Rockstar

Sofia is a star. She now has 3 heart caths and 2 surgeries under her belt. I really don't think yesterday could have gone any better. Ok, if the whole day didn't involve a heart cath, then yes, it could have been better, but as far as days involving surgical procedures go then this one was pretty good.

After leaving Sofia sedated in the cath lab, we were due to get our first update 2 hours in and I was just about to get a little nervous when we hadn't heard anything by 3 hours. I started to notice that it seemed like all the families that were originally there in the waiting area had gotten updates and moved into patient rooms. So I was relieved when we soon got paged and heard that the doctor was actually finishing up and would be out to talk to us soon. It was almost exactly 3 hours from start to finish. Right after we had left her in the cath lab and started our long wait in M20, I put on Pandora Radio and the first song was called Live High by Jason Mraz and I chose to take this as a good sign.

Dr. Golden was very pleased with how things went. He said that the pressures they checked were even lower than they were when last checked during her 2009 cath. The pulmonary arteries looked good. I believe it is the right PA that looked a little narrow at one spot, but he also wasn't sure if that was a glitch in the program capturing the image or if it was actually a little narrow, but he said that is where they will put the conduit in the Fontan surgery anyway and was not concerned with it. There is some occlusion in her vessels (not sure if it was veins or arteries), but he was able to get in through the groin after doing ultrasound to look at the blood flow, and he definitely got a good picture of the inferior vena cava and a long stretch of that looks good. She did not have any collateral vessels. (A lot of single ventricle patients' hearts develop collateral vessels and they sometimes coil them off during caths). She had a stable heart rhythm throughout - back in 2009, she had needed epinephrine for irregular rhythm during her cath. Before the cath I had asked Dr. Golden about the potential of this repeating itself and he assured us that her heart is bigger now and she's more stable hemodynamically, so didn't anticipate any problems. He was right. She did just fine. He pulled out his cell phone and showed us 3D video of the imaging. Looked interesting, but you could have told me it was her brain and it's possible I might have believed you.

She was very groggy waking up from the surgery, but we were right there with her. She had to stay flat for 6 hours, which was a little challenging at times. She did really well, particularly while being fed orange popsicles (her choosing). We chilled in the familiar pods on M40 for awhile and saw some familiar faces and it was pretty relaxed. Dr. Golden stopped by to check on her and said he already shared the good news with Dr. Edwards (Sofia's cardiologist) and Dr. Stewart (surgeon). We really like him and are glad we found him. Dr. Stewart ended up stopping in as well and we talked briefly about surgery and we're all set for May 10.

It was nice to get home. I really didn't anticipate her being released the same day, so it was a very nice surprise. Oliver had his overnight bag back just in case. I had a headache probably from the lack of sleep, worry, and not drinking enough, and I can't imagine how Sofia felt so it was nice to be back home. At one point I told Sofia I was sorry she had to spend the day at the hospital and that it wasn't fun. She corrected me and told me it was fun. I think we may need to redefine fun in this household. At least this made me think that maybe it wasn't a horrible experience for her. We ended up getting home at 7:30 last night, which wasn't bad. Sofia was back to her usual self and had fun playing with Adelaide before bedtime. I'm not sure anything can hold her back.

Live high
Live mighty
Live righteously
Taking it easy
Live high, live mighty
Live righteously

Just take it easy
And celebrate the malleable reality
Nothing is ever as it seems
This life is but a dream



Arriving at the Clinic



Waiting before the cath

Cath is Underway

It was just a little harder to shut my mind off as I was falling asleep last night. The alarm was set for 4:30 this morning, but surprisingly I feel pretty good right now. About 20 minutes ago we left Sofia and her cath has started.

Dr. Mehta was the cardiologist that saw her first and we liked her. Had not met her before this. Then she was seen by anesthesiology, Dr. Rivera. It was alot of waiipting and Sofia said a couple of times that she wanted to go home. But she was calm and very well behaved. The child life specialist named Ashley came to see her, which was good timing because I wanted to meet with someone from child life just to see if they had any suggestions on how to talk with Sofia about surgery. Sounds like we are doing the right things. Sofia got a dose of liquid versed, which had her very calm within 10 minutes. She blew bubbles with us and Ashley. She got to pick a flavor of chapstick to coat the inside of the mask they were using. She played with the mask.

I wasn't expecting that they planned on intubating her because she wasn't for her cath at 6 mos. Dr. Rivera said it has something to do with the CO2 and not getting good reads for some numbers without intubation. Anyway, I hope she handles it all well.

Dr. Golden came In and saw us. He is the cardiologist performing that cath. His plan is to go in throught the neck so he can see the pulmonary arteries. He said he will try to get an artery in her groin. From her last cath, her right side of groin was occluded, but the previous doctor had not attempted the left side and chose to go through the liver instead. He said he can ultrasound it and see if it looks like a go. If not, there is a chance he might get what he needs just from going through the neck. If not, he'll go through liver.

We were able to go to the cath lab, which is new since we've had her last one. Apparently with new equipment. Sofia was very calm from the versed. It took maybe a minute or two for her to be out after putting the watermelon mask on. Love, love this girl. Not sure how long it will take, but we will get update after 2 hrs.

Cath Scheduled as Planned

What a week. We had a great time in Niagara-on-the-Lake last weekend for Easter. We met Oliver's parents there and had a really nice time. The weather was great and I felt like I got to spend some needed time with the girls. We were able to go to the falls, check out a butterfly conservatory, and see the town of Niagara. It was busy, busy back to work on Monday for a big 51 hour work week. UGH. It's hard not to have at least 1 day to recover and just get the needed things done around the house like laundry and planning for the week. I noticed that at the end of last week the girls might be getting a little cold, but it didn't really develop into anything. We called the cardiologist on Tuesday anyway because her heart cath is scheduled for Monday 4/16. They were ok with seeing how she did through the week and checking in with us yesterday. She seems to be fine so the cath is going to take place as scheduled.

I'm a little anxious, but more just ready to have it done and behind us. She is the first cath on Monday, so we have to be there at an unGodly hour, but that's alright. Little by little, we've been trying to prep Sofia on what is going on, but it is hard to assess how much a 3 year old can really understand. My sister asked her about what is going to happen on Monday and she did say that Dr. Golden is going to take special pictures of her heart while she is asleep. So, I think she did indeed absorb what I talked to her about earlier in the day!

We are kind of lying low this weekend in preparation for Monday. It was really kind of sad that today I decided not to take Sofia to my good friend Jessica's daughter's 3rd birthday party. I know she would have had fun, but I felt like we just couldn't risk her getting exposed to any germs so close to her cath and surgery. I know there will be other opportunities though once surgery is over! I'm trying to get a list together of things I know I will need for Monday at the hospital for a potential overnight stay. It's been so long (thank God) since we've had to do this.

We're not really sure how things are going to proceed on Monday with the cath. When Sofia had her last cath (in 2009 prior to her Glenn surgery) the doctors weren't able to go through the blood vessels in her groin because they were occluded. I'm not entirely sure if this is because of her heart condition or damage was done to them during her first cath when she was 1 day old. I think it might be a combination of the two. Regardless, they had to go through her liver in 2009, which automatically bought her an overnight hospital stay for observation. I'm not sure about what they will do on Monday, but I'm hoping they will attempt going through the groin. I guess I don't really care how they gain access as long as what they see in the cath looks good. There are things they are looking for in the cath, like different pressures and the size of the pulmonary arteries. I love this girl so much and I hope that it is as easy on her as possible.

Please keep Sofia in your thoughts and prayers on Monday!

Favorite pictures from Niagara...

Tricuspid what?

I sometimes get questions about Sofia's heart and what her condition is. She has tricuspid atresia with pulmonary atresia. Two of the heart's four valves are called the tricuspid valve and the pulmonary valve, and atresia means absence of. Her heart, for reasons unknown, developed in utero without a tricupsid or pulmonary valve. I'm going to try in my own terms to describe it (not a very easy task) but will rely on the Boston Children's Hospital website for some back up.

I'll start simple. The heart has 4 chambers: 2 upper ones called atriums and 2 lower ones called ventricles. Don't lose me here...In a normal heart, blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs, then blood returns to the left atrium from the lungs, passes into the left ventricle, then is pumped through the aorta out to the body.



Because Sofia was born without a tricuspid valve, blood can't get from the right atrium to the right ventricle and to the lungs as it should. No blood to the lungs? Houston, we have a problem. Essentially, there were walls where valves should have been. And surgeons cannot put a valve where there is a wall. So because blood can't travel through these valves because she doesn't have them, a surgical repair needed so that both a baby's body and lungs can get oxygen. This is usually staged in 3 surgeries. (It is kind of a moot point that she has pulmonary atresia in addition to the tricuspid atresia because she without the tricuspid valve, they would be bypassing the right ventricle anyway). She is referred to as a single-ventricle patient because she only has a functional left ventricle. There are several types of single ventricle anatomies and tricuspid atresia is just one of a few. And there are different variations of tricuspid atresia too.

In her first surgery that she had at 4 days old, she had a BT shunt put in which allowed her lungs to get oxygen. A connection was made, which allowed some of the blood traveling through the aorta towards the body to "shunt" through this connection and flow into the pulmonary artery to receive oxygen. This is a temporary fix which allowed her to grow before having her next surgery. A baby will eventually outgrow the shunt because it is made of Gortex and does not grow with the body.

Her second surgery, called the Glenn, was done at 5 1/2 months old. Babies are more stable after the Glenn because the surgery reduces the left ventricle’s workload and sets the stage for the Fontan Procedure to come. In her Glenn surgery, they took down the shunt and and replaced it with different connection to the pulmonary artery. They basically took her superior vena cava (the large vein that returns oxygen-poor blood from the head and arms back to the heart) and surgically connected it to the right pulmonary artery so that blood can get to the lungs to receive oxygen.

Is anyone still with me? We're almost there...

This final operation, the one she is having in May, is call the Fontan (named after the surgeon who invented it). The principle of the procedure is that it's not necessary to have a ventricle that pumps to the lungs so long as the lung arteries have a low resistance, are well developed and are of good size, and so long as the single ventricle fills at a low pressure. Surgery involves directly connecting the returning blue blood into the pulmonary arteries. This can be done in a few different ways, and Sofia will have what is called the extra-cardiac Fontan, involving a synthetic Gortex tube called a conduit that will be big enough for her to live with as an adult. She will also be pink after the surgery because she will have more oxygen rich blood where she needs it and her pulse ox will be near normal. I should also mention that these surgeries have been modified over the years to get the best outcome, so a Fontan isn't the same in all T.A. patients.

Because her blood will be passively getting to her lungs, not pumped by the right ventricle, like yours and mine, she needs to be on a thinner (right now just aspirin) to prevent clotting. Aspirin is the only medication she is on. She is at risk for developing arrhythmias (abnormal heart rhythms) that could have to be treated with medication or pacemaker down the road. We pray that everything continues to go smoothly for her. We are thankful that she has a functional left ventricle which was designed to pump to the body. Some babies that are born with only a working right ventricle (opposite of Sofia's and is called hypoplastic left ventricle), which was only designed to only pump to the lungs,, can have more difficulties and their first surgery is generally more complicated.



There you have it. Modern medicine and surgery. Please don't ask me to repeat this without my notes.

http://www.childrenshospital.org/az/Site508/mainpageS508P4.html

Sofia's Room

I've been wanting to take some pictures of Sofia's bedroom for awhile, but it's never been totally finished. I'm not sure anything in our house will ever be, so after I put up a new shelf in her room I got to snapping a few photos. First, a funny one of the girls at dinner tonight, and then Sofia's room. I hung the shelf myself!

Lockdown

As Sofia's cath approaches (4/16) we are more and more aware of the germs that could threaten to push back cath and/or surgery dates. Her cath is two weeks from tomorrow, so we are trying to all stay in good health until then! This has not been a particularly good winter for us germ-wise, so it will be challenging. Trying not to obsess too much about it. We're not to the point where we are on lockdown (yet), but that may come soon. We had a nice weekend. Started off on Friday night when Grandma came over to babysit so Oliver and I could go out to dinner. That does not happen often enough, and it was nice to get out. On Saturday morning, I took the girls an Easter Egg Hunt at the park near my parents' house. The girls enjoyed it, even though it was cold and threatened to rain. Who would have known that early March would have been the best time to have an Easter egg hunt...the weather just a few weekends ago was perfect.

This is Sofia and cousin Camryn. Sofia is her little shadow and loves to follow her around. And here is Adelaide showing off her basket of eggs.

My parents had everyone over for a nice spaghetti dinner today and now it's time to catch up on some things before the week starts again. It's going to be a busy week, but Easter is next weekend and I know the girls are excited to see their Oma and Opa!

...just looking through last year's Easter pictures and they're too cute not to post.