What is PPMS (Primary Progressive Multiple Sclerosis)? Let's talk about it simply!

You may have heard of a condition called ` Multiple Sclerosis (MS)`. PPMS (Primary Progressive Multiple Sclerosis) is a specific form of MS. In this case, your symptoms don't come on suddenly, subside after a few days, and then come back again and again. Instead, your symptoms gradually, gradually, and gradually increase over time . Like climbing a staircase, these symptoms gradually get worse. Don't worry, this may sound a little complicated. Let's talk about everything clearly and simply, okay?

What exactly is PPMS?

Simply put, `Multiple Sclerosis (MS)` is a condition in which our own immune system, the system that protects us from disease, mistakenly attacks our own central nervous system (`central nervous system`) – the brain and spinal cord (the nerve cords inside the spine). This damages the protective covering (myelin) around nerve fibers.

Now, there are several main types of MS.

• Relapsing-Remitting MS (RRMS): Most people have this type. In this type, symptoms suddenly get worse (we call this a `relapse`), and then after a while, the symptoms either go away completely or go away completely.
• Primary Progressive MS (PPMS): In PPMS, the type we're talking about today, there are fewer clear `relapses` like in RRMS. Instead, symptoms gradually get worse from the start. Sometimes there may be small `fluctuations`, meaning symptoms get better and worse. But overall, the condition tends to get worse gradually.
• Secondary Progressive MS (SPMS): Some people with RRMS can develop the disease into SPMS after several years. Their symptoms then gradually worsen and relapses become less frequent.

PPMS and SPMS are sometimes referred to together as `Progressive MS` because both have a progressive nature. In PPMS, there is some level of inflammation in the nervous system, and nerve cells are damaged and gradually lose their function. This is what we call `neurodegeneration`.

How common is PPMS?

There are millions of people with MS around the world. It is estimated that there are about a million people with MS in the United States alone. About 10% of them, or about one in ten people, have this type of MS called PPMS. So, PPMS is a little less common than other types of MS.

What are the symptoms of PPMS? How do they feel?

The main feature of PPMS is that the symptoms of MS gradually get worse. These symptoms can vary from person to person. Also, the way the symptoms start and the rate at which they progress vary from person to person.

Here are some of the most common symptoms:

• Difficulty walking: This is the first symptom that affects many PPMS patients. They may experience stiffness in their legs, weakness, and loss of balance. They may also feel tired quickly when walking.
• Tingling or numbness in various parts of the body: This can occur in the hands, feet, face, or anywhere else on the body.
• Vision changes: Blurred vision in one eye, double vision, or pain when moving the eyes may occur. However, these vision problems are less common in PPMS than in RRMS.
• Feeling tired all the time (`fatigue`): This is not normal fatigue. You may feel like you are not getting any better no matter how much rest you get. This can be a major obstacle to your daily activities.
• Difficulty urinating and defecating: This can include sudden, sudden urge to urinate (`urgency`), difficulty controlling urination (`incontinence`), and difficulty emptying the bladder completely. Constipation is also common.
• Feeling like someone is holding your chest or stomach area tightly: Some people also call this the `MS hug`.
• A sensation of an electric shock running down the back, arms, or legs when the neck is bent forward: This is called `Lhermitte's sign`.
• Muscle stiffness (spasticity) or weakness: This can make it difficult to manipulate and move your limbs.
• Speech problems: You may feel like your words are slurring or your speech is changing in pace.
• Swallowing problems.
• Thinking and memory problems: Feeling like your brain is foggy (`brain fog`), which means it's hard to remember things, focus, and make decisions.
• Mental problems: Depression , anxiety , and irritability may occur.

The important thing is that these symptoms may not be very obvious at first. You might think, “Oh, it’s just fatigue.” But if they gradually become more severe over time, you should be concerned.

Why does PPMS develop? What is the cause?

Scientists still don't know the exact cause of MS (including PPMS), but there are several factors that are thought to contribute to it:

1. Genetic Predisposition: It is thought that certain changes in our genes (DNA) may make us more likely to develop autoimmune conditions like MS (a disease in which the immune system attacks the body). However, this does not mean that if you have MS, your children will also develop it. The influence of genes on MS is not as great as you might think. Therefore, the risk of children passing on the disease is relatively low.

2. Environmental Factors: Some research suggests that certain things in the environment we live in may contribute to the development of MS. For example:

• Viral infections : Some viral infections, such as the Epstein- Barr Virus (EBV), are also being investigated for a link between MS and certain types of infections.
• Vitamin D deficiency : There is also a belief that people with low levels of vitamin D from sunlight are at higher risk of developing MS.
• Smoking: People who smoke are at higher risk of developing MS and may experience a faster progression of the disease.

3. Immune system function: Somehow, these genetic and environmental factors combine to create something wrong with the immune system. That's why it starts attacking our own nervous system.

Simply put, PPMS is not a disease caused by a single cause. It is caused by a combination of many factors.

What additional complications can occur due to PPMS?

As PPMS symptoms progress, other problems and complications may arise. Some examples are:

• Worsening muscle stiffness (spasticity): This can lead to increased pain and difficulty moving.
• Risk of complete loss of vision (rare).
• Worsening bladder control difficulties: This can lead to frequent urinary tract infections.
• Increased sexual dysfunction.
• Further decline in memory and thinking ability.
• Mental health issues: Conditions like depression and anxiety may worsen.
• Frequent falls and resulting injuries such as fractures.
• Pressure sores caused by spending too much time in bed.
• Risk of respiratory infections: Especially when the disease is severe.

How is PPMS diagnosed? (Diagnosis)

There is no single test that can definitively diagnose PPMS. Your doctor will take into account your symptoms, your medical history, a physical exam, and a few other specialized tests, and then come to a conclusion that "this could be PPMS."

Here are some of the tests that are commonly performed for this:

1. MRI (Magnetic Resonance Imaging): This is the main test used to diagnose MS. It can look for damage (lesions) in the brain and spinal cord caused by MS. In PPMS, the pattern of these lesions and how they change over time are important.

2. Lumbar Puncture (also known as Spinal Tap): This involves taking a small sample of the cerebrospinal fluid (CSF) from your lower back. This fluid is examined to see if there are any changes that are specific to MS, such as proteins called oligoclonal bands. These are not specific to MS, but they can be a sign of inflammation in the brain or spinal cord.

3. Blood tests: These help make sure you don't have other conditions that cause symptoms similar to MS.

4. Evoked Potential Studies: These measure the speed at which electrical signals travel through the nervous system. When nerves are damaged by MS, the speed at which these signals travel can be slowed.

5. Optical Coherence Tomography (OCT): This is a painless eye scan that can check for damage to the optic nerve and retina at the back of the eye due to MS.

To diagnose PPMS, doctors consider the gradual worsening of symptoms over a period of at least a year and the presence of certain specific features on an MRI scan .

At what age does PPMS usually occur?

Most people are diagnosed with PPMS in their 40s or 50s. This is a slightly later age than RRMS. However, it can occur at any age, both in younger and older people.

What are the treatments for PPMS?

There are two main goals in treating PPMS:

1. Disease-Modifying Therapies (DMTs): These try to slow down the rate at which the disease gets worse.

2. Symptom Management: This helps reduce your discomfort and improve your quality of life.

Disease-Modifying Therapies (DMTs)

There are several types of DMTs available for RRMS, but there are only a limited number of DMTs approved for PPMS.

• Ocrelizumab (Ocrevus®): This is the main DMT currently approved for PPMS. It is given as an intravenous infusion. Studies have shown that this drug can help to control the worsening of symptoms in PPMS patients to some extent.

Doctors believe that PPMS is less likely to be affected by immune system inflammation than RRMS, which is why DMTs that target it are less effective. However, there is a lot of research going on about MS, so there may be more treatments for PPMS in the future.

Controlling symptoms

This is a very important part of managing PPMS. This treatment is determined by your specific symptoms.

• Physical Therapy: This can be very helpful for things like difficulty walking, muscle stiffness, and weakness. Exercises and stretching can help improve strength, balance, and mobility.
• Occupational Therapy: Introduces techniques and equipment to help people perform daily tasks (e.g., dressing, eating, writing) independently.
• Medicine:
• Medicines for muscle stiffness (spasticity).
• Medicine for fatigue.
• Medicine for bladder problems.
• Pain medication.
• Medication for depression or anxiety.
• Speech Therapy: If you have difficulty speaking or swallowing.
• Mobility Aids: You may need things like a cane, walker, or wheelchair.

Your doctor will explain the possible side effects of any medication or treatment before you start it, so don't be afraid to ask any questions you have.

Is there a way to prevent PPMS?

Unfortunately, there is no way to prevent MS (including PPMS) because the exact cause is still unknown.

However, if you have MS, there are some things you can do to reduce the number of flare-ups (`relapses` – although these are rare in PPMS) and control the rate at which the disease gets worse:

• Taking prescribed treatments (especially DMTs) exactly as prescribed by a doctor.
• Following a healthy lifestyle (eating well, sleeping well, exercising).
• Completely abstain from smoking.
• Trying to reduce stress as much as possible.

What kind of experiences does someone with PPMS have to face?

PPMS is a chronic condition that can have a big impact on your daily life. As symptoms worsen, you may need to make some changes to your lifestyle to protect yourself and avoid accidents.

For example, if you have difficulty getting around on your own, you may want to talk to your doctor about a mobility device, such as a wheelchair. These things may be hard to accept at first. However, these devices can help you become a little more independent and stay active in your life.

As well as the physical effects of PPMS, living with this chronic illness can also have a significant impact on your mental health. It's common to experience feelings of sadness, anger, hopelessness and loneliness. Some people find great relief from talking to a mental health professional , such as a counsellor or psychiatrist. So, if you feel the need, don't hesitate to ask for help.

Remember, there is no cure for PPMS yet. However, research is ongoing to learn more about it and find new treatments that may bring you relief. So don't give up hope.

How quickly do PPMS symptoms get worse?

This is a question that many people ask. However, everyone with PPMS experiences it differently. For some people, symptoms may worsen very slowly, over years. For others, symptoms may worsen more quickly.

This is difficult to predict exactly. If you notice any obvious changes in your symptoms, or if they get worse quickly, be sure to talk to your doctor about it right away.

What is the life expectancy of someone with PPMS?

This is also something that many people fear. However, PPMS does not directly affect your lifespan. That is, having PPMS does not mean that a person's life expectancy is shorter than that of others.

However, serious complications of the disease (e.g., severe infections, problems caused by prolonged bed rest) can have a life-threatening impact if not managed properly. Therefore, it is very important to follow medical advice and take care of your health.

When should I see a doctor?

If you have PPMS, it's important to have regular medical checkups. In addition, see a doctor if you:

• If your PPMS symptoms are getting worse and affecting your daily life.
• If new, severe complications such as paralysis occur.
• If you have persistent pain or numbness in your limbs, or if it is increasing.
• Because PPMS can affect your balance and coordination, if you fall and injure yourself, see a doctor immediately or call emergency services.
• If the medication you are taking causes side effects .
• If you are feeling mentally ill (such as depression, anxiety).

What questions should I ask the doctor?

When you go to see the doctor, it's a good idea to write down the questions you have. Here are some examples:

• Do I have PPMS, or another type of MS? How do I know for sure?
• How can I manage my symptoms? What specific things can I do?
• Would you recommend physical therapy or occupational therapy for me? Where can I get those services?
• What medications do you recommend for me? What are their side effects? How long should I take them?
• Will I need to use a wheelchair or other mobility device? If so, for how long?
• Do I need to change my diet?
• What should I do to take care of my mental health?

Final Take-Home Message

PPMS, like other types of Multiple Sclerosis, is a disease that can have a significant impact on your life. That's true. But don't worry, you're not alone.

Remember, there are treatments and methods that can help slow the progression of your symptoms and improve your quality of life.

At first, you may not notice many symptoms, but over time, they may increase. You may even need to use equipment like a wheelchair to help you get around on your own. It's not the end of the world. Your doctor, physical therapist, and other healthcare providers can help you along the way. They can help you manage your symptoms and guide you to live as healthy and active a life as possible.

So, stay positive. Get informed. Ask questions. Get the help you need. Talk to your family and friends about this. Their support will also be a great strength for you.