April 25th Cardiology appointment

Thank you for everyone who has kept tabs on our family over the last several months. We all appreciate the prayers more than we can express. AnnaSophia received her last Synagis injection for RSV last Tuesday. It seems that the cases of RSV in Colorado Springs has significantly decreased, and she will not need anymore injections until October. She continues to gain weight at a steady pace, 11lbs. 12oz.! The cardiologist is amazed at her weight gain and how healthy she looks. She charmed everyone in the office with her smiles and cooing. If it were not for her oxygen, someone looking at her would never know that she has serious issues with her heart.

Now for the bad news. Her echo on Friday showed that her heart function is not normal, in fact, quite bad. Her left ventricle is still having to work too hard. It has now caused a leak in her mitral valve. The aortic valve is a bit leaky, but that was to be expected after her recent heart cath. The aortic valve is the culprit causing the issues with her poor heart function. Also, the portion of muscle in her ventricular septum (VSD repaired with gortex) is becoming increasingly narrow. This is a huge problem that is only correctable with surgery. The plan was to help AnnaSophia make it into her teen years before she needed a valve surgery, but now everything has been moved up. It looks as though she could require surgery as soon as a couple of months from now to a couple of years from now. I personally feel that as quickly as things change with her heart, we are probably looking at major surgery sooner than later.

The cardiologist and I spoke about doing the Ross Procedure at the same time that her septum is repaired. The hard part right now is finding a surgeon who can do the Ross Procedure. It is a very complicated surgury, and only a few surgeons perform it. The other issue is that a good Ross is very good, but an ok Ross is very bad. Instead of one bad valve, she could end up with two bad valves. Our cardiologist is looking for the surgeon who seems to have the best outcomes for us. The best Ross surgeon in the world was working at the Cleveland Clinic, but has since retired. His surgical team is still in tact at Cleveland, so efforts are being made to contact them.

This is a really hard time for Steve and I. If she has surgery in Cleveland, Steve will be able to be there for the initial surgery, but will need to return to work quickly. It will be better for our other children, Ethan and Emily, if Steve remains in Colorado Springs during AnnaSophia's recovery. We also have some insurance benefits for out of network providers, but there will be a tremendous out of pocket cost for us.

There is a surgeon at Denver Children's who has experience doing the Ross Procedure, but not the same level of experience as the team in Cleveland. We have had the highest level of care at Denver Children's and have been so impressed on many different levels, but when it comes to the life of our infant, we would like to have the best. I can't help but think that if we went to Denver for her surgery, and she didn't make it, we would be kicking ourselves for not attempting to give her the best options for survival.

At any rate, Steve and I are very emotional right now. We know that she is the Lord's child, and that He loves her more than we do. We are just very scared for her. I have continuous butterflies in my stomach and flutters in my heart when I think about the road ahead for her. We need to move forward before irreversable damage occurs to her heart. We pray for wisdom in the choice of doctors for her, and that the Lord will make a way for her care. God is good, and we continue to trust in Him.

I look up to the hills,
but shere does my help come from?
My help comes from the Lord,
who made heaven and earth.

Psalm 121

Mary

Feeling better!

Little AnnaSophia is doing much better since her heart cath. She had a hard time for the first 24 hours after her procedure. Thursday, my best explanation would be that she had the wind knocked out of her. She was pretty droopy all day. Friday, she turned the corner, just in time to see the cardiologist. Her echo showed that the ballooning of her aortic arch and aortic valve accomplished what the doctors intended to do. The left ventricle looks much better, but the pressure gradient in her aorta is up. That is generally not a good thing, but in her case, her heart function is much better. He said that if she were 20 years old, he would not be happy with the results, but at her age, he is overall pretty happy.

She was started on the drug Propanalol, a beta-blocker, to slow down her heart rate a little. She was running in the 140's before her procedure, and now she is running in the 160's. Still, not as bad as when she had JET, but it needs to be managed. Because of her small femoral arteries and poor circulation in her lower extremities, she will be starting aspirin therapy to thin her blood and, hopefully, increase blood flow.

The doctor warned that the Propanalol could cause her to become drowsy and sleep more. The interesting thing is that she was so exhausted from her poor heart function and anemia prior to her surgery, that she is so alert now. Even the new drug can't seem to keep her down. Before her procedure, she was awake for about 4 to 5 hours a day. Now, she has more of a normal wake and sleep pattern for a baby her age.

AnnaSophia is also taking an iron supplement for her anemia. The other amazing thing is that she is so pink now. Her little nail beds are pink, her feet are pink, and she just looks beautiful. I had noticed that she was pale before her procedure, but I didn't know what was causing it. Praise God for pink babies!

She will most likely require another heart cath within the year to keep her valve operating well. There is a concern for the muscle tissue beneath the valve. It has narrowed and is in the way of the bicuspid valve. The only way to fix this is through another open heart surgery. We just pray that as she grows, this problem will resolve itself, rather than become more of a problem.

I know this is a lot of information, but there is also more. As I research options for her valve surgery, I will report what the doctor and I have discussed. Right now, we are joyful and optomistic about her recent outcome. Thank you to everyone who has prayed for our little miracle girl. We appreciate all of the intercession on her behalf.

Shout for joy to the Lord, all the earth.
Worship the Lord with gladness;
come before him with joyfull songs.
Know that the Lord is God.
It is he who made us, and we are his;
we are his people, the sheep of his pasture.

Enter his gates with thanksgiving
and his courts with praise;
give thanks to him and praise his name.
For the Lord is good and his love endures forever;
his faithfulness continues through all generations.

Psalm 100

Mary

Going home!

The discharge process is underway! The doctors are pretty confident that the neurological symptoms have passed. Praise God! AnnaSophia is smiling again too!

The next step is to pray that she's O.K. once she's home and that there are NO emergencies! She will see her cardiologist in Colorado Springs on Friday for a follow-up echo and consultation.

Thanks to everyone for your continued prayers.

Steve and Mary

Noon update for April 9

We offer our apologies for the delayed report -- lots of stuff happens here.

AnnaSophia had a rough night, including a shuddering episode that was similar to a mild seizure. One of the not-so-desirable side effects of catheter surgery -- especially one as invasive as the type that was performed on AnnaSophia -- is damage to the arteries leading to the brain. That damage can be temporary or permanent. We're obviously hopeful that any disturbance was temporary, and so far, it seems as though it is. Aside from a strange head bobbing issue earlier today, she seems to be doing better on the neurological front.

As a precaution, the doctors are keeping us here for a while longer while they monitor her neurological functions and review her latest echo. Speaking of which, we did receive word that AnnaSophia definitely has an issue with a muscle underneath the aortic valve. The muscle problem is causing a continuing murmur. This could rectify itself over time, or need to be corrected with another open heart surgery. She also has a recurrance of some heart rhythym issues. Please pray that this will subside and not require more surgery. AnnaSophia doesn't want to see the hospital again for a while!

So, that's the latest. Please continue to keep AnnaSophia and the entire family in your prayers.

Steve

Tough afternoon on the 9th floor -- 4:15 p.m. update

We're now on the 9th floor with AnnaSophia, but she has been having a really tough time this afternoon. She has been extremely pale and very fussy/screaming. The nurse gave her another dose of morphine. She still has another two hours to go on the blood transfusion and has tubes/wires hanging off of her everywhere.

Mary was finally able to hold her a few minutes ago and that seems to be helping.

Please continue to pray for AnnaSophia tonight; that she starts getting some of her color back and is able to rest.

Taken a few minutes after the catheter procedure:

Taken at approximately 4:20 p.m. today (finally -- some rest for our little one):

Steve

Surgery complete -- met with the doctor

After more than 4 hours of surgery, AnnaSophia's procedure is done! The cardiologists used a balloon at two locations -- the aortic arch and aortic valve -- to relieve narrowing. Also, her hematocrit was very low, so she will be undergoing a blood transfusion over the next several hours.

The cardiologist is very pleased with the results. He feels that by fixing her "plumbing" a lot of her issues -- need for oxygen, paleness, overall energy level -- will improve drastically.

A picture of her femoral arteries showed that they were abnormally small. The doctor used the words "thread-like" to explain her condition. This is why it took them an hour and twenty minutes to get the catheter inserted.

Thanks to everyone for your continued prayers! If she handles the blood transfusion well and heals as expected tonight, she will likely go home tomorrow. The next 72 hours will be key as to the overall success level of the procedure. An echo will be performed tomorrow and then again on Friday.

Mary and I both feel like zombies, but we're anxious to see AnnaSophia soon. They haven't brought us back yet to see her.

A couple of pictures!

Mary took these a few days ago...

..

10:18 a.m. update -- catheter is in! Praise God!

I just received a call from the cath nurse at about 10:15 a.m. -- the catheter was successfully inserted! Praise the Lord! Thank you for the prayers. The surgeons are taking pictures of the aortic arch right now to determine next steps.

Steve

9:20am Update Prayers needed

We just received word from the nurse in the Cath Lab that they have not been able to start the cath yet, because they cannot get the second arterial line in. The doctor paged another physician to try to get the line in. It has been one hour and twenty minutes. We pray that the Lord will guide the doctor's hands, and that the next try will be successful. Please pray for our little girl. The doctor thinks that the stress on her left ventricle could be caused by the arch repair narrowing significantly. He is trying to fix this narrowing through the catheter. The other option is open heart surgery. We pray that the Lord will see this cath through with great success, so that she will not need another open heart surgery so soon.

Praise be to the Lord,
for he has heard my cry for mercy.
The Lord is my strength and my shield;
my heart trusts in him, and I am helped.

Psalm 28:6-7

Mary

Catheter surgery begins -- 7:50 a.m. report

We arrived in Denver last night and stayed at a local hotel since we had to be at the hospital very early this morning. AnnaSophia's check-in was at 6 a.m. today!

God has blessed us with an amazing little girl with a gentle and calm spirit. AnnaSophia couldn't nurse after 4 a.m., yet she was flashing smiles the whole time she was getting prepped for her catheter surgery. The Lord has filled her with His Holy Spirit and has used our little girl to show His grace. I am honored to have her.

She was taken back to surgery at approximately 7:45 a.m. We are expecting about a three to four hour surgery, with updates coming every hour. She'll go under general anesthesia and have a breathing tube.

The doctor who will perform the surgery told us this morning that he will be looking at two portions of her aorta. He will look at both the aortic valve and the surgically repaired portion of her aortic arch, which he believes may have narrowed like the valve has. It's possible that the aortic arch may be the larger contributor to the left ventricle issue Mary spoke of in the last update. If that's the case, the doctor may just attempt to enlarge the aortic arch instead of the valve. He doesn't want to touch the valve if possible, because he doesn't want to over stretch it and cause a leak.

Anyway, all of this is easier than it sounds. The truth is they're messing with areas of the heart that are extremely sensitive, not to mention the potential complications of entering a major artery with a catheter. Please pray that the Lord guides the surgeons through this procedure and that AnnaSophia comes out of it O.K. I'll try to post updates as we get them.

Last cardiology appointment

We intended to update the blog sooner, but out internet was down for days, so I apologize for the late update. AnnaSophia had a cardiology appointment on Friday, March 28th. She had an EKG, echocardiogram, and we repeated another 24 hour Holter monitor. The EKG looked normal except for some residual long Q T intervals. The doctor does not think this is a concern in that she does not have Long Q T Syndrome. This syndrome is actually quite dangerous, and we were relieved to know that this is not a concern for AnnaSophia.

The echo showed that the pressure gradient in her aorta had not changed, meaning that the aorta had not narrowed since her last echo two weeks ago. The bad news is that the left ventricle was enlarged, indicating that it is being adversly affected by the bicuspid valve and the aortic stenosis. After consulting with the interventionist, both cardiologists decided that it is prudent to proceed with the heart cath sooner than later. Monday, we were told that her procedure was scheduled for Tuesday, April 8th at Denver Children's Hospital. The plan is to gently stretch the narrow part of her aorta, relieving the narrowing. The doctors are attempting to make her bicuspid valve last through childhood. After she has finished growing, we will then need to replace her valve.

The good news is that the doctor called today to tell us that her 24 hour Holter was completely normal! We do find it amazing that despite her enlarged ventricle and bicuspid valve, she has no signs of arrythmia.

Please pray for AnnaSophia and her doctors that the cath procedure may go smoothly and accomplish what it needs to. We're hopeful that we can all be home again after a couple of days at the hospital. Also, pray for AnnaSophia's brother and sister. This has been so tough on them. They are very frightened and sad for their sister.

Steve and Mary