Expecting more for people with primary progressive multiple sclerosis
Written by: Professor Jerome de Seze, Department Head of Neurology and Clinical Investigation Centre at the University of Strasbourg, France
Over the past 20 years I’ve worked as a neurologist in multiple sclerosis (MS) focusing more recently on the primary progressive form of MS, also known as PPMS, a highly disabling form of MS.
When I look back to the early 2000s when I first entered the field of MS, there was about a four year delay between first experiencing symptoms of MS and a diagnosis – sometimes even longer for people with PPMS. Time to diagnosis has gradually improved, particularly in PPMS, due to advancements in diagnostic criteria, understanding of the disease and accessibility of magnetic resonance imaging (MRI).
Due to these clinical advances, more and more people are being diagnosed with PPMS.
Difficult to diagnose
Unlike in relapsing forms of MS (RMS), where there can be a new clinical symptom from one day to another, we see a steady progression of symptoms in PPMS, which are sometimes more difficult to recognise and attribute to MS. Difficulty walking is one of the main symptoms of PPMS, however physicians sometimes think this is due to rheumatisms (joint pain) or back problems, and not MS. Weakness (61%) and pain (59%) are also commonly reported as symptoms that have the greatest impact on people with PPMS1.
To understand the physical impact of MS, we measure the degree of disability using the Expanded Disability Status Scale (EDSS). This looks at different functional tests, such as walking ability and motor function.
A patient is usually already experiencing walking difficulties when we diagnose them with PPMS, unlike most patients with RMS, who are diagnosed earlier. If walking ability is affected, we conduct disability tests more regularly – around twice a year, to monitor disability progression.
Arm, wrist and hand function can also be affected during the course of PPMS, becoming more severe in later life. Around half of people living with PPMS experience upper limb disability.
Both difficulty walking and upper limb disability significantly impact everyday life for people with PPMS. My patients often talk of difficulties in daily tasks like cooking and eating, writing and using a computer. Most are keen to keep working, but these physical disabilities can mean they struggle to stay in work.
We should encourage people with MS to continue working and recommend they talk to their employers about adaptations they can make to help enable them to remain working for longer. 85% of people with PPMS have had their working lives impacted by MS, with 23% reporting that they had to give up work altogether due to MS1.
Limits on life
In my experience, people with RMS and people with PPMS tend to have similar limits on making long-term plans. People with RMS may have a new relapse that’s difficult to manage and cause them to cancel plans at the last-minute, while, people with PPMS experience unpredictable walking and upper limb disability, as well as urinary disturbances, which can significantly impact work and social plans. 39% of people with PPMS avoid making long term plans because they can’t predict how they will feel. 38% limit their social commitments due to the unpredictability of MS1.
People with PPMS are usually aware of the need to treat the cause – underlying disease activity – to limit disability progression that they may already be experiencing at diagnosis. With my patients, I try to reassure them that new disease-modifying therapies are in development.
However, there currently are no treatments approved for people with PPMS in Europe, and so I worry that by the time new therapies become available, their MS may have progressed further.
People with PPMS frequently discuss their walking and urinary problems. From one appointment to another, they say that their disability hasn’t gotten much worse, but when you ask them to reflect one to two years back, it’s clear there has been significant progression. They appear to accept the disability as the new ‘normal’.
It’s important we ask patients many questions about their MS symptoms during appointments, as well as involve support partners, who often remember much more about the changing of symptoms over time than the patient themselves. It’s hard to see the progression of the disease when you are living with it day after day. Asking patients to track the daily fluctuations in symptoms, particularly tracking changes in walking ability, would be hugely valuable to aid effective disease management in PPMS.
- MS MindSet Survey results.