceDaily as saying: “When we analysed the survival curves, the data spoke for themselves, especially for trisomy 18, the number of babies that survive more than doubles after surgery.”

Most infants in the study were admitted at less than a day old, and 51 percent of infants in the study who had congenital heart defects died in the hospital or were discharged to hospice.

The researchers also found that in-hospital mortality decreased in infants who were older at their admission date, heavier and female, corroborating previous findings.

It is reported that Collins’ goal is to ‘challenge the narrative’ surrounding these two conditions, much like how the story of trisomy 21, or Down’s syndrome, has changed in the last 40 years.

“Back in 1975, folks would’ve said there’s nothing we can do to help those babies,” he said.”

But now people have proven if you do heart surgery early, patients with Down’s syndrome can live to adulthood and be active members of their community. The difference it makes for them is tremendous.”

 

The Scanlan’s family have set up a fundraising page to help them identify and secure the treatment that could save her life. Click here to donate.