Neurology and GI visits

Hey guys!

On Tuesday, I saw the neurologist at Randall Children's.  She's the doctor who went through a year long mitochondrial medicine fellowship up in Seattle.  It was a good appointment, and she's really nice. :)  I'm so blessed to now have a doctor who will take care of all my mito issues locally.

I mentioned that I've been dealing with worsening myoclonus (muscle jerking)---it can be brainstem related, spinal-cord related, or from the muscles.  It could also be from seizures.  So she went ahead and ordered a standard 30 minute EEG which is scheduled for June 11th.  If it's normal (it often is because it's such a short study), she'll look in to admitting me for a longer EEG at the hospital.

She's also going to write up an ER protocol letter for whenever I have a "mito crash."  She'll explain what type of IV fluids I should receive, what meds to stay away from, etc.  I'm sure the letter will be really helpful for the ER doctors!

Today I saw my GI specialist.  He's going to decrease the TPN fluid volume/calories again because the amount I'm currently receiving is still too rough on my neurogenic bladder, and I'm now at a good weight. :)  If I start losing weight again, then he said we'll have to go back up on the TPN.  We also discussed how I may not be able to get off of it if the stomach/intestinal issues don't improve.. :(

In one and a half weeks, I have my 3rd sleep study and 1st multiple sleep latency test.  The exhaustion has just been getting worse. :(

In other news, we leave for San Diego in 23 days!!

Update 5/20/2015

Hi friends,

A short update for you---thank you for praying for me and my family. <3  Things still aren't going well.  Abdominal pain and distention has been coming back with a vengeance.  I continue to deal with daily fevers (it went up to 101.6 one time) and lots of other symptoms.  I've had 13 tubes of blood drawn so far just for lab work ordered by my hematologist.  He's going to be out of town the rest of this week but plans to call me next week to go over results, etc.  Not sure if something will be found at this point.... :/  I wish I wasn't so complicated. :(

To make matters worse, I have a sore/growth on my arm which has been getting bigger and leaking/crusting a ton.  I saw dermatology yesterday.  They're not sure if it's a lymphangioma or what, so they wanted to do a biopsy of it.  The results should be back next week.  When the doctor left the room, he told the resident, "That was unusual!"  I don't like being rare. :(

We also got my blood copper level back, and it dropped once again, even though I'm on twice as much IV copper in my TPN.  Go figure!  I mentioned before how copper deficiency can cause numerous neurological and hematological issues which is probably why I'm still dealing with bad headaches and nerve pain every day.  My feet have been burning so horribly. :(  Next Thursday, I have a follow-up with my GI specialist.  I also see neurology (the local mito specialist) at Randall Children's this coming Tuesday.

P. S. In exactly one month, my parents and I leave for San Diego for the third time to see my mitochondrial disease specialist!

This journey's been rough, and all I can do is be strong, take heart, and remember that the Lord fights for me!

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! :)