The Farmer, the Camel and the Cow with a Bad attitude.

farmer sickleFarmers in Ireland are bred tough. They don’t come in to see their Rheumatologist with a bit of muscular pain. They don’t come in with a few Heberden’s nodes. When they do arthritis, they do it properly.

The typical encounter with a farmer would be a chap who hobbles in ruefully, asking politely ‘Sorry to bother you doctor, but is there anything you can do for my knees?’. I usually rub my beard sagely and say –  ‘Hmm, let’s have a look at your hips, then’. Chances are that his knees are fine but his hips are shot to pieces. By the time they stumble into clinic you can guarantee that their hip joints are done for. It never ceases to amaze me that someone can develop severe osteoarthritis (OA) of the hips without ever having experienced pain in the hips or groin. And by the way, I still don’t know why OA of the hips is an occupational hazard for farmers. You see people from other heavy occupations who don’t seem to have the same problem. Perhaps it is something to do with the heavy work most of them did on the farm as young teenagers.

Another common example is the farmer with a seemingly straightforward shoulder capsulitis or tendon rupture. When you get down to the story, however, you find – for instance – that he has been dragged halfway up the field hanging on to the back of a tractor driven away by his 12 year old son who had found the accelerator instead of the brake. Perhaps the most memorable ‘rheumatological case’ was a farmer who hobbled painfully into clinic leaning precariously on two walking sticks with the worst knee joints I’ve ever seen. The valgus deformity on each side was at least 45 degrees and the knees had rubbed together with such violence that he had developed huge suppurating rheumatoid nodules.With each step he had to swing from one side to the other using his stick a bit like a pole vault. When I got him up onto the couch his legs seemed to be connected to the thigh by no more than half an ACL. How this man had continued working on the farm for the previous 5 years I will never know. To my amazement, he recovered well after his knees were replaced and he is probably still working on his farm to this day.

camelAnyway, I digress and really must return to the subject of my story. I could barely contain my excitement when a farmer come in to clinic and showed off his impressive knee swellings. I immediately recognised this as the little known ‘Bactrian camel’ sign. The ‘double hump’ is a dead giveaway. Given the usual yarns, I was not surprised when he told me the story of how this all started with being kicked on the shin by a mischievous young cow. It’s never something mundane like an ingrown toenail. For a farmer this is a regular hazard – but his story was tame compared to one of my patients recently who had barely escaped with his life after having been trampled over by half a dozen stampeding cows. Anyway, our farmer had developed a cellulitis in his ankle which was eventually treated in the local hospital. I say eventually because it is highly unlikely that a farmer would go to see the doctor when there was only a little redness around a scratch. He recounted how the swellings had started after the kick and now they were getting a bit painful. When he took his trousers off I was taken aback. The swelling on top of his right kneecap was like a six inch ‘bap’, and just below that he had a second fluctuant swelling above the tibial tuberosity. Both the pre-patellar and infra-patellar bursae were swollen and clearly displayed in all their glory. The profile was unmistakably that of a double hump-backed or Bantian camel. I wish I had taken a photograph for posterity. A large amount of clear fluid was aspirated from each swelling and steroid injected, to the patient’s evident relief.

As an educational aside, it is well known that infected bursitis often occurs in association with cellulitis, and a good long course of antibiotics is called for. However, this is not the first time I have seen ‘post-infective’ bursitis persisting long after the infection has cleared up. There isn’t a strong evidence base when treating bursitis, but in my experience a small dose of steroid can do the trick quite effectively. Some experts counsel weeks of inactivity, but in my experience if you tell a farmer to rest it is likely that you will be wasting your breath.

The old Irish farmer belongs to a stoical and dependable tradition, in touch with the land and in command of their over-worked but hardy frames. But are they a dying breed? I now see so many of them working on well into their 80s because their children have moved away seeking greener pastures and easier lifestyles. As a rheumatologist who sometimes tends to their ‘war wounds’, I salute them.

The Robber of Memories: Memory loss in Art and Medicine

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…and he recalled that the Magdelena, father of waters, one of the great rivers of the world, was only an illusion of memory’
Gabriel Garcia Marquez ‘Love in a time of cholera’

Probably the most embarrassing experience I will never remember
My first personal experience of a significant memory lapse took place when as a medical student I took part in a study of oral Midazolam. I didn’t feel any different for a few hours after taking the drug and decided that I would make my own way home. An hour later I started to feel very sleepy, and everything for a few hours after that became a blur, a black hole in my memory. I was later told that I had walked into a packed meeting 20 minutes late, sat down in the front row beside the speaker’s wife and had promptly fallen asleep for the rest of the meeting! No matter how hard I tried, I could not piece together one thing the speaker had said or indeed how I had made my way home. Even though this was a trivial event, it was strangely frustrating and my mind kept churning the events over and over for weeks to try and get it back. I felt as if I had been robbed of something precious. It made me wonder what it must be like for people who begin to lose their memory, especially the most treasured memories of their loved ones.

A memorable Film informs an improbable clinical encounter

One of the most striking descriptions of memory loss in art has to be Christopher Nolan’s Memento. In the film the main character tries to compensate for his dramatic and specific deficit in short term memory by tattooing all over his body aide-memoire notes to himself as part of an obsessive and desperate search for the truth. I have encountered many patients with general memory loss in clinical practice but I did wonder if this dramatic failure of short term memory was just a fictional construct or if it could really happen. My question was recently answered when I came across someone with exactly the same deficit, brought on by a bout of encephalitis. She had suddenly become unable to form new memories whilst her past memories remained unaffected and her higher mental function was mostly intact. She continued to work out her puzzles but she  could not retain any new information for more than a couple of minutes. She just could not understand why she couldn’t go home or why people weren’t telling her what was going on. The frustration and anger that this caused was already beginning to distort her normally placid and cheerful character. Apart from ‘locked in syndrome’, I can’t imagine too many conditions more terrible to develop. The ability to remember (and forget) is definitely something we take for granted.

Probably the most memorable book I will always struggle to remember clearly
This leads me in a roundabout way to some of the books I have read this summer. The first was the Gabriel Garcia Marquez classic ‘100 years of Solitude’ which begins with the memorable line “Many years later, as he faced the firing squad, Colonel Aureliano Buendía was to remember that distant afternoon when his father took him to discover ice.” The novel revolves around the Buendia family and the people of Macondo, who have been afflicted by a plague of insomnia whose side effect is a loss of  memory.  We read how the people in this imaginary village had to resort to labelling everyday objects and even write instructions on how to use them.  In the words of Jacob Silverman, “Garci­a Marquez, who has described himself as a professional of the memory, that awareness (i.e. of slippage of memory) must be especially piquant, both because his work is so predicated on notions of memory, history, and ancestry, and because neurological conditions run in his family.” There are many references to memory in his works, and when reading ‘100 years of Solitude’ I almost felt as if I was being transported into a world where I myself was losing my grip on reality and getting lost in an impossible jumble of characters. The thread of history gets tied into so many knots that it takes an effort to unravel it (including regular reference to the family tree, for instance). In spite of the use of magical realism, some of the insights from these books are now being reflected or confirmed by research. For instance, we now know how important sleep is in developing and shaping our memories. Garcia Marquez’ books are also strongly influenced by the author’s experiences and travels in the area of Columbia near the Magdelena river.

Probably the least likely but most memorable travel itinerary in the world

This novel was the inspiration for another book I read this summer: ‘The Robber of Memories’ by Michael Jacobs. Jacobs is another writer with a life long obsession with memory, not to mention his childhood interest in tales of the Spanish exploration of South America. In his own words “The older I got the more I appreciated the role of travel as a stimulus to memories, and the way in which journeys even to new places were somehow always awakening memories of places seen in an ever-receding past”. This book is an intensely personal travelogue/meditation but should be of interest to those of us who want to gain an insight into the experiences of patients or relatives with memory loss or dementia.  ‘Escaping’ for some respite from his role of carer for his elderly mother with dementia, Jacobs embarked on a mission fraught with danger, following in the literary footsteps of his hero Garcia Marquez. Unusually, his journey was a quest to retrace his hero’s love affair with the Magdalena river, ignoring the threat of FARC guerilla attacks to make his way to the source of the river. The story begins with a meeting with the aged author, now himself in the lonely hinterland of early dementia. Garcia Marquez himself has had to let go of so many precious memories, but images of his beloved river had remained. His 81 year-old literary agent Balcells was quoted as saying of him:  ‘Gabo carries a constant glare of nostalgia in his eyes’. That description would have fitted my patient to a tee.

Mingled through the narrative Jacobs reminisces about his parents’ loss of memory and frets with anxiety and guilt about having left his mother. A dread of what might happen to him if he was to follow his parents into the fog of dementia seems to be an undercurrent throughout. He re-reads ‘100 years of Solitude’ and finds evidence that the author may have had a premonition of what was going to happen to him. He meets Marcela, whose mother has Alzheimer’s disease. She tells him about the tales of the disappeared, about the days when two or three unidentified bodies floated down the river every day. In itself, this is a powerful metaphor for the brutal ravages of dementia, separating sufferers from their loved ones in a way that reopens old wounds and prevents them from getting closure for their grief. She said that her mother could still recall some of the trauma but she couldn’t recall where or when they had happened. Jacobs quizzes the locals about the local myth about a ‘robber of memories’ who would visit you in your sleep. It is almost as if he was challenging the robber in his own backyard!

As we are led slowly up the river Jacobs reveals his plan to visit the village of Yarumal, where so many of its inhabitants are struck down early in life with Alzheimer’s disease. The seemingly fanciful idea of a whole community being struck by a ‘plague’ of memory loss is actually based in the strange but real world of the Magdalena. He meets and talks to Fransciso Lopero, a leading researcher who has made the sudy of this unique tribe his life work. Genetic discoveries here have allowed susceptible people in this town to be identified so that in future  potential treatments or vaccines can be tested in this group before they develop clinical symptoms of Alzheimer’s disease.

Many seek for hope at the end of the rainbow: Jacobs sought hope at the source of the Magdelena. And after all, the best research is essentially an adventure into the unknown inspired by a passionate hope and anticipation of discovery. Sadly, Jacobs died from cancer earlier this year. At least he was spared a house call by the ‘robber of memories’.

Further reading
The town of Yarumal and Alzheimer’s disease: Telegraph article by Michael Jacobs
Telegraph Obituary for Michael Jacobs
BBC Article (2011) about Francisco Lopera and his research in Yarumal
Selected quotes and memories of ‘Gabo’ – Huffington Post

More about Alzheimer’s disease from the Alzheimer’s Society

What are we doing to make our service safer?

scaffold safetyI was speaking to a man recently who had worked all his life as a scaffolder. I asked him about advances in safety since the early days. He told me the story of when he worked on scaffolding 23 stories up on a building site in London many years ago. He seemed proud of the raw bravery you had to have in those days, but in hindsight he can’t believe the risks they took at work every single day. One day his foreman called him to join him on some work on the next floor. For some reason he didn’t go immediately and just a few minutes later his shocked workmate informed him that tragically the foreman had just walked over some loose boards and fallen 24 stories to his death. The scaffolder spoke about the incident as if it was yesterday and he still wonders why he hadn’t died that day. Safety regulations on building sites have dramatically reduced deaths and workmen these days wouldn’t dream of taking those risks. In the case of the health service, we as health professionals are not taking risks with our own safety but with that of our patients. So reducing risk – ‘first do no harm’ – should always be at the core of our professional integrity. In today’s uneasy climate it doesn’t take much to bring the great weight of ‘quality inspectors’ and intense media attention to bear on a hospital and its services. At the moment this level of scrutiny is still quite infrequent, and people may get the impression that patient safety is not embedded into routine practice. It is high time that we all started to reflect on what we are doing to improve patient safety and let people know what we’re doing. High risk specialties involving high risk surgery or obstetrics are well used to reporting deaths to confidential enquiry programmes such as ‘CEPOD’. In some cases, particularly in the U.S., these statistics are available to the public and not anonymised. In lower risk specialties such as Rheumatology the measures of safety and quality are harder to measure. The safety measures listed below are gradually being incorporated into routine practice. Here is a provisional list of some of the measures we currently use in our Rheumatology unit to promote patient safety:

  • We report serious drug adverse effects using the ‘Yellow card’ post marketing surveillance run by MRHA. This is a voluntary reporting scheme: some report more than others, but it can help to pick up problems that haven’t surfaced in the drug’s development. We also contribute to a national ‘risk registry’ for the newer biologic drugs (BSRBR).
  • Morbidity reporting for infusion reactions. In the past year we noticed quite a high incidence of allergic reactions in our unit to Iron Dextran infusions: following discussions with specialists and pharmacists we have changed to another preparation with a lower risk of reaction. None of the reactions were serious, but it is still a patient safety issue.
  • Clinical Incident reports – actual harm or near misses. Our hospital has a database for this and the risk management team rate incidents as green/yellow/red by risk level. We contribute to this, mainly for the more serious incidents. These incident reports are looked at centrally and there isn’t normally much discussion at a local level. It is therefore difficult sometimes to see whether anything changes when problems are identified.
  • In our unit, we have started holding regular ‘Mortality and Morbidity’ multidisciplinary meetings – using a clear unambiguous format. These meetings take place once a month and take a few hours to prepare and write up.
  • Multidisciplinary clinical discussions about unusual imaging reports or difficult diagnostic or treatment decisions. We have a good meeting with radiologists to discuss x-rays and scans, but we could probably do better in discussing difficult cases with our peers. It is very difficult to find a time when all clinicians are free.
  • We use (and helped to develop) agreed regional shared care guidelines for ‘disease modifying drugs’ which used to be thought of as drugs with a lot of side effects. We record in the notes that these have been issued with every prescription and we check that monitoring is carried out when patients attend clinics. In our system the General Practitioner or Family doctor has responsibility for monitoring blood tests. We try to record telephone calls for advice, but this is an area we can improve on, perhaps by using email more often. We also standardise patient information about these drugs – we give out booklets but make sure that the patient understands the most important issues.
  • We are responsible for monitoring blood tests for patients on biologics drugs – our pharmacist and specialist nurses take responsibility for this and use an agreed protocol.
  • When we conduct research, we adhere to ‘good clinical practice’ standards and the international statement of Helsinki. We are also regularly monitored by the Trust’s R&D department and have to provide annual reports. Each study has to have prior approval by a regional (and very detailed) ethics review body.
  • We have a discussion of major drug safety alerts or other safety alerts at our monthly multidisciplinary meeting.
  • We carry out regular audits – for instance, this year we looked at the accuracy of Methotrexate prescriptions in patients admitted to hospital. This has led to several proposals to help ensure that mistakes are reduced/identified before a patient can come to harm.
  • Each doctor who works in the unit has to undergo an annual appraisal. For consultants, this includes a review of statistics including mortality & a comparison with peers. There is also a discussion about any complaints, incidents or legal cases. Each doctor has to sign a probity and health statement. Every five years a doctor in the UK now has to undergo revalidation. This assessment has to include two confidential surveys about the performance of that particular doctor – one from patients  and the other from health professionals with whom they work.

This is not an exhaustive list, and it doesn’t mean that there aren’t areas in which we need to improve. For instance, we don’t yet use a clinical database, and we don’t have any link to the patient’s prescription list held by their GP. We don’t participate in a peer review programme – this doesn’t yet exist in our region. These are significant developments that may contribute to patient safety – but they have to be properly resourced and supported by our hospital’s management.

When is a DAS28 NOT a disease activity score?

I’m a big fan of the Disease Activity Score (DAS) and if you’re interested at all you should check out this excellent article in ‘The Rheumatologist’ about how Piet van Riel and Desiree van der Heijde developed the score. As much as I like this tool, I think people need to be aware of its limitations in clinical settings. There is no such thing as a perfect disease activity score, and similar problems arise with other scores. I use the DAS28 (ESR) regularly in clinic, and for the majority of patients it is a very useful tool that matches what the patient and physician think is happening to disease activity. I am written this article mainly for clinical health professionals in Rheumatology.

…when the patient has Fibromyalgia (as well as RA)

This one may be a bit obvious, but it can catch out the unwary. I have made this mistake more than once. As many of you know, the incidence of FMS is increased in chronic diseases like RA and SLE and it can seriously skew the DAS28. In a busy clinic we can be so busy doing the DAS28 and sorting out the patient’s problems that we don’t check for muscle tenderness in a patient with known RA. So how can we avoid this mistake? If you see a patient where the tender joint count and VAS scores are very high but the ESR and swollen joint counts are low/normal – look out! This is the typical pattern with FMS symptoms. A quick check for muscular tenderness will usually confirm your suspicions. Of course, the symptoms of pain and fatigue are genuine and need to be addressed, but just not with anti-TNF drugs or more Methotrexate! You are welcome to check out my little shared spreadsheet tool on Google Drive that shows the COMPONENTS of the DAS28 as slices in a pie chart. This tool uses the official formula for DAS28 (ESR) but splits up the components and allows you to experiment with different values and see the results. It makes it a lot easier to see when the Tender joint score and VAS score are dominating the DAS28.

…When the patient has more tender than swollen joints

I’m not going to make a big issue of this, but it is good to be aware that the DAS28 score puts twice as much emphasis on tender compared to swollen joints. This is good in that it makes the DAS28 more of a ‘patient reported outcome measure’ but not so good when you see a patient with quite a few swollen joints and an inappropriately low DAS28 score. And do remember that the DAS28 was not developed to be used in a system where there would be a clear incentive from having a higher score (i.e. access to biologic drugs) – it is open to some bias on the part of both patient and physician.

…When the patient has a low esr

The DAS scores were developed using data from clinical trials. With handy calculators around, most people don’t pause to check the formula. The ESR score is log-transformed to take account of the way an ESR rises. This is all very reasonable, but it has to be remembered that patients were selected for these clinical trials because they had very active disease with a lot of tender and swollen joints. In most of the trials the starting ESR was very high. The ESR during the trials fell significantly but often not to ‘normal’ levels. So the DAS28 was not really properly validated in the population of RA patients we see day to day in clinic: those with fewer active joints and low/normal ESRs.  Which do you think would cause a greater change in DAS28 score: a change in ESR from 40 to 20 or from 15 to 2mm/h? The change in DAS28 score is actually three time larger in the latter example – a reduction of 1.51 – greater than the NICE/BSR ‘response’ criterion for biologics. There is no cut-off for ESR values within the normal range, and even insignificant changes.

In the UK, NICE guidelines dictate which of our RA patients can get access to biologics. The ‘cut-off’ for access is a DAS28 score of over 5.1. This may be no problem for most patients, but for some men with particularly low ESRs this is an issue. I have a couple of patients who have definite ultrasound proven synovitis in multiple small joints but a low ESR of 2mm/h. Now I challenge you to try and get a DAS28 of 5.1 with that! Have a go on the DAS component spreadsheet. Try entering 15 tender, 15 swollen joints and a VAS score of 90. Now this doesn’t affect a lot of patients, but there’s a handful in our department who need treatment but can’t get it because of this.

…When the patient is in ‘Remission’ (DAS28 less than 2.6)

I would guess that most of you are already aware that the DAS28 doesn’t work too well when it comes to defining remission. This is partly because it completely misses out joints such as the feet and ankles which can be important to people with partly controlled arthritis. It is also partly because of the under-valuing of swollen joints (mentioned above). One of my patients had 5 swollen joints, no tender joints, ESR of 8, and VAS of 10. His DAS28 is 2.23 (i.e. in remission). I think it is better to use the new ACR/EULAR remission criteria.

…When it is done by someone inexperienced

Once people have learned how to examine the patient properly to complete the score and practiced using it regularly for a few weeks they will be able to get quite repeatable results. There is an instructional video on the official website http://www.das-score.nl/ and this site also contains a list of the ‘official’ reference articles on the various DAS scores. However, there are grey areas and there can be quite a bit of difference between observers. So if we’re checking DAS28 scores regularly in clinic, it is a good idea to have it checked by an experienced observer rather than a trainee. If we’re relying on a crucial result (e.g. to determine if a patient is to get biologic treatment or not) we need to make sure that the person doing it is experienced. To measure ‘response’ it is best for the observer to be the same person before and after.

So that’s it. All my moans about the DAS28 all in one page. Am I the only one who has these issues?

The mark of a good doctor: The Caring Quotient (CQ)

What is the essence of a good doctor and how can we choose the right applicants at medical school?

The traditional way to choose doctors was mostly based on knowledge/memory tests. Choose the brightest students and you will get the best doctors, or so the theory went. However, there is a problem. Some very clever people have ended up as very poor doctors. Whilst it is likely that it will always be necessary to have and maintain a high level of learning in order to be a competent doctor, this is clearly not the whole picture. The general theory that IQ is the key to success has been undermined in recent years, and it has been clearly shown that there are other important aspects of intelligence that are not measured by IQ tests or tests of memory recall. Some would argue that today’s easy availability of vast data banks of knowledge will further erode the preeminent position of the learned doctor.

One area of active development has been to look at ways of testing for ’emotional intelligence’ or EQ. These theories are based on the work of a several psychologists over the past 50 years or so, particularly that of Daniel Goleman’s 1995 Book called ‘Emotional Intelligence’. His domains of EQ may be summarized as follows:

  • Knowing your emotions.
  • Managing your own emotions.
  • Motivating yourself.
  • Recognizing and understanding other people’s emotions.
  • Managing relationships, i.e., managing the emotions of others.

This does seem to make a lot of sense, but some have expanded the concepts to encompass such a broad sweep of ’emotional competencies’ that the term seems to lose all validity. To my way of thinking, desirable characteristics such as fairness or trustworthiness cannot be considered as subtypes of intelligence. Studies have shown a poor correlation between the EQ scores of employers and their employees’ assessments of their employer. A person with a high EQ will not always act with empathy/respect/fairness to the people around them although they should be in a good position to understand and practice these positive behaviours.

The media coverage of medicine in the UK over the past decade has been coloured by the dark shadow of Harold Shipman. A clever doctor who knew how to get patients to trust him (high IQ and EQ) but one who fundamentally lacked compassion for his patients. There has been a growing recognition that we need to take more care to recognize and weed out doctors like this but nobody seems to have any clear idea as to how this could be achieved. Appraisal, revalidation and multi-source feedback have been introduced but many feel that as currently set up it is not able to identify the next ‘Shipman’. Reflective practice is a good thing to encourage, but for many doctors their reflection relates mainly to their professional educational activities.

How can we encourage doctors to reflect more on the things that influence their quality of caring? I propose that it would help if we introduced the concept of a ‘caring quotient‘ to complement the IQ/EQ domains. We all recognize that doctors should be caring, but how much and in which situations? This could be tested by proposing challenging clinical situations and getting the opinions of a number of physicians as to what they would do in the same situation.

Is it possible to be TOO caring so that professional boundaries are crossed to the detriment of the patient or other patients under our care? Is it possible for a doctor to be very caring 90% of the time but ‘lose it’ from time to time? If this behavior is not a fixed characteristic it may be useful to consider it as a continuum that can vary, perhaps in predictable ways. Research has shown that, in general, doctors and healthcare workers tend to be biased against patients with alcoholism, dementia or the ‘heart-sink’ patient with the thickest file but the lowest rate of serious pathology. I have seen a number of medical errors over the years that have arisen because an alcoholic has not been accorded the same level of care and attention as others. Difficult behaviour by patients often provokes a negative reaction in us and if we don’t learn to recognize and compensate for this we will make mistakes. Some of our individual biases are so deeply ingrained that we will transgress again and again. I try and teach medical trainees to actively watch out for these biases – as I try to do – and change their behavior to compensate.

So, can a doctor actually learn to recognize situations or patients that ‘make their blood run cold’ and change their behavior accordingly? Doctors would benefit from building up a self-reflective narrative portfolio to include their reflections on difficult or challenging interactions with patients. We could hope that this would encourage more insight into where we are going wrong, where our ‘CQ’ was inappropriate for the particular situation we were faced with at the time. I have put together a mind map to begin to tease out some of the positive and negative factors that could affect a doctor’s ‘CQ’. This is a work in progress!