Wow, I’ve been really really bad about updates lately. To say that life has been a little crazy would be an understatement. Since my last post we had just returned from the hospital and Annabelle had not been eating well. We had really hit a brick wall and I was beyond discouraged with the whole situation! Thanks to some great advice from another more experienced PA parent we have totally revamped the way Annabelle eats. The problem is that Annabelle has to have a set amount of formula every day to help her stay within the normal range for protein; there’s no getting around that issue. What we are doing now is a continuous feed overnight through her g-tube so she gets half of her daily formula while she sleeps. By doing this we are able to knock out a bulk of the formula at night so that she isn’t fed as much formula during the day, allowing us more opportunities to focus on feeding solid foods. Since her night feeding goes from 9pm – 5am we are also able to let Annabelle sleep in until 7:30am (I still wake up at 4:45am to give her more meds), where as before we were waking her up at 6am to try to give her a bottle. Now she is able to wake up and play for an hour or so before we have to feed her at 8:30am. Twice a day we give her 0.25g of protein through some type of solid food then tube 130ml of formula about 15 minutes after she eats her solids. Once a day we just tube the formula without any solids. Our dietician gave us a great 8-page sheet with most of the brand name baby food items and how much of each of those items equals 0.5g protein. We are getting more comfortable weighing her food and measuring it all out (disposable 2oz containers have become our best friend). This feeding plan has been a game changer, and it has allowed her tummy to start feeling the difference between full and empty! Before we were trying to give her four bottles a day by mouth and also trying to feed solid foods in-between – we felt like all we were ever doing was trying to force food down her throat without much success! This new format allows us to focus on her solid foods without worrying about her formula (as we just tube it all now – bye bye bottle!). We’re only having to do “Meal Time” three times during the day so she has more time to play, nap, and be a happy baby. We also had a feeding specialist come evaluate Annabelle’s eating to give us some pointers. This has also helped, and we can happily say that Annabelle’s eating by mouth is great! So far she loves green beans, peas, sweet potatoes, and carrots; she could care less for the rice cereal or fruits.
Medically, Annabelle has had a few ups and downs the past few weeks. As you may have noticed in some of her pictures she had an awful rash on the inside of her arm folds. It started to spread further on her arms and we took her to see the pediatrician. She diagnosed Annabelle with a fungal skin infection (since she had been on antibiotics recently), and she prescribed an oral anti-fungal along with continued use of nystatin and over-the-counter lotrimin. However, after a few days the rash got much worse and started to spread all over her face and chest too. Luckily the pediatrician was able to get us an emergency appointment with a dermatologist who isn’t even taking new patients! He actually diagnosed her with severe eczema and prescribed a heavy-duty steroid cream and everything we use for Annabelle has to have no fragrance (laundry detergent/body wash/shampoo, etc.). Within days we saw improvement, and although it hasn’t healed completely she looks SO MUCH better!
When we did take Annabelle in to see the pediatrician regarding her rash Annabelle happened to do this weird eye roll thing three times in front of the pediatrician…her eyes will quickly roll to the back of her head for a split second. We never thought much about this before because it only happened when we were outside and when the sun hit Annabelle’s eyes (we just assumed she didn’t like the sun in her eyes). However, the eye rolling has gotten more frequent where it’s happening almost daily. This worried our pediatrician who had a nice long talk with our geneticist at Duke. They are both concerned that it could be small seizures or that some propionic acid could be building up in the tissue of her eyes. Therefore, they wanted to get some emergency appointments with neurology and ophthalmology.
We met with neurology Friday, July 14th and it was basically just a consult. The frustrating thing is that we had to wait an hour and a half to see the doctor who just did the same examination as when we were in the hospital a few weeks ago (a neurology team came to examine her during our last hospitalization because they had never seen a PA kid before and wanted to check her out). The neurologist found nothing wrong, and she was never able to see Annabelle’s eye rolling. She said it could be very mild seizures or it could be a weird baby tick she’s just learned how to do. She did recommend an EEG, and we have that scheduled for July 27th.
We met with an ophthalmologist on Tuesday and he did a thorough examination. He also wasn’t taking new patients, but sometimes Annabelle’s rare disorder has its advantages (most doctors want to see her since they have never met another PA kid before – she’s famous at Brenner Children’s Hospital and I don’t think there are many doctors there that don’t know about Miss Annabelle). The ophthalmologist couldn’t detect whether there was acid build-up in her eye tissue but nothing looked alarming to him. Everything checked out great with her eyes!
In light of the eczema, eye rolling, increased fatigue, increased ketones, and increased lack of appetite over the past few months I decided to touch base with our transplant coordinator in Pittsburgh. Mike and I both feel like Annabelle’s PA symptoms are starting to become more apparent the older she gets, which is somewhat reassuring that we’re making the right decision in terms of transplant. We really thought we would have had a call by now, but since Pittsburgh is the best in the country they have the highest number of Status 1B listings so the wait time in that region is sometimes longer (for example, there is currently 1 status 1A and 9 status 1B listings in the Pittsburgh region and there are NONE in North Carolina’s region). The transplant coordinator did suggest getting Annabelle listed through the “Living Donor” Campaign they have there (good samaritans that are willing to donate a portion of their liver for transplant since wait times are so long). Mike and I cannot donate to Annabelle since we are PA carriers; she needs a non-related donor with an A blood-type. Annabelle would remain on the national list for deceased donors, but by dually listing her this gives her a better chance of getting a call sooner. Unfortunately, since Annabelle has had so many changes the past few months the doctors in Pittsburgh want to see her again soon. So it looks like we’ll be making the trek to Pittsburgh for a transplant clinic, bloodwork, and a hepatology appointment, likely on July 31st.
Sorry for the long update; I will try to update more frequently to avoid these mini-novels!
Some Prayer Considerations:
- Pray that Annabelle’s eczema would heal completely. The dermatologist told us that the eczema leaves her skin exposed and opens the door to infection. Therefore, Pittsburgh will probably not want to do a transplant if she is in the middle of a bad eczema outbreak.
- Pray for our upcoming mini-trip to Pittsburgh on July 31st (safe travel, availability at the Ronald McDonald House, wisdom for the doctors).
- Pray that Annabelle’s eating continues to improve and that eating solids continues to be an enjoyable experience for all of us.
- Pray for the right liver at the right time for Annabelle – a perfect match for her little body (whether that be from a deceased or a living donor). Pray that it is soon; we are ready!
- Pray for our COTA team as they have put in so much time and effort to make sure this campaign has been a success. We are so blessed to have them!