The BIG surgery

After a week of discussion, planning and testing, it was Monday, surgery day. We knew she was an add on for the afternoon which meant she could be called to OR anytime from 12 to 10 at night. The suspense was hard to deal with. Audrey's Opa came to she the stress and worry with me. Audrey was blissfully ignorant of what was going to happen. She had had teaching on Friday where they made a doll with an ostomy and a bladder catheter in the belly button and we told her where everything was going to come out. She seemed indifferent to the teaching but played with the doll all weekend and was able to tell others all about the ostomy and bag. So Finally at 3 it was time to go!! We went to pre-op and spoke to a dizzying number of people. Going over the conditional plan, allergies and mitochondrial requirements with the OR nurse, the pediatric surgeon, the urologist, the anesthesia team and the pain team. Everyone seemed well read on Audrey and I was as comfortable as I could be handing over my daughter. Surgery was supposed to be 2.5-3 hours.

At hour 2.5, I was called and notified she was stable and surgery was progressing fine. At 3.25 they called say urology was done so the surgeon was closing, finishing the ostomy and then she'd be done. Finally after 4 hours of surgery she was done and going to recovery. Both teams of doctors reported a smooth surgery with no complications. Audrey was very sleepy in recovery but since she was inpatient she didn't need to be fully awake. She was responding to questions and asking for only her princess blanket (she refuses to use hospital blankets) so we knew she was ok. The pain team had placed an epidural so her pain seemed well managed which was a huge relief!! We were back to her room by 10. She had a busy night and her vital signs were a little off but she rested well! Let the healing begin!

Back from OR, there's no more room on this tummy!

Post Op Day 1-
The days after surgery are often the most difficult. Swelling peaks at day 3-5 as does pain and discomfort. With mitochondrial disease, the body has to recover from this huge strain placed on it which has depleted any extra energy it may have had. This was complicated by Audrey's primary team of docs not knowing her well. Of all the days for her to have new doctors this was not a good one. So Audrey slept ALOT! Her body was frantically trying to make enough energy. Her heart rate was high, her blood pressure was low. She had a low grade fever and needed her oxygen. She needed extra fluid. Her pain was mostly controlled unless we had to move her but she was very itchy from the epidural. It was a difficult day for her. Some good things happened though. First her new stroller came! Yay! It had been ordered for her 2 months ago so it was much anticipated, and it was pink! Next, after major surgery, patients have to do the dreaded incentive spirometry. This forces big deep breathes to help open the lungs and get rid of anesthetics. This being a Children's hospital this mean...blowing bubbles!! Same action but much more fun! Audrey willing participated and finally her oxygen started going up and temperature went down. Surgically everything was going well. Her
mitrofanoff was draining well. Her ostomy was pink and happy though still sleeping. We did her first
bag change with the ostomy nurse. By the end of the day, we got our first smile!

Very tired mitochondria!

             The new PINK stroller!

Incentive spirometry Children's style!

,

Finally, a smile from my girl!

Post Op Day 2-
Audrey woke up bright and early asking to watch Mickey Mouse! I love my girlie! And surprise, surprise, her ostomy was working!! Yay! Today, everyone is cautiously optimistic that we are finally on the right path. It was decided to start a small amount of pedialyte, give her some blood and we were still trouble shooting the annoying itching. Overall it was a good day. We were able to get her up out of bed into a chair and she was awake most of the day. She tolerated the pedialyte well and an advancing plan was made to get us home. Home!!! After 16 days our discussions finally included home! I was feeling so hopeful!

Happy girl first thing in the morning!

Post Op Day 3-
A day of rest! Audrey finally woke up free of the relentless itching and is still pain free. Since her epidural site looks great the pain team decided to leave it in place and let her have a good day. We had to switch her pedialyte to her jtube from her g because of retching so we did not start formula yet. It was decided her anticipated discharge date will be Monday 11/25, 3 weeks from when she was admitted.

Up in her stroller!

Post Op Day 4-
We are counting the days till we can go home! Audrey woke up again in great spirits and pain and itch free! We were even able to get her to stand on her own to weigh her. The pain team decided to leave her epidural in another day since she's doing so well and the site looks good. Audrey started half strength formula in her jtube! Yay!! Now we have to see what happens with her ileostomy output since it's been high. Urology also came and took out her foley so it's just her mitrofanoff draining urine now.

Post Op Day 5-
It's finally time to remove her epidural. Her dressing is lifting now so it's not safe to leave in. Everyone is nervous but hopeful since it was able to stay in so long she has missed the worst of the post op pain. They started around the clock pain medicine before taking it out so she wouldn't get behind in pain. Her feeds were advanced to full strength!! For the first time in nearly 5 months, Audrey is getting formula!! Her pain ended up being well controlled all day. What a relief! Our biggest problem was getting the ostomy bag to stay on. It's close to the dressing for the mitrofanoff so there isn't a lot of skin to make a good seal. By the 4th bag we both had our break down. She cried that she was scared and so was I. This is one more problem and complication in her life. We are trying so hard to make things better for her, I pray this was the right path.

A big leaky mess!!

Post Op Day 6-
Today we are not changing her feeds. This is what she will go home on and we will plan to advance her very slowly. We are going to start capping her mitrofanoff today and draining every 4 hours. This will be the plan for the next 2 weeks so I hope she does well. Her pain medicine is now PRN and she's tolerating that well. It was a restful day in preparation for a busy week!

She can sit up alone now (to play princesses of course)!!

Post Op Day 7-
Going home today!!

Surgery

I'm terrified. It's 630am but I've been up since 4 and was tossing and turning the hours before that. I'm filled with doubts about the choices that have been made and where we are going with her medical care. With each step we've taken, it's been the one I said I wouldn't do to her. Today will be a life changing day for Audrey and I hope with all of my heart it will be for the better.

So Audrey was supposed to have a cecostomy tube placed last Monday. This tube would have enabled us to flush her colon from the beginning rather than torturing her with enemas to clean her out from the end. However, thanks to the brilliance of her surgeon, a contrast enema showed the valve between her small intestine and large intestine didn't work. I was very disappointed since this really limited our options but I was thankful we found this out ahead of time because it could have meant months of trying to make something work that was never going to work to begin with. After discussions between her team it was decided that an ileostomy would be her best choice. This is where the end of her small intestine is brought to her abdominal wall and the poop drains into a bag. This would bypass her dysfunctional colon completely and hopefully allow her to tolerate feedings. The decision took a few days for me to come to grips with. It was ultimately the surgical fellow who reminded me that this doesn't have to be permanent. This surgery can be reversed. That was a great relief to my mind. I'm terrified of making the wrong decisions, especially ones we can't undo. My only requirement for this surgery is that we also place her mitrofanoff at the same time. A mitrofanoff is where they take her appendix and run it from her bladder to her belly button. Then when it's time to cath her and drain her bladder, we can do it from her belly button. This is much easier for her, not something anybody can see and will allow her to cath herself easily. But this is a major surgery. Both of these procedures are and a mito kid does not handle big procedures. So if we are going to do one to her, we are doing them both so she doesn't have to do this next year.

So after much discussion and planning, today is that day. Sometime this afternoon, my daughter will go to the OR and they will disconnect her small intestine and put it on her tummy and then create an opening in her belly button to her bladder. She will no longer poop from her bottom.. She will no longer need daily enemas and suppositories and buckets of miralax and milk of magnesium, we hope, and pray that this surgery will dramatically change her life so we don't live in the hospital for bowel clean outs and abdominal pain and distension. We will see...

This is Audrey with her ostomy doll. She learned what it's for and why it's there and how to put the bag on. She was also site marked for the location of her ostomy, where it should work best. X marks the spot!

Motility testing

This week had a big note on my calender for the past few months. This week Audrey's motility test was scheduled. We have been waiting for this since the beginning of April. While at that time I was on the fence, it became more and more obvious that this was something we had to have done. I came to the hospital with mixed emotions. First, I was so nervous that the test would be "normal." Not that I won't something to be wrong with her, but her GI motility is anything but normal. In the mito world we are faced with test after test saying "it's not normal but it's not completely abnormal." Second, I worried the results would be awful. What if her motility was so bad nothing worked? What does that mean for her long term? Finally, I worried about how she would handle the procedure. What 4 year old wants rectal probes in place all day long? Would she be able to tolerate the pain? This was before I knew exactly what was entailed in this testing.

So with all this on my mind, Audrey was admitted on Monday for two days of "cleanout." She could not even tolerate this so it was quickly scrapped. She hasn't been fed in 4 months, how much stool could there be? Wednesday she went to the OR to place the motility probes. They pulled out her GJ tube (which we did not anticipate and it was only 2 weeks old!) and fed a catheter into her jejunum. The catheter has the pressure sensors on then every few cms. They run water through the sensors so as the intestines move, the water is moved and the pressure is measured. Then they placed the same type of catheter (only much longer) into her colon all the way through to where the small intestine and large intestine meet (the cecum for those who know). Each catheter has 8 probes so a total of 16 probes were measuring her activity. She came back to her room and was hooked up to the machine by the GI nurse who stayed with her all day. For 4 hours they just monitor her. This is the "fasting" state. What her intestines do when shes not being fed. At hour 4 she was given IV erythromycin which makes the stomach contract. At hour 5 she was supposed to be fed 8 ounces of formula in 30 minutes in her stomach. This was a huge problem since Audrey has not once in her life consumed 8 ounces at one time, hasn't had feeds at all in 4 months and hasn't tolerated feeds in her stomach for the last 18 months. We comprised to see "as much as she could tolerate." This ended up being 4 ounces in 30 minutes. She was very uncomfortable, nauseous and in pain at this point. At hour 6, a bisacodyl enema was given through the colon probe. We suspected this would work well since this was her home regimen. Sure enough with in 30 minutes her intestines moved. Unfortunately she vomited all of the formula we had given her one hour and a half before and then some. Thankfully at this point the test was over. Now I had to anxiously wait till Thursday to get the results back. Here are some pictures:

   This is a picture of the screen reading a very quite tummy.

 This is Audrey being entertained

This is the catheter that is in her stomach and jejunum. The little blue caps are attached to each sensor.

Yesterday the motility doctor and her GI doctor came to speak with me about the results. What they found was on its her intestines did very little moving. As you can see in the graph above. Thankfully her stomach did respond to the IV erythromycin and there were a few contractions made into her small bowel from that. Feeding her did not make her intestines do anything at all. The bisacodyl showed the only the very proximal part of her large intestine has movement. The distal part (the descending colon) had no movement ever. Normally the recommendation is for a distal colectomy but Audrey has mitochondrial disease. This is a progressive disease. Cutting out the piece that doesn't work may help right now but in 6 months or 12 months she may need more out and more out. We just don't know. The next option is to do a c-tube. This is where they place a button in the very begining of her large intestine which does have a little movement and through there we can do the enema flushes. This is not a long term option because we know at some point this will stop working. Our last option is to do an ileostomy. This is where we stop using her colon completely and let her stool out from her small intestine. They also found that her g-tube is located too close to the exit of her stomach. This may be contributing to her pain and may be blocking her stomach. The hope is by relieving the "downstream" obstruction in her colon, her stomach and small intestines will function "normally."

This is very hard news to hear. Though I was expecting her to have poor motility. Being told that she will keep losing what little response she has now is heartbreaking. Having to make these decision is impossible. What if it's the wrong decision? What if it only causes her more pain and suffering? With every step we take with Audrey, I am constantly weighing her quality of life. Right now the has spent 3 of the past 4 weeks in the hospital and the one week she was home her stomach was huge and she was getting around the clock pain medicine. So something needs to happen. The plan is now to move forward with the g-tube relocation and to place the c-tube. I am not ready for the colectomy and I don't think she is ready for the ileostomy. Ultimately we may end up doing one or both, I may end up regretting the c-tube. But this is the least invasive we can be with her at this time and I hope that this will buy us time to come up with a better solution for her. I have no illusions that this will fix anything or make her better. But I hope this will make her more comfortable and keep her out of the hospital for longer periods of time.