Primary progressive MS

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). There are different types of MS and the type you're diagnosed with depends on the pattern of your symptoms over time.

Traditionally MS has been split into three types: relapsing remitting MS (RRMS), primary progressive MS (PPMS) and secondary progressive MS (SPMS). Each type of MS encompasses a wide range of experiences of MS. 

Here are some key facts about primary progressive MS.

What is primary progressive MS?

About 10-15 in 100 people diagnosed with MS are diagnosed with primary progressive MS (PPMS).

In this type of MS, symptoms gradually worsen and accumulate over time, known as progression. In primary progressive MS this progression occurs primarily from the outset of the condition, which 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, though for some it can be more rapid. There can also be periods of minor improvement or times when symptoms stay the same.

Who gets primary progressive MS?

It is estimated that 10-15,000 people have primary progressive MS in the UK. Primary progressive MS is typically 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.

A diagnosis of primary progressive MS is often a clinical diagnosis – based on your medical history and the symptoms you're experiencing, rather than on diagnostic tests. A neurologist will be looking for a pattern of worsening symptoms due to the slow and progressive loss of nerve cells (neurodegeneration).

Other ways your MS might be described

Increasingly the way MS is described is changing. The following terms are being used more widely to describe what is happening at an individual level, 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 of primary progressive MS?

There's a wide range of possible symptoms which vary from person to person. The symptoms you experience depend on where MS has caused damage in your brain and spinal cord (central nervous system).

In more than 8 out of 10 people with primary progressive MS, the first symptom experienced is difficulty walking. This might be stiffness, weakness or a heavy feeling in your legs which gradually gets worse and can lead to 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, loss of desire 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.

Some other common symptoms are:

  • fatigue – this is not normal tiredness but an exhaustion which is out of all proportion to what you've been doing, it can be mental or physical tiredness
  • spasticity and spasms – stiffness of the muscles
  • cognitive problems – which might include problems with concentration, memory or planning
  • emotional difficulties – feelings of anxiety or altered mood
  • altered sensations – unusual feelings in the skin such as numbness, tingling or burning sensations, including a type of pain known as neuropathic or nerve pain.

Some symptoms of MS such as fatigue and altered sensations, aren't always 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 for primary progressive MS?

Often people with primary progressive MS are under the impression that there aren't any treatments for them. This is not true. There's a wide range of treatment options for individual symptoms and, for some people with early or active primary progressive MS, a disease modifying drug (DMD) may be appropriate. 

Symptomatic treatments

Symptomatic treatments help relieve the physical or mental symptoms of a condition. They don't treat the underlying cause, or change the course of the condition itself. Symptomatic treatments for primary progressive MS 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
  • management techniques – for example pacing yourself to minimise fatigue
  • rehabilitation – 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
  • complementary and alternative medicines (CAMs) – for example acupuncture, Pilates and relaxation techniques such as mindfulness or meditation. These are treatments which fall outside conventional medicine but are generally accepted as being helpful in improving physical and mental wellbeing.

Learn more about treating MS symptoms.

Disease modifying drugs

Disease modifying drugs (DMDs) treat the underlying condition, or change the course of the condition itself.  There is currently only one DMD, called Ocrevus (ocrelizumab), available for people with primary progressive MS. If your PPMS is active, you've had it for 15 years or less (early), and you can walk at least 20 metres (with or without a walking aid) you may be eligible for Ocrevus. You can read more about Ocrevus for PPMS.

If you've had primary progressive MS for more than 15 years, your MS isn't active and you're unable to walk a short distance then it's unlike you'll be offered Ocrevus.

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.

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. A change in your symptoms doesn't always mean that your MS is getting worse, but you may be concerned about how well you’ll feel on a particular day.

People living with MS say that the best approach is to try and put aside your worries as much as possible, live each day as it comes and deal with symptoms if, and when, they happen.

Day-to-day living

It’s important to look after your general health. This includes your mental wellbeing as well as your physical health. 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. 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.

You may be concerned that your MS will get worse very rapidly, but 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. 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. So it’s important to keep as well as possible and seek treatment promptly when needed.

Most people with MS live into old age, although lifespan, on average, is about six years less than the general population. This gap appears to be getting smaller all the time.

Diaries and reviews

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 before your review to see if you need to discuss anything in particular.

You might find it helpful to keep a simple diary to record any new or worsening symptoms, when they happened and whether they went away or have persisted. You could also include when you started (or stopped) any medication or treatment, if you experienced any side effects and how much it helped your symptoms.

A diary could be paper-based or an app on your phone. It's useful to use something that's easy to take to your appointments so you can share a summary with your MS team. They can then see at a glance what's happening with your MS and support you better. You could also add the dates and any outcomes of appointments in your diary so you have a record of what was said.

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.

Often living with primary progressive MS can mean finding a slightly different way to enable you to 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 if you have balance issues. Or using a walking aid or wheelchair some of the time might help you conserve energy if you have walking difficulties so you don't become as fatigued. Making these types of changes could mean you still be able to do the enjoyable things in life such as socialising, as well as the everyday things that need to be done.

Getting support

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.

Going forward

Being diagnosed with 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.

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