The Rarest Patients on This Sixth International SPS Awareness Day

Sunday, March 15, 2020 was the sixth International Stiff Person Syndrome (SPS) Awareness Day. The patients who often are forgotten on this day of awareness are the rarest, most severely ill warriors like me who are without any effective treatment options after years trying everything available and, therefore, living in intractable acute pain and body-wide muscular seizing, compromising our ability to breathe as our muscles squeeze into our ribcages and seize and spasm throughout the throat, neck, head, diaphragm, everything that makes breathing possible and easy. This respiratory distress in the midst of vehement seizures leads us to extended stays in the ICU and hospital rooms. It compounds the basic functions of our already immobilized bodies dangerously.

Our Awareness Day happens to occur during the start of COVID-19 outbreaks all over the USA. SPS is a neuroimmunological disease, meaning our immune systems, especially without any available medical treatment other than high levels of benzodiazepines, which often may not work either, lump us in the “high risk” category of contracting COVID-19. It means this virus is potentially deadly for us. Most of us are self-isolating at home, something we live every day anyway. If we need a doctor or an ER, we are incredibly susceptible to the severe consequences of COVID-19. Most of us just skip needed medical visits, anyhow, as we know medical staff is laser focused on possible infectious COVID-19 patients.

Most medical staff know nothing to very little about how to help us anyway, so we just suffer through the worst of seizures, muscular spasming, violent jerking, the resulting bone fractures and agony, difficulty breathing, and intractable pain that has made me spend an entire day and night vomiting, which frankly takes everything out of us. I am still recuperating from this latest vicious pain attack from last week. I am lucky that my husband is now permitted to work from home due to the COVID-19 outbreak. He is now here throughout the day to offer me help, or I’d just deal with it all on my own, at which I have become expert, though no one should have to be forced into this physically threatening and neglected reality.

A devoted mother in Mexico reached out to me for companionship and advice while her little boy is living in a hospital bed with severe pediatric SPS and nothing the doctors have tried has worked or helped her son. She is in a constant state of panic over his seizures and muscular rigidity, not to mention the looming presence of COVID-19 invading her son’s compromised immune system. I also have fellow rarest SPS warriors struggling for proper respiration, with acute pain management, and with total immobility at home because we have learned there is no place for us to receive medical help from hospitals who treated us like we were a drag on their system long before this infectious pandemic. My SPS friends cry out to me for friendship, empathy, any thoughts or advice I can give. I give all I can as we all push well beyond our limitations to help ourselves and, then, pay for this over-extended effort with the resulting worsening muscular rigidity. My heart breaks for all of us as we essentially have to be our own doctors and caretakers while suffering through the incurable, vehement, progressive variation of this neuromuscular disease.

In the midst of all of these phenomenal challenges, I’ve had exactly one perfectly healthy, able-bodied friend, now an absent acquaintance, say to me “hey, too bad your SPS isn’t contagious because they’d (the medical community) be working around the clock to develop research, treatments, and specified cures for you! Too bad SPS is not contagious, right?!” Just in case it’s unclear, this statement is horrible and unacceptable in many ways. Let me say thank goodness ‘my type of SPS’ is absolutely not infectious nor is any variation of SPS.

We patients should be able to receive the same medical attention, research funding for our SPS, and available effective treatments without having to force the medical world’s hand by describing our disease that crudely. What we have is more than enough to capture medicine’s attention, but we are too rare and complicated to justify or generate research and the gain of medical knowledge for the rarest SPS cases, or so we are told by our specialists. Every day, I hear and see stories online about the latest extremely rare and complicated diseases actually receiving medical care. Hundreds of patients like me at any age, whether infants, children, adolescents, or adults, must face this injustice with the knowledge we all deserve equal time from the medical community but that is not reality when you’ve spent your whole life fighting and pleading for help from specialists who tell you the brutal truth: “you all are on your own. Good luck.” Doctor language for get lost.

My primary care doctor years back was the most honest when she told me during an appointment “the medical community has failed you. You have come to the point where you have done everything for help. We just do not have the knowledge or the resources to help you.” Nothing deserves more awareness on this sixth International SPS Awareness Day than the fact that patients and patient advocates have done all we can do to grab the medical world’s attention, and we still continue to fight as the rarest “unicorns” we are until we win true medical care.

Until then, we battle onward and try not to contract COVID-19 in the coming weeks. Remember we are the most vulnerable among you and we need your cooperation in the prevention measures of COVID-19. Most of all, keep washing your hands and remember you can call us, say hello on Facebook, Skype or FaceTime us, email us, even send us little cards, letters, care packages, so we are not forgotten in the midst of this pandemic. We are usually far too ill and immobile to initiate all communication with others. Others have to care.

It’s just my husband and I in this SPS fight, and we sure appreciate any gesture of communication, connection, or thought sent our way as we continue to live the near impossible and help our fellow SPS warriors who do the same. All of us need basic empathy and kindness at this time of SPS Awareness now more than ever.

Angela Davis

Angela Davis

Angela Davis is a rare disease writer and advocate. She was published in last year’s congressional press release to support and fight for the ultimate passage of Cures Now, and she has 16 published pieces on The Mighty. She is also a published fiction writer and is working on her first novel every time her disease permits. Her pieces on rare disease have been shared by prominent organizations such as Global Genes and NORD. Once a busy writer, Angela grew progressively debilitated over 22 years with the rarest 20% of a rare neuromuscular disease, Stiff Person Syndrome, which affects one in a million and has no effective treatment or cure. She lives and battles for medical care in Bar Harbor, Maine with her devoted husband, Devin. They have one sassy rescue cat named Maeve. After care at UCLA Neuromuscular Clinic and Johns Hopkins, Angela is now without any specialist attention since she has exhausted help from top rare neurological doctors in the USA. Angela’s Twitter: @DavisAngesdavis, Facebook: angela.s.davis.37

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