Wednesday, July 4, 2018

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!


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