Treasure Box

A dear friend of mine, Pam Miller, has a daughter with DiGeorge as well. She was telling me about this great idea to do for Arianna's hospital stay. Her daughter recently had a mouth surgery and this was a hit for her.

We make a Treasure Box and every time Arianna does something brave or courageous she gets to pick out a present! We were already planning on buying toys and gifts for her to have after surgery so why not make it fun and exciting with a treasure box!

So today for our craft project that is exactly what we made!

Still no word from her cardiologist about a surgery date so we have not told her yet. She asked a few times what we were making this for. Broke my heart. I told her we will be using it soon and left it at that.

Since Arianna has her dental work on Thursday she is asking about that today. She likes to know the exact date it will happen. She knows she needs to go to sleep and have a tube in her throat. :( It breaks my heart for my daughter to have to know all of this but she is one smart cookie and put two and two together last time when she woke up with a sore throat.

Blogging is something that really helps me so I'm sure to be posting alot over the next month or so!

It's Time

On September 21st, 2012 I got the call from Dr. Barber regarding Arianna's cardiac MRI results. Her right ventricle pressures had increased from the 130's-140's to 160's-170's! This is a huge jump in her pressures! She has officially hit the danger zone which means it is time to get her a pulmonary valve. The last few months she has slown down so much. She has also started getting daily headaches. I've pulled her out of school to homeschool to help her the best I can. I knew it was time but its still so hard to hear. The doctors are going to have a surgical consult tomorrow with Dr. Teodori and will be scheduling her surgery in the next few weeks.

Cardiac MRI

Arianna had her 6 month check up with the cardiologists in August. The echo showed her an increase in her right ventricle size, which warranted another MRI to be done. On Monday, September 17th we took her to UMC for her cardiac MRI.

 On our way to the hospital. She has "Coco" and her Beads of Courage!

She did amazing this time during the wait. She was not nervous or anything. She knew she had to "go to sleep" for the procedure. It wasn't until I walked her back that she freaked out and clung to me like a little monkey. As always, it was the hardest thing to do. I sat her on my lap and pinned her down while I forced the mask on her face. She cried and cried and asked me to take her home. Finally she feel asleep and off we went.

The scan took almost 2 hours! Since she always experiences headaches we asked for them to give IV Tylenol before waking her up. She also gets her antinausea medicine ever since her exercist episode a few years back! She woke up great this time without the delerium or headaches. With my girl in my arms we were able to go home!

My mom is always at all our procedures, appointments and surgery. Arianna makes a point to call her and make sure she will be there. Here they are at home after she watched a movie and relaxed. My mom always makes these fun crafts with her! Thanks Mom!

Chest pains

Arianna had some very scary episodes of chest pains and blue spells. She even managed to land herself a trip to the ER in August. So the cardiologists did another 24 hour Holter monitor to check things out. Everything came back normal!

Here she is wearing her Monkey packback which is actually holding her monitor!

The dreaded conversation

Today Arianna put on a tank top that really showed off her scar. She was standing in the mirror looking at her chest and asked, "Mama, what are these bumps?" I've told her many times before but I know as she gets older she will ask and I just remind her. I told her, "Those are the wires holding your sternum together." She asked, "How did they do that?" This is the question I've been dreading since her surgery. I had a feeling it was coming since she is maturing so fast. Without even thinking I said, "Well they had to cut open your chest..." I have NEVER said the word "cut" before because I know that would be scary but it just slipped out. I normally just say open and she is okay with that. Her poor eyes got as wide as golf balls and she interrupted me. "THEY CUT ME OPEN?!?!" she said as she stared into my eyes. All I could say was "Yes baby they did." She then asked, "How did they do that?" Another question I've dreaded for 5 years. Instead of giving her the great details I was hoping I could just say "They used tools." and see if that was enough for her at this time. Thank God it was. I then proceeded to explain how the sternal wires were placed. "You see they had to get to your heart which is deep inside your chest so they had to get past your sternum and ribs. Then when they fixed your heart they pulled your ribs back together and used the wires to keep your bones from moving." She just looked at me. I told her as I placed my hands upon her chest and traced her scar with my finger "You see baby, that is why you are so brave and strong. You went through something so big and scary and overcame it!" She then flexed her muscles and said, "I AM strong! Look at my muscles!" I just LOVE her!

I'm sure this is the first of many conversations we will have about her heart and all she has been through. From the very beginning Robert and myself have decided we would be honest and never lie to her about what she has been through. There are certain ways to say things at different ages. I know that God will continue to guide us through life and how we raise Arianna. He is always present and knows exactly what she needs to know and when she needs to know it in order to cope with her chronic medical condition.

GI Appointment

I am so thankful for Dr. Pratt. He is the most conservative GI doctor I have meet. We took Devin to him when he was a baby for his reflux and when we had to take Arianna back to GI we knew it would be with Dr. Pratt. So what transpired from that appointment???

We gave him a full medical history on Arianna and he listened to everything I had to say. He was very open and honest with us. There is not a true test to accurately diagnose aspiration. Aspiration is when the stomach content is brought back up and goes into the lungs. This is a very serious and dangerous condition.

So his initial thoughts were this: Arianna could have a birth defect called Transesophageal Fistula.

This is a congenital abnormality in which there is a connection between the trachea and the esophagus. Saliva and gastric secretions may be aspirated into the lungs through the abnormal opening in the trachea.


We are also suspicious that she is refluxing since she has never slept through the night and recently started exhibiting symptoms of reflux. Now if she does not have TEF its possible she is simply aspirating from reflux.

The frustrating part of this is we can do these tests but if she does not aspirate during the time frame we do the test, which some kids dont aspirate all the time, then we won't be able to diagnose it. I'm praying that God is at work here and will allow the doctors to see what they need to see in order to diagnose whatever is going on with my baby girl.

PLAN

She needs to have several tests done in the upcoming weeks.

• Bronchoscopy - They can actually check to see if any fat or large number of macrophages are in her lungs, which would indicate  the presence of food and confirm aspiration.
• Endoscopy - If she is indeed refluxing we need to see if there is any damage to figure out how to treat her. This will help in diagnosis reflux.
• NG Tube with dye - They will place an NG tube down her nose and pull it back spraying dye. If the dye sprays off into an opening then they can diagnose TEF.
• pH probe - They will place an NG tube down her nose into her stomach. She will go home with this in place for 24 hours and it will measure the level of acidity to see if she is having episodes of reflux.
• MILK Scan - She will drink a radioactive drink and then go back 24 hours later for xrays. If the radioactive material shows up in the lungs then she indeed is aspirating.

Again, we are hoping that if she is indeed aspirating that she does it during the time of these tests. 

The next steps would be rather drastic on either end. If she has TEF then we will have to talk to a thoracic surgeon about going a repair. I've looked into this some but got overwhelmed so stopped.

If its not TEF but she is aspirating on her own then we can attempt to thicken her liquids and see if that helps. If it does not then she would have to have a GJ-tube (feeding tube) placed and she would not be able to eat by mouth any more.

I'm giving you guys all the fact and telling you exactly what the doctors have told me. During her appointment with the pulmonologist today I explained everything that the GI doctors told us and he is in agreement with this plan so I have to accept it and place it in God's hands.

Pulmonary Function Tests

 Arianna had her PFT's done a few weeks ago. The nurse, Darcie, was so impressed with how well she did. Normally they don't do this test until the kids are about 10 years old but because we are trying to figure out what is going on with her lungs we wanted to give it a try. She had to do 3 different tests. One of them is the one to the right and is very simple, like the one she does in clinic. Deep breaths and blows until her lungs are empty.

 The second test sounded like popcorn and I was suprised she wasn't scared by it. She had to hold her cheeks as the mouthpiece would cut the the airflow on and off. You can see she follows direction very well here. It also helps that Darcie is the same nurse that does her tests in clinic and she has had alot of practice with those tests already.

 The next test is in "The Box" and I was not sure how she would feel about that one. She did GREAT! She has to follow more detailed directions for this test. At one point the machine will cut off the air flow and she has to keep breathing. We did it a few times but she got it down!

 

RESULTS

She has both obstructive and restrictive asthma.  Obstructive asthma means the flow in and out of the air is impaired. Restrictive asthma means her lungs are small and do not fill up well. The elasticity is not working well. 

We continue to do the Pulmicort twice a day since this is the best treatment for now. However, this does not really answer the unknown question of why the base of her lungs look so bad on CT and xray. Also why she gets so sick and easily gets pneumonia. We are concerned that a GI component could be at play so that is our next step..