Muscle biopsy update and other news..

Hello everyone!

The mito coordinator down in San Diego was finally able to speak with my mito dr., and he told her he's almost positive there's more muscle.  So she's going to look once more, but in the meantime, my appointment that was scheduled with him in November has been postponed...we should hear more soon when that new date will be.  But whatever the outcome, whether there's more muscle or not, I trust the Lord completely. :)  If I do have to have another muscle biopsy surgery, I'm going to ask my pain doc if I can have my ears pierced while under anesthesia.  No joke. ;)  Because I have Complex Regional Pain Syndrome and any type of trauma can cause a nervous system flare-up, getting ears pierced under general anesthesia would be ideal! ;)  So maybe this muscle-biopsy-put-in-the-wrong-solution is a blessing in disguise!  Having a jewelry etsy shop and making earrings all the time makes me want to have pierced ears. :)

Last Wednesday, my GI surgeon had to get me in because I've been having raw tissue growth near my j-tube.  She had to use silver nitrate to chemically burn off the granulation tissue!  Hopefully that did the trick, but she said it may need additional treatments.

I mentioned in previous posts how my neurogenic bladder has been getting worse (my nervous system is not sending signals to my bladder and this could eventually cause kidney death).  Well, this morning, I saw the neuro-urologist.  She's excellent and so nice which I'm very thankful for. :)  And she spent more than an hour with us!  Next month, I have to undergo a urodynamics test to check muscle strength, pressures, etc., and in her own words, she said it's very unpleasant.  I have to be awake, too.  It involves catheters and having more things stuck inside of me.. :P  She also wants me to start a medication, and all this is very important to do to prevent kidney damage.  I have to get permission from cardiology first since the medication lowers blood pressure (I already have low blood pressure).

So she's very glad I see cardiology tomorrow!  And I am, too, because my high heart rates have been getting worse.. :/

I will keep all of you posted!  Thank you for checking in and praying for me! :)

More Setbacks.

Last Monday, we received some unfortunate news. :(  I heard back from the mito coordinator down in San Diego, and she explained that the mtDNA sequencing (one of the numerous tests that was supposed to be done on my muscle) at the lab in Georgia was not completed.  We're not sure if it was a new lab person who made the mistake or something else, but somehow, the muscle was sent to the lab in the wrong solution, making it completely untestable. :(  What's worse is that there is no extra muscle left (even tho' several pieces were biospied....some was sent to New York, etc.).  We're now waiting to hear from the dr. about all this and what needs to be done.  Worst case scenario is I'll have to get yet another muscle biopsy surgery..  As you can imagine, this is discouraging news for all of us to hear, but I'm trusting in the Lord's sovereignty!  He is faithful.  This is just yet another test of patience. :)  Hopefully we hear something by next week, and I will keep you posted!

This past Monday, I got my feeding tube changed to a low-profile "button"!  It was a very unpleasant and painful procedure....basically, they pull the long tube all the way out of the stoma (hole) in my intestines and then "stuff" the new tube back into the hole. ;)  But it was definitely worth the pain because this new button is so easy to manage and handle!  That, the new tube feeds formula, and my feeding pump switch to a lighter one is all so nice!  Here are some pictures. :)

This is a j-tube pad around my mic-key button.  Many people sew these---they're super cute and comfy!  And it keeps the granulation tissue/leakage at bay!

hooked up to tube feeds :)

And this is my new enteralite infinity pump!

Yesterday, I had a lidocaine and magnesium infusion for the CRPS pain.  We weren't expecting any surprises because I've had both before, but you know me... ;)  I have to keep things exciting!  Long story short, things went terribly and involved very high heart rates, slurred speech (that was scary), head-throbbing, and a feeling of passing out twice.  The pain team had to quickly give me a bag of IV fluids and stop all the medication drips.  So I didn't really receive a good infusion... :/

If my insurance approves it, my pain dr. will be starting me on another pain medication.

In addition to all this, I've been dealing with a huge and painful mouth ulcer.  I haven't been able to eat much at all!  It's hard to talk, and this ulcer is my biggest one yet. :(  I've been getting mouth ulcers regularly for a long time now, so I have to see a dermatologist who specializes in oral mucosal disorders because we're wondering if something else is going on.

This has been a challenging week, but I continue to press on!  To close, I want to share this beautiful quote that I read just today. :)

"Suffering is not the absence of goodness, it is not the absence of beauty, but perhaps it can be the place where true beauty can be known" ~ Kara Tippetts (a sister-in-Christ who recently wrote a book about her journey battling stage 4 cancer....she knows all about suffering!)

Long overdue update

Hello friends,

I'm sorry it's taken me so long to post.  My weeks have been filled with doctors appointments and therapy, and I've been needing to sleep past 2 pm some days!

I just wanted to thank all of you who purchased through my Etsy shop!  It encouraged me so much. :)  It has definitely helped lighten my huge medical bill pile.

Warning: this will be a long medical update!

I haven't been tolerating j-tube feeds very well, and the gastroparesis has been causing a lot of stomach pain (all organs need energy to function....because of mito, my stomach isn't getting enough energy, so the food I eat just sits in my stomach or moves very slowly).  I saw my GI dr. on Monday, and he set up a new plan.  He's such a wonderful doctor!  He put in a new tube feeds order to see if a less calorically-dense formula will be better tolerated.  He also mentioned I could be dealing with Small Intestinal Bacterial Overgrowth (SIBO), but we will try this first.  Regarding the abdominal distention from the gastroparesis, he wants me to do a trial of increased j-tube feeds and less eating orally (by mouth).  That's a big step, but hopefully it will only be short term!  I won't be starting this yet until I get the new formula and until I can switch feeding pumps.  My current pump is quite heavy and makes it difficult to carry (because of the muscle weakness) so his staff is contacting other companies for a lighter one.

Unfortunately, my neurogenic bladder has been getting worse, and my GI surgeon explained how my kidneys could die if I don't get this treated.  Wasn't expecting her to say that!  She put in an urgent referral for me to see a neuro-urologist at OHSU.  That appointment's still a month away because she only works part-time and this specific dr. is the one I need to see.

I had a follow-up with my PCP today, and she's referring me to a cardiologist at OHSU who specializes in arrhythmia because I've been dealing with high heart rates, excessive night sweating, and heart fluttering.  That's how bad mito is. :(  It basically affects every body system!

Last week was Mitochondrial Disease Awareness 2014, and I didn't get a chance to blog, so you can consider this my awareness post. :)

P.S. The mito conference in Seattle two weeks ago was amazing!!  I'm so thankful for all the specialists studying this complex and extremely challenging group of diseases.  My mito specialist was there as well, and I was able to say hi to him.  He told me that I'm a conundrum (medically)...that's never good news.. :/  I asked him if he remembers me from June (he sees a ton of patients), and he said, "Of course I remember you!" :)

A neurologist who trained under an excellent mito specialist at Seattle Children's is now practicing at Legacy Emanuel's Randall Children's Hospital in Portland, and she's trying to get approval to open a mito clinic.  She also hopes to see older kids and adults as well!  She took my contact info, and I'm to call her office about all this within 6 months.  Oh that'd be so wonderful to have a local mito specialist! :)  This would technically be the first mitochondrial disease clinic in Oregon!

A thick binder full of notes and slides they gave to each attendee!

In front of the Bell Harbor Pier and conference center :)

This is how close the conference center is to the water!

Sad news

Hi everyone,

Thank you so much for your prayers and support!  It means a lot to me.  I am doing a little better than before. :)  After I wrote my previous post, I only had to go back to the ER one more time (that was a total of 3 trips to the ER and 1 urgent care visit in less than 2 weeks....).  The GI dysmotility got pretty bad, and I was in a lot of pain.  So Surgery told me to go to the ER to check for obstruction.  Thankfully, that was not the case, but the x-ray results did show that I was very backed up. :/  The doctors ordered more fluids, blood work, and IV meds, and I was able to go home with an updated medication regimen about 6 hours later!

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Today, I saw my pain dr. because the CRPS burning pain has been so very severe in my hands. :(  I have to hold an ice pack at night because my hands over-heat and burn like crazy!  They've been flaring up because I had so many IVs and blood draws the last several weeks, and needles always make things worse.  My pain dr. ordered an IV lidocaine drip combined with ketamine and magnesium which will be done on Oct. 8th.  So far away because he's booked out.. :/  Hopefully there will be a cancellation soon!

To my great sadness, Dr. S told me that he is leaving OHSU the end of October and moving to Washington to practice at the University of Washington Medical Center beginning in December.  I wasn't expecting this at all. :'(  I'm so very sad!!  It's not because he doesn't like OHSU anymore...he's been here for 18 years.  And he likes change, so he made the decision a month ago.

He told me at least he's not moving to Cincinnati or San Diego.  Washington is just a few hours away, and I'm not completely heartbroken because I get to see him up there. :)  But this news is still hard...change is hard.  I've been seeing him at OHSU for almost 4 years!  Out of all my doctors, he's my favorite.

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The next several weeks, I have many big follow-ups with Neurology, Surgery, GI, and Internal Medicine.  So I may not be able to update much.

Also, next Friday and Saturday, my parents, sis, and I get to attend a two-day mitochondrial biology conference presented by Seattle Children's Research Institute!!  Many mito specialists from across the US will be coming to speak to families and doctors.  I'm so excited!  The second day is a family day where you can meet other families with mito. :)

P.S. My etsy shop is open!  Check it out. :)  5% of profits will go towards MitoAction and mitochondrial disease research.  And the rest will be for all the increasing medical bills I have!

Another trip to the ER....this time by ambulance :(

Hey friends,

Please pray for me.  It's a long story, but since Saturday, I've been dealing with bad headaches every day and just feeling plain crummy.  I thought maybe I had another bladder infection, so my PCP's office told me to go to urgent care to check Tuesday evening.  Well, it turns out I don't have one, but I think I'd rather have that since it's treatable!  TMI, but I'll just state the facts: they found I have high ketones in my urine (my liver is forming too many ketone bodies and excreting it in my urine).  This is what that means---my GI dr. and the urgent care dr. said my body is basically in a starvation mode and is burning fat (instead of sugar) for energy.  Mitochondrial disease, protein-calorie malnutrition, and failure to thrive (all things I'm dealing with) don't go together. :(

Yesterday, things went downhill.  My weakness got worse, I was so hot from burning so much energy, and the nausea was so bad.  My parents had to call 911.  I thought I was going to pass out! :(  The paramedics had to use a sling to carry me out and put me on a stretcher.  I was taken to OHSU where they gave me a bunch of IV pain/nausea meds and IV fluids with dextrose.  In just 2 weeks, I've been poked a total of 8 times just for blood draws and IVs.

I'm home now because I started perking up after all that they gave me, but I am still so sleepy and weak.

My GI dr. and the nutritionists recommend that I start using tube feeds 24 hours around the clock, so please pray that things will start looking up and that I'll feel better.  I may have mito, but it won't have me!

I have touched the scars upon His hands, 

To see if they were real.

He has walked the road before me, 
He knows just how I feel.
When you feel there is not anyone, 
Who understands your pain, 
Just remember all of Jesus' suffering.

-from one of my favorite songs by Jeremy Camp ("Healing Hand of God")

Another trip to the ER.....

Suffice it to say, I am "ER-ed" out.....I just can't stay away! jk ;)

But first, let me rewind a little bit:

On Thursday, Aug. 7th, I had surgery for a jejunostomy feeding tube placement.  Thankfully, everything went well, but there were still some complications and setbacks.

1. After surgery, my heart rate was way "too high," as the nurses put it.

2. I had a low grade fever, something the team monitored closely in case it turned into Malignant Hyperthermia (which I'm susceptible to based on genetic testing).

3. I had to spend several long hours in the PACU (post-anesthesia care unit) because of breathing problems which continually set the alarm off.  The team ended up having to use my CPAP machine.

4. I had almost a whole LITER of fluid in my bladder!  The doctor was astounded!  I couldn't empty it on my own (because I have a neurogenic bladder), so they ended up having to place a Foley catheter---such a painful procedure! x[

5. During surgery, they wanted to place an arterial line in my arm to continually monitor blood gases....my blood work got so out of whack.

6. Over the course of this hospital stay, I had to be given 6 liters of IV fluids because my potassium kept dropping very low!

7. The dr. said I bled a lot during the surgery, even though this was laparoscopic (minimally-invasive).

Anyways, I got home from the hospital Saturday evening!  I was sent home with a long list of medications to take, including some stronger pain meds because having CRPS makes my nervous system more sensitive than normal.

Monday night, I noticed the J-tube site was bleeding a little bit, but I just tucked some gauze near the area and went to bed.  Well, at 1:30 in the morning, I woke up to wet blood on my sheets and pajamas.  I groaned, knowing where this was going to lead me.  My mom called the GI surgery resident on-call, and he said to go to the ER if I was bleeding at the central part of the J-tube.  So to the ER we went!  As you can imagine, this was unwelcome---here I was sleeping peacefully! ;)

When I arrived at the OHSU ER, they got me right in and even put me in the pediatric side (Doernbecher's) so that I wouldn't have to wait at all.  It's a bad sign when the emergency dr. knows you and is familiar with your case.. ;)

The nurses were concerned about all the blood.  The dr. quickly called Surgery to come.  What followed was very unpleasant and so painful!  The dr. washed and cleaned the area aggressively to try and see where the bleeding was coming from.  She had to use scissors to remove thick blood clots....that part wasn't nice to watch. ;)  The bleeding still kept coming, so she eventually had to suture the place up again.  Thankfully, that did the trick!

I have a follow-up with Surgery on Friday.  They're going to remove these things at the J-tube site called bolsters (which are currently anchoring my small intestine to the wall of my abdomen).  And in a few weeks, I get to have the tube changed to a "low-profile button." :)

Today I started feeds at a very slow rate.  Here are a couple pics:

This is how I put medications through my tube!

I receive high-calorie formula through the feeding tube. :)