If you don’t have advanced MS, you may find the contents of this newsletter upsetting. It is not for everyone. Only read on if you want to know about managing advanced MS.

Question

I am at EDSS 8 and need to get my affairs in order. I cannot do anything really anymore, so is this the beginning of the final stages? I know you won’t sugarcoat the reply, so? I can no longer go to the toilet on my own and need to use the Peristeen system with my partner’s help. My partner uses a hoist to move me to and from bed/chair, etc. I have just had pneumonia, and the doctors said if I had a heart attack, they would not step in on medical grounds, even though I protested. What would you advise?

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Prof G’s response

Thank you for your questions and for being so honest about what you are going through. I get the sense that you are exhausted and anxious. I am sorry you are having to navigate such a heavy burden, especially after battling pneumonia and having such a distressing conversation with your doctors about end-of-life care. These conversations are best conducted well in advance, not during an acute crisis. This is why I urge all of you to complete an advance directive early on in your disease course and make sure your family knows about it and has a copy.

You asked me not to sugarcoat things, so I will give it to you straight. Reaching EDSS 8 is a significant and difficult reality. It represents the advanced stages of physical disability in MS, which is why you are facing the loss of independence, needing the hoist, relying on the Peristeen system and your wife’s support. However, being in this advanced stage of disability does not necessarily mean you are in the final weeks or months of your life. Many people live for years and decades with an EDSS of 8. However, it does mean you are more vulnerable to complications, as evidenced by the flu.

Given that vulnerability, you are right to get your affairs in order. Doing so is not a sign that you are giving up or that the end is near. It is simply about taking back some control, ensuring your exact wishes are legally documented, and giving you and your wife some peace of mind so you don’t have to worry about the administrative side later.

Regarding the doctors telling you they would not step in if you had a heart attack or, by implication, another life-threatening event, I can only imagine how surprising or jarring and angering that was, especially as you protested. In the UK medical system, doctors can legally place a “Do Not Attempt Cardiopulmonary Resuscitation” decision on your file if they clinically believe the severe physical trauma of CPR would not be successful or would cause you more suffering due to your body’s current frailty and only if you and your family consent to it happening. However, your voice still matters. You are entirely within your rights to ask for a second medical opinion regarding this decision, to complete an advanced directive contrary to their advice and to demand a clearer conversation about your ongoing care plan going forward.

Please try to be mindful of yourself. Your feelings and frustrations are valid, but anger will not help. Your neurological team should be there to support you through this. I would also recommend that they refer you to the palliative care team that has access to community services that can help you. For example, you would benefit from some form of counselling.

As you are aware, from a treatment perspective, we don’t have any therapies to stop your disability from worsening or to reverse it. We think treatments such as cladribine may slow the rate of worsening, which is why we are conducting the CHARIOT-MS trial in advanced MS. We also know that comorbidities such as recurrent infections, pressure sores, poor metabolic health, poor sleep hygiene, low mood, etc., worsen MS. This is why you, your GP, and your local MS service need to focus on optimising the small things.

I have looked after many people with advanced MS who have done this, and when the small things are looked after, they often notice an improvement in their quality of life.

I hope this helps.

I now realise the information I have given you is quite superficial. I have therefore produced a longer newsletter that covers topics you and others with more advanced MS may find helpful.

Advanced MS

As you know, the EDSS ranges from 0.0, indicating a normal neurological examination, to 10.0, indicating death due to MS. The transition to an EDSS score of 8.0 represents a watershed in the clinical trajectory. At an EDSS of 8.0, the pwMS is usually restricted to a bed or a chair, or requires assistance in a wheelchair for mobility. While they may be out of bed for much of the day, their independent ambulatory capacity is usually curtailed, unless they have an electronically controlled wheelchair. At EDSS 8.0, pwMS generally retain effective use of their arms, with some preservation of many self-care functions. This clinical profile distinguishes them from pwMS at EDSS 8.5, who are restricted to bed for much of the day with only some effective arm use, and those at EDSS 9.0 or 9.5, who are totally confined to bed and face severe bulbar dysfunction, including problems with communication and swallowing.

Because pwMS at EDSS 8.0 retain upper-extremity function and cognitive awareness, preserving their autonomy is a clinical priority and the reason we launched our #ThinkHand campaign almost a decade ago. We don’t think pwMS who are wheelchair users should be ignored. As part of the #ThinkHand campaign, we have been involved in designing and starting the O’HAND (ocrelizumab in PPMS), CHARIOT-MS (cladribine in advanced MS) and OCTOPUS trials. All these trials use the 9-hole peg test as the primary outcome measure or part of the composite endpoint.

When you lose the use of your legs, your arms and hands function are essential for mobility, and explain why pwMS with advanced MS prioritise upper extremity function over lower limb function.

At this stage of advanced MS, care must focus on supporting daily living and preventing complications (preventive neurology). The philosophy I promote relies heavily on the aggregation of marginal gains—the understanding that meticulous attention to seemingly “small things” yields improvements in the pwMS’s dignity, survival, and psychological well-being. Furthermore, the relationship between the pwMS and their carer(s) becomes a central focus of care. The psychological resilience, physical safety, and emotional well-being of the carer are inextricably linked to pwMS outcomes, necessitating support, respite strategies, and environmental adaptations to mitigate the burden of providing chronic care.

Comorbidities

The management of a wheelchair-bound or bedbound pwMS does not occur in a vacuum. People living with advanced MS exhibit a higher incidence of comorbidities compared to the general population. The most prevalent conditions are high blood pressure, diabetes, heart disease, fibromyalgia, chronic lung disease, and mood disorders such as depression and anxiety.

The presence of these comorbidities has been clinically linked to a faster worsening of disability, reduced overall quality of life, more frequent hospital admissions, and a regrettable shorter lifespan. Clinical studies indicate that pwMS who also suffer from vascular disease tend to require assistance with walking much sooner than those without vascular comorbidities. Therefore, the active management of metabolic syndrome and vascular health should not be peripheral to MS care, but a central part of it.

Because a pwMS with EDSS 8.0 is often entirely sedentary, their risk for developing metabolic syndrome—a constellation of high blood pressure, elevated blood glucose, excess body fat around the waist, and abnormal cholesterol levels—increases due to a forced sedentary lifestyle. Maintaining a good relationship with your general practice or a primary care provider is, therefore, essential as most MS services don’t have the resources and expertise to manage these comorbidities.

Respiratory function

Respiratory complications are recognised as one of the leading causes of death in pwMS, accounting for approximately 50% of all deaths in longitudinal studies. Emergency, unplanned, or non-elective admissions for respiratory crises in pwMS cost an average of £216,000 annually per NHS Clinical Commissioning Group (CCG), with £75,000 attributed to admissions for aspiration pneumonia. Prior to their dissolution, there were 106 CCGs in England; therefore, the cost to NHS England was ~£23M annually for this problem alone.

The decline in respiratory function in MS is typically insidious and begins long before the pwMS reaches an EDSS of 8.0. Pulmonary function is affected even in the early stages of disability, primarily through weakening of core stabiliser muscles. This reduction in core strength translates into impaired pulmonary mechanics, diminished health-related quality of life, and worsening fatigue. However, the transition to a wheelchair-bound or bedbound state precipitates a much sharper decline in respiratory function. This manifests clinically as a weak and ineffective cough. When the expiratory muscles fail to generate sufficient force to clear the airway, and the inspiratory muscles are too weak to provide adequ...