Background to Charlie's Law

Today many adults and children are helped by new medical treatments for serious conditions and accidental injuries but many are too ill or injured to be helped. Families in shock or distress about a loved person may then put their trust in experimental treatments, even where there may be no evidence as yet that these work. While initially treated with mechanical ventilation as doctors assess conditions or injuries, there comes a point when a discussion about withdrawing treatment is needed. 

A complicating factor is when other countries offer to treat the patient without seeing the medical records and test results.  

We don't hear about the many cases where the patient and/or family and doctors agree on this point but where there there is disagreement, recently families have made the issue public to put pressure on doctors to continue treatment, even when the patient will not recover. 

The parents of Charlie Gard have since set up the Charlie Gard Foundation to work a bill for Parliament on 'Charlie's Law'. They see an urgent need for changes in the law, policy and legal and health practices to support both families and medical and legal professionals involved in emotive, difficult and life-changing circumstances. They have been working with NHS medical professionals, world-leading ethicists and legal experts to develop draft legislation with the aim of preventing further long and painful conflicts between hospitals and families with sick children. 

Three key changes proposed.

• Prevent cases reaching court. Both families and medical professionals would prefer this. The new legislation would provide access to clinical ethics committees throughout NHS hospitals, access to medical mediation where there are differences of opinion, and speedy access to all medical records including raw data.
• Help parents get the support they need for better access to advice on ethics and their rights, independent second opinions, and legal aid so families do not have to face having to pay for costly legal representation, and are not forced to rely on funding from outside interest groups.
• Protect parental rights by restricting court involvement to cases where there is a risk of significant harm to the child.

Terminal genetic conditions. Some children are born with life-limiting genetic conditions. While doctors are able to use mechanical ventilation to help breathing and tube-fed nutrients, no treatments for these these conditions are currently available. Doctors will aim to keep the child comfortable initially but at some point physical deterioration means that they will suggest removing medical help in the best interests of the child.

This was the case with Charlie Gard (4 Aug, 2016 to 28 Jul. 2017) who was born with mitochondrial DNA depletion syndrome (MDDS), a rare genetic condition that causes progressive brain damage and muscle failure, for which there is no treatment and usually causes death in infancy. While being cared for at Great Ormond Street Hospital, doctors contacted New York neurologist Dr Hirano, who was working on an experimental nucleoside treatment and thought there was a "theoretical possibility" that it would help. Dr Hirano and a member of the GOSH medical team discussed the case and agreed that the experimental treatment was unlikely to help if there was irreversible brain damage and that a baseline MRI scan was needed to rule out "severe brain involvement". to rule out . The GOSH team intended to try the treatment and started work on an application to the hospital ethics committee and invited Hirano to visit and examine Charlie (the visit did not happen till July 2017).

When in January 2017 Charlie had seizures that caused brain damage, doctors were of the view that further treatment was futile and might prolong suffering and began discussions with his parents about ending life support and giving palliative care. However, his parents still wanted to take him to the US for experimental treatment and raised funds for the transfer. In February 2017 the hospital asked the High Court to override the parents' decision. The parents appealed but British Courts and the European Court of Human Rights supported the hospital position. In July 2017 Dr Hirano visited Charlie but after examining scans of Charlie's muscles, determined that it was too late for his treatment to help. On 27th July, by consent, Charlie was transferred to a hospice, mechanical ventilation was withdrawn and he died on 28 July 2017.

Brain death. This is permanent, meaning that the affected person will never regain consciousness or start breathing on their own. They are legally confirmed as dead with the time of death on their death certificate logged when they fail a set of tests. This can be confusing to many people because brain dead people can still have a beating heart and their chest will rise and fall with every breath, which is due solely to life support machines - not because they have miraculously regained the ability to do this themselves. Occasionally also the limbs and torso can move, due to reflexes triggered by nerves in the spine that are not linked to the brain - this does not indicate that the brain is working. [By contrast, the the brain stem still functions in someone in a vegetative state.

When the brain stem stops working, it cannot send messages to the body to control any functions and cannot receive messages back from the body. This damage is irreversible. Six tests are used to determine brain death, including pupils not responding to light, having no cough or gag reflex and being unresponsive to pain. MRI scans can also show areas of damage and necrosis.

On 7th April 2022 12-year old Archie Battersby was found unconscious by his mother with a cord round his neck at their home in Essex. (His mother thought he might have been trying a challenge seen on TikTok.) Taken to Southend Hospital, he had suffered severe brain injuries and needed life-sustaining support, including mechanical ventilation and drug treatment. He never regained consciousness. On 8th April he was transferred to the Royal London Hospital in Whitechapel. With no sign of improvement, the hospital was granted High Court permission on 13th May for Archie to undergo brain stem testing. On 16th May two specialists tried to administer brain stem function tests, but were unable to as Archie did not respond to a peripheral nerve stimulation test, a precursor to the brain stem test. Continued life support would only delay cessation of breathing and other body organs would also deteriorate. With the parents still opposing removal of mechanical ventilation, believing that the spinal nerve triggered movement showed consciousness, further court cases gave doctors permission to use MRI scans, which took place on 31st May. Despite medical evidence from the scans, the parents still refused permission to end life support. During June 2017 further court cases in the UK, including the Supreme Court, gave permission to the hospital. On 3rd August the European Court of Human Rights deemed it a matter for UK courts. The family wanted him to be moved to a hospice for this but the High Court, taking medical evidence into account, ruled he was too unstable to be transported by ambulance. Archie's life support was withdrawn early on 6th August and he died at 12.15 BST with members of his family by his bedside.

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Self-sterilising Plastic Kills Viruses Like Covid

Scientists at Queen's University Belfast have developed a plastic film that is self-sterilising and could make it harder for infections, including Covid, to spread in hospitals and care homes. The plastic film is cheap to produce and can be fashioned into protective aprons and other clothing. 

The plastic film works by reacting with light to release chemicals that break viruses, killing them by the million, even in tough species that stay on clothes and surfaces.

Studies had shown that under certain conditions, the Covid virus was able to survive for up to 72 hours on some surfaces and under certain conditions. Other viruses can survive for longer - the norovirus (winter vomiting bug) can survive outside the body to two weeks while waiting for someone new to infect.

The aim of the research was to create a material that viruses could not survive on. While copper is known to kill microbes on contact, it is not very flexible. The new plastic contains nanoparticles of titanium dioxide, which react with ultraviolet light (which can be the tiny amount emitted by a fluorescent tube) to release molecules called reactive oxygen species. Theses react with the genetic material of the virus, the proteins it uses to enter the body, and the fatty sphere around it, resulting in a dead virus.

In the laboratory the material was tested against four types of virus: two influenza viruses, the Covid virus and a picornavirus, which is a very stable virus outside the body. Large amounts (far more than would start an infection) of each of these viruses were placed on the new plastic and went down down from one million viruses to nothing. The effect is seen in less than one hour and maximum death in two hours. In real conditions far fewer virus particles would be present, so it is likely there would be an effect in the first few minutes.

Prof. Andrew Mills of the university's chemistry department said "This film could replace many of the disposable plastic films used in the healthcare industry as it has the added value of being self-sterilising at no real extra cost."

Current personal protective equipment used in hospitals works well, but infections can take place when taking off or putting on PPE. The team are also investigating its use for hospital tablecloths and curtains and use in the food processing industry. Real world trials will be needed to work out how much of a difference it would make.

Source: Self-sterilising plastic kills viruses like Covid by James Gallagher, BBC News 9th Sept. 2022

Sticky tape eczema test for newborns

 Italian researchers have developed a simple test using sticky tape that can predict whether newborns will go on to to develop childhood eczema.

Their research showed that newborns with higher levels of the compound thymus and activation-regulated chemokine were more than twice as likely to to have developed eczema two years later.

The condition affects up to one in five children, causing cracked and itchy skin which can become infected. Discovering the risk early on would help doctors to treat and prevent the skin problem.

Source: Sticky tape eczema test for newborns gives medics an early warning about the painful skin disease. Daily Mail Health Notes: 14 Sept. 2022.

New Discovery on Causes of Dementia

Researchers at bio-tech firm Neuro-Bio, based in Oxford, have made a significant discovery about the causes of dementia. Almost 900,000 people in the UK suffer from dementia, and nearly 70,000 die from it every year. The number of suffers has been projected to rise to 1.6 million by 2040.

Research to date has focused on developing drugs to destroy amyloid plaques (protein deposits) that build up in the brain, affecting memory and cognitive function. However, recent studies show no improvement in symptoms after taking these drugs. 

Research by a team led by Dr Susan Greenfield's on the causes of Alzheimer's dementia has led to the hypothesis that something else could be the initial cause and that amyloid plaques build up well after brain degeneration has begun.

New studies have shown that cells at the centre of the brain, in the isodendritic core, are the first to begin dying in the brain of someone with Alzheimer's disease (the most common form of dementia) often ten to 15 years before they experience symptoms. When these cells die, it sets off a snowball effect, causing more and more cells to die.

While scientists are still unsure why this process begins, Neuro-Bio claims to have found a molecule responsible for causing the damage, called T14. They have developed a drug to limit the cell damage caused by T14 and initial trials on mice show this is effective. 

Human trials are expected to follow, to investigate whether it is safe and effective in humans. The treatment is given as a nasal spray. 

T14 can also be measured in blood and Neuro-Bio is also developing this biomarker as a companion diagnostic.

Source: Oxford scientists hail major breakthrough which could provide relief to 900,000 Britons with dementia by Ethan Ennals, Daily Mail, 2nd April 2022.

Air Pollution and Cancer

 Researchers at the Francis Crick Institute in London have shown that air pollution does not cause cell damage directly but 'wakes up' old damaged cells. This transforms our understanding of how tumours develop and it may now be possible to develop drugs that stop cancers forming.

The classical view of cancer is that it starts with a healthy cell, which over time acquires more and more mutations in its genetic code, or DNA, until it reaches a point where is becomes a cancer and grows uncontrollably. However it is also known that cancerous mutations are also found in seemingly healthy tissue, and many substances known to cause cancer, including air pollution, don't seem to damage people's DNA.

Research has now produced evidence of a different idea. The damage is already there in our cell's DNA, as we grow and age, but there needs to be a trigger to make it cancerous. The research focused on exploring why non-smokers get lung cancer. While the vast majority of lung cancers are caused by smoking, one in ten cases is due to air pollution.

Focusing on a form of pollution called particulate matter 2.5 (known as PM2.5), which of far smaller than the diameter of a human hair, they carried out a series of detailed human and animal experiments which showed the following.

• Places with higher levels of air pollution had more lung cancers not caused by smoking.
• Breathing in PM2.5 leads to the release of a chemical alarm (interleukin-1-beta) in the lungs.
• This causes inflammation and activates cells in the lungs to help repair any damage.
• But around one in every 600,000 cells in the lungs of a 50-year-old already contains potentially cancerous mutations.
• These are acquired as we age but appear completely healthy until they are activated by the chemical alarm and become cancerous.

And crucially, the researchers were able to stop cancers forming in mice exposed to air pollution by using a drug that blocks the alarm signal.

People who had never smoked but developed lung cancer often had no idea why. To give them clues about how this might work is important, since 99% of people in the world live in places where air pollution exceeds the WHO guidelines, so it impacts everyone.

The results also showed that mutations alone are not always enough to cause cancer. It can need another element. We even need to rethink how smoking causes cancer - is it just the known DNA damage caused by the chemicals in tobacco or is the smoke causing inflammation too? The idea that mutated DNA is not enough to cause cancers and that they need another trigger to grow was first proposed by Isaac Berenblaum in 1947.

Source: Air pollution cancer breakthrough will rewrite the rules. By James Gallagher, Health and science correspondent, BBC News. https://www.bbc.co.uk/news/health-62797777