Energy for Life Walkathon

It's amazing how an item on a list, an idea mentioned in passing, a challenge between friends, ended up bringing so much joy and  satisfaction floor my family and I. A minutes worth of conversation bore much encouragement. You see, I'm a coach in the non profit organization called I Run for Michael.  My buddy, a NP named Betsy, runs in my name. She does all types of interesting distance races in various states and laces like Epcot and posts photos and adds up each mile. When she saw one of my bucket list items was to walk (well, roll) for a cause important to me, she told me to find a local one and she'd come up for it. I learned there was going to be an UMDF Energy for Life walkathon a few months later, a few weeks ago now in April.  We signed up and began fundraising with a modest goal of a few hundred dollars, and hoping to get a few people to walk with us.

Immediately, people began to donate and sign up to walking with us.  My good friend and fellow POTSy took the helm and began organizing and ordered the t-shirts. Another booked a flight from Canada and had rubber bracelets made to sell to make more money for the UMDF.  I raised our donation goal as well as my private goal multiple times as it was smashed over and over. The final total was over $4,600!!  And we've only begin to sell the bracelets. (Email me at  tradingsorrows@gmail.com for info if interested). Altogether $60,000 was raised collectively. These walls are held all across the country to raise money for research and to support mito families in need. I'm so proud of everyone, and praising God for the blessing it was to be apart of it.

I am so honored by generosity and touched as 23 people including my family made the walk.  we had more but last minute issues kept them out of it, a big disappointment.  As dad began pushing me down and around the curved the path, I looked back up the hill to see all those people in our bright blue shirts carrying balloons and small posters of friends and other children lost to mitochondrial disease.  I stated crying harder, so very touched and also feeling sad mixed in with the joy of the moment as I thought of those kids, my friends that I missed so much.  I also cried for my friend Danna. Brave, kind Danna who has been constantly checking on me despite her short prognosis due to severe and imprenatrable infection. It seemed so unfair. But knowing we were doing something was empowering and learned the sing ever so slightly. To know that all those people had stepped into my world and took the time to fight mito with me, some without even knowing me personally, was overwhelmingly beautiful. Danna is at peace. She's living her life achieving all the things she possible could if her bucket list, spending countless hours with family, and reaching out constantly to her friends. She wants you to remember, don't take life for granted.

You see, I always feel like I'm jamming myself into the regular world. Like a key trying to turn the wrong lock. I never feel I belong, always out of place. I'm never strong or fast enough or saying the right things or doing the same steps. Not that I particularly feel unaccepted, it's just that my life is so different.  But that day so many of my friends walked with me in our broken world and it felt so, so good.

We did the balloon release with biodegradable balloons and I had the pleasure of meeting Corynna's parents. I didn't know this brave young lady personally but several of my friends were her friends. She used the same bipap equipment as me, and her mom said it hit her seeing me, I rejoined her off corynna so much. It was a wonderful thing to meet them and im so glad we got to do the release at the walk they attended. God knows all things, he orchestrates each detail! He knows what His children need. The idea came originally from one of the mom's of the kids we honored. She saw his name printed with thee dates of his life. She said it hurt seeing that, like it diminished and reduced him and his story. All that he was and went through just in black in whit e keys and numbers?  That really got me and it sparked the idea in my heart, a God given one.  Wyatt was her sons name. He also lived on bipap, but what defined him wasnt that. No, Wy was a superhero - SuperWy. He loved his family and vacations and wanted to be a roller coaster designer when he grew up . I wanted to share the stories and smiles of as many who loser their earthly lives to mito as possible.  Last year I list my good friend Melissa after she went into cardiac arrest from sepsis post surgery. She had a type if mito called MNGIE. Our symptoms were nearly identical and I was able to tell her about Jesus. She was searching for peace, I can only hope she found Him. His peace.  My mom carried Gavin's photo, a brave little guy who never got to grow up, who is free now from a body stacked with constant infections and more tubes than little boy. He had the best smile.

Maybe one day with the money you helped raise, I will be taking you about mito kids who all grew up and achieved their dreams.

I am finishing this very tired so please excuse any errors, it's been a long time coming and I just rally want to finally o
Post it!

Again, thanks to everyone.

Love
Amelia

Q&A

Greetings friends.
I finally am doing the Q&A post!  It's been in the works for weeks.  As always please excuse any typos or errors. As usual this is a product of insomnia.

Are you on bipap constantly, not just at night?
-anon

Yes, I am on bipap all of the time. I can always tolerate being off for seconds to minutes depending on the day. Sometimes, removing the mask for moments causes me to immediately start gasping and struggling for a breath. This happens almost solely during a flare, its usually not to hard for a few minutes if I'm quiet and still.  My daytime breathing is always better than night and I can usually handle being off few minutes to half an hour on average so long as I'm still and not talking, although certainly very uncomfortable.  Often my breathing becomes shallow with long pauses between breaths when I try to do things with no bipap. I get dizzy and disoriented.  My record off bipap is one hour, and that happened as I tried to push my limits.  When I'm lying down or especially sleeping I will sometimes wake up because I had an apnea. They are much less frequent now from what I can tell. I don't have that alarm set anymore to let me sleep better. 

(1) how old are you and (2) whether you've been symptomatic of mito your whole life or from what age did your issues arise? 

(OH and one more - is the wheelchair for you due to fatigue? how long have you had one?
-Momttorney

I'm in my early 20s. Symptoms from birth are esotropia, weak eye muscle that drifts out/crosses. As long as I can remember I've had POTS symptoms, mottling, subluxing joints, hypermobility. My sister has the latter as well and had severe pain growing up.  I had chronically twisted ankles and could replace any joint half asleep, the time it happened the most. Things progressed as I aged till I was constantly sick and in pain 11-13 years ago and totally disabled at 18, almost 5 years ago. I finally became  TPN dependent 2 years ago after battling it for a year.  Eighteen, about 5 years ago, is also when I first needed a wheelchair.  I'd been struggling awhile but a sudden progression made it impossible for me to ambulate myself.  As I'd collapse trying to walk from pain and absolute exhaustion and weakness. I've needed one for various reasons including ataxia and hemiplegia/hemiparesis.  Now it's mostly because I get tired vs just muscle weakness as my respiratory status makes everything a struggle.

what do you do with your j tube now that you are on TPN?
-Sarah

Going to give the long answer so it makes more sense. I received my jtube 2.5 years ago. I was on tpn a few times once just a short Time inpatient another several months before becoming dependent from what we thought was an overcome-able flare. I had been on NJs for 7months and my one nostril is swollen shut if I get the least but stuffy from the damage. Every time I swallowed u cried it hurt so bad, & an ulcer was developing. Dr. Gisela pulled it right away and started TPN, and tried to get me on oral food with a motility med before going ahead with a surgical option.  I was happy to try and when it failed we decided on the J in October.  I never did tolerate feeds well either. She found my small bowel makes no MMCs on it's own. We added octreotide a few months later in January. Tried gastric pacing. In may I was on tpn again due to weight loss etc. My J now is so so helpful. With my two motility meds I can sort of absorb meds for my bladder, POTS, complex migraines, and one of my pain meds. These meds are either not available IV or not safe for me to do IV.

I also frequently get asked what are the conditions I have. 

I have suspected Mito or some multi system genetic disorder.  This causes chronic respiratory failure, severe GP and intestinal dysmotility,   HMS (and all the issues that comes with it like esotropia, scoliosis/lordosis, subluxations, and more).  A main issue I have is dysautonomia (temperature instabilities) and hypovalemic postural orthostatic tachycardia syndrome.   A channelopathy or stroke like episodes has caused a lot of hemiplegia in the past, for long stretches plus short episodes. This supposed channelopathy responded to meds along with meds for complex migraines so it's hard to know as meds overlap.  CMs are an ongoing problem (confusion, ataxia, and aphasia at their worse. Thankfully fairly controlled to just the first symptom being present & short episodes).  I also deal with Neurogenic bladder, anemia and chronic hypokalemia.

Thanks for reading and being apart of my quest to educate!
God bless,
Amelia