Prediction of Death and Diagnosis

A year or two back I stumbled across a case which seemed to solve an ongoing medical mystery. I feel like recording it here in case it is eve proven correct so that I can say "I told you so" ;)

In December 2013 Californian girl Jahi McMath underwent a tonsillectomy. It went hideously wrong and four days later she was declared brain dead. Her family, however, refused to accept this and fought to keep her on life support. This 'futile care' was resisted but ultimately allowed...not least as people do not live very long on life support. In many cases, if death does not result from other causes, the dead brain will rot causing the body to expire from massive infection. Four years later Jahi McMath remains on life support, apparently showing none of the usual signs of deterioration. One of the most notable features of her case is the onset of puberty and menstruation experienced while brain dead - such developments being supposedly being unique to life, and controlled by the pituitary gland within the brain.

This has led to her family feeling vindicated and others questioning how death can be diagnosed if we still don't understand it. In September 2017 a judge even ruled that Jahi might still be legally alive (http://www.latimes.com/local/lanow/la-me-brain-dead-girl-20170907-story.html).

A year or two ago I was following a hunch when I felt that I had solved the mystery.

A child, identified only as TK, (Nicanor Pier Giorgio Austriaco in The Linacre Quarterly 2016: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5102206/) survived for 20 years on life support / critical care, despite having been declared brain dead at just 4 years of age.

"He remains the individual kept on life support the longest after suffering total brain failure."

There is a condition, extremely rare but well documented, called Lithopaedion. When a foetus dies in utero it is usually expeller from the mother's body as a miscarriage or stillbirth. When this doesn't happen spontaneously medical procedures (D&C or induction of labour) are used. If left for an extended length of time the foetus will degrade and the mother will die of infection. In the case of a Lithopaedion this is circumvented by the mother's body sealing the foetus in calcium - somewhat like a pearl developing inside an oyster - meaning no infection can take hold as the baby becomes 'petrified' hence the colloquial term for the condition - 'stone baby' (Daniel Ramos-Andrade, Caterina Ruivo, M. Antonia Portilha, Jorge B. Brito, Filipe Caseiro-Alves, Luis Curvo-Semedo in Science Direct, 2014: https://www.sciencedirect.com/science/article/pii/S2352047714000082)

In reading the longer account of TK's condition - with autopsy results and imagery in case that bothers you - (Susan Repertinger, MD; William P. Fitzgibbons, MD; Mathew F. Omojola, MB, FRCPC; Roger A. Brumback, MD in the Journal of Child Neurology, 2006: https://hods.org/pdf/Long%20Survival%20Following%20Baterial%20Meningits-Associated%20Brain%20Destruction1.pdf) you may see the connection between Jahi and lithopaedion:

"the specimen [his brain] was seen to consist of a hollow hard-calcified shell containing mostly semisolid and some cystic areas [...] semisolid areas consisted of tan (and scattered intermixed orange, red, and brown), grumous, focally mineralized material, with no identifiable cerebral structures"

TK's brain had essentially been sealed up like a calcified foetus and I feel sure this will prove to be the case with Jahi McMath.

• TK brain death at age 4; Jahi brain death at age 13
• TK brain death from meningitis; Jahi brain death from massive post-operative bleeding
• TK 20 years of life support; Jahi four years and counting
• TK "developed minimal pubic and axillary hair but little other evidence of secondary sexual characteristics" - whilst the lack of full puberty is unsurprising the fact that hair growth occured at all is perhaps surprising; Jahi's puperty is reported but not in detail. It is likely that given her age at brain death these changes were already happening and occurred due to the developmental stage of her ovaries.

I may not be the only one to have realised the connection, of course. In researching this post I found an article listing TK and Jahi's cases in the same sentence yet somehow failing to address the likely connection:

"...there are at least 30 known cases of pregnant women having been physiologically supported for up to 107 days to gestate a fetus; a young boy meeting 'brain death' criteria was physiologically supported for 20 years; and more recently, a young woman has been maintained on home ventilation for over 3 years following the diagnosis of 'brain death'"

(Michael Nair-Collins, Franklin G. Miller in the British Medical Journal, 2017: http://jme.bmj.com/content/early/2017/08/28/medethics-2016-103867). Note the 107 days for brain dead pregnant women. And that's not 'just' because the women are allowed to expire on delivery of their babies - their medical teams usually have to fight to keep them alive long enough to deliver! In comparison, Jahi has been 'brain dead' for at least 1482 days so far. There seem to be no published dates for TK but 20.5 years is approaching 7500 days. (Dr Alan Shewmon in transcripted interview for The President's Council on Bioethics, 2007: https://bioethicsarchive.georgetown.edu/pcbe/transcripts/nov07/session5.html)

EDIT:

Jahi Kelis McMath passed away (permanently this time) on 22 June 2018 - four years six months and ten days (1653 days total) after she had originally been declared brain dead. She is survived by her mother Nailah and stepfather Marvin Winkfield, and a sister, Jordyn. A second death certificate indicates bleeding caused by liver failure. 

(Sources: CNN https://edition.cnn.com/2018/06/29/health/jahi-mcmath-brain-dead-teen-death/index.html Mercury News https://www.mercurynews.com/2018/06/30/jahi-mcmath-death-could-have-costly-implications-in-civil-case-against-hospital-doctors/ East Bay Times https://www.eastbaytimes.com/2018/07/03/of-jahi-mcmath-mom-says-that-little-black-girl-from-oakland-made-history/)

As legal cases remain ongoing at this time (September 3rd, 2018) results of full autopsy regarding the condition of her brain have not been revealed.

My hope is that her brain was calcified and she was utterly unaware of her incapacitation. I also hope that the court cases will be found in the family's favour and they will be compensated for their suffering and Jahi's lengthy treatments will be reimbursed. I don't know that I would like for her case to significantly alter how we define death. The body living on with no functional brain is, in my humble opinion, no life at all. However, my sincere sympathies are with Jahi's family.

Unpopular Opinion Piece

So...here in the UK the Charlie Gard case has been all over the media for ages and now, following his death, I feel it's time to express my thoughts.

And as the title suggests...this is not going to flow along the same lines as the popular opinions on the subject. If you are easily offended I suggest you click away.

Brief(ish) background to the story for non UK / living-under-a-rock type peeps:
Charlie Gard was born on 4th August 2016 and died on 28th July 2017 - his life support was finally switched off after a number of legal battles.
He was hospitalised from the age of two months. Initially admitted for breathing difficulties it turned out he had Mitochondrial DNA Depletion Syndrome which was considered, in his case, to be terminal.
Charlie was unable to move, breathe, open his eyes, cry (so there could be no consensus on whether he was in pain) and so forth.
The world renowned Great Ormond Street Hospital (GOSH) in London where he was being treated believed he should receive only palliative care and be allowed to die; his parents (Connie Yates and Chris Gard) not entirely unreasonably wanted to take him to the US for experimental nucleoiside therapy.
A number of hearings were held; ethics committee, High Court, Court of Appeal, European Court of Human Rights...all agreed with GOSH. Eventually Charlie was allowed to die, aged 11 months and 24 days.

Disclaimer: I have based my opinions on my personal ethics and philosophy and information on the case available in the public arena; I have no medical expertise.

So...

My take on it is that Connie and Chris never had Charlie's interests at heart. I have little faith in the medical profession but despite every damn expert telling them, telling the courts that their son had no hope of life they believed blindly in a 'cure'.

THERE WAS NEVER A CURE. The EXPERIMENTAL nucleoside therapy offered at best (speculatively, as it had never been tried on a child so severely affected) a 4-10% improvement in muscle tone. He would still have been utterly immobile, non-verbal and requiring mechanical ventilation for life (life meaning probable death within early childhood anyway). Connie & Chris were clinging to a dream of Charlie being a normal healthy child and at every stage they seemed to fail to recognise the gravity of his situation.

Repeatedly they stated that if Charlie was suffering they would stop fighting for him...how could they know??? Of course he seemed peaceful. He couldn't move. He couldn't cry. If severely brain damaged (as all medical testimony asserts) it's possible even his blood pressure wouldn't spike to indicate distress. No parent likes to hear their child cry but to have my child unable to express fear, suffering, pain...NO. And yes, if you haven't read my other posts I am a mother. I would definitely bury a child rather than endure them suffering that.

All the expert opinions were that Charlie had suffered catastrophic brain damage from seizures; seizures were nigh on impossible to detect after he became immobile. In their final front-of-court statement Chris Gard said: "we now know had Charlie been given the treatment sooner, he would have had the potential to be a normal, healthy little boy" (http://www.huffingtonpost.co.uk/entry/charlie-gards-father-chris-delivers-heartrending-statement-outside-court_uk_5976159ee4b0e79ec19ad93d) This is fundamentally untrue. The best case scenario for Charlie was paralysed, ventalated, entirely dependent. The same statement said: "far from showing catastrophic, structural brain damage, Dr Hirano and other experts say his brain scans and EEGs were those of a relatively normal child of his age" ...if true* that is terrible. Charlie's life would have been effectively locked-in syndrome; an intelligent conscious mind trapped in an unresponsive body. No sane parent could ever want that for their child.
*There is a definite question as to whether this is true as no statement to that effect was made by ANY medical professional / expert at ANY point. We can only take this as the opinion of his parents. Every professional / expert opinion preceding was that catastrophic brain damage had already occurred.

Connie and Chris' final legal fight was to allow Charlie to die at home...which imho only confirms their insanity. People on life support simply do not die at home. At a time when the NHS is considered to be stretched to breaking point they wanted an incredible amount of money to be spent on pandering to their wishes after incredible amounts of money had already been wasted on court cases and futile care...I'm afraid I don't have a lot of sympathy. Dying in hospital, where he had spent most of his short life, or hospice made little difference. To die at hope was purely for the parents' 'need' with no consideration of the cost, either as financial or potential distress to their son.

Thankfully the courts saw sense and poor Charlie is finally at rest. My heart bleeds for what he endured.