LIVING WITH HIBM

What it's like to live with a progressive neuromuscular disease

Wednesday, April 27, 2016

emetophobia* (of a different kind)

It’s like that feeling you get when you know you are going to throw up, but you don’t know when. You try to convince yourself that the nausea will pass. You distract yourself. You wonder if maybe your body’s just being hypersensitive. You are desperate to avoid the extreme unpleasantness that comes with vomiting up your insides. And yet, you know that you’re inevitably going to have to endure it if you want some relief. Once it’s over, your stomach will eventually settle. But it’s so scary. And so uncomfortable. You hate it. And there’s nothing you can do to stop it.

I’ve been trying to avoid the metaphorical throwing up for a few weeks now. In other words, I’ve been desperately clinging to denial. It sounds something like this…Am I really getting worse? No, I’m probably just feeling weaker because I’m recovering from the flu. Am I really having trouble lifting my arm up to hang clothes on the higher rack in my closet? No, this jacket is probably just particularly heavy. Am I really going to need to start using a cane full-time? No, it’s just the shoes I’m wearing today that are making it difficult for me to walk. When I get home, I’ll be sure to add them to the pile of shoes that no longer work for me.

I have become a master at this. I will go to great lengths to avoid coming to terms with the truth. I will distort my perception of my reality for as long as I can to avoid feeling the pain that comes with confronting it.

When I’m comfortably in denial, it usually means that my disease progression has slowed for a bit. It means I have moved through grieving my most recent loss of function and adjusted to my new limitations. It is during these glorious times of disease stability that my frenemy, Hope, pays me a visit. She says to me, “Sure, this is bad, but you can live with this. And, let’s not forget that there is always a chance that you won’t get any worse than this.”

I try not to get too close to her because I know she can hurt me, as she has so many times before, but I also feel reassured by her presence. She’s the one who helps me turn up the volume of everything else when I’m trying to drown out the voices of truth. The voices that yell out at me when I stand up from the our living room sofa, not wearing my leg braces, and I literally cannot take a step in any direction without losing my balance.
“Turn it up! Louder!” we say.

She’s the one who tries to help me breathe when the biologically-programmed panic of not being able to fight, flee or protect my young sets in. She tells me that even though I may feel as if I’m vulnerable prey, there are no predators to fear in my modern day life. I can and will continue to adapt. I will be responsible about enlisting help. And ultimately, I will survive.

But over time, I notice she’s not hanging around as often. She’s not there to help me in my quest to convince myself that it’s going to be okay. Changes start to set in. Painful experiences become harder to look past. New social situations present themselves and I realize I’ve lost any chance of trying to “fit in” or not have my disease be obvious. Those days are over. And eventually, the whispers become increasingly louder. That feeling sets in. I know it’s coming. I know I’m going to have to start to feel the sadness again. The pain. I don’t want to. I’ve just gotten comfortable here. I don’t want to feel sad. I don’t want to cry. I don’t want to live in the truth. It feels so bad. It feels so icky. Repulsive almost. I’ll do anything to avoid it.

And yet, I’ve been through this cycle enough times to know that if I want to get past it, if I want to reach the land of acceptance, there is only one way to get there.

We try to do everything we can to bypass the journey or even just make it more tolerable- binge on TV shows or food, get high or drunk, lose ourselves in work or just try to fall asleep and hope that when we wake up, we’ll have arrived.

But nothing can ever quite do the trick.
I know I have to let it all come up and out.
I know I have to stay in it until it’s over.
I know that despite it getting exponentially harder to settle back into the new baseline, there is no other way to keep moving through.

I also know that most of my blog entries are just collections of (sometimes overdone) metaphors trying to capture the pain that comes with moving through my own personal 3 stages of grief: denial-depression-acceptance. Sometimes, in these raw, non-distorted moments of truth, it feels as if the writing is the one thing I have to hold onto. So, I guess I’d just like to thank you for holding onto it with me.

 

*emetophobia: an intense phobia that causes overwhelming anxiety pertaining to vomiting

Thursday, March 10, 2016

Finally, I’m here. I’ve arrived!

When I was first diagnosed with HIBM (or as it’s now often called, GNE myopathy) almost 10 years ago, I couldn’t even say the name of the disease out loud. I could barely accept that I had it. I didn’t want anything to do with it. I didn’t want to read about it, I didn’t want to talk to any other patients, I didn’t want to attend any fundraisers and I most certainly did not want to meet any other patients. It was all I could do to cope. I was barely hanging on. It was all too much. Too scary. Too painful. Too real. So I didn’t push myself. I went at my own pace. I knew it was to the dismay of some loved ones who thought it would benefit me to reach out to other patients for support. Especially those who seemed to be doing quite well living with the disease. But I wasn’t ready. I admittedly felt a bit self-conscious of my process. It seemed that other newly diagnosed patients were able to jump right in and access any and all resources that were available to them. But I wasn’t.

In the early years, I cried all the time. I cried not only about what was happening in the moment, but also about what I realized was never going to happen in my future.

I cried every time I thought of not being able to dance at my kids’ weddings. (For whatever reason, that one came up a lot at the beginning). Then, as I continued to progress, I cried when I thought about possibly not being able to stand up on the bimah at their bar-mitzvahs.

I cried (and became angry and defensive) the first time somebody suggested I consider surrogacy as a way to have a second child to complete my family.

When I watched my mother and mother-in-law act as night nurses for my boys when they were babies, helping me throughout the night, voluntarily exhausting themselves for the love of their grandsons, I cried because I knew I’d never be able to do that for my own children and their babies.

I cried myself to sleep the first time I went to the snow with my 10 month old (who will be turning 9 in May) because I knew then that I’d never be able to ski down the mountain alongside him the way my parents did with me. And when someone so lovingly told me there were adaptive ski programs at most mountains, I became angry and defensive because I wasn’t interested in some special program to help me. I was interested in having my old skiing body back.

I used to cry a lot. And I still do my fair share of crying, but it’s different. My mind travels to all of those same places in the future, 10 or 20 years down the road, but when it gets there, it sees something very different from what it saw back then. I was wearing different lenses then. I couldn’t see through the pain and the loss. It was cloudy and dark. I was too angry to try to envision any other scene. I simply couldn’t.

But of course I couldn’t. I hadn’t yet honed any coping tools at the start of this journey. I couldn’t possibly imagine tolerating where I am now when I was all the way back there. Quite frankly, it would have been odd if I had been able to embrace it. I just wasn’t there yet. And no matter how many people tried to reassure me that I would eventually figure it out or that it would be okay, I didn’t buy it.

Here’s where I am now…

For the last few years, I have taken my family to the snow and managed to ski standing up, with some simple modifications, for a few hours each trip. The last time I did, I lost control and gently crashed into a group of young girls in ski school, one of whom said so adorably and sympathetically, “It’s okay, don’t worry, we were all beginners once!” As I smiled at her sweetness, I felt that familiar pang in my heart that comes with every new loss. I knew that was the last time I would try to ski standing up.

Spring break is approaching. My younger son is turning 5 and he is ready to get serious about skiing. And so am I.

A strange thing has transpired. I am excited. I am excited to enter into this new world. I am giddy just imagining myself skiing down the mountain with my family. I spent 30 minutes on the phone with the head of the Mammoth Mountain adaptive ski program yesterday. She was awesome. Who knew there were so many options? A ski bike? A mono-ski? A bi-ski? I’m so curious to see which one will work for me. I am so excited to just get on the mountain. To show my boys what I can do. To feel the cold and work up a sweat. To get to earn my apres-ski hot chocolate. It’s strange. It almost feels as if I’m betraying my younger, more skeptical, more defiant self. As if I shouldn’t be okay with this. And yet, it also feels as if I’m giving her a precious gift. One I couldn’t receive until now.

I wish I could give this gift to newly diagnosed patients. I wish I could say to them, “Look at me! I’m doing it! Don’t worry, you’ll figure out a way too! Your own way!” But if they are anything like I was then, they will likely just cry while reading this. Or feel angry. Or hopeless. And I wouldn’t blame them.

Everyone has his or her own process. I am only here to share mine. Mine doesn’t always feel as easy or as fast as I would like, but it got me here. I’m here. I made it! Right now.

And for that, I am so grateful.

 

 

Monday, February 15, 2016

Pain is inevitable. Suffering is a choice.

I’m coming up on 10 years of knowingly living with this disease. An entire decade. One fourth of my life.

Throughout these past 10 years, I have mourned losses, adjusted to new baselines, adapted new ways of coping, advocated on behalf of patients and invested a tremendous amount of emotional energy in trying to keep perspective and stay in touch with the gratitude for all my blessings. I am proud of myself for tapping into resources within me that I never knew I had.  I am proud of myself for learning how to hold the pain and the joy in one hand. But also, I am exhausted. It feels like I’ve been at it for long enough. Too long. I want to be done with the mourning and just focus on the coping. I want this part to be over.

It would be so nice if that’s how it worked. You put in your time, you pay your dues, you demonstrate your unexpected resilience in the face of adversity and then you get to be done. And by done, I don’t mean done with all the challenges and disappointments that are inherent in life. Those are inevitable. I mean done with some to allow room for others. Because there are always others.

Life as an adult is hard. It’s hard to navigate the complexities of parenthood. It’s hard to grow older and confront the losses of loved ones as our mortality becomes more real. It’s hard to navigate the waters of the work-life balance and not feel exhausted by the swim. Add extra stressors to the mix and it can feel overwhelming. And then add ones that are chronic and it can feel despairing. That is what I think I have been in touch with lately, the despair.

I’m sick of the process. I’m sick of having to talk myself down from the panic that sets in when I get a glimpse of what’s coming based on what’s already here- more frequent and more serious falls, more difficulty taking heavy things out of the fridge, more difficulty getting out of a chair. I am tired of not knowing whether I should renew my office lease for another three years, tired of rushing to plan vacations despite them not being the ideal time/stage for my family simply because my physical abilities are on a ticking clock. I’m exhausted by constantly trying to pull myself back into the present moments. In each of those moments, I have to run through the five stages of grief at an accelerated pace. I have to constantly hope that maybe this disease won’t ever get as bad as I think it will. As bad as I know it can get.

But that hope comes with a cost. It’s increasingly harder to hold onto it in the face of continued progression. So rather than invest in the hope that I won’t get exponentially worse, I am going to try to start shifting my resources towards hoping that despite how much worse I get, I will still be able to hold onto me.

That’s what I’ve been afraid of all these years. Losing me. My smile, my upbeatness, my desire for adventure, my spontaneity, my silliness, my capacity to feel carefree, my ability to have fun. I have feared that I will become so disabled that I will no longer have the capacity to feel truly happy. And so as a result, I have desperately held onto a need for me to not get worse in the service of maintaining my emotional health. But that in itself is exhausting. The holding on.

I am going to try to loosen my grip. Let go of what I think I require to be happy and wonder if maybe there’s more to it. I will stop trying to convince myself that my body is going to be “okay” and instead accept that maybe it won’t be.

I do not perceive this as a defeat. I do not see this as me not having a positive attitude or me giving up on hope. It feels empowering, like an act of self-compassion, a way to preserve my strength and my emotional energy.

I am tired. I want to stop struggling to stay afloat in this sea of unknowns and instead gently turn over and try floating on my back for a while. I want to catch my breath. I want to feel the calm. I want to see where it takes me.

Friday, November 27, 2015

Thank you

I was going to post yesterday, but I chickened out. I was going post about how, for someone who feels in touch with gratitude on a regular basis,  it was disappointing to not feel it to the degree I normally do on Thanksgiving.  Thanksgiving is like my gratitude Superbowl. It’s when we are all so in touch with all the ways in which we are blessed.  But I haven’t been feeling as grateful in this past month.  It sounds so taboo. I guess it’s not that I haven’t been feeling grateful,  it’s just that it’s been harder counteract all that I’m not  grateful for. And yesterday,  I didn’t want to be a buzzkill. Or even offend anyone. So today,  I wanted to share. I wanted to tell you about some experiences that have felt hard in this last month. And where I have ultimately found the gratitude.

In October, my husband and I got away to Portland for the weekend. It was so great, except for the sad realization, once and for all, that I can’t really explore a new city on foot the way I used to, the way I wish I could.  Even stepping up onto a curb has proven to be a challenge. While we were there, on that Saturday night, we waited in the hour-long line in the drizzling rain at a famous ice cream shop. We were having so much fun, except for when I walked out of line to go to the bathroom, slipped and fell, had a crowd gather around me, heard a bystander so sympathetically say, “oh no, she has a cane” and somehow ended up getting helped up by a police officer.

A couple of weeks ago, I attended an annual psychopharmacology conference at the Skirball. It’s always a wonderful opportunity to see old friends from residency, but this time, it was also an unwelcome opportunity to see how much I’ve progressed since the last time I was there. I can handle the more “minor” challenges like difficulty clipping my name tag onto my lapel or trying to keep up with everyone while heading to the luncheon area. But sitting down for lunch and watching everyone at my table so effortlessly cut into their food, while I sat their acutely aware of how awkwardly I now hold my utensils and struggle to cut my own food, did not feel like something I could handle. It took all of me to not flash forward to a time where I might have to sit down to a meal and ask the person next to me if he or she wouldn’t mind cutting my chicken for me.

 

 

If you’re still here, if you’re still reading, if you are still with me, I am grateful for you.
I have tried to hold these experiences quietly within myself. But I can’t. I can’t hold them alone. They are too heavy. They will crush me.

Thank you for holding them with me. Thank you for not running away out of discomfort or fear. Thank you for receiving my invitation and for choosing to attend.

I am grateful for you and I am grateful for the one thing this disease has forced upon me- the strength to share and be vulnerable. Because if I didn’t have it, if I couldn’t do what I’m doing right now, I would be so lonely. And the loneliness of carrying this by myself would disable me in a way that, unlike all the rest, I wouldn’t be able to adapt to.

Sunday, November 1, 2015

Kitchen dreams (and nightmares)

As I sat down to write this post, I realized how often the topic of my home has come up on the blog. Or at least, that’s how it feels. Probably because I’m so intimately acquainted with it on a such a micro-physical level. It plays an integral role in my daily life and overall feeling of well-being, as I’m sure many homes do for many people, but in different ways and for different reasons.

We recently embarked on a kitchen remodel. We wanted a new kitchen not only because we love cooking and being in the kitchen (especially with Noah’s recent into foray baking bread), but because as I slowly get worse, I am becoming increasingly impatient with my limitations. I am continuing to have to increase my tolerance for frustration and, quite frankly, I’m over it. I love cooking, I love coming home from work and making dinner for my family, but as the only adult home during that time, I can’t function as efficiently as I used to.  As my calf muscles melt away, it’s becoming harder to stand on my tippy toes to reach the dishes on the second shelf of the cabinet. Or as balance becomes trickier, it’s getting harder for me to walk over to the trashcan holding food waste and then press the pedal that lifts open the lid. And it’s been a while since I’ve been able to fill up a big pot with water and lift it up onto the stove by myself.

I initially justified the expense to myself by saying I needed my kitchen to be accessible and adaptive to my needs. Naturally, right? Makes perfect sense. Then once we decided to move ahead, I quickly forgot that part.  I had told myself that my job in this process would be to be mindful not only of my physical needs now, but also to try to anticipate my future needs with this disease. But who wants to do that?  That’s no fun. The fun part is going to appliance warehouses to pick out cooktops or staying up late to tag photos on design sites. It is the two of us embarking on this journey together, for the first time ever. Creating something together. Making something completely our own. Customizing our physical space. I now fully appreciate why people who have the funds often go that route, building an entire home. It’s such a luxury- building your vision, watching your dreams transform into reality.

In the first couple of weeks, when questions came up from the contractor about what would work for me physically, I told myself in a very quiet but very strong voice, “Oh come on, you don’t really need to worry about that. You won’t really need to check the counter height to see if it would be suitable for a wheelchair, because you’re never going to get there.” I tried to believe it.  Was that my defense mechanism kicking in and trying to prevent me from confronting the truth? Buying me some time? Or was it a rational voice that genuinely believes that the scientific community will figure something out before I get to that point? Of course, there is no one, not one person on this planet, who can answer that question. It’s a scary place to be. I would think it feels lonely, but it’s surprisingly, reassuringly not. Because I am not alone. I have people next to me who hold it with me, no matter how heavy. And wherever I go, they follow along right beside me, still carrying it. Of course, they didn’t just appear alongside me, ready to work. I had to invite them to come along. I had to let them in. I had to expose my vulnerabilities. [See previous blog post on the benefits of vulnerability and love being everything, http://livingwithhibm.com/?m=201409]

Although I have people to share the load with me, that doesn’t mean I am always willing to hand it over. Sometimes, just sometimes, I want to let the people around me relax. Take a break and just take the load off. Sometimes I just don’t want it to be about my disease. Not because they need that, but because I need that. Sometimes I just want to pretend. Try on what it would feel like to not have this disease. I don’t want to have to mention to my husband in an attempted nonchalant way that we may indeed have to choose our flooring based on whether or not it will leave an uneven surface between the dining room and kitchen which will potentially make it difficult for me to walk with my future gait.

I want to be able to choose things because I like them, not because my disease and my progressively weakened body requires me to need to have them.

And yet, that simply isn’t an option for me.

So I will feel sad about it for a little while. I will feel that vague bummed out feeling.  That feeling you aren’t always conscious of  until you’re reminded of it and realize, “Oh yeah, that is what’s been feeling hard lately. ”

And then I will presumably get back to my happy, grateful, I’m-so-lucky-I-even-have-the-opportunity-to-do-this-kitchen-remodel baseline.

Yes, I know I can do it. But that doesn’t feel as satisfying as it used to,  just knowing I can do it. Sure, I know I will move through this. I just wish I didn’t have to.

And yet.

Wednesday, October 7, 2015

The million-dollar question

Exactly a month ago, I was walking over to say goodbye to my younger son and our nanny at an indoor play area and I tripped on a toy and fell. I went down hard and my left hand, the much weaker one, bore the brunt of it. A bunch of people saw and rushed over to me, but per my usual, I played it cool. I told them I was fine. I can’t stand that kind of attention, probably because it’s on top of what is now a new baseline of daily unwanted attention. Luckily, my son didn’t see and I decided to skip the kiss goodbye and instead wave from afar. I needed to get out of there as quickly as possible. I came outside and saw blood leaking out from under my rings on my ring finger and discoloration on my skin. It hurt like hell. I walked to my car, not just feeling the physical pain but the emotional angst of realizing that I probably wasn’t going to be able to go exercise that morning. (Exercise is no longer an optional luxury but rather a means of muscle preservation and survival.) Within minutes, my ring finger started to blow up. Luckily I had the sense to take my rings off right away. I started driving. I didn’t know where to go. All I wanted to do was drive to the gym. But two blocks in, it was getting so bad that I had to pull over at a random Taqueria and ask for a cup of ice.

I really did not feel like spending the morning at the urgent care. So I started driving towards my physical therapist’s office. Maybe she would tell me it didn’t look that bad and I could go about my day. But of course, she was worried it was fractured and told me I needed an x-ray. Of course I did. I knew that I did. So I cried to her and she gave me a hug in that most tender, loving compassionate way that she always does when she sees first-hand how unforgiving this disease is and I headed to the urgent care at Kaiser.

It’s amazing when you enter a hospital setting and see so many others using a cane. And then it’s even more amazing to see that the majority of people using a cane are all elderly or seem very infirmed.

I sat in the waiting room, wondering how many more times I would find myself there. In the next year. Or the next five years. Would it be for a for laceration on my forehead like another HIBM patient I know? A broken arm or a leg? Not catastrophic fears. Real outcomes in real patients. The hardest part of that urgent care visit was when I had difficulty arranging my already weak fingers in the correct positioning for the various views needed for the x-ray. The reminders never stop. There’s never a pause in the awareness that I have this disease. Or that I will always have this disease.

For all intents and purposes, I had fractured my finger. Though he couldn’t detect a break on that initial x-ray, he said it’s common for a fracture to show up later on and based on how it was presenting, we would need to treat it as such. By that time, the entire finger and parts of the surrounding ones had turned purple and black and my finger was swollen in a way that was rather fascinating to me. He put my finger in a splint and that was that.

That was a month ago. Though the swelling has reduced somewhat and the discoloration has started to fade, it remains incredibly painful and I have almost no range of motion in that finger. On the one hand, my joke has been that it’s such a weak finger anyway, it doesn’t really matter what happens to it. On the other hand, because it is already so weak, I need to try to preserve strength as much as possible. To be quite honest, the most frustrating part is that I haven’t been able to wear my wedding and engagement ring.

So a few days ago, I decided I should probably go in for a follow up and figure out what’s going on. I had the appointment this morning. The doctor was a lovely middle aged Persian man with kind eyes. He looked at me then looked at my cane and I quickly explained that the cane had nothing to do with why I was there. Well, not really. He asked me what the cane was about. I put on my doctor hat and told him about HIBM. He was shocked. He’d never heard of it. He wanted to write it down. He asked questions. He asked if I had seen the neurologist at Kaiser. I said there was no point in being followed by a doctor. He said that’s true, but he was actually thinking about for other patients as there are a fair share of Persian patients at Kaiser. Of course, I said. Then he started to examine my hands. It’d been a while since I’ve had that kind of exam. He asked me to bend the tips of my fingers on my left hand. To resist against his. I told him and showed him that I couldn’t. He looked up at me and when his eyes met mine, I saw in them a mixture of disbelief and confusion. Then he asked me if I could bend my fingers at my knuckles. Of course, I couldn’t. I could see him trying to take it in. Not understanding how this mysterious disease worked. I saw him compare my two hands and feel where the atrophy has melted away the muscle between my thumbs and forefingers. The area my eyes are always checking in on but my mind is constantly trying to ignore.

I felt my doctor hat slipping off and my patient hat coming on. I tried to resist. I told myself not to cry. I repeated it to myself like a mantra. Don’t cry. Don’t cry. I swallowed it back down. We talked about how there isn’t a whole lot to do. We can “buddy tape” it, but the fractured finger doesn’t have a healthy ‘buddy”
to move around with it. We could put on a night splint, but unlike a normal finger, my fingers don’t really bend much anyway so it’s not really indicated.

He said he would refer me for hand therapy and we left it at that. Lastly, I asked him when he thought I might be able to wear my rings again. Or if my finger would just remain this deformed permanently. He said that was the million-dollar question. My least favorite answer.

My rings. My beloved rings. I’ve been so hung up on the fact that I haven’t been able to wear them. Why? Why has that been such a big deal? It’s so minor in the grand scheme of things. But then I let myself unpeel that piece. And underneath was hiding the realization that when I leave the house with those rings on, they serve as my shield out in the world. They protect me. They reassure me. They scream out that I am loved. That I don’t just have this thing going on. This unsightly gait. (Please allow me to hate it.) I wear my ring and I am reminded I have have a whole life that balances this. This what you see before you. They represent the innocence and carefreeness I was so blessed to live in during my pre-disease life. They are my security blankets. Without them I feel vulnerable. I feel insecure.

I wish i didn’t find myself in a position where this is something that I care about. Something I find myself relying on. And yet, here I am.

 

Saturday, August 8, 2015

Damn you, Acceptance

It’s been four years since we moved into our new home. Our first house. We knew it was the perfect fit when we found it. Big enough that we don’t feel like we’re on top of each other, yet small enough for me to not feel stressed walking from one end of it to the other. It also satisfies our joint need to live somewhere that straddles the line between residential and urban. When you hang out in the front yard, you feel the comfort of being on a tree-lined street with houses on all sides. But walk one long block down the street and at your disposal are restaurants, coffee shops and even a mall with a movie theatre.

Four years ago I was physically progressed enough that I felt worried about buying a house with even a few steps to front door. Thus, I never really let myself fantasize about actually taking advantage of the convenience of our location. Sure, it was great because it meant it only took me a minute to drive to buy the kids’ shoes, but I still had to deal with the parking and all the hassles that come with mall shopping. Then, last year, I took the plunge and moved through another level of acceptance and bought myself a shiny red “mobility scooter.” It doesn’t get much sportier or zippier. Most random people who see me on it ask how they can get one, without realizing it’s not purely for recreation. (Side note, it amazes me how many able-bodied individuals look for ways to avoid having to walk.) It always feels good when I can get away with not appearing disabled. It feels like a relief. We’ll come back to that in a bit.

Last night, my husband and I had plans to see the movie, “Trainwreck.” Yes, my obsession with Amy Schumer is still very alive. Because the kids are away and we weren’t on babysitter time, we found ourselves running a little late. And the thought of having to drive and deal with parking while rushing was so unappealing. And of course, the movie theatre (which happens to be our favorite, the Landmark) is only about 2.5 blocks away. My husband, for the first time ever in the last four years mentioned that it would be nice to be able to walk. Not in a disappointed way, not in a resentful way, just in an honest, non-judgmental sharing kind of way. I realized I had never even thought about that. Because I had never let myself. Then it hit me-if I didn’t have my disease, would we be living the dream and walking to dinner and the movies all the time? I had never even considered it. But Noah, on the other hand, confessed he does think about it. He also confessed, somewhat jokingly but also seriously, he sometimes wishes we had a simple fold up wheelchair because then he could realize his dream of us being able to walk places. What? I mean, how could he be so level-headed and practical about it? Sure, what he was saying made sense, but slow down a minute!  Did he expect me to just get on board with that plan? Of course not. But could I see how it makes sense that he’s just patiently waiting for me to get to the next phase of- wait for it-acceptance? Yes, I could.

Instead though, as we stood in our driveway contemplating what to do, I told him to run in the house and get me my walking sticks. I probably use my cane about 30-40% of the time now, depending on what I’m doing, but I knew if I were going to take on this own personal challenge for myself, I would need the sticks (Leki trekking poles to be exact).

We started down the street, each of us regularly  wondering aloud if Noah should just run back and get the car. My stubbornness, of course, would have none of it.  It felt empowering. It felt like a gift I was giving my husband. He didn’t need it nor did he even ask for it. But I wanted to give him the experience. And it was actually okay. Until…

…we turned onto the main boulevard. People everywhere. Couples, families. Potentially people I knew. Patients even. I wouldn’t say I felt panic (luckily that emotion’s not really my bag), but rather extreme discomfort and self-consciousness. Not only did I have two trekking poles while out on a Friday night date with my husband, but at this point my gait is /feels so exaggerated, so obviously off. Dare I say weird? I felt embarrassed. I felt like I didn’t want to make eye contact with anyone. I felt like I didn’t know if I could go out like this. Noah said he would hold my poles and I could just hold onto his arm like we usually do. And then I felt ashamed. Disappointed. Saddened by the fact that maybe I’m part of the problem?

What is all of this? Where does this come from? When did disabilities-or better yet- when did  being different become such a bad thing? Something to fear, to try to hide, to feel embarrassed about? Is it me and my own personal hyper-self-consciousness I feel at times with this disease or is it born from a society where it doesn’t always feel safe to be different? Both? Neither? When did we start caring so much about what others think? Obviously, one interpretation would be that when we are uncomfortable with what others may be thinking about us, it’s because we aren’t fully comfortable with it ourselves. But I pride myself on putting it all out there. On not caring what others think because all that matters is how I feel about the life I’m living. Right?

I mean, didn’t I just post a blog one week ago about reaching acceptance? What the hell?

Lo and behold,  it turns out that was just the first layer. Of apparently many. Damn it. It sure would be nice if I were there already.

I’m not going to use this post to  try to solve any questions or provide any deeper understanding of why this is such a pervasive, universal experience, disability or not. Maybe next time.

For now, I’ll just say that we thoroughly enjoyed the movie and I walked all the way back. Turns out when I’m not in a rush, my disease is a whole lot less annoying.

Oh and I’m really hoping for my sake that last night was the last time I intentionally reserve us movie seats towards the top of the theatre, up those treacherous stairs.

Friday, July 31, 2015

Chapter 14: The 9.5 Year Phase

Wouldn’t that be so nice? If there were actually an entire textbook dedicated to HIBM? One with an index where I could look up what to expect a year, two years or 10 years from disease onset? With chapters like, “How Much Exercise is Okay?” Or, “What is the Role Hormones Play in Disease Progression?”

Because HIBM is so ultra-rare, there are relatively few scientific groups in the world working on it . We simply don’t have enough information on HIBM to fill up a textbook. Maybe a thick pamphlet. And though we have some general guidelines and a basic understanding of this disease, what each of us desperately seeks to know is the rate at which our own personal bodies will deteriorate. And no one can tell us that. There is only one line we patients get to read over and over again in terms of statistics and numbers; hard facts. It’s the one that’s been haunting me for these almost 9 1/2 years: “On average, HIBM leads to severe incapacitation within 10 to 20 years of onset.”

Tonight, while starting to wind down from a long, busy, active week, I had what I guess I would call an epiphany. I realized that for these last 9 1/2 years, without conscious awareness, I have been holding my breath. Not to the point of respiratory distress, just to the point of not being able to fully inhale and exhale. In eight months from now, I will officially enter into that feared chronological phase. And yet, though I can’t guarantee it, it appears extremely unlikely that I will fall into that category, the one of “severe incapacitation.” Doesn’t mean I never will. And doesn’t mean I won’t even within 20 years. It simply means that at the 10 year mark- the one I’ve been dreading all along, the one I can just barely make out in the distance- I will not be where I so desperately feared I would be, back when this all started.

Let me tell you what “severe incapacitation” has meant to me all these years. The vision is one of me sitting in a wheelchair with my hands folded on my lap, needing to have someone feed me and having to use a water bottle with an extra long straw so I don’t have to bend my neck. I don’t know if I imagine this scene because it’s my own personal worst-case scenario or because I’ve actually seen HIBM patients progressed to such a state. When I have, those moments have been traumatic. And if you know me, you know I don’t mean that in any offensive or disrespectful way. I mean it in the most honest, authentic, compassionate way.

I am slowly yet suddenly becoming conscious that these images have been flashing in my mind for the last 9.5 years. And so it helps me explain why tonight, in this moment, I feel almost a kind of elation. An intense surge of gratitude. It may be the first time that I don’t feel angry at the fact that at age 40, I have to feel grateful for not being physically incapacitated. Instead, I feel joy. Relief. It feels as if I am finally fast-forwarding through that vicious soundtrack. The one that’s been on repeat: “But I shouldn’t have to deal with this disease in the first place.” I think it feels like true acceptance.

I do have this disease. That part already happened. It’s over. This is what’s happening to me, now. It’s time to let go.

I can not tell you how many times a day I use the word “acceptance” with patients. And so often they look at me, quizzically. “But how do I do that? How does that happen?” I don’t blame them for the confusion. It’s vague and abstract and there’s no how-to guide. But I always offer the same response, “you have to mourn the loss first. You have to hold the feelings, look at them, let yourself feel them and then move on. Set them free.” I tell them that only then will they will no longer distort their thoughts. They will no longer engage in as many cognitive distortions, all the catastrophizing and “fortune-telling” that triggers the panic and anxiety. I can’t promise them it will work. But internally, I can vouch for it.

We do not have crystal balls. We cannot know the future.
This is not my opinion. This is not me trying to make a convincing argument.
This is simply a scientific fact.

Tonight I am just where I am.
It’s that plain and simple.
And it feels really good.

Friday, June 5, 2015

What goes up must come down

I’ve had a good few months. Like, really good. If you’ve hung out with me lately, you’ve probably had the fleeting thought of, “Um, is Jen on stimulants?” As in, I’ve been talking fast and thinking fast- what I jokingly refer to as feeling “high on life.” And though the only stimulant I’ve been using lately is coffeeI have managed to sustain such a happy baseline and keep such a positive outlook. I have whispered things to myself like, “maybe in my lifetime there will be muscle transplants.” Or even, “I’m sure the FDA’s going to approve a treatment any day now.” Basically, I have been how I think people hope that I am- feeling grateful and happy for all I’ve got. Feeling blessed to have an incredible husband, feeling joyful to have two healthy boys and feeling relieved to  have a job that so suits my physical limitations. I have been a model happy, brave person.

But what if I want more? What if I’m sad that I can’t have all of that and my body too? Would that be okay? What if the line I’ve been telling myself these last 9 years-the one that says I’m sure the universe just had to give me this disease in order to give me all the amazingness in my life-isn’t true? Or the one that says, “Well, everyone’s got something, right?” Sure, everyone’s got something,  but everyone’s something isn’t as bad as this something. And I recognize that mine definitely isn’t as bad as others’. But I’m starting to wonder if maybe, in fact, it didn’t have to be this way. That I wasn’t given this disease to be an advocate for it or to “be the one who could make a difference.” That the perspective I would gain from living in a constant state of uncertainty couldn’t only be achieved by confronting this kind of adversity. But what if, what if that’s complete bullshit? What if I would be the same person, with the same personality, values, gratitude, etc without this disease? What if I would have an amazing husband, healthy kids and a great job and my physical strength? What then? How am I supposed to cope with that? What am I supposed to tell myself in the darkness of the night when I’m having trouble rolling over from one side to another? Or when I can’t wiggle my toes? What then can I desperately grab onto for reassurance? That this is it? That this is actually happening? To me? At this age? Well, fuck that.

I’m sorry. For the language. I’m not sorry for what I’m saying. But I wish it didn’t have to be so intense. Painful. I know, it’s so uncomfortable. But these are the realities we need to hear. These are the feelings we need to let ourselves feel.  As much as I wish I weren’t crying right now, on a Friday night at 8:15pm, I am equally as relieved to know that once I purge these feelings tonight, once I let them take me over, once I carve a hole for them to pour through, I will then probably laugh at a funny movie with my husband later and have a really good night’s sleep. Because that’s what happens, every time.  Not because I have some magic power to turn on and off my feelings, but because I can stop investing in trying to convince myself that it’s fine or that it’s going to be fine and instead invest in making room for all the scary dark feelings to move on through. This is essentially what therapy is. This is what I have dedicated my life to- teaching people to learn how to feel their feelings. You’d be surprised at what an epidemic difficulty feeling feelings is.

If you’re wondering where all of this is coming from, in three days I will be leaving for a week long vacation in Hawaii with my family.  I mean, is there anything in the world that sounds better right now? Except that rather than feel the excitement, I feel the dread of not knowing how I’m going to navigate the beach every day. Especially after seeing how hard it was for me last weekend here in LA.

It would be so nice, just once, to not have to hold both.

I always know that this is the cost of letting myself feel so happy for so long. But I always forget how rough it is to come back down.

The thing is, it’s still worth it. And I have to believe it will always be.

Tuesday, April 7, 2015

Where to invest

I haven’t written in a while. Not since January in fact. Lately I’ve been spending some time thinking about why that is.

It’s definitely not for lack of difficult or painful experiences related to living with HIBM.  I have come to realize that such experiences are built into my daily life and will continue to be a part of my daily life, for the rest of my life. In some ways, these last few months have been harder than most- I have attended a conference where I met a handful of other HIBM patients, all of whom were wheelchair-bound, except one. I have stepped out into the world now on numerous occasions using my new cane, forcing me to not only confront the fact that I am getting physically weaker, but also forcing me to endure extreme levels of self-consciousness and emotional discomfort. A level of vulnerability I have not experienced until now. And I recently returned from an annual vacation destination where evidence of my progression, as manifested by my difficulty in climbing up the same little hill I’d been able to climb so much more easily in years past, made it extremely challenging to stay calm and not fast forward to the day I won’t be able to climb it at all.

So why then haven’t I been depressed? Why haven’t I been living in that pain? Why hasn’t it been consuming me? How is it that I can have so many days, more days than not, when I feel truly, genuinely, authentically happy? It’s almost hard to believe. Or maybe it doesn’t seem possible. I can assure you though, I’m not one to minimize my feelings or deny my truths. When I feel sad and overwhelmed, I let it in and I put it out there. This phenomenon-experiencing true joy and inner peace despite such upsetting and at times, terrifying circumstances -is one that even I have trouble understanding. But here’s how I think it works.

If you spend less time and energy fearing the bad that may happen and instead invest more emotional energy in cultivating the good that is or can happen, then, when the bad stuff strikes, as it inevitably does, it will only take a part of you rather than the whole of you. It may derail you off your tracks,  the ones you just assumed you’d always travel on, but you will take with you all that is good in your life. That part is still intact and gets to come along for the ride, no matter how scary or unpredictable it may be. The message here is not one of, “look on the bright side” or “just be happy for what you have and don’t dwell in what you don’t have,” because of course, one never has to choose. We can be grateful for our blessings  while simultaneously feeling angry for our losses. What I’m referring to is something different. It’s not about how to think about your life but rather how to live your life. Invest in the aspects of your life that you can control. Cultivate meaningful relationships, seek out true love- familial, friendly or romantic. Find fulfillment in how you spend your days. Don’t let others interfere with your vision of what you want your life to look like. Because when loss occurs or when tragedy strikes or when circumstances beyond your control arise, you will lean back into that same life,  just with the addition of this new scary dimension. It adds a challenge. It doesn’t subtract the good. But if you don’t have the good, if you don’t invest in it, if you don’t seek it out,  then the bad can easily go from an added dimension to the whole of it. It can consume you. The imbalance can be so great that no matter how many steps back you take and look in, you can’t find that perspective you’re so desperately seeking.

I sometimes used to tell myself it would be much easier if I were a miserable person with such a miserable disease. That being such a happy person with a miserable disease makes it so much harder to cope because the cognitive dissonance is too great. But the truth is, being a happy person with a miserable disease is what allows me to be just that.

Please visit the NDF website for more information about HIBM and how you can help fund a cure

Neuromuscular Disease Foundation

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