Tuesday July 5th

Another day in the books at Egleston, but not without some excitement of course. At night when Audrey is sleeping her heart rate naturally slows down. The past couple of nights Amanda and I had noticed it slipping below the 80 mark which struck us both as odd considering her pacer is set so that it will ensure that is the low point and will kick in to ensure that. This is concerning because Audrey's type 3 of Long QT is known to be triggered during low heart rates and sleeping. We have come to ignore a lot of the readings on the monitors because many are false readings so we didn't read too much into it. Today when Amanda brought it up to the doctors during rounds they interrogated the pacer and found that both the leads running from the pacer to her heart are fractured and not operating correctly. This occurred in the past few days here at the hospital somehow so they went ahead and turned off the pacing and we are relying on medicine alone until surgery on Thursday when they will replace those leads along with the ICD. This definitely led to our anxiety levels ratcheting up slightly further today. We should find out tomorrow what time on Thursday Audrey is scheduled for.

Yesterday Avery and Ryan came down to visit with the intentions of going up to the 6th floor and watching the July 4th fireworks together from the window but with the heavy rain delaying things we decided to bag that idea and head home instead after hanging out for a little while.

- Mark

Sunday July 3rd

This picture was taken on Wednesday in the CICU, two days after Audrey's episodes occurred.






This picture was taken in Audrey's step-down room when the kids visited.











Audrey is officially scheduled for ICD implantation surgery on Thursday 7/7. We don't know what time of the day and the date could still be shifted if any emergency surgerys bump us out or if someone ahead of us drops off of the schedule. The same doctor that implanted Audrey's pacemaker, Dr. Kanter, will do the surgery once again and will remove her pacemaker and replace it with the new ICD. They will examine the existing leads and reuse them for pacing as long as they are in good shape and they will have to also put in an additional lead from the ICD to her heart as well. If everything goes well then they estimate that the recovery will take 3-4 days following the surgery where they will also be monitoring her rhythms closely and adjusting the settings to ensure that it is pacing correctly.

We're still in discussions around the denervation procedure but we've established that we are not going to have that done here at CHOA. They want to build up that experience and ability here but we are unwilling to have Audrey be one of their first. We are working with a doctor at the Mayo Clinic in MN that is one of the leaders in the Electrophysiology field and especially Long QT and has extensive experience with this procedure. We believe that when we take that step then we will travel to Minnesota to have it done.

Audrey is really doing pretty well now. Her rhythm has been very stable and she's as happy and active as you can be when you're stuck in a hospital. We walk her all over the hospital and outside as much as possible. It is crazy how quickly perspective and patience levels can change when you are here. Not only do the thoughts around Audrey's situation and how it could have been tragic make us really cherish time with the kids and serve as a reminder that we must have more patience with them during those times when they are just being wild kids. Being here you also see all of the other children that are battling so many other awful conditions as well and its just such a reminder to not ever take the health and happiness of your children for granted. It is definitely a reality check on priorities.

- Mark

Friday Update

Audrey enjoyed her first full day of being in step down rather than the CICU. With her telemetry monitoring she is fully mobile and able to crawl around and be active again. The nurses setup a nice mat with a blanket on it for a play area with her toys. She is her old self again as far as her activity level, mood, and eating again. She does get pretty fired up with each new person that comes into the room to take her vitals or an ekg. We're not sure if it is due to a new stranger complex at her age or just being sick and tired of another machine being wheeled in to poke and prod some more. I definitely couldn't blame her if its the latter.

Avery, Ryan, Grandmommy, and Granddaddy came down to visit Audrey today. She loved seeing their smiling faces and we took all three kids for a ride in the wagon outside and around the hospital. We ran into the hospital assistant golden retriever dog which the kids got to pet as well as the helicopter transport pilots were getting some coffee and took some time to talk to the kids and give them some pins. Avery and Ryan also got to meet with the child life specialist who sat with them in the playroom and did crafts and explained to them a little about Audrey's condition using some fun props and tools. It was a great experience for them all.

We also feel like we made some great progress today on our research into the suggested treatments for Audrey. Our great friends at the SADS foundation instantly connected us with one of the Long QT experts at the Mayo clinic and we are now working closely with him in conjunction with our CHOA doctors on a plan. After today we really feel like we have consulted with some of the most expert and renowned doctors worldwide in the Long QT field and are much more at peace with the proposed treatment options. Although there are quite a few specifics to hash out still, we currently are planning for Audrey to have surgery sometime next week to replace her pacer with an ICD to protect her in case of cardiac events such as the one she had Monday. We are so thankful to have so many resources such as the SADS foundation, friends in the medical field, and Doctors willing to spend their time to help us determine the best path for Audrey.

- Mark

Thursday Update

Pretty uneventful day here at Egleston. On one hand that is a good thing as Audrey's rhythms stayed pretty stable and she took all of her medications well. Her wide and bright eyes were back today and she was in a great mood and very lively all day.

On the other hand, I dont feel like we made very much headway toward our decision on a plan. Our EP spoke with a couple of other doctors, one in Italy who is one of the biggest proponents of the cardiac denervation and one in NY who is the EP for a family that we have kept in close contact with as their 4 year old son has the same condition and has followed our same path. Both gave some interesting input that we are taking into consideration. We were hoping to have another meeting outside of the CICU in one of their offices to have further discussion with a couple of the EP's but didnt get the chance. Almost every minute of the day and every conversation Amanda and I have, we are talking through our options and feelings around the procedures. It's so very intense and consuming for me right now as we attempt to make the right decisions for Audrey. As morbid and harsh as it sounds, the impact of these decisions could truly be life or death for our sweet little girl. We are trying to make certain that we talk to as many resources as possible and challenge our doctors on the options both presented to us and not presented. It is such a fine line as we want to ensure we do not offend our closest doctors, that we respect and value greatly, but also make sure we do our homework as Audrey's parents to explore the pros and cons of every option on procedures, locations, surgeons, etc.

We did get moved to the step down unit at the very end of the day so Audrey and I are sitting in our own room now with a couch/bed in the room. She was able to get some of her lines removed and has telemetry for her heartrate monitoring which allows us to take her out of the room and all over Egleston now, all while her heartrate is still being monitored. She is now able to crawl around in our room, sit in a high chair for meals, and play in a playroom across the hall. Crazy coincidence, it ended up being the same exact room that we had a year ago after Audrey was born. This was a huge step for Audrey as it was becoming painful to keep her held down in the CICU once she was her self again and had her energy back. Avery and Ryan are dying to come back down to the hospital to see Audrey, and visit the almighty gift shop.

Again, we sincerely thank everyone for all of your well wishes, prayers, and assistance.

- Mark

Recent Pic of Audrey with Avery and Ryan

Taken at Ryan's Tball game...

Wednesday Status

Audrey had a relatively stable overnight on Tuesday evening and then again on Wednesday. The last runs of arythmias occurred on Tuesday around 5pm although she has still had some occasional premature beats in the past day. She has transitioned once again back to the same two medications she has been taking at home although in a slightly higher dosage now. She has started once again drinking milk and we are attempting to get her to eat some solid foods such as cereals. We were waiting the results of her bloodwork that was sent off to Utah to analyze the level of Mexilitine in her system because one possible explanation could have been attributed to a switch in compounding pharmacies just two weeks prior. If her levels were very low then that could have given us a good cause to help explain Mondays events. The results were returned and her Mexilitine levels were good so that eliminates that possibility and we really have no true leads on the cause, outside of the fact that she has Long QT type 3 and it will always be a real possibility.

At this point our EP's are strongly recommending that we replace Audrey's pacemaker with an ICD (implantable cardioverter defibrillator) that would provide insurance against events such as Monday's by providing a shock to break the rhythm pattern. It would also operate as a pacemaker for the lower end pacing to ensure that her rhythm does not drop too low where LQTS type 3 triggers occur. At this point we are pretty comfortable with that surgery and the need for this device. The EP's are also recommending a cardiac denervation procedure that would ablate the nerves running to the heart that contribute to the triggering of these dangerous arrythmias. Although they are recommending that procedure there is quite a bit of research and discussion to take place before we are truly comfortable with this one. The ICD alone would provide some protection but we would not have done anything to try and prevent the incident requiring a shock. Our doctors and us are currently reaching out to some other leading doctors to discuss these recommendations.

If Audrey remains stable and continues to take her medicine correctly then the plan is to move us from the main CICU room into the step down unit where we are still monitored but would have our own room and a little more freedom. I believe Audrey would remain there for monitoring until we have a solid plan.

Thank you to everyone for the support, prayers, and well wishes. We truly appreciate it and feel very loved and supported.

I'll continue to update here rather than sending out emails and forgetting to send to someone or another.

-Mark

Back in the CICU...

As many of you know, we had Audrey's 1 year cardiology checkup scheduled on Monday following a great weekend where we celebrated her 1 year old birthday with a fun party. During that checkup she got pretty agitated and was crying quite a bit and then surprisingly threw up quite a bit. Despite that, the EP's were able to acquire all of the data needed from her pacer and saw that all of her rhythms from the past month continued to look good just as her first 9 months of life. We got her cleaned up and wrapped up our appointment and were on the way out of Egleston when I told Amanda I wanted to quickly use the restroom. When I came back out Amanda exclaimed to me that something was definitely wrong and was holding Audrey up out of her stroller. Audrey had clinched up and then passed out and Amanda ran back over to the Egleston desk and said she needed some help and Audrey continued to throw up and gag herself. We returned back up to the pacer clinic where they immediately hooked her back up for an ekg and noticed that she was having some runs of tachycardia that had not occured previously. While watching the monitor the Dr got startled and quickly said "Audrey! Audrey!" and kind of grabbed her to shake her out of a run of VT. They concluded that she needed to head over to the CICU for at least a day. When they were getting her settled into her bed there, they asked us to leave the room while they put her line in. When we returned the Dr told us how her heart had gone into complete fibrillation where she had no pulse requiring that they do compressions and provide shocks using the defibrilator a couple of times. The doctors termed that incident a "recessitated sudden death incident" that now puts her in quite a higher overall risk category. Extremely scary moment but at the same time very thankful we happened to already be there in the building where she could be cared for.

Fast forward to the email below that I sent on Tuesday 6/29 recapping that days status...

A quick update on Audrey. They have been monitoring her closely to watch for any more arrythmias after yesterdays events while they investigate also for a possible trigger to the event. She did have some runs of tachycardia this morning around 6 or 7 and then was pretty clear until about 5 when she had some additional runs. These were short bouts that she would come out of without any assistance but not good and not anything we have seen her first year. Although the decision has not been fully made yet, the conversation is starting up about the probability that we'll have to have surgery to put in an ICD rather than her current pacemaker to protect her against future incidents such as yesterdays. The harsh reality is that it was what they called a "sudden death incident" and would have likely been fatal in a lot of scenarios. The ICD alone will only protect her against those incidents and will also serve as a pacer as she has now but there also has to be some steps taken to try and avoid the fibrillation that requires those shocks. So along with adjusting the medications we have been just starting to discuss an additional procedure called cardiac denervation that will ablate some of the nerves connecting to the heart that trigger some of these events. That is not certain but discussions with some of the nations leading doctors on those procedures are being explored. We have always figured that the ICD would be probable someday but we were hoping to wait as long as possible but if Audrey absolutely needs that at this point then we'll definitely do it. We should know more in the next day or two as they continue to monitor her closely.

She is pretty stable now and doing fine and the biggest struggle is trying to get her to relax and rest as she is so active and wanting to move around. One of us is staying here with her each night and we both are here with her during the day as well and it comforts her a lot to be held and played with. The doctors and nurses are great with her as well and she is in awesome care and a great facility. We are so fortunate to live here near CHOA.

I'll give Wednesdays update in the next post.