I just received a howler of an email from an MS-Selfie reader accusing me of neglecting people with primary progressive MS (pwPPMS) and of being a charlatan for running a subscription model. They concluded by asking me to unsubscribe them from MS-Selfie to stop receiving future emails.
So the questions I have for you are, do you think I am neglecting people with primary progressive MS? What additional information do you need me to cover? And do you think the two-tiered funding model of MS-Selfie needs changing? The regular funding from subscriptions supports the development of the MS-Selfie microsite, MS-Selfie infocards, the MS-Selfie Self-Management Guides and future MS-Selfie initiatives that are planned. Without funding, the development of these would have to stop.
In response to this person’s email, I have prepared the following for people who have recently been diagnosed with PPMS. It may also be helpful to read some older MS-Selfie Newsletters:
* Diagnosing MS: what to expect? (10-03-2023)
* The annual MS follow-up consultation (26-06-2023)
* What to do about MS follow-up consultations that are too short? (07-08-2024)
Receiving a diagnosis of MS and PPMS in particular is a life-changing event, and it is entirely normal to feel overwhelmed. I would therefore suggest going into your neurology appointments prepared with questions, which can help you feel more in control, understand your condition, and partner with your healthcare team to make informed decisions.
Here is a list of questions to ask your neurologist or healthcare professional, organised by category. You don't need to ask all of them at once; pick the ones that are most important to you for your next appointment.
Tips for your appointment
* Take someone with you: A family member or trusted friend can provide support, help you remember what was said, and ask questions you might not have thought of.
* Write down the answers: Or ask if you can record the audio of the consultation on your phone to review later.
* Prioritise your questions: Star or highlight the 3-5 questions that are most important for you to have answered during this specific visit.
* Be honest: Be open about your symptoms, fears, and what you hope to achieve.
Category 1: Understanding Your Diagnosis
These questions help clarify the specifics of your situation.
* Certainty of diagnosis: How certain are you that I have PPMS and not another form of MS or a different condition? What in my MRI, spinal fluid (lumbar puncture), or other tests points specifically to PPMS?
* Meaning of PPMS: Can you explain in simple terms what "primary progressive" means and how it's different from relapsing-remitting MS?
* Current status: Based on my scans and symptoms, what is the current state of my MS? Can you show me the lesions on my MRI and explain what they mean?
* Cause: Do we know what might have caused my MS? Is there anything I did to cause this? (The answer is almost always no, but it can be reassuring to hear.)
Category 2: Prognosis and Progression
These questions are about the future, but remember that a neurologist cannot predict the future with certainty. They can only speak about general patterns.
* Typical progression: What does a typical course of PPMS look like? Since progression is a key feature, how will we monitor it?
* Personal factors: Are there any factors in my specific case (e.g., age, location of lesions, initial symptoms) that give you an idea of how my MS might progress over time?
* Key indicators: What are the key signs or symptoms I should watch for that would indicate a significant change or progression?
* Life expectancy: Does PPMS affect life expectancy?
Category 3: Treatment and disease management
This is a critical area, mainly since treatment options for PPMS have recently emerged.
* Disease-modifying therapies (DMTs): What disease-modifying therapies are approved for PPMS? Am I a candidate for them (e.g., Ocrelizumab/Ocrevus)?
* Treatment goals: What is the primary goal of this treatment? Is it to slow progression, manage symptoms, or both? What level of success can I realistically expect?
* Risks and benefits: What are the potential short-term and long-term side effects and risks of this treatment? How do these weigh against the possible benefits for me? What can be done to derisk these issues?
* Administration and monitoring: How is the treatment given (e.g., infusion, injection)? How often? What kind of monitoring (blood tests, MRIs) will I need while on this therapy?
* Alternative options: If I am not a candidate for a DMT, or if I choose not to take one, what is our plan?
* Clinical trials: Are there any clinical trials I could consider?
Category 4: Symptom management
This focuses on your day-to-day quality of life.
* Specific Symptoms: I am currently experiencing [list your symptoms: e.g., fatigue, leg stiffness, balance issues, bladder urgency, pain]. What can we do to manage these?
* Mobility: What can I do to maintain my mobility and balance for as long as possible? Would physiotherapy be helpful?
* Fatigue: My fatigue is a significant issue. What are the best strategies for managing MS-related fatigue?
* Pain and spasticity: What medications or therapies are available for nerve pain, muscle stiffness (spasticity), or spasms?
* Bladder, bowel and sexual dysfunction: Are bladder and bowel issues common? Who can I talk to if these become a problem? What can be done for my sexual problems?
* Cognitive and Mood Changes: Should I be aware of potential cognitive changes ("brain fog") or mood changes like depression and anxiety? What support is available for this?
Category 5: Lifestyle and wellness
These are questions about what you can do to help yourself.
* Exercise: What type and amount of exercise is safe and beneficial for me? Is there a type I should avoid?
* Diet and nutrition: Is there a specific diet you recommend for people with MS? Are there any foods I should avoid?
* Supplements: Should I be taking any vitamins or supplements, such as Vitamin D? If so, at what dosage?
* Stress and work: How does stress impact PPMS? Do you have any advice on managing my condition at work?
Category 6: Your healthcare team and support
A team best manages MS.
* MS Team: Besides you, who else should be on my healthcare team? (e.g., MS Specialist Nurse, Physiotherapist, Occupational Therapist, Psychologist). How do I get referrals?
* Communication: Who is my main point of contact between appointments if I have a question or a new symptom? What is the best way to contact them?
* Urgent issues: What symptoms would you consider an emergency that requires immediate medical attention?
* Support resources: Can you recommend any reliable resources for information and support, such as the MS Society, the MS Trust, or local support groups?
Category 7: MS research
* Is there any research into PPMS that I need to know about?
* Is there anything I can do to help participate in research?
Choosing the right questions for you will help you turn a daunting appointment into a productive conversation about your health and future. You are your own best advocate.
An alternative view of PPMS
I have made the argument over and over again that MS is one, not two, three or four diseases. You either have MS or you don’t. PPMS is simply MS, and people who are diagnosed with PPMS miss out on having relapses. About a quarter of pwPPMS will subsequently go on to have relapses and are referred to as having progressive relapsing MS. As we move to a biological definition of MS, the clinical descriptors are likely to be relevant in terms of treatment targets, i.e. we want to render pwMS NEIDA (no evident inflammatory disease activity) and NESDA (no evident smouldering disease activity).
How many of you use the search function on the MS-Selfie Substack website? When I put in the search term ‘primary progressive’, I get back a pretty large number of newsletters dedicated to PPMS. In addition, there is the MS-Selfie microsite you can use. A helpful section is the one on ‘What type of MS do I have?’.
I have hypothesised that the real MS is smouldering MS, and the immune response to what is causing MS is what drives focal inflammation (relapses and MRI activity). The clinically-apparent progressive phase of MS is simply a manifestation of a loss of reserve in that particular part of the nervous system, and the reason why progressive MS tends to affect the motor system in the lower limbs relates to the length of the axons subserving lower limb function. The longer the axons, the more likely they are to have multiple hits and the more likely they are to degenerate early. I, therefore, refer to MS as a length-dependent axonopathy.
So if you are newly diagnosed with MS and are an MS-Selfie newbie, I would spend time on the MS-Selfie microsite and then cover the following old MS-Selfie Newsletters.
* Is your MS salvageable? (03-11-2022)
* Relapses versus Progression (16-12-2024)
* Is primary progressive MS a different disease? (20-09-2022)
Subscriptions and donations
MS-Selfie newsletters and access to the MS-Selfie microsite are free. In comparison, weekly off-topic Q&A sessions are restricted to paying subscribers. Subscriptions are being used to run and maintain the MS Selfie microsite, as I don’t have time to do it myself. You must be a paying subscriber to ask questions unrelated to the Newsletters or Podcasts. If you can’t afford to become a paying subscriber, please email a request for a complimentary subscription (ms-selfie@giovannoni.net).
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General Disclaimer
Please note that the opinions expressed here are those of Professor Giovannoni and do not necessarily reflect the positions of Queen Mary University of London or Barts Health NHS Trust. The advice is intended as general and should not be interpreted as personal clinical advice. If you have problems, please tell your healthcare professional, who will be able to help you.
