Severely brain-damaged patients are commonly misdiagnosed, often aware and may well recover, says authoritative new report

People with severe brain damage are difficult to diagnose reliably, not uncommonly recover and are often much more aware than we think. Specifically:

      Four in ten people who are thought to be unconscious are actually aware

      One in five people with severe brain injury from trauma will recover to the point that they can live at home and care for themselves without help

      The term PVS should be dropped and that pain relief should be given to patients affected

These are the startling conclusions of a new US practice guideline for managing prolonged disorders of consciousness (PDOC) issued earlier this week.

The guideline is acutely relevant to the UK, where on 30 July 2018, the British Supreme Court made a landmark decision, that food and fluids can be withdrawn from patients with PDOC provided that both doctors and relatives agree it is in a given patient’s ‘best interests’.

According to Prof Derick Wade, a consultant in neurological rehabilitation based in Oxford,  there could be as many as 24,000 patients in the NHS in England alone either in permanent vegetative state (PVS) or minimally conscious state (MCS), most of them in nursing homes.

People with PVS are awake, with eyes open, but do not exhibit behaviour suggesting they are aware either of themselves or their surroundings. Those with MCS show definite signs of awareness of self or surroundings, but often, these behaviours may not be obvious or may not happen regularly. These signs include tracking people with their eyes or following an instruction to open their mouths, but the behaviours are often subtle and inconsistent.

A summary of the guideline, together with an accompanying literature review on which it was based, was published online on 8 August 2018 in the medical journal Neurology. An accompanying press release summarises the main points.

The new guideline updates the earlier 1994 AAN practice parameter on persistent vegetative state and the 2002 case definition for the minimally conscious state (MCS), some of the recommendations of which ‘probably no longer hold true’ according to lead author Joseph Giacino.

The guideline carries weighty authority because it has been issued by the American Academy of Neurology (AAN), the world’s largest association of neurologists and neuroscience professionals, with over 34,000 members.

Experts carefully reviewed all of the available scientific studies on diagnosing, predicting health outcomes and caring for people with disorders of consciousness, focusing on evidence for people with prolonged disorders of consciousness—those cases lasting 28 days or longer.

The majority of those with PDOC are young people who have suffered head injuries or older people with hypoxic brain damage (lack of oxygen to the brain, for example during a cardiac arrest).

Errors in assessing awareness

As neuroscientist Adrian Owen's research demonstrates through the extraordinary testimonies in his book 'Into the Grey Zone', reviewed here by Chris Willmott, some patients with PVS and MCS have far more awareness than we might possibly imagine.

The guideline, based on the latest research, states that about four in ten people who are thought to be unconscious are actually aware. This 40% rate of misdiagnosis is because underlying impairments can mask awareness, argues Giacino, and can lead to inappropriate care decisions as well as poor health outcomes.

‘An inaccurate diagnosis can lead to inappropriate care decisions and poor health outcomes. 

Misdiagnosis may result in premature or inappropriate treatment withdrawal, failure to recommend beneficial rehabilitative treatments and worse outcome. That is why an early and accurate diagnosis is so important’, he argues.

It is therefore essential that assessments of these patients are carried out only by real experts.

The guideline states, ‘People with prolonged disorders of consciousness after a brain injury need ongoing specialized health care provided by experts in diagnosing and treating these disorders.

Problems with diagnosis

To get the right diagnosis, a clinician with specialized training in management of disorders of consciousness, such as a neurologist or brain injury rehabilitation specialist, should do a careful evaluation. This should be repeated several times early in recovery—especially during the first three months after a brain injury.’

The guideline enlarges on this as follows (see here for the accompanying academic references):

‘The range of physical and cognitive impairments experienced by individuals with PDOC complicate diagnostic accuracy and make it difficult to distinguish behaviours that are indicative of conscious awareness from those that are random and nonpurposeful.

Interpretation of inconsistent behaviours or simple motor responses is particularly challenging. Fluctuations in arousal and response to command further confound the reliability of clinical assessment.

Underlying central and peripheral impairments such as aphasia, neuromuscular abnormalities, and sensory deficits may also mask conscious awareness.

Clinician reliance on nonstandardised procedures, even when the examination is performed by experienced clinicians, contributes to diagnostic error, which consistently hovers around 40 percent.

Diagnostic error also includes misdiagnosing the locked-in syndrome (a condition in which full consciousness is retained) for vegetative state and minimally conscious state (MCS).’

But it is not only diagnosis that is problematic. Prognoses too can be widely mistaken.

Difficulties in predicting outcomes

‘The outcomes for people with prolonged disorders of consciousness differ greatly. Some people may remain permanently unconscious. Many will have severe disability and need help with daily activities. Others will eventually be able to function on their own and some will be able to go back to work.’

 According to the guideline, approximately one in five people with severe brain injury from trauma will recover to the point that they can live at home and care for themselves without help. Those with a brain injury from trauma have a better chance of recovery than those with a brain injury from other causes.

What about treatment?

Treatments do exist

‘Few treatments for disorders of consciousness have been carefully studied. However, moderate evidence shows that the drug amantadine can hasten recovery for persons with disorders of consciousness after traumatic brain injury when used within one to four months after injury.’

There are also advances being made in the treatment of some acute brain injury because of brain cooling techniques, intracranial pressure monitoring and neurosurgery.

Commenting on the new guideline, an editorial in Neurology, agrees that the term PVS should be dropped and that pain relief should be given to patients affected.

Authors Joseph Fins and James Bernat, argue in their review titled ‘Ethical, palliative, and policy considerations in disorders of consciousness’ that PVS should be renamed as ‘chronic vegetative state given the increased frequency of reports of late improvements’.  

They also advocate ‘routine universal pain precautions as an important element of neuropalliative care for these patients given the risk of covert consciousness’ and ‘applaud the Guideline authors for this outstanding exemplar of engaged scholarship in the service of a frequently neglected group of brain-injured patients’.

Conclusions

The publication of the new guideline, casts further doubt on last week’s UK Supreme Court decision and will no doubt lead to further criticism.

I have argued previously that doctors should not be starving and dehydrating non-dying brain damaged patients to death in any circumstances at all. The fact that a substantial number of these patients are misdiagnosed, feel pain and will later recover only makes the case more strongly.

This state of affairs was predicted in 1993 by Andrew Fergusson, former CMF General Secretary, when he argued that the Law Lords in the case of Hillsborough victim Tony Bland (pictured above) had made three key false assumptions: that food and fluids is treatment and not basic care; that death is in some people’s ‘best interests’; and that it is not euthanasia when food and fluids are withdrawn with the explicit intention of casing death by starvation and dehydration.  Andrew’s whole article, ‘Should tube feeding be withdrawn in PVS?’ is well worthy of further study by all with an interest in this area.

The new guideline, issued by the American Academy of Neurology, has been jointly published by the American Congress of Rehabilitation Medicine and the National Institute on Disability, Independent Living, and Rehabilitation Research and has been endorsed by the American Academy of Physical Medicine and Rehabilitation, American College of Surgeons Committee on Trauma and Child Neurology Society.

I wonder how our own British medical organisations (and in particular the BMA and Royal College of Physicians) will respond. Given that they backed the Supreme Court decision, they most certainly ought to be asked for comment.

Supreme Court to rule on whether doctors can remove food and fluids from brain-damaged patients without going to court

Should doctors be able to withdraw food and fluids from severely brain-damaged patients who are not imminently dying? And if so, in what circumstances?

The answer to these questions has changed dramatically as a result of recent decisions by the Court of Protection which are due to be appealed in the Supreme Court on 29 January 2018.

The case of Tony Bland in 1993 (who was in a permanent vegetative state (PVS) after being injured at the Hillsborough Stadium Disaster) established that clinically assisted nutrition and hydration (CANH) is a form of medical treatment that can be withdrawn in some circumstances.

In that case it was also held that, in England and Wales, prior court approval should be sought for the withdrawal of CANH in all such cases. This now also applies for patients in minimally conscious state (MCS).

A vegetative state is when a person is awake but is showing no signs of awareness; they may open their eyes, wake up and fall asleep at regular intervals and have basic reflexes; they're also able to regulate their heartbeat and breathing without assistance.

A person in a vegetative state doesn't show any meaningful responses, such as following an object with their eyes or responding to voices; they also show no signs of experiencing emotions.

Permanent vegetative state (PVS) is diagnosed if these features persist for more than six months if caused by a non-traumatic brain injury, or more than 12 months if caused by a traumatic brain injury. If a person is diagnosed as being in PVS, recovery is extremely unlikely but not impossible.

By contrast, somebody in a minimally conscious state (MCS) shows clear but minimal or inconsistent awareness and may have periods where they can communicate or respond to commands, such as moving a finger when asked.

Prof Derick Wade is one of the country's leading experts in this area, a consultant in neurological rehabilitation based in Oxford. He estimates there could be as many as 24,000 patients in the NHS in England either in a permanent vegetative state, or minimally conscious.

Patients with PVS and MCS are severely brain-damaged but they are not imminently dying and with good care can live for many years. But if CANH is withdrawn, then they will die from dehydration and starvation within two or three weeks.

In such cases, doctors can withhold food and liquid - if they consider there's no likelihood of improvement, and if the family agree. But to do that, they need to wait - six months for cases with brain injury caused by disease or a year in cases of traumatic injury (see diagnostic guidelines here).

Then the patient has to be assessed by a specialist unit, before being diagnosed as being in PVS or MCS. Then, the procedure is to seek permission from the Court of Protection to take out their feeding tube.

The applications are made by the local Clinical Commissioning Group (CCG) and usually cost around £50,000. Only about 100 such applications have been made in more than 20 years.

Two recent legal judgments have held that there is no requirement for treating clinicians to seek the court’s prior approval to withdrawing CANH for a patient in PVS or MCS where existing professional clinical guidance has been followed and where the treating team and those close to the patient are all in agreement that it is not in the patient’s ‘best interests’ to continue such treatment.

The Official Solicitor is contesting the second of these two judgements in the Supreme Court.

The first case concerned M, a woman who had suffered from Huntington's disease for 20 years and was now in MCS.  It was argued that it was in M's best interests not to continue to receive clinically assisted nutrition and hydration (CANH), with the consequence that she would die. 

The application was supported by M’s family, her clinicians, and an external specialist second opinion. At a public hearing on 22 June, Justice Peter Jackson made the orders requested, giving short reasons and reserving fuller judgment. On 24 July, CANH was withdrawn from M, who then received palliative care, and on 4 August she died. She was 50 years old at the time of her death. 

In his fuller judgement on the case on 20 September, which was widely reported in the press (see Guardian and Telegraph), Mr Justice Jackson said in future judges should not be required to make rulings in similar cases - where relatives and doctors were in agreement and medical guidelines had been followed.

The second case involved a 52-year-old man (Y), married with two adult children who in June 2017, suffered a cardiac arrest after a myocardial infarction (heart attack) as a result of coronary heart disease. It had not been possible to resuscitate him for well over ten minutes, resulting in severe cerebral hypoxia and causing extensive brain damage (See Guardian, Times (£) and full judgement (£)).

Mr Y had been in a prolonged disorder of consciousness since his cardiac arrest and, in July, was admitted to a regional hyper-acute rehabilitation unit under the control of the claimant NHS Trust. 

Two medical experts with extensive qualifications and experience in the field of neurological rehabilitation agreed that Mr Y was in a very low level of responsiveness, he had no awareness of self or his environment, and it was highly improbable that he would re-emerge into consciousness.

The clinical team and Mr Y's family, including his wife, Mrs Y, agreed that it would be in his best interests for clinically assisted nutrition and hydration (CANH) to be withdrawn, with the consequence that he would die within a period of two to three weeks.

The NHS Trust sought a declaration that there was no mandatory requirement to seek consent from the court to the withdrawal of CANH, which the court had upheld. However, the Official Solicitor has appealed this decision and, as noted above, the Supreme Court hearing is expected to take place on 29 January.

I was asked to comment at the time of the first hearing on 20 September and my words were later picked up by Life Site News.

I said that the court decision had set a dangerous precedent and should be appealed. Taking these decisions away from the Court of Protection removes an important layer of legislative scrutiny and accountability and effectively weakens the law.

This will make it more likely that severely brain-damaged patients will be starved or dehydrated to death in their supposed best interests and that these decisions will be more influenced by those who have ideological or financial vested interests in this course of action.

I think that the key issues legally and ethically separating right and wrong in end of life decisions are:

1.   What is the intention of this particular action or omission?

2.   We can say a treatment is futile (ie burden outweighs benefit measured in terms of cure/relief) but not that a life is futile

It seems to me that these recent decisions have been made with the intention of ending the life of a person who is not imminently dying because their life has been judged futile. This is a very dangerous precedent indeed.

Furthermore, there are still significant uncertainties about diagnosis and prognosis in both PVS and MCS. These have increased rather than decreased since the Bland case and this is why continued court endorsement of the withdrawal of life-sustaining nutrition and hydration in such cases is necessary. Well-intentioned people - relatives, carers and clinicians - often make mistakes about diagnosis/prognosis and accordingly, agreement between all of them about withdrawal of CANH is not adequate protection.

Life Site News noted that whilst starvation and dehydration certainly hasten death, this is not a painless procedure. In 2006, a British euthanasia activist gave up her own freely chosen attempt to starve to death after 19 days, saying it was too painful. ‘I would not wish what I have been going through on my worst enemy,’ Kelly Taylor said.

At the moment, because of the current policy of involving the court in all such cases, the number of cases we see are very few (fewer than 100 in over 20 years as noted above). But if the court were to be removed from the equation, this could very well lead to a huge escalation of cases, given how many people in Britain have either PVS or MCS.

Doctors are already moving in this direction as a recent article in the Journal of Medical Ethics argues. Furthermore, in the light of these recent court decisions, new interim guidance for health professionals in England and Wales has been issued by the BMA, Royal College of Physicians (RCP) and General Medical Council (GMC). They plan to publish new definitive guidance in May 2018 which will make this new direction official.

In their interim guidance they note however that it 'may need revision if a case concerning these issues is considered by the Supreme Court'.

This is now happening and we await the hearing with great interest.