Reality check

That was really hard.

We were invited to my uncle's house for shabbat dinner tonight. My husband Noah couldn't go because he had his holiday party for Kaiser at the same time (doctor's and nurses only). My dad was also going to my uncle's and together we decided he would come over early so we could drive together, mostly because I was concerned about coming home alone with both boys and getting them both inside safely.

So, all was running according to plan, (except for the addition of my 4pm visit to the ENT doc to evaluate my month-long laryngitis spell). At the last minute, I decided that my dad and I should just take our own cars. I was going to leave around 9pm (that's early for Persian shabbat dinners) and I didn't think it was necessary for my dad to drive all the way home with me, then pick up his car and then head back to my uncle's for the rest of the evening. Plus, he was on-call and what if he had to go in to the hospital for a delivery? I honestly thought it wouldn't be that big of a deal.

But it was.

When I brought both boys home, Lev was already asleep in his car seat. Asher was easy. He got out of the car and just wanted me to let him in the house because he was cold and tired. I said he could climb into bed and that I'd be there to tuck him in. I never made it in time. He eventually fell asleep. But that was not such a big deal. Better, in fact, because then I could take my time strategizing how I was going to get this 22+pound baby into the house without waking him. Meanwhile, Noah kept texting me asking if he should leave the party to come home and help me, but I genuinely thought I'd be fine.

The first challenge was just getting the car seat out of the car. Because my left wrist and fingers on my left hand are weak, I can't really get a strong enough grip on the bottom end of the car seat, so I always have to be right next to the stroller and hope my fingers don't slip. (I would never risk his safety, I somehow always manage, but it's always a major accomplishment. Luckily, tonight was fine). Then, I strolled him up the path and got to the two steps. I usually use the other stroller when walking (or someone's always there to help me) and in that moment I realized that no matter how many different ways I tried, I wasn't going to be able to get the stroller up the stairs. I tried to tilt it going one way forward and another way backwards. Then I decided I would just lift the whole seat off the stroller, and onto the top step. Okay, I was able to do that. Then I carried the stroller up separately. And that's where I really got stuck. I thought I could just put the car seat back onto the stroller and stroll him right into his room. But I cannot lift the car seat up off the floor. No matter how hard I try. Then I thought if I shifted the big handle forward, I could hold it by the handle rather than from underneath. The problem with that plan was that my left hand isn't strong enough to squeeze down on the part of the handle that releases it to pull it up. So I tried to release it with my right hand and quickly move my left hand there to keep it, but I just couldn't do it. By then I turned off the bright light on the patio because he was waking. I stood there in the dark, staring out into the sky, trying to juggle wanting to give up and break down and feeling proud of myself for not completely losing it in utter frustration.

Then I saw some dinner guests leave the house of the neighbors across the street. Ahhhh, this was my chance. My neighbor came out with her dogs and her girls and I called out to her. But then another neighbor with a dog walked up to her and their dogs started playing, loudly, on their front lawn. I called out to her a few times, but with my scratchy, quiet laryngitic voice, she just couldn't hear me. It also didn't help that I was wearing all black while waving and standing in the dark night.

By then I gave up. After all, was I seriously doing all of this in an effort to avoid waking my sleeping baby? Yes, but it was clearly more than that for me. I bent down to the floor, unbuckled him, and with all the strength I had, lifted him up and out and managed to stand back up. Of course, because I did it so clumsily, he woke up, but I managed to get him into his crib where he fell right back asleep.

If you're reading this, I imagine you may be thinking of all of the practical, wiser ways I could have avoided this: have my dad drive home with me, tell Noah to come home and help me, hire a babysitter, decline the invitation to go out, etc. But it's not the rational thinking that's hard. I know all of that. It's times like these when I am reminded of how hard it still is for me to accept that this process is happening to me, to my body and that I have to take responsibility for it and act accordingly.

I know the game I'm playing with myself. I know that I still sometimes turn to denial to cope with this disease because that's the safest place for me. I'm trying to hold onto the hope, maybe even the fantasy, that I can still do this. That I can be like so many other moms, like so many other people. That I can still be independent, that I can still be capable, that I don't have to inconvenience others.

But the simple truth is, I can't.

And sometimes there's just no way around it.

When a cold was just a cold...

This last month has been tough. I have literally been sick with one cold after another for what has felt like an eternity. And though, like most people, I've never enjoyed being under the weather, ever since being diagnosed with HIBM, being sick or suffering some sort of physical discomfort has taken on a whole new meaning.

In the old days, pre-HIBM, being sick meant feeling temporarily miserable. It was an annoying interruption in my day-to-day routine, but nothing that I ever really spent too muh time worrying about. Sure, it was frustrating not getting to the gym, but I could force myself to enjoy the down time and even the side benefit of not having an appetite and maybe losing a few pounds. Most of all, I could look forward to when I would feel healthy again and get on with my life.

These days, I long for when it was simply annoying to be sick. Now, being sick triggers a cascade of anxious thoughts and fears, some irrational and some, unfortunately, completely legitimate.

The first stressor is the fact that I can't exercise. Between having two kids and working more and more, exercise has been cut down to about once, maybe twice a week. People talk about how exercising keeps them sane, but for me it's so much more than that. It is almost the only way for me to have some peace of mind that my body can still do certain things. It is one of the primary ways in which I cope. It used to be about staying in shape and keeping my figure. Now it is just about trying to momentarily reassure myself that my body isn't completely failing me.

But it's not just the psychological benefits that I gain from working out. The truth is, my muscles are always atrophying at a slow rate. If I don't actively work on maintaining what I have, I will lose that strength. And thus far, that loss is irreversible. It's not as if I can get back in the gym after a few weeks and regain that strength or rebuild that muscle. So every day that passes without exercise brings with it a heaviness, a deeper awareness that I am losing strength and not doing anything to prevent it.

When I'm sick, I can't afford to not eat right or get enough sleep, despite my lack of appetite or tossing and turning in the night. I don't get to not take care of myself without paying a high price. I don't get to skip breakfast or eat cereal for dinner or be up half the night coughing without wondering how that's affecting my disease, my muscle strength, my future. The pressure can get intense. Sometimes, I just want the luxury of not caring.

When my body is even weaker and clumsier than usual, I am more dependent on others, namely my husband, and that awareness catapults me into a dark place. As I lay in bed in the early morning and hear the footsteps of my four year old down the hall or the cries of the baby who needs to be picked up from his crib, I am often overwhelmed with panic. The thought of pulling my body out of bed and shuffling across my room is too much in that moment. And when I'm sick and I can't even force myself to motivate to get up and out, I start to have flashes of what my future may look like. I start to hear in my head the words that are always used to describe HIBM, "severe incapacitation within 10-15 years of onset." I start to quickly re-calculate for the umpteenth time how long I've had it and how much longer until then. Worst of all, I start to wonder/dread what it will be like for my dear, sweet, loving husband, who already has to bear so much of the burden on my behalf. I look over at him, knowing he just wants to stay in bed for 5 more minutes and I am so in touch with the hatred I have for this disease and the fact that he has to be in this with me. No matter how many times he reassures me, I always return to the same thought, "he doesn't deserve this."

Living with HIBM means constantly trying to balance staying engaged in the present and feeling grateful for all that I do have against being overwhelmed by my reality, by the ugliness and inevitability of this disease and by the frightening unknowns of my future. I know I am always saying this, but it's the greatest challenge for me. I devote so much psychic energy to trying to stay calm, to trying to live in the now, to trying to not fast-forward every time there's a new loss. I suppose it's no wonder that I always get sick these days. I can only imagine the stress hormones that are being released on a daily basis as I try to keep the low grade, baseline level of anxiety under control. When my defenses, both psychological and immunological ones are lowered, I am much more in touch with what's really happening to me and I have much less strength to fight it. I become too aware of the fact that even when I do recover from my cold, I will still have to continue to fight this disease.

There's just no way around that reality. Sometimes all I can do is accept it. And that's just so incredibly unsatisfying. Especially when I'm sick.

Bittersweet

It's been a while.

Life has been a whirlwind and I haven't really had a moment to take it all in. I am grateful for the chaos though, as it keeps me too busy to get stuck inside my head for too long...

Today marks the beginning of an exciting new chapter in my life. After eight years of marriage and with our two beautiful healthy boys, my husband and I moved into our very first home. And in so many ways, it is all just so perfect- sunny and bright little Spanish-style, a nice quiet street, shops and restaurants near by and friendly neighbors with young kids all around us.

But as hard as I've tried to just stay with the joy, this stubborn, scary, nagging little voice has been haunting me. And it's been getting louder. I've done my best to ignore it until now, but I'm afraid that what it's been saying all along may be true.

You see, we spent many months looking for a house. There were some basic requirements, such as it having to be a one-story and on the smaller side, for obvious reasons. But in the neighborhood where we were looking, it seemed that we couldn't avoid a house with just a few steps to the front door and a few steps to the backyard. And all of them had layouts that included a hallway of sorts. At a certain point I realized that I had to accept that I wasn't going to find everything I wanted in one house.

Since I wear leg braces every day now, I had only ever gone to open houses with them on, partially because I didn't want to accept/believe that something as silly and minor as a couple of steps could come between me and my dream or worse, my husband's dream. So, we found this lovely little house. Just what we were looking for. And I convinced myself that the steps in the front and the back would be manageable. I decided that if I ever got to the wheelchair phase, then I could easily put ramps in. And if not, then I would just get railings installed on the sides of the stairs. And because of the long hallway, I would just keep my leg braces on all day and night, despite the callouses and the sore spots. That was my logical, rational mind talking.

And here I am. Our first night. I have been on my feet all day, moving since early this morning. It's 9:30pm and I have yet to take off my sneakers and leg braces. I am too afraid. Too afraid of how my body may (poorly) navigate through this beautiful new house and too afraid of how I will then handle it emotionally. Though I have tried to prepare myself for this moment for months- talking myself through the possible problems and potential solutions- for now I want to just live in the fantasy/ denial just a little bit longer: what if it's not as big of a deal as I thought? What if I won't trip on the slight elevation in the floor between the tile and our new hardwood floors? What if I actually will be able to carry a bag of groceries through the front door by myself without losing my balance?

Eventually, I will have to stop stalling, get off the computer and enter into my new life, but not because I want to. Mostly because I have no other choice. As anyone with HIBM can tell you, it's the initial feelings of loss and shock that come with navigating new territory that are the hardest, but eventually we find a way to adjust.

I am still amazed by how I can feel so extremely happy and grateful and blessed while simultaneously feeling so angry and sad and scared. How could I have looked forward to this day with as much passion as dread? How can I be so lucky to get to own a new home, be in love with my husband, have these two smiling little faces to wake up to and be surrounded by such wonderful family and friends AND have this nasty disease that continues to rear its ugly head in my most joyous moments?

I try not to question it.

I try to hold onto both extremes in one hand and try not to interfere with process that takes over in my mind and heart.

And most of all, I try to be in touch with the truth that some things are beyond the physical...

Just ask me.

A couple of days ago, I asked my four year old son if he wanted to come change his baby brother's diaper with me. As I slowly made my way over to the changing table, he ran (as he often does, because everything seems to be a competition these days) and arrived there first, stating, "I won Mommy! I'm faster than you. You're slow. You wear leg braces."

As I carefully placed the baby on the table, I was surprised by how little his statement bothered me. I didn't burst into tears. I didn't feel hurt. Nor did I feel angry. But why not?

Maybe because what he said was not a judgment, not a criticism, and not mean-spirited in any way. It was simply a descriptive, factual statement. He was merely commenting on what he has observed in his most innocent, untainted four-year old way.

It's been said plenty of times before- children bring a fresh, unfiltered honest perspective to our reality. They have not yet been influenced by the stigmas that seem to plague our culture and society. After I was diagnosed with HIBM, one of the many thoughts that kept recurring in my mind was the impact my disease would have on my future children and/ or on my relationship with them. I imagined angry outbursts on their behalf, "Why can't you just be like all the other moms?" Or sad moments, "I wish you could come ______ing with us (insert your favorite family fun sport- skiing, playing tennis, hiking). Or most devastating, "I'm embarrassed." Let's face it, kids don't like to be different. But where did that come from? Why is it that having a disability often provokes embarrassment, shame and a desire to hide? I should re-frame that. Why is it that I felt such feelings when I was diagnosed?

I suppose it's no surprise, for in my culture, disabilities, weaknesses, diseases and defects of any kind are considered taboo. But it's not just within the Persian community. Based on my personal experience, it seems quite pervasive. People are so uncomfortable around those of us who are different in this way. In the beginning, it took me a while to "come out." I went from keeping my HIBM diagnosis a secret from most to now wearing my AFO's (lower leg braces) out in public with a dress and sneakers for the world to see.

It continually surprises me how comfortable people are to stare at me or rather, to try not to stare at me, and yet how uncomfortable they are to ask me about my braces for example. There's a natural curiosity to their stares. And I get it. And that's okay. So just ask me. Ask me what they're for, how they work, what they feel like, etc. It's as if asking me about them or what's going on with me will somehow be upsetting to me or "make me feel bad" about it. But I am living it every day. It's already upsetting at times, whether we talk about it or not. It's when people don't ask, that it can feel hard. It can feel lonely. It feels as if it's too much for other people to hear, to take on. That's when it feels like it actually is a bad thing, something that shouldn't be discussed and something that I should try to hide.

Life can be difficult and challenging at times, but it doesn't have to be bad. We don't have to be afraid of the hard stuff. Sharing experiences and trying to understand what it's like to walk in someone else's shoes is what brings people closer and ultimately what fosters acceptance.

I happened to have been born with a certain genetic mutation that caused certain symptoms in my body. That's all. I have accepted that. I have made peace with it (or at least I try to most of the time). I wish that others could more easily accept it too. And not be afraid to look, to ask and to try to understand...

It's My Birthday

I have never blogged before. Maybe because it felt exhibitionistic or maybe because doing so presumes people will be interested in what I have to say. But recently, I have felt a need, an urgent and desperate need to express myself- to share what's going on with me and my body, to help people understand what it's like to live with a progressively debilitating disease and ultimately, to help raise awareness and call attention to this orphan disease. The truth is, hardly anyone even knows the name of this disease that I suffer with every day.

Five years ago I was diagnosed with a progressive neuromuscular disease called Hereditary Inclusion Body Myopathy. It is a very rare muscular dystrophy that typically strikes men and women in their 20s or 30s and then, according to all of the literature, leads to "severe incapacitation" within 10-15 years of onset. There is currently no treatment or cure. But there are labs in different parts of the world that are either very close to establishing one or simply waiting for funding to start human clinical trials on treatments already discovered.

I have not always felt comfortable talking or writing publicly about my condition. When I was first diagnosed, I was scared, embarrassed and ashamed. I did not tell anyone. I was having a hard enough time processing the information myself, let alone having the headspace to process other people's reactions to it. But after a couple years, once I had moved closer to acceptance, I gave a "coming out" speech at the Neuromuscular Disease Foundation fundraising gala in June of 2009 and I shared my private thoughts and fears about living with HIBM. As vulnerable as I felt going public about my life with this disease, it was equally liberating and therapeutic. I have given two more speeches since then. For me they have evolved into an opportunity to educate people about HIBM, to chip away at the stigma (especially in my community) of having a genetic disease, and to inspire people to spread the word and ultimately, be a part of a cure. For the last few years, the annual speech felt like enough for me, like it was as much of myself as I was willing to share for the cause. But now that I am farther along in my disease progression, the anticipatory anxiety and fear that I regularly feel make it hard to feel like anything is enough. It's becoming harder to find ways to cope with not knowing how much worse I am going to get and how long it will take. Because it never stops.

My hope is that if I keep up this blog, it can be yet another tool to help me process and cope, it can be a source of support for others struggling with the same or similar challenges, and it can help inspire people to join me in raising awareness and working toward a cure.

I am launching this blog on my birthday because for me (and I imagine for most people living with a progressive disease) birthdays have evolved into something very different from what they used to be. In the past, I looked forward to my birthday - a day to celebrate and be celebrated, a day that felt carefree, light and fun. I was never one to fret about getting older or to wish for anything beyond what I already had. But now, I almost dread my birthday. Mostly, it just feels like a painful reminder that I am getting one year closer to worse. Another year of new losses. Another year to look back and remember how much stronger I was on my last birthday. This is my second birthday wearing leg braces. And this is my first birthday that I am experiencing weakness in my right hand that makes it harder to write with a pen.

You see, it is not hard to fall into the deep dark vortex on my birthday, especially when I can painstakingly imagine all of the birthdays ahead of me and all of the losses I will have accumulated each year. What is hard, is to see beyond the darkness, to perceive my reality through different lenses. When I am able to do that, I can celebrate that this is my first birthday as a mother of two. On this day last year, I was convinced I was never going to be able to realize my dream of having a second child. This is another birthday I get to celebrate life with my husband, someone who always seems to make me feel like it's going to be okay. And, as my sister pointed out to me, this is another birthday I get to celebrate the fact that I can still walk, with or without leg braces.

That's the funny thing about life, we get to choose how we are going to frame it, how we're going to interpret it, what we're going to make of it and how we're going to celebrate it.