Wednesday Night

Well, better late than never!  :)  Rebekah had a fairly good afternoon once we got some medications switched around and a little more pain medicine on board.  She was finally able to get some sleep, which would make anyone happier!  :)

I'll see if I can recap everything that happened today, partly because all of you are interested, and partly because I want to remember what the doctors said today.  Yes, I freely admit that if I don't write something down, I won't remember it!!

Before we saw Rebekah this morning, she had an echo in the PCICU.  This is perfectly routine, and all PC kids have an echo and a chest x-ray pretty much daily.  On Rebekah's echo this morning, there were three things that the doctors noted.  One, the function in Rebekah's right ventricle was diminished from yesterday's echo.  Two, there appeared to be a small area at the edge of the new conduit (the one that connects her collateral artery to her pulmonary conduit) that could have been the beginnings of a blood clot.  That is serious because this new conduit is only 6mm in diameter.  It would take only a very small clot to block that conduit completely, and that would be very, very bad; potentially fatal, even.  Third, Rebekah's pulmonary pressures appeared to be higher than what they were when she came out of surgery.  Also, during Rebekah's chest x-ray this morning, as I mentioned in my earlier post, she had several areas of atelectasis (collapse) throughout her lungs.

Also today Rebekah's potassium was on the low side, so she did have to receive a bolus (one single dose) of replacement potassium.  As of this evening's lab, her potassium was at a low-normal level.

So, as I said in my earlier post, Rebekah began CPT (chest physical therapy) today to try to open up the collapsed areas of her lungs.  She is having CPT every six hours around the clock until her lungs clear.  Tonight's x-ray did show some improvement, although the right lung is still quite hazy.  It's hard to get a really good look at her left lung because her heart is so large!  :)  Another thing that is helping with Rebekah's lungs is that she had negative fluid output today, which is great!!  That means that she expelled more fluid today than she took in.  Normally that's not a great thing, but it is terrific right after surgery! She needs to get rid of the extra fluid that has accumulated over the last couple of days.  The negative output is a direct result of the two diuretics (Lasix and Diurel) that Rebekah has been getting regularly for the last 24+ hours.

If you have followed Rebekah's blog since her last surgery, you may remember that Rebekah has taken Sildenafil (more commonly known as Viagra) for about a year.  It was discontinued right after surgery, but with the higher pressures in this morning's echo, the doctors decided to resume Rebekah's Sildenafil.  They also increased the dosage to 4mL's three times a day, which is a significant increase from her previous dose.  It would seem that the Sildenafil is already beginning to work since this evening's echo showed the pressures down to the level that they were when Rebekah first came out of surgery.

As for the small area that might potentially be the beginnings of a clot.....Rebekah is now a member of the Aspirin club!  Many, many heart kiddos take aspirin, and I have truly been surprised that Rebekah has avoided it this long.  However, she will be on a small dose of aspirin from now on, probably for life.  It is vital that her conduit not clot off, as it truly would be a life-threatening condition.  The only change that the aspirin means is that we will have to be a little more careful about any cuts, scrapes or bumps that Rebekah might get as she will certainly bleed more easily.

The thought for the decrease in right ventricle function is that the heart is simply bruised and hurt from Monday's surgery.  It will take a little time for it to heal and be completely back to Rebekah's "normal" function.  As of this evening's function, the right ventricle was already showing some improvement from this morning.

Rebekah continued to gag and retch for a good bit of today, even with little to nothing in her stomach.  I asked (begged, pleaded, pretty much demanded) for the doctors to switch her nausea medication from Zofran to Phenergan.  The Zofran is a more mild medication, and it just was not helping Rebekah at all!  After Rebekah received her first dose of Phenergan (around 2:00), she really seemed to relax and rest more easily. She still had periods of retching and gagging, but far fewer incidents than before. We were so thankful that the Phenergan worked to bring her some relief!

Dr. Bradley came by to check on Rebekah around 5:30 this evening, and he is still very pleased with her progress.  In fact, Rebekah is doing so well, that he decided to remove her pacing wires and chest tubes.  The pacing wires are actually leads he puts into her heart during surgery and leaves the ends exposed on the outside of her body in order to hook them up to an external pacemaker if necessary.  Thankfully, Rebekah's heart rhythm has remained normal and she has never needed to be "paced."  The chest tubes drain fluid that accumulates in the chest cavity following surgery, and Rebekah had a surprisingly small amount of fluid over the last 48 hours.  In fact, she had less than 20mls of fluid output from her chest tubes all day!  The lack of fluid is definitely a huge answer to prayer, as this was one of the more troublesome complications that we were warned could be a problem this time around.  Thank you all for praying!!!

Before Dr. Bradley took the chest tubes out, Rebekah got some IV morphine and some Versed so that she would be more relaxed and not feel too much pain from getting the tubes out.  She was great!! She cried just a tiny bit, but for the most part she lay very still and let Dr. Bradley do his job.  Rebekah has been scratching at her incision often today, so she got some Benadryl a few minutes after Dr. Bradley was done.  We were joking that she had quite the cocktail of drugs tonight!  Hopefully it means that she will sleep well!

And speaking of sleep, I think I finally have everything documented from our day.  Now it's time for me to sleep!  :)  Sorry this was a very technical post, but I didn't want to forget anything.  I never know when some obscure detail will be important later on!  I will make sure I get some more pictures up tomorrow.

Thank you all again for so faithfully praying for Rebekah.  We are forever in your debt!

Nancy

Update #7

This should be the final surgery update for this surgery!!!  Yippeee!!!  Dr. Bradley surprised us by coming into the waiting area before we got the text that he was finished.  It was a very nice surprise to see him!  :)

The first good news we had was that Dr. Bradley was smiling!  Those of you who know him in real life know what a good sign that is!  :)  He said he was very, very pleased with how things went, and that Rebekah tolerated everything well.  They did put her on the heart/lung bypass (ECMO) as we suspected, and they did replace her original conduit.  Dr. Bradley was able to put a little bit bigger conduit in than what she had before.  I believe she had a 10 or 12mm conduit before, and he replaced it with a 14mm.  So, not a big change, but one that he felt was necessary.

One surprise we had was that in the sedated echo right before surgery, the doctors noticed an area of enlarged muscle just under her conduit.  It could be that this area was also a contributing factor to the high pressure in her heart/lungs.  Anyway, Dr. Bradley was able to cut away some of that muscle to allow better blood flow and he removed some scar tissue around it.  The new conduit that Dr. Bradley put in actually has a human donor valve in it this time around.  Rebekah's previous conduit had a bovine valve.  He said there should not be any noticeable or medical difference to Rebekah with one valve versus the other.  I get the feeling that it is more of a decision of what they have on hand to use.

SO....the big question....YES!  Dr. Bradley was able to attach Rebekah's collateral artery to her pulmonary conduit.  He had to use an additional conduit (tube) to make the collateral stretch far enough to attach it.  Drew asked Dr. Bradley if that smaller tube/conduit would need to be replaced sooner than Rebekah would have otherwise needed her larger pulmonary conduit replaced.  Dr. Bradley just smiled and nodded a little bit and said, "It's likely."  We'll cross that bridge when we get to it.  For now we're thrilled that she will be able to have some use of her right lung!!!

So, all total Rebekah was in the OR about 10 hours, with somewhere between 8 and 9 hours of actual surgical time.  We know that she is back in the PCICU because all the other parents got kicked out when she started rolling.  (For those of you not familiar with PCICU procedures, when a case comes back from the OR, or when a new case comes into the PCICU for the first time, it is called "rolling."  As in the bed is "rolling" into the PCICU.  It is the only time that parents/visitors are not allowed in the PCICU.)  We were told that we could go in to see her around 5:00, so we have about 20 minutes or so until we can kiss her little cheeks.  :)

The plan is to take recovery very slow.  The doctors are going to monitor Rebekah's pain, breathing and heart rhythms for a few hours.  When they are sure she is stable, they will slowly allow her to start waking up.  From there it will just depend on how fast her body wakes up and recovers from surgery.  Dr. Bradley did do a lot during surgery today, so it will take a little while for her body to adjust.

Thank you all for praying for us and Rebekah today.  We are just so, so thankful that God worked through Dr. Bradley to help our little girl.  We are certainly blessed beyond all that we could ask or hope.

Nancy

Heart Cath. August 16th

The summer is coming to a close around here, and we are preparing to start home schooling next week with Rebekah's brothers. We are also heading to Charleston next week for Rebekah's scheduled heart catheterization on Tuesday at MUSC. Rebekah is scheduled for first case on Tuesday, so we have to check in at the hospital at 6:15 in the morning. She should be going back to begin the catheterization around 7:00. If all goes well, it should be a three to four hour procedure. We are anticipating that Rebekah will be spending one night at MUSC for observation, and we should be coming home on Wednesday.

Please pray for us as we travel to MUSC, about a 3 to 4 hour trip, on Monday. Also pray that the doctors would be able to find a cause for Rebekah's fatigue and come up with a plan to help her. We are also praying that the pressures in Rebekah's heart and lungs will have come down from the dangerous levels they were at in the spring.

We praise the Lord for his providing and care of our family. We trust and know that the Lord will carry us thru all of our trials.

Thanks for supporting our family in prayer and love,

Drew

Disappointed

Well, this has been quite a week! Monday morning Rebekah's EI (early interventionist) called and said her daughter was sick with a stomach bug. So, Rebekah had a day off from therapy! :) This week Tuesday should have been our Ladies' Bible Study at church (it's roughly every other week), but our teacher was sick, so that was cancelled as well. Then came Wednesday and cardiology. I'll get to that in a minute. This morning I got a phone call from Rebekah's OT (occupational therapist) who was running a fever and wasn't coming over today. What a mixed-up, inside-out, upside-down week! :) I'm glad we don't have anything scheduled for tomorrow!

Rebekah is "feeding" her babies.

I snapped these pictures post-cannula-incident,

so that's why she doesn't have her oxygen on. :)

Wednesday, dear Wednesday, where do I begin? Rebekah and I headed out to her cardiology appointment first thing in the morning. It was a routine visit, yet we were anxious to see if the weeks of oxygen therapy had lowered Rebekah's pulmonary pressures any. Once we arrived at the appointment, we went through all the preliminary stuff (height, weight, blood pressure, o2 sats - 100%!!, etc.), then we waited our turn to go to the echo lab. I was excited to have one of our favorite echo techs. Rebekah cooperated fairly well, and only got a little fussy at the very end. Then Dr. Lucas walked in. I knew when his first words were, "I'm not pleased with the results of Rebekah's echo," that it was all downhill from there.

Time for Baby #2 to have a drink.

We really need to come up with names for these babies!

Drew and I were very disappointed to learn that the oxygen therapy Rebekah has been on has not lowered her pulmonary pressures at all. As a matter of fact, Rebekah's pulmonary pressures are significantly higher than they were at our last appointment in December. Whether the oxygen helped and kept the pressures from going even higher, or whether it did nothing at all, we aren't sure at this point. But, just in case it is helping keep the pressures from getting any higher, Rebekah will continue to stay on oxygen until a decision has been made about where to go from here.

Oh, this little girl is so cute and sooo sweet!

Nope, I'm not biased at all! :)

So, the elephant in the room.....where do we go from here? That's a good question, and one we are waiting to have answered. Dr. Lucas was concerned enough to make a call to Charleston, and we are currently waiting to hear back from them. Rebekah already has a heart catheterization scheduled for March 7. It is likely that we will keep that appointment. However, Dr. Lucas does not want to be the one making that decision. He wanted to let Dr. Bradley and the surgical team in Charleston evaluate Rebekah's pressures and decide whether or not they are going to bump her cath date sooner. The only reason that we are not currently in Charleston at this moment is that Rebekah's heart, despite the ridiculously high pressures, is "happy." As long as Rebekah's heart is tolerating the increased pressure, then she is not in imminent danger of heart failure. If we begin seeing signs of heart failure (hopefully not!!), her case will begin to take precedence over other, more healthy children. There is a definite pecking order among heart patients. This is one pecking order that you would rather be on the bottom of! Those at the top are the most serious, critical cases, and that's never a good spot to be in!

Look at those curls!!

Okay, so once we get to Charleston (sometime between now and March 7), there are a few options for Rebekah. The first, and by far the best, is that the cath will reveal that the pressures are not as high as the echo is estimating them to be. The echo is a great tool, but it is not an exact measurement. Basically, it reads sound waves bouncing off blood flowing through the heart and gives a reading which is then translated into a formula to give a close estimation of the pressures in Rebekah's heart. During the cath procedure, there is a catheter that is inserted into the conduit in Rebekah's heart which gathers an exact measurement of the pressure. Even if the echo is estimating the pressures higher than what they really are, they would still be too high.

Just a side note here, Rebekah's blood pressure on Wednesday was 88/67. The 88 (top number) is the systolic. The 67 (bottom number) is the diastolic. Rebekah's pulmonary pressure should be somewhere between 1/3 (normal) and 1/2 (acceptable) of her systolic number. So, that would put her pulmonary pressure somewhere in the range of 29-44. Rebekah's estimated pressures on Wednesday were in the 90-100 range. So, even if the estimate was, say, 20 or even 30 points high, that would still be too high to be safe.

We are really beginning to suspect that Rebekah will be left-handed!

Since Rebekah's pulmonary pressures have been almost equal to (or sometimes greater than) her systolic pressure (also called systemic) since she came out of her last surgery, it is doubtful that there is anything that the doctors will be able to do in the cath lab to relieve that pressure. Do you remember almost exactly three months ago, I wrote this post detailing our conversation with Dr. Bradley about Rebekah's high pressure and the possibility of some narrowing around her conduit? Well, the result of that conversation was a trip to have a CT angiogram which showed no signs of narrowing anywhere around the conduit. That was good news. The bad news was that there was nothing to balloon or stent in the cath lab to open up the conduit more. It was wide open and still Rebekah's pressures were high. In that post, I wrote that Dr. Bradley told us his goal was to get Rebekah stable enough to come home and recover from surgery, and in about three months come back and re-open, or partially re-open, her VSD if her pressures were still high. That's where we're at, folks.

On the (all too infrequent) days that I scrub my kitchen floor,

the kids get to enjoy a tent in the living room.

Rebekah thought it was great fun!

One of the other concerns that Dr. Lucas and I talked about is Rebekah's restless sleeping patterns. She is no longer the great sleeper that we had before her last heart surgery. Now she tosses and turns, moans and groans all through the night. She is "asleep" during all of this, but it is obvious by the dark circles under her eyes that she is not getting quality sleep at night. One potential reason is that the pressures in Rebekah's heart keep her in a constant state of adrenaline. If you or I had a few cups of coffee every night before bed, it is likely that we would be very restless sleepers, as well!

The other possibility that we discussed for Rebekah's restless sleeping is that she may have sleep apnea. She does not have the classic "freight train" snoring of typical sleep apnea sufferers, but that alone does not rule out the possibility. Because of the atypical formation of Rebekah's palate and pharyngeal arch (basically the back of Rebekah's mouth and throat), it is quite possible that she does have a degree of sleep apnea. In fact, I just read an article this week, in the VCFS newsletter that documented the higher incidence of sleep apnea in children with DiGeorge and velocardiofacial syndromes. I won't bore you with all of the medical details, but in a nutshell, the physical formation of the mouth and throat, combined with low tone in the soft tissues of many kids with DiGeorge and VCFS, is a prime combination for sleep apnea. Once Rebekah's current heart issues are resolved, we will be referred for a sleep study if we do not see a dramatic increase in the quality of her sleep.

Such a sweet girl!

Drew and I are understandably disappointed by this week's findings. But we are so thankful to know that none of this took God by surprise. He knows Rebekah's heart. He made Rebekah's heart, and we can rest in Him, knowing that He loves Rebekah even more than we do. We are so blessed that God trusted us to care for our sweet baby girl. She is such a joy, even on days that she is not feeling well.

Our biggest request for now is wisdom. Please pray for wisdom for Dr. Bradley and each of the doctors that will be discussing Rebekah's care. Pray for Drew and I to have wisdom and discernment in Rebekah's care. It would be so easy to jump at every little sniffle or "off" day, thinking that Rebekah is showing signs of heart failure. Pray that we will know when Rebekah is just being a normal toddler and when we need to call the doctors.

In closing, Isaiah 25:1 has been on my heart this week. It so perfectly states what I have been reminding myself of all week.

O LORD, you are my God;

I will exalt you; I will praise your name,

for you have done wonderful things,

plans formed of old, faithful and sure.

~ Isaiah 25:1

Nancy

P. S. I just learned of a new little (unborn) baby who was recently diagnosed with Truncus. His name is Lukas, and his button is now on the left sidebar of Rebekah's blog. I know his family would appreciate your prayers as well. And, our sweet buddy, Owen, will be leaving Feb. 8 for a significant surgery that will determine whether he is a candidate for the next surgery he needs, or if he will need a heart transplant shortly. Please pray for Andrea and Owen as they fly, and especially for the surgery to go well. I sure hope you get to "meet Buzz Woody," too, Owen!!

Happy New Year!

I just realized that it has been a while since I last posted an update on Rebekah. The bad news is that I am falling behind in my blogging. The good news is that there hasn't been anything to update on! :)

January brings in a round of doctors visits for Rebekah. I know, what's new, right? :) This coming week Rebekah will see her pediatrician (for a very belated 15-month check up), her audiologist for a re-check of her ears, and her gastroenterologist for a routine visit. We are not anticipating any changes or issues at the pedi or audiologist appointments. During the GI appointment, I am hoping to discuss a plan to wean Rebekah to a much lower volume of formula through her g-tube, and ultimately, a plan to wean the g-tube use permanently. After being given the go-ahead from Rebekah's cardiologist to cut back her g-tube feeds, we have decreased the volume Rebekah gets overnight from 40 mL's per hour to 35 mL's per hour. I know that doesn't sound like much, but over the course of her 12 hour feed, that adds up to two ounces less per night. And Rebekah is definitely responding to the decrease by eating more! Like all of us, she has days when she eats more and days when she eats less, but overall we have seen an increase in the volume of food she is taking in orally. Please pray that this continues and that Rebekah continues to make forward progress in the growth department while decreasing her g-tube feeds!

Rebekah also has a pretty significant cardiologist appointment this month. On January 11 she will go in for a regular check-up and echocardiogram which will tell us if the oxygen therapy is doing anything to help lower her pulmonary hypertension. Please, please pray that her numbers have begun to come down. Pulmonary hypertension will take a toll on Rebekah's body at some point, and we would love to see those numbers lower. In the same regard, please continue to pray for wisdom for all of Rebekah's cardiac team in knowing when/how to treat her high pressure.

Also on the calendar for January is a follow-up appointment with Rebekah's ophthalmologist. We last saw him in September and decided to wait on any possible treatments until after surgery. Now we are post-surgery, and we will be discussing possible patching and/or glasses to try to strengthen Rebekah's right eye. I have noticed that her right eye has been turning inward more than before, especially if she is looking at or playing with something closer to her face. I am definitely going to be mentioning it to the doctor at our appointment.

One other thing that is not on the schedule, but will be soon is a trip to see the orthopedic doctors at the Shriner's Hospital here in Greenville. Our physical therapist has mentioned it a few times, but again, we were waiting until Rebekah was post-surgery to schedule that appointment. In the last few weeks, Drew and I have felt Rebekah's hips popping when we play with her. It is probably nothing of significance, but we want to make sure there is not a problem we need to address.

Rebekah is all cleared to begin her therapies again this week. We are excited about that! It will be good to see our therapists again. And I must admit, I'm anxious for them to see how far Rebekah has come since surgery. She is really beginning to take off in all areas of her development.

I guess that's all for now. Rebekah has been well since her last hospital visit, and we are praying that continues! For all of our SRBC family, please know that if we are not in services it is because we are trying to keep Rebekah well. Happy New Year! We love you all!

Drew, Nancy, Zac, Justin, Aaron, Caleb and Rebekah Grace

There's no place like home!

I just wanted to post a quick update to let you all know that we are home!!! Rebekah was very excited to see her brothers, but she was very tired. She went to bed at 7:00 and we haven't heard a sound from her since! I'm sure she is enjoying being back in her room and in her crib. I know I am looking forward to sleeping in my bed tonight!

Rebekah did come home with oxygen, and she will be staying on it until at least her next cardiology appointment. Unlike before her surgery, Rebekah does not need the oxygen to keep her sats up. Rather, this is called "oxygen therapy." It some studies, oxygen has been shown to lower pulmonary hypertension, so we are going to try oxygen for the next month and then see if it has helped. If Rebekah's pressures are lower, she will likely remain on the oxygen until her heart catheterization in March. If there is no change in the pressure, Dr. Lucas will probably take Rebekah off the oxygen. We are hoping that the oxygen will lower the pressure in her heart at least a little bit. If going back on oxygen is a way to do that, then we are willing to try it. I'm hoping that she will be on oxygen until March!

Thank you to everyone for your prayers while we were in the hospital. Please continue to pray for Rebekah. She still has a cold, but it seems that she is getting over it. Pray that she will continue to be as healthy as possible through the winter months.

Nancy

December 2010

It has been a few weeks since our last prayer request post, so I thought I would update the list! We are so appreciative for all of you who check on us and faithfully pray for our family.

Rebekah is doing so well, and we are thrilled to see her almost back to where she was prior to her surgery! She has been walking with one of us supporting her, even though she tires easily. We are still hoping to see Rebekah's endurance level bounce back some, but we are aware that with her current heart configuration and high pressures, that may be some time in the future.

As you know from reading previous posts, Rebekah is now scheduled for a heart catheterization on March 7 to check the pressures in her heart. Our prayer between now and then is that the pressures in the right side of her heart will go down on their own and not require any surgical intervention. Also, please pray that the doctors will have wisdom in making decisions regarding how high is "too high" for Rebekah's heart pressure.

In regards to the catheterization itself, please pray that it will go smoothly and Dr. Baker will be able to get the IV access he needs. After Rebekah's last cath, we found out that Dr. Baker was not able to get all of the information he had hoped for because he could not thread the catheter everywhere that he needed to. Rebekah will be almost a year older this time around, so I'm hoping that will have given her (very tiny) veins some time to grow a little more.

Please also pray for wisdom for Drew and I. Before surgery, we knew Rebekah's limitations and were comfortable working within them. Now we are having to learn a new set of parameters to work with. We are still trying to get a feel for when to push Rebekah harder and when to back off and let her rest. Over time she will show us how she can handle things, but right now we are still a little unsure of what her limits will be.

Rebekah's eating will be another area we need to address in the next few weeks. Again, if surgery had been a complete success, we would have been given the green light to push Rebekah to eat more orally. Rebekah has an appointment with her cardiologist on Dec. 8 and her GI doctor on Dec. 13. We will be discussing how far we can push Rebekah to eat orally and how much we will still rely on the feeding tube until at least March. It is amazing when you stop to think about how intricately our bodies are made. Everything depends on everything else! I am continually reminded of Psalm 139:14 "I praise you, for I am fearfully and wonderfully made. Wonderful are your works; my soul knows it very well."

Thank you all for the prayers and support you have given our family. So many of you we have never even met, some of you have taken care of our sweet girl in the hospital or doctor's office, and others of you are close friends. We love and appreciate each and every one of you!

Nancy

One Week Post-op

Today marks just one week since Bekah had her open heart surgery. What an amazing difference a week can make! As I type this (well after Rebekah's normal bedtime), she is sitting up in her crib and playing with everything in reach. She is "talking" about whatever it is she finds. This is the best she has looked since before she had her surgery. It is absolutely amazing how just a few hours make a huge difference. Literally five hours ago, Rebekah had a 101.1 fever, had to go up on her oxygen a little bit, and was very miserable. Now, the fever is gone (she has had Tylenol), she is down to 0.2 liter of oxygen, and she is having a blast playing in her crib. Thank you all so much for praying for our little Bekah-boo this afternoon. Obviously, prayers were answered!

So many people have asked us when we are coming home, and the short answer is: we just don't know until we are walking out of the hospital! As with today, any little thing can change at any minute, and the best plans can go awry. The best answer I can give is that the doctors would like Rebekah to totally be off oxygen, her lung to reopen all the way, and she has to be fever-free for 24 hours. Rebekah had another echocardiogram today to check the function of her right ventricle. We have not yet heard what that looked like. At the very least, it will need to remain unchanged, or preferably better than the last echo, in order for us to go home. If the echo shows the heart function is declining, the doctors will have to decide what they are going to do to stabilize her heart function.

Rebekah is on a blood pressure medication now to lower her overall blood pressure. Most likely she will be on it for at least a few months, or unless her heart function improves dramatically. So, she will be going home on an increased dosage of Lasix and a blood pressure medication called Enalapril. She has had two days of the Enalapril so far in the hospital and seems to be doing well on it.

I think that's about the extent of today's news. By the time I reached the end of this post, Rebekah is now fussy, tired, and feels like she is starting to get warm again. The nurse should be back with some pain meds soon. Here's hoping we can both get some sleep tonight!!

Nancy

Quick update

We just kissed Rebekah and tucked her in for the night. All things considered, she had a pretty good day. Rebekah was awake for a good portion of the day, although she seemed to be very restless and unable to settle into a deep sleep today. The nurses were good about staying on top of her pain medication, so we aren't sure if she was in more pain that we realize, or she liked the morphine drip a little too much, or if something else is brewing. Her lungs still sound pretty junky this evening, but she has been sitting up (with assistance) and has had a few good coughs today.

One of the big questions we had after Rebekah was extubated was how orally averse she would be. So far, she has not wanted her little taggie blanket or her fingers in her mouth. Prior to surgery, she would stuff a corner of her taggie in her mouth, along with two of her fingers and that's how she would fall asleep. For her not to want that in her mouth is a big deal. So, we were concerned about how well she would go back to feeding. Rebekah did take her sippy cup a few times today and drank a few swallows of milk. That was a great start! She refused any cheerios, and she gets very agitated when she sees anything coming toward her mouth - even my finger with some lip balm, which she loves! So, we may have to work on getting her used to things being in and around her mouth again.

Rebekah is also very weak now compared to where she was pre-surgery. At this point, she cannot sit up without assistance, or even hold her head up on her own. She is scooting around in her crib just a bit, mostly just moving her legs or arms around. We will definitely have some ground to make up once she is feeling up to moving. Today was unbelievably busy in the unit, but I am planning on talking to the doctors tomorrow about when we can start PT and OT in the unit.

Rebekah's pressures remained high pretty much all day. Her heartrate and blood pressure were also higher than they has been in the last few days. Right now the doctors are waiting for another day to see if her numbers go down any. There is a possibility that Rebekah may start on a blood pressure medication, but the doctors would like to give her one more day to get her lungs a little more clear and a little more fluid off before determining whether or not she needs the blood pressure medicine. Rebekah's temperature has also been climbing throughout the afternoon, and when we left it was around 100.6. It has been up and down since she came out of surgery, but we're hoping it starts staying down soon!

Thank you all for your prayers for Rebekah! It means so much to our family!

Nancy

A New Week!

I know it's small, but here's another picture of our sweet girl. :) If you look really closely (or click on the picture and make it larger), you can see she's wearing her pink pearls today with a pink bow in her hair. Cuteness!

Oh, if you look really closely, you can see Rebekah's "Jaxon's blankie" underneath Rebekah. We love the blanket that Jaxon's mom, Lacey, made for Rebekah. Congratulations, Lacey, on the homecoming of sweet little Arina!

Pretty in Pink!

Rebekah sailed through her CT scan this morning. Actually, she had no clue where she was or that anything was happening! You have no idea the production that it was to get Rebekah moved! Literally, it took us 30 minutes to get Rebekah ready for transport, transported, and settled for the CT scan. The scan itself was about 10 seconds. Literally. There were two doctors, two nurses, a nurse's assistant and a respiratory therapist who had to go with us. We were moving Rebekah in her crib, two IV poles with 2 brains each (that's the system that runs the IV pumps) and each brain has 4 pumps on it. Then we had 2 monitors to keep track of all her numbers, plus the oxygen and the 2 emergency airway bags in case she stopped breathing. Oh, and the whole time we were gone, the respiratory therapist was bagging Rebekah because the vent isn't portable. We barely managed to fit all of us in the elevator, and we took the largest elevator in the hospital. I took some pictures of the procession and will try to upload them when I get home. It was a sight to see!

Anyway, the end result of all that effort is that Rebekah's conduit is fine. There is no narrowing anywhere in the conduit that could be resulting in higher pressures. We were thrilled to hear that!! Dr. Bradley came by (very) early this morning and messed around with some of the probes monitoring Rebekah's pressures. Right now, the pressures are measuring much lower than they were yesterday, so we are waiting for an echo to accurately read the pressures and let us know if they are truly that low. It would be great if they were!

Rebekah's morphine drip was turned off about an hour ago, so she will be gradually waking up over the next few hours. The plan is to extubate sometime this afternoon. We're very hopeful that Rebekah will do well off the vent and will be able to keep her oxygen levels up where they need to be. That will certainly be a good test of how the pressures are going to do in her heart when she is having to do more work on her own.

That's all the news for now. Please continue to keep Rebekah in your prayers!

Nancy

Dr. Bradley

First, I have to apologize for the blurriness of the picture. My laptop needs a new hard drive, so it's not working. Drew's computer does not have any working USB ports, so between the two, we can't upload any pictures from our camera. I was able to get this picture from my phone and text it to blogger. That's why it's not such a great picture. But, I figured it's at least a picture for everyone to see! :)

Daniel & Kelsey - your bear is being put to good use! She's holding down Rebekah's legs! :)

Anyway, we finally were able to talk with Dr. Bradley this afternoon. He checks on Rebekah frequently, but we never know when he might come in, and we have happened to miss him each time he has been here. He is concerned about the high pressures in Rebekah's heart, and said that if left for a long period of time, it would "definitely take a toll on her heart." That being said, he agrees with what the cardiologists are already doing in waiting to get more fluid off to see if the pressures improve. (Which, by the way, Rebekah has been doing a great job today getting rid of fluids!!)

One thing that we were sort of misinformed about is Rebekah's conduit. Yesterday we had been told that the conduit was narrowing, which seemed a little odd to us. Today Dr. Bradley told us that it is not the conduit that is narrowing. It is either the area where the left pulmonary artery attaches to the conduit, or the area on the left pulmonary artery where Rebekah's band was that had to be patched. Either of these two areas could potentially narrow, but not the conduit itself. To get a good look at any potential narrowing, Rebekah will be having a CT angiogram done tomorrow morning at (roughly) 9:00. This will give Dr. Bradley a clearer picture of exactly what, if anything, has narrowed. If there has been any narrowing, a trip to the cath lab to place a stent and/or balloon the narrowed area should fix that problem. Dr. Bradley's hope is that Rebekah's heart can tolerate any potential narrowing for a few months so that she does not have to have another invasive procedure so soon after surgery.

The other (bigger) concern is the pressure in the right side of Rebekah's heart. Dr. Bradley did not specifically say one way or the other, but from his comments Drew and I both get the feeling that he is anticipating having to open (or partially open) the patch on Rebekah's VSD. This would be a full blown open heart surgery again. Again, Dr. Bradley is hoping Rebekah can tolerate the pressures for a few months before we have to go that route. Dr. Bradley's end goal is for Rebekah to tolerate the pressures for now, go home and recover, and in 3 months or so come back for another heart surgery to open her VSD. Of course, the possibility is great that once Rebekah starts weaning off the sedatives, her pressures will become higher than her heart can tolerate, even for a few months or weeks, and she has to return to the OR sooner than expected. This is just something we will have to watch and see what happens over the next few days.

As a whole, Dr. Bradley is very pleased with how Rebekah looks. Her lungs are doing well, although still "junky" from surgery, she is starting to breathe over the vent, and her oxygen and heart rate are stable. Dr. Bradley said if you don't look at the pressure numbers, you would never know by looking at her that her heart isn't "fixed."

Rebekah is now on the lowest ventilator setting, and she will begin CPAP trials tonight. That is when they stop giving her breaths on the ventilator and she has to take each of the breaths on her own. She will still be breathing in concentrated oxygen, but she will have to do all of the breathing on her own. As long as her numbers look good, they will CPAP her for an hour and then draw a blood gas to see how well her body responded to breathing on its own. As long as she tolerates it well, they will probably CPAP her three or so times through the night. Rebekah does have to remain on the vent for her CT scan in the morning, but she could be extubated tomorrow afternoon if she does well during her CPAP trials.

Overall it has been a nice, quiet day today and we are hoping for a quiet night. Thank you to everyone who has been praying for Rebekah this week. We love you!

Nancy

Sunday Morning

Good morning to all! Rebekah had a very stable night, but made no improvements. She had done so well peeing off fluid for a couple of hours last night, but overnight slowed down considerably. The doctors have started a Bumex drip (constant IV drip) in addition to the Bumex and Diurel meds she was getting every six hours. The doctors don't want to make a lot of adjustments or try anything new to get Rebekah's pressures down until the fluid is resolved. So, right now we need lots and lots of fluid output!

In other news, the pressures that we thought had dropped some yesterday afternoon have not really dropped at all. Late last night (much later than my last post), Rebekah's nurse and I realized that the pressure probe inside Rebekah's conduit was not reading accurately. So, after flushing the line, we got a very accurate reading. Rebekah's PA pressure is almost equal to, and sometimes higher than, her systemic pressure. This is not the trend that the doctors wanted to see! They had really hoped to see those numbers growing farther apart. But, again, they are not ready to make a move toward intervention at this point until a lot of that fluid is resolved and we can see how her heart reacts under less stress.

We are at the hospital now, but have not been able to see Rebekah because the doctors are doing a procedure. Whether or not it is on Rebekah or another child we have no idea, but it will be a little longer before we are able to see Rebekah this morning.

Hope you all have a great Sunday!

Nancy

Update

The good news is......Rebekah has started to pee!!!!! It is with the help of some heavy-duty doses of diuretics, but at least she is getting some of the fluid out of her body. We are very excited that she is responding to the medications finally.

As far as the pressures go, Rebekah's LAP and RAP have come down considerably. She has been hanging out in the 15-17 range except when she moves or wakes up when the numbers shoot up to 30+. The PAP (measures the pressures in her new conduit) has still remained high, at times only 3 or 4 points above the systemic pressure. We are still in the post-operative waiting period, though, and the doctors want to wait and see how her heart is doing over the next couple of days before attempting any other interventions.

Rebekah's oxygen saturations have dipped a little lower today, but still much higher than we are used to seeing them. :) Mostly today they have been hanging in the 95-97 range. Rebekah's heart rate has been very steady all day, and her nurse told me that is a good indication that her heart is doing well and there is no bleeding inside her heart.

The goals for Rebekah overnight are to rest calmly, continue to get rid of more fluid and rest calmly. :) For the last few hours, Rebekah has been resting much better. We hope that trend continues overnight.

We enjoyed spending time with Danny and Kelsey Rowan today. They drove down from Greenville to see Rebekah and bring her an adorable teddy bear. It was nice to visit and have some adult talk that didn't completely revolve around medical terms. :) Thanks, Danny and Kelsey, for giving up your Saturday to come and see us!

Please continue to pray for Rebekah tonight. She is stable, but still has a lot of recovery ahead of her. Also, please keep two other families here in the PCICU in prayer. Their little ones are not doing as well, and I am sure that they would appreciate your prayers.

Due to the other issues going on in the PCICU, and a very sick baby in the PICU as well, the doctors have not officially rounded yet tonight. Drew and I were hoping to talk with Dr. Graham about Rebekah's heart function and the time line that he has in mind for monitoring the pressures. It could be quite late when he is available, so we'll see how long we can stay up. We may wait until tomorrow if it gets too late.

Nancy

Not out of the woods yet....

So overnight Rebekah was stable, albeit with high pressures. Today, not so much. She has been having frequent breakthrough periods of startling awake, which causes the pressures in her heart to jump dramatically. The LAP and RAP pressures that I talked about in the previous post (normal is high single digits, post-op normal is low teens, Rebekah has been running close to 20) are jumping up into the 50's during these startle periods that Rebekah is having. Yeah, that's super high and not really great on her heart.

The doctors have also been doing echocardiograms every six hours (at the bedside). Overnight, they noted moderate dysfunction in the right side of her heart. The good news is that it had not gotten any worse this morning. The bad news is that it has not gotten any better, either. You can be sure this is something that the doctors are keeping a very close watch on. They also confirmed that the conduit has narrowed, and they are watching it closely to see if it is narrowing any further.

Believe it or not, Rebekah's heart pressures and function are not the main concern of the doctors right now. The biggest concern is that Rebekah is not peeing. I mean, like almost not at all. Like only 9cc's over two hours. And that was her "big" amount of the morning. The previous hour was only 3cc's. Rebekah has been getting two "big gun" diuretics since midnight, and she is still way overbalanced in her fluid intake/outtake. That fluid is also contributing to the already high pressures in Rebekah's heart. She is also much more swollen today than when we saw her yesterday due to all of the fluid retention. There is another diuretic that the doctors are probably going to try later this afternoon if the noon doses of the other diuretics don't produce the results we need.

So, the two biggest concerns of the day are for Rebekah to start urinating (in large amounts) and for her to keep calm and still so the pressures in her heart don't skyrocket. As always, we will update with other news as things progress.

Good Morning!

Thank you all so much for your prayers, emails, comments and texts! Drew and I were able to get some much-needed sleep last night. I fell into bed around 10:30, and I heard nothing until about 7:30 this morning. It was wonderful!!

Rebekah, on the other hand, did not sleep so soundly last night. Her nurse said she tossed and turned through the night. They have not been able to come up with an effective cocktail of drugs to keep her sedated. She is on a morphine drip, IV injections of Versed and morphine in between, and they also added in Precedex. All of that, and she still had a restless night! Our little girl is a fighter! The doctors would still like her to be sedated throughout most of today to let her heart and body heal from the surgery. Also, all of that moving and thrashing has the potential to rip out tubing, IV's, pacing wires and chest tubes, not to mention the ventilator. So, if you would like to pray specifically, please pray that Rebekah can rest peacefully. I'm sure her nurse would thank you for those prayers. :)

We were able to talk with Dr. Graham (one of our favorites!!) before we left the hospital last night. He said the pressures on the right side of Rebekah's heart were disappointing, but they are going to hold off a few days to see if her heart begins to lower the pressure on its own before doing any more interventions. Basically, Dr. Graham gave us three potential outcomes.

1) The pressures in Rebekah's heart come down on their own and no intervention is needed. (Best case scenario)
2) The pressures in Rebekah's heart remain high, but her heart tolerates the increased pressure for a few months (with or without medication), and in a few months we come back for a heart catheterization to measure the pressures and make adjustments as necessary. (Not the best, but certainly not a bad option)
3) The pressures in Rebekah's heart remain high, and her heart begins to tire and doesn't want to keep pumping against that much pressure.....also known as heart failure. In this scenario, we would have to intervene immediately. Hopefully a procedure could be done in the cath lab, but there is a possibility of another trip to the OR if the pressures cannot be lowered by a cath procedure. (Worst case scenario)

As of last night, Rebekah actually had three different pressures that were high. The most concerning is the PAP (pulmonary artery pressure). She has never had this measurement before, because she did not have a pulmonary artery before yesterday's surgery. This measurement is a measure of the pressure that is in the new conduit taking blood from the right side of her heart into her left lung. Normal PAP pressure is about 1/3 of systolic blood pressure (the top number on a normal blood pressure reading you might have at the doctor's office). Rebekah's systolic pressures yesterday were in the mid 70's to high 80's. Her PAP was in the high 60's to mid 70's. As you can see, it is significantly higher than normal, and too high even for Rebekah's anatomy. The doctors would like to see it at least as low as half of the systolic number. So we are looking for numbers that are somewhere in the upper 30's to mid 40's. Based on a bedside echocardiogram that Dr. Bradley did yesterday, he feels that for some reason this conduit is beginning to narrow. Not good! This conduit is the passage way for an amount of blood that is already too large for the space that it is flowing to. That in itself is enough to create higher pressures, which Dr. Bradley had warned us about on Tuesday. If that conduit narrows, it becomes an even smaller space for that blood to flow through. I think this is one of Dr. Graham's more pressing concerns. If that conduit continues to narrow, or the pressures continue to remain as high as they are, or higher, we got the impression that Rebekah would be headed to the cath lab in the next few days for them to try to balloon that conduit open.

The other two pressures that are high are the LAP (left atrial pressure) and RAP (right atrial pressure). These pressures are a measure of the pressure in the top two chambers of Rebekah's heart. You know, the chambers which now have a hole between them to try to equalize some of the pressure building on the right side of Rebekah's heart. I'm not really sure what the plan is for correcting, or lowering, that pressure, except that the doctors are going to give it a few days and see if that pressure drops as the heart heals. Normal readings for those pressures would be high single digits (8 or 9). Normal post-op readings would be as high as the low teens. We saw Rebekah's pressures as high as the low 20's last night before we left.

Other than those numbers, everything else looks great! Rebekah's oxygen saturations are reading between 97 and 99%; those are numbers we have never seen! When she came in for surgery, her sats were between 75 and 80% on half a liter of oxygen, so we are very excited to see "normal" saturations!

That's about all for right now. I'm sure we will have more information to share as the day progresses, but I just wanted to get a quick post to update you on where we are right now. Thanks for praying!!

Nancy

This day is history!

Well, our history-making little girl has come through again! We just met with Dr. Bradley, and he is pleased with the outcome of the surgery. Given Rebekah's unusual anatomy, there really was no perfect solution or repair to her heart that could have been done, but we feel that this is a best case scenario.

Rebekah is in ICU and will remain sedated probably through the night and into tomorrow. They will gradually start weaning down the ventilator and Dr. Bradley is hopeful that Rebekah will no longer need oxygen after she recovers from surgery. No promises, but he is very hopeful.

As we discussed with Dr. Bradley yesterday, the pressure in Rebekah's right ventricle was a large concern today during surgery. Dr. Bradley was able to completely close Rebekah's VSD, but Rebekah does have high pressure in her right ventricle now. However, Rebekah's heart does seem to be tolerating the high pressure well. To help relieve some of that pressure, Dr. Bradley made a small hole in the atrial septum (between the two upper chambers of her heart). There is less risk to Rebekah's heart to have a hole in the atrial septum rather than the ventricular septum, so this is the better of the two choices. Rebekah will need to continue to be monitored by frequent regular cardiology visits once we come home, but Dr. Bradley feels that her heart can tolerate this pressure over time. Eventually the plan is to let the left lung grow and keep watching the pressure in her heart to make sure she does not show signs of heart failure. Dr. Bradley did mention the likelihood of a return trip to the cath lab after a few months to recheck the pressures in her heart and maybe tweak things a little if the pressures go any higher. At this point, he does not see that Rebekah will need extra medication (for the high pressures) when we go home, but it is a possibility. Right now Rebekah is calling the shots, and the doctors are just adjusting to how her body responds to these changes.

Dr. Bradley did search for, and did not find, Rebekah's right pulmonary artery. We were all pretty certain that it wasn't there, but he wanted to look for himself, since he did not do Rebekah's first surgery. We asked if he looked at Rebekah's right lung to see if it looked healthy, but he said that there was a lot of scar tissue in that area that he didn't want to disturb today.

So, we are going to get a snack downstairs (mmm....frozen yogurt!) and then we should be able to see Rebekah around 4:30 if all is going well.

The bottom line is that Rebekah's Truncus is officially repaired, but at the cost of an atrial spetal defect and higher pressures in her right chambers. All in all, this is probably the best scenario for what could have happened today, and we are very pleased with the work Dr. Bradley did on Rebekah's heart.

Unless something else of significance happens tonight, this will probably be our last post of the day. We are going to spend some time with our little girl and try to get some rest tonight. Now the long days of recovery are ahead of us, so we will get some rest while we can.

Thank you to everyone who followed along with Rebekah's surgery today and kept us in your prayers. We appreciate each and every prayer, comment, post and email that we have gotten.


Drew and Nancy