I have been living with HIBM for almost five years now. And one of the ways I’ve coped with living with a progressive neuromuscular disease has been to try to focus on all that I do have and on all of the ways in which I am lucky. But once a year, I stand up and give a speech about my experiences living with this disease and I am forced to have to go deep within myself and tap into all of the pain and loss I have suffered in the past year. I have to accept that at least this one time a year, I won’t be able to protect my loved ones from hearing the painful truth about the extent to which I suffer. And hardest of all, I have to try to reconcile the anger, frustration and sadness I feel in life as a result of having this disease with the joy, fulfillment and true inner peace I experience as a result of that same life living with HIBM.
On the one hand, I feel so lucky to have been given this opportunity to be a voice for people everywhere with a disease of this nature, and on the other hand, I still can’t believe it is my life that I am talking about up here and that I am that person whom people will be talking about when they go home tonight, feeling so grateful they are not me.
The strange thing is, I can make that statement and accept it as true and not be devastated by it. Not anymore at least. Not because it’s not devastating, but because I have been living with HIBM for four and a half years now. In the beginning, I could not and would not accept that this was happening to me. In fact, for the first two years, I couldn’t even say the word “disease” in the context of me. My sister and I literally referred to it as “the thing.” Anything else just sounded too frightening and so real. After the first few years, I gradually realized and accepted that this was going to be my reality-one plagued by ongoing losses and physical limitations. But despite having an intellectual awareness of that fact, I still desperately tried to cling to some semblance of my “normal” pre-HIBM life to avoid the emotional pain that comes with true, complete acceptance of a loss.
I got leg braces to help me try to walk more “normally,” I switched to a different car that would help me get in and out more easily, and I started parking in disabled spots to help make going to places like the mall or the supermarket a little less taxing. And for a while, that all helped.
But with each new day, I move farther and farther away from being able to maintain the “normal” life I once had. And the short-terms fixes never seem to keep up with the rate of change. The leg braces don’t work as well as they used to- after all, they are really just designed for people with lower leg weakness, not full leg and hip weakness. Plus, you can only wear them in certain shoes and even then they are painful by the end of a long day.
The convenience of getting in and out of my car more easily has been overshadowed by my more recent difficulty opening and closing the heavy door. And being able to park closer to the entrance of a store is helpful, but then there’s the walking and shopping that I have to contend with once inside a store.
There is no getting around it. I am now deep into this disease- the beginning stages seem so distant and the end is literally nowhere in sight. The anxiety about where this is going, how quickly it will progress and how much more I will have to tolerate is something I think about every day. Sometimes I am overwhelmed by it and sometimes I can tolerate it, but it’s never not there, I’m never free of it. No matter what I do, I can never escape it.
People often ask me how I am and I never really know quite how to respond because it never feels like there is a simple answer. So I will try tonight to give you a sense of what it’s like to live in the body of someone afflicted with this disease.
If you’ve never experienced it for yourself, I will tell you that it is a strange and surreal, not to mention horrifying feeling, when one day you realize you’ve lost the use of a once very strong and healthy part of your body. The helplessness and fear you feel when you stare at your foot, for example, and try to will it to rotate up or side to side and it doesn’t move, is emotionally paralyzing. I have often thought of HIBM as causing a type of complicated, chronic post-traumatic stress disorder. Every time I experience or discover a new loss, it’s another microtrauma that I have to acknowledge, process, mourn and then somehow incorporate on a practical level into my life.
Every single loss I have suffered is etched into my memory: the first time I realized my left index finger could no longer press the “T” key on the computer keyboard; the first time at the gym I realized I didn’t have the strength to put my foot into the pedal cage of the stationary bike and had to manually place it there; the first time I couldn’t zip up my son’s sweatshirt without asking someone for help; the first time I couldn’t hold him while walking without first putting on my leg braces; the first time I noticed that even my neck muscles were weak and as a result, I could no longer lift my head off the ground without placing my hand under it; the first time I saw the outlines of the bones in my forearms after all of the muscle tissue had wasted away or the first time I looked in the mirror and saw my left clavicle protruding out of my shoulder joint and realized there was a big divit where the muscles on the top of my shoulder used to be; the first time I mustered up enough courage to see the neurologist to ask him for a disability placard and had to check the “permanent “ versus the “temporary” disability box; the first time I truly accepted the fact that I was not going to be able to carry another baby in my body because it would be too risky; the first time I had to ask my husband to kneel down and manually uncurl my toes in my shoes because I was no longer able to wiggle them; the first time last summer I tried to flex my right foot while lying down during a physical therapy session and realized that not only could I no longer move it, but that there was no sign of even a contraction in it, which is what is referred to as a “complete foot drop”- a complete, permanent and irreversible death of that part of my body.
I could stand here all night and continue with this list of “firsts,” but I imagine for many or at least some of you, you’re wishing it would end. Me too. That’s the thing about HIBM-there is no finite amount of losses with which one can finally and definitively come to terms. And even if there is a period of time when symptoms do plateau, there always seems to be new loss around the corner.
Keep in mind, you are hearing the level of loss in someone who’s only four and a half years out from diagnosis. I am only 35 years old right now. I can’t help but wonder what my life will be like in my 40’s and 50’s when it is already so hard right now.
So how do I try to keep my mind off all of this? What do people do to cope when life just gets to be too much? All of the things I used to do to relieve stress or “get away from it all” are now tainted, for “it all” comes with me wherever I go. Things like sleeping, working out, or taking a vacation almost seem like more trouble than they’re worth sometimes.
Sleep is tricky because it’s the one thing that is actually required; and on a nightly basis. For some people, sleep is a way to avoid dealing with life or, at the very least, to catch up on some rest. For someone with HIBM, it can be the most dreaded part of the day. Sleep to me means having a hard time finding a position that is comfortable enough to stay in before I have to shift again, regardless of how expensive the mattress; it means waking up in the night with an entire leg tingling in pain because it has “fallen asleep” due to my inability to move certain muscles when I’m lying in most positions; it means tolerating the annoying sensations of muscle fasiculations- involuntary contractions that take place when muscle cells are firing and trying desperately to stay alive; and most painfully, sleep for me means having to confront a deep, scary, unavoidable awareness that every night will be the same, if not worse, for the rest of my life.
When it comes to exercise, the frustrations lie not just in being prohibited from doing much of what I used to, but from having difficulty doing most things that I can do now. The tightness in my muscles, the swelling in my ankles from repeatedly flopping my feet down uncontrollably on the floor and the constant clicking and snapping I feel in my joints every time I bend down, reach up or try to lift a weight make it hard to just lose myself in a workout.
So, sleep and exercise are not what they used to be; but for me, and I truly believe for most people with disabilities, it’s traveling that exacts the greatest physical and emotional toll. Going outside of our “comfort zone,” if you can call it that, to a new place with a physical landscape that has not yet been navigated is hard, especially when that place is a vacation destination for you and your family.
I have been lucky enough to have taken a few vacations this past year and I was grateful for each opportunity to do so, but vacation for me has evolved into something very different. This summer we went to Hawaii. It was my first summer since getting leg braces a year ago and one that I dreaded. I quickly realized that if I wanted to be safe and keep up with everyone, I would have to wear my leg braces and sneakers down to the pool with my swimsuit and sundress. That part I could handle. I could accept the inconvenience of strapping my leg braces on and off multiple times a day. And I can also tolerate going out into the world and facing the stares and awkward questions. I do it all the time. It’s when it’s time to go into the pool, or walk on the sand, or, worst for me, stand in the ocean that I have a problem.
Like most of you, I never really appreciated how helpful it was to be able to hop or even walk quickly along hot pavement or sand or to be able to go in the ocean by myself before I was physically incapable of doing so. And I definitely never anticipated how that inability would impact my feelings of worth as a mother. What good am I when I have to stand by helplessly and watch my son cry as he burns his feet while walking on the hot sand before someone else can come rescue him? Or when I can’t even stand in two inches of ocean water without losing my balance and falling over, let alone trying to do it while holding my son’s hand? I cannot keep him safe. It is that plain and simple. Vacation for me feels like a painful reminder of how I can’t fuIfill even some of my basic motherly duties, like carrying my son away from a crowded pool when he’s kicking and screaming during a tantrum.
During those moments, when I feel the eyes of strangers on me, all I can do with that feeling of embarrassment and unwanted attention is take a deep breath and hold back the tears as I watch all the other moms glide across the pool effortlessly, carrying their babies in one hand, holding the hands of their toddlers in the other, completely unaware of how lucky they are that their bodies work in the simplest and most basic way.
I never thought that those would be the kinds of things I would dream about and wish for- being able to carry away my tantrumming child safely from a hotel pool. After I was first diagnosed, I used to have a recurrent dream that I was running in a field. In the dream, I was always confused by how that was possible, but somehow it just was. I haven’t had that dream in over a year.
These days, the daydreams I have are quite simple- I wish for the day when I can walk barefoot from the kitchen to my son’s room to check on him without tripping over my own toes. I fantasize about the day when I can receive an invitation to a wedding and not have my first thoughts be worries about what I’m going to do for shoes or how much walking will be involved. I long for the day when my son begs me to chase him “like that other mommy is doing” and I laugh and say “okay” and run after him; the day when I can wake up in the morning and take my first few steps without having to consciously tell my body to lift up my legs high enough so I don’t trip and fall or without having to wonder if today will be a day that I get worse.
I am not giving up on these dreams, but I am running out of time. I am constantly assessing my body with each new loss to see if I would be able to “survive” if my progression stopped here, in this moment, right now. And I am quickly reaching my max.
I have lived the past four and half years trying to remind myself that we cannot control what happens to us-the losses, the tragedies, the heartaches; that we can only control how we’re going to cope with them. And in almost all dimensions of life, I believe that to be true. But in the past year or two, the research and science of HIBM has been shattering what I once thought was an absolute truth.
Recent advancements, which were facilitated in large part due to the money raised by the NDF, seem to prove that we actually can control how this disease affects us physically. We are so close to a treatment. But the reality is, even though we know that a treatment, or even a cure, is entirely within our reach, HIBM is still an orphan disease that primarily affects the Persian Jewish community, which means that it doesn’t get the attention that many other, more-well known diseases do. For me, knowing that we are right on the cusp of finding a treatment that could stop the merciless progression of this cruel disease fills me with hope that maybe there is an escape after all.
When I look at all of you tonight, I know that while I have this “orphan disease,” I am not alone. I am part of this community and I see our collective strength. I see our potential and our opportunity to make change happen, not just for me, but for all those suffering with HIBM and their families, now and in the future. Together I know that we can do this.
On behalf of all patients with HIBM, I want to thank the NDF, Hadassah, Malka and Haifa Foundation and all of you for your generosity, support and for being here tonight and listening to my story.
