In case you don’t know what manometry means – let me tell you what a few synonyms for it are:
And I know its not that bad for everyone. But it was my worst fear going into this test – that it would be so overwhelmingly painful for Kendall, and indeed, it was. I am not going to go into a whole lot of details about that day simply because it was truly one of the most horrific things I have ever watched her have to deal with. I did not even take pictures of it because I never want her to remember the horrifying details. Basically they removed her GJ-tube/button that is always in her tummy, and replaced it with a tube containing 8 smaller leads/wires. They also attempted to place a foley catheter in the same stoma, and none of the nurses could do it, so the doctor said “let me try to jam it in.” Which indeed he was successful at. These wires threaded down her stomach into her intestines similar to the path her GJ tube takes, except it extended just the slightest bit past where her tube goes. Horrifically, a similar tube was also placed up her rectum and threaded to the junction where her large and small intestine meet. This sounds like no big deal until you watch almost 4 feet of thick tubing get pulled back OUT at the end of the test. I just about threw up. She had more tubing in her than she is tall.
On top of all this funness, she was grumpy because it took enough anesthetic to knock her out to put an adult to sleep. Typically she is able to get morphine coming out of anesthesia to help combat the grumpiness, but for this test, she could have no meds that might interefere with her motility. No morphine, no tylenol, no ibuprofen. No Zofran for nausea, no venting of her tummy for pain relief, not even numbing cream around her stretched out stoma. She couldn’t even keep her eyes focused in front of her at the beginning due to the pain and nausea. She could only give me glares every few minutes in between arched back screaming fits. We had to try to convince her to lay down/lay still during the entire test (supposed to be about 4-5), and she was not having it. She was just grunting/screaming in sheer pain. At one point I just finally laid down on top of her, we both had our heads buried in pillows crying – she crying AT me, I crying FOR her. After an hour, she had finally calmed enough to stay laying down or at least reclined enough for one of the nurses to step in and distract her, while the other one pulled me away and sent me down the hall to regain my composure.
At one point almost five hours into the test, we had still not seen any kind of good pattern, contraction or rhythm on the test. It was very tense in our room. We had given the first medicine meant to induce stomach contractions and it was not working. The second had just been administered, Kendall was finally just about to pass out from the pain, and it happened. One semi-weak, but rhythmic contraction. The nurses cheered, paged the doctor who came right in and looked at it and had a half-grin on his face. We NEEDED that contraction he told me. Three hours later, the test was complete. Three more hours of horrific screaming that had people on our floor shutting their doors and avoiding our room like the plague. They gave her a small bolus of her formula into her stomach and her jejunum, and then gave an enema from the top of her colon down and we waited again…..and FINALLY saw/heard/witnessed the five contractions we needed from her lower colon (meaning that she pooped! luckily it was just water, since she had a $50,000 tube blocking the exit…gag!)
The initial results we received in the one hour meeting we had with the doctor leave us with much hope. She CAN have normal function. She does not right now, and the normal function we did see obviously came at a very high price (her pain and nausea levels are not sustainable right now for the payoff of still very weak and too few contractions). But we were able to eliminate a lot of bad stuff from the table right now (transplant, organ failure, surgical anatomical changes to her GI tract, ostomies, among others). In my heart, I always knew Kendall still had SOME function. I have never once given up on her gut. It has been horrid and crappy from the start of her little life, but i have always known that it was still working on some level. Even on the TPN, we have retained some very good function. She is able to get about 1/4th of her nutritional and fluid needs met with the J-feeds we are able to run (well, at least half of the time…). So the results are not shocking to me that there is the capability of normal function. She does not have an inherent motility disease – meaning that the nerves inside the GI tract and the muscles of the GI tract CAN contract in an appropriate fashion and play nice together – IF the right environment for such is created for them.
She does, however, have an outside or secondary neuropathy. The nerves from her GI muscles to her brain are not communicating in an appropriate fashion. They are misfiring at times (leading to the increased pain or the reverse motility patterns we saw more often than not), or not firing at all at other times (leading to the times when everything that we are able to get into her gut comes pouring right back out any exit hole it can find). This is believed to be due to her autonomic dysfunction, which is a part of the larger (quite poorly understood) connection to the mitochondrial disease. Our motility doctor was not comfortable giving us an overall picture of what to expect from her gut simply because he is not a mito doctor or even a genetics doctor – he is a motility doctor. and right now, we CAN get some motility. What I find to be ironic is that these are the exact nerves that we get corrected by our family chiropractor every week. And while I know some people’s thoughts on chiropractic range from very positive to thinking they are just this side of quacks, for us, it works. I have seen it help countless times. I know that the limited function we ARE able to see is because Kendall’s nerve pathways are constantly being reset back to as good as we can get them. I shudder to think of how bad her testing or her function in general would be without the benefit of direct therapy to those nerves each and every week of her life. Because while we got SOME good contractions – it was essentially less than five minutes worth. Out of almost 8 hours of testing and stimulating and recording and watching….five minutes. But we will cling to the hope that those five minutes offer, and pray that we can turn it into ten. And then fifteen. And then an hour.
Essentially – the capability for normal function is there, but the pain it causes to reach that function are extremely ABnormal.
It will not come easily.
But if, in the end, it gets Kendall off the TPN and helps relieve her pain and lets her LIVE her life again, it will be worth it. A team of Kendall’s doctors from Milwaukee conferenced me in on a call yesterday where we came up with Phase 1 of our plan. We will be trialing a few different medications in an effort to help slowly stimulate those same contraction patterns we did see. We are doing the trials in a very methodical and controlled manner, which I appreciate. I hate nothing more than when doctors just throw meds around at symptoms and you never know the real story about what is helping what or what isn’t working about a particular plan. Especially with the ramifications of what happens if we CAN’T find a good medication option, I want to know that we have given each and every one a good hard look at what is working and what isn’t. Once we have ramped up the first medication and kept her steady on feeds, we will add in a second one slowly. If those go ok, we will begin advancing her rate of feedings very slowly and taking nutrients out of her TPN very slowly. The impatient side of me (ok that’s pretty much the only side there is to me) wants to just stop TPN next week and turn up feeds and beg and plead with her body to just please please please work.
The way it was explained to me though is that what we are trying to do is work around a bad nerve pathway that has been created. If we change things too quickly, we risk really pissing those nerves off even more, and losing any ground we have been able to gain or maintain. Right now, she is on a good enough rate of enteral feeds that we have been able to really protect her liver from having to do ALL of the work. (Liver failure is one of the biggest and most real risks of TPN as a long-term nutrition solution.) If we lose any of that ground, it would be devastating to her system. So everyone else (besides me) wants to move very slowly on this. I think the biggest shock to me about how serious they are about going slowly is that one of her doctors on the conference call who has been very “upbeat” about kendall’s issues (brushing off some of my concerns, telling me she’s way too healthy for this that or the other thing, etc.) – was one of the biggest voices in the room on how slowly we will go with this, how we aren’t looking at getting rid of the line for many many months, if indeed she ever is able to get rid of one. So while the situation overall is very much full of hope and ambition, there is still a very long road to “success”. And it will be a bumpy road. And it will probably have setbacks. And at the end of the day, it still might not totally work.
But we HOPE it will. And our HOPE lies in the Author of all HOPE. And as has always been our view on this journey with our strong leader in the valley,
“Hope is a good thing – maybe the best of things. And no good thing ever dies. I hope…I hope…I hope.”
So in spite of the absolute horrific trauma that the testing was, we are so very grateful for the awesome team of doctors we have, who can interpret this information into a real and hopeful plan for rehabilitating Kendall’s GI tract, and allowing her a chance to get off the TPN, if even for a little while. As Dr. S (the motility doctor) put it to me – without that one good contraction we saw, you and I would be having a VERY different conversation right now, one that I hope we never have to have. So we rejoice that her system showed us that indeed it is Kendall’s. It may not feel like doing a whole lot, but it’s got the fight in it that is so totally her! I really really really do not ever feel like even talking about that day again. I know some of my special needs momma friends have had to witness so much worse, and on a near constant basis. But I still feel afraid to close my eyes or have it be too quiet. I can hear her screaming if it is…(does that sound like Hannibal Lecter to Clarice? “can you hear them clarice…the lambs, do they still scream??? run run run…..run run run……”)
anyways – now that i’ve completely lost it (and proven it on the interwebs once and for all!), I’ll wrap it up.
Thank you to everyone who was praying for us on that day. As you may have noticed, I was SO dreading it I couldn’t even really talk about it in advance. Every time I would think about it I would be in tears. So to the few who knew ahead of time, thank you. To those who prayed even without knowing, thank you too. And to everyone of you who is hoping and praying for us that this new information and new plan is the KEY to getting things working again – THANK YOU!
I have so much more on my heart and mind to share –
and I will hopefully get the chance to soon, but please keep the Hilliard family in your thoughts and prayers. Eithene is a little girl who has battled against mitochondrial disease her entire four and a half years on earth, and now she is weary. Her family is preparing to allow her earthly body to earn its rest in this coming week. Eithene’s mom has been an amazing friend to me, helping me navigate some of the more crazy aspects of this disease and the weird things it can do to kids’ bodies, sharing of her spirit and her amazing love for God in the midst of her own storms raging around her. She, her husband, and Eithene’s little brother will need to be lifted up in ways I cannot even begin to imagine over the coming days, weeks, and months.
So this just seemed like a fitting video for today. I don’t know why. Cause it usually makes me cry pretty good. Anyways.
That’s about all I have to say about that.