Summer and Orthopedic Visit

Hello Ava friends! We have been having a great Summer. Ava took a swimming lesson last week. She did so well. It was the first time (ever) that she got tired and exhausted to the point she had to stop and rest due to her lungs. Swimming is excellent for her lungs and also for her scoliosis. She learned all the styles of swimming. She made us laugh by not understanding quite how to dive. She thought she had to fly into the water like Superman and they wanted her to put her hands straight down and fall in. She is a silly girl. Brian and I stayed during her lessons. (Brian was a swimmer in high school, so she always wanted his praise.) I did not tell them about her medical history as most of the instructors were young teenagers and she had a new instructor almost every day. We were so happy when she told us that while swimming her teacher asked her why she was "resting"... she replied, "I only have one lung, and it takes me a little longer sometimes." She is advocating for herself! That is huge for me. I am very proud of her.

She may kill me but here is one of her videos of swim lessons. She loves the water but is not so sure about diving quite yet!

The other day I noticed her left shoulder blade was a lot further out then her right one. It scared me, and I called and got her in to see Dr. Weinstein. She had an appointment in August, but there was no way I was waiting that long.  I was so glad they were able to get her in yesterday. They did the "usual" hand bone age scan and also the full body scan. She had her brace on, and they told her to take it off.

It was extra busy in the office, and we had to wait. At one point Ava sat on the floor. She doesn't care at all about being uncomfortable. Give her some music (She LOVES to do the music videos.) and a pair of headphones and is good to go for hours! She amazes me!

I was not surprised when she measured almost 5'6. I can't believe how crazy fast this little lady is shooting up! Finally, Dr. Weinstein came in and examined Ava. He asked her to lay down. He wanted her to take off her brace and put it back on while she was laying down. (We learned this back in January when she first got the brace, but she gets lazy and does it standing almost all of the time.) He told her that she must put her brace on laying sown to get the most benefit out of it. He was calm and reassured her that he was very sorry the brace was so uncomfortable and hot. He is the kindest man. I asked him how her curve was and he said it is holding stable. GREAT NEWS. The shoulder blade is uneven due to her scoliosis.

I told Dr. Weinstein what Children's Hospital of Philadelphia's orthopedic doctors said about him... they oh and aww-ed about how Dr. Weinstein was the BEST orthopedic doctor in the WORLD. They told us he had written many publications, had done a lot of research for scoliosis and wrote the books on how to treat scoliosis. Plus he has received a ton of Awards for his achievements. We KNOW without a doubt that yet again God gave Ava the best! How awesome is that? I seriously am in awe over how great God has planned things out for us. First getting the best pediatric surgeon in Philadelphia (at birth) to now the best orthopedic surgeon/doctor today. MANY of our prayers answered regarding Ava's health and life!

Here is her latest scan. They said her curve is now a 20 degrees. (It measured 22 last appointment) I was a bit shocked as it started at 26 degrees in December. They told me that it could differ from each person that measures it and also from each scan. AND her scan made her diaphragm look a lot better as well of course to me... I'm not a doctor by any means. Also, we were there for a spine scan, not an x-ray. Either way, I'll take it! 

Here are the "official" scan findings from her July 30th appointment.

Impression: External brace has been removed since prior exam.

Thoracic dextroscoliosis, from superior endplate T2 to the superior endplate of T7 measures 20 degrees, previously 15 degrees.

Levoscoliosis from superior endplate T7 to the superior endplate T10 measures 18 degrees, previously 16 degrees approximately at this level.

Here is the scan results from her very first body x-ray on December 4, 2017-

Findings / Impression: There is levoscoliosis of the thoracic spine with Cobb angle of 26 degrees between T6 and T11. Negative sagittal balance with an offset of 9.1 cm.

The good news is she is NOT having any back pain. So now to get her to wear her brace for 14 hours a day is the goal. She does good wearing it at night but not so good wearing it during the day. Right now the brace is working!

Thanks for checking in on her. We are blessed to have so many supporters, followers and friends that think and pray for Ava. We love that you care!

I'll leave you with one of her cute snap chats!

Thanks again. We appreciate your love and support!

~Terri Helmick

Cardiologist... what is normal?

Happy 4th of July! I hope everyone has a great day. Ava and I kinda just chilled today and poor Brian had to work. We thank anyone reading today in the military for their service. We are so happy to live in America! God Bless you all! Thanks for checking in with us!

It is very hot and humid here in Iowa. Where is Iowa you ask? We are in the in the Midwest! What are we known for? Yep corn and farms. It is slow paced here. We live in a town of 25,000 people. We rarely ever have to wait in traffic. We are great with that. So now you know!

Ava had a cardiology appointment yesterday. I was a little nervous about this appointment with Dr. Reinking as Children's Hospital of Philadelphia had requested Ava see her cardiologist as a few of her tests indicated possible pulmonary hypertension. She is now 5'5. (98th percentile) Yes I got passed up. Hey I'm okay with that. The rest of her stats... Blood pressure 103/63. Weight 95 pounds (61st percentile) BMI 15.89 (17th percentile) Temperature 99.3 (She was wearing her scoliosis brace so she was warm.) Pulse was 100. Respiration was 26 and oxygen was 100%

They did an electrocardiogram (ECG or EKG) test. It measures the electrical activity of the heartbeat. That was normal. Or maybe it wasn't do you see that face? Scary! She is silly she took all the stickers off herself and put them back on the paper and brought them home. Why? She said, "I've never taken them home with me before." Yes only Ava!

Everyone and I mean EVERYONE told us they loved her hair colors and curls. A few weeks ago she asked... I said of course and did purple, pink, teal and blue hair color. (At home.) I can't believe how much she is growing. She's turning into a beautiful young lady. I signed her up for swimming lessons this Summer. She will be starting middle school in the Fall. 

What is pulmonary hypertension?

Pediatric pulmonary hypertension (PH) is high blood pressure in the arteries of the lungs (the pulmonary arteries). PH is a progressive disease where the pulmonary arteries continue to shrink, making the right side of the heart work harder as it makes the higher pressure needed to force blood through the narrowed arteries.

So in simple terms. The blood on the right side of Ava's heart is pumping blood into her lungs... she was born with pulmonary hypoplasia (small lungs) but due to her left lung being very small there is limited "working parts" meaning the blood has to pump a little faster because it has a smaller area which actually functions. (If it functions at all. It may only just pass air.) They told us that her lung could grow and expand however since it didn't grow right inside utero. She has less alveoli, the small air sacs in the lungs so her left lung is not normal and never will be. Good news is you can live with one lung. Her right lung is very strong and can sustain her! Here is what Ava's lung may have looked like at birth. It now fills the lung space but they don't know just how well it actually performs. 

Here are a few reasons the doctor thinks she could have Pulmonary hypertension. The obvious one was she was born with diaphragmatic hernia. (While growing inside of me her organs migrated to her left lung area due to a very large opening in the left diaphragm muscle.) Pulmonary hypertension was one of the things she nearly died from in the NICU. They had to stable her heart and lungs to even begin to think about her repair surgery. It took 2 whole weeks for her to be strong enough and well enough for her Gortex repair. After her first surgery she no longer had it. So she may just run a little higher than normal kids... another theory is she may be having a little bit of a hard time at night. Ava gets into a very deep sleep and sometimes irregular heart pressures can be due to sleep apnea. And finally the one that probably fits... it could be associated with her scoliosis. Her spine is growing to the left and it may be putting stress on the right side of her heart. So those three things. 

Dr. Reinking wants to do a cardiac catheterization, which will give a definitive diagnosis of PH, to determine the severity of PH and guide her treatment. Because cardiac catheterization is an invasive procedure he will require the assistance of cardiac anesthesiologists and often a night’s stay in the Intensive Care Unit. During the procedure, a catheter is threaded through blood vessels into the right side of the heart to the pulmonary artery. With this catheter, they measure the pressures in the pulmonary vessels and the blood flow into the lungs to help them determine the severity of the condition and confirm the diagnosis. They will give Ava medications during the catheterization so that they can see how treatment will affect the pressure in the pulmonary artery. Catheterization can also help predict disease progression.

He also said she has a leak as well but he is not concerned about that. Her heart is also pushed to the right side of her body... again due to her organs growing in her chest. Here is a diagram that might explain  Pulmonary hypertension a bit better.

They will be calling us soon to set up the procedure. We thought doing it during Summer break will be the best. She is never afraid. I thank God for that. We have a lot of appointments in September. She will see her spine doctor, her GI doctor and her lung doctor. 

And as always we make a trip to Hurts donuts! LOVE them!

Please keep Ava in your prayers. You guys keep up with her journey. I keep waiting for it to slow down. We are grateful she is so healthy right now. We know how quickly things can change. We have no doubt that God holds her close each and every second. Thanks for dropping by!

~Terri