“Is this a pain in her jaw or a pain in my neck?”

In textbooks, guidelines and letters from specialists to generalists, life can seem clear cut. Symptoms, signs and tests all mesh together; a diagnosis appears. Everyone is relieved, except the poor patient who must wrestle with the consequences.

In Primary Care, it is not like that; patients may come with some, any, none or all of the symptoms of not just one, but several conditions. The ANALYSING quadrant of the Map of Uncertainty in medicine can help here. This addresses the WDYDWYDKWTD moments that arise when one doctor and patient are working together and they still don’t have a working diagnosis.

Agnes is a 36 year old kick boxer. She often competes and accepts the bumps and bruises that follow. She ignored an achy pain in her left jaw for a few days, putting it down to a fall in a bout she lost. It didn’t get better, she started to feel unwell, with a slightly sore throat. A friend noticed a swelling in Agnes’ neck and recommended a visit to the GP.

This unusual set of symptoms may give the clinician a WDYDWYDKWTD moment! Is her jaw broken? Is the swelling an abscess, a haematoma, the start of a lymphoma? Why does Agnes feel vaguely ill? It is tempting to take rapid “dysfunctional ways out” here.  As she is not that unwell, reassurance that all will settle with time may seem appropriate.  An X-ray in the A and E department to rule out a fracture uses referral as a quick way out. When the X-ray is normal she will go home none the wiser about her problem. Avoiding a diagnosis and saying vaguely, “it’s probably a virus”, or giving an antibiotic “just in case” are other “dysfunctional ways out”.

Better to use the skills of accurate hypothesis generation, thinking rationally about the differences between ruling things in or ruling them out. Marshall Marinker famously characterised the work of the generalist as being to “marginalise danger, explore probability and tolerate or manage uncertainty” (reference 1)

Could the worst case scenarios here be ruled out?  The trauma cannot be discounted; is there a head injury, jaw fracture? Could the swelling be something serious, an early lymphoma perhaps?

As for probabilities, a fracture was not that likely; although stoical, Agnes would probably have sought help immediately if she had pain on eating, with swelling or bruising over the jaw. The neck swelling may indicate inflammation; is there a local infection? Or are there, as yet unnoticed, swellings elsewhere, in the axillae, groin or liver?

Clinical reasoning can help in deciding where to go next Doctors are keen to rule things OUT, because it reduces worry about serious issues, if they can be reasonably ruled out. Patients are naturally keener for answers and would like to know what has been ruled IN. Examination helped here.

Agnes was fully functioning and alert and had had no period of unconsciousness or head trauma. With no bruising or localised tenderness over the jawbone it was reasonable to “rule out” trauma as the cause for her current problems.

The neck swelling felt like a reactive lymph node and the absence of other enlarged glands, spleen or liver, made a lymphoma less likely. This left some inflammatory possibilities. Careful examination of Agnes’ face revealed a few subtle pinky red spots, all on the same side of the cheek, her skin being otherwise healthy. The ears and throat appeared normal and Agnes repeated that she was not her usual fit and active self.

Could anything be ruled IN? The spots were nonspecific. Pinkish, small and perhaps a bit tender. Was the gland in the neck reacting to an infection there and if so what kind?  The malaise and sore throat could indicate some kind of mild viral process, but that would not explain the spots nor the unilateral lymph nodes. Perhaps the pain was early shingles.

So in the WDYDWYDKWTD moment, what could the clinician do next? When things are not clear cut, follow up and reassessment are powerful diagnostic tools for managing uncertainty proactively. When handled correctly, this can build a strong doctor patient relationship and therapeutic alliance.

When these thinking processes were articulated explicitly with Agnes, she was happy to accept some pain relief and an early telephone review. 36 hours later her face was looking awful, “as you said doctor, things might change quickly”. When she returned, the diagnosis became obvious; there was a patch of shingles across her cheek.

Using explicit reasoning skills and timely reassessment, unnecessary X-rays, antibiotics and an Accident and Emergency attendance were avoided. Uncertainty is an important sign in clinical practice; holding uncertainty and working through it methodically can yield effective results.

  • Marinker M. Medical Audit and General Practice. London 1990

“Are we letting her starve to death then?”

A carer in a nursing home asked this question on seeing Mrs J, an 82 year old lady with Alzheimer’s disease, push away food and clamp her mouth shut when offered a drink. Mrs J had been deteriorating for many months. Thin, bed bound, incontinent, unable to speak except to grimace or occasionally shriek, she needed help with all activities of life, able to do nothing for herself. Her appetite, never huge, had gone entirely. Now she was refusing everything, pushing carers away when trying to feed her, turning her head away when drinks were brought to her mouth. The staff were having a “What Do You Do When You Don’t Know What To Do” moment, looking to me for an answer; I felt I was having a WDYDWYDKWTD moment of my own.

Effective Palliative care can be tricky; knowing when palliation turns into end of life care, even harder. Could a structured approach to uncertainty help me here?

Mrs J’s care involved both diagnosis and management issues. The types of uncertainty here were ANALYSING (what were the diagnoses?) and TEAM-WORKING (arranging the right care with a group team or family) because Joan lacked capacity to plan or negotiate care herself, except perhaps, by refusing sustenance.

Using ANALYZING skills from the Map of Uncertainty in Medicine I first hypothesised and then ruled out intercurrent illness. Apart from the feeding issues nothing else had changed, examination was unremarkable. Although a little dehydrated she did not appear to have an infection, or be in pain, or have any mouth issues. Her dementia had been relentlessly progressive, with less of her old self apparent every week. Even offering her favourite foods did not help. Thus, I was able to rule in a diagnosis; this was a development of her disease, a sign that we were reaching the end stage of dementia, when even food is no longer recognised.

Then I turned to the TEAM-WORKING quadrant. This was not just about me and Mrs J.  Decisions involved her carers and nurses, and her two sons, who lived nearby. There are different ways to plan care. Pathways are for straightforward and slow moving conditions (a hernia repair in a fit 40 year old). Algorithms help when things move fast but are single conditions (childbirth), whereas pattern recognition helps more when things are fast moving and complex ( ITU, major trauma). This was a slower moving, complex situation which is also strongly preference sensitive, meaning care needs to be individualised to the person. Scenario planning is what’s needed here.

Fortunately, when Mrs J was admitted we had met with her family to do just that, and staff had regular updates with her sons as things deteriorated, creating an Advance Care Plan (1). Although saddened, they recognised that Alzheimer’s was taking their mother inexorably away from them. They wanted Mrs J to stay in situ; her last hospital admission had made her fearful, distressed and isolated on a busy ward. They also wanted to “Allow a Natural Death” (AND), a kinder and more accurate phrase than “Do Not attempt Resuscitation”. We had anticipated various scenarios including this one; how eventually the dementia could even result in her failing to recognise food and drink.

We called them once more, sharing the perception that Joan was approaching the end of her life; they were relieved to have this in the open having thought as much themselves. She was nursed tenderly in her own bed with her familiar carers and her sons visiting often. She slipped into a coma and died peacefully a few days later.

And no, we didn’t let her “starve to death”. She died from her Alzheimer’s disease. Emaciated patients with cancer do not starve to death, but die from their disease too.  Working with the whole team, recognising her family as a crucial part of that team, meant that, for Mrs J, the uncertainty generated by the pursed lips and refusal of drinks, resulted in careful planning and a peaceful death.

Her sons sent the staff a big bunch of flowers and thanked them for their care.