Alfie Evans and Charlie Gard Case Studies

These are two case studies about life support and who has the final say in end of life care. To answer end of life care questions, see: Switching Off Life Support.

Definitions

Encephalomyopathic Mitochondrial DNA Depletion Syndrome (MDDS): This is caused by a mutation of a gene which produces a protein to regulate mitochondrial DNA. This reduces the quantity and efficacy of the mitochondria in cells causing many energy related issues.

Nucleotide Bypass Therapy: A treatment which uses modified nucleoside molecules to increase the production of healthy mitochondrial DNA.

Alfie Evans Case Study 2016

Alfie Evans was diagnosed with a degenerative neurological condition which led to breathing difficulties.

At 7 months, he was admitted to the Alder Hey Children’s Hospital and was placed on a ventilator to keep him alive. He was on life support for almost a year. The parents and doctors disagreed about what was in Alfie’s best interests:

  • The parents found an Italian hospital (linked to the Vatican) which offered Alfie life support for an “undefined period”. The Pope offered Alfie Italian citizenship.

  • The hospital ran brain scans which discovered “catastrophic degradation of brain tissue,” and concluded “continued ventilator support was not in Alfies best interests”.

After 11 months in hospital, Alder Hey Hospital Foundation Trust went to the High Court to request switching off his ventilator. On the 23rd of February, the court ruled in favour of the hospital to withdraw Alfie’s life support to grant him ”peace, quiet and privacy”.

The parents applied to the Supreme Court to appeal the case but this was rejected with the judges stating they couldn’t find a violation of human rights. After the parents met with the Pope, they tried to re-appeal the case but it was rejected.

Eventually the High Court judge decided to switch off Alfie’s life support on the 23rd of April 2018. Alfie died on the 28th after 5 traumatic days with the parents arguing he was still fighting so should be given support.

Alfies Army

Alfies army was a social media campaign whose supporters gathered regularly outside Alder Hey. They abused and intimidated NHS staff causing a police investigation to be launched. Recruitment of staff at the children’s hospital decreased and the staff morale fell.

End of life ethics

  • As the patient was a child, the parents made the decision on Alfie’s behalf. However, the Hospital used the 1989 Children's Act to argue this decision isn’t in Alfie’s best interest and, therefore, they took it to court.

  • Although figures are uncertain, sources suggest one day for special palliative inpatient care costs the NHS at minimum £415. If each year on life support is 0.1 QALY with a 75% chance of survival: Cost per year is £151,475. Cost per QALY (adjusted for survival) is £2 million. This is very far above the £30,000 per QALY NICE recommendation.

  • This was the deciding factor for the case. The court ruled that it was in Alfies best interests to die in “peace, quiet and privacy”.

New legislation in 2018 enabled doctors to switch off life support, without a court case, if it was not in the patient’s best interests. However, if the parents and doctors disagree, then it should be taken to High Court.

Charlie Gard Case Study 2016

Born with Encephalomyopathic Mitochondrial DNA Depletion Syndrome (MDDS) he rapidly began to deteriorate with partial paralysis and severe brain damage. His heart, kidneys, liver, and lungs were affected and he had to be placed on a ventilator in Great Ormond Street Hospital (GOSH).

  • The parents wanted Charlie to have an experimental treatment called Nucleotide Bypass Therapy which was offered in the US for a fee of £1.3 million. They crowdfunded this money.

  • GOSH believed the treatment was futile and Charlie should be allowed to die with dignity. Nucleotide Bypass Therapy had never been used on patients (or animals) with MDDS and although it had been used for a similar genetic disease, Charlie’s brain damage was too severe.

The doctors applied to the High Court which agreed that it was uncertain if Charlie was in pain and, thus, he should die with dignity. This caused a public outcry with both the Pope and Trump tweeting about the case. Donald Trump’s team contacted Professor Michio Hirano to see if anything could be done. He suggested Nucleotide Bypass Therapy could work.

This led to a second court case, “in light of new claims of evidence.” During this time Professor Michio Hirano flew to England and, on seeing an MRI, thought it was too late to save Charlie. The parents abandoned the second court case. Many argued that Professor Hirano was financially motivated and should have come to England weeks earlier when requested.

On the 27th of July aged 11 months, Charlie was moved to a hospice and his ventilator was switched off. He died the next day.

End of Life Ethics

  • Again, as the patient was a child, the parents made the decision on Charlie’s behalf. However, the Hospital used the 1989 Children's Act to argue this decision isn’t in Charlie’s best interest and, therefore, they took it to court.

  • Nucleotide Bypass Therapy cost £150,000. However, it would not have reversed Charlie’s brain damage and so each subsequent year was rated 0.3 QALYs. As the treatment had not yet been tested, Charlie had a 10% chance of survival, with an estimate of 50 years. Therefore, QALYs (adjusted for survival) is 0.3 x 0.1 x 50 = 1.5. Cost per QALY is £100,000 which is over 3x that of the NICE recommendation. Although privately funded (and, therefore, not applicable) this can still be used as an indicator of distributive justice.

  • This was the deciding factor for the case. The court ruled that it was in Charlie’s best interests to die as he was likely in “continued pain, suffering and distress”.

Outcomes of the Charlie Gard Case

After this case, once again many NHS staff in Great Osmond Street Hospital received death threats and staff moral fell. There was also a loss of public trust in the NHS.

Although Court cases can only consider the patients best interests, the Charlie Gard case brought two other suggestions for who should be considered:

  • The parents intervention of choice - if it is privately funded and does’t harm the child then the treatment should be allowed.

  • Future patients with the same disease - no patient with Encephalomyopathic Mitochondrial DNA Depletion Syndrome has been treated with Nucleotide Bypass Therapy. Therefore, by enabling the treatment, this could help future patients and give the family hope.

However, no law has been changed yet.

After the case, the Royal College of Paediatrics and Child Health (RCPCH) also released guidelines for end of life care for a child:

  • Independent medical mediators should be introduced early on to help regulate and decrease the trauma of difficult conversations between doctors and patients/parents.

  • More psychological support for the families.