EP checkup

We were looking forward to taking Audrey to her Electro-physiologist appointment down at Egleston today. It's been a full month since we got a true look at how her heart is truly doing. They first weighed her and she was up to 7 lb 7 oz, which was up 12 ounces from last Tuesday's pediatric appointment, just a week ago. That was great news. As I have mentioned in the past, the pacemaker not only paces the heart but it also collects all of the data. Her data looked great! She did not have a single instance of tachycardic heart rate, above the 180 bpm range. The pacemaker was pacing the upper chambers 56% of the time and the other time the heart was recharging and sending the signal on its own all the way through the lower chambers. The doctor was really pleased with the results. Due to her growth and development they have upped her medication dosages to keep up with her size gain and also reduced the lower limit of the pacer to 100 bpm rather than 120. We go back in another month for our next follow-up. It was definitely very positive results and a great feeling.

Childrens Healthcare also asked us if we would be willing to meet with their media department to document our entire story of Audrey. They would like to use it in some variety of fundraising efforts, since they are a non-profit hospital. They said it could entail them coming to our house to take some photos of the whole family. They were not sure what format exactly it would be documented but could be some glossy brochure, website, or something like that. We told them that we absolutely would do anything of that sort as we are extremely appreciative of the care we have been provided throughout.

I know these posts are few and far between these days but that is really because thing are somewhat calm and stable and we love it.

- Mark

Growing and Baptism

It's so hard to believe Audrey hit 5 weeks old this past weekend already. Time really flies by when you are in a constant sleep-deprived stupor. Audrey continues to do great and is eating and growing like a champ. Amanda took her to a follow-up at the pediatrician yesterday, 10 days after our last visit, to make sure all was continuing on the right path with her eating and growth. She had put on a whopping 10 ounces in the past days and went from 6pounds 1oz to 6pounds 11oz. We were thrilled as an ounce a day was at the top level of what they described as possible for expected growth. Amanda and I can really tell just holding and looking at her that she is filling out and growing. She is also starting to become more awake and vocal which we find refreshing. Being premature, on medication, and through surgery we felt like all she did was sleep. I think she must be realizing that she's going to have to start making some noise to hang in there with the constant levels of screaming and yelling coming from Avery and Ryan. I never imagined I would welcome the sound of a baby crying but with Audrey it is another feeling of normalcy.


Audrey was baptized at our church, Roswell Methodist, this past Sunday 7/18. We were lucky that one of our good friends had the spot reserved for her own daughter's baptism and let us know before officially giving it up so we could jump on it once she did. We were talking to the preacher beforehand in the Welcome Center area and gave him a recap of Audrey's story. During the ceremony he held her and walked her around the church to introduce her to the congregation. Doing so, he was telling everyone her story and he got a little choked up which caused me to get a little teary-eyed as well. It was certainly one of those moments that we found hard to imagine reaching but there we all were. The ceremony was really nice and Audrey did great.


We're looking forward to going back to the EP doctor's visit on next Tuesday to take a detailed look at how her heart is doing and if there is any changes to be made. They mentioned that there is a good chance we will modify the pacer rate settings to be a little lower as she is growing.


- Mark




Audrey at 5 weeks...

All five of us...

Baptism...

With Audrey's cousin, Katherine...

A big Yawn...

Interesting Article about Long QT

Amanda came across this article from the WSJ talking about Long QT risk running in a family and how genetic testing can be beneficial in diagnosing the condition. As I mentioned yesterday, we are still awaiting Audrey's genetic test results and before Audrey was born Amanda's blood was drawn for testing and came back negative for all of the known mutations. Once Audrey's results come back there is a good chance we could have Avery and Ryan and myself tested as well. Having her specific results will narrow down the tests that would need to be done rather than testing for the full spectrum of possibilities on each of us.

The article just mentions Long QT in the broad sense without going into the different types and specific symptoms of each type. The risk and treatments do vary quite a bit through the different types. Nonetheless, the article provides an interesting overview on a specific family being affected by Long QT and provides some high level insight into what we are juggling.

http://online.wsj.com/article/SB10001424052748703283004575363070206768854.html

- Mark

4 weeks old today!

Our very loyal blog followers have been telling me I am slacking on the blog post updates. I have definitely slowed down in the number of updates and posts but that is really attributed to the fact that we are now home and things have been stable and gone really well with Audrey. We just haven't had much in the way of new developments over the past week, which is really a great thing. We are able to just focus on getting into the swing of the normal newborn baby duties as much as possible.

Amanda did take Audrey to her followup Pediatrician appointment on Friday to see how her weight gain was going. They decided to go ahead and do her 1 month appointment since it was just a few days after that. While at the 1 month appointment, the nurse was running through the normal things such as her bottom being a little pink. It's so hard to feel like that is really an issue to be concerned about at this point. Her weight had increased up to 6lb 1 oz which was good and we feel like we can definitely tell when looking at her and holding her but they want to see a faster rate of weight gain. They want us to feed her more often than the 3 hour intervals we have been doing. Once she starts gaining more weight we'll have to modify the medicine dosages we are currently giving too. Otherwise, they thought she was doing well and were impressed with her 'tone' for her age, which I believe was her muscle and head control. We have our followup with the EP's in two more weeks and we're still waiting on Audrey's DNA testing results to find out her exact type of Long QT.

I can't believe Audrey is already a month old today! This has definitely been one of the more chaotic, emotional, frantic, sleep-deprived months that we have experienced in our lives but as I have mentioned before, we are extremely grateful to be on the positive side of the possible outcomes right now.

Thank you again to everyone for all of the help and support we have gotten. We truly do appreciate it all.

- Mark

July 4th Weekend

Audrey is doing great and had a good 4th of July weekend at home. She is continuing to eat really well and sleep even better. We are usually waking her every 3 hours to eat except for the 10pm-12am timeframe in which she tends to be wide awake and ravenous. We have adjusted the two medications to be on an identical 4 times-a-day interval to simplify things and started actually mixing it in a bottle to give to her rather than directly into her mouth using a syringe. Being that she is premature and still tiny, the pediatricians wanted us to bring her back this upcoming week to make sure that she is continuing to get enough food and growing. Overall, she is really doing great and tolerating her crazy loud two older siblings perfectly.

We were able to take her to see the Roswell fireworks on the 4th. We had to run it by the EP doctors last week to see what they thought because we had read that loud startling noises are a trigger of arrhythmia's for some types of Long QT. They said they thought it would be OK but once we were there it did make Amanda a little more nervous than she expected and she tried to keep Audrey's ears covered as much as possible.

We still await the results of Audrey's DNA testing to find out what exact type of Long QT she has which will clarify a more precise long term treatment plan. That generally takes about 6-8 weeks so we have probably another month or so.

- Mark

Followup Appointment recap

We had our followup with both the Surgeons and also with the Electrophysiologists on Wednesday. Surgery group thought all looked great with Audrey's healing. They removed the remaining tape and it really has healed up nicely. There are no stitches, clamps, or anything else remaining there. We are officially done meeting with that group for now until the next time the pacer needs replacement. They have said on average that the battery lasts about 5 years, at which point we will have to replace it with a new unit. They will only need to replace the unit itself at that time and the leads going to the heart will stay in place and be connected to the new pacer so it will be a less invasive procedure then.

The meeting with the EP's was what we were looking forward to so that we could see how her heart was doing. They both did an ekg to measure the rhythm and they collected all the data from the pacemaker about how her heart has done since last week when we left. Audrey's QT interval still measures long on the ekg even with the medication, somewhere in the 500 millisecond range. Anything above 440 milliseconds is considered prolonged. After birth Audrey's interval was up in the high 600's to 700 millisecond range so the medicine has definitely helped to shorten it a good amount. The good news is that the pacemaker helps to keep her rhythm normal despite her long QT interval. We were able to see a lot of the downloaded information from the pacemaker and overall the stats were really good and was about the same as when we left the hospital last week. We were relieved to see that nothing unexpected was occurring that we weren't able to see at home. Her heart was staying in a sinus rhythm right around in the 120-130 range. Things were good enough that we aren't going back for our next followup for 4 weeks. In the meantime we will be able to upload the information from Audrey's pacer through the phone line that they can view.

There was one conflicted feeling I left with. Audrey's case is so high profile and rarely seen that all of the doctor's were involved, some much more than others, but all were aware of it at least. When we are there it's like we are celebrities and they are all so extremely nice and stop by to see us. Everyone from the big group's chief of cardiology down to all of our specific cardiac doctors came by and said Hi. They were really glad to see that both she and us were doing well and one even told us how our story was being told in some fundraiser events with donors. On one hand I have welcomed as many doctors opinions as possible during our journey, especially with it being a rare diagnosis. On the other hand, just as things were starting to feel somewhat better and normal (as much as it can), having all of those doctors come by was just a reminder of how rare Audrey's case is and makes us feel that the odds are not on our side. We left feeling really good and relieved about how Audrey is doing and the care she is getting but just a little conflicted at the same time.

- Mark