What I Want My Fellow SPSers to Remember on Our Awareness Day

Imagine the thief who continues to tighten, seize, and hold your every muscle in eternal rigidity. Your relentless muscular spasticity fractures and collapses bones in your feet, fingers, knees, legs, and arms — even in your ribcage and your back. You constantly lose your grip on your cup full of water. You spill food and break your plates, bowls, and anything ceramic or the slightest bit fragile throughout everyday. You cannot stand up in the shower to wash yourself, even your arms are far too rigid to lift in order to wash your hair. You need a wheelchair and/or walker to be semi-mobile all of the time. No treatment or pill works to ease your debilitation, and you feel it progressing with each passing day for years. Specialists are very few and they do not know how to treat you. They send you home with a few basic anti-seizing meds and a pain med that you fight for even with all of your diagnoses. The endless acute pain is hardly ever controlled; you just somehow work to live in it and rally on to chase away morbid thoughts like, “what is the point of this existence?”

Sounds like amyotrophic lateral sclerosis (ALS), right? That is usually what people assume I and one in one million other patients must have. No, though my past specialists have informed me this disease, a rare manifestation of stiff-person syndrome (SPS), is very likely a sort of cousin to ALS, given all they share. With the extraordinary Stephen Hawking’s passing after living a long, remarkable life, I am prompted to recall SPS patients like me whose muscles have squeezed so ferociously upon our ribcages, that respiratory failure occurs. SPSers in this situation die, and no one, including the few physicians who attempt to treat and understand this vehement SPS, talks about it much, if at all. I fight the ribcage squeeze daily and push forward during severe asthma attacks and the seizing they induce.

Imagine with every physical exertion you make, from just trying to walk through cement-like rigidity or trying to rise up out of a chair, your muscles become more and more rigid. They become more spastic and disabling just from basic attempted movements you need to make in order to live, to exist. Vehement variations of SPS, like “SPS plus” or PERM, are so insidious, dangerous, and often fatal, for infants, children, and adults. Not much is available or offered medically to help the more severe SPS variations. You’ve heard doctors tell you to “just to do your best,” especially since they have little to no medical research or knowledge to help them care for you effectively.

On this fourth International SPS Awareness Day, March 15, 2018, I advocate especially for the most neglected and rare SPS patients like me who are just trying to survive, dreaming and battling to thrive in their lives with little to no medical options. Our SPS is the thief that steals away life’s fun moments and memories. We cannot enjoy hiking the outdoors, the vacations, the anniversaries with our spouses, the celebrations of life because they require physical energy and movement we simply cannot conjure — no matter how profoundly we want to celebrate, enjoy the beautiful events in life most people can take for granted.

I just want a vacation with my devoted husband of 20 years. Our 20th anniversary is in July, and I just saw yet another doctor who was kind, prescribed my regular “keep out of severe seizure” danger meds, but could not offer anything more. Again, I had to educate, explain, hope my new doc would hear and understand me. All I wanted was to celebrate a milestone anniversary with the love of my life, but we will probably end up watching movies at home like we have done for 22 years. We are so grateful to have one another and our time together. It sure would be wonderful to go on a true vacation that did not involve my SPS limiting my energy because of the physical demands of travel or my husband having to work so hard and so long and, then, come home to help me with basic tasks every day.

As we know from this past Rare Disease Day, SPS needs more in-depth, thorough medical research funded. This will allow our specialists and PCPs to be empowered with the information and knowledge, from diagnostics to complications of harsh treatments, to provide targeted, effective SPS medical care. Just like most SPS patients, I have to provide the bulk of the medical facts about SPS and how it works in my body. When you are incredibly ill and continually have to be the “doctor” in the room for every medical appointment, it often creates a tendency to feel these endless efforts are futile. It often leads SPS patients to stop going to the doctor because it is beyond exhausting to fight and plead for rightful medical attention every time we haul through the doors of a clinic.

On this SPS Awareness Day, though, I want to stress the importance of perseverance, of just going to clinic and having doctors hear you, of making certain you secure the best care you can find. We deserve medical attention just like every other patient. By insisting upon doctors’ visits, SPS awareness is increased tremendously and you can access the best available care to live your best life. We need to continue being the persistent SPS patient in the exam rooms, in the ERs. When we inform doctors and medical staff one on one about SPS and how widely it varies from patient to patient, we grant ourselves the gift of a much more educated medical community and our own personal access to the most effective care available. We encourage and grow a medical team mentality that integrates SPS patients as the most essential partners in medicine.

Here’s to the doctors who do not simply dismiss SPS warriors or shrug their shoulders at us, then just tell us, “Well, good luck with that.” Here’s to the physicians who know they must listen to us and really read our medical transcripts in order to help us to the best of their ability. These doctors know they need to listen to our individual experiences in order to provide any kind of care.

Most of all, here’s to all SPS patients and the people who love them from infancy to adulthood. We need the powerful devotion of friends and family to survive. These exceptional folks know they cannot ignore SPS warriors or just wish we would disappear. They know the mighty key to our refusal to give up happens to be in their sincere love and care for us. Too often we are fed the lie that our lives are not really worth the trouble or the demand for knowledgeable specialists. This lie is the block to our medical needs and to lasting, truly helpful SPS research, which is so desperately required in order for this insidious neuromuscular disease to be treated effectively. Researchers and doctors do have to care about SPS patients in order to offer true medical care, to offer us medical care that delivers relief from suffering and even release from the grip and, for warriors like me, the untreated progression of a terribly neglected rare disease.

Let today’s fourth International SPS Awareness Day expedite enlightenment of effective research. Let the information it yields quickly reach the doctors and specialists who fight to treat us as tenaciously as SPS patients fight to participate in life fully, not just persist until the next hospital visit. To my fellow SPS warriors, I am amazed by your strength, and we will continue to improve our care and even realize cures! Happy SPS Awareness Day!