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!


Tuesday, May 1, 2018

Back to Children's Hospital for Research!

Good day friends and family. We traveled to Philadelphia last week for Ava to be in a Pulmonary Hypoplasia (Babies born with small lungs.) Research at CHOP. We also got two second opinions. (One from Ava's very first surgeon, Dr. Adzick and one from Dr. Denney an Orthopedic doctor about her scoliosis.)

Brian, Ava and I drove to St. Louis (3.5 hour drive) to board an American Airlines flight 522 to Philadelphia. I wanted to do ONE non stop flight as many of you know I'm extremely afraid to fly. It was Ava's first plane experience. I was able to take medicine to help relieve my anxiety. I was so glad Brian was able to come with us. He's my rock. We are always a team when it comes to Ava and her medical care. So here we are getting ready for the flight. This was our plane Ava was standing in front of. She was so excited!

Ava loved the plane. It was a 2 hour flight. I slept most of the way but when we were circling Philly I opened my eyes and saw Ava taking pictures and loving the view so I decided to look out, Being the "photographer" I am I grabbed my Canon and started shooting. WOW the view was AMAZING!

When we landed we picked up our rental car and went to eat at Red Robin. We don't have one of these where we live so this was a treat for us. Didn't my chicken sandwich look amazing?

One of Ava's favorite part of our visit was the hotel swimming pool. Sadly it wasn't heated so she froze but she still swam three times.

So we were shocked that so much had changed in twelve years. CHOP had so many new buildings and a HUGE research center. All the offices were new except the surgeon's office. It was exactly how it was all those years ago. Here is the front of the building and Ava's NICU unit was right in front here. Oh the memories!

So our first appointment last Monday was with Dr. Deeney an Orthopedic doctor. He looked over her scans. (They took a new scan while we were there too.) He agreed the the brace she was in was what she needed. He suggest that she wear the brace for 18 hours a day instead of the 14 that Dr. Weinstein recommended. We were so happy that Dr. Deeney said that Ava was in excellent hands at the University of Iowa. He is known as a "legend" of Orthopedic bone doctors. What a relief!

Ava first had to have a full body scan and then a chest x ray.

We had a long wait for this doctor so she decided to eat some snacks we brought along. Good thing we did! She's always hungry.

So as you can see here is her last three body scans. Her curve is above her heart which is a little more unusual. You can also see a good/bad look at her diaphragm. The right side is normal and the left side is a lot lower and is not even seen on the side at all. (She was born with left congenital diaphragmatic hernia- Missing her left diaphragm allowing her stomach to move up by her heart and her intestines, spleen and a small portion of her liver up in her left lung cavity.) She has to continue to wear her brace for 14-18 hours a day. We are praying that the brace helps to keep her curve stable. She's at a 26 degree curve without her brace right now.

Next appointment was with "one of the greatest men that ever lived" in our eyes, Dr. Adzick. He was her surgeon at birth. We were so excited to see him again! He has not aged one bit. I asked him how it came to be that we got blessed to have him as her surgeon. He said the surgeon that does the "consult" is the surgeon who takes on the case. I know without a doubt God gave him to us! I've heard amazing things about all the surgeons at CHOP so any of them we would have loved to have. He just happens to be the director of surgery at CHOP. He does so many wonderful things to further the study of not just CDH but all of the birth defects.

We wanted a clear and honest about Ava's diaphragm. For the past year or so I've felt things were changing with the x rays. When she was diagnosed with scoliosis in December 2017 the doctor ordered a MRI. Dr. Adzick looked at all her scans and tests and he too thinks that the very left side has reherniated. However he thinks that she has a lot of scar tissue that is keeping things in place. I worry that if there is a hole that intestines may get up there and become obstructed. This would be a disastrous storm. If she needed to be repaired they could not go in on the side but would have to go above her ribs to fix her diaphragm. (We would then want the side muscle used to repair her diaphragm so that we don't have to deal with patches.) He is optimistic looking at how well she looks that she "could" possibly never need fixed. He said the same thing that her Iowa surgeon said, "Her intestines are like cement" they just are terribly compact and scarred. He was also surprised at all she went through from 2 to 4 years old. She has many lives! He wanted to discuss what he thought with Dr. Hedricks the surgeon that is doing this research study. (We've yet to hear their collective thoughts but are eagerly awaiting them.) Dr. Adzick always instantly calms me. He has this amazing way about him that is soothing. He talks very softly and I'm hard of hearing so I caught bits and pieces. Brian filled me in on the things he said later. It was so good to see him and show him our little miracle girl he had a hand in saving! I just can't believe how fast the time has gone.

Next she had to do an Autism Assessment. She had to go with a lady and they asked her questions. She said it was easy... all they did was talk.

Then we went to do a Physical therapy assessment. She had to do the strangest things. Like stand on one foot and balance while closing her eyes. She also did some eye hand coordination tests. She did a 6 minute fast walk. They took her blood pressure and oxygen before and after the test. 

She has been having problems with her right heel. We went to see her orthopedic NP before we left. She said that with her bones growing so fast  her muscle is hurting as it needs stretching to adjust to the fast growth... basically growing pains. I felt some of the physical therapy she could have done better if that heel wasn't hurting. She did her best!

We took time for lunch and as her sisters she enjoyed the huge CHOP lobby. The ball dropper thing was Lexis and Emeline's favorite thing to watch. It brough me so much joy to see AVA standing there... right above her was where she fought for her life. It was like coming full circle and standing in God's Amazing Grace. I felt so very blessed!


You can barely see her but Ava is at the bottom of the stairway here. And the second picture is her view looking up. It hasn't changed much at all!

Silly Ava taking her own blood pressure.

Of course we had to take photos of this glorious occasion! Happy, Happy Day!

The last thing we did last Monday was BNP blood sample. We did not tell her about this test like we did with the others. I didn't want her worrying about it. She was so scared but did really well. The nurse was just amazing. She sang to her and was so funny. You can see the look on her face she wasn't ready. It was done before she even knew it began!

We were so tired after that first day. Ava was so excited to go back to the pool. She froze... her hands turned purple and her lips too but she LOVED that pool! I took advantage of their work out room. Only one machine worked but hey as long as I was the only one in there it was good.

Tuesday was also a FULL day.  We went to the echo lab for an ultrasound of her heart. When Ava was born her stomach and other organs pushed her side to the right of her body. What we didn't know was that the position of her heart never moved back over and her heart is also laying some what slanted... the technician had to have Ava lay on her side in order to get a clear view of her heart. She then asked... was she born with congenital diaphragmatic hernia? Why yes! All is well with her heart!

Next we went for Developmental testing. They wanted to see what Ava knew. This test we could not stay for so we walked around a bit. They gave us about 5 forms to fill out. Strange questions? Yes they were. They wanted to know stuff like... can Ava think for herself? Can Ava order her own food at restaurants? Can she tell you directions to get somewhere she knew? Can she use a credit card? Can she email, text or message people? Does she keep to herself? Is she social? It went on and on. I filled out 4 an Brian took the extra long one. After we got those all filled out we started wondering if we could find Daisy. She would clean Ava's room and be so happy and positive. We asked a cleaning lady that we saw in the hall. She sent us to the basement. We must have looked confused because a couple people asked if they could help us. They led us to the head of housekeeping. We told her our story and asked if there was anyway to find Daisy. She called and sure enough she was working that day. We set up a meeting time in the hospital lobby. Ava was still in testing but at 11:00 we were reunited with our lost friend Daisy. As soon as I saw her I burst into tears. All the emotions I was holding back about Ava and all I felt just came out. Seeing her brought back so many memories. She was our light at the end of the tunnel. She gave me the biggest hug. Brian and her hit it off twelve years ago. They teased each other and it gave us something to think about instead of our child hanging onto life. What a JOYOUS reunion it was! "We love you Daisy. Thank you for giving us so much love and attention!" When Ava was done testing we told her that we had caught up with Daisy. Her manager told her to spend as much time with us as she wanted. YAY! We had lunch with her. To say she changed our lives is an understatement. She made a difficult day worth smiling about! It's a great lesson to all... show kindness where ever you go. You never know when someone needs you so go above and beyond for strangers and your loved ones. God gives us the ability we just need to share His love! "We will never forget you Daisy and we are so glad that Ava was able to meet you!"

We went to see General Surgery to visit with Dr. Hedrick. She is the doctor that is doing the research study. She thought Ava looked great. She said of course she remembered Brian. No one seemed to remember me. Perhaps it is because I lost so much weight. (I hope.) I think I'm worth remembering! Everyone was shocked that Ava was so tall. We heard that with every appointment! We thought that she was going to go over the results with us of the last two days but it wasn't so easy. They told us they would send us the results in about 6 to 8 weeks. We can't wait to see how Ava did. We know she's better than average... she our survivor!

Last thing we did was do her Pulmonary lung function tests. They did it a little different that Iowa City. The lady kept telling Ava to keep trying harder. She didn't give her any breaks either, which was different as well. She finally wrapped it up and we were done. We were sad to leave our hostess Rachel. She took us to all our appointments and saw that we were taken care of. CHOP's research team is amazing. Ali helped us with travel arrangements. We were so appreciative of all their help! We are excited to be part of this study. More doctors and parents will be given answers. The thing we can certainly give them all is HOPE as CHOP did to us at the start. 

Ava had to get a picture of Rachel. "Thanks again for making us feel comfortable and informed! You were always there for us!"

Here are a few pictures Ava took during her trip. She may be learning a few trick from me! The first picture is the elephants in the hall at the surgeon's office. The second is out the window of the new building in the Pulmonary clinics. And of course the last is Philly.

Wednesday we didn't set any plans. We wanted to do a few things but just felt we'd see how everything fell. I talk to a lot of people online from my facebook page, "Raising Healthy Congenital Diaphragmatic Hernia Children" and occasionally I get the opportunity to meet survivors and their parents. Well we got to meet another family! Sandra and her son Anthony. It's always such an honor and I find myself quickly bonding with them. They came to our hotel. This was the first time that Ava has been old enough to ask and answer questions herself. To see them talk back and forth gave me goosebumps. Anthony had never met anyone who was born like him. He still deals with the effects of cdh. "Thanks Sandra and Anthony for taking the time out of your day to come meet us!"

So next we decided to take Ava by the Ronald McDonald house we stayed at before and then as she was getting treated at CHOP. Wow was that a blast from the past. So much has changed. They have added on and are making things even better for the families. We were just shocked by all the wonderful additions. This is the back of the house. The building to the left was not there when we stayed. The area is changing around them as well. It was very scary looking when we lived there... come on it was Camden, New Jersey. It felt safer... so much better than before. We talked with Teddy the lady who runs it. They are celebrating 35 years of service! How great is that? We sure appreciated all they did for us 12 years ago. It was surreal bringing Ava back and showing her around. It truly was our home away from home!

Next we were so excited to visit another one of our places we took Lexis and Emeline while they were there with us... the Adventure Aquarium. Such good and bad memories. The last time we were there I was pregnant with Ava and we all didn't know her future. Now here we were bringing her through the doors and showing her all the exhibits the girls LOVED. We had a great day. It was one I will never forget. This was right outside the Aquarium.

Here is a cool comparison I did with the first time we were there and now. Lexis and Ava look go much alike. How can time pass so quickly?

 Here was another great place we ate while we were here before., PEOPLE'S PIZZA. So good!

When I was almost ready to have Ava it was time for Lexis and Emeline to leave and go back home. I was very sad when they left. They had hugged me goodbye and hugged my belly and told Ava they loved her. It was so sad not knowing if it would ever get to see her alive. So I decided to go to Target there in Cherry Hill NJ. I walked the isles praying and asking God to give me the strength to get through all that was to come. I was fearful that I might go into labor before Brian could return. So my mind was very solemn that day. So as we were driving I saw that exact same Target. I told Brian that I wanted to go in. As we walked up to the store I put my arms around Ava and explain the day I was all alone. As we walked the isles tears filled my eyes. Ava hugged me and said "It's okay Mom, I'm here now." It was such a precious moment for us. All the hard work... all the prayers...all the hospital surgeries and stays. She is alive and she did survive! Amen!

A few trips to the mall. We managed to find some of the same malls we took pictures with all those years ago. Time changes so much!

Here are Lexis and Emeline in 2006 at the same fountain. How precious are they? They were pulling so hard for their baby sister. They are all so close now!

So then we flew back Thursday morning. We had the best time! Ava learned so much. We are so proud of her for putting so much effort into this study. We are truly blessed. I can't say enough times how emotional and wonderful it was all at the same time.

We continue to see how great our God is! We are blessed... so very blessed!

~Terri L. Helmick