Waiting

Hi guys,

Just wanted to check in and let you know that I'm hanging in there!  Haven't posted an update in a while because I'm waiting to hear from my hematology doctor again....he called last week and wanted me to keep a record of my fevers every 2 hours so that he can look at the pattern and decide on further work-up.  He plans to touch bases with me on Friday..  Not going to go into details yet, but something new is going on as well and it doesn't seem very good.

Last week, I finished my 5th IV iron infusion....now we watch and see how my blood counts do.  I'll also be getting my copper level checked again in a couple of weeks to see if I'm still deficient.

Still not tolerating tube feeds or solid food, so I continue to remain on IV nutrition.  I will be trying a "blended real food" tube feeds formula soon to see if I can tolerate that.  The elemental formula I'm currently on is even more broken down than the blended food formula, so we're not sure how this will go..

I saw the pain team this afternoon.  I've been dealing with bad headaches every day, and the CRPS nerve pain in my feet is worse because of not feeling well.  So they came up with a good plan. :)  I will be increasing one of my pain meds to twice a day, and if that doesn't help, they'll have me try a different narcotic analgesic---it's safe, non-addicting, and would be given at a very small dose. :)  Thankful for my doctors!  They're so sweet and always decide on the best and safest route for me!

P.S. My etsy shop (www.etsy.com/shop/BeautyInItsTime) hit 105 sales this week!!! :D  And I'm currently putting together a custom order for someone which will be my 106th and 107th sale. :o)  I love making jewelry for people. <3

Hematology and sleep medicine visits

Yesterday, I had my first hematology visit at OHSU.  We found out (through lab work that my GI dr. ordered last week) that I have a copper deficiency which is known to cause many hematological and neurological issues.  So twice as much copper is now being added to my IV nutrition.  By the way, I've been on Total Parenteral Nutrition for about 4 months now with no end in sight.. :(

At this point, we don't yet know why I'm dealing with daily fevers, night sweats, and other symptoms.  My hematologist is looking into rare blood disorders and researching more.  He ordered lots of common and uncommon lab work.  By tomorrow, I will have had 8 tubes worth of blood drawn!

This afternoon, I saw my sleep medicine dr. for a follow-up.  I'm still dealing with fast breathing and shortness of breath.  I'm also very sleepy and not able to tolerate my CPAP.  So he's ordering another sleep study and something called a multiple sleep latency test.  He wants new data to see if I need BiPAP (bi-level positive airway pressure) now.  Unfortunately, they're completely booked, so I'm not able to get the studies done until June. :/

Here's some good news! :)  I mentioned last year how I was able to meet a neurologist at the mitochondrial biology conference at Seattle Children's.  She completed a mitochondrial medicine fellowship and is practicing at Randall Children's Hospital in Portland.  She's a pediatric neurologist but is now able to see older kids and adults at her mito clinic.  In February, my neuro-ophthalmologist referred me to her.  The referral process took 10 long weeks, but I received a letter last week saying that I can now see her!  This is a blessing from the Lord! :)  She's booked out, so the appointment isn't until May 26th.

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