Adjusting to TPN

Hey everyone!

My GI appointment on the 5th went well. :)  As my GI stated, TPN is a blessing and curse.  The abdominal pain, distention, and nausea has been nothing like before, and I'm so grateful to the Lord!

Oral eating is recreational now rather than a necessity, so my small intestine has been getting better rest than before.  But, I still have hypoactive abdominal sounds which is not very good.  Also, as I mentioned in the past, we always have to be on the watch for infection since my PICC line goes towards my heart.

1 1/2 liters of TPN fluids and lipids every 12 hours has been hard on my neurogenic bladder, but aside from that, I've been tolerating TPN well. :)

Every single time I shower, I have to cover my arm in saran wrap and tape!

All the TPN and lipids that get delivered weekly...they have to be refrigerated.

  
Haven't noticed any extra energy still, and I've been sleeping even more---TPN can destroy red blood cells, and some of my blood counts have been lower than normal which could explain the worsening fatigue, so my GI is watching that.

I saw the pain team on the 6th for the CRPS around the PICC line area.  The slightest touch caused so much nerve pain in my arm!  So my pain dr. added yet another oral pain medication and ointment to my list of meds.  They have helped a lot! :)  It is a win/lose situation, though, because pain medications make GI motility worse.. :/

This past Tuesday, I had physical therapy, and yesterday was a follow-up with my PCP.  She is referring me to ENT for my lingering cough/swallowing issues.  Today, I saw an orthopaedic foot dr. regarding the lymphangioma (vascular tumor) that was in my right foot back in 2010.  He ordered an ankle/foot MRI with and without contrast to check for/exclude recurring tumor.  This will be done after my parents and I get back from San Diego.

Speaking of San Diego, it's finally here!  I have a follow-up with my mitochondrial specialist to go over all the results/tests on January 20th.  My parents and I leave on Monday the 19th and come home on Thursday the 22nd---a quick trip!  Please pray that the dr. will have wisdom and insight regarding all the results.  My case is very complicated, and more than one thing is going on which makes things so tricky....I won't be surprised if he has to order more genetic testing.. :(

Thank you all for your faithful prayers!

Kerissa

A New Year.

Happy new year, everyone!! :)  I'm sorry I never wrote my usual last-day-of-the-year "reflections" post.  I was admitted to the hospital the day after Christmas and got discharged this past Tuesday, so I didn't quite feel up to blogging.

The jejunostomy tube feeds trial failed on Saturday---I have very slow small intestinal motility and hypoactive abdominal sounds. :(  It causes terrible abdominal pain, distention, and nausea.  The IV team came by Sunday to place a PICC line (peripherally inserted central catheter) in my upper right arm.  And Sunday night, the doctors started TPN (IV nutrition) very slowly to bypass my GI tract.

Once I got home, 3 different TPN nurses from home health came by Tuesday, Wednesday, and Friday to teach me and my mom how to administer and hook up to TPN.  They also drew blood work (which my GI specialist wants completed weekly) to monitor all my electrolytes, triglycerides, etc.  I also got my PICC line dressing changed twice---a nurse will be coming once a week to do this as well.

The CRPS has been flaring up a ton in my right arm from all the trauma. :(  Dressing changes are so painful with all the tape removal, etc!  So my pain dr. will be squeezing me in soon to try and figure out what to do about this.  I can't use my arm well, and the slightest touch causes severe nerve pain. :(

TPN initiation has been a big step.  I see my GI specialist this Monday to go over everything and discuss continued tube feeds trials as it's not good to stay on TPN long-term.  And for the first time ever, the hospitalist gave me and my family a serious talk about disease progression, prognosis, and how I need to get my PCP more involved because of all these changes.  It was different hearing that for the first time..

Yes, I have been and will be facing a lot of unknowns in the future, but the Lord is Sovereign!  Since 2010, each year has been getting more and more difficult as you can see, but that's the beauty of a NEW YEAR. :)  I look back on the previous year, and it always gives me so much hope, knowing that a gracious and faithful God will continue to carry me and my family through these ongoing challenges and trials.

It's already 3 days into 2015! :)  I've shared this before because it's my favorite verse!  "...it is my eager expectation and hope that I will not be at all ashamed, but that with full courage now as always Christ will be honored in my body, whether by life or by death.  For to me to live is Christ, and to die is gain "(Philippians 1:20-21).

Getting admitted this week.

Hey everyone,

I just wanted to let you know that I will be getting admitted the day after Christmas.  I'm very thankful for my GI dr.---he's working at the hospital and not in clinic all this week, but he arranged and coordinated everything so quickly!  I'm not doing very well.  The pro-motility drug I've been taking hasn't helped at all, and it's getting harder to tolerate oral intake.  All my back muscles have been getting weaker and forming tight, painful knots to compensate---my physical therapist said it's because my muscles aren't receiving enough nutrition.  I've had to sleep past 2 pm every day now because I've been so tired.  All this and more is going on, and yet, I look so normal on the outside!

On top of all that, I still cough and gag all day and night.  We wonder if my secretions are going down the wrong way and ending up in my lungs.  I got the overnight oxygen testing completed last week, but we won't get results at the moment because my sleep dr. is out of town these last two weeks of December.

We're not sure how long I'll be admitted.  But I'll keep you posted!  Everything is up in the air regarding how long I'll be on TPN (IV nutrition), etc.

I hope all of you have a blessed Christmas!  I'm so thankful for you and your steadfast prayers.

P.S.  I thought this was so neat!  Last week, CNN wrote an article, and here is part of what they said: "We know parents whose children are in remission from cancer and others whose children have mitochondrial disease. Their dream present would be an end to childhood disease. Give to their causes if your family is lucky enough to be untouched by serious illness."

More tests and referrals.

Hi everyone,

We thought this week would have been my scheduled admission to the hospital since I haven't tolerated glutamine, but we're trying one last thing to re-mobilize my small intestine.  My GI specialist really wants to avoid TPN (he recently told us he has two critical patients in the hospital because of sepsis from being on IV nutrition).

I'm to take an antibiotic at a low dose for 10 days---this specific antibiotic can help the motility of the small intestine.  It doesn't benefit everyone, but he just wants to exhaust all options.  If I don't notice anything, then the plan is to get admitted to do a tube feeds trial and/or TPN (but it's always possible he may think of another option..).  I'm still losing weight and feeling bad, so 10 days seems so long. :(  I just need to keep waiting on the Lord.  I know His timing is perfect.

Still gagging and coughing, too, especially at night.  It wakes me up from sleeping. :(  Very thankful for my sleep dr.  He even emails me back on the weekends or late at night (btw, he made Portland Monthly's Top Doctors list for 2015 again! :)  He will be referring me to Pulmonology and also ordered overnight oxygen testing which will be hooked to my CPAP and done this week.

Today I saw my physical medicine specialist because I've been dealing with something called "foot drop" (can be seen with many neurological disorders).  It makes me trip when I walk around in our house.  He ordered an ankle-foot brace which is similar to the kinesio tape that my physical therapist used on me in the past.  It helped a lot, and I look forward to using the AFO brace!

My dr. also referred me to his colleague who is a foot and ankle surgeon.  Not planning to have foot surgery, but I'm not sure if the lymphangioma in the bottom of my foot (I had it surgically removed in 2010) grew back because the "bump" looks bigger to me and hurts a lot.  Dr. C isn't sure what type of MRI would be good to order for this specific problem, so he is referring me.  Lots of appointments in the future! :/

Worsening problems.

Hey friends,

I'd appreciate your continued prayers!  My small intestine is in bad shape.  Still can't tolerate j-tube feeds or solid food orally.  And I'm now getting distended with just liquids... :(  The dysmotility in my small intestine has gotten so much worse.  Mitochondrial disease is horrible!  I'm being followed very closely by my GI dr.  We've been trying a lot of different things, but none have been helping.  He wants to exhaust all other options before we move on to TPN (total parenteral IV nutrition) which is always a last resort because it comes with many risks and complications, including sepsis (blood infections).

I'm to try an amino acid called glutamine for 4 days to see if it will help the mitochondria in my GI tract.  And we're also trying another tube feeds formula since the elemental one wasn't tolerated well last week.

If these last things don't help, then we're moving on to TPN.  I'll have to get a central line placed and be admitted for the start of it for monitoring, daily lab work, and hourly blood sugar checks.

I'm so thankful for my GI specialist!  He's even trying to get in touch with my mito specialist in San Diego.

Today I saw my pain dr.'s colleague, and she is so sweet! :)  I'm so glad she can keep in touch with him.  The whole pain center misses him a lot!  If I have to start TPN, I'll be seeing the inpatient pain service at OHSU to receive ketamine infusions for CRPS pain control.  The CRPS has been flaring up because I'm not getting enough nutrition.  I'm also dealing with daily headaches and low blood pressure again from lack of food..

On top of all this, two weeks ago I started dealing with forceful coughing bouts to the point that I gag and retch. :/  I'm not sick, though.  We're not sure if something lung-related is going on now.  See how bad mito is? :(  I emailed my sleep dr. today to see if he can refer me to pulmonology.

Next week, I will be getting my j-tube changed yet again to a different-sized mic-key button.  And I also have a follow-up with cardiology to go over the holter monitor results.

I continue to press on---the Lord's got this! :)  And like always, He carries me through the roughest of times.

Good news and bad news.

Hey friends,

We got the CT scan results yesterday!  The images showed that I had something called Buried Bumper Syndrome (a rare complication with feeding tubes)....basically, the balloon on the j-tube (which is inflated with 1 ml of water to hold it in place) got stuck in my abdominal muscle.. :-O  No wonder I am dealing with pain and spasms!  So my GI surgeon's office got me in that afternoon.  I've been having appointments every day....I'm tired to say the least.  My surgeon pulled the tube out and replaced it with a temporary balloon-less tube to let the area heal.  And she'll later switch it to a different size in 3 weeks.  We're still not sure if this will resolve all issues as my GI specialist said there may be more than one thing going on, including the motility issue.  But at least we can take care of this specific problem now.

Because I'm losing weight again, my GI dr. is going ahead and starting me on an elemental tube feeds formula which is more broken down and usually easier for patients with dysmotility to tolerate.  I see him again in 12 days!

Today I had urodynamic testing (super thankful that's out of the way!), and the results aren't good..  My nervous system is not working well at all, and she thinks this is from a cerebellum issue and/or a spinal cord problem (which my mitochondrial specialist suspects as well when I saw him back in June).  My specific bladder issue is hard to treat, but she is starting me on a medication....sometimes it works and sometimes it doesn't.  If it doesn't help, she'll re-evaluate and go up the ladder from least invasive to invasive.

Now for some good news---more of my muscle was found which means the mitochondrial DNA testing can be started as soon as my insurance approves it! :)  If all that goes well, I have a tentative appointment with my mito specialist in San Diego on January 20th!

The first week of December, I have GI, an appt. with my pain dr.'s colleague, and physical therapy.  So I will be enjoying next week off! :)

Have a blessed Thanksgiving next week!

Love,
Kerissa