Getting a feeding tube

Hey friends,

Just a short update!  I had appointments with GI and Nutrition this past Tuesday, and it's not great news---my weight's still steadily dropping..  So my GI dr. has to put in a nasojejunal feeding tube which will bypass my stomach and go directly into the small intestine.  I'm not getting enough nutrition because my stomach isn't moving food well, and that's making all my symptoms worse.  This NJ tube placement will be done in the OR on May 16th!  It's kind of a big step, but I'm past a medication stand-point and I'm losing weight too fast.  When I see the mito specialist June 17th, we'll see if he thinks I need a permanent feeding tube.

*54 DAYS!*

;)

I read this beautiful quote a while back from a small book that one of my friends let me borrow, and I wanted to share it with you.  I hope it's an encouragement to you. :)

"If God had told me some time ago that He was about to make me as happy as I could be in this world, and then had told me that He should begin by crippling me in arm or limb, and removing me from all my usual sources of enjoyment, I should have thought it a very strange mode of accomplishing His purpose.  And yet, how is His wisdom manifest even in this!  For if you should see a man shut up in a closed room, idolizing a set of lamps and rejoicing in their light, and you wished to make him truly happy, you would begin by blowing out all his lamps; and then throw open the shutters to let in the light of heaven."

~Samuel Rutherford

It's Official!

Wonderful news! :)  My appointment with the mitochondrial disease specialist at UCSD Medical Center is set in stone for June 17th!  My parents, sis, and I will be there at least a week since the muscle biopsy is scheduled for Friday, June 20th as well.  And after I see the dr. that Tuesday, we'll know more then if he will order any additional tests, etc.

this is what the hospital looks like!

*2 MORE MONTHS*

:)

Yesterday, I had physical therapy and also saw my pain dr.  The CRPS pain in my feet has still been really bad.  And this past Friday, my pinky finger and side of my right hand (my good hand!) accidentally got cut, and now the CRPS has been flaring up in that area. :/  That's how bad this pain syndrome is---the smallest cut can set a limb "on fire."  So Dr. S is changing one of my meds and switching it to a stronger one.  He also ordered some labs---one is to check my lactic acid level (at the request of the mito specialist in San Diego).  The last time I had it checked a year ago, the results were elevated..

This coming Tuesday, I have appointments with GI and Nutrition.  Unfortunately, the new medication I started a couple weeks ago hasn't been helping, and I still can't eat much.  I'm steadily losing more weight. :(

P.S. Please continue to keep Justina Pelletier and her family in your prayers.  Her health is getting worse by the week, she's losing her hair, and she's swollen all over.  This is a great article (written just today) by someone from MitoAction about the difference between medical child abuse and mitochondrial disease: http://www.mitoaction.org/blog/why-mitochondrial-disease-looks-nothing-medical-child-abuse  It also includes an update on Justina.  If you can't finish the whole article, read paragraphs 3 and 4.  We need to get her home!

Latest update!

I mentioned in my last post that I've been dealing with severe vertigo and a bunch of new, ongoing GI issues (nausea, weight loss, decreased appetite, stomach pain).  Well, my GI dr. and neurologist quickly got me in this week, and I'm so glad I was able to see them.  They're wonderful!

On to the not-so-good news:
I am down to about 79 lbs.  And I can hardly eat without getting terrible stomach pain and nausea.  It's most likely that I'm dealing with delayed gastric emptying which is dysmotility of the stomach..  This is common with mitochondrial disease.  See how mito can affect basically any part of the body? :(  It especially affects organs that demand a lot of energy like the brain, heart, muscles, GI system, etc.

Because of this, I have to be on a mainly soft food/liquid diet for now, and that's not good news since I'll probably lose even more weight.  Dr. P couldn't even hear any abdominal sounds with his stethoscope---this means the food isn't moving well out of my stomach.  He's putting me on a proton pump inhibitor drug and a compounded medication that will hopefully help this dysmotility issue..  I see him again and the nutrition therapist in less than 3 weeks!

My neurologist has come to the conclusion that the vertigo I've been experiencing for a whole month now is from an inner ear disorder (which usually happens after whiplash or concussions!).  I've never had either of those, but you know me. ;)  I'm not the typical patient!  At the appointment, Dr. G induced vertigo by doing these different maneuvers, and it wasn't fun at all!  She put in an order for me to have intense vestibular rehab.  She also prescribed a vestibular suppressant for bedtime to see if it will help me sleep better.  As you can imagine, it's very hard to get restful sleep with vertigo.

The CRPS pain has been ramping up again, too---my feet get really discolored, and the burning pain is just horrible.  I sent a message to my pain dr., and he told me he needs to see me for a visit.  I called to schedule an appointment, and he is booked until May 8th!  But do you want to hear an answer to prayer? :)  He has a cancellation in 11 days!

These symptoms on top of everything else has been challenging to manage all at the same time, but I thank and praise God for His mercies that are new every morning!

I just want to close with a quote a dear friend shared with me.  The last sentence that I highlighted gives me chills! :)
 

And so, perhaps in your life, you say, "I do not understand why God let my dear one be taken.  I do not understand why affliction has been permitted to smite me.  I do not understand the devious paths by which the Lord is leading me.  I do not understand why plans and purposes that seemed good to my eyes should be baffled.  I do not understand why blessings I so much need are so long delayed."  Friend, you do not have to understand all God's ways with you.  God does not expect you to understand them.  You do not expect your child to understand, only believe.  Some day you will see the glory of God in the things which you do not understand.

~~J. H. McC

Symptoms and Setbacks

Hello friends,

I'm sorry it's been almost two weeks again since I last blogged.  I always have bad news to share. lol ;)  The disease process (whether it's mito or something else) is progressing, and I'm dealing with way too many symptoms.  I look so normal, and yet I deal with all of this continually:

-Migraines
-Complex Regional Pain Syndrome
-Nystagmus
-Ptosis
-External Ophthalmoplegia
-Tinnitus
-Myoclonus
-Sleep-disordered breathing/sleep apnea
-Hypersomnia
-Muscle weakness/wasting
-Hypotonia
-Fatigue
-Premature Atrial Contractions
-Postural Orthostatic Tachycardia Syndrome
-Slight Tricuspid Valve Regurgitation
-G.I. dysmotility
-Dysphagia
-Hypermobility
-Cervical spine instability
-Mild scoliosis
-Dysautonomia
-Tremor
-Vertigo
-Muscle spasms/cramping in right hand

THIS is the nature of mitochondrial disease.

I mentioned in my last post that I've been having breathing problems at night, so my sleep dr. did increase the CPAP pressure this past week.  It's really strong, but it does help. :)

I've also been having some setbacks in physical therapy and occupational therapy.  My brain isn't recognizing where my left foot is, and I'm having sensory/proprioception problems.  The dizziness came back again, too.  But my physical therapist is awesome, and I know he'll do all that he can to try to help these issues.

My occupational hand therapist is wondering if the muscle spasms/cramping in my right hand is from dystonia, so he's having his colleague take a look.

In addition to all of this, I'm experiencing bad stomach pain after eating.  And it doesn't change with the type of food (gluten-free or not).  I really hope this isn't delayed gastric emptying..  Last year, I had that gastric emptying scan which came back normal, but I didn't have this symptom then..  If it's not better within the next week or so, I'll have to tell my GI specialist.

I'm just getting worse as each week passes, but the Lord has been so faithful.  I couldn't do this without Him.  I know I shared this before, but one of my favorite Scripture passages from the book of Isaiah says this, "Fear not, for I have redeemed you; I have called you by name, you are mine.  When you pass through the waters, I will be with you; and through the rivers, they shall not overwhelm you; when you walk through fire you shall not be burned, and the flame shall not consume you" (Isaiah 43:1b-2).  That's what the Lord has been to me.  Always there. :)  And with His help, I know I can take on these challenges!

I will end with something that encouraged me!  Last week, a rep from Courtagen Life Sciences (where I got my DNA testing) emailed me and said he found my blog on the web! :)  He wanted to share it on Courtagen's facebook page which has over 26,000 followers!  I thought that was so cool and neat!  I took a screenshot of it. ;)

MRA results and more!

I'm sorry it's been a while since I last updated!  I just want to thank you all for your continual prayers!  It's been a tough couple of weeks.  At the end of February, I was dealing with blurred vision for several days, so my neuro-ophthalmologist squeezed me in for a follow-up.  She thinks I was experiencing an ocular migraine.  It's either that or mito-related.

Last Monday, I had an MRA done of my neck.  The test was pretty much finished, and one of the techs even took out my IV.  But one of the other techs came back and said they needed to do some brain sequences.  I was a little puzzled why (since I just had a brain MRI the beginning of February).  But I got that done and then went home.  When I woke up the next day, my mom said that the MRI place called again and they wanted me to come back that day for more brain images!

Well, I received the results on Friday, and they're somewhat abnormal:

This is me in vascular form. ;)  That blue arrow I drew is pointing to my left vertebral artery---see how it's kind of coiled and looped?  That's called tortuosity which is a new med term that I learned. ;)  People with tortuosity are prone to aneurysms/strokes, and my neurologist said it can be seen in patients with Ehlers-Danlos Syndrome.  My cervical spine instability is putting stress on my arteries....so my neurologist wasn't very surprised by this new finding.  She said it's just something to keep an eye on.

I've also been dealing with decreased appetite and weight loss again. :(  I've been doing all that my GI dr. told me to do, but it's not helping.  So he referred me to their clinic's nutrition therapist, and that appointment's next month.  We'll see if she has any other insight to offer..

After PT and OT today, my mom and I were able to ride the tram up to the OHSU hospital again because I had to drop off my CPAP sd card at the sleep clinic front desk (my sleep dr. wants to take a look at the data to see if he needs to increase the CPAP pressure because I've been experiencing breathing problems due to tired respiratory muscles).  OHSU gives free passes to patients! ;)  The ride was especially fun today because the weather was sunny and beautiful!

For those who don't live in Portland, this is what it looks like!  Took this while riding the tram. :)

That's about all for now!  Haven't heard more about the plan from the mito specialist in San Diego...the coordinator is waiting for tests like the muscle biopsy to get pre-authorized.  And you know how long insurance matters take.. :P

*Good news/Bad news*

I have good news and bad news....what would you like to hear first? ;)  Let's start with the bad:

I got my MRI results, and the report says I have levocurvature of my cervical spine which wasn't there before.  I also have wasting of several vertebrae, and my C5-6 disc narrows the ventral CSF space of my spinal canal.

When I got those recent MRIs, the doctors forgot to order an MRA (magnetic resonance angiography) of my neck which my neurologist requested.  Sooo....guess what.  I have to get yet another MRI which will be my 12th one so far. x[

For the past few weeks now, I've also been dealing with daily cramping/stiffness in my right hand which makes it difficult to write, text, hold a fork, crochet, play piano, etc. :(  And this was my good hand!  My physical medicine dr. referred me to occupational hand therapy again, and he also wants to do a bilateral EMG/nerve conduction study which will be my 6th EMG.  That's scheduled for March 11th.

I also started experiencing dizziness/blurred vision this past week, and it's been horrible....my neurologist still suspects possible TIAs.  Thankfully, my MRI is scheduled for this coming Monday.  The fatigue has been really bad, too, and I had to sleep 16 hours yesterday. :[

I know I'm getting worse, and I hate that my body's not functioning well....but are you ready for the good news? :)

I have a tentative appointment with a mitochondrial disease specialist in San Diego on June 17th!!! :D :D :D  So very thankful!  The Lord answered my prayers!  I got referred to this dr. last July, and we were all beginning to wonder if this would ever happen!  This specialist has been working with mito patients for over 40 years!  He's also on the scientific and medical advisory board for the United Mitochondrial Disease Foundation.

I should be hearing more about the plan soon. :]

P. S. This is my "loaner" gait trainer until I get my own.  Like it? :)  I am determined to learn how to walk normally on my own, even if it's just for short distances!

P. P. S.  Please pray for 15 year old Justina Pelletier and her family.  What happened is terribly sad, unreal, and tragic.  If you haven't heard, her story has made national news.  Google her.  This hits home because she has mitochondrial disease. :(  Her parents lost custody of her, and her health has dramatically declined.

Here are a couple of good articles:

http://abcnews.go.com/Health/advocates-fight-teen-justin-pelletier-held-state-pysch/story?id=22312907  In this article, Dr. Richard Boles of Courtagen (where I got my DNA testing) spoke.

http://www.huffingtonpost.com/cristy-balcells/first-do-no-harm-how-we-f_b_4843997.html  This article was written by Cristy Balcells, executive director of MitoAction.