A Step Out of Hibernation

A tremendous amount of things have happened since my last blog post. It has all been a lot to think about and I honestly didn’t even know where to begin.

The best way I could think of summing things up was by creating a chronological list. In my last update I discussed my new diagnosis and how CHOW was planning on operations in July and August. Well the plans changed and my surgeries began the week after my birthday.

1. Shortly after my birthday we received a call from my fantastic motility doctor at Milwaukee. She said that the upcoming Monday would be a perfect day to have my GJ tube operation. It was quite sudden to hear, but I was so excited to have a chance to get off of TPN. My whole family was too. This was such a hopeful period. On Monday June 20th I received a second chance at being fed through my gut. That was a beautiful thing. I came out of my two hour long surgery very sore and full of sutures inside. They took three full thickness biopsies. I woke up looking at my G tube and hopeful for better days with no TPN. I remember almost crying with joy because my belly was so flat again. In the operation they decompressed my intestines.
2. After I recovered I had my G tube converted to a GJ tube. This way I would be able to start feeds into my small intestine while venting my stomach. We then began feeds. We started very slowly and worked our way up. I was put on a very broken down formula called Vivonex Ten. I was then put on over four new medications to help my small intestine move to accept feeds. I was started on more stimulants after not having a bowel movement for 12 days. I also got another injection of Botox into my pyloric sphincter of my stomach. I received over four iron transfusions which improved my color immensely. During the stay my J tube moved back into my stomach twice and it further confirmed my diagnosis of severe gastroparesis. I also had many images done of my brain and spine. We found cysts of my spinal fluid along my cervical and lumbar spine. Some of these cysts on my lumbar spine were pressing on the nerves that control my bladder and bowels. We also found abnormal lesions in my brain. Last week I had surgery to remove the lower cysts. We were able to get a few of them. Another huge step in this journey was my diagnosis of Classic Ehlers Danlos. It was hard to hear this, but it explains why my systems have been so severely effected. I was so excited when I could finally go home. It was a long two weeks. We all felt rejuvenated. I was tolerating close to my full nutritional feeds through my new feeding tube, I vented through my G tube, and I was only needing fluids through my PICC. It was amazing to leave without TPN.
3. Going home went very well. I was able to smoothly transition to being back with the people I love. I felt so liberated to no longer be dependent on TPN. I was able to not deal with the 10 pounds of water weight. I was able to enjoy those few days at home. I had a wonderful Fourth of July surrounded by people I adore. I was able to even eat up to 600 calories by mouth. The days after showed the signs of what was to come.
4. A few days after the Fourth I began to have acute nausea. It was debilitating. I needed the help of both of my parents to get myself into bed. My pain also began to soar through the roof. I was becoming so ill I was needing pain medication to allow myself to sleep. The next day I went in for an image because my abdomen was so swollen and big. The image showed my J tube had migrated into my stomach for the third time, hence the formula pouring out of my G tube. My Dad and I drove back to Milwaukee the following day to get it fixed. The days following those incidents were quite bad. My abdomen was incredibly swollen and hard. My stoma began to bleed from the tube being pushed out. My GI doctor tried everything he could to decompress my stomach. My  image showed that my stomach was extremely swollen and stretched all the way out. It covered my whole abdomen. I was later admitted back to AFCH that evening.
5. That night I was put through another thick and hard NG suction tube placement. Each day we pulled out over a liter from my stomach. My bowel sounds continued to decrease. My J tube moved again into my belly.  It was declared that we were in crisis management. I had another surgery later that week and I got another feeding tub exchange. As we started feeds I grew more miserable.  I was on pain medications around the clock and was constantly hunched over a barf bucket. My GI doctor sat down with my parents and I and we discussed my quality of life. We realized I simply can not tolerate feeds. So we stopped them. I was put back on full TPN and lipids.

After those storms I finally came home. This has by far been the hardest homecoming yet. My body is very tired and ill. I can no longer tolerate much of anything by mouth. I rely fully on my fluids and TPN. I am kept alive through my veins now. My GI tract is not being used anymore as it currently does not work. I am needing lots of heavy medications to keep me comfortable. Medications to tame the nausea, to tame the pain, to allow me to drift off to sleep. I constantly have a huge and hard belly. It is always so swollen and protrudes from my figure. I can no longer where any pants that button. My doctors have all realized I have a very severe case of pseudo obstruction.

This upcoming week I have a few appointments at Childrens Hospital of Wisconsin. I am looking forward to being able to ask more questions about what is next.

I am forever grateful for my family and friends. My sisters and parents have become my full time caregivers. They have so selflessly given up their time to make sure I am comfortable. I can not help but feel my heart ache as I see them changing their lifestyles for me. I can only hope that one day I can be able to provide them as much joy as they give me.

As this disease progresses so rapidly I learn to treasure things even more. I notice how beautiful my sisters are. The way my parents work tirelessly to make us happy. Seeing the way my friends are able to laugh with so much passion and joy. I treasure each day I am not on an operating table, being put to sleep, or having another tube shoved into my body.

So with all of this news I come to this conclusion: a lesson. A lesson to love more. Laugh more. Smile more. Simply live more. Let people know how much they mean to you. Go outside and soak up the weather. Be happy. Be free. Things can change so quickly, so rapidly. So embrace now. Because truly, now is all we have.

Love to all,

Isabelle