Poor Maddy

I wanted to quickly recap the last week with Maddy while it is still fresh in my mind!

Last Wednesday was the day of her surgery to place her feeding tube. She was not nervous. (One of the benefits of having a brother with a tube) She was happy that she was not going to have to take her yucky medicine anymore. They took her back for surgery around 8:00 and she was done pretty quickly. When I got to recovery they told me she was having quite a bit of pain and had already given her  2 doses of fentanyl. When we got up to her room she was grunting in pain and really struggling so we gave her a dose of morphine. While we were doing our intake with the charge nurse, we realized that Maddy's anesthesia report showed that she had gotten lactated ringers (which is extremely dangerous for a mito patient). Our nurse stopped intake and scrambled to get risk management involved as well as check labs to be sure Maddy was doing okay. I also asked her to check Alex's paperwork since he had anesthesia as well. After the investigation, it was determined that the lactated ringers was pulled , but they caught their mistake before she actually received it. However, Maddy's blood gas came back not great. All afternoon we struggled to try and get her comfortable. We increased her dose of morphine 2 times and also added in hydrocodone. She was so pitiful. She laid completely still in the same position all day and barely said anything to us. She only talked to ask for more meds. Her little HR stayed in the 150-170 range. That evening we started pedialyte through her tube and she seemed to tolerate it well.

In Pre-Op

Not feeling good

Thursday they really wanted her up and moving. We tried and tried and finally got her to take a few steps. She was crying and begging to go back to bed. WE put her back and she immediately fell asleep and slept 6 hours! While she was sleeping we tried her first feed of formula. When she woke up she  had a fever. We had been on the fence about whether to discharge or stay one more night, but a fever made the decision for us! It was decided to try one more small feed in the evening, but hold overnight feeds in case the fever was related. 

Big brothers make everything better

Friday she had had no more fevers and was a little more chatty, but still did not want to move at all. But the doctors and I decided that we felt like she was well enough to go home. We made it home around 3:00 on Friday. Maddy was still not sitting up or walking. She just wanted to lay flat or slightly inclined. We got home and I gave her another small feed. She fell asleep and I struggled to wake her up so I decided to check her temp. Sure enough her temp was back up to 101.8. I could not bring myself to drag her right back to the hospital so I decided to give her some meds, let her sleep and reassess in the morning.

Checking out her tube

Home

When she woke up Saturday morning she still had a fever and her pain was horrible. She laid totally flat on her bedroom floor for two hours because it was too painful for me even to pick her up and move her. I called the doctor on call and he told me I needed to bring her back in. Both the Dr and I questioned whether something was wrong with the tube. At the ER, they started with some labs and fluids. Most of her labs looked okay, but her Sed Rate and C-Reactive protein were both very high. We went to X-ray and I was shocked when the doctor came back and said it looked like she had pneumonia. We had had some low O2 sats in the hospital, but she was not coughing or wheezing. Next we went to ultrasound. That didn't really give us any answers so they decided to proceed with a CT Scan. The CT didn't show anything with the tube, but confirmed pneumonia in both lungs as well as a pleural effusion on the left. They admitted us under the diagnosis of double pneumonia. It was decided to hold feeds through the night and resume in the morning.

Back in the ER

Sunday we saw one of our favorite hospitalists. He assured me again that the new tube was fine, but I told him my mom gut was that we were missing something. Her significant pain just didn't seem to fit with pneumonia. He ordered labs for the morning, but didn't tell me exactly what he was ordering. We just had a quiet day on Sunday and let Maddy rest. It was decided that we would start slow feeds Sunday night at just 10 cc/hour (2 tsp) and if she tolerated them to start increasing in the morning.

Monday we woke up to the realization That her lipase was high and she has pancreatitis. I am so glad that Dr. W has enough knowledge of our family to think to check her lipase because I had tunnel vision on the tube! We once again stopped feeds. She continued to have pain, but as the day went on she was a little more chatty.

By Tuesday morning she was begging us to eat. Her lipase Tuesday morning was the same, so we decided to let her try and eat. She ate a little bit of breakfast, but never ate anything else all day. The only other development on Monday was that she was finally pain free enough to attempt to stand. When we tried to get her up she was too weak to hold herself up. That sparked a PT consult. We also restarted some feeds Tuesday afternoon and evening.

Wednesday morning her pain was a little worse because we were feeding, but her labs had stayed the same so after some discussion with the doctors we decided to come home. She continues on antibiotics for the pneumonia. The pancreatitis will either resolve or get worse.,. only time will tell. It didn't make sense to sit in the hospital just to wait and watch to see what direction she is going to go. We are happy to be home. Sweet girl is still having some pain, but otherwise tolerating her feeds. She is extremely weak. Alex's nurse helped me give her a bath yesterday, because she is too weak to hold herself up unsupported.

Happy to be home...hopefully to stay!

We follow-up with her pediatrician today as well as her hematologist.

Thanks for keeping us in your prayers.
Ali