It's MAGEC!

Anya’s scoliosis surgery went well. The recovery time was longer than I had anticipated; I had been told that little kids usually got back into the groove pretty quickly. Anya refused to try to walk for two weeks after surgery, if we tried to put her on her feet she would pull them up at the knees so she couldn’t stand. However, she did decide to do this:

 

 
 

And then she wanted to go to the park and climb on the rock wall:

 

So I ruled out that she had some kind of weakness issue, and after talking to the orthopedic NP several times we figured she had to do her own thing. Now she wants to walk everywhere, before she would get tired and need to be carried. Her favorite thing is going down to Main Street and walking among the stores.

She is so much straighter than she was! At her follow up they told me she had gone from a 74 degree curve to a 34 degree curve, a 40 degree improvement! In January they will start the lengthening, and that will straighten her out further. She will lay on her stomach and a device will lengthen the rod with a magnet. They will do this every few months until she outgrows this rod in a few years, and then she will get a longer one placed.

Before

After!

 She is avoiding so much surgery with this MAGEC rod! With typical growing rods you have to have surgery a couple times a year and that would be very difficult or even dangerous for Anya. I am so thankful MAGEC was available to her, and she had an excellent surgeon to place them. Most scoliosis patients aren’t three years old, we are so fortunate to live close to a pediatric hospital like Childrens, that has so many specialists.

Praise him from who all blessings flow!

And Now MORE Surgery...

Little monkey doesn't know she needs surgery!

The day after we got back from Gainesville we had a routine follow up appointment with orthopedics. They have been seeing Anya every 4-6 months to check her scoliosis. Anya’s curve started showing up while she was still in the NICU; she is very young to have this problem. No one is sure of precisely why; it can be more common in kids who have had a lot of surgeries to the chest, but it could be something else. Dr. Kays has said he has never seen such a severe case of scoliosis in a CDHer that has survived.

Lovely.

Still, I wasn’t worried going into the appointment. I couldn’t tell a difference in the way she carried herself, she was gaining more gross motor skills, and there hadn’t been any change in the curve at her last visit. I was completely caught off guard when they told me her curve had increased by 14 degrees and was now at 79 degrees, and it was time to do surgery.

‘Can’t it wait until the spring; I don’t like to have her recovering from surgery in flu and RSV season.’ I pleaded.

‘I don’t want to have her go through a flu RSV season without this being corrected.’ The doctor said firmly.

Well shit.

Scoliosis can get severe enough that it affects the lungs, and we are at this point. This is especially an issue with Anya, as she needs every bit of lung function that she can get. I have noticed some small signs that she is not improving as she should be, and it could be the reason she still needs oxygen when she is ill. It also could be affecting her eating, as her esophagus is angled differently. Anya can’t run yet, and when she walks it is easy to see how hard she has to work to keep her head midline.

Thankfully they are going to use the device I have talked about before, the MAGEC rod system. Traditionally growing rods are put in and then they need to be surgically changed every 4-6 months or so as the child grows. The MAGEC rods are placed once, and then they can be magnetically adjusted in the office every few months. Because Anya is so small in a few years she will need to have larger ones placed, but this still dramatically less surgery than she would have had otherwise.

When we are doing the pre-surgery workup a few weeks ago I got a call from the surgeon’s nurse practitioner. She had enough platelets, but some of the results came back that Anya was predisposed to bleeding.

Um, okay…

She has had so many opportunities to bleed out and hasn’t done so! I get that we had to cover all the bases and make sure it wasn’t anything to be concerned about, but it was kind of freaky going to the Oncology/Hematology office to get worked up. They did more blood work and determined they would give her some kind of replacement factor or something before surgery. I still don’t know what it is and I don’t think it has a name.

To make things more interesting, Anya decided to spike a low grade fever Sunday night. One dose of Tylenol settled it right down, but surgery was only four days away. She has a cough but has not had any more fever; Dr. Eaton saw her today and her lungs sound good, so we are moving ahead. As the flu/RSV season moves along, there are going to be more complex and sinister bugs making the rounds, so we don’t want to wait any longer.

Tomorrow is the day! We have all been dreading this and I can’t say I won’t break down in tears tomorrow because I will, but it will be nice to have this done. It could solve so many of her problems and help her developmentally. I have always worried about Anya’s scoliosis treatment, thinking about people I knew in school who had to wear braces and hating the idea of something that set her apart. Now she will have this started before she is even aware she is different.

So again I request prayers please and thank you for all of your support!

Discharge Day!

Anya is out of the hospital! We will start driving back home tomorrow.
The last half of this hospitalization has been amusing. I expected Anya to need oxygen longer than was typical, we even brought some tanks with us just in case, but even I was surprised by the amount of oxygen Anya needed. She was on 0.1 L for about a day. This is a ridiculously small number. Newborns go home on this, but Anya is three for goodness sake. Most kids can go from 0.5 L to nothing when they wean off. Not our kid!


We picked up some sandwiches and went for a ride!

When I cared for adults after surgery, we did all kinds of things to get patients to work their lungs: an incentive spirometer, that encourages breathing deep, and we also made patients get up and walk on the first day. I suppose kids don't usually need these exercises, but two days after surgery Anya was still hanging out in a crib watching movies. She was not using her lungs much at all. So late yesterday we went on a Mommy Daughter Date around Shands. She had a blast going to the food court, the gift shop, and wandering up and down the halls where I spent so much of my time three years ago.

After the trip it was like I had flipped a switch. Anya started being more playful and talking more. Only a few hours later I took her off the oxygen, and she has been off since. Her stomach has settled down and she is eating pretty well (for her). Her pain is well controlled with Tylenol. I am a little anxious about how well she will tolerate the car ride. Tomorrow we will drive less than half to ease her in to it.

After she was discharged we went and visited the NICU. We saw a few of her old nurses and met some families who have CDH babies in the NICU now. We have connected on Facebook but have never met face to face. Our children all have complex cases, and it was nice to talk about all the similar things we have faced, and they are now facing. In the world we live in, your child needs a nissen fundoplication and a g-tube placed before they can be discharged home. Events that others are horrified by are normal milestones. It is a strange place to live and I am so thankful for social media, it really helps make this experience more bearable.



Yummy noodles!

We saw Dr. Kays while we were in the NICU waiting room as well. Anya was playing with one of her Doc McStuffins toys, and Dr. Kays referred to it as an 'ear thingy'. Anya immediately corrected him, that it was called an otoscope. I remember three years ago watching this man examining Anya with concern, discussing all the hurdles in front of us. I think it is satisfying for him to hear Anya speak with such confidence now.

I hoped for the best and planned for the worst with this trip, so now we are getting back home earlier than I expected. When we get back there will be a whirlwind of preparations for BOTH girls to go to school!
So thankful for this smooth recovery. Thank you everyone for your thoughts and prayers!

Back at the Ronald McDonald House, settling in for the night!

Post Op day 3

This morning we were told Anya may be ready for discharge tomorrow or Monday! We still need to get her to take more of her normal tube feeding and get off oxygen.
Anya continued to eat well for breakfast and lunch today, and then we gave her normal tube feed after lunch. She didn't want to eat any dinner. At 8 PM we started up her normal evening feed and let it run for a few hours before we stopped it. Her stomach has been kind of distended all day and I was a little nervous about giving her entire feed. In order to get her to gain weight for surgery, we were giving her 780 calories worth of tube feeding in 24 hours. She is supposed to get 1000-1100 calories a day. So only a few days after surgery we were starting her on 80% of her calories, when she had already eaten about 20-30% of her calories. Also, the tube feeding she is on is very concentrated, possibly making it harder to digest.
While the tube feeding was on hold, only 1/3 of it done, the Fellow working with Dr. Kays, Dr. Solomon came in, despite it being 11pm! He looked her over and said he was comfortable doing whatever made sense to us. We decided to stop the feed for now and see how tomorrow goes.
Anya has been so close to getting off oxygen! She is only needing 1/4liter. This evening we kept her off for an hour and she was 89-93%, not quite where they want her. She hasn't been needing much pain medication but she hasn't been doing anything but laying in bed. Our goal tomorrow is to give her pain meds routinely and get her up and moving. That might help her breathe deeper and get off the oxygen.
This morning I asked more about the pulmonary hypertension and the echo they did in the OR. Dr. Solomon said it was actually at her baseline, so I guess that is a non-issue. I am still a little concerned but will follow up with cardiology in St. Louis when we get home.
I have to say this has been a very smooth hospitalization, and I have been very happy with the team of people taking care of Anya. Looking forward to getting her home!

Post op day 2

Another good day! This morning Anya was still not allowed eat or drink at all. This afternoon she started out on ice chips, and then was allowed to try a regular diet. She ate nearly a whole easy mac and cheese bowl and a few fries! They capped her IV and took off her monitoring leads, she is still on a pulse ox. Her oxygen has been weaned to 1/2 a liter.
The floor staff have been asking about our home tube feeding schedule, so I think they may start that back tomorrow in some way.
Anya has really perked up in the last few hours and it is so good to see!

Post op day 1

At her visit with Joy and Dr. Kays

Anya had her hiatal hernia repair yesterday and everything went well. She needed a little more oxygen than they expected, but they were able to take the breathing tube out right after the surgery. She was on a normal nasal cannula and had 'blow by' oxygen running from a tube by her face. By the time we got up to her room she didn't need the blow by anymore, and we were able to wean down on her oxygen overnight. Her pain is under pretty good control, she is on around the clock Tylenol and can have the hard stuff in between. She has been watching a lot of movies and chewing on her blankets. She gets upset when staff try to mess with her but everyone is surprised how she takes everything in stride, and isn't afraid. We spent the night in a monitored unit and today they moved her to the floor.

Waiting for surgery to start

Yesterday was really painful for me. Right before it was time to take her back for surgery, the anesthesiologist started discussing her pulmonary hypertension, and said it was 'significant' according to a echo that had been done the previous day. This scared the shit out of me because at last year's cath, and then an echo six months ago, I was told her pulmonary hypertension was resolved. It was a huge comfort for me to think this was over, it hits so many of these kids so hard, changing their quality of life, and it made me feel better about the surgery.
They actually repeated the echo in the OR and Dr. Kays felt comfortable enough to do surgery. I trust him but am still worried about this new development. Right now he is leaving this discussion for after this recovery period, and then we will work with cardiology in St. Louis to see what we do next. I cannot think of a clinical reason why this would be happening, but I am no expert here. It is a comfort that she hasn't gotten worse in any way, if these numbers were real it would seem she would be showing symptoms.
Dr. Kays told us she would be in the hospital for four to five days. Tomorrow they will let her have ice chips and maybe eat a little. The main goals are pain control and getting her to tolerate food slowly so it doesn't damage the repair. So far these goals are right on track.
Adam and I were able to get a room in the Ronald McDonald House for the whole of Anya's stay. This is so helpful! I spent the night with her last night and came back to the house today to take a nap and shower. We are only a couple of blocks from the hospital here and it is nice to be able to take turns this way.
So generally, things are doing well, but there is this huge question hanging over my head. Praying for peace and healing. Thank you everyone for your support!

More Surgery

It seems like something dramatic has to happen to get me to post nowadays.

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.