Sickle Cell Disease – New Guidance

New guidance out on sickle disease from NICE in the BMJ

I must admit to having a bit of an interest in sickle cell disease and the ED. There are two reasons for this.

Firstly, and most importantly, it is a really important disease to manage well. It’s a proper disease that we have learnt about since the very first weeks of med school, one for which the pathology, pathophysiology and treatment is well described and understood.

Secondly, I am constantly intrigued to see new docs arrive in the ED with preconceptions about the disease and it’s sufferers that I cannot understand or explain.

So, back to basics. Sickle cell anaemia is a ‘proper disease’, it can kill you, and shortens your life expectancy which is bad, very bad indeed. Living with sickle disease can be tough and patients suffer painful vaso-occlusive crises that may require them to come to our EDs for help. If you work in a multi-ethnic practice like Virchester then you will meet many patients with Sickle disease so it’s important that we understand how to help them. In our ED that means that we have a really good working relationship with our haematologists and in particular a specialist nurse in sickle disease who knows our local population really well and who has encouraged the development of personalised treatment plans. These are shared with the ED (the pink file), the patients, and with the haematology team. If not then we have a great pathway for the treatment of ‘unknown’ patients with sickle disease. It’s safe, evidence-based based and it works. Many of our local patients manage their disease very well at home and only come to see us when home management has failed. With few exceptions they only come to us when they really need help.

So, in Virchester everything is rosy. Except it’s not because I still occasionally come up against ideas which I find difficult to explain and understand, and this is almost always around the use of analgesia in vaso-occlusive crises.

So, let’s think about analgesia. In many ways I think that sickle disease is the poster boy for good analgesia management in the ED. Get this right and you probably have an ED that manages most pain well (there is a poster girl as well – but that’s for another day).

Why is it the poster boy? Well, because the management of sickle disease is one where analgesia is a cornerstone of management, where there are few physical signs, but where I see clinicians manage patients in very different ways. Such variation is not just an issue in my practice, but also in past studies and papers where the experiences of patients attending EDs is far from optimal. Take this quote from a 1999 paper in the BMJ

“The experiences of patients with sickle cell disease of hospital care are characterised by mistrust, stigmatisation, control, and neglect”

Now that was a long time ago (1999) and I like think we have made fantastic progress locally, but I am not convinced that this is the case everywhere because I still see suspicion and concern amongst some junior docs rotating in from St Elsewhere. Why is this I wonder? Where and how did they learn it? Why is it that we have a disease that causes pain, suffering, shortens lives but for which I sometimes see docs and nurses ‘not believe’ that the patient is genuinely in pain and as a result their analgesic management is suboptimal.

Why then? Is it the disease itself? Well perhaps as there are often few physical signs in sickle disease that ‘justify’ the pain to the physician. There is no bleeding, deformity and often few physiological signs. I hear odd comments such as ‘nobody in that much pain has a pulse less than 110’……! Really, do you want me to demonstrate whilst I take your pulse….? Until we have a pain-ometer that can quantify pain then we have to presume that the patient is telling the truth unless we know otherwise. In law we are innocent until proven guilty and the same should apply here. Similarly, on a general approach to pain then we must decide whether analgesia is something that we should freely offer, or should it be something that is earned? What do I mean by this? Well, its that I believe that analgesia is one of the most important things that we can do to improve the patient experience of illness and injury. We should be seeking out pain and treating it, not waiting for our patients to beg for it to be taken away.

This cannot be right and we cannot accept it, but how can we make it better.

I would suggest the following.

  • 1. Speak to your local haematologists and devise a protocol for the management of sickle pain. Why not use something like that advised by NICE and abstracted in this weeks BMJ
  • 2. Use sickle as a teaching tool for analgesia. Ask your colleagues about their attitudes towards sickle disease and the patients. Challenge, argue and question any attitudes that trouble you.
  • 3. Role model the care of sickle patients in the ED. As a senior these are great cases to see with a junior doc. Great for the patient as they get great care. Great for you as it tells you a lot about their attitude towards the disease and analgesia in general. Ask them how they ‘measure pain’, you might be surprised at the results…
  • 4. Talk to your local haematologists. Establish a mechanism for them to tell you about difficult or complex patients.
  • 5. Train the whole team. Analgesia is something often prescribed by docs but prioritised by the nurses. If you only train one of the tribes then you will fail.
  • 6. Listen to Dr Gentile.

When the patient is comfortable ask them what they felt about their experiences in your ED. It’s highly unlikely that this will be their first attendance at the ED. Ask them how you did, ask them about good and bad experiences in the past. In general they will tell you.

Lastly, another plug for the unbelievably fantastic podcast from Dr Gentile on pain management in the ED. Whilst not strictly about sickle disease you just know that if you did have sickle disease (or anything else painful) you would want Dr Gentile to look after you.

I am told that my posts might be too thought provoking where some people just want the answers. I think not. I’d rather write about things that we all find challenging. Where there are answers we can give them, but so much of our practice is dependent not just on the evidence but also on ourselves. We should never stop questioning the evidence, we should never stop questioning ourselves.

PS. This is our current management protocol for ‘unknown’ sickle patients. Time for a review I think, but even so this works pretty well at the moment. Advice on changes welcome.


Cite this article as: Simon Carley, "Sickle Cell Disease – New Guidance," in St.Emlyn's, July 21, 2012,

8 thoughts on “Sickle Cell Disease – New Guidance”

  1. While I totally sympathise with docs coming from elsewhere with little sickle in the population – I totally agree re pain management. Pain is subjective, and our only measure it what the patient says it is. While it might be seen as ‘cool’ to be cynical about patients who are complaining about 12/10 pain, I would rather give too much pain relief than not enough!

    The podcast quoted is indeed excellent – I incorporated this advice into my practice the day I heard it:

    “Would you like some more pain medicine?”

  2. We have a large sickle cell population that attends our paeds hospital and we see a fair share of crises in the ED. Another contributing factor is that some Drs are reticent about giving parenteral opiates to children – which is what these children require when they attend with severe pain.

  3. Absolutely John. What are you using as first line opiate? We would go for Intra-nasal diamorphine.


    PS. I give it 3 seconds before someone thinks about IN Ketamine….it seems to be the answer to everything at the moment 😉

  4. IV Morphine for severe cases (pain score >7/10). Moderates will get PO morphine if not settled with paracetamol, NSAID and codeine.
    But an RCT is underway here comparing IN Fentanyl with IV Morphine for the severe group – so watch this space

  5. My thoughts…
    1) >98% of sickle cell patients are genuine patients who really would rather be doing anything else other than presenting to the ED. The screaming opiate seekers are the extremely noisey minority.
    2) The vast majority of sickle cell sufferers know what works and what doesn’t and will tell you. Listen
    3) Realistically who will sit and give 2mg s/c every 3-4 minutes until it has an effect..?
    4) Those who suffer regular crises have crap veins. Don’t try to cannulate them for IV opiates, just give them s/c to get pain under control. It is far easier to spend 40 mins finding access in someone who is calm and pain controlled than someone in agony.
    5) If they are not from area, are very loud and demand morphine and cyclizine then by all means be suspicious, document, investigate (contact their local hospital) but if its their first time in your dept always give benefit of doubt and threat like anyone else regardless.
    6) Never forget simple measures like oxygen and warm blankets.

    DOI – EM Registrar who had privilege of being the Sickle Cell SHO some years back for Dr Jo Howard

  6. Pingback: SGEM#166: Which febrile child with sickle cell disease should get a chest x-ray? | The Skeptics Guide to Emergency Medicine

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