Referral to Hematology.

Hi everyone,

Your prayers would be appreciated!  The past two weeks, I've been feeling worse than usual...very sleepy especially. :(  To be honest, I just feel plain crappy, and whenever that happens, the CRPS always ramps up---the nerve pain has been really bad lately.  I'm still pale and short of breath as well.

We found out that my white blood cell count has been dropping (it did go up a little bit this week, but it's still not my normal), so my primary care physician is referring me to OHSU hematology.  We're not sure if this is mitochondrial disease-related or if something new is happening.  She wanted the soonest appointment available, so it's scheduled for next Tuesday the 21st.  I see God's goodness in this---the hematologist I'll be seeing is another Top Doctor.  He's nationally renown, and even my GI dr. has heard of him. :)  I'm just so blessed to have a knowledgeable and caring medical team for my complex case!  And it makes this rough journey a little easier, knowing that I'm in good hands.

I had another follow-up with my GI specialist this afternoon.  Unfortunately, I still haven't noticed any extra energy with the IV iron infusions, so he is ordering a bunch of different labs to see if I'm deficient on certain minerals while on TPN.  He's also ordering a carnitine panel.

I tried tube feeds last week at 10 mls per hour, but after receiving just 16 mls, I felt sick to my stomach and got super distended.  So I have to decrease to a rate of 5 mls----that's one teaspoon per hour..!  That's not enough to live on, so I still have to receive TPN and lipids.

I will be sure to update next week after the hematology and sleep medicine appointments.  The Lord is my hope and strength! :)

This is what iron looks like.  My GI dr. said 5 small bags of this is equivalent to receiving about 8 pints of blood!

GI update

Hello everyone!

Thanks for stopping by and for your continued prayers. :)  I'm so blessed!

Haven't felt up to blogging in a while because the iron deficiency anemia makes me ever so tired, short of breath, dizzy, and pale.

I've been needing to breathe fast as well which makes it hard to use CPAP at night.  I messaged my sleep dr., and he called me back.  He really is the sweetest and so kind! :)  He's the co-medical director at the OHSU sleep program and a Top Doctor.  Can you believe this---he's booked out 4 months!!  He thinks a lot of new things are going on with me sleep-wise, so he's having his office double-book to squeeze me in April 22nd.  He wants to order more tests and re-evaluate.  He's astonished that I have to sleep 16 hours a day. :(

I had a good follow-up with my GI dr. last week.  He always spends so much time with me...very thankful for a doctor who cares. :)  For the anemia, he is having me receive IV iron infusions once a week for 5 weeks to bring my blood counts back up.  He warned me that the iron looks like motor oil....that's gonna be interesting. lol ;)  Some patients experience body aches as a side effect, but I hope I tolerate it well.

Still receiving TPN every day for 16 hours, but my GI is having the TPN volume/calories decreased by 20%.  We are going to introduce j-tube feeds again at a very slow rate of 2 teaspoons per hour.  And we'll be using the elemental (broken-down) tube feeds formula.  Not sure yet how this will go as I still deal with hypoactive abdominal sounds, etc.  But I'm game to try!  I see my GI specialist again to follow up on this in 1 1/2 weeks.

This morning, I had my cardiac MRI.  It's different---they have to capture the images according to each heart beat, and you wear EKG leads and a respiration belt.  During the scan, I was told to take a deep breath, blow it out, and hold for 10-15 seconds....this cycle repeated over and over again!

Tomorrow I have an orthopaedics follow-up, and Friday is my first IV iron infusion at the hospital.  After that, I'm done for the week!

To close, I wanted to share this with you.  My sweet aunt emailed this to me recently, and the words are so encouraging!  And perfect for this Easter! :)

Cardiac findings and ENT update

Hey friends,

Thank you for checking in and for your continued prayers!  So much keeps happening, and this journey just gets more and more complicated.. :/

But this I remember: the Lord fights my every battle, so I will not fear.  He goes before me and never forsakes me!

Some of my blood counts have been steadily dropping since December, and I'm now anemic.  That and low grade fevers every day hasn't been fun.  My primary care physician ordered a bunch of labs, and we found out that I'm very deficient on ferritin (even while on TPN).  I should hear soon what the plan is for that.  And, I see my GI dr. next Thursday for a much needed follow-up.  If the ferritin deficiency is not what's causing the anemia, then my PCP will be referring me to Hematology.

Last month, my cardiac electrophysiologist referred me to General Cardiology to discuss my recent heart echo results.  That appointment was this past Monday.  The dr. went over the echo images and explained that the wall of my descending aorta (in the abdominal area) is very irregular and "jagged" which is not normal.  It's supposed to be smooth.  She's not sure what to make of this and whether it will cause problems, so she ordered a cardiac MRI to investigate this further.  This will be my 17th MRI scan...

Today I had a modified barium swallow study, esophagram, and ENT follow-up.  Long day!  The results of the two tests show that the movement (peristalsis) of my esophagus is very slow....food and pills I swallow get stuck and don't move down well from my mouth to the stomach.  This is called esophageal dysmotility.  My ENT dr. isn't surprised that I have this because I've already been dealing with small intestinal dysmotility and large intestinal dysmotility....my whole GI tract is affected. :(  This is because there aren't enough mitochondria in my GI tract (caused by the mtDNA depletion).

I had these two tests done last year, and my ENT dr. compared them.  Things have worsened since then.  Unfortunately, we won't know what will happen a year from now..

Mitochondria are in every cell!  That's why mitochondrial disease affects SO much.  It's not just a muscle disease or heart disease....it affects the intestines, muscles, heart, brain, autonomic nervous system, eyes, and anything else you can think of that requires energy!  I recall hearing a talk by a mito specialist in LA, and he explained that "energy is the difference between life and death."  Many people often say I look so normal...that's because mito is a disease that affects at the cellular level.  Anyways, enough of my rambling....just want to raise awareness. ;)

Every time my doctors discover more and more that's wrong inside my body, the only thing I can do is rest in the Lord.  He has a good purpose in every single thing that happens!

"You go before me every step I take..."

Hey everyone. :)

This past Thursday, I had a neuro-urology follow-up.  I tried two different medications for my neurogenic bladder, but both caused awful side effects.  So at this appointment, my doctor discussed what needs to be done.  It looks like I may have to have surgery #9 this year in order to protect my kidneys/bladder.  She recommends this be done sooner rather than later.  It involves neuro-modulation (the spinal cord stimulator that I had implanted for CRPS pain back in 2011 was a neuro-modulation device).  So this surgery is very similar---it corrects the abnormal signals in my brain and helps the sacral nerves in my spinal cord.  We won't be making the final decision, though, until after I see my mito specialist in June.

My neurology appointment today went well.  I updated her on all that's been going on.  I've been dealing with an increase in shock-like muscle jerks, and she explained that they could be caused by many things, one of them being seizures..  If the movements worsen, she'll have me get an EEG (electroencephalogram) to try and pin-point where these are coming from.

Regarding the low-grade fevers I experience that come and go, I'm to monitor them.  If they reach beyond 100.1 degrees, I have to let my PCP know to get this investigated further.  My neurologist said fevers can be caused by numerous conditions, and she believes they're not from dysautonomia.

TPN overall has been a blessing.  But, it also causes something called fluid shifts which result in very painful charlie-horses!  I had over 20 long-lasting muscle cramps (sometimes each last up to 10 minutes long) all over my body yesterday.. :(  My GI dr. is continually trying to lessen these by adjusting the TPN infusion rates, but lately, this issue seems to be getting worse.  I have another follow-up with him in a couple of weeks.

Yesterday, my pastor shared this passage from 2 Corinthians.  It's a familiar section of Scripture to many, but hearing it again was so encouraging.  This has been on my heart:

"So [I] do not lose heart.  Though [my] outer self is wasting away, [my] inner self is being renewed day by day.  For this light momentary affliction is preparing for [me] an eternal weight of glory beyond all comparison, as [I] look not to the things that are seen but to the things that are unseen.  For the things that are seen are transient, but the things that are unseen are eternal." ~2 Corinthians 4:16-18

Another ER visit and more appointment updates.

Hi friends,

This past Monday evening, I started dealing with 100 degree fevers, severe nausea, and headaches.  So my mom called my GI doctor who recommended that we head back to the ER to get blood cultures drawn in case of a line infection.  Can't catch a break, and ER visits always wipe me out. :(  We spent over 8 hours there---the ER was jam packed!  The doctors ordered labs, IV dilaudid, and zofran.  The meds helped a lot.  Thankfully, they couldn't find signs of infection in my blood, although blood cultures do take a couple days to come back.  They came to the conclusion that this was either a virus or the dysautonomia (autonomic nervous system dysfunction) that I deal with on a daily basis---my body could now be having trouble regulating temperature..

We got home after 6 in the morning on Tuesday, so I've been so exhausted!  I did have two appointments yesterday with my surgeon and a new ENT dr.  I had a lot of granulation tissue growing at my j-tube site, so my surgeon used more silver nitrate sticks to burn it off.  The ENT visit went well.  I had a laryngoscopy done---one of the doctors inserted a scope down my nose to look closely at my swallowing muscles and voice box.  My ENT dr. noticed that all the muscles in that area have atrophied a lot, so he thinks this may be what's causing my chronic cough/swallowing issues.  He ordered another modified barium swallow study with x-ray to be done in 3 weeks.

Here is some good news: I am now off both blood thinners!  I'm so thankful. :)  I had a follow up with the anti-coagulation clinic at OHSU on Monday, and the nurse practitioner consulted with one of their hematologists.  They all agree that I've been treated long enough for this type of clot.

I have a lot of upcoming appointments next month with Neuro-urology, Neurology, ENT, and GI..  This week has again been rough, but I strive to keep my eyes fixed on Jesus and not on all these continuing problems.  One day they will end! :)

"Days have come, days have gone
I know one thing’s sure
Hanging on by the words of Your promise, Lord
You are good, You are true
Even in my pain

And I’m thankful for this suffering
‘Cause it’s brought me right here on my knees...."

~partial lyrics from Perfect Love by Jeremy Camp

Never a dull moment.

Hi friends,

Just an update for you!  For those who don't know, I had to go the ER yet again this week. :(  My PICC line site was red, hot, and swollen, and I was experiencing low grade fevers when my home health nurse visited last Thursday and when I saw my GI specialist this past Tuesday.  He feared a line infection and wanted it to get pulled.  So Wednesday I was told to go to the ER.  When the doctors and nurses know you, you've been there too often...... :/  They drew blood cultures and labs to check for infection and ordered an ultrasound to rule out clots in my upper arm.  And guess what----a clot was found in my cephalic vein!  That's one of the complications when you have a central line.  They admitted me overnight in the ER observation unit for IV fluids and potassium because my potassium levels were low.  They also pulled my line and started me on two heavy duty blood-thinners to prevent it from traveling to my lungs and causing more problems.  Yesterday morning, the PICC team came by to place a new line in my left arm.  I was also taught how to give myself anti-coagulant shots twice a day....not pleasant. :(  This certain drug burns horribly!

Today I had a follow-up with my cardiologist.  We discussed my recent heart echo results, and he is going to consult with another cardiologist because they found thickening of my aorta (that was a surprise because it wasn't there on my two previous echos!).  Also, they found a mobile mass in my right atrium, but he thinks this is congenital and shouldn't cause problems.  Still, that was another surprise since it wasn't found there before!  I am a puzzle, and I keep my doctors on their toes. lol ;)  My cardiologist is also going to get in touch with my GI specialist next week to see if more sodium can be added to my TPN because my high heart rates, dizziness, and heart fluttering has been getting worse.

Monday I have an appointment at the anti-coagulation clinic to get labs drawn and see the doctor there.  And Wednesday, I have surgery to get a central line placed in my chest!  They wanted to do this soon to prevent a clot from happening again.....but who knows, I'm not the typical patient. ;)

It's been crazy hectic, and my arm where the clot is still doesn't feel well yet.  But I continue to trust the Lord and persevere!  He is good.

Thank you all for your love and prayers!

P.S. A famous mito specialist from Ohio came to speak at OHSU the same evening I was in the ER.....the ER docs were so kind to let me go to it since the lecture was literally just down the hall! :)  It was very informative.  I was able to ask the mito specialist about my mtDNA depletion results.  He looked over the report and told me that my depletion is 99% which is very high....no wonder my GI tract is so affected. :(  I really hope these results and the ones pending will help me be eligible for a future clinical trial!

My new PICC line right after it got placed :)