End of life care
It is part of every doctor’s role to be involved in end of life care. In the early days of my career I recall trepidation at the thought of caring for patients who are dying and their families. As an inexperienced first year doctor, I was worried it would be such a difficult time for them and that the last thing they would need would be to talk to an inexperienced first year doctor. Over time, as I gained in experience, I realised how rewarding this aspect of care was. Working with families through a difficult time like this has challenges, but families are often thankful for your attention making their family member as pain free and comfortable as possible. End of life is a sensitive subject that can be difficult to approach. This article aims to open up this important aspect of healthcare for discussion within families by drawing on some of my observations and experiences and describing some of the research and options to consider.
The reluctance to discuss the end of an individual’s life has not always been as strong as it is for many people nowadays. Past cultures, such as the ancient Egyptians, were so pre-occupied with the afterlife and the importance of death, that they would spend their lives planning for what comes next. But it is rare for us, in our society, to think or talk about the end of our lives; yet it is something vitally important to get right. An apt way to put it is that death is one of the few life events that can only happen once to a person. This is an unusual way to think of death, but a highly relevant one when considering all aspects of the last moments of an individual’s life, from where they want to be, to decisions on management of their symptoms.
End of life care is also known as ‘palliative care’ and it is a subject that has been in the news several times over the past year. There has been ‘an assisted suicide bill’ debated in the house of commons, international surveys comparing practices in a number of countries and significant social media activity regarding individual cases of decisions around prolonging life or allowing death1. Conversations around end of life care can be very difficult for both health professionals and individuals and families to broach in short clinic appointments. News outlets, social media and the internet do not always help with this.
‘Day-to-day’ aspects of end of life care and decision making are rarely reported in the media, although in 2015 palliative care in the UK was voted to be the best among eighty other countries2. The study identified that both the quality and availability of UK services were among the best in the world. There were however, also areas highlighted in which the UK could improve, including how the NHS plans for end of life care.
SMA and life expectancy
There is a range of information available on the internet about life expectancy for those diagnosed with different types of SMA, some of which is conflicting. It is widely accepted that babies with SMA Type 1, the most severely affected, have a reduced lifespan, although this is improving with better care standards. Individuals with SMA Type 2 may have a reduced life expectancy, but with improvements in care, the majority of people can expect to live long and productive lives3. Individuals with SMA Type 3 typically do not have any overall reduction in their lifespan as a result of the condition3. However, as end of life care is likely to be important for everyone at some point, talking with healthcare professionals early on can help to ensure that the best possible care is considered and planned in advance.
The word ‘palliative’ comes from the Latin ‘Palliare’ which means ‘to cloak’. Put simply, the aim of palliative care is to protect individuals from pain or suffering. Being referred to palliative care means that you have a condition that for most people brings with it deteriorating health, however the referral may well be to help manage symptoms that are difficult to control or for other support. Referral to palliative care does not necessarily imply that there is expected to be deterioration in the near future. Similarly, ‘hospice’ is often an emotive and alarming word but being referred to one may be an opportunity for respite care, a chance to see a doctor specialising in symptom control or to access other specialist services such as hydrotherapy, a sensory room or counselling. If some of the negative associations can be removed from these words, people might be more ready to accept care and services that would greatly alleviate pain and distress.
Common symptoms of SMA that are treated by palliative care
The most common symptoms of SMA that can be treated with medication are feelings of shortness of breath, pain, anxiety and build-up of secretions (saliva or mucus in the throat).
-Shortness of breath
There can be many causes of shortness of breath (often also referred to as ‘Dyspnoea’); this can be due to an infection or difficulties breathing due to weakness of the muscles. It is uncomfortable to be unable to catch your breath and can lead to alarm. Garcia and colleagues identified shortness of breath as the most common symptom in infants with SMA Type 14. Shortness of breath may be helped with different types of morphine, a medication that is usually given for pain. Different types of morphine may also be used to reduce breathing rate. Medication such as this can be taken as a drink or via a tube into the stomach if required.
Pain can be alleviated with several medications and due to their side effects these should always be given with caution. The World Health Organisation (WHO) has a guideline for prescribing pain relief in a stepwise manner. This begins with medications that are available over the counter (such as paracetamol) and then increases in strength to morphine and other medications. The stronger medications have strict doses and regulations. Individuals may develop a ‘tolerance’ to these stronger medications, meaning that they require higher doses to get the same pain-relieving effect.
-Build-up of secretions
Secretions such as mucus and saliva can build up in the airways and mouth, either causing drooling or a rattling noise when breathing. Relief is usually achieved with medications called hyoscine or glycopyrronium - two medications that work by affecting receptors in the body, which causes a reduction in secretion of water from cells. Studies comparing these medications have shown them both to be effective at reducing secretions5.
-Emergency care plans
Emergency care plans are being increasingly used to identify what should be done in the event of an emergency or a sudden worsening of a condition. The idea is that the team of doctors and nurses that know the condition best may not work locally, or simply may not be at work in the hospital when an individual develops a sudden illness. Also, it recognises that an individual, or the parents of a child at a time of crisis, are unlikely to be in a position to be able to advise of their wishes and discuss the complexities of treatment options. In this event the doctors and nurses who are present on the day need to be clear about what to do and what has been previously discussed and agreed upon with the individual and/or the family. These emergency care plans are not binding and can be changed by the individual or family at any time.
Emergency care plans (ECPs) are included as a requirement of best practice within the standards of care for some types of SMA. ECPs are widely used in the UK and USA and are advised by several local authorities. Local templates can often be found on council websites. Studies looking at the use of ECPs have reported that users who required emergency care and their families found them useful6. In this study by Jones and colleagues they did report that some families found the conversations around ‘levels of care’ (how much care would be given in the event of a sudden illness) difficult6.
When to talk about end of life care?
SMA is considered to be a spectrum of disease, meaning that there is a wide variation of impact and severity within each ‘type’; therefore, making this a difficult question to answer. Medical professionals have often had discussions about changing the classification of Type 1, 2, or 3 to another system which appreciates the wide differences between individuals. An example of this would be the differences between infants with very severe SMA Type 1, who struggle to breathe from birth, to an infant who has less severe respiratory problems and is not diagnosed until six months of age. The wide variation in symptoms and progression makes it difficult to know when starting to talk about end of life care would be the most appropriate. Obviously there is no ‘one size fits all’ guideline as to when to involve palliative care teams.
As mentioned earlier, it is best practice for end of life discussions and planning to begin early following a diagnosis of SMA Type 1, but there are a lot of interventions and care that can still occur and which may prolong life. The very difficult questions and decisions families still face, however, include, ‘How much is too much for an infant to go through?’, ‘Should we consider all treatment options every time our child gets sick?’ or ‘Should we say ‘no’ to antibiotics, ventilation or other supportive therapies?’ These are extremely difficult decisions to make and underline why early discussions are necessary between a family and doctor or other professionals.
Studies looking at individual and family perspectives have given support to early discussions allowing families to plan ahead7-9. Current guidelines imply that these conversations should begin from the point of diagnosis for families of children with SMA Type 17.These conversations can range, for example, from initially ensuring a family has an awareness of palliative care services and the possibility of access to local hospices, through to developing a specific care plan in the event that a baby’s health suddenly gets worse.
A study by Monterosso and colleagues, interviewed 28 families of children with life-limiting, non-cancer-related diseases. This study highlighted the importance of families being told the truth about their condition at an early stage. A clear barrier to seeking palliative care was that they felt they first needed to accept that their child was not going to survive10. Another similar study looking at SMA involved interviews of families, highlighted that around end of life there may be a lack of co-ordination of care, consideration of siblings and bereavement support11.
Another important part of any discussion may be choosing, if possible, where to die. A study in 2015 by Garcia and colleagues4, reviewed the care of nine infants with SMA Type 1 and found that end of life care was possible at home for all of them and that this was important to the families involved.
End of life discussion may also be helpful for those with other types of SMA which bring with them severe health care challenges and the potential of significant or critical episodes. As described above, it is best practice for anyone in this position to have a personal emergency care plan in place. These discussion times can be a helpful time to think about these ‘what if’ possibilities and talk about preferred palliative care options.
End of life care is a difficult topic. I hope this article may help individuals and families with their thinking and with their discussions with healthcare professionals. It is important to view palliative services and hospices as facilities that specialise in providing care and helping reduce symptoms in individuals who need this. Fear of such services and discussions around end of life is perfectly natural but can form a barrier which may prevent individuals from getting care that may help them. It is important to remember that it isn’t necessary to completely come to terms with what is going to happen in order to seek help for symptoms. For families with infants with SMA Type 1, it is important that conversations about end of life planning happen early. For anyone affected by other forms of SMA who may have to manage life threatening crises, it is important that everyone knows what course of action is required and preferred in an emergency.
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