Monday, September 24, 2018

Pulmonary and GI check ups

Hello everyone! Ava has been doing so well. She needed her annual pulmonary and GI check ups so today was the day! I was glad Brian was able to go with us. She was also able to get her flu shot as well. We had her take it in the leg this year. Last year hr arm was sore for a week or longer. I don't know if they hit a nerve or what but she was not thrilled about getting one this year.

First thing of the day was the dreaded lung function test. She has to take a huge deep breath and then blow all the air out of her lungs. She did good in the end!

Ava keeping herself busy...

She is 5'6 and growing. She weighs 96 pounds. This is good for her. 

The Nurse Practitioner was so impressed with how Ava looked. She was shocked she had grown so much. Looking back from last year she grew a little over 4 inches! That is crazy. She also praised us for being such great parents who advocate and always stay on top of things. It is nice when the healthcare team recognize us. Is is by God's grace that Ava has come this far. We give Him the Praise! 

We also saw her GI doctor. She refilled her Prevacid and Zantac for reflux. She was happy that Ava liked school and that she was healthy. It was nice to have a boring check up. We will take boring ANY DAY!

Thanks for stopping by. We thank you for your love, support and prayers.

Monday, September 17, 2018

Heart Catherization

Hello friends. Ava woke ready to tackle this procedure head on. She was super calm and was not worried at all. I on the other hand was worried but felt like it was in God's hands. We got to the hospital at about 6:30 am. She had her same silly attitude. You gotta love her joyous and carefree spirit. Her smile lights my heart. I felt a little calmer since she was in such a good mood. She brought her penguin along for the ride!

We got checked in and went to her room. She changed into the gown. She was still so calm. (Of Course we have to get a selfie!) I love this little lady so much!

Calm and collected... so peaceful.

It's so hard to see your child go through tests, procedures and surgeries. It just doesn't seem fair.

I had to take a couple deep breathes and pray that God would help us through all of this. Kate was Ava's nurse and she was so gentle and kind. She tried very carefully two times to get an IV started, but with no luck. So then the anesthesiologist assistant, Andrew came in and tried to look her over. He too couldn't seem to find one that would work so they decided to take her back and give her a little laughing gas to lessen the pain so that they could get one in. I asked if I could walk back with them and he agreed. (I've always walked her back.) She is older but I felt I needed to say I love you to her just right before she went in the procedure room.

Next we waited. My mother came and that helped get my mind off of worrying. They gave us a pager and would text us updates. First one was, "We got access to the vein and have started the procedure." Then about an hour and a few minutes after we got another one saying, " We are finishing up and she will be done soon. All went well."

When they strolled her in they were all saying how great she did. She did not looked medicated and I was a little confused. Katelyn her NP said that she did awesome. They told us that they started by using laughing gas to get her sleepy and relaxed while they got her IV line in. She said Ava was giggling. (No surprise there!) They then asked her if she felt alright and she said she did. They asked her if she wanted any medicine and she said no that she was fine.

Going into this procedure we had discussed with her that there was two options. Light sedation- doing the procedure with medicine to calm her and her other option was heavy sedation- this meant doing it while being on the ventilator. We were persuading her to do the light sedation.

Well apparently to Ava there was a third option (God's calming medicine.) She did it with no sedation at all. I was just blown away that she did it with no medicine besides the first initial laughing gas. She got confused and thought that sedation meant that she would need to be on the ventilator and she told us that she wasn't interested in that. Leave it to Ava to come up with her own way of doing it.

God calmed her! We were so happy to hear the news from the doctor that her heart looked great and that all her pressures were in the normal ranges. She had no problems with her heart and no pulmonary hypertension. Praise the Lord! He gave us yet another miracle today.

So with no medicine used she was able to drink and eat right away. She got out of the bed and into the chair. It was under and hour after her heart cath that were were leaving the hospital. I was just so humbled and felt so blessed! Thank you for all your prayers. She does have to rest for a few days. But then she can return to normal activities.

I was able to ask for an image. This is her beautiful, loving, caring and giving heart!

Grandma D came and brought her a little gift. That made her day. I'm so thankful for my mom!

She got the royal treatment with a ride to the car. She enjoyed that. She looks so grown up! Where did my baby girl go?

All in all a wonderful, easy procedure. This little lady is as tough as they come. I'm so proud of her. She tackled it with such grace and was not intimidated by any of it. She remembers everything about the procedure and said that she could feel the line inside of her but that it didn't bother her at all. I'm so lucky to call her my daughter. I'm thrilled she sailed through this so good... thanks to God. He was indeed the real medicine. He gave her the peace that exceeded any medicine the doctors could ever give her. (Less risk and many less complications!) Praise His Name! We love and thank Him! Thanks again for your thoughts, well wishes and prayers.


Saturday, September 15, 2018

How can she be 12?

Hello friends! I am a little late posting this but we have been extra busy with Ava starting middle school. She loves it by the way. God has given her amazing teachers. I adore her IEP teacher. (She was the teacher that came to our house and helped Emeline when she was out of school for several weeks with her jaws wired shut...due to breaking her jaw.) Mrs. Vandenburg remembered Ava and her story. I was so thankful at open house and relieved to know that she would be looking over Ava and helping her a lot.

Mrs. Vandenburg and Ava at Open House.

Mrs. Menke, is her school nurse. I met her when Emeline was in middle school. We spoke to her during open house. I knew that Ava would be in excellent hands. It turns out her and I have a thing or two in common. She loves to write too! Many years ago, I had a strong passion to write a book about Ava's journey. Ava was one at the time. I posted a note on my desk and said that "I will write a book one day." So here it is 11 years later and during a text I told her about Ava's blog. She read it and said, "You are a great writer. You should write a book." It was another sign that yes I should dust off those pages and try to get to it again. Ava has had so much happen in her life. A dream of mine would be to write a book and then get it made into a movie. It was neat that Mrs. Menke brought it up out of the blue. God sends us angels and she and Mrs. Vandenberg are two of them!

But Jesus beheld them, and said unto them, With men this is impossible; but with God all things are possible. -Matthew 19:26

So for the purpose of this blog post. Ava turned TWELVE on August 31! It is just crazy to think about her birth and those first several days/weeks/months. I still am humbled by the fact that God sent us to the hospital and that led us to getting Dr. Adzick the number one children's surgeon in the world. I mean that in itself is a miracle! Ava doesn't want to revisit her birth or look at the pictures. (I do every year as it keeps me thankful for all she went through and all that God brought our entire family through.)

Ava and I do one thing together every year on her birthday. We do a photo shoot. I love taking pictures, especially of her. It calms me and brings out my creative side. This "ritual" brings us together and it helps me heal and it bonds us as well. I think our Children's Hospital of Philadelphia visit this Summer made Ava realize just how blessed she is to be alive.

So here are a few of our favorites of that day. (Disclosure... this road is very secluded and does not get very much traffic.)

I used the road to symbolize her journey

We just had a nice family Supper of home made chicken Alfredo and bread sticks. (Her choice) Aunt Marsha made her mermaid cupcake birthday cake. So sweet!

So she was pretty surprised with her birthday present. I had to share this with you all. She is such a grateful young lady. And yes we got her a helmet. It goes 15 miles per hour. (On the sidewalks of course.) She was shocked to say the least!

I picked this special necklace out for her. It was exactly what I want her to know each and everyday. She is ALL of these things and more!

"You Are Braver Than You Believe, Stronger than you seem, and Smarter than you think, and Loved more than you know. "

She has a helmet and wears it whenever she rides. Safety is key!

Something HUGE changed in her medical care on her birthday. Our "mycharts" an electronic health chart site had a drastic change with her age of 12. They want to "protect" the child by not allowing parents electronic records. It is so that doctor and patient safety is number one. (AKA... child age 12 wants to be on birth control or has a drug issue they can talk about it without their parents knowing... bottom line. My summed up version.) I was so upset about this. I'm so used to getting online and looking at all her medical records and viewing all her tests. I could message her specialty doctors and get immediate response. It was so easy to look over all her care in one place. Now I have a lot more "red tape" to cross. I am going to try and fight it. Ava saw me upset and said, "Mom I want you to have full access of my records. You save my life." That was sweet. Ava filled out the form for her to get her own my chart that way I can go on and see it there. It is just so much more work. I'm sorry but in my opinion 12 is a minor. I'm still fully responsible for her.

She has a heart catherization on Monday September 17th. She is very nervous about this. They realistically want her to have just enough sedation medicine so that she is responsive but not aware of what is going on. She was very upset when the nurse practitioner used the word awake to describe how she would be. Sedated is not awake. Her heart doctor led us to believe that she would be put under during the procedure. It was a shock. The bad thing about full sedation (She would be put on the ventilator.) Her normal pressures will not be quite accurate not breathing on her own. Also with the tube it is longer recovery time and a lot more drugs and risks involved. We will make the final decision on Monday. This is the first real procedure that she will have done that is a big deal. Pray that the anesthesiologist will do what is best for her. We want no mistakes and we want her to leave just as she came in.

The reason for the heart catherization is to look at the right ventricular of her heart for hypertension. Her echo at CHOP over the Summer showed strain on the right side. They will insert a line in either her arm, neck or groin and go directly to her heart and measure her exact pressures. Pray that she finds peace in the decision she makes with the help of doctors and us. Brian will be with us so that eases my mind so much. He is my rock. Thanks for stopping by and I will update you as soon as I can about her procedure! Much love to you all.

- Terri

Tuesday, July 31, 2018

Summer and Orthopedic Visit

Hello Ava friends! We have been having a great Summer. Ava took 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 great 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 really 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 everyday. 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 to 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. They got us in yesterday. I was so glad. 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 musically videos.) and a pair of head phones and she's 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 the brace. 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. Apparently the shoulder blade sticking out is similar to her shoulders being so uneven both because of 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 wrote many publications and has done a lot of research for scoliosis. He basically 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 out a 26 degrees in December. They told me that it can 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 is 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 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

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!