I have been living with Primary Progressive Multiple Sclerosis Active (PPMS active) for 40 years, since I was 8 years old. PPMS active is the most aggressive of the types of MS in it’s progression. It is also the rarest, occurring I only 5% of cases. This type of MS is defined by steady disease progression with occasional exacerbations that can be “fast-forwards” in disease progression.
PRMS (Progressive Relapsing) is the former name of PPMS active.
So does that mean others reading this with a diagnosis of PPMS active should prepare for the worst? It means no two cases of MS are the same, and some of you will work for decades before it cuts your dreams short. Many of you will marry and have children. The time of diagnosis is not the time to hault your life.
For those not familiar with how the level of MS disease progression is measured, for many decades the EDSS (Expanded Disability Status Scale) has been used. The EDSS scale ranges from 0 (normal neurologic examination) to 10 (death due to MS) in half-point incrementsthat represent higher levels of disability. Scoring is based on an examination by a neurologist.
My goal was throughly educating myself on proper treatment, nutrition, fitness, and maintaining function through my chair yoga, water aerobics, chair circuit classes, and the recumbent bike. Although I used a walker, I stayed active and loved swimming and activities with my family. In the back of our minds, we know the new symptoms and longer naps are progression. However, we try to keep getting in each precious memory that we can.
As you can imagine, when our number rises it can be quite scary. Especially, when you approach the last few numbers before 10 (death due to MS).
4.0–Fully ambulatory, self-sufficient, up 12 hours a day despite relatively severe disability. Able to walk ≥500 meters without aid/rest.
4.5–Fully ambulatory, able to work a full day, may require minimal assistance. Able to walk ≥300 meters without aid/rest.
5.0–Ambulatory for 200 meters without aid/rest; disability that impairs full daily activities (eg, to work full day without special provisions).
5.5–Ambulatory for 100 meters without aid/rest; disability precludes full daily activities.
6.0–Intermittent or unilateral constant assistance required to walk 100 meters, with/without resting.
6.5–Constant bilateral assistance (canes, crutches, or braces) required to walk 20 meters without resting.
7.0–Unable to walk >5 meters even with aid; (restricted to wheelchair); wheels self in standard wheelchair and transfers alone.
7.5–Unable to take more than steps; restricted to wheelchair; may need aid in transfer; wheels self but cannot use standard wheelchair a full day; may require motorized wheelchair.
8.0–Restricted to bed/chair or perambulated in wheelchair, but may be out of bed itself much of the day; retains many self-care functions; generally has effective use of arms.
8.5–Restricted to bed much of the day; has some effective use of arms; retains some self-care functions.
9.0–Helpless bed patient; can communicate and eat.
9.5–Totally helpless bed patient; unable to communicate effectively or eat/swallow.
10.0–Death due to MS.
In February of 2016, I had a bad exacerbation that moved me up the scale from an 8.0 to an 8.5. It was a scary time for both me and my family. I was under 24 hour care. Diaper changes, sponge baths, and being turned to prevent pressure sores was our routine. This became my view day after day…
Over the next year, I continued to progress day after day…sliding toward a 9 with several organs going into failure.
My care team had uncomfortable conversations with my husband Bernard and parents, like nursing homes. My MS specialist discussed getting my affairs in order. In May of 2017, I heard the words I had been dreading from my doctor.
“YOUR PROGNOSIS IS GRIM.”
I was staring down the barrel of an EDSS 9 as I was losing all arm function very quickly. Make it stop, please! My goal of seeing my sons graduate was slipping from my grasp. My oldest son has multiple disabilities, including Cerebral Palsy. My youngest is disabled as well as Autistic. That’s too much to leave on their father and elderly grandparents! The thought of my sons saying goodbye to their mother at such a young age was just…unbearable.
But what can I really do to change this? There is no cure for the RNA gene mutations responsible for the disease process of MS once triggered by an environmental factor. There have been medication and dietary changes put symptoms into temporary remission until acted upon again by the triggers. I did not qualify for the new “game changing” medication Ocrevus that was on DMT (Disease Modifying Therapy) available to treat PPMS patients. It had just been FDA approved in the US on March 28th, 2017.
HSCT can give me 5-7 extra years if I am one of the lucky 80% initial success rate cases, and I can raise over $100,000 for the whole process overseas. Let’s face it, my EDSS is too high for that as well. My kidneys were in failure and too weak for a new MRI. My lung function was down to 25%. My heart was in and out of A-Fib. For the first time in 40 years, I felt like MS was really beating me.
This had to stop! I am the host is a radio show that is blessed to interview the most incredible MS scientists and clinicians from around the world. There has to be a way of using all of this front line information and my God given talent for research to find a way to give my immune system a fighting chance!
I spoke with friends in the field, researched, spoke with my own doctors about my findings to make sure everything I did was safe for me. My lung function is now down to 18% and this is a battle I cannot afford to lose. My dietary, fitness (yes a quadriplegic can exercise), medication, supplement and therapy changes were trial and error. No two MS patients are alike, not even identical twins. The RNA mutations are the same, but the environmental triggers are different.
I started to get stronger. My kidney function improved. My heart is rarely in A-Fib. My digestive function slightly improves. Does that mean I am not progressing, no. I have too many other autoimmune diseases I am living with that complicate matters. The short list of what I refer to as my “scroll” of conditions is MS, Lupus, Parkinson’s, Rheumatoid Arthritis, Sjogrens, EDS, Fibromyalgia, Hashimoto’s, Trigeminal Neuralgia…well, the list is long.
I started to feel sensation and get movement in parts that were long since dead. I didn’t have any control or strength, but the intense therapies were working. There were setbacks along the way. The biggest was Hurricane Irma passing over as a CAT 3 storm. What does that have to do with MS? Many of us react to changes in barometric pressure, like increased symptoms when a storm comes through. Also, we can have an exacerbation with change of seasons. A hurricane is the mother of all pressure drops! My doctor had ordered me to the hospital to weather the storm. It threw me into a deep relapse.
Now what? What is the key to gaining ground. For mild courses of MS, changes in diet and exercise alone can give an immune system enough of a break to allow itself to remeyelinate and heal itself from the MS damage. In my case it was going to take more drastic measures. I MUST qualify for Ocrevus and give myself a chance.
In the meantime, no need for that end of life “Bucket List”. I am taking my life back! I am going on a journey and turning it into a “Taking My Life Back” bucket list. I still spend most of my time in bed and have trouble sitting upright for long periods of time. I am working diligently on that one.
Beginning last Fall, I started checking off bucket list items. My mission was delayed by the hurricane, but I got back on track in February. We were flown out to San Diego, CA for a business meeting. My first time flying, so I was really nervous how I would react to the pressure.
The key to traveling as a quadriplegic is letting the airline know you will need assistance from beginning to end. I called the day before to confirm they understood my needs. We were instructed to tell the airline employee at curbside that I was a wheelchair transport that required assistance.
The pressure did impact me on takeoff and landing. My husband and I reminisced about our conversation when were were engaged about our marriage bucket list. One of our dreams was to go to California. He majored in film direction in college, so we talked about visiting Hollywood together. It was so beautiful when landed in San Diego. It’s like God had His hands all over this trip! FLY ON A PLANE…check!
After my business meeting, we were blessed to be asked to be part of the press coverage for the ACTRIMS (Americas Committee for Treatment and Research in Multiple Sclerosis) Forum. It was being held at our hotel. The view from our hotel was AMAZING!
I was in AGONY by the time we arrived. The flight and being upright for that long had taken it’s toll on my body. The edema was extreme and my oxygen levels had suffered during the flight. The ACTRIMS exhibits started early each morning. Deep down, I wasn’t sure I could serve on the MS & Me Media team to bring the latest in research and treatments back to our followers AND check off the personal goals my husband and I had set for us while we were in California. I was going to go down fighting!
I did it! Being in my manual wheelchair instead of having the tilt/recline and head support of my power wheelchair was quite painful, but we brought the cutting edge science and MS treatment information to the people living with MS. BROADCAST FROM #ACTRIMS2018…check!
I expressed to an MS friend, Dave Bexfield, at the conference that I was exhausted and hurting, but I really wanted to check off another item from my list. My husband and I had never taken a real honeymoon, so we were taking personal time on this trip as well. One item on my list was fulfilling our dream as a couple to kick back with our toes in the sand and watch the sunset over the Pacific Ocean. We had chosen the beach before we ever left our home in Florida, La Jolla Cove. I wanted to see the sea lions on the rocks and he wanted to climb the rocks and caves. Dave encouraged me to go for it when I was so tired and ready to give into my pain. WATCH THE SUNSET OVER THE PACIFIC OCEAN…check!
The next morning we had a private press conference with the President of ACTRIMS Dr. Jack Antel of McGill University, the Ptesident Elect Dr. Jeffrey Cohen of the Cleveland Clinic, and the Programs Chair Dr. Benjamin Segal of the University of Michigan. To a journalist who strives to bring accurate and cutting edge information to people living with MS around the globe, that was like Christmas and my birthday all wrapped into one! To have it come on the heels of being given a prognosis of “grim” it was confirmation that there is always hope! DISCOVER MS RESEARCH AND TREATMENT INFORMATION THAT GIVES ME HOPE AND A REASON TO KEEP FIGHTING…check!
That afternoon after another ACTRIMS presentation, we headed to Los Angeles! The leg of our trip to which my husband was most looking forward. It was a beautiful drive. Of course, we had to get a photo of the Hollywood sign and act like tourists. My husband, Bernard, pushed me down Hollywood Boulevard in my wheelchair to see some of the stars on the Walk of Fame. He loved exploring! We even saw an episode of America’s Next Top Model being filmed on the street. It became a little scary after dark and I was once again gave out, even with our planned rest breaks. We ate dinner and travel back to beautiful San Diego. VISITING HOLLYWOOD…check!
One star on the Walk of Fame very important to me personally that I see, actress Annette Funicello. Annette also lived with PPMS until her death at the age of 70.
Her husband was a great care advocate at a time when there wasn’t a lot of treatment options for patients living with Primary Progressive MS. Annette became a wonderful Ambassador for the MS Community.
The next morning, after more ACTRIMS presentations and exhibits, we packed for our flight home. With the treatment and research I had gathered, the goals I had accomplished, and the memories we had made…I was ready to go home and love on my boys! LEAVE A LITTLE PIECE OF MY HEART CALIFORNIA…check!
Stay tuned for the next chapter in my Taking My Life Back Bucket List!
For more on my ACTRIMS coverage and my weekly interviews on MS & Me Interviews, visit outer YouTube archives https://www.youtube.com/playlist?list=PLaLImm9tBMVf-g9ffDK_IQvnNdqnjVIUV