What do you do when it is 3AM and there is no one to talk to?...Watch Food Network.
Bedtime, All In a Day's Work
I just finished making dinner and truthfully this was about the only thing I did today. Pippi laid faithfully by my feet to catch any droppings. I think she thinks she's helping mom so she doesn't have to bend over.
We just got back from LA last night and I am emotionally and physically tired. I slept nearly all day today and had a hard time getting around in my house. I was in LA for almost a week and 4 of the days were without Jason. Every time something changes in my daily routine, or life, my body has to go through some major adapting and compensate to any little change.
Personally, I love change but my body votes heavily against it. The "change" can be anything from sleeping in a different bed, showering, how far I have to park my car, how the street inclines and differs from what I am used to, counter heights, chair heights and the list grows every day.
My visit was busy and I had lots of meetings, many of which were with LA Times.
Obviously this is great for awareness, but emotionally and physically it got hard towards the end of my visit. I cried alot on this trip. In fact I cry more recently and for some reason this year has been tough. I share this because I know there are some patients that read my blog and I think it is important to be open and honest. I don't want them to think that I'm perfect. that I never get upset about it.
In fact, if I wasn't expressing sorrow, I'd worry more about that. No matter how positive I may seem it is still hard. The positive face is not a facade, what you see is what you get with me, but I still experience really difficult times.
There is a statement that I hear often and it is very telling. "Kam, wow, you look great. You look healthy..."
I realize visually I may look fine. I look kept, my hair isn't crazy, I'm smiling and I don't have food all over my face (or do I?).
But my body is anything but. On the inside I am deeply conscious of all my physical struggles and it is a constant challenge. When my body is physically challenged to its brink it can be hard on the spirit. I think it is because I am at a point where going into a wheelchair would relieve me physically and in essence potentially gain more independence, but I am trying to keep myself walking. I need to keep myself walking.
A chair presents problems, too. In some ways I would gain the ability to move around faster but lose independence as well.
Another one of my friends I met with was shocked to hear that HIBM was debilitating.
Now, I've told this person more than a few times that my condition is debilitating, progressive, could reach paralysis-like symptoms, quadriplegic state (though HIBM is not nervous system oriented). I'm not sure how many ways I can explain what "debilitating" means and sometimes it is truly frustrating. I feel like no matter how much I talk about it, no one really understands what I am saying.
It has only been recent that I have started describing the potential prognosis as quadriplegic state and I know this may be hard to hear, but I just don't know any other way to explain the seriousness or the sense of urgency attached to it. I imagine it is an abstract concept to many.
How can someone so young, someone I went to high school with, someone I went to college with, someone I played soccer with, someone who is walking right now in front of me get to a debilitated, bedridden state?
***I must note that not every patient ends up this way and every patient progresses differently.
I have to stop typing now. I'm in bed hunkered over my laptop and having a hard time with my neck. This is a recent weakness. If anyone was here watching me type they probably wouldn't notice that it was hard for me, but every muscle in my neck is working overtime to do something as simple as holding my head in this position.
It's bedtime. BED, we shall continue our day-long love affair.
Out of Love...and Black Licorice (Roadtrip to LA)
I am up early and on my way to Los Angeles for some ARM business and meetings; including another meeting with an LA Times writer who has been pursuing my story for a couple months now. In addition, it's ARM's 10th Anniversary and we are celebrating and fundraising in Gala fashion. Let's see if I can encourage others to attend and support the event. This is always the difficult challenge - getting people to come. We haven't sold many tickets which makes me nervous because the event is right around the corner (June 27, in Los Angeles).
Jason packed the car before he left for work. This is Pippi's first road trip and I'm wondering how I am going to manage her and all my appointments, but there is no other option but to take her. Good thing I have good friends in LA :). Unable to sleep, I have been up since 3am so I'm already tired. I just stopped to get a coffee drink (shhh, don't tell Jason. He hates that I drink these overly sugared coffee drinks). I'm in the parking lot trying to unwrap a straw so the deliciousness can begin, but I'm having trouble. Ahhh, it's going to be one of those days. I find myself focusing on forcing my languid fingers to unwrap a silly straw.
I just arrived at San Francisco airport and waiting for my friend. I can't do road trips by myself anymore which makes me sad because it has ALWAYS been one of my favorite things to do. I love driving long stretches alone because it is where dream up all my best ideas. A drive from San Fran to LA is about 6 hours but I can't do that drive, so I'm at SF airport picking up my friend who flew up so she could drive me down to LA. Since I moved to northern California this will be our second time doing this. Her name is Sandra and she is the wife of Dr. Babak Darvish; an HIBM patient, ARM Co-founder and President. She's so much MORE than just "the wife" but I'll formerly introduce her in another post. She paid for the plane ticket out of her pocket just so I could come and out of pocket I pay for the road trip and Jason's ticket so he can fly down to LA and drive back up to San Fran with me. Currently this is how we handle me traveling to LA. Sandra's only condition? That I buy her some black licorice for the road trip. ;)
ARM is a nonprofit that works hard to achieve every dollar donated, so it is these little volunteered acts that helps them in little ways. ARM and HIBM Research Group (HRG) are made up of only a few people who have accomplished much for the future of HIBM medical research - despite many struggles and financial barriers. It seems like we constantly do so much for so little, in hopes that around the corner someone with means will take interest, invest and help take HIBM research farther.
It's really nice that Sandra picks me u, but then does it on her own dime. I love her and the Darvish family. These selfless acts are out of love. They work patients like me; out of love and empathy knowing what it is like to be us because they ARE us. Knowing how emotionally painful it is for me and then knowing there are others experiencing the same moments makes the experience even worse. Generally I am a go-getter. If I see something that needs to be done, I'll do it but witnessing this sort of love is what makes me work harder. It's not just for myself but out of love, gratitude and inspiration to those who work for me. Seeing those who have sacrificed their life and well-being for the greater good of this condition is not an everyday occurrence.
As time has progressed, I realize that not everyone responds to pain with love and giving. Some become inverted, angry and selfish, which is understandable in many ways, but I can't see it this way. How can I just stand by and do nothing? It would be like me ignoring this firsthand life experience, this opportunity to learn despite the struggle. I think it's love and will that keeps us going. That has to be it. That can only be it. Because it would be too difficult without it. I've found that giving comes from inspiration and vision of potential. If you're really inspired, grateful and humbled - giving is genuine; a natural act and less calculated. If it's a conscious etffort to give then it is forced accompanied by a lack of understanding of what is truly important. Love, grace, selflessness, understanding, tolerance, compassion: less about "me" and more about "you".
Emotionally and physically dealing with our condition and then trying every day to work for a cause that is constantly hit with barriers, down moments, not enough money, not enough "hands". It has to be love that keeps pushing our tired bodies forward. I wish you inspiration today, and if you do feel inspired then hand it off to someone else. Even if it wasn't me affected with this condition, if it was my family, friend...I would hope I would do the same.
Crayon Pirate
"There was a single blue line of crayon drawn across every wall in the house. What does it mean?, I said. A pirate needs the sight of the sea, he said and then he pulled his eyepatch down and turned and sailed away."
- Crayon Pirate-
Don't forget to use your imagination today, it will be your best source of direction. It usually is but we tend to get in the way.
Softer Days
I had a co-worker/friend that I would sometimes talk about my condition with, and most of the time I would spin it in a positive light even if I had just fell in front of her. One day I fell and she said to me, "Kam, you don't always have to be an after school special. It's ok to be sad".
I'm not my usual after school special today, for awhile, for that matter.
It's getting harder.
There are days when I have so much perspective and I can see way beyond myself. I see potential out of nothing, blissful in the little things, see the sheer goodness in others despite their glaring faults, sense love deep down, think life is too short and couldn't possibly house all my dreams and ambitions and take everything in stride, while devaluing what I am experiencing because I think anyone can do it.
And then there are other days when I’m just trying to keep it together. This is one of them.
I don’t feel like I belong anywhere.
"My grandma used to plant tomato seedlings in tin cans from tomato sauce & puree & crushed tomatoes she got from the Italian restaurant by her house, but she always soaked the labels off first. I don't want them to be anxious about the future, she said. It's not healthy." -Brian Andreas-
I'm sure the world will appear a little softer soon.
4AM
I feel shitty right now. My body feels like a corpse that I'm dragging around and I just happen to be along for the ride. It's 4am. My body aches, each limb feeling disconnected and I can't sleep. The air is warm and stale and there's the drone of snoring in the background. Uggggh, the snoring, at least I don't snore. No floppy tissue for this girl.
What the Heck Is HIBM, Anyways?
The textbook version of Hereditary Inclusion Body Myopathy (HIBM) looks like this:
HIBM are a heterogenous group of rare/orphan genetic disorders which have different symptoms and could be transmitted in an autosomal dominant or recessive fashion.
Generally, they are neuromuscular disorders, unlike MD which is skeletal muscle weakness, characterized by muscle weakness developing in young adults, causing progressive muscle wasting and weakness that can lead to very severe disability within 10 - 20 years. HIBM is also known as Distal Myopathy with Rimmed Vacuoles (DMRV), Quadriceps Sparing Myopathy (QSM), or GNE related muscle disease.
Now I could go into it further, talking about HIBM linked to chromosome 9, the defective gene regulating bifunctional enzyme GNE, that is the rate-limiting step in the biosynthesis of sialic acid and that Sialic acid modification of glycolipids and glycoproteins at the cell surface is crucial for their function in many biological processes BUT I'm guessing no one wants to read that or understands the "meaning" except for those in biomedical research.
The experience is so much different than the textbook image. In 2003, over the phone of all places, Mayo Clinic gave me my HIBM diagnosis. But my symptoms probably began in 1997/1998. Due to having 5 previous incorrect diagnosis', and a series of bad medical experiences which I will probably share later, I wasn't even sure if HIBM was the correct diagnosis. Clinically, though, I treated it as if it was, so at the very least I could seek next steps...if there were any. I'm the researcher type so naturally that's where I started. I remember sitting in a dimly lit college computer lab perusing online medical journals for information only to find the same bits of word clusters. I read the textbook definition, the prognosis, the symptoms and the black insubstantial words staring back at me; RARE, WHEELCHAIR, WEAKNESS, DEBILITATING, GENETIC, NO CURE, LIFELONG. I thought, "Well this can't be all of it. It doesn't make sense, there has to be more information...something". In actuality, it wasn't the information -- I just hadn't attached meaning to it yet. I was alone in my space - in that college lab feeling neither sad nor angry, but detached. I had been through a number of diagnosis' before, the poking and the prodding, the muscle biopsies, so to me this was just another diagnosis. I had a life to live and these words meant nothing to me, at least not as much as they do now.
In 2004 I remember visiting my physiatrist with this new diagnosis in hand thinking perhaps he would create some understanding out of all my research efforts. He evaluated me and asked me weird things like, "Can you be in a sitting position and rise without needing to hold onto anything?"
I thought, "What a silly question. Of course I can" and would demonstrate it for him in an almost cocky-like manner. "Hmmm, I can do this all day, Doc. Keep it coming".
Today, getting up and down from a seated position is difficult and slowly turning into non-existent. That moment in the physiatrist's office is suddenly clearer and more real than it was when it actually happened. As the years progress, so does my body and when I recognize those measurable moments of progression such as; climbing stairs becoming difficult to non-existent, to not being able to ascend curbs, to not always being able to roll over in bed by myself or having trouble with unscrewing a toothpaste cap, I grab from those insubstantial black words I read in that dimly lit library and I start to attach meaning to them - slowly, like adding photographs to a scrapbook.
de·bil·i·tat·ing (dĭ-bĭlˈĭ-tāˌtĭng) adj. Latin debilitatus, past participle of debilitare to weaken, from debilis weak
"Ahhh, yes. I know what that word means. Gosh darn it, I went to college and Webster tells me what YOU mean", but I so didn't. I so did not.
People who know me would probably say I'm upbeat, full of ambition, positive, optimistic but the truth is it can't possibly always be that way all the time...for anyone. It's not as easy as it may seem and everything is not "ok" all the time. There are days when it is hard to gracefully swallow the reality. Throughout my posts I hope that my condition will become less of a name and more of an experience. It's not meant to drum up pity or sadness for I have no use for such things and that crap is counter-productive, but perhaps I can paint a picture for the strangers who may be reading this or other fellow patients. So, what is HIBM, anyways? It's a part of me but not all of me. It can't be explained in just one post or one definition and the definition isn't as important as the experience. I'll keep sharing - I hope you'll keep reading and let's see how this thing turns out. :)
GreenGreenGrass - First Blog Post
I'm the one with the hat on the bike decorated with streamers :).. My youngest brother is right next to me. I actually remember this day vividly and the way that Michigan sun felt.
So, why GREENGREENGRASS (name of my old blog)?
I hate shoes and if I could walk barefoot all day, I would. I've always been like that and perhaps that is why I never placed much emphasis on shoes as an adult - even if I had the choice to wear any shoes I want, and choices weren't so limited due to leg braces.
Of course the cliche is we never appreciate the simple things until we lose them, and I am that cliche to a degree. I actually DID enjoy this simple pleasure when I was a child, though. I loved, loved, loved to walk through freshly mowed grass with my toes curling between the cool grass blades. At the end of the day I loved that my little feet were dirty and sometimes blistered. This showed it was a day of playing hard and nothing beat the Indian summers and the smell of freshly cut grass.
I have three brothers, which meant I grew up a tomboy. My biggest memories are running around with them, sometimes till sundown, all dressed in army gear, holding our plastic guns. I miss this simple pleasure so much. Nowadays, I pretty much can't walk without my orthotics/braces and shoes on. I named my blog GreenGreenGrass, because most things break down to simplicity...it always goes back to simplicity, just look at nature and it's design.
A couple years into my condition my balance was getting shaky and little things seemed to increasingly be an obstacle. I recall a memory of my younger brother, Josh, poking fun at me as I was walking out of the house. "Watch out Kam, the grass hasn't been mowed. It's extra long today", he said. I think I fell trying to walk through grass one day and that is where this joke came from. It's also when I realized, "Oh shit, that's weird...grass is a problem?". Josh was of course kidding and when he would say this joke, I would laugh so hard that the muscles in my back would stop me from walking. That's the way we are, we make jokes of everything and so do I. If you can't make fun of yourself then you're in trouble. He and I soon graduated to the joke of him rolling small pebbles under my feet as an "obstacle course". Apparently I was in training :)
I digress. Anyway, that moment was a simple one...but it stands out.
Because I had "it" and now I don't, I've become extremely observant. I see people differently, I'm aware of my surroundings because I have to be so I don't fall. I place importance on different things and I miss the feeling of running, the wind in my face and walking without having to think and planning EVERY SINGLE S...T...E...P. I miss it. I sometimes catch myself intently watching people walk or run and it is such an amazing thing to me. "How do they do it with such ease?", I wonder. If I see a group of runners or soccer players playing I will stop and watch their legs and feet in movement. I break down that miniscule moment into a system and it's so amazing. It is almost like I am trying to remember and gain that satisfaction through them. Why must we love when it's too late and gone?
I wouldn't say love something out of fear of losing it, because fear only holds you back, but love it because you can. Most of the time we are not aware that we are living a special moment.
If you're not conscious, then you're not alive whether you have a body or not and ultimately it is about observing observation. Really think about what it is to "see", the essence of what it is like to be alive. With that, despite the enormity of my struggle, I've never felt more alive and never have asked as much from life as I do now.
Please enjoy all the little things. Bask in them. Ridiculously relish in them even if it is a blade of nature in between your toes. Use it. Live it. Love it.