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.

Growing & Shrinking

Growth isn't linear. That's something I remind myself of constantly. It's actually a lot like grief. I will have times of great growth, times of dormancy and times of regression. It's not a beautiful upward swoosh like a Nike logo. It's more like that hair ball clogging your drain. And it can feel that way at times too.

I've lost 24lbs since early June of this year. As of today. As of right now. That number goes up and down at times, but it's on a trend that I like. Each of those pounds has felt like a Herculean effort on my part. Resisting a parade of temptations (and sometimes giving in), swimming as much as I can, weighing myself weekly, then every few days, then daily, then every few days, then weekly... It's a dance that I'm doing with myself. Sometimes light on my feet, other times stepping on my own toes.

I always read that it's harder to lose weight the older you get as your metabolism goes. Back when I was "healthy" it was hard for me to lose weight because of my sluggish raisin of a thyroid. Even with synthetic replacement. Add a mitochondrial disease on top that hugely affects my energy and muscle ability and I was in for a bigger challenge than I realized.

Hence the drain clog of a process. Up and down and all around, back upon itself. But it is change. And I feel a lot of pride. I've lost a small toddler so far! Only a full adult to go. 

I see myself being pleased with about 82 more pounds lost, but I'll see how it feels.

I have vacillated between being a happy, thick adult woman and wishing to be a thinner version of myself my entire adult life. There's an important distinction there because I've never wished to be anyone other than me. Not even when I had a brain tumor and started to need the help of a wheelchair. Social media doesn't give me FOMO or make me covet other women's looks. Rather it gives me an appreciation for the feminine diversity and makes me even happier in my skin than I was before. 

Any desire to be thinner has always come down to health for me. In the past I would notice things such as my lack of stamina going up stairs. Joint pain or muscle pain after doing a Zumba fitness class. How I felt I should and could be more flexible. That hit an all time high with my mitochondrial disease. 

Self sympathy played a big part in my weight staying higher than I like once I had compromised mobility and muscle use. I told myself that if I couldn't have an orgasm I could have a massive Taco Bell feast or a whole batch of rice crispy treats. Not that I had those things very often. My big problem was just eating too many calories on a regular basis. Not particularly anything unhealthy regularly.

And then there was COVID. Everyone I know gained weight during 2020. I was no exception. But when I passed 300lbs red alarm bells went off in my head. I had come to a crossroads. Was I going to be a life-long big woman who would eat what I wanted and not worry about the consequences? I have many friends who have chosen this path and they are very happy. They look stunning! But I knew it wasn't for me.

The more weight and pressure I put on my joints and muscles, the unhealthier I personally am because of my disease. My body has to work that much harder to maintain any mobility and I have a lot more pain. I couldn't do that to myself. I love myself more than the liberated feeling I get from being able to eat what I want.

I want to feel as good as I feel in the water, on land. This can only happen with weight loss. A serious amount of weight loss. I'm fine with going slow, not going in a linear direction, but I have a destination in sight. I want to be a might lighter version of myself, for myself. For my health and for all of my muscles. 

Brain Fog

There are many symptoms that go along with my mitochondrial myopathy, but one that I find to be rarely discussed is brain fog and short term memory issues. It's one of the many reasons I can't work anymore at the job I used to do.

"The highest concentration of mitochondria are in the nervous system, brain system, and the spinal cord. For this reason, the most commonly experienced symptoms are of a low functioning brain or nervous system. This might present as brain fog, memory issues, poor concentration, mood swings, and sleep challenges."

Or even more cheerful news...

"The symptoms of mitochondrial myopathies include muscle weakness or exercise intolerance, heart failure or rhythm disturbances, dementia, movement disorders, stroke-like episodes, deafness, blindness, droopy eyelids, limited mobility of the eyes, vomiting, and seizures." (from NIH)

My migraines can also cause some memory issues, so I unfortunately get a double whammy. My memory becomes much worse as my body symptoms increase. This means if I'm battling an illness or even just having an asthma flair because of the weather, my brain becomes very "foggy." This is the perfect word because I do feel like I'm behind a fog. Like I'm a few drinks in (which is one of the reasons I don't drink alcohol).

My auditory memory is especially affected. Recall of recent things (working memory) is very challenging for me when I'm feeling this way (like right now. Thanks crappy air quality!) My family are all aware of this challenge for me and are sweet and patient. My grandmother suffers from severe dementia, so this is a very scary symptom for me.

Like all of my symptoms there seems to be very little that I can do about it. I take my supplements and push through regardless. Since it is an invisible symptom though it can be hard for me and the people who love me to remember and address. I can get quizzical looks of disbelief when I can't remember something very simple, even from my family. It's understandable. I'm young(ish) and "shouldn't be having challenges like this." But it is a part of my muscle disease and I have to remember to be kind to myself when it happens. That's something I still find challenging. 

Thank you to this girl on Instagram who talked openly about her memory problems with her Mitochondrial Disease today. It's always helpful for me to find others who have similar challenges. 

Try ALL the Things!

It's a new month. August. The bridge between summer and fall. A perfect time to try something new. I've been told (and my own research confirms) that I am not eating properly for my muscle disease. What I should be doing is eating many small meals through the day that are high in protein and "good" fats and low in carbs. But what I like to do is eat two large meals (especially dinner! My favorite) and nothing in between. Breakfast to me is a banana or just coffee. Not so good. 

Soooo... Since I want to be my best healthy self that I can be. And I'm also very fortunate to have access to food. I want to at least try eating the way that my doctors recommend. With gratitude in my heart that I have the financial stability to be able to do so. Especially now during the pandemic.

Soooo... I did a lot of research and here's what I found:

1. I should try eating 6 smaller meals a day. 
2. I will prioritize foods naturally high in COQ6 since that's where my major genetic mutation is.
3. I will focus on foods that are high in protein and fats and low in carbs.
4. I am going to pay close attention to my symptoms and how I feel.
5. I am going to try this for the entire month of August.

Yes. I already started!

I read that foods naturally high in COQ10 are:

• Trout
• Herring
• Mackerel
• Sardines
• Spinach
• Cauliflower
• Broccoli
• Oranges
• Strawberries
• Sesame Seeds
• Pistachios
• Eggs
• Soy
• Peanuts

(I got this instead of Taco Bell the other night. One small choice at a time!)

Some basic "good fats" are:

• Avocado
• Salmon
• Peanuts
• Olive Oil
• Eggs
• Dark Chocolate (85%)
• Macadamia Nuts
• Tofu

Foods high in protein are:

• Anchovies
• Eggs
• Yogurt
• Tuna
• Salmon
• Pumpkin Seeds
• Cottage Cheese
• Peanuts
• Tofu
• Beans
• Halibut
• Peas
• Oats
• Hummus
• Broccoli
• Popcorn
• Avocado
• Asparagus
• Potatoes
• Shrimp
• Guava
• Sun Dried Tomatoes

Food helpful for inflammation are:

• Flax Seed
• Green Tea
• Turmeric
• Walnuts
• Pineapple
• Kale
• Garlic 
• Broccoli
• Lemon
• Berries
• Avocado
• Cantaloupe

These lists aren't all inclusive, but are helpful for me. I also notice a common theme. Like rich fish, eggs and tofu. Those will be a staple for me. 

My hope is that maybe I'll have more energy with eating this way and even a little less pain. That would be lovely. It could also make some weight loss easier too. Fingers crossed. I'll keep you posted!

Botox Injections for Migraines - Take 2!

I FINALLY got to have my second round of Botox injections. The first round went very well, even though it took almost a month for me to notice a difference. My neurologist said that I have to wait the full 90 days both because of insurance and because of research showing any sooner than that could be dangerous. Damn.

It went well, though I think I got more shots than last time. My husband lost count and I forgot to ask. I know she was doing a bit larger dose in some specific areas. I have the most problems with my left side (no surprise since that's where my plates are from my brain surgery.) 

It did feel a little more painful than last time, but it's also hard to remember from three months ago. I ended up coming down with a raging migraine and my head feeling like it had been stung all over by bees, but it's all worth it if I can get the same relief (or more) than last time. 

Funnily enough I can live with the migraines, it's the chronic vertigo that did me in. Also the "Alice in Wonderland Syndrome." Vestibular migraines are no joke.

With my Metabolic/Mitochondrial myopathy I have learned that anything can set my body off. I become very sore, very stiff and have an incredibly dry mouth during the night. When these things happen I know something is "off" with my body and I need to just rest. That's what happened yesterday after the shots. I came home and crashed out for almost six hours. Then woke up every hour last night in terrible pain and with serious dry mouth. 

Today is also a rest day.

No shame in listening to your body!

Mine speaks a whole language all her own, but it's one I'm slowly learning.

Curious to read more about Botox for migraines? Click here (no affiliation).

The Curse is Lifted!

I have my days back

I'm thrilled to share that I FINALLY found something that keeps me awake all day long without any side effects. It's THIS! Orgain Organic Nutritional Shake.

This is the first one I bought and tried.

I have no idea why it works for me. 

My guess is that my body just doesn't absorb nutrients correctly to get them to my mitochondria. So I would be totally depleted of energy by the afternoon. But when I consume a lot of them in liquid form my body can somehow use it more efficiently than by eating. 

I'm so grateful that I found this product and that it works. Now I'll be looking around to see if I can find other things that work just as well but for less. Each drink costs me between $1.90 and $2.50 depending on the flavor. I use it as a meal replacement, so it's not as bad as it sounds. But since I'm unemployed right now I want to try and save us as much as I can. I'm also curious if something like a vitamin water would work just as well or if it has to be with protein too.

At first it felt really strange to have a full day back. Like I flew to another country where their days are twice as long. Now after about two weeks of it I'm getting more adjusted. It's wonderful! I still have to be careful not to do too many things at once. 

If I spread my activity out with resting a lot in between I feel much better. If I push myself I'll end up with shaking muscles and sweaty. Then it will take about an hour for that to stop. Ugh.

I have a check in with my muscular neurologist this week where I'll let her know about it. I want to discuss my increasing pain and muscle spasms with her. 

I haven't used the CBD/THC pen at all since I discovered the drink worked even better with no side effects. One pen cartridge is the same price as 12 drinks, but it's much better for my lungs. I also never drive with the pen and I can drive a little in town normally. That's a big thing to have to give up to stay awake if I used the pen instead.

I can't tell you what a relief it is to have a big chunk of my life back. I'm so grateful that I stumbled on this product. Fingers crossed that it keeps working!

I have zero affiliation with any product I ever talk about on my blog and received no incentive to write about it. 

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. 

What's New?

This time last year I was responding well to my Mito Cocktail. I had a real increase in energy and stamina. I suspect what happened with the Mito Cocktail is that my body just adjusted to it and it stopped working as well. 

That happens to everyone with almost all medications, even recreational drugs. I'm no longer taking the Idebeone. I tried it for three weeks and it didn't work for me. I had an increase in cramping, spasms and muscle pain, plus it upset my stomach. So two weeks ago I went back to my COQ10 and I feel like I'm still adjusting.

I continue to be less fearful than a year ago and am still rocking a bikini. I also take care of my "tasks of daily living" independently. I even made a quick store trip with my son yesterday, just the two of us. That felt really good.

As far as medications go, here's what I'm currently taking:

Prescriptions Taking

LevoTHYROxine 125 mcg AM

(Pregabalin) Lyrica 150 mg BID

(Duloxetine) Cymbalta 60 mg PM

(Lioresal) Baclofen 20 mg TID

Albuterol Inhaler PRN (apx 6 puffs spread out daily)

PRN

Acetaminophen 500mg +2 PRN

(Rizatriptan) Maxalt-MLT 10mg PRN

Albuterol Nebulizer (DME) PRN (not used yet)

5:1 (CBD:THC) Tincture or Edible PRN

Supplements

COQ10 300mg 5x a day (1,500mg total)

L-Arginine 500mg AM

Vit. D3 2,000IU AM

Wild Alaskan Fish Oil 1,400mg BID AM/PM

Biotin 1,000mcg AM

Magnesium Citrate 250mg BID AM/PM
Potassium 99mg BID AM/PM

MigreLief supplement BID AM/PM

B-2 400mg

Magnesium (citrate and oxide) 360mg

Feverfew (whole leaf & extract) 100mg

I have a fantastic A Team behind me now including multiple people who know about Metabolic/Mitochondrial Myopathy. My neuro muscular specialist just suggested I try epicatechin to see if it helps my muscles at all. She said there's some good results with it helping protect the heart of people with MD. I will give it a try since it's an over the counter supplement.

She also reminded me about the muscular benefits of Epsom Salt Baths. It's not easy for me to get in and out of the tub, but it is possible. I will try and add these to my regular routine as well. We have a big bag of it from Costco.

The biggest news for this month is I got adjustments for my big chair "Dory". I got a new seat, new headreast and (*insert drum roll here*) KNEE ABDUCTORS! That's a huge deal. No more leg straps for me!

Also fantastic is that she's on a bit of a tilt back so I don't slump over myself when my trunk muscles fatigue. Stamina is not my friend.

"But what about Plan Kick Ass?" 

I've integrated a lot of it into my daily life. I try and eat more fish and veg then I do land animals. I move as much as I can and in general make my health a priority. Of course I still have things that aren't the best for me, but I try and notice how food makes me feel after I eat and stick to the things that make me feel good.

Here's some things I'd like to change, work on for the future:

• Stretch twice daily
• Take Epsom Salt baths
• Try the epichatechin
• Explore CBD more
• Go back to smoothies every morning

Lastly what's new with me is that I'm trying some high CBD, low THC products to help with pain and muscle cramps/spasms issues. I don't like taking Acetaminophen for pain all of the time and I don't think it honestly helps that much anyway. It's hard on my liver and kidneys and can cause stomach irritation if I take it too often. I feel there's less of a physical risk to my health taking a more natural product. So far I have tried oils, tinctures and gummies. It's legal in my state and very available. I'm open to trying almost anything that could improve my quality of life.

So that's where I'm at medically right now. Taking things day by day and focusing on the positive. I'm keeping connected with my friends from my Muscular Dystrophy support group and family. That's always helpful for keeping my mood up. And my mom has been stuffing me full of delicious foods. I try and get outside to get some sunshine when I can. Now I'm off to make a smoothie!

Wheelchair Van Shopping

Terrifying, daunting, uncomfortable, overwhelming... maybe those words begin to describe what it's like to shop for a wheelchair van. These are just a few reasons I suspect most wheelchair users choose to either not go out much, or to take public transportation. The other reason? COST! DAMN! I mean come on! The prices of these vans will take your breath away.

My husband and I began thinking about pulling the trigger on a wheelchair van this summer. We're fortunate enough to have actual options in the city we live in. Most people I think are stuck with a single dealer or buying private. I thought I educated myself and was prepared ahead of time from internet research. Nope. So let me break down my experience for you so far in the hopes that it might help you out in your search.

That's some serious "RBF" going on there!

Step 1: Find what kind of ramp you want.
Electric? Manual? In-Floor or out? There are many options here. I want an electric "Fold Out" which is not the same as "In Floor". The reason is because if the electric equipment has a malfunction (and we know how common that is) then you can still manually get your ramp up and down. You're not trapped in your chair till some magical help comes along.

Step 2: Will you ever be driving?
I've heard a lot of stories from people who thought they would drive, but then due to their progressive condition only drove for a year or two. It ended up not being worth the huge expense of making the drivers side accomidate their needs. Case in point. When we first started looking I was still driving. I was interested in a "Transfer Drivers Seat" meaning I could easily transfer from my chair to the drivers seat to drive. Well, I'm no longer driving and I don't need that expensive feature.

One of the many benefits of buying a van from a dealer vs private party is that as your needs change you can have your van modified. For example I don't need a transfer passenger seat yet, or a chair lock down in the front instead of a passenger seat. If that changes in the future I can have our van adjusted.

Step 3: What kind of van do you want?
This is called the "Chassi" in the wheelchair van world. Common makers are the usual. Honda, Toyota, Chrysler, Dodge. Each comes with their own pluses and minuses. I am very tall with long legs and an "ample bottom" so having a comfortable, roomy passenger seat is really important to me. Also due to my chronic pain, seat heaters are really a big plus. Because of these and a few other important features I'm hoping to buy a Chrysler Pacifica Touring-L Plus.

Step 4: What's your budget?
Wheelchair vans range wildly in price just like regular cars. Also like a regular car you get what you pay for (in my opinion.) The older the car, the more miles on it, the less expensive it will be. You will need to consider--The Chassi Price, Conversion Price, Equipment Cost (tie downs, modifications to your chair to be locked in, transfer seats, driving mechanisms...) and Warranty. Then of course there's taxes, documentation fee, license fee, smog, registration and every other nickel dime fee there is. 

Some Chassi brands will give you a small rebate. Usually around $1K and only if you're buying brand new. A super small drop in the bucket. In my state there are no programs to help you with the cost. It's considered a "non medical necessity". Though my dealer mentioned we might be able to deduct the cost of the conversion and equipment as a DME (durable medical equipment). We'll check with our accountant about that.

And some cars have lease options instead of buying. But I have found them to be too rich for my blood. However if money is no object and you just want to have the best you can buy it should be an option to consider.

I was happy to hear that there are programs where veterans can get a van almost completely covered (they should be able to get whatever they want for free if you ask me). And my dealer said "Victims of violent crime get a 30K payout to put toward the cost of a van". 

The van we're considering is a 2017 (two years old) with super low miles. "Out the door" it will cost $61,094.87 to purchase. Luckily we have excellent credit, but I was told that financing a van can be difficult as it isn't treated like a typical car purchase. You can also get financing for up to 10 years to make the monthly payments a little bit easier. For us those payments are still 4x our current monthly car payment so that's a huge, scary undertaking.

The next steps are to talk to our banker about it, sell my husbands car and pray that my student loan debt is forgiven due to permanent disability. I'm checking the status on that daily. Hopefully we can pull the trigger soon because it would really improve my quality of life to have "Dory" with me whenever I need her. 

Help is HERE!

I had three medical appointments last week, each good in their own way. The great news is that my meningioma (the benign brain tumor from last year) is all clear, no new growth and my brain bounced right back in to fill the cavity. YAYYY! At least my brain is athletic! So that's fantastic news. I also don't need another brain MRI for another year. 

Getting medical help is very exciting

My neurosurgeon recommended I do 23andMe genetic testing. He said they can be pretty thorough and may find something that we don't know about yet. So that's in the works right now.

The next bit of good news is a have a neurologist that I love. He was super compassionate, a great listener and had a lot of fantastic ideas on how to help me NOW. I told him most of my doctors get so caught up in wanting to find out what the underlying cause of my issues are that they overlook how to help me suffer less today. He was awesome and did both. 

He reviewed my chart, asked questions and went over my timeline with me. He asked about my symptoms and was upfront with what he knew and didn't know. He's an MS specialist and he wants me to see a Myopathy specialist in the same office next week just to talk genetics, but he's all about helping me now. Also many of my symptoms are similar to MS, even if the cause is different. 

The biggest takeaway for me was that he said I have Mitochondrial Myopathy (MM). He said "The two hallmark symptoms of MM are muscle weakness with any kind of exertion" (check) and "Muscle energy crash with any kind of illness" (double check). No one had ever put it to me that way before. I asked him to officially change my diagnosis in my chart and I hope he does so soon. It's helpful for me and to keep everyone on the same page. 

BUT he also had some other ideas and suggestions. First off he thinks my dizziness, foggy brain and vision problems may or may not be connected to the myopathy. He thinks it's just as likely that I'm suffering from "Intractable Migraine". He said with this type of migraine it's more likely that you'll just have my symptoms instead of the classic traditional migraine. He said it's also incredibly common to have this type of migraine after my kind of brain surgery and/or with my myopathy... but bottom line he considers it a different beast from MM. Interesting. 

Bruce wants to help too

Help take a nap that is

Before diving into a new medication for the possible migraine he recommended I try a combination of Vitamin B-2, Magnesium (800mg a day) and Feverfew. It comes in one pill (thank God!) in something called "Migrelief". So I'm giving that a go for a month till we move try a possible prescription for it. 

He also gave me a lot of referrals for services that might help my CURRENT symptoms. YAYYYY!!! I can't even tell you how amazing that was to hear. 

So here were my main takeaways and diagnosis complete with links:

• Intractable Migraine. Could be causing the dizziness and foggy headedness.
• Dysphasia. Fancy name for choking on your own spit like a big dork for no reason.
• Physical Medicine and Rehabilitation. A magical place that will help with my tools and other mobility needs.
• Mitochondrial Myopathy What the neurologist thinks is my overall diagnosis and what's going on with my muscles (due to a genetic mutation.) 
• Speech Therapy For my issues I'm having lately with talking.
• Temporal Muscle. Issues with scarring and muscle tensing from brain surgery last year.
• Therapies for Mitochondrial Disease

(I received no incentive, kickback, discount or payment for ANY of my links.)