Unlocking the World

On August 17th, 2018 I had my first ever assessment for a wheelchair. That chair would turn out to be my Quantum Edge in Ocean Blue (of course). I love her very much.

How can you love a wheelchair?

Much the same way people love their cars.

For me she's so much more than a chair that moves. She's everything about freedom that's so important. She literally unlocks the world for me. Without her my life would be smaller than small. 

She also means independence.

I'd be relying on others to push me everywhere if I didn't have her. Not only is that tedious for them, but it feels awful for me (literally, I get motion sick this way.) As my mother once put it when she had to push me before we purchased our wheelchair van. "Awwww, it feels the same as when you were little and I would push you in your stroller." For me too mom! It feels the same for me too. Like I'm an infant. Ick!

My biggest fear is that she will break and I'll have to wait months and months (if not a full year) to get a new one. Repairs and delivery of medical devices is shocking. A few companies have a monopoly so they can take their sweet time, lose your order or screw it up as much as they like. You're at their mercy. It's such a racket.

I have many fantasy businesses, but one of them is running my own wheelchair supply and repair business. 

Anyway... I digress. 

If something happens to my chair (and it already has in the past) I'm very limited. I do have my travel chair as a backup, but she's not nearly as comfortable or maneuverable. She's good for an emergency, or rugged travel. Not really for every day use.

I'm also curious if I could get a new wheelchair that raises and lowers up and down. Not just tilts front and back. And, as much as I love this chair, I didn't get the full assessment of an occupational therapist (OT) when I was fitted for her. Just a sales man for the company who ordered her for me.

I contacted my insurance company to see when I could qualify for a new chair and was told "Anytime you need one." WOW! That's great news. So then I reached out to my muscular neurologist and asked how it would work. She said she would need to see me, but that we could do a video visit. Then she would send me to my fabulous muscular disease OT for an assessment. Bingo!

This all means I'm hoping to get an order in for a new chair soon. That will give me some new options and a solid backup should anything happen. And IF I ever travel again on a plane, I can bring my big chair and not be in a panic if something should happen to it during the flight.

My world just keeps getting bigger.

Turn for the Worse

On April 20th, 2022 I had my Botox for Migraine as usual. I get it every three months. It's a big deal when I'm eligible to have it done again as I'm usually a good month past needing it. This time I felt I was around six weeks past needing it.

Each time I get it the results are different. But typically every other time it is a solid 6-8 weeks of no migraine headaches and greatly reduced other symptoms (like aura's, vertigo and other vision issues.) The last two times were a bit of a dud, so I had high expectations for this round.

This must have been what Norm felt like rolling into Cheers every day.

This Botox session was different from the start. It hurt a lot more and I bled a lot, which had never happened before.

So that was April 20th.

Two days later on April 22nd a small blood vessel burst in my left eye. No big deal. I call the left my "bad side" because that's where I had brain surgery three short years ago. Although I haven't had a burst blood vessel in my eye in five years.

Three days later I could tell I was getting some kind of mouth and tongue infection. It felt like thrush maybe? But I had blistering too. Shit was starting to get a little strange.

Blisters under my tongue.

Webbing and redness inside my cheeks.

"Geographic tongue" (the spotting) and coating.

I tried treating it myself with salt water gargles, but by May 3rd it was bad enough for me to just go to my local Urgent Care. The doctor there wasn't so sure it was thrush and thought it might be an autoimmune reaction. She prescribed me two disgusting mouth washes and I went along with my life.

When I went in I had tongue pain, a sore throat and pain deep in my left ear.

Just four days later on May 7th, a large blood vessel AGAIN in my left eye burst. WTF! Now I was getting a little freaked out. I messaged my doctor and he confirmed it was just a burst blood vessel. Nothing to do but wait it out. It wasn't painful. Just a little itchy. And I felt like I looked like a zombie for Mother's Day. How fun!

My husband took this graphic picture for me.

It looks like it burst up, then the blood trickled down.

Nasty!

You'd think that would be the end of it.

But wait! There's more!

Now the excruciating migraine headaches started.

The first one lasted 24 hours. From May 9th-10th. I took my usual trio of medications to try and help. Compazine (for pain), Naratriptan (for nausea) and 1,000mg of Ibuprofen (just over the counter stuff.) I call it my "trio of helpers." I can't take them more than three times a week though. 

The second migraine was thwarted by the "trio of helpers" and only lasted five hours. That was the very next day on May 11th. On May 12th I was hit again, but couldn't take my meds so close together, so I tried to ride it out. Nope. Now I was getting scared. On the 1-10 pain scale I was at about an 8 and seriously considering going to the hospital. 

During the middle of the night on May 12th I awoke in the middle of the night to the familiar tingling feeling of a fever blister forming on my top lip. I hustled to the bathroom to dig out my herbal treatment and "doctored" it. Shaking my head I went back to bed. 

Hello fever blister.

Welcome to the party!

During the night on both the 12th and 13th it felt like something was draining down my throat. I started to wonder if I had formed some kind of abscess from one of my shots and it was draining now? The pain in my left inner ear was worse, but that could be from some kind of inflammation or pinched nerve. I was also now 100% sure that my poor immune system was being severely compromised. 

My nurse practitioner who did the Botox shots has ordered a shot of Compazine and Toradol for the head pain. I wanted to go and get it yesterday before the weekend hit, but my insurance didn't approve it yet. Seriously!?! She also ordered me a nasal spray to try through our local compound pharmacy. But it's a special order and will take some time to create for me. As much as I appreciate her solutions, it's not helping me right now. 

Today is May 14th and I'm typing this with a fever blister, migraine headache (though it is slowly improving) left ear pain, mouth discomfort and a sore throat. I'm constantly torn between trying to "ride it out" and going to the hospital. I also can't stand not knowing what's going on with my body. I like answers and solutions. 

UPDATE (June 1st):

So I believe I had a severe sinus infection that I just couldn't feel because of the Botox. What a weird thing to have happen. I suspect it was caused by my BiPap machine, so I have completely stopped using it.

After three days of "drainage" (nice word for puss) down my throat all night long I felt much better and the pressure was off my ear. I think the pressure from the infection caused those blood vessels to burst in my eye. Then my body fighting the infection caused all the other things (tongue issues, fever blister, etc...)

I had my doctor test me for sepsis just to be on the safe side. Everything was negative. I still have a strange rash that he'll check out for me Friday. I was pretty sick for almost the entire month of May. I may never know what really caused it, but I'm being much more cautious now. I'm also very impressed that my body fought it off all by itself.

Life Goes On

 Sometimes life can click along with nothing changing for years. Then suddenly I can have months, weeks, days where things change so fast it's hard to feel present.

I recently lost my precious Sweetie girl. She was my canine companion, my fur baby for 16 years. I feel lucky to have had her so long. I was also aware that super old age really sucks... even for animals. I would say she was "not her best self" for the last two years of her life. Joint pain and dementia had set in. We did what we could to keep her happy and comfortable, but in the end she didn't even want to be touched much.

From the earth we are given and to her we one day return.

In the end she went quickly, thanks to veterinary medicine and our fast actions. I am grateful for her precious soul to have been a part of my life.

Now my grandparents are also facing a difficult end. Death that comes swiftly in the night is a rare blessing. Usually fog and pain are there years and years before death finally comes for us. I for one will welcome Death like an old friend and relish the sweet release when it's my turn. Pain is for fighting. Not death. Death is there to set us free. I never understand people who want to fight death. Even as a child that made no sense to me. 

They are very far away from me, both in distance and in life but that doesn't stop my love for them.

And then there was joy!
I found a new little boy needing a home and love. This is our brand new Miniature Pinscher named Max.

Animals give us so much love and teach us so much about life. Like all of nature, we just have to pay attention to learn.

What has also been going well is the help I've been getting for my health. My migraine neurologist is still out on maternity leave, but I'm receiving my Botox every 3 months. I also just had my bi-annual check in with my muscular neurologist. That went well. I'm having a lot of pain and weakness in my neck so I'm getting fitted with a special neck brace to wear sometimes. They think that will help. I'm also getting new wheelchair tires and batteries soon.

I had my eyes checked and the optician is recommending I try "Prism" lenses. Apparently my eyes aren't tracking together and the prism helps that. It may also help my migraines.

Life is going quickly.

The holidays will be here soon. Thanksgiving, then Winter Solstice and Christmas. I feel like if I even blink I will miss them. I'm just trying to be mindful and enjoy each moment as they come. Being present in the present.

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).

Vestibular Migraine Update

April 14th, 10 weeks ago now I had my first round of Botox to see if it would help my Vestibular Migraines. Here's what happened.

• Weeks 2-4 my headaches slowly faded in both duration and intensity.
• Week 6 the dizziness went away overnight. I felt the best I'd felt in a very long time.
• Week 9 the headaches came back. First just one normal feeling one, then a worse one that lasted 48hrs. Now they come on daily and I'm taking a Rizatriptan Benzoate ODT 10 mg tablet as soon as I feel it come on. That's been helpful.
• Week 10 the dizziness is back.

My next injection round is scheduled for July 14th. I asked if I REALLY have to wait that long or can I get it sooner. The answer as with all things in the US is my insurance will only pay for it every 90 days. F*#&!!!

Now I have a little less than three weeks to go and I'm feeling shitty. Although it is great to know just how effective the treatment is! I was also told that the more I have it the more effective it will be and the longer it should last.

My only side effect has been a strange muscle cramp I get sometimes in my forehead. I think that's just part of my myopathy because I'm constantly getting muscle cramps and spasms. But it looks and feels very strange. Fortunately it only lasts about a minute.

Don't lie. I look like a Klingon!
Normal is top. Muscle cramp is bottom. Really funky sensation.

I'm so grateful to have a team of doctors figure out what the heck is wrong with me. I never would have guessed all this was migraines. Now I'm counting the days till I can get 32 shots in my head again. 

I'm the zebra

I remember early on at a medical appointment, the forgettable doctor told me the following. "We have a saying in the medical industry that if you hear the sound of hoofs look for horses, not zebras". She explained that what I was experiencing was common and not likely to be something rare. She was dead wrong.

I later looked up her strange "zebra" analogy to find it is taught in medical school in regards to "a very unlikely diagnostic possibility". I'm not a doctor, but do have a masters degree in my field of practice, mental health. What I was trained was to "consider all possibilities, listen to your client and rule nothing out". So why do we treat the mind any different than the body? Shouldn't doctors be looking for horses AND zebras? And are zebras really so rare after all? I mean... if you're on an African plain wouldn't you be shocked to see a horse instead of a zebra? 

To put that in medical terms. Maybe doctors aren't finding zebras because they're too busy looking for horses. Seriously. The example of the "unlikely diagnosis" is "When someone develops a cough, a virus or infection is a logical cause and tuberculosis is the zebra". As someone who has been treated for TB I find that to be a bad example. How about testing for all of it?

I mean how can a middle class Caucasian person living in a "safe" small city with good food and clean air not only contract tuberculosis, but also have Rheumatoid Arthritis, survive a brain tumor AND have a very rare muscle disease? (Yes, I'm talking about myself here.) I don't think I'm the only zebra at this watering hole. 

Doctors would be wise to rule nothing out and look for horses AND zebras. To take their patients concerns (and not "complaints" as another doctor of mine called them) seriously. And to admit when a horse really is a zebra. Come to think of it... I'm not a zebra after all. I'm a freaking zebra, unicorn, pegasus that poops rainbows! 

Botox Trial Begins

Yesterday was my first round of 32 shots to see if it will help my migraine symptoms. Specifically I'm hoping to get relief from my constant dizziness, fatigue and headaches. The shots were all around the crown of my head and two in each shoulder. They stung a bit, but it wasn't awful (though I also have many tattoos.)

I was very concerned about the potential side effects listed on the Botox site, but my neurologist is a migraine specialist and does these all the time. She assured me that they have to list every side effect for every use, not just for migraines. The side effects sound scary... but I'm not having the shots in my chest so I should be fine. So far (24hrs later) I've had a headache, minor soreness, tenderness and stinging. 

No washing your hair or rubbing your head for 24hrs, then when you do wash, do it gently. I made the mistake of towel drying my hair after. Ouch! Don't do that next time. Lesson learned.

My doctor said it could work as fast as one week, or take as long as six months. I will go back in three months to do the whole thing all over again. 

I told my husband on the way home yesterday that I really admire my optimistic nature. Even after everything I've been through and only about 15% of things I try helping, I remain hopeful. Hopeful enough to get 32 pokes in the head, cross my fingers and wish it will help.

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!

Starting Idebenone

My mid-day exhaustion is a serious problem. Not being able to do anything "all day" has more of an impact than you would think it would. I miss out on a lot of life by sleeping from 3PM to 6PM every day.

I talked to my new muscular neurologist about this and asked if there was some kind of "ATP Booster" that could potentially help lift my afternoon curse. (If you don't know what an ATP is, check out this short, link to Mitochondrial Disease. It's really fantastic). She suggested I try a supplement called Idebenone. Actually, she was the second person to tell me about it. The first was a brilliant man in my MD support group.

Today's my first day on it, replacing the COQ10 that I take every day... all day every day. I'll take 500mg once a day for two weeks, then two of those a day for a month to see how it goes. My hope is that I'm able to sustain my energy and make it through an entire day without a nap.
"...idebenone is thought to increase the energy production inside cells as well as protect the mitochondria (and the cell) from damage."
Now you might think "well, what's wrong with taking a nap every day? Who wouldn't love that?" I'm guessing that's what you might be thinking because once upon a time I would have thought that too. I love naps actually. But that's not what happens to me. 

What happens is my body slowly shuts down and I "crash" more than nap. My muscles get weaker, it takes more effort to talk, my short term (working) memory gets worse and it becomes difficult to focus my eyes. Once my head hits the pillow I'm out like someone chloroformed me in an Agatha Christie book. Out-out. See the difference? Not fun at all and totally mandatory for my body.

This has been happening since late October (about four months) and I'm frankly concerned about it getting worse. A one hour nap increased to two then to two and a half and now three hours is pretty common. What if one day I just can't leave the bed? These are the thoughts that scare me.

On the plus side Idebenone has had some positive results in research, so I'm hopeful. I typically respond well to new things at first, but then adjust over time and they stop working as well. But I'm strong, brave and hopeful.

February - Where I'm at

I'm not kidding when I say I'm a full time patient. In one day I had three medical appointments. In one of them I learned the joys of what barium in all its forms tastes like for a Barium Swallow Test. 

(And why is the woman grinning madly? Why it's because she's trying not to vomit while holding massive amounts of barium in her mouth of course!)

Don't throw up or you'll have to do it all over again!

I also got to have this lidocaine nozzle shoved up my nose before a camera tube followed. Who knew they look at your throat through your nose? Now I sure do! (Kidding, he didn't "shove". He was very gentle and I'm being dramatic because I can.)

This was the petite nozzle that went up my nose. 

Only about 4 inches.

TAH DAH! My voice box and surrounding muscles. 

See the resemblance?

The tests all went well and they found some interesting things. Myopathy that affects my voice and muscles in the back of my throat that tire just like the ones in the rest of my body. They want me to see a speech therapist to get trained in some lung exercises to help protect me from pneumonia. I asked if I could just blow bubbles and pinwheels instead and the dude didn't even crack a smile. Doctors are just too serious sometimes!

So here's medically where I'm at:

• Referral for EMST (fancy for lung stuff) through a speech therapist
• Occupational therapy for my tweaked shoulder
• Occupational therapy to maintain muscle strength (even though strength isn't my issue, it's stamina)
• March 11th is my neurologist consultation for Botox for migraines (not the actual procedure. Hold your horses Ms. rushy rush! You have to be consulted first!)
• March 19th is when my wheelchair will finally get picked up for the modifications I've been trying to get since October (a higher headrest and knee abductors)
• March 22nd I check back in with my primary care doctor about everything
• Working on getting a nebulizer for home after needing urgent care and the hospital last year for breathing issues (mostly after travel)
• My teeth are clean, but I need a few procedures, so that continues in April

But what about FUN!? What's going on that's NOT related to medicine or illness?

• I'm enjoying the new show Lego Master with my honey
• My mom and I have a blast at our weekly Trader Joe's run
• I try and take Sweetie out every day, even if it's just around the block
• Daily I sit on my shower stool and pretend to be a mermaid while in the shower
• My husband and I are going to a Capitola beach house with friends in early April for the weekend
• My mom is taking myself and my son to Monterey during his spring break (one of our most favorite places in the world!)
• I just planted a little herb garden by my door
• The nature here is already springtime beautiful! I try and get out in it as often as possible
• I painted my toes rainbow and they make me incredibly happy

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.