My Body

My illness has a few different names, but they all mean the same thing. Mitochondrial Myopathy, Mitochondrial Dysfunction and Mitochondrial Disease. My neurologist explained the differences just refer to severity with "disease" being the most severe. Here's a tiny bit about what I struggle with. 

What are mitochondrial myopathies?

Mitochondrial diseases are caused by defects in mitochondria, which are energy factories found inside almost all the cells in the body. Mitochondrial diseases that cause prominent muscular problems are called mitochondrial myopathies ("myo" means muscle and "pathos "means disease), while mitochondrial diseases that causes both prominent muscular and neurological problems are called mitochondrial encephalomyopathies (encephalo refers to the brain).

A typical human cell relies on hundreds of mitochondria to meet its energy needs. The symptoms of mitochondrial disease vary, because a person can have a unique mixture of healthy and defective mitochondria, with a unique distribution in the body.  In most cases, mitochondrial disease is a multisystem disorder affecting more than one type of cell, tissue, or organ.

My challenges appear as severe fatigue, brain fog and muscle weakness in my entire body. I also struggle with cramps, spasms and constant severe pain. These are all of the reasons why I can no longer work. Yes... even a little (a question I get a lot.) "Can't you just?" NO!!!

I've had this diagnosis for five years now. In that time my muscles have become progressively weaker.

On top of my mitochondrial disease ravaging my body, I have severe head problems. "Intractable migraine" and "neuralgia" (occipital and temporal). Icepick migraines can come and visit me too. They feel just how they sound. No one flat out said as much, but I expect these are all after effects from my brain surgery in 2018. I certainly never had any of these symptoms before.

So that's my body in a nutshell. All of these issues have combined to turn me into an incredibly mindful person. I fully live in each and every moment (that I'm awake). I also actively look for the beauty and joy that is everywhere to keep me going. These coping mechanisms are incredibly effective. Well, that and a lot of drugs and medical treatments.

The down side of is I struggle to plan into the future. Not intentionally. Not in a depressed way. But like my brain is too Zen and tuned in to what I'm doing in the now. It can make even little things like what to eat or watch on TV impossible for me to decide. It's a strange feeling. Maybe my brain is simply busy coping with the war raging in my body.

The week of May 11th, 2020 in Pictures

Fatigue Rescue?

I'm still recovering from a recent bladder infection. Those strike me from time to time and are common with wheelchair users, especially women. I have an issue with my bladder muscles not wanting to let go when I... um... GO. It's called "retention" and that also puts me at a higher risk for infection. Hey... Aren't those tulips beautiful! I thought we could all look at something pretty while talking about bodily functions.

When I'm sick my afternoon "naps" (or "curse" as I really think of it) eat up more and more of my day. Makes sense. We all need rest when we don't feel well, but I end up sleeping my life away. 

I'm excited at the prospect of something new that might help me. Epicatechin. As I said before my neurologist recommended I give it a try. It's an over the counter supplement derived from chocolate, so why not?

This is what I went with.I've had three doses now and honestly can feel a difference. I took my first dose last night and indeed it did keep me awake! I took another dose this morning with a banana and another with lunch. I feel much less tired than I normally do at this time of day. Like how I used to feel if I ate some chocolate covered espresso beans. The thought of having found something that can possibly wipe out my 2-4hr nap every day for $38.95 a month makes me excited. I hope, hope, hope this stuff really does help and I don't just acclimate to it. 

How I've Changed

Humans are bags of squishy bacteria and water. We're sensitive and vulnerable. It's amazing we live as long as we do given our ability to succumb to the slightest virus, infection or change to our bodies. And we do change. All the time.

My body has been through a lot in its 47 years. Car accidents, a pregnancy, multiple surgeries, TB, brain surgery and a genetic mutation. Without modern medicine I would have died a hundred times over by now. But not only am I still here, my serious health issues have changed me... for the better.

I believe I am a much better person now than I was before I had Latent Tuberculosis or my brain tumor. I am far more patient. I let little things go very quickly. I have a lot more perspective and love for others. I'm way less shy and more outspoken. I am fast to let people know I love them (animals too). I smile a lot more. 

But why?
How?

Because I know now what's most important. It's not being first in line or looking young and beautiful. What's most important to me now is that the people I love KNOW 100% that I love them. That I leave strangers feeling happier and in a better mood than before they interacted with me. That I make the world a little bit better just by being alive. By being here and a part of it. That's what matters most to me. Not superficial, shallow stuff.

Each day I'm grateful to still be here. I'm appreciative that I have my beautiful wheelchair and medicine to help me feel better. Doctors who try and help me live my best life and a family I'm devoted to.

I hope I'm here for years to come because I love my life.

Guarding my spoons

I love "spoon theory". As a visual person I can relate to energy being represented by spoons. Lately I've been learning how to guard my spoons better.

For example, if I have to take something to the back of my house (even though it's not that far) I'm learning to leave it on the table until I have more than one thing to go back. A big one that has been helping me lately is when I have to get up and down out of a chair.

If I need to do something that will require me to get up physically, more and more I will ask someone else in my home to do it for me (if they're around), or again wait until I have multiple things I need to do (like I have to pee AND I'll switch the laundry AND get myself more water). This does not come naturally to me. At all.

Did I mention this doesn't come naturally to me? I'm a multitasker for sure, but listening to my body and adjusting what I'm doing based on my what my body needs is incredibly hard. And I'm guessing that's not just for me, but for a lot of people.

I've noticed that now my spoons are now more dedicated to smaller things. 

One year ago 1 spoon would go to taking a shower and getting ready for the day. Now it's more like 1 for showering, 1 for putting on a bra, 1 for putting on makeup, 1 for the rest of my clothes, 1 for fixing my hair... which makes me much more selective about what I choose to do (not go around naked) and not do (wrangle on a bra every time). 

For me this is a huge part of learning day by day how to live with my chronic illness. Paying attention to what I can do that day, that moment and what's worth my spoons. 

Make Life Easier

Over the past two years I've learned ways to tweak my environment to save spoons. For example, opening a dresser drawer takes WAY more effort than putting something on a hook. I also have learned to not sweat the small stuff. Like taking time to turn my clothes all right side out all at once. It's easier for me to just do it one at a time as I wear things.

Here's a few modifications that have been incredibly helpful for me.

• Sit whenever I can instead of stand.
• Lean instead of free stand.
• Plan ahead.
• Think outside of the box (like my dresser is now just storage for things I don't use often like sunglasses and extra blankets).
• Work around your energy needs - if showers leave you exhausted, take them before bed instead of first thing in the morning.
• Use empty wall space as storage.
• Keep the things you use the most within easy reach and out in the open. (I love using a variety of pretty little bowls for things like hair clips, jewelry and my anti-vertigo bands.)
• Use bins instead of hanging things up.
• Use wall hooks for clothes or pajamas you wear the most.
• Using "travel size" instead of full size products.

In the kitchen I have a stool that's adjustable to sit on when cooking, but honestly my husband and mom feed me most of the time. It's not a job that I miss. When I do cook I keep it simple. That makes it healthier and saves my energy. We also meal plan weekly together, that way there's no stress around dinner time.

In the bathroom I love my shower stool and bars. I feel like everyone needs both in their shower even if they don't have any health issues. They didn't cost much and I use them every time. I also use bins, bags and hooks in the bathroom for my stuff. I've learned that "travel size" products are lighter and easier for me to use, so that's what I tend to buy. I've also cut WAY back on the number of products I use. "KISS" in the bathroom. 

Each spoon of my energy is precious and these minor tweaks add up fast for me. 

The A Team

It's all coming together now. The "A Team" is assembled and ready to fight my Metabolic Myopathy. 

I'm so happy I could burst!

Yesterday I had my much anticipated appointment with a place called PMR (Department of Physical Medicine and Rehabilitation). There I met a fantastic doctor who not only had heard of my condition, but has other patients with the same thing across a spectrum of disability. 

She heard me when I talked, had read my chart ahead of time and had fresh insight into my care. It was so wonderful I almost cried. She reviewed my tests and records with me and took a baseline of my lung functioning. Something no other doctor had done before. She ordered some new labs for me and gave me recommendations for things to try.

At this clinic I will be assessed and followed for future mobility tools that will work the best for my needs and body. I'll also be connected with the MDA (Muscular Dystrophy Association) for additional resources. During my appointment she shared that there are 600 discovered muscle diseases related to MD as of right now, but more are being discovered all the time. FINALLY! FINALLY someone agreeing with the research I had been doing for years on my own and offering me real help. 

So now I have a great neurologist, this new knowledgable neuromuscular doctor, my fabulous PCP (Primary Care Physician) and my patient advocate who helps coordinate my care. My husband and I call them all "The A Team" (because 1-It's funny and 2-My name starts with an A).

Knowing that I have medical professionals who understand my illness, are looking for ways to help me live my best life and who listen to me is such a relief. It took about two years to get to this point and a TON of work on my part so I'm savoring this feeling of being helped and supported like never before by my A Team.

Daily Rhythm

After all of the holidays, my mom's birthday, my birthday, my son's birthday, I think I have something of a daily rhythm forming and it's really helpful.

I start my day off with a cup of coffee and a hand full of pills, like most Americans, but especially those with any kind of chronic illness/disability. 

Then I spend about 30minutes on some kind of household chore while I have the spoons. Laundry, dishes that kind of thing. After that I move into computer work. These days it's compiling my medical files for SSDI appeal of denial. 

Now I have all my relevant medical records on my hard drive and on my Google drive. There my mom and a family friend who is a PCP can both easily access my files and give me feedback if they notice something missing. AND if anything ever happens to my computer it's all backed up and organized. 

I have some other things in the work as far as SSDI goes, but it requires me leaving the house and is more complicated. It also requires more spoons. This is something I can do bit by bit every morning.

After that I eat something. Usually a banana, but sometimes avocado toast. Or sometimes it's actually lunch time and I have some kind of fish. I shower after I get my grub on.

When I emerge all soft and shiny fresh I usually switch gears and move onto something creative or go on a small errand or adventure with Sweetie and my Mom. Yesterday was rare in that I had the spoons for both. 

My Mom came over and while she was on an important phone call I made my FAVORITE dessert and something I was craving, rice krispy treats. Yes, it is hard for me to make, but it's a challenge I can still do. 

After her call we took my 13 year old "puppy" Sweetie out to our local Arboritum for a very short walk (Mom) roll (me). It was the first time Mom got to drive Pablo. She's in love.

After that the rest of my day is devoted to napping. Then my husband is home from work and the Man Cub is home from college. We all have dinner, watch a show and it's bed time. And no, I have no trouble taking a 2-3 hour nap and going to bed for the night soon after. Not. At. All. 

Not every day follows this flow 100%. Sometimes my Mom comes over in the morning and we spend the whole day together. Other times I don't see her at all and I do my own thing the whole day and maybe roll Sweetie on my own around my neighborhood. But a typical day is tending to follow this pattern and I love it.

Today I have my second cement person coming out. I hope it goes better than the first who totally flaked on me. I'm starting to feel a little more settled in my daily rhythms and even though I'm still struggling a lot with pain, fatigue, dizziness and other issues it does bring me a new feeling of calm that I welcome with open arms.

Painsomnia

Painsomnia is when you're in too much pain to sleep. I've had a nasty head cold lately (and a beauty of a cold sore) and when I'm sick my body pain level usually skyrockets. The last few nights I've been in bed trying everything I can to get to sleep. Night time cold medicine, Ibuprofen PM... nothing seems to help.

When my pain gets this bad I usually just try and distract myself into a state of ultra sleepiness by messing around on my iPhone. Pinterest is my go-to. I found myself doing some last minute Christmas shopping last night. "Design Home" is my favorite app. I've played it for years. I find it relaxing and a fun way to unwind. 

If I'm getting busy brain or feeling worried looking at animal "pins" or humor is helpful. Baby animals cure everything. 

My sweet girl is also helpful. 

I can't force sleep. She will come when she's ready. Bottom line is I'm lucky to not have to get up and go to work on only a few hours sleep. It really screws me up. If I'm up late with painsomnia I feel super dizzy and foggy headed the next day. I will also likely have a migraine. 

I'm lucky not to have this happen every night, but it does seem to happen back to back nights when it does come up. Being sick with a bug is almost always a guarantee that it will happen. It also seriously affects my mood. Bah Humbug!

Good Doctors and Not So Good

I've learned a lot from having a chronic illness. Specifically about the American medical system and those who work for it. I think I had some serious rose tinted glasses when it came to doctors. Overall I'd had good health and anything wrong with me had been very straightforward. Even my years of endometriosis. But when it comes to having something that there isn't a clear test for or that requires years of multiple people guessing at what's going on with you, then that's where things get more dicy.

Doctor's don't like the word "guess" even though that's what they do with me. I have a rare condition that they "think" they know something about even though all they're really doing is guessing. It's a "best guess" but still just a guess none the less. 

I've learned that some people really suck at bedside manners. Some doctors think I'm doing a "self fulfilling prophecy" and "not trying hard enough to be well" (real quotes.) Others think they know exactly what's wrong with me and respond in frustration when I continue to be sick. Some are fantastic in person and then never respond to my messages between my three month appointment wait times. Some say they're going to give me referrals for services they think will help and then don't, or do it a month+ later (typically after I message them repeatedly). 

All of this takes a ton of time and energy. I told my husband the other day that when people ask me what I do for a living I should just say "I'm sick" because it's seriously a full time job.

Because I'm a visual person I'll show you how most of my appointments start out:

And then it can go one of two ways.

Poorly...

Or

Well...

I've only walked out of an appointment once. And let me say that it took me a very long time to even comprehend that just leaving is an option. They are there for me and if they're not helping, or worse... I can always just get up and go. No need to waste my time or theirs.

In general my encounters have been very positive. Most doctors genuinely care and are trying their best to help me. Some even admit that they don't know much about my condition and are just trying their best to help my symptoms. I seriously appreciate that.

Pain

I hate that question that the nurse always asks at every doctors appointment. "Are you having any pain today? Where? On a scale of 1-10 how bad is it?"

Pain is subjective, personal and really hard to measure. I'm always in pain, everywhere. I can't remember a time in my life when my back didn't hurt. My best friend growing up knew this and would rub my back when I asked her to (the sign of a TRUE friend.)

A real friend has your back... Literally

I take Cymbalta 60mg every night for joint and muscle pain. It helps tremendously. I also take Lyrica twice daily for neuropathic pain. That also has been a life saver. Occasionally I'll take Acetaminophen if the pain gets too bad. Ibuprophen is supposed to help with inflammation, but I haven't found it to be useful for my pain. I'm not interested in taking anything else for pain management medically due to the risky side effects and dependence.

Pain has always been a part of my life but I'm not ruled by it. I find new symptoms much more disturbing and disruptive. Like the chronic dizziness, lack of stamina, weakness, etc... 

I long for the day when we can all just be scanned and have a report come out saying exactly what's wrong with us. Then a machine will pop out one pill that you take a day that will fix everything. I mean they did it in Star Trek! Why isn't that a thing yet!?

Transitions

My new transportation cards

I don't feel like I'm "transitioning" into my life of being on disability and not working. Not at all. Instead I feel like I've been distracted from really coming to terms with anything. Part of that has been from being so busy with my mother moving to town from overseas and the other part has been caused by having such a complicated illness.

When you are healthy one day and paralyzed the next, or have a clear diagnosis or are undergoing serious treatment for a condition complete with walks and ribbons, you know what to expect. Your prognosis is clear. But even though I have something serious, it's rare enough that it's not clear at all.

I have accepted that I likely won't find a magical treatment that will put me "back to normal" (at least my normal). I've also accepted that I will probably continue to have degenerative, progressive muscle weakness. But what I'm not sure about is if that will always keep me from working?

Right now what's keeping me from working isn't my body at all, but the dizziness, fatigue and foggy headedness. Both my ENT and the neurologist think at least some of this is from migraine. The pink glasses helped and I ordered a pair of blue light blocking prescription glasses yesterday hoping that helps even more. 

My new specs - Yes, I'm a hipster

The Topamax DID NOT HELP! It made my dizziness so much worse that I had to just go to bed. It also made my headache much worse. Ugh. After three days of that BS I just stopped taking it and messaged my neurologist.

I think what will help me transition is:

• Some time to myself
• Applying for SSDI
• Talking to the new neurologist who knows more about myopathy
• Time