A Matter of Life and Death. Sue Armstrong

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СКАЧАТЬ his writings Dr González-Crussí has considered death a great deal. What are your own thoughts about death and dying?

      I’m not a religious person, so I have tried to look at the process of death from the most scientific angle. But even so I recognise there are certain things completely beyond our scientific understanding. And I have had my own personal experiences – I don’t make too much of them, but others perhaps could.

      I was called to do an autopsy on a baby who had a disease that required very rapid intervention in order to make the diagnosis, and to provide genetic counselling to the family. The only option was to go immediately after her death into her heart and take some parts for examination. The clinician called me before the baby died, asking, ‘Would you be available if she dies in the night?’ He called me later and said, ‘We’re going to interrupt artificial life support in half an hour, okay?’ So I drove over in the night, and they came with the baby a few minutes after she died.

      When you die, cells are still viable in many organs, and when you stimulate certain tissues that are contractile they can develop mechanical activity. And what happened was that I was doing the autopsy with the head of the paediatric intensive care unit at my side, and when we got to the heart it started moving. The baby was dead, but the heart was sort of beating … Not really, but in a disorganised fashion there was a beat. I felt at that moment like an Aztec prince taking out the heart of a human sacrifice, if you will. And I wondered at that moment, ‘What am I doing here, opening the heart of this little baby?’ Of course, that was just for a flash second, and I understood that what I was doing was very important. The diagnosis was made, and the family now knows what type of disease they might carry, and understands what to do. It was a very valuable autopsy. But the experience was very chilling.

       Is it something that has lived with you?

      It has. It has. You wonder … In many autopsy rooms there is a statement in Latin that says something like, ‘This is a morgue, the place where death helps those that are still alive.’ And that’s exactly what came to my mind – the contribution this baby was making to her family and to humankind was huge. So I see autopsy as the most exhaustive, and final, medical exam that a doctor can provide for a patient. I don’t see autopsy as something horrendous and macabre.

      But it was just too close to the event of death – was that what got through your professional defences?

      It was too different. When you do an autopsy, usually the body has been in the refrigerator for several hours, it’s cold, it has changed colour and there are many familiar things. But with this little kid I saw something different. She was dressed as a baby. She was pink and warm. It is very difficult to open a body like that – and just very different.

      People can lose track of what life is all about. Frequently I see a case where the clinicians have spent weeks, or sometimes months, treating a patient. They know all the parameters – respiratory, cardiac, etc. – and they follow those and watch the curve of progress up, or down. Then finally, when the patient dies, they come to me and say, ‘What happened? We don’t understand how this patient died.’ And my answer on several occasions has been, ‘Well, I can’t understand how this patient lived.’

      Our practice of medicine nowadays is so fragmented into different specialities and little tasks that we as doctors lose track of the patient. So they come and say to me, ‘But this patient was doing fine. The blood pressure was this and that, the urine was so …’ But when I just show them the heart, the lungs, the whole thing … I mean, you can no longer recognise the human body. The baby weighs two or three times the normal weight because it has been inundated with fluids. It has seven or eight catheters in different places. It has been operated on three times… What I’m trying to say is that medicine is now a practice in which lots of specialists lose track of the big picture, and frequently it is only the pathologist that can try to bring all the loose ends together.

      There are situations in which specialists put too much trust in their results. They are not sceptical enough, and they make mistakes. A memorable occasion was when a genetics counsellor stormed into my office very upset because I had issued a report saying, ‘Fetus without significant abnormalities’ after an autopsy was performed. She said, ‘What do you mean? We saw polydactyly in the ultrasound; that’s why we interrupted the pregnancy.’ Polydactyly is more fingers or toes than five. To interrupt a pregnancy on the basis of polydactyly may or may not be justified, but that’s not the issue. The issue is that I knew the specialist was going to challenge my findings, so when I performed the autopsy I put both hands on a glass slide – they were so small that they could fit into this little space – and you could see five fingers on each hand. So I didn’t open my mouth; I just took the slide and gave it to her in response to her question.

      The point is that with ultrasound – as much as it is very advanced and you can trust a lot of the results – you should never lose track of the fact that those are virtual images, and pathology has real images. This is where things stop: there is no more accurate and real exam than a pathological exam.

      Do you feel people are relying too much on technology, almost in order to cheat death?

      I recognise that if we do not apply our marvellous technology to these very sick patients, we would not be making the progress that we are making. There are voices that argue very strongly against the application of advanced technology to sustain life, to treat certain conditions in childhood or adulthood. I disagree with that because, as you know, our quality of life is much better now than it was just 20 years ago. And 50, 80 or 100 years ago, when there were no antibiotics and no anaesthesia and things like that, if the doctors of the time had not applied all their efforts to pursuing a particular question, we would never have got to this point. So I think that although the end does not justify the means, we should have the curiosity and the will to apply this marvellous knowledge. But we should do it in a respectful way.

      Tell me a bit about your own research.

      Well, I have been interested in a particular group of disorders that involves the development of the neural crest. The neural crests are transitory structures of the embryo that are the excess tips of the closing borders of the neural tube. The neural tube gives rise to the brain and to the spinal cord. But as the neural tube closes – because it first begins as a groove – the excess tissue remains there instead of disappearing, and it migrates to different parts of our bodies through a very complex process.

      Neural crest disorders are of different types. There are neural crest diseases that are cancer-like, or cancer. And there are neural crest disorders that are the result of an abnormal migration or survival of the cells. The bowel, for example, has a brain of its own. The peristaltic movement of the bowel is coordinated essentially by this brain in the gut, and this nervous tissue, which has many millions of neurons inside, requires for its development the migration of the neural crest into the bowel, and the proper development and survival of the cells in a coordinated fashion. If you look at it under the microscope, it looks like a little piece of brain sandwiched between layers of other tissue in the bowel.

       That’s amazing! And is there a lot of it?

      Yes. It continues from the oesophagus all the way down to the exit. And the proper development of this gut brain is necessary for our bowels to move. There is a childhood condition called Hirschsprung’s disease, in which babies, usually, are unable to move their bowel, and become distended. And they can explode, literally, with a colon like an anaconda. Just imagine what it is like not to be able to move your bowel for five, six days. There is no peristalsis, because there is an area that is lacking these neurons in the bowel.

      The disease was described in 1888. It was poorly understood for the first 50 years and then it began to be unravelled by a surgeon at Boston Children’s СКАЧАТЬ