Author: Liz Herrievan / Editor: Charlotte Davies / Codes: CC1, CC11, CC24 / Published: 13/11/18
Before my daughter came along I knew very few people with learning disabilities. Of course I saw people with LD at work, but I’m afraid I was pretty ignorant of the issues they faced and didn’t really attempt to think about how I could make their visit to the ED less problematic. My priority was assessing, diagnosing and managing in a short a time frame as possible and I stuck to the same systems I used with all my other patients.
Then Amy arrived.
She has Down syndrome and autism and, over the last 12 years, has taught me more than I could ever have imagined about learning disabilities, patience, determination and prioritization.
Approximately 2% of adults have a mild intellectual disability and 0.35% have a severe or profound disability. There are many, many causes for these disabilities including chromosomal problems, congenital infections, neurodegenerative conditions, post-traumatic conditions and those which we still can’t properly explain but which definitely aren’t caused by vaccines. Many of these, as well as causing learning difficulties, also have physical symptoms and comorbidities, so you will meet plenty of people with LD in the ED. Some conditions causing LD are “invisible” – you may find no obvious physical clues that your patient has difficulties which may impact on your assessment and management. Conversely, some patients who appear very physically disabled may have no learning disability. It’s so easy to get basic things like communication wrong, making the rest of our job so much harder.
People with LD die, on average, 20-25 years earlier than people without LD. Median life expectancy for everyone is increasing, but that gap isn’t narrowing. CIPOLD (the confidential inquiry into premature deaths of people with learning disability) found many reasons for this, including reduced access to screening and interventions for conditions such as obesity, low uptake of vaccinations including the ‘flu jab and delays in investigations, diagnoses and treatments. The biggest discrepancy between people with LD and those without, with regards to cause of death, is that those with LD are three times more likely to die from something amenable to treatment, compared to those without (most often a respiratory infection). As a doctor, this saddens and angers me. We need to do better.
The ED Environment
When an emergency physician enters an ED we know where we are. We belong. The noise, the hustle and bustle, the general organized chaos feels strangely comforting and familiar. To everyone else though, it can be terrifying. Add in being in pain or feeling unwell. Then add in a lack of understanding, sensory processing difficulties and a loss of your usual routine (which might be the only thing that gets you through the day) and we’re getting close to how a patient with LD might feel when they arrive at the ED. Sensory processing problems are common, particularly in autistic spectrum disorders. Sudden or loud noises can be overwhelming and there’s plenty of those in the ED – buzzers, alarms, beeping monitors, ringing telephones, shouting, crying, screaming… People with LD can find it almost impossible to filter out sensory stimulation.
It can make the world of difference if the patient and their carers are offered somewhere quiet to wait or a place to escape to when the waiting room gets too much. Waiting itself can be a massive problem, particularly with conditions such as ADHD. I’m not suggesting patients jump the queue purely because they have a learning disability, but wherever possible explaining the situation, keeping carers updated and providing an appropriate environment to wait in can go a long way to helping the situation.
This Canadian toolkit is a really useful resource for helping to ensure your ED is LD friendly.
We commonly provide forms of translation for patients who do not speak English. We need to think of people with LD in the same light. Depending on the specific type of LD, people may have a difficulty with speech and language to a greater or lesser extent. As clinicians we have a duty to find out how best to communicate with our patients. Ask what you can do to help them. Some people, such as those with Down syndrome, may have a relative strength in understanding visual media such as signs or symbols but find understanding and remembering auditory information far more difficult. Would using gesture help your patient? Could you use pictures to help explain procedures or treatments? Do you need a British Sign Language translator or does your patient use Makaton (a more simple system, with many signs similar to common gestures)? Keep speech clear and emphasize key words. Wherever possible, keep background noise to a minimum to enable your patient to concentrate. Use simple, one-step instructions. Break things down – explain what will happen, when, how, who will be involved. Avoid jargon. Give time for processing information and for formulating a reply. Your patient may need several seconds to understand what you have asked, decide on an answer and form the words or gestures required to give you that answer. Don’t rush them. Pay close attention to non-verbal communication – sounds, facial expressions, body positions. Wherever possible ask for input from family or carers. They will know far more about how their loved one communicates than you do and may pick up on cues that indicate pain, anxiety or discomfort. Pain is a difficult concept for anyone to describe, but may express itself as aggression, agitation or restlessness or your patient may instead become withdrawn or tearful. For more on communication read this great link.
Some people with LD understand far more than they are able to communicate. Do not assume your patient cannot understand you, given help. As @Alan_Saunders57 tweeted:
Being unable to think quickly does not make you stupid. Speaking quickly before thinking does. Tell them to look in the mirror.
— Alan Saunders (@Alan_Saunders57) June 22, 2018
It may sound obvious, but don’t forget to talk directly to your patient. Read this, by a young woman with Down syndrome.
Does your patient have a Hospital Passport? These are similar to the “All About Me” documents you may be familiar with from patients with dementia. If possible, get it entered onto their electronic patient record. Do they have an emergency care plan? Both these things can save patients and carers from having to answer the same questions over and over again and can help you prepare for your consultation.
Don’t be afraid to admit what you don’t know. We can’t know everything about everything and families or carers may be able to give you important information about the condition your patient lives with. Similarly, a few moments on Google can help to guide you in the right direction particularly with some of the rarer conditions. For infographics on two rare conditions, Kleefstra syndrome and Pitts Hopkins syndrome, plus one on Down syndrome, take a look here.
The Learning Disabilities Mortality Review (LeDeR) programme revealed that problems with early identification of serious illness are common. Families are often better than clinicians at recognizing that their loved one is unwell, but we need to listen to them and empower them to be able to give us the information we need. More on the LeDeR programme can be found here.
Depending on the cause of your patient’s learning disability, they may not present how you would expect them to when unwell. People with neurological conditions may not mount a tachycardia or pyrexia. Your patient may not be able to explicitly describe their symptoms. They may have comorbidities which mean that their usual vital signs or NEWS score are not in the same range as everyone elses.
It’s important not to assume, however, that things like abnormal oxygen saturations or blood pressure, respiratory secretions, hypotonia or poor peripheral perfusion are “normal for them”. Wherever possible try to find out where your patient’s normal baseline lies. Family and carers are invaluable for this, as is any documentation such as the hospital passport or previous clinic letters.
All this means that our usual assessment tools such as sepsis screens can’t always be relied upon. We may have to look for “softer signs” instead – difficulty in urinating, changes in gait or inability to weightbear, new confusion, reduced appetite, family or carer concern, for example.
Causes for any change in behaviour should be sought rigorously. It’s not enough to assume they are due to the underlying condition or an emotional disturbance. Altered behaviour may be due to pain, nausea, lightheadedness, seizures, changes in visual acuity…. You get the picture. Of course, you will only know that behaviour has changed if you know what the normal baseline is for your patient – again, don’t be afraid to ask family and carers if what you are seeing is normal for their loved one. There’s more on diagnostic overshadowing, where new symptoms are attributed to a pre-existing diagnosis, here: http://www.intellectualdisability.info/changing-values/diagnostic-overshadowing-see-beyond-the-diagnosis
The practical aspects of assessing your patient can be challenging. Things which are second nature to us, such as saturation probes or blood pressure cuffs, might be terrifying for someone with LD. Sensory processing disorders may make these things actually painful for some people. For some they will be unfamiliar and for others far too familiar and associated with previous bad experiences. Some people may have specific issues with personal space or being touched. Take time to explain things whenever possible, demonstrate equipment or show pictures and use things like distraction or getting family members to help you. Leave uncomfortable or distressing interventions until the end of your assessment. There’s some great work going on in the community involving desensitization for procedures such as blood pressure monitoring and there is easy-read information available for people with LD about what to expect in hospital.
Do what EM physicians do best – a good A-E assessment – but be prepared to adjust things slightly where needed. You may need to be opportunistic in your examination, especially in patients with sensory processing difficulties, for example starting with a “less threatening” body area and working centrally, or listening to the chest whilst your patient is settled and quiet. Be flexible and resourceful – another quality of EM docs – embrace the challenge, don’t run from it.
When taking blood, consider topical anaesthetic creams and ultrasound or vein viewers. Make this experience as easy as possible for both yourself and your patient. Do take bloods though, if they are needed. Do get access if it is needed. I recently heard a story about a patient with LD who was left with a low blood pressure for several hours because no-one wanted to upset them to get intravenous access. Another patient had to suffer 17 attempts at cannulation – for any other patient wouldn’t we have opted for central access or intraosseous by this time, depending on the situation?
Don’t forget that some conditions which cause LD may be associated with specific comorbidities which may affect presentation or risk of serious illness. Beware medication interactions, particularly with drugs you may be unfamiliar with.
Consent and Information
The Mental Capacity Act of 2005 states that people should be given help to make their own decisions, wherever possible. This may mean providing information in an easy-read format, using photos, pictures or diagrams, getting a sign language interpreter to the department, asking family members to help relay information or simply just allowing a little more time for decision making. If you think it might be useful for your department to have some easy-read copies of frequently-used paperwork such as information leaflets or posters, this guide is pretty easy to read itself!
If your patient is unable to make a particular decision for themselves, then it is imperative that any decisions made for them are in their best interests. Don’t make assumptions about your patient’s quality of life. Speak to people who know them. Families, carers and hospital passports can all help with this. Can you get hold of the Consultant who regularly cares for them? Find out if there’s an emergency care plan in place. Learning disability liaison nurses can be an invaluable source of information and support. Involve senior colleagues where needed and don’t forget the Independent Mental Capacity Advocate Service if your patient has no other form of support. Remember capacity is decision-specific and can vary with time. Does the decision have to be made now? Can it wait until your patient is better able to understand? Is there a less restrictive management option? For more on the MCA see here.
Treatment and Discharge
Consider whether your patient might need sedation or distraction for any procedures. Remember that just because pain isn’t verbalized it doesn’t mean it isn’t felt. If giving medications consider whether your patient might need a suspension or soluble form rather than a tablet. Make sure any instructions about how and when to take them are clear and simple.
The LeDeR programme has identified issues with recognition of illness by people with LD themselves. Specific and understandable safety-netting advice must be given. The ED can be incredibly busy and there are many demands on our time but, when possible, giving health promotion advice has to be part of every doctor’s job description.
Families can be your biggest asset when caring for a patient with LD. They know their loved one and will pick up on soft signs of illness that aren’t so obvious to others. They’ll be able to help you with communication, examination and procedures. Listen to them, respect them and gain their trust.
People with LD often have far more outpatient visits and hospital appointments than is healthy. Even just contemplating a trip to the ED with a loved one with LD can be exhausting for families – transport issues, loss of routine, sensory overload, equipment for those who need portable oxygen, suction, ventilation….these families don’t take their loved ones to the ED unless they believe it absolutely necessary.
Kristine Flint, mum to Nieve, a young lady with Rett syndrome who has spent far too much time being unwell, has been working hard to raise awareness of sepsis in people with LD. She says:
“Please listen to us when we say something is not right, we can see the subtle changes in her health and behaviour that may not be apparent to the casual observer or even health professionals like yourselves. Nieve, and children like her, cannot speak for themselves therefore as parents we have to ensure that we advocate for them in the strongest possible terms. We do not think we are better than the team, nor are we full of our own self importance, but we are simply trying to give a voice to our children as they don’t have one of their own.”
That seemingly “difficult” family in cubicle 3 may be scared they won’t be listened to, worried about their loved one, reliving previous bad experiences or all three. Families are sadly used to having to fight for services and support. It’s an exhausting, lonely, 24/7 job. Acknowledge this and their experience and work together for your patient. This article has some more great insights from parents of children with LD.
Top Tips for caring for people with LD in the ED
In summary, considering a few simple things when managing a patient with LD can make the whole process much more rewarding for everyone concerned:
Huge thanks to Kristine Flint, Jim Blair, Helen Laverty, Suzanne McCloud, Paul Herrieven and all the lovely people on Twitter who suggested tips for EM physicians caring for patients with LD.