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.
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 it is very rare to have any relapses (periods where symptoms flare up) and remissions (periods of good or complete recovery). Instead symptoms gradually worsen from the outset (progression). This is why it is called 'primary' – because progression happens first, rather than having a relapsing remitting phase which can be followed by a progressive phase called secondary progressive MS. 'Progressive' is the word used to describe the change towards more disability in MS.
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 is more common in women.
What are the symptoms?
In more than 8 out of 10 people with primary progressive MS, the first symptom experienced is stiffness and/or weakness in both legs.
It gradually gets worse and results in minor problems, such as tripping on kerbs or when going up steps, but can then change to more obvious walking difficulties.
Bladder problems or bowel problems are quite common in primary progressive MS. These can be linked to sexual difficulties in both men and women such as maintaining an erection, decreased sex drive or finding there is less genital sensation.
Fatigue is very common. This is not normal tiredness but an exhaustion which is out of all proportion to the task that has been undertaken. Fatigue can be mental or physical tiredness.
Not all symptoms are obvious to other people. For example, bladder symptoms, and worries about getting to the toilet, can have a big impact on the person who is experiencing them but they may be invisible to others.
There are other symptoms which people with primary progressive MS may experience. 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.
What treatments are there?
Some people have the impression that there are no treatments for people with primary progressive MS. This is not true.
The confusion may arise because the disease modifying drugs for relapsing remitting MS are not available to people with primary progressive MS. These drugs can decrease the number and impact of relapses and, because people with primary progressive MS do not usually have relapses, these treatments are not appropriate.
Although there are no disease modifying drugs licensed in the UK for primary progressive MS at the moment, there are a number of drugs that are under development.
There is a wide range of treatments for individual symptoms which you can discuss with your health professionals. The treatment options include:
- symptom management - this may be drug treatments, rehabilitation therapies (such as physiotherapy or cognitive behavioural therapy) or a combination of the two.
- prevention of complications - such as treatment for bladder infections.
- management techniques - for example pacing yourself to minimise fatigue.
- promoting general health and well being - 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.
Is there disability in primary progressive MS?
The rate at which progression occurs varies widely between individuals. At times it may be at a standstill, or it may even improve very slightly for a short period. For others the progression is more rapid. However, onset is typically slow with symptoms steadily increasing over a period of months or years
What is disability?
You may be worried about becoming disabled. Disability means different things to different people. What one person considers disabling may not count as disability to someone else.
A dictionary would typically define disability as any result of having a condition that limits what you can do. For example, if you have a bit of numbness in your fingertips, it could make it hard to fasten a button. Some people might class this as disability but many wouldn’t.
The public’s image of disability can be different again. People often look for outward symbols of disability like a walking stick or wheelchair. This isn’t surprising, given that the symbol of a wheelchair is often used to represent disability. This can be frustrating if your symptoms are less visible ones like fatigue or pain.
Often disability is not about you personally but the environment in which we all live. For example, if a building has steps, then it will not be accessible to all. If products in the supermarket have labels with tiny writing, then not everyone will be able to read them.
The Equality Act covers people with MS from the point of diagnosis even though they may not see themselves as disabled at all.
Will I become disabled?
As each person’s MS is different, no one can predict how much your MS might, or might not, 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 can be a question of doing the same thing but in a slightly different way. For example, to manage fatigue, you might find it helpful to sit on a stool or chair to prepare food or do the ironing, rather than standing.
Although the word ‘disability’ may feel uncomfortable or inappropriate, it can help you access support. It may be particularly helpful at work or when considering whether any government assistance or financial benefits apply to you. There is no countrywide scheme to register as disabled so each benefit has its own definition of disability.
The Equality Act covers you as soon as you are diagnosed with MS even though you may not see yourself as disabled at all. It covers physical difficulties but also symptoms that affect your thinking, like memory problems and fatigue. At work, the Act requires employers to make reasonable adjustments so that you’re not at a disadvantage because of any difficulty caused by your MS. Examples of reasonable adjustments may 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 part of the office.
You may find that you are eligible for some government benefits especially if you have difficulties with walking, driving or key activities like washing or dressing. Some benefits are awarded according to the impact of your MS rather than being linked to your level of income or savings.
Diaries and reviews
You might find it helpful to keep a simple diary to chart your pattern of symptoms. You could list any new or worsening symptoms, when they happened and whether they went away or lingered. You could add in when you started or stopped any medication and whether this improved your symptoms or caused any side effects. You could add the dates and outcomes of your appointments with health professionals.
A diary could be on paper or an app on your phone, tablet or PC. It may be useful to choose something that’s easy to carry with you to your appointments.
It can be good to look back over a year and see if you need to discuss anything in particular with your health professionals. A summary of your diary can help your MS team to see at a glance what’s happening with your MS and then advise and support you better.
A review of your MS once a year is recommended but if you have substantial change in your symptoms you may be seen more often. If you haven’t had a review for a while, you could take the initiative and ask your GP or MS nurse to arrange one, rather than assuming that the system will contact you at some point.
Living with primary progressive MS
Living with progressive MS means living with uncertainty. Symptoms can go up and down and you may be concerned about how well you’ll be on a particular day. People living with MS say that the best plan is to put aside your worries as much as possible, live each day as it comes and deal with particular symptoms if, and when, they happen.
You may be concerned that your MS will get worse very rapidly but this is rarely the case. It’s important to look after your overall health which includes managing psychological factors, like stress, as well as your physical health. This will help you to do the things you enjoy, as well as the things you have to do.
MS is not a terminal condition but it is one that you will live with for the rest of your life. It is unlikely to be a direct cause of death. Most people with MS live into old age although their lifespan, on average, is about six years less than the general population. If you are more severely affected, you may get more infections than healthier people and develop complications that affect breathing and circulation. This can decrease your life expectancy so it’s important to keep as well as possible and seek treatment promptly. A well-balanced diet, regular exercise or activity and not smoking will help you stay well and reduce the risk of infections. People with MS are recommended to have the annual flu vaccination.
Diagnosis 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.
The bottom line
None of us know what life has in store for us. Try not to focus too much on what might happen or assume that MS will have an effect on some particular aspect of your life. This time and energy could be spent in a more positive way on something that is important to you right now.
Primary progressive MS book
Explores the physical and emotional aspects of living with primary progressive MS and the ongoing management of the condition.
It is only with the help of our donors, supporters and volunteers that we can continue training MS specialists, funding vital research and providing trusted information to the 130,000 people living with MS across the UK.
Questions about MS?
0800 032 3839
Open Monday to Friday, 9am - 5pm.