This account clearly shows how complications after an operation can be life-threatening, but not necessarily fatal. Most complications can be treated successfully, if they are recognised in time and the correct action is taken. They only become life-threatening if they continue for too long or if one complication leads to another. In the case of the shah, both of these things happened. The pancreas was damaged, leading to the development of an abscess. That was treated far too late, haemorrhaging occurred and the unfortunate patient died.
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Fever
Human beings, all other mammals, and birds are warm-blooded. Our bodies continually burn energy to keep our temperature at around 37ºC. Our thermostat is buried deep in the brain, in the hypothalamus. It can be disrupted by a protein called interleukine-6, which is released by an inflammation. That causes fever, by raising the setting of the thermostat. The body then has to work harder to keep warm and feels too cold. The hypothalamus passes this incorrect information on to the brain, so that we feel cold, even though that is not the case. We start to shiver and shake, while the thermostat raises our body temperature. When, after a time, the impact of the interleukine-6 reduces, the process is reversed: the temperature falls, we feel too warm and start to sweat. It is not clear whether fever performs a function. Should we let it run its course and do its work, though we do not know what that is, or should we fight it and try to cool the patient down? Fever always has a cause, but it can sometimes be difficult to find. Different inflammations have different patterns of rising and falling temperature. A virus infection typically generates a high fever of over 39ºC, and a bacterial infection between 38 and 39ºC. If bacteria cause an abscess containing pus under pressure, short peaks of high fever also occur, especially in the evenings. Fever resulting from pus will only disappear if the pus is removed surgically. Tuberculosis produces little fever but it does cause profuse sweating, especially at night. A typhoid infection creates a pattern of fever peaks known appropriately as ‘brontosaurus fever’. A bladder infection produces no fever at all.
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Michael DeBakey lived to be very old. When he felt a pain in his chest on 31 December 2006, at the age of ninety-seven, he almost reconciled himself with the fact that he would die of a heart attack. But when he noticed that the pain persisted and he was still alive, the father of aortic dissection surgery realised that he himself had an aortic dissection. He became the oldest patient to undergo the complex major operation that he himself had developed. And he survived the ordeal. Two years later, just shy of his century, he died a peaceful death.
Special forceps that he designed, the DeBakey forceps, are still used by surgeons around the world on a daily basis. DeBakey was genuinely a great surgeon and an example for many of his colleagues everywhere. But clearly, even great surgeons can sometimes make a mistake. Complications are, after all, part and parcel of operations and the risk of problems can never be counted out, no matter how great you are.
Marlène Dietrich also lived to a great age. She died in Paris in 1992 at the age of ninety – thanks to DeBakey – with two healthy legs.
14
Dissemination
Two Musicians and Their Big Toes: Lully and Bob Marley
CONDUCTORS DID NOT start using the small baton that is so familiar today until the nineteenth century. Before that, they would stand in front of the orchestra beating time with a long staff, topped with a ludicrous, decorative ball. The maces waved around by drum majors at the front of marching bands hark back to that practice. Jean-Baptiste Lully, court composer to the French King Louis XIV in Versailles, also used a long staff when conducting. On Saturday 4 January 1687, while banging his staff on the floor in time to the beat, he suffered an unpleasant industrial accident which, seventy-seven days later, was to cost him his life.
The baroque age was at its peak. Versailles was the centre of the world and, in that centre, Lully was the master of baroque music and French opera. His boss, the Sun King, had just survived an operation on his anus, two months previously. Lully was to perform his Te Deum at the beginning of the new year to celebrate the king’s recovery. Especially for the occasion he had reworked the sacred ode, originally composed in 1677, into a magisterial masterpiece. It was to be performed for the king and a large audience on Wednesday 8 January in the Église des Pères Feuillants in Paris. The Saturday before was the final rehearsal. Trumpets and cymbals echoed through the empty church. There were fifty musicians and a choir of more than a hundred of the best voices in the country. In front of them stood Lully, his long staff taller than himself.
A typical feature of baroque music is the basso continuo, a rhythmic succession of chords providing a basis for the whole piece. The musicians had a certain freedom to improvise, but Lully would have intervened as much as possible in the performance of his own work, and most certainly at rehearsals. With a little imagination, you can see him standing there, passionately keeping the beat of the basso continuo with his enormous staff and demanding the attention of the musicians every now and again by striking the ground. At one such moment, he struck his own toe. Whether Jean-Baptiste clenched his teeth and carried on or screamed out in pain, or whether the musicians and the choir, caught up in the imposing music, noticed nothing of the incident or burst into laughter, we do not know. Perhaps the final rehearsal of the Te Deum was interrupted to carry him from the stage, screaming in agony. In any case, the performance of 8 January went ahead, with Lully leading the proceedings, and was a great success. Afterwards, he was seen limping towards his coach and, in the days that followed, his big toe became infected. He developed a fever and his wife sent for a physician, Monsieur Alliot, who advised him to have the toe amputated to prevent gangrene. Lully refused.
The infection spread slowly from the toe to the foot, and from the foot to the leg. Amputation could still have saved his life, and Lully must have known that. And yet, he ignored the wise advice of Dr Alliot and allowed himself to be treated by a quack for the princely sum of 70,000 francs. At first, he recovered, but the fever returned. By then, the charlatan had made off with the money.
Why did Lully refuse the amputation that would have saved his life? Was he too vain to live without a leg? Lully not only wrote operas and ballet, but was also a musician, actor, dancer and choreographer. He was a top entertainer, and not only on the stage. Jean-Baptiste was an Italian of very humble origins, who had worked his way up in France from a simple guitarist to a celebrity. He was a respected composer, husband and father, and a personal friend of the Sun King. But he was also a much-loved character on the Parisian gay scene, who brightened up seventeenth-century France not only with his art, but also with a series of minor and not-so-minor scandals. With only one leg, his career, his pleasure and his status would all have been wiped away.
Or was Lully simply reckless and underestimated the severity of the situation? Seventy-seven days is quite a long time for an infection to ultimately prove fatal. It could not therefore have been gas gangrene, at least not at first, as that spreads like wildfire and, without amputation, will kill you within three days. So it must have been a simpler infection, caused by less aggressive bacteria that spread slowly and with fewer symptoms – perhaps so few that Lully did not see the danger.
The description suggests that the cause was an abscess with lymphangitis and blood poisoning, or a progressive infection that begins as local (the toe), becomes regional (the leg) and then systemic (the whole body). This process of spreading is known as dissemination. An abscess is in essence a closed infection containing pus. What pus is and how it arises has been explained earlier. It is the soup of dead tissue, dead white blood cells and bacteria, which flows from an infected open wound as a creamy, beige-coloured, stinking fluid. But pus can also develop deeper in the body, below the skin. It can then not find a way out and comes under pressure. That causes an abscess. Mostly, with open wounds and closed abscesses, the bacteria in the pus are streptococci or staphylococci, which live on our own skin. In
the case of an abscess, they must have somehow penetrated into the deeper tissues below the skin. That can only occur through a wound, which is known as the point of entry. It could be a nail that you have stepped on, or a dog bite, an inflamed sebaceous or sweat gland, an ingrown hair, a wound caused by scratching an itch or eczema, or a crack in the skin. With fingers and toes, damage to the cuticle can provide a point of entry, which was probably the case with Lully’s toe.
Moreover, Jean-Baptiste’s socks would have been swarming with streptococci and staphylococci. Washing or changing your clothes daily was not done in the seventeenth century, and the French court was no exception. There was a good reason why wigs, perfume and toilet waters were so popular. They were necessary to disguise the unwashed hair and the stench of the body and clothing. It was not until a hundred years later, in the time of Napoleon, that some understanding of hygiene developed, leading to the laying of sewers and the provision of facilities for people to wash themselves and their clothes, practices that had disappeared from Europe with the Romans. It is difficult to imagine how filthy the otherwise so colourful life at the court of the Sun King must have been. Jean-Baptiste Lully’s sweaty sock must have undoubtedly offered an ideal breeding ground for bacteria.
When an abscess develops, the bacteria below the skin initially only cause an inflammation. The skin swells up and becomes warm, tense, red and painful. But then the bacteria defeat the inflammatory cells and pus forms in the inflammation. At that point, the infection is maturing. The increasing quantity of pus pushes the surrounding tissue away, and the body attempts to halt that process by forming connective or scar tissue. The pus then becomes sealed in by an abscess wall, which temporarily stops the development of the infection. But, because blood can no longer flow to the pus, the immune system cannot combat it. Antibiotics would also have no effect. The patient develops a severe fever and the accumulation of pus feels like a hard ball. If you place two fingers on the swelling and one finger is pushed outwards when you push the other inwards, you know for certain that it is filled with liquid. This is known in surgery as fluctuation. If the swelling fluctuates, the infection is mature and ready to be cut open.
If you cut open the abscess wall and allow all the pus to flow out, the wall has a chance to heal per secundam like a normal open wound. This is called incision and drainage. If you do not drain the abscess in time, the bacteria will ultimately break through the abscess wall and be released into the surrounding tissue. That causes an infection of the subcutaneous fatty tissue, known as cellulitis.
The subcutaneous tissues are criss-crossed by minuscule vessels that do not carry blood but tissue fluid known as lymph. These are the lymph vessels, the smallest of which are known as lymph capillaries. In these vessels, lymphangitis develops, an infection that follows the course of the lymph vessels and can be seen on the surface of the skin as a red line leading away from the abscess. This line will be longer each day.
Lymph vessels come together in the lymph nodes, small glands less than half a centimetre across that are found bunched together and act as hubs in the network of lymph vessels. The closest group of nodes to the toe is in the hollow of the knee. The next one is in the groin. The infection causes the lymph nodes to swell up, so that they can easily be felt from the outside as small hard lumps below the skin, the first day behind the knee and the following day in the groin. From the groin, the lymph nodes continue upwards behind the abdomen and finally enter the blood circulation in the chest.
Without antibiotics, an infection of the lymph vessels, lymphangitis, will therefore irrevocably lead to blood poisoning, as large quantities of bacteria will end up in the blood. That will enable them to infect other organs and form abscesses in, for example, the brain, the liver or the adrenal gland. And the whole process will then start again in these abscesses. Whether the patient survives all that will very much depend on his general state of health. A healthy individual will have a healthy immune system and will survive for longer. Lully must have been a healthy man to have held out for seventy-seven days.
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Barrier
An important condition for any living thing to survive is being able to maintain a barrier between itself and its environment. That requires energy, which in the case of animal life needs a continual supply of oxygen. A living cell can survive only while its cell membrane is intact. Complex multicellular animals like humans also have barriers to protect them against the outside world, such as skin on the outside, mucous membranes on the inside and the immune system in between. Cancer can develop only if dysfunctioning cancer cells break these barriers down. A good example of barriers being maintained in our body is the pancreas, which can digest meat but – thanks to its own barrier – does not digest itself. The gastric mucosa, the mucous membrane layer of the stomach, even produces pure hydrochloric acid, but is itself resistant to it. Infectious diseases occur when living pathogens break through barriers. That can be caused by an open wound in the skin or mucous membrane, or by an inadequate supply of blood. The latter causes a shortage of oxygen in the body’s tissues, which can no longer generate sufficient energy to maintain their barriers. Physical damage and a shortage of oxygen are the main mechanisms leading to barriers being compromised. Understanding these mechanisms is the basis of solving the challenge facing modern surgery – to restore the barrier breached by the scalpel as effectively as possible when performing an operation. This means that the tissues in the area around the operation wound must retain a sufficient supply of blood and, while the wound is open, it must be kept free of living pathogens.
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Lully’s leg finally turned green and black. He sent first for a notary to draw up his will and then for a priest to hear his confession. On his death bed, this father of ten children, who had been promiscuous with many men, composed a piece of music entitled ‘Il faut mourir, pécheur, il faut mourir’ (It is time to die, sinner, it is time to die). Lully died on 22 March 1687.
Three centuries later, another great musician died from a disease of his big toe. This man’s music was even more influential than that of Lully. He was the father of a completely new musical genre, even though his oeuvre amounts to only a few hours of music. He, too, refused to have his toe amputated, even though it would have saved his life. But, in his case, it was not pride or vanity that held him back. It was that it was not permitted by his religion. And, like Lully, he sought salvation from a quack, who was equally unable to save his life.
It started with a pain in his toe. He could not remember having stubbed it anywhere. At first, he could make the pain bearable by smoking marijuana. For a while, he thought he had damaged his toe playing football, but the pain did not go away. Doctors diagnosed a tumour below the toenail. A small operation was performed to remove the small growth and study it under a microscope. It turned out to be a malignant melanoma, an aggressive form of skin cancer that develops in the melanocytes, the pigment cells in the skin. He was advised to have the toe amputated, but he rejected the advice and decided to tackle the disease by fasting, smoking and using herbal salves. For two years, he ignored the severity of his illness, even when he developed complaints in other places. The cancer in his toe had spread throughout his body. Eventually his symptoms became so bad that he could no longer ignore the fact that he was going to die. He expressed his acceptance of his fate with one of his most beautiful compositions, ‘Redemption Song’.
Bob Marley spent the final eight months of his life in Germany, in the clinic of a charlatan who believed he could cure the cancer, which had now spread to his lungs and brain, with a special diet and ‘holistic’ injections. When the end was near, he wanted to return home to die. During the flight from Germany to his homeland, his health deteriorated further. In Florida, he was too ill to transfer to the plane to Jamaica. He died in a hospital in Miami on 11 May 1981, three years after the diagnosis. The religion that forbade him from defiling his body with an amputation was Rastafari, an important feature of which is to avo
id all association with death. Lethal diseases, for example, are therefore denied. Marley was thirty-six years old.
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When the body is invaded by cancer, tumour cells spread in the same way as bacteria during an infection. In both cases, a local attack becomes regional and, ultimately, affects the whole body. The mechanism of dissemination is the same. In the case of cancer, this process is known as metastasis, which literally means ‘displacement’. Cancer has three malignant properties. The tumour cells escape the body’s control mechanisms by moving away from their original position. They are able to find their way through other, healthy body cells. This is known as invasion. How far the invasion of tumour cells has developed is a measure of the stage that the disease has reached. The life cycle of the tumour cells also evades the body’s control mechanisms. They multiply indiscriminately, meaning that there are steadily more and more of them. Thirdly, tumour cells lose the properties of the cells they originate from. The less recognisable they are, the more malignantly they behave.
Although tumour cells disseminate through the body in the same way as bacterial infections, they do so much more slowly. Lully survived for seventy-seven days, Marley for three years. Both diseases start locally, where the intruders succeed in penetrating the body’s barriers. Bacteria have to wait for their chance and enter the body through damaged skin or mucous membrane, while tumour cells actively force their way through the barriers, even if they are still intact. In both cases – with an infection or cancer – the body is attacked, and takes the form of rapid multiplication of the bacteria or tumour cells and there is active damage to the body’s tissues, which provokes a response from the body. The immune system tries to repel the attack. White blood cells, antibodies and macrophages – cells that clean up damage to tissues – combat the bacteria and the cancer cells. At this stage, the attack is still local, extending no further than the place where the infection or tumour originated. The invasion can be stopped surgically by means of total (in toto) excision, or resection, of the source. An infected wound with dead tissue (necrosis) can be cut out (necrosectomy), an abscess can be cut open (incision and drainage), and a tumour can be cut away (tumourectomy).
Under the Knife Page 14