To sum up the past eighteen months: Anya has been doing great! She has stayed off oxygen most of the time, every three months or so she might catch an illness that required her to need oxygen for a week or so. She had RSV this spring and we kept her in the hospital for one night. Considering her medical history I was very impressed with how she handled it.
Last summer Anya had a cardiac cath to check out her SVC (superior vena cava, it is a big blood vessel right next to the heart). They thought it had narrowed, causing her face to get all puffy. This can happen after ECMO, a cannula sits right at that spot and can cause irritation, and scar tissue develops that doesn’t allow the blood vessel to grow normally. They found out that the SVC had COMPLETELY shut and there was no blood flow coming through. Blood was finding its own path to the heart through other blood vessels, sometimes even going the opposite way to get through! They placed a stent to open it up and it is fixed. She has already had a follow up echo and it looks great. She will continue to have cardiology appointments yearly, but her cardiologist thinks she may never need it to be dilated. The size it is may work for her as an adult!
Anya has been moving right along developmentally. She started a two day a week preschool program this year and loved it. Now that she is breathing better she has so much more energy to work on doing all the things most kids take for granted. She is talking more and more each day and every day her speech is getting clearer. She has a very large vocabulary but it can be difficult for others to understand her; she stops for breath in the middle of a sentence and may drop sounds that are difficult for her- like the F sound. Try to make it right now and notice the breath you have to take in and let out to get the sound. That is difficult to coordinate for Anya, but she is working on it! It has been very rewarding to hear some of the thoughts coming out of her head, after so much time.
|She didn't want to smile for the camera.|
Anya is still behind in her gross and fine motor development. She is walking more confidently but falls easily. Still, she has come so far. Using the upper body is hard when you have lung issues and Anya has started crawling around more and trying to swim. She loves to climb up on the furniture. We are still working on her ‘wonky thumbs’ but we think surgery can be avoided in this case. Her thumbs are getting stronger and she is figuring out different ways of grasping things. I have always been worried that she would find it difficult to write, but she has started holding her crayons correctly for short periods so we are on our way with that as well.
Anya is now three (!) and is aging out of the First Steps program. After age three the school district is responsible for providing therapy. This was nerve wracking as Anya had to be tested in order to see what she would qualify for, and no one was sure what that would include. Thankfully she qualified for a classroom at United Services. They include PT, OT, and Speech as part of the school day. The class has other children with special needs, but no one too different from Anya. There is a nurse there at all times who I can trust to take care of any special medical crazy that might happen. This is where her preschool was this year so she is used to going there.
Anya has severe scoliosis and at some point will need surgery. They will delay it as long as they can. I am hoping for a procedure that uses a device called MAGEC growing rods. Google it if you like, it is revolutionary. Most scoliosis surgeries require surgical adjustments every six months or so, this system allows doctors to make tiny adjustments with a magnet over years, with no extra surgery.
So, now we get to feeding issues. Lovely, lovely, godforsaken feeding issues. Anya has been a complete mystery with this one. At the end of last summer we thought she was ready to be off supplemental feeding, and took her off all tube feeds gradually. She did ok for a while but then started to dramatically lose weight. We started supplementing back, but had to keep increasing feeds to get her to gain any weight at all!
Then the ‘discussions’ started. Without getting too detailed all of Anya’s doctors and I went around and around, and around again, with how to address this. I balked at more tube feeds because there is no real reason Anya shouldn’t be able to take all her calories by mouth. There is a prevailing attitude in much of the medical community that does not seem to value weaning kids off tubes, and I have always felt very strongly about getting Anya off tube feeds as soon as it was safe. Tube feeding is not a normal way to be fed, and not a normal way to develop good eating habits and attitudes toward food. I wanted to get her off tube feeds so she could develop normal eating as soon as possible.
I ended up consulting GI at Children’s and they came back with a huge nearly impossible sounding goal: Anya needed to gain a pound and a half every month for three months. WTH. What three year old can gain that much weight? They had a theory that Anya’s metabolism had slowed down because her calories had been too low, and dramatically increasing her tube feeds would restart it. Appetite be damned, it was my idea to consult GI so we gave it a shot. She gained two pounds in about six weeks.
|For some reason Anya likes medical toys.|
Which leads me to the ‘point’ of this post, if there is one. Two years ago we discovered Anya had a hiatal hernia, where the stomach comes up through the diaphragm. This is a common condition that doesn’t always need surgery, but of course in a CDHer it is different. CDHer’s don’t have the normal anatomy that keeps the stomach down where it is supposed to be, and in fact Dr. Kays told us that with growth some of this could be expected. Dr. Kays wanted to wait to operate until she was older. Then when she started losing weight we saw that the hernia was getting bigger, and I was concerned that it was affecting her appetite. No one will commit and say this is probably the reason why she stopped eating, but I am hoping!
They wanted Anya to gain all that weight before surgery for safety. Even though she didn’t gain the original amount, Dr. Kays and GI talked this week and agreed that surgery could proceed. Dr. Kays is going to do the surgery at the end of July. She will be in the hospital for about 4-5 days or so.
Dr. Kays is doing the surgery rather than someone up here because he is the one who put the jigsaw puzzle that were Anya’s organs together originally. He is the best man for the job. This isn’t going to be straightforward, and it is a scary prospect. I have been re-reading early portions of this blog reminding myself of what Anya has made it through, and how much stronger she is now compared to where she was.
Please keep Anya in your thoughts and prayers as she jumps this next hurdle.