In my last post I was expressing concern that my MS might not be the remitting recurring type that most people get, but might be another form called primary progressive. This is a problem in that there are currently no disease modifying medications approved for PPMS. One drug for RRMS, Gilenya, was in late trials for PPMS and many people had their hopes pinned on it. My doctor had been talking about switching me from Tecfidera to Gilenya anyway so my thought was that I might be covered whichever form that I actually had.
Unfortunately, the day before my doctor’s visit, Novartis announced that the Gilenya trial for PPMS failed. It did not have a statistically different effect than the placebo had. I brought up the possibility of PPMS with my doctor.
My doctor made a case that I do not have PPMS and that it makes sense to treat it as Secondary Progressive MS. This is the form where a person has remitting recurring MS and at some point it doesn’t remit anymore and gradually gets worse. My original MRI scans showed active lesions on the brain and were more typical of RRMS. Then I got on disease modifying drugs fairly early. She felt that this may have prevented new flareups that are common in RRMS. It’s just that my initial symptom never completely remitted, which can happen sometimes.
Given that there are not any different treatment options available for PPMS, I guess that sounds good to me and we will go with it.
As I mentioned in my last post, MS comes in four flavors. Since 85% of those afflicted have the relapsing-remitting type (RRMS), most people start with an assumption that this is the version that they have. Some of us hit a point where we have to start to question if we actually have Primary Progressive MS (PPMS) instead. I am at that point now.
Let’s start by looking at some of differences between RRMS and PPMS.
RRMS has periodic flare-ups that largely or completely go away, only to come back again later. PPMS has just a steady deterioration. Looking back on the progress of my disease, it has been a slow steady deterioration. I may have some days that are slightly better or worse than others, but I have never all of the sudden had an exacerbation. Nor have I woken up and found that my leg was back to normal. My strength and coordination in my left leg has just very slowly gradually gotten worse. I used to think that my MS medications were just preventing any flare ups of new symptoms. More and more I am suspecting that this is not the case; it may just be PPMS.
Demographics. RRMS occurs in two to three times as many women as men. It usually first pops up it’s ugly head in someone’s late teens to 20’s. PPMS on the other hand, usually begins in someone’s 40’s and afflicts men and women equally. I am a male and my MS began in my late 40’s.
RRMS activity tends to show up in the brain more and the lesions have more inflammation. PPMS tends to show up more in the spinal cord and have less inflammation. I remember my doctor looking at my MRI done with contrast last year and saying that it looked good with little evidence of new inflammation. We attributed it to the Rebif that I was on working. It could be. Or it could just be that it’s PPMS and taking the Rebif was coincidental since it’s generally accepted that the interferons like Rebif don’t do much if anything for PPMS.
MS can manifest itself in many ways. RRMS runs the gamut from eyesight, strength, balance, cognitive, bladder control, sexual issues etc etc. Any of these can also occur with PPMS, but the most common symptom is trouble walking and mobility. To date, walking/mobility is the only symptom that I have experienced.
What difference does it make and what does it all mean from a practical perspective? The only advantage that I can think of for PPMS is that it is more predictable. You know what you have and that it will gradually get worse. There is less of a concern that you will all of the sudden wake up one day unable to see out of your left eye for a week. The biggest disadvantage of PPMS is that it doesn’t respond as well to medications. In fact, all of the disease modifying drugs approved for use in the U.S. currently are just for RRMS. With some luck, that may be changing.
In my next post, I will dive deeper into my treatment options if we suspect that I may have PPMS.
By far the most common is called Relapsing-remitting MS (RRMS). Approximately 85% of people with MS have this type. It’s main characteristic is that the individual has symptoms that come and go. After a flare-up or exacerbation of a symptom, the symptom will partially or completely go away. Early on, this gives many people false hope that the disease has passed them by. Unfortunately, the symptom or new ones will eventually come back.
Most people with RRMS eventually progress to Secondary-progressive MS (SPMS). The individual’s symptoms may have remitted and relapsed for 10 or 15 years, but reached a point where they stopped going away. Typically from this point on, the symptom or symptoms gradually get worse going forward.
The third type is Primary Progressive MS (PPMS). With this version the patient does not have distinguishable flare-ups, nor do their symptoms ever go away. There is just a steady progression of the disability.
The fourth type is also the least common. Progressive-relapsing MS (PRMS) is characterized by steady deterioration as in PPMS, but with occasional flare-ups on top of it. The flare-ups may or may not improve, but the basic disability underlying it continually progresses.
There are a few common differences between RRMS and PPMS. That will be the topic of my next post as we try to determine which variant I have and the path of my treatment.