What does it mean to be diagnosed with primary progressive MS?
Multiple sclerosis is a lifelong condition which affects the central nervous system (the brain and spinal cord). MS affects everyone differently and there’s a wide range of possible symptoms. There are different types of MS and the type you're diagnosed with depends on the pattern of your symptoms over time.
Here are some key facts about primary progressive MS.
What is primary progressive MS?
About 1 in 8 people diagnosed with MS are diagnosed with primary progressive MS (often abbreviated to PPMS).
In this type of MS symptoms gradually worsen and accumulate over time, known as progression. In primary progressive MS this progression occurs from the outset of the condition, this is why it is called 'primary' – because progression happens first. This contrasts with secondary progressive MS where an initial relapsing remitting phase is followed by a progressive phase. With PPMS it's rare to have any relapses.
The rate at which symptoms become more severe varies, but typically the changes are slow and gradual over a number of years. There can also be periods of minor improvement or times when symptoms stay the same. However, for some people the changes can be more rapid.
Who gets primary progressive MS?
It is estimated that 10-15,000 people have primary progressive MS in the UK. Generally primary progressive MS is diagnosed when someone is in their 40s or 50s.
Relapsing remitting MS is usually diagnosed under the age of 40. People with relapsing remitting MS typically move into the secondary progressive phase about the same time as others are diagnosed with primary progressive MS.
About equal numbers of men and women are diagnosed with primary progressive MS whereas relapsing remitting MS and secondary progressive MS are more common in women than men.
Other ways your MS might be described
Increasingly the way MS is described is changing. You may also hear the following terms to describe what is happening with your MS at a specific point in time:
- active or not active - these terms are used to describe whether or not you're having relapses and/or if new lesions (characterised by inflammation and demyelination) can be seen on your MRI scan
- with progression or without progression - used in progressive MS to describe whether your symptoms are increasing or staying the same over a period of time.
You might think that active MS would only apply to someone with relapsing remitting MS. However, although inflammation is seen in PPMS to a lesser extent than in RRMS and SPMS, it can be present - particularly in the first few years after diagnosis. Some people with PPMS also experience a single relapse. In these circumstances PPMS would be described as active. If you have no evidence of inflammation on your MRI and don't experience relapses your MS would be described as not active.
What are the symptoms?
In more than 8 out of 10 people with primary progressive MS, the first symptom experienced is stiffness, weakness or a heavy feeling in your legs.
It gradually gets worse and results in minor problems, such as tripping on kerbs or when going up steps. You might also experience unsteadiness or problems with your balance or coordination which can contribute to more obvious walking difficulties.
Bladder problems or bowel problems are quite common in primary progressive MS. They may also be linked to sexual problems in both men and women such as maintaining an erection, decreased sex drive or you may find you have less genital sensation.
These symptoms are commonly seen together as they're all caused by MS lesions in the spinal cord.
Fatigue is also very common. This is not normal tiredness but an exhaustion which is out of all proportion to what you've been doing. Fatigue can be mental or physical tiredness.
There's a wide range of other symptoms which people with primary progressive MS may experience including spasticity and spasms, cognitive problems and emotional difficulties such as anxiety or altered mood. As with all types of MS, the symptoms vary a lot from one person to another and not everyone will have all of them. Symptoms may also vary from day to day. This makes MS rather unpredictable and can take some getting used to. Not all MS symptoms are obvious to other people even though they may have a significant impact on your life. They're often referred to as invisible symptoms.
What treatments are there?
Some people have the impression that there are no treatments for people with primary progressive MS. This is not true.
There's a wide range of treatment options available for individual symptoms and, for some people with early or active primary progressive MS, a disease modifying drug (DMD) called Ocrevus (ocrelizumab) may be appropriate. You can read more about Ocrevus for PPMS here. There are more disease modifying drugs in development for PPMS, so hopefully in the future there will be more choice of treatments to treat the underlying condition, or change the course of primary progressive MS.
Although symptomatic treatments can't change the course of your MS, they can help relieve the physical or mental symptoms. Options for PPMS include:
- drug treatments - such as medication for pain, spasticity or bladder issues
- therapies - like physiotherapy to help with muscle stiffness, or cognitive behavioural therapy for pain, anxiety or depression
- prevention of complications - such as treatment for bladder infections
- management techniques - for example pacing yourself to minimise fatigue
- rehabilitation services - which can help you to live life as fully as possible and provide practical support to overcome barriers to your daily activities. The services you interact with will vary depending on your personal needs but might include services such as physiotherapy, speech and language therapy, cognitive rehabilitation therapy, or occupational therapy which looks at activities you find difficult to see if there are other ways you can do them such as by making simple lifestyle changes, or whether there are useful aids, equipment or adaptations that can help
- promoting general health and wellbeing - such as advice on diet and exercise.
If your symptoms are changing, you can ask for a review if you haven't got an appointment lined up fairly soon.
Living with primary progressive MS
Living with primary progressive MS can mean living with a degree of uncertainty. Symptoms can sometimes get more or less intense, or fluctuate, over time so you may be concerned about how well you’ll be on a particular day. People living with MS say that the best approach is to put aside your worries as much as possible, live each day as it comes and deal with symptoms if, and when, they happen. A change in your symptoms doesn't always mean that your MS is getting worse.
It’s important to look after your overall health which includes your mental wellbeing as well as your physical health. Taking good care of yourself will help you to continue to do the things you enjoy, as well as the things you have to do. A well-balanced diet, keeping your brain and body as active as possible, drinking sensibly and not smoking will help you stay well and manage some symptoms. It's also important to go for health checks, such as cancer screening, and any vaccinations you're offered.
You may be concerned that your MS will get worse very rapidly but this is rarely the case. For most people the changes are very gradual over a number of years.
MS isn't a terminal condition and it's rarely a direct cause of death, but you will live with it for the rest of your life. Most people with MS live into old age, although lifespan, on average, is about six years less than the general population. If you're more severely affected by your MS, you might be more susceptible to developing infections or complications that affect your swallowing or breathing. This can decrease your life expectancy, so it’s important to keep as well as possible and seek treatment promptly when needed.
Being diagnosed with primary progressive MS sets you on a lifelong journey. It will take time to learn about your MS, how to manage it well and to become your own expert.
None of us know what life has in store for us. It’s good to live in the here and now and make the most of today. Try not to focus too much on what might happen or assume that MS will have an effect on a particular aspect of your life. This time and energy could be spent in a more positive way on something that’s important to you right now.
Diaries and reviews
You might find it helpful to keep a simple diary to keep a record of your symptoms. You could make a note of any new or worsening symptoms, when they happened and whether they went away or persisted. You could include when you started or stopped any medication and whether this improved your symptoms or caused any side effects.
A diary could be paper-based or an app on your phone. It can be useful to use something that’s easy to take to your appointments so you can share a summary with your MS team.
It's recommended that you have a review of your MS once a year but if you have a substantial change in your symptoms you might be seen more often. It can be good to look back over the previous year and see if you need to discuss anything in particular with your MS team. A summary of your diary can help them see at a glance what’s happening with your MS and then advise and support you better. You could add the dates and outcomes of your appointments so you have a record of what was agreed.
If you think your review is overdue, or you've experienced significant changes to your MS, consider contacting your MS team or GP rather than waiting for the system to contact you.
Is there disability with primary progressive MS?
You may be concerned that primary progressive MS will impact on your ability to do what you want, when you want. It’s natural to worry about whether it will change your physical capabilities or if you’ll become disabled. Although progression does mean that disability will continue to increase over time, for many people it will occur slowly over many years and doesn’t mean that you will inevitably experience a rapid decline in your health or capabilities. However, regrettably for some people, primary progressive MS can lead to significant disability.
What is disability?
Disability means different things to different people. What one person considers disabling another may not.
The medical model of disability defines disability as the impact of a physical or mental condition on your ability to do daily activities. This might be something as simple as numbness in your fingertips making it hard to fasten a button. Some people might class this as a disability but many wouldn’t, even though this symptom does impact on their lives.
Typically people consider disability to be difficulties with mobility and tend to look for outward symbols such as a walking stick or wheelchair. This isn’t surprising, given that the symbol of a wheelchair is often used to represent disability. However, disability can include less visible symptoms such as fatigue or pain.
People are becoming increasingly aware of the social model of disability which, rather than focusing on the condition, considers the wider barriers in society and your environment which disable you. This might include negative attitudes, a lack of understanding about your condition, or physical barriers such as a lack of public toilets making it difficult to go out if you have bladder issues. This highlights the need for society to change to create equality, more independence and choice for everyone.
Will I become disabled?
As everyone's MS is different, it's difficult to predict how much your MS will affect you. You’ll have your own way of thinking about disability and it will be up to you to choose how you define yourself. MS may affect what you can do but only you can decide if this is disabling. As the symptoms of MS vary so much, you may have different views of yourself at different times.
If symptoms affect your mobility, a walking stick or wheelchair can be helpful. In reality, three quarters of people with MS never use a wheelchair full time and this number is expected to change because the care of people with MS has improved, and this is reducing the level of disability experienced.
Many symptoms can be helped by trying different treatments. For some activities, it may be possible to find a slightly different way to do things so you can carry on doing the things you enjoy. For example, you might choose to sit on a stool or chair to prepare food or do the ironing so you're left with enough energy later in the day to go and see a friend or do some exercise.
Although the concept of being disabled may feel uncomfortable or inappropriate, it can help you access support. The Equality Act covers you as soon as you’re diagnosed with MS, even though you may not see yourself as disabled. It covers both physical difficulties and symptoms that affect your thinking.
This can be particularly helpful at work, for example if you need any adjustments made to be able to carry on with your role. This might include allowing you to return to work by gradually increasing your hours or, if heat makes your symptoms worse, allowing you to sit in a cooler area of the office.
If you do have extra care or mobility needs as a result of living with MS, there may be benefits, grants or other financial support available to you. Some benefits are awarded based on the impact of your MS rather than being linked to your level of income or savings. Social care services can carry out an assessment if you need support to help you maintain your independence.
Primary progressive MS book
Explores the physical and emotional aspects of living with primary progressive MS and the ongoing management of the condition.
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