Sunday, September 1, 2013

Teratoma: An Extract

This week, Teratoma was in the news (in the New York Times on the 7th of November 2008). My Teratoma case as described in The Cockroach Catcher now makes sense!

I am therefore taking the unprecedented step to publish on this blog a long extract from my book The Cockroach Catcher.

Also I would like to pay tribute to our eminent yet formidable Professor of Medicine, Professor MacFadzean, 'Old Mac' as he was 'affectionately' known by us. He taught us two important things right from the start:

First - One patient, one disease. It is useful to assume that a patient is suffering from a single disease, and that the different manifestations all spring from the same basic disease.

Second - Never say never. One must never be too definitive in matters of prognosis. What if one is wrong?

Here is the extract:

Chapter 29 The Power of Prayers

“According to old Chinese advice, it is wise never to discuss politics or religion even amongst best friends. Religious belief can often blur judgement in the wisest of people. In psychiatry it is sometimes not easy. This is particularly true in cases of florid psychosis, which often presents with symptoms of hallucination, delusion and even vision

.…….Some time in early February of 1978 I was called to do a Home Visit on a thirteen year old girl by Dr Pinkerton, a paediatric consultant. Dr Pinkerton had been the local Paed for years and was generally well regarded. She had, in my short time as consultant, referred a couple of cases, most notably that of a Tourette syndrome and a boy with non-stoppable hiccups. Both cases put me in her A-list and I gathered that not many were on that list. Needless to say I realised too that her cases were never straightforward or simple. Those she would have dealt with herself. The girl had upper arm stiffness on the left side and Dr Pinkerton could not find much else wrong with her, and so it crossed her mind that perhaps there was something psychiatrically wrong. The girl was also carrying out some strange rituals around the house and Dr Pinkerton did wonder about psychosis or even catatonia.

One of my two clinics was in this so called “new town”. Basically it was an idea conceived after the war in about 1949. The idea was that if people were moved out of the inner city their life would improve. Because they often moved the same people from the same area to the exact same street in the new town the problems travelled with them. Old foes stayed in the same streets as warring neighbours. Yet generations of Local Councils continued to move people into newer housing estates, not understanding why they never managed to solve the problem. I had visited a few of these new towns.

The family I had to visit luckily did not have any enemies but they only moved three months ago and felt very isolated. They moved from a very tough neighbourhood in London not to get away from difficult neighbours. They moved because their daughter did not fit in. She was a timid shy adolescent who did not do normal South London teenage things and was becoming ostracised.

She was not into drugs, smoking or drinking or even sex. To her peers she was a weirdo. After the move, father was able to find a job at the local airport and mother worked part time as a dinner lady at the local school.

Feeling isolated, they went with a neighbour to a local church group and both parents had recently been converted.I was asked very early on by mother, although father did try to stop her, if this might be the work of the devil. She heard that the devil was always trying to do nasty things to anyone who had just become a Christian although she also heard that it could sometimes be God himself wanting to test her faith.I saw what mum meant.

The girl was ignoring my presence. She was mumbling to herself and pacing around the room with a semi-fixed gaze. She held her left arm stiff in a half-raised position and was going round the room as if looking for bits of dirt on the wall and rubbing it. It started about two days before when the parents came home from a church prayer meeting to find her non responsive. Since then she had had sips of water but hardly ate anything. Dr Pinkerton came out straight away to see her and called me in.There was really no significant medical or psychiatric history in the family. She was an only child with the history of the usual childhood ailments. She was average at school though the year before she was not performing well because of problems with other girls.

Both parents were healthy although I noticed that mum was nursing a cold sore.

I did wonder if catatonia was making a come back but the golden rule in psychiatry, as in General Medicine, is: if in doubt, observe.I told mother that it might be better if we got her into hospital for observation. After all they probably needed a break as they did not have any sleep properly since this started. The parents did try to take turns to catch some sleep but as father still had to go into work it was very exhausting.

“But it would not be the mental hospital.”

“No, it would be one of my beds in the paediatric ward, although it would not be the same hospital as Dr Pinkerton’s.”

“Anything would do, Doctor. We leave it in your hands.”

Even when we did not know what was going on, we had learned how to keep that from our patients. Was it cheating or was it just good doctoring? Patient’s confidence in you is as important as your medical knowledge. Perhaps that is why doctors are not doing so well nowadays.

“You will sort her out, won’t you, doctor?”

“Sure we shall. In hospital we can run a few tests including those on the brain just in case and then we can proceed with treatment.”

“Have you seen cases like this before?”

“Sure, not that many but we sure have.”What else could I have said? To be honest, I am only a junior consultant and I have never seen anything like this before, any further question?

“I know you are good. You have helped the boy with the swearing at our church. He now hardly swears.”

My goodness. It is a small place. I have been here only three months and people already know.

All I knew was it would be easy enough if it was indeed the start of a psychotic illness and all would be all right though sad. I must first exclude rare but serious neurological conditions.I had no idea what was to hit me in the next twenty four hours.Sister Clark used to be at University College Hospital in London where I had the good fortune of gaining some paediatric training. She moved here to look after her eighty eight year old mother. We knew we were in safe hands as there was nothing to replace a good Sister on any ward. They reminded us of important things to look out for and basically if we were not too pompous they would look after us. That way we tended not to miss a thing clinically.

When I reached the ward after my day’s clinic, Sister took me to the nursing station. She said the girl was either pregnant or she had a full bladder. A quick examination revealed a soft mass up her umbilical level. How stupid of me.
Remember: every female of child bearing age is pregnant until proved otherwise.Mother’s reassurance that she was not like the other girls fooled me.

She must have found it difficult to tell her parents and therefore was in such a difficult psychiatric state. Faking mental illness would be one good way out.I thought: great! At least I could deliver. Pregnancy test and OB consult and that would be it.But hang on. Would mother not notice her sickness if she was this big? Would she not have complained about other symptoms? Something was not fitting in. And she still looked pre-pubescent.

Perhaps we should catheterise her. She had not been seen to use the toilet for hours although she was not drinking much. She was still going round in her room – we gave her the side room and a nurse – and we put on an input output chart so we knew. The new junior doctor’s car broke down so she was late in examining her.Bother, I forgot it was changeover time, when new doctors came in for their new six-month rotation. This is one of the days of the year not to be ill.

“Good work Sister. What do we do without you?”Sister did the catheterisation but only got about 150ml. The mass was still there. I phoned Ob-Gyn. The consultant had left for home, but I got her Senior Registrar. He came over. Yes, it was possible that she was pregnant but unlikely as there were no breast changes. He would hate to do an X-ray but that seemed justified in the case of an undiagnosed abdominal mass.My mind was racing now. Sometimes you do have to believe what you see. Sometimes you have to believe the parents. She was not one of those girls. She could not be pregnant. So now we had to go through the differential diagnosis for abdominal mass in a young girl of thirteen.

Ovarian cyst was the obvious one. This big?Possible. No. It cannot be. The x-ray came back. The tell tale tooth was there and yes – a Teratoma , the distinctive type of tumour that can include teeth, hair, sometimes, even a jaw and tongue. I guessed just a split second before the results came back. How annoying.

Working diagnosis: Teratoma with possible toxic psychosis.

Emergency operation was arranged. Yes, she would be fine a little while after the operation, I reassured the parents.The paediatric junior arrived and took some history and did a quick physical before she was prepared for the theatre. This petite doctor with a very babyish face told me that on her first day in her last job she had to do an emergency tracheotomy. This time she had been on call for the last three nights and the battery in her old Mini could not cope with the heavy frost so she had to wait for AA before coming. She was most apologetic for not having got in earlier.She asked if I had seen many toxic psychosis cases and I asked if she had come across any in her psychiatric placement. As with all good psychiatrists answering a question with another is in our blood and here it worked well.

Neither of us knew what was to hit us next.At 2 A.M. I had a call from her.“Your patient – I mean our patient could not be aroused after the operation. Yes they removed the teratoma, complete and intact. It is bigger than any specimen I have seen but she could not be aroused. Any ideas?”

“Call the paediatrician on call in the regional paediatric unit and I will be in.”

What happened? I asked myself as I drove to the hospital.What had we done? This was fast becoming a nightmare situation. What was I going to say to the parents?Something else was going on here, and I was not happy because I did not know what it was. I was supposed to know and I generally did. After all I was the consultant now.Thank goodness she could breathe without assistance. That was the first thing I noticed. I saw mother in the corner obviously in tears. She asked if her daughter would be all right. I cannot remember what I said but knowing myself I could not have said anything too discouraging. But then I knew I was in tricky territory and it was unlikely to be the territory of a child psychiatrist.A good doctor is one who is not afraid to ask for help but he must also know where to ask.

“Get me Great Ormond Street.”

“I already did.”She is going to be a good doctor.

“Well, the Regional unit said that they had no beds so I thought I should ring up my classmate at GOS and she talked to her SR who said “send her in”.”

Who needs consultants when juniors have that kind of network? This girl will do well.

“Everything has been set up. The ambulance will be here in about half an hour and if it is all right I would like to go with her.”

“Yes, you do and thanks a lot.”

I told mother that we were transferring her daughter to the best children’s hospital in England if not in the world and the doctor would stay with her in the ambulance. She would be fine.

When I got into work later that day, my secretary asked how my patient was as she heard from her friend that the church was going to hold a 24-hour vigil for her.Trust my secretary. She knew someone from the same church and she always had the knack of extracting information first hand. “They say this may be the work of the devil as the doctors and surgeons all did the right things and removed this big tumour but the devil must have got to her.”

I did have a vague fear that there might have been some anaesthetic accident but quickly told myself off for thinking along that line. I knew all the anaesthetists and such a thing could never have happened. I was back at the hospital to deal with an overdose case. The junior was there and we had a chat in Sister’s office.They had to ventilate her. That was the first thing she told me. I thanked her for going up there and she said it was scary but she felt important and the mother who was in the ambulance could not thank her enough. She was impressed with mother’s faith and trust in God. She said mother was near to tears. It was bad enough to have such a large Teratoma and then to have the patient unconscious with no one knowing what was going on was very frightening.

“I have seen some deaths as a medical student but never since I was registered. I do not want this to be my first.” I knew the feeling well but what could I say? A doctor has to face it some time.

“Do you believe there is God?” She asked

“Do you really think I can answer that one?”

“Well, you have more experience.”

“To me it is like reading a good book. You would not know until the end.”

“So you mean I am not going to know until then.”

……“But do you think this girl is going to live though? I do not want this girl to be my first death. It would be so awful.”

“Neither do I. I keep saying to myself that it is now over seventy two hours and she is still alive and I do know that some cases of viral encephalitis can be very dramatic in presentation and recovery.”

“But which virus?”

“The nearest I have is Herpes.”

“Mother’s cold sore.”

“You have noticed that too.”

“I was with her for a long time.” We had our own prayer for her too. Let it be Herpes encephalitis and all would be well.I left the hospital feeling slightly strange. I just had a philosophical encounter with a young doctor. How strange it is that threats of death always get one thinking about these things.The girl remained unconscious although the word was that the EEG was more hopeful than was first thought. GOS decided to transfer her next door to Queen Square - National Hospital for Nervous Diseases. A lumber puncture was done and the initial findings were in keeping with viral encephalitis. They were now trying to grow the virus. They also wanted Queen Square to decide on assisted ventilation.There was now a candlelight vigil at the church and it was hoped that there would always be a lit candle until she came home. The story was in the local paper and radio.

Faith was now on field test if not on trial.

The doctors were not. They had done their best.On the 10th day the ventilator came off, and she was able to breathe without support.They then started a vigil in the girl’s home.By the 23rd day, as my optimism was about to give in, word came from the hospital that she became conscious. It became big news in the papers.When mother came home from London, she came to see my secretary to give her the details. She told my secretary that she always knew that her daughter would live.No virus was ever isolated and her diagnosis on discharge was that she had a variant of Encephalitis Lethargica .

“Did you agree with the diagnosis?” The junior asked me when I saw her next.

“Why should I be arguing with the best neurological centre in the world? It is harder to argue with a variant of Lethargica. However the next few months or years will be important. If she is well then Herpes fits in better and often it is an allergic type of reaction on first exposure. But if she is like those in Awakenings , then Encephalitis Lethargica.”

I saw her at the local hospital rehab a couple of times. Initially there were a good deal of residual symptoms including awkward gait and dis-inhibition. She became better and was moved to a specialised centre and that was the last I heard of her.Ten years later mother came to see my secretary and left a photo. It was a photo of her daughter and her new baby. She had been working at the local bank since she left school, met a very nice man and now she had a baby. Mother thought I might remember them and perhaps I would be pleased with the outcome.I was very pleased for them too but I would hate for anyone to put faith or god to such a test too often."

Exactly 30 years on we may indeed have an answer

Cahalan's new memoir is called Brain On Fire-My Month of Madness.                                

Chapter 29  The Power of Prayers                                       

Brain on Fire      


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1 comment:

Anonymous said...

Finished it in one breath.