Apologies

Hey friends, 

I haven’t used this blog in a long time because my brother made me my own website called painwithpurpose.com a few years ago.

Well, I was deleting over 1000 spam subscribers on painwithpurpose.com, and I accidentally also deleted ALL my email subscriber friends (that is, those who wanted a notification whenever I posted an update 😭😭).

I am SO very sorry that this happened!  And I tried looking everywhere to see if I could reverse this “action.”  But I can’t.😭

So, since most of you who are subscribed here went to painwithpurpose.com and subscribed over there a long time ago, I just wanted to let you know what happened. :( 

If you’d like to still get notifications again over there (I just posted a new blog update about my yearly mito appointment in San Diego), you can head to the right side of my painwithpurpose.com website, scroll down a little, and find the “follow by email” box.  When you enter your email to get notifications again whenever I post a blog update, you’ll receive a VERY IMPORTANT EMAIL where you will need to CONFIRM your subscription (check your spam folder in case you don’t see it)....just to clarify again, if you don’t press “confirm,” you will not receive any notifications of blog updates.

Again, I’m so very sorry this happened!! :(  Please let me know if you need help with any of this!

Love, Kerissa

Notice: New Website

Just another short reminder that my blog has moved! :)  I will no longer be posting here on this website.  Just wanted to clarify as there have been new readers signing up on this blog.

I WILL however be blogging on my new website--- painwithpurpose.com :)

On the new site, there's a little area on the right where you can enter your email address to receive updates.  But, BE SURE to activate your subscription (you'll hopefully receive another email to do just that), otherwise you won't receive any new updates!

See you at painwithpurpose.com! :)

Kerissa

My blog is.....moving!!! :)

GUESS WHAT?!? :D

My awesome brother Curtis made me a website with my own domain which means I no longer have to use Blogspot!! :)  He put together this new website for me as a 2015 Christmas gift.  It's amazing and such a lovely writing spot.

Ready to see what it looks like?!  I love it. :)  And I'm sure you will, too.

Click this link:

~~~~~~~>>>>> www.painwithpurpose.com

For those who follow my blog through email, you can still do that with my new site! :)  Just go to the right side of my page and find the little box that says: Follow by Email.  Enter your email and be sure to check your inbox!  You'll need to activate the subscription.

So long, Blogspot!  I'll try to post a new update on my new page later this week. :)

Keeping on.

Last Wednesday, I had a follow-up with my pain dr.  The IV ketamine I was able to receive in the hospital last month helped the nerve pain so much, but as soon as it was stopped upon discharge, the pain ramped up again. :(  Unfortunately, getting IV ketamine in the clinic is not an option currently due to insurance issues, but my pain dr. is working on this!

In the meantime, if insurance pre-authorizes it, I will be switching to an even stronger, anti-convulsant pain medication.  I'll also be getting another bilateral lumbar sympathetic nerve block which is a procedure where the doctors inject anesthetic medications around the sympathetic nerves to temporarily "block" them and try to decrease the nerve pain.  She's booked out so it won't be until the end of May.  My pain dr. also referred me to Palliative Care, and I now have an appointment with one of the palliative doctors next month.

On Thursday, I saw my nephrologist.  She was hoping my blood pressure numbers were high enough so that I could start a medication to help my kidneys hold on to magnesium, but unfortunately, my BP is too low.  Putting me on that med would dangerously lower my blood pressure, and I'd land in the ER.  Given how much IV magnesium my body needs, my nephrologist doubts I'll ever be able to stop the infusions entirely. :(  I'm disappointed that I can't even cut back on my daily IV fluids with Mg (it's no fun lugging around an IV pump for 3-6 hours every day), but I know God's timing is best.  She is starting me on an oral Mg supplement in addition to the infusions to see if we can get my blood Mg levels stabilized better.

This morning, I had a follow-up with the Infectious Disease dr. since I'm off IV antibiotics.  I'm completely wiped out and sleeping til 3:00 pm most days.  I feel like a walking zombie. :(  The doctor said it's because I'm still recovering from being septic.  And it will take longer for me to get back to normal because of the mitochondrial disease.  On top of that, I'm anemic, so my GI is running some other labs to figure out if this is iron deficiency anemia or hemolytic anemia.

Next week I have appointments with Wound Care (for my raw feeding tube site) and Cardiology.

Thank you for checking in!

P.S. This is my 200th blog post!  I started this blog December 31, 2011.  Thank you so much for reading and following my journey!  It means a lot to me. :)

"I have said these things to you, that in me you may have peace. In the world you will have tribulation. But take heart; I have overcome the world.” -John 16:33

My first blood infection.

Hello all,

I'm sorry I've been MIA here!  I'll just cut right to the chase. :)

On February 26th, I started dealing with vomiting.  We didn't know what was going on, and my GI specialist was unsure if this was due to worsening GI dysmotility.

I had 3 more episodes of vomiting and dry heaving, and very early in the morning on March 8th (around 12:00 am), I began experiencing a fever of 101 degrees, terrible low back pain, severe chills, headache, and heart rates over 150 bpm.  I was taken by ambulance to Legacy Emanuel ER because OHSU's ER was filled to the brim, and they couldn't even take ambulance patients.  I had lots of tests ran all throughout that early morning, and we found out that my blood cultures came back positive (in my blood and central line) for gram positive cocci in clusters (more specifically, Staphylococcus Capitis).  I was septic and spent 9 days in the hospital.  I had to have a spine MRI to make sure the infection didn't spread to my back.  The doctors also ordered a heart echocardiogram to look at my heart valves to make sure the bacteria didn't stick there.  On Friday (March 11th), I had a central line removal procedure in Interventional Radiology.  Then, Saturday, I was transferred to OHSU since almost all of my doctors are there.  I continued to receive heavy duty IV antibiotics---they were very hard on my veins. :(  On Monday, I had a venous duplex ultrasound study to look at all the veins in my neck and arms to help the doctors figure out the best central line access.  And on Tuesday the 15th, I had surgery #11 to get a new central line placed.  I got discharged on Wednesday the 16th. :)

I'm weak and exhausted but slowly recovering here at home.  I'm also still on IV antibiotics, and the Infectious Disease doctors will let me know when I can stop them....they are looking at my labs weekly to see if my body is ready to be off antibiotics.  I have a follow-up with them in clinic on April 6th.

Yesterday I saw my GI doctor, and we had lots to discuss.  Several of my liver function labs are high, and he thinks my liver took a hit from the infection.  My bone marrow also got affected, so my blood counts are lower than my normal.  I get labs drawn again tomorrow, and we'll see how things are looking.  My next infection in the future (and it's not "if," it's "when".......infections are a given with long-term IV access. :(  You can be so sterile with your central line and still get sepsis...!), I probably won't be getting another central line.  My GI specialist spoke with the hospitalist, and they both agree I should get a port surgically placed in my chest because it can be less risk for infection.  But, there are pros and cons with a port, just like there are pros and cons with a central line..

Next week, I have 3 appointments with Sleep Medicine, Pain Medicine, and Nephrology.  My nephrologist did discover that my kidneys are spilling magnesium and potassium which is why my blood MG and K levels can't hold steady.  What's going on is similar to something called Fanconi Syndrome (this can be seen with mitochondrial disease).  She's looking into starting me on a medication to help my kidneys hold onto Mg, but it lowers blood pressure.  Since I already deal with low BP, she wants me to get my BP checked for several days (right before I get up from sleeping).  She'll see what my numbers look like, and we'll discuss the next step when I follow-up with her next Thursday.

Happy Easter, friends.  Have a blessed Sunday as we celebrate the Lord's resurrection! :)

Hello, March.

Wow, it's been a whole month since I last posted here.  Did you miss me? ;)

February was very rough, and I'm glad I can now look back on it.

Here are all the latest appointment updates.  Beware, this is super long!!  If seeing doctors was my full time job, I'd be rich. ;)  For those on facebook who already read my "mito" update, just scroll down to pass that one. :)

Mitochondrial medicine (posted Feb. 16th):

1. My hypomagnesemia is related to the mitochondrial disease---a lot of his patients deal with the same thing and need daily IV fluids with magnesium just like me. Still going to be seeing nephrology at OHSU on the 29th.
2. He wants my doctors to aggressively treat the Wilson's Disease and start me on another chelating drug in addition to the Zinc Acetate I'm taking. Unfortunately, there have been no studies showing that WD causes a mitochondrial depletion (which was found on my muscle biopsy). This means treating WD probably won't help improve the mitochondrial symptoms, but he's still interested in how treatment goes!
3. He is going to ask the lab to check my whole exome sequencing analysis (done last year) for Wilson's Disease gene abnormalities.
4. I will be starting Alpha Lipoic Acid in addition to my other mitochondrial co-factors and antioxidants.
5. He thinks that in the next 2-3 years, I'll be eligible for a drug trial. So far, one that he's involved with looks promising.
6. I'm dealing with another painful jejunostomy feeding tube infection... :(  Thankfully I didn't have to go to the ER here! He was able to prescribe antibiotics for me, but he said I need to follow-up with my GI surgeon right when I get back!
7. One more thing, he said he won't forget me and that I'm very memorable! ;) :D

GI Surgery:

I followed up with my GI surgery team the day after I got back from San Diego.  To my surprise, I had to have a small unexpected surgery called "incision and drainage" at the site of my j-tube infection.  It was very painful, and for several days after that, I had to pack the wound with gauze twice a day.  The resident cultured the abscess, and we received results the same day I was in Seattle on the 22nd to see my pain dr.  My cultures grew a bacteria called Enterobacter Colacae Complex, so I was switched to a different antibiotic.  I was on antibiotics for a total of 11 days.  Thankfully, the infection is now gone, but my j-tube site is raw and super painful due to bile leakage.  This has been a problem since November, so my GI surgeon referred me to Wound and Ostomy Care.  Haven't seen them yet, but hopefully soon..

Neuro-Ophthalmology:

The ophthalmoplegia (eye muscle paralysis) in my left eye is stable which is good news. :)  The vision in my right eye is continually getting weaker, though. :(  My right eye is either working too hard because my left eye doesn't move well, or this is due to the copper accumulation from Wilson's Disease..

Pain Medicine:

It was soo good to see my pain dr. at the UW Medical Center on Feb. 22nd.  He gave me a big, long hug!  For those who don't know, he's my favorite doctor out of all. :)  He spent over an hour and a half with me.  From the appointment and neuro exam, he came to the conclusion that I'm currently not dealing with Complex Regional Pain Syndrome.  He thinks I have Small Fiber Polyneuropathy which is very similar to CRPS but also indicates nerve damage.  The plan is to try and switch to a different and new medication....similar to the one I'm on, but he's seen better results.  I'll also be getting another bilateral lumbar sympathetic block, but it will be done here at OHSU.  We talked about Spinal Cord Stimulation, and sadly, he doesn't want to put one in yet because my health isn't stable, I'm on IV nutrition, and I just got over an infection...all big risks.  If I were to get an infection in my spinal cord from the surgery, he would never be able to forgive himself.  I trust him completely and understand, but I AM disappointed....hopefully things look up and I can get one down the road!

Gastroenterology:

I had a GI follow-up after we got back from Seattle, and my dr. ordered lots of blood work to be done to check on all my vitamin levels and minerals since I'm on TPN.  We should get results on Friday.

Hepatology:

My GI dr. spoke with my hepatologist a couple weeks ago.  He said that my hepatologist will be calling sometime soon to tell me that he wants to repeat some Wilson's Disease tests.  He wants to see how the Zinc therapy is going and if it's helping remove copper from my body.

Nephrology: 

I saw my nephrologist for the first time this past Monday, and she is amazing!  So blessed to have her on the team.  She ordered lots of lab tests to be done tomorrow to try and get to the bottom of why I'm dealing with Hypomagnesemia.  I really want to cut back on my IV fluids with magnesium, but that's going to be hard to do if my level keeps dropping.  Hopefully these tests give us some answers.  She's looking into 2 kidney disorders, and I pray I don't have either.  I don't think I can take another diagnosis!!

Pulmonology:

Today I saw my pulmonologist for the first time to assess my weak respiratory muscles.  I had another pulmonary function test, and we compared the results with the one done at Cincinnati Children's.  It wasn't good news.  One of my values showing lung strength decreased from 72 to 46.  That's a big decrease, and this level is seen in chronic respiratory failure. :(  So I have to get another PFT done in 6 months to keep an eye on this.  Taking it one day at a time, and I know I'm in the Lord's hands.  Here is something good---all my other pulmonary tests (including chest x-ray) are normal. :)

Tomorrow I have a follow-up with my hematologist to discuss my latest blood counts.  My GI dr. is glad that this is tomorrow because something is going on with one of my white blood cell types..

After that, I'm done for the week!! :)

Busy busy.

Hey guys,

Just wanted to post a short update for you.  Thank you for continuing to pray for me. :)

My magnesium dropped AGAIN even though I'm receiving extra magnesium in my daily IV fluids!  Something's clearly not right. :(  Yesterday, I was given an additional 250 ml bolus of magnesium, so I was on IV fluids for 6 whole hours.  My GI team referred me to OHSU nephrology to make sure I'm not losing Mg through my kidneys..  The clinic already got back, and I'm blessed to be able to see a Portland Monthly "Top Doctor" in nephrology.  My appointment with her is on the 29th.

Picture #2: what it's like to live with mitochondrial disease....I receive the big clear bag of TPN every other day, the white bag of TPN with lipids on alternating days, and the little IV bag of fluids every day....I have to flush my central line with saline (syringes) before and after every infusion.  All the other supplies in the picture are what's needed every day!

This month is crazy busy.  I have 4 appointments next week (physical therapy, kidney/bladder ultrasound, neuro-ophthalmology, etc), and then my parents and I leave for San Diego on Monday the 15th.  The follow-up with my mitochondrial disease specialist is on the 16th.  We come home on the 18th.

In addition, my parents and I will be driving up to Seattle on the 21st to see my pain dr. who moved from OHSU to the University of Washington Medical Center.  This was planned last minute.  The CRPS nerve pain has been horrendous, and my local pain dr. is so booked out.  It's so hard to deal with this CRPS flare-up day in and day out. :(  Nothing is helping, and chronic pain is so different from acute pain in that regular pain meds don't help.

I can't wait to see my pain dr. again....it's been more than a year!  I need to update him on all that's happened, and I also hope to discuss spinal cord stimulation again (for those who don't know, I had an SCS system implanted in my back in 2011 for the CRPS, and it helped the pain tremendously....I had to have it removed in 2012 because I needed MRIs....after waiting more than 3 years since then, there are now MRI-compatible SCS implants!). :)

After we return from Seattle, I have my GI follow-up that Friday.  I'm glad he'll be back....he's been gone (out of the country) way too long!

And then the following week, I see Nephrology, Pulmonology, and Hematology....too many appointments! :(

The rest of this month, I won't be blogging.  You know why. ;)  I'll be sure to post some short updates on facebook.  And I will return here next month!

Until then!

*Oh man, that was supposed to be a "short" update... :D *

Kerissa

Trekking on.

Hey friends,

Just wanted to share some news---my liver enzymes dropped and are now in the normal range!  My hepatologist thinks I had autoimmune hepatitis which resolved quickly (must be because of all your prayers! :)  Thank you, Lord!!  He really does hear our prayers!  My magnesium level is still not where we would like it to be, but my hepatologist (he's my GI dr. for now until my main GI specialist returns from out of the country) added even more magnesium and 2 different types of potassium to my daily IV fluids which I now receive for 3 hours.

He also called me last week and restarted me on prescription zinc because one of the copper blood tests for Wilson's Disease came back even higher. :(  He explained that high copper in the body compromises liver mitochondria which is not what I need since I already have a mitochondrial depletion.  He plans to talk to a Wilson's expert at Yale and possibly a geneticist in Canada regarding treatment because he's not exactly sure what's the best way to proceed since my case is so complicated.

Sadly, the CRPS pain in both of my hands and feet has flared up a ton, and I'm not sure why.  My feet are swollen and discolored.  It's so painful to even stand or sleep because the bed sheets cause burning pain.  That's how bad CRPS is. :(  My pain dr. is booked out till March, but thankfully, she had a cancellation.  So I now have an appointment scheduled in 2 weeks (it's at 7:30 in the morning!!).  I wish it were even sooner. :/  I took more of my as-needed pain medications to calm the CRPS down, but they worsened my GI dysmotility.  Even super soft food gave me horrible abdominal pain all day. :(

My mom, dad, and I leave for San Diego in exactly 3 weeks!  I'm glad I'll be seeing my mitochondrial specialist---I need to update him on all that's happened!

Picture #1: what it's like to live with mitochondrial disease....these are all my meds and supplements...!

News we didn't want to hear.

Hey friends,

These past two weeks have been super rough....still not feeling well at all. :(  We found out 2 weeks ago that my liver function blood test results quadrupled suddenly which indicates liver damage/inflammation.  The results have improved since then, but one of the liver enzymes is still higher than normal.

I saw my GI specialist last Tuesday, and he expressed that he's worried about down the road.  You never want to hear a doctor tell you that. :(  I've been on IV nutrition for more than a year now, and that definitely doesn't help things liver-wise.  I'm still attempting j-tube feeds, but I can only tolerate 2 teaspoons per hour at the most.

He's not exactly sure what's going on and why my liver suddenly took a turn for the worse...is it from Wilson's Disease....or is it from being on TPN (IV nutrition)....or is this something totally different?

We also found out that my magnesium and potassium levels dropped yet again.  We're not sure why that's happening as well because I continue to receive extra Mg and K in my supplemental IV bag of fluids which is infused through my central line every evening.  Is this liver-related, or is there a problem with my kidneys not being able to hold onto Mg?

There have been lots of questions lately with few answers.  The day after my GI appointment, I saw the hepatologist (liver specialist).  He wants to pursue genetic testing for Wilson's Disease because some specific things related to the diagnosis are puzzling him.  He also ordered lots of lab work to rule out common illnesses and figure out why I'm not feeling normal.  One of the blood tests came back high which indicates that I either have autoimmune hepatitis, chronic active hepatitis, or mononucleosis.  Unfortunately, my GI dr. is currently out of the country for 4 whole weeks, so I'm waiting to hear what the hepatologist thinks about the latest finding.

I wish my GI dr. were here because he knows my situation the best.  At my appointment, he brought a smile to my face when he said he'll be thinking about me on his trip. :)

I'm thankful to the Lord for His mercy and compassion.  He is still God, even when things aren't going well, even when you receive bad test results, even when you don't feel 100%.  He is mighty!!

A rough start to the new year.

All last week, I wasn't feeling my normal and just felt "off."  I kept losing my balance and had nystagmus/dizziness.  This past Saturday, I was feeling so bad with horrible body aches, headache, and nausea, so I ended up going to the ER to make sure I didn't have a central line infection.

The ER docs followed my mitochondrial specialist's ER protocol letter, so I received through my central line a dose of IV carnitine and more than 3 liters of continuous D10 with sodium chloride and potassium.  I also had a ton of labs drawn.  It turns out I was nearing a metabolic alkalosis state (opposite of metabolic acidosis), and I was diagnosed with hypomagnesemia (lower-than-normal levels of magnesium which is an electrolyte).  This happened even though I still receive daily IV fluids with extra magnesium for 2 hours...!

I just had labs drawn with home health 3 days before Saturday which showed some abnormal things, but my magnesium level was fine.  So it dropped quickly.  Hypomagnesemia can be very serious and cause seizures and even cardiac arrest, so my GI dr. is glad I went to the ER to get checked out.

I got admitted to the ER observation unit for 2 nights and received 3 different IV pain medications because the pain was so bad.  I also had to be placed on a continuous cardiac monitor to watch my heart rhythm and high heart rate.  Though short, it was a rough hospital stay---all the IV fluids overwhelmed my neurogenic bladder (and when I'm in a mito crash, body system functions don't work well)...I had to be cathed because I had over 1 liter in my bladder which I couldn't empty. :(  TMI, but mito is no fun. :(

To correct the low magnesium, the doctors gave me 4 grams of IV magnesium.  That brought the levels back up, but they soon dropped again.  So I had another magnesium infusion early Monday morning before I got discharged.

My GI dr. thinks the prescription zinc I was taking 3x a day for Wilson's disease caused my body to get depleted of magnesium.  But, if my MG levels start dropping again while off of zinc, then he said something else is going on..  I had weekly labs drawn yesterday.

This coming Tuesday, I have a monthly follow-up with my GI dr.  Wednesday, I finally see the hepatologist (liver specialist) after waiting 2 long months.  And Thursday, I have an appointment with my neuro-urologist.  It's gonna be busy! :/

I wasn't expecting a hospital stay right at the beginning of the new year, but the bright side is, I went without an ER visit for almost 11 months which is a record! ;)  I'm thankful to the Lord for His protection this past weekend.  My condition could have been a lot worse if my very low MG wasn't corrected!

Reflections on this past year 2015

This year alone, I had a total of:

• 51 doctor appointments
• 21 physical therapy and occupational therapy appointments
• 5 MRIs (brain, cervical spine, thoracic spine, foot, and heart)
• 2 more surgeries (central line placement and liver biopsy) which makes a total of 9 so far
• 2 overnight hospital admissions
• 1 blood clot in my right cephalic vein
• 14 days of blood thinner injections
• 52 home care nurse visits
• 52 central line dressing site changes
• 3 sleep studies which showed worsening sleep apnea
• 1 skin biopsy
• 2 trips to San Diego to see my mitochondrial specialist in January and July
• 2 genetic tests (whole exome sequencing and a periodic fever syndrome panel)
• More than 150 tubes of blood drawn (thankfully, most of the blood draws were through my central line, so I didn't have to get poked for all of them :)
• 5 weeks of IV iron infusions for iron deficiency anemia
• 2 ER visits
• 2 electroencephalograms in June and August
• 1 pulmonary function test
• 1 modified barium swallow study/esophagram which showed esophageal dysmotility
• 2 EKGs
• 2 new medical diagnoses (Mitochondrial DNA Depletion Syndrome and Wilson's Disease)
• 1 jejunostomy feeding tube infection
• 2 multi-organ abdominal ultrasounds and 1 venous duplex ultrasound study
• 4 "24-hour" urine tests to check histamine and copper
• And more than 365 hook-ups to IV nutrition and IV fluids

I look at all these numbers, and I'm humbled by God's grace---I truly couldn't have made it through another hard year without the Lord's help.  He has been my Rock!  I'm reminded of the verse in 2 Corinthians 12---But He said to me, "My grace is sufficient for you, for my power is made perfect in weakness."  I was weak many times this past year, but the Lord made me strong (2 Cor. 12:10).

2016 is upon us...  It's always a little hard when a new year begins because of the unknown, but I know the Lord will give me courage to face whatever comes next!  Plus, He blesses me with such loving and supportive friends and family. :)  Thank you for staying on this journey with me!  I couldn't do this without you!

Happy new year, friends.  May we hold fast to the Lord and trust Him in everything these next 12 months---He is faithful!!

Saw this quote recently and wanted to share.  Perfect as we begin 2016!

Another diagnosis (and hopefully my last..)

Hello friends,

For those who didn't hear, my grandma passed away the day after Thanksgiving.  We had a beautiful service for her this past Saturday.  We all miss her so much. :'(  It sure is different not to have any more grandparents living.

On November 20th, my GI dr. called, and I got diagnosed with Wilson's Disease, also known as hepatolenticular degeneration.  He thought WD very unlikely, but you know me, I'm a puzzle. Wilson's disease is a rare, genetic copper overload disorder where the body lacks or has an abnormal copper transport protein due to two mutations.  This causes copper to build up in high amounts in the brain, eyes, liver, kidneys, and other organs.  The excessive copper causes damage (hepatic and neurological) and even failure of these organs if not diagnosed early enough.

The normal copper content in the liver is 10-35 mcg...my liver biopsy copper result from Mayo was 934 mcg....!  That's extremely high. :(  Also, my liver biopsy histological findings, though mild, are consistent with WD.

My GI dr. referred me to a hepatologist (liver specialist) for input on treatment, but unfortunately, this specific dr. is booked out until January 13th.  I do see my GI dr. this coming Monday, and we'll talk more about everything.

I don't know why I'm the one to end up with all these mutations and rare diseases (especially since, as far as we know, there's no past family history of these genetic disorders), but I won't stop trusting the Lord.  I am in His hands, and I know that the Lord will turn even this into good.

I do hope this is the last piece to the puzzle!  I'm not sure how many more diagnoses I can take.. :/  One of my friends told me I'm an age 18+ 20,000 piece jigsaw puzzle. lol  Mia, you always make me laugh. :)

Wilson's disease is known to cause mitochondrial dysfunction, so my doctors are wondering if this is a separate issue or related to my Mitochondrial DNA Depletion Syndrome.  I will ask my mito specialist when I see him in February. :)

Gratitude...a precious offering.

Hi everyone,

Just wanted to let you know that things medically are going ok with me, but my grandma (my only remaining grandparent) is not doing well at all and is in the last days of her life due to congestive heart failure. :(  It's been so hard to see her decline...the hospice nurse thought maybe she had 1-3 days left, but our grandma is a fighter and is still hanging on even though she has not had food or liquids since last Monday the 16th. :'(  She's in a drugged-up state and cannot talk or swallow, so the liquid morphine has to be rubbed around in her mouth.

I did receive my liver biopsy copper results back from the Mayo clinic last Friday.  My GI dr. called me, and it's not good news.  But I'll share about that another time.

Have a blessed Thanksgiving, friends.  Hold your loved ones close as things can change in the blink of an eye.

Love,
Kerissa

Pathology results

Hey friends,

My liver biopsy last Friday went very well!  Thank you so much for praying!  The doctors were able to obtain two specimens without complication.  After the biopsy, though, my kidneys took a hit that weekend, and I suddenly gained 4 lbs. of fluid. :(  We weren't sure what was going on and if this was a new symptom related to the mitochondrial disease.  Thankfully, they slowly recovered and I'm getting rid of the fluid!  My local mito specialist whom I saw on Tuesday suspects this was caused by the one of the drugs used for sedation during the biopsy.

Yesterday, my GI dr. called me with the initial liver pathology results (the copper results from Mayo aren't back yet).  Some mild inflammation was found in my liver but no sign of cirrhosis, hepatitis, etc.  That's good news!  He thinks the inflammation is from TPN (IV nutrition can damage the liver which is why it's not ideal).  Speaking of TPN, next month is one whole year of being on it!  Hard to believe.  I know there are big risks and it's only used as a last resort as my GI dr. states, but lots of blessings have also come as a result of TPN---I haven't had an ER visit for a "mito crash" in over 8 months (I used to have to go every 2-3 months!).  The IV fluids, amino acids, extra vitamins/minerals in the TPN have helped so much.  The nutrition has also prevented all the coughing/retching I used to deal with every single night due to a weakened esophagus.  It was horrible, and I hardly got any rest.  So I'm very thankful that went away. :)

Still working hard in physical therapy every week.  My mito specialist at Randall Children's thinks my "foot drop" (a neurological symptom) has improved somewhat. :)

This coming Monday I have another follow-up with my GI dr.  Hoping the Mayo results will be back.  I'm dying to know my liver copper level!  It never gets any easier waiting for results.. :(

Every single Wednesday, this huge stack of boxes gets delivered to our door.  I wonder what the UPS delivery guy thinks! haha ;)  The boxes contain all my IV nutrition, IV fluids, and central line supplies!

Liver biopsy

Hey guys,

Just wanted to let you know that my liver biopsy is scheduled!  My GI dr. put the order in as "urgent," so it's for this Friday the 6th.

This specific dr. performs complex image-guided biopsies.  He's only 13 years older than me (wow!), and yet, he's a Portland Monthly "Top Doctor." :)  Very thankful I'll be in good hands.  And in the Lord's!  A piece of my liver will be sent off to Mayo, and we should we getting results back the following week.

In addition to receiving TPN (IV nutrition) for 16 hours every night, I also now get infused with IV fluids every day for 2 hours.  *See pic below*  It's a cute little bag! ;)  It has extra sodium, potassium, and magnesium to try and raise my low blood pressure and prevent the painful muscle cramping I experience.

I will let you know what the biopsy results show!  I also see my mitochondrial neurologist at Randall Children's next Tuesday and will be sure to update on that as well.

Thank you so much for your prayers! :)

__________________

P.S. I thought this was so neat and wanted to share!  OHSU's fall newsletter came in the mail today, and this was the featured story. :)

Upcoming

Hey friends,

Here are the latest appointment updates for you. :)

Rheumatology--- The new rheumatologist I saw at OHSU is very nice!  She's not 100% sure that I have the periodic fever syndrome "Familial Mediterranean Fever" because I only have one mutation versus two (FMF is recessive).  But, 30% of patients with FMF only have one identifiable mutation anyways.  Soo, regardless of the genetic results, she's starting me on the standard FMF treatment to see if we can get these fevers to go away, and I'm to monitor my symptoms while on the medication.  It's a very small dose, but I started experiencing lots of achy muscle pain in my back, so I have to email my dr. about it.. :/

Gastroenterology--- I'm still dealing with horrible "charlie-horses" (muscle spasms).  I don't cry, but tears automatically come out of my eyes because the pain is disabling! :(  And they last minutes rather than seconds.  My GI dr. is adding more magnesium and potassium to my IV nutrition to see if this will reduce the frequency of them.  In other news, we are going forward with the liver biopsy to know for sure whether I have the copper overload disorder Wilson's Disease.  He is going to speak with my GI surgeon to see if she can do it.  If not, then interventional radiology will be doing the surgery.  Currently waiting to hear what the plan is.

Sleep Medicine--- Using BiPAP is going very well!  I now have less than 5 apnea episodes per hour (it used to be an average of 17 episodes per hour), and anything less than 5 is good!  I have been experiencing really bad nasal congestion at night, so my sleep dr. recommended I turn up the humidification settings on my machine.

P.S. I'd appreciate prayer for my grandma.  Her health has declined the last two weeks due to worsening congestive heart failure.  She's now under hospice care.  It's really hard to see her go through this, and she's my only grandparent left. :(  Pray that my family will be an encouragement and support to her in any way during this time.

Such an encouraging quote!

Thoughts on waiting.

Hey friends,

Tomorrow, rheumatology is getting me in for the Periodic Fever Syndrome, and I'm so thankful to the Lord that it's much sooner than January. :)  The appointment is at 1 PM.  Please pray that this specific dr. will be able to start treating it.  The numerous fevers are hard on my body, and I've been dealing with episodes of joint pain and inflammation since August.

I received my repeat 24 hr. copper test results, and sadly, the level increased from 77 to 106 in just a month (normal range is 3-50).  We have lots to discuss with my GI dr..  I see him this coming Monday.  I also have a follow-up with my sleep medicine specialist next week.

I will update after those appointments!

To end, I recently came across this little "devotional" I wrote years ago.  I can't remember if the homework assignment was to paraphrase a passage or an original composition.  I don't even recall when I wrote it (my bad for not putting a date!)...maybe it was after being newly diagnosed with Complex Regional Pain Syndrome (aka Reflex Sympathetic Dystrophy) or when I started dealing with all my other numerous rare diseases.  Nevertheless, I wanted to share it in hopes that it encourages you. :)

The Hardest Thing to Do

by Kerissa

          Wait.  Sometimes, this word can be the hardest to accept when in times of trial or tribulation.  There will be times when we ask with little faith, "Why do I have to go through this?" or "God, why am I suffering?"  What can be done about these questions when one is anxious for answers or does not know what path to take?

          Although waiting on God and humbling one's self can be the hardest thing to do, God states in His Word that His "plans...stand firm forever, the purposes of his heart through all generations."  We should not worry about anything because He always intercedes at the right time.  Psalms 27:14 says, "Wait for the Lord; be strong and take heart and wait for the Lord."  Complaining like the children of Israel did against Moses will do nothing.  Waiting in quiet patience and faith, even when under affliction, will bless God and make Him proud.

          If we accept our difficulties with humbleness, we will be able to say with confidence, "Now, Lord, not my will, but Yours be done.  I do not know what to do.  But I will wait until You drive back my foes.  I will wait, for my heart is fixed on You alone, O God, and my spirit waits for You in the full conviction that You will be my joy and my salvation, my refuge and my strong tower." ~Charles Spurqeon

          So wait on the Lord.  He really does hear our cries and prayers.  Know that God is always by and on our side.  In the end, we will all be able to understand why God placed those terrible thorns in our path, and someday we will thank God for the word "wait" in each of our lives.

Dealing with infection.

Oh I just can't catch a break. :(

Last week, I had to say goodbye to my home care nurse. :(  She changed my central line dressing site and drew labs at my house every single week for 9 months.  Long story short, the home health agency she worked with got bought by a huge company.  She didn't really like all changes involved, so she found a new job.  I'm going to miss her!  She was a very caring and compassionate nurse!

For the past week or so, I started experiencing severe pain, intestinal spasms, and other symptoms near my jejunostomy feeding tube.  The pain's been so bad that I wanted to go to the ER on Tuesday!  Thankfully, I was able to avoid going because my GI surgeon squeezed me in this past Wednesday.  She thinks I have an infection because the whole area is inflamed and bled a lot.  That would explain why I haven't been feeling super great lately.  She had to do a "needle and drainage" procedure right then.  That was so painful, and the needle was huge!  I've been started on 3 days of antibiotics.  If things don't improve soon, she wants to see me back.  She'll order more imaging like an abdominal ultrasound to look into this further.  Praying this gets better because the pain is horrible! :(

I had a total of 5 appointments this week.  Saw Cardiology, and my dr. is pleased to hear that the additional sodium to my TPN helps reduce the dizziness and high rates.  Still haven't had a chance yet to dissolve sodium chloride tablets and flush them through my tube, but hopefully next week!  I see him again in 6 months.

I met my new home care nurse yesterday afternoon.  And so far I like her---she's very easy going and laid-back! :)

The Periodic Fever Syndrome mutation I have is related to a rare auto-inflammatory disorder called Familiar Mediterranean Fever.  And the funny thing is, I'm not Mediterranean. lol  Basically, the gene MEFV is defective and can't regulate inflammation.  My rheumatologist spoke with his colleague to see if she can get me in sooner than January.  She said she'll look at her schedule and try to get me in asap.

I received a copy of my continuous video EEG report, and my neurologist noted that I have diffuse cerebral dysfunction and diffuse "slowing"...  Sounds lovely, right? ;)  Just another finding to add to my long list..

Continuing to rely on the Lord for His help because there's nothing else I CAN do.  He is my rock and fortress!

Unexpected

Hello friends,

Yesterday, I received some unexpected news.  A few months back, my hematologist referred me to rheumatology to get their input on why I'm still having persistent fevers.  I saw them end of July, and the doctor ordered an extensive genetic panel to test for all 7 Periodic Fever Syndromes that there currently are.  It was completed through the same lab that did my Whole Exome Sequencing.  Well, yesterday, my rheumatologist's office called and told me that a mutation was found.  *sigh*  I can't believe my doctors continue to find more and more mutations in my DNA. :( 

Both Hematology and Rheumatology thought it was unlikely that I have a Periodic Fever Syndrome to add to my long list of conditions, but you know me....I'm rare.  Mitochondrial disease is rare.  Ehlers-Danlos Syndrome is rare.  Complex Regional Pain Syndrome is rare.  Dysautonomia is rare.  And before all this, I never heard of any of these disorders!

My rheumatologist didn't tell me what exact PFS I have, but they are sending me the report.  He referred me to his colleague who specializes in the disorders.  Now, here's the sad part---this specific dr. is booked out until January...! :(  So, more waiting to do..  I was placed on her cancellation list in case any of her other patients cancel.

Still processing all of this.  But I know that the Lord has a good and perfect plan behind each and every mutation.  I know that I am fearfully and wonderfully made (Psalm 139). :)  One day I will have an answer to all of my questions!  But until then, I will continue to serve my Savior in any way I can. :)

On top of all this, we found out that my repeat 24 hr. copper results are still elevated.  This week, my GI dr. spoke with another copper expert at Yale.  He suggested we do a third 24 hr. copper test (hopefully my last!) the beginning of October..  If the results have dropped, then Wilson's Disease is more unlikely..  If they're still high, I guess we go from there..  I just want to rule it out once and for all!  And if I do have Wilson's, I'd like to begin treatment quickly before my brain/liver gets damaged..

Not much else happening. ;)  Still doing weekly physical therapy and occupational hand therapy.  I'm thankful for my therapists who work with me so much!

Once I receive a copy of the PFS results or hear back from my rheumatologist (I sent him a message today to get more info), I will let you know! :)  Thank you for continuing to pray!  It means a lot to me!

"But for you who fear my name, the sun of righteousness shall rise with healing in its wings..." ~Malachi 4:2

Repeat copper test.

Here are some updates for you all. :)  Thank you so much for continuing to pray!

The past two weeks, I started experiencing sharp pain in the ball of my right foot---it became very painful to bear weight on it.  I saw Orthopaedics, and the doctors think I'm dealing with something called metatarsalgia which is pain, swelling, and inflammation of the metatarsal bones/joints in the foot.  Thankfully, it's improving!  Ice and rest has helped. :)  The doctors also referred me back to occupational hand therapy because my right hand has been cramping up a lot again, even when I wear my splint that the therapist made for me.  I see them next week after physical therapy.  My left hip/leg muscles have also "shortened" a ton which is causing severe charlie-horses (muscle spasms) and an inflamed sciatic nerve.  So my therapist is working on this.

Using BiPAP at night has been going so well!  I'm tolerating it much better than CPAP. :)  I have a follow-up with my sleep dr. next month.

My neurologist called me yesterday with my overnight video EEG results...thankfully, no sign of seizures!  I'm glad I don't have to add another problem to my plate...the jerking I deal with daily is non-epileptic. :)  She said she still saw brain wave "slowing," but it's not constant which is a good thing.

On Tuesday, I saw my GI specialist.  We discussed how my 24 hr. copper results came back very high. :(  He wants to repeat the test, and if it's high again, he said we have to get a liver biopsy done. :/  He doesn't want me to have to go through that, but if the 24 hr. copper results are high this week, it's a big indication that I probably have Wilson's Disease, a genetic copper metabolism disorder that causes copper build-up in the body and damages the brain, liver, and other organs.  We'll hopefully get results next week..

The GI dysmotility has been really rough lately. :(  Soft food I eat is not moving well down my esophagus, and the abdominal distention and pain is lasting longer each day.

I've been on TPN (IV nutrition) for more than 8 months now..

I wanted to share a few pictures I found on ThriveRX's facebook page (a company that makes TPN).  I thought it was cool how TPN is put together!

Everything has to be sterile because TPN goes directly into the bloodstream.  This reduces the chance for sepsis (infection).