Happy Saturday!

I'm happy to say that I think we are all on the mend at our house! What a crazy couple of weeks of being sick! Rebekah ended up having an ear infection before all was said and done, but she is feeling much better now after a few days of antibiotics. Please pray that Rebekah (and the rest of us!) stay well between now and next week!

We received "official" papers in the mail this week regarding Rebekah's heart catheterization. I know it's been scheduled for months, but it always makes it seem more real when we get the official paperwork. I still don't know what time Rebekah's cath is scheduled, but I'm hoping that it's first case. Last March Rebekah was scheduled for 2nd case (11:00) and the first case went long. It was between 1:00 and 2:00 when she finally got back for her case. We also don't know what to expect from the case itself. The last cath Rebekah had was strictly exploratory. This time, there will be procedures done, if there is anything that can be done to help relieve Rebekah's high pressures. As of the last discussion our cardiologist here had with the surgeon, Dr. Bradley did not really think there would be anything that could be done in the cath lab to relieve the pressure, but they will be checking. Dr. Baker will be the doctor doing the cath. He has done all of Rebekah's caths so far.

In other (great!!) news, Rebekah has tested out of occupational therapy!! We love Rebekah's OT, but are thrilled that Rebekah has made such great progress in just 18 months. When we looked back at Rebekah's goals from last April, there were things such as "clap hands" and "roll over" listed for her goals. Now, Rebekah is almost age-appropriate in every area. We are so thankful for our great therapists and their hard work with Rebekah. We're pretty proud of our little superstar, too! Rebekah has so much determination and desire to do more. She rarely lets anything slow her down!

Rebekah's speech is exploding by the minute! I'm going to attempt to list words and phrases that Rebekah says. I'm sure there are words that I'm forgetting, but even so, it's getting to be a long list!

Words Rebekah Says

mommy

daddy

zac (sounds like gac)

justin (ju-in)

aaron (sounds like "hen" without the "h")

caleb (sounds a lot like bubba)

baby

up (means up or down)

please (peese)

thank you (ga-ooo)

eye

bye

night-night (ni-night)

apple

buckle

bubble

outside (side)

drop it

got it

all done

love you

grampy (sounds like bampy)

grammy

dog (woof-woof, yep she barks for dog)

nana

papa

uncle david (day-ya)

no

look

potty (this one compliments of potty-training Caleb!)

Words Rebekah Signs

play

eat

milk

yes

no

jump

ball

cookie

please

Rebekah's receptive language is astounding. Even though she can't say a lot of the words, she can recognize most common animals (pets and farm animals), almost all her colors, and most of her toys. We're working on her choosing between two or three things. For example, if she has a doll, a ball and a book in front of her, we ask her to pick up the ball correctly the first time.

Rebekah is really walking all over the place now! She is walking at least half of the time without prompting. We are so excited that she is getting stronger and more stable. What a miracle this little girl is!

The other night at dinner, Aaron was playing with Rebekah. He was holding his nose and snorting, so this is Rebekah's attempt at holding her nose and snorting. I thought it was so funny! Then Rebekah shows off her knowledge of body parts. See? Even though she doesn't say the words for all of those things, she knows what they are and can point to them.

Better get some Saturday chores done now. Hope you all enjoy this beautiful weekend!!

Nancy

Surprise!!

Our little super star is walking!!!!

Thought that might be a nice surprise for a Monday morning! :)

Nancy

Quick Update

Just to quickly let all of you know that the colds continue. :( All of us have in one way or another been sick with this round of colds. The current status is: Zachary and Justin appear to be over, or mostly over, the colds; Aaron and Caleb are somewhere in the middle with runny noses and occasional coughs; Rebekah still has some runny nose, and has developed more of a cough. I also detected some wheezing in both lungs tonight when I listened to her breathing. So far she is managing well with only a slight drop in her oxygen saturations. Normally she sats in the mid-90's on room air, and at 100% on oxygen. Tonight she was 91-92% on room air and about 97% on oxygen. We will continue to watch her, but unless she takes a drastic turn for the worse, we are much better off at home than at a doctor's office or hospital!!

Mom and Dad are feeling pretty rough tonight. I have been fighting cold/allergies/sinus symptoms for a few weeks now, and I think this last round was enough to knock me out. I'm planning on getting to the doctor tomorrow if I can get an appointment. Drew is also not feeling well, and we would both appreciate your prayers.

Thankfully, even though many of our friends and church family have been battling the flu, our sickness appears to just be a bad head cold. No one has had fevers, headaches, or body aches that are associated with the flu.

Also, please be praying that Rebekah recovers sufficiently from this cold before we head to Charleston in two weeks (2 weeks!!) for her heart catheterization. Given the seriousness of her pulmonary hypertension, this heart cath is not something that we want to delay if at all possible. I'm not sure what the threshold of "sick" versus "too sick" for a heart cath is, but we don't want to be anywhere near that!

And to throw out a little teaser, I have an exciting video to post of Rebekah...tomorrow! I haven't gotten it downloaded from the camera yet, but I will get it done tomorrow. :)

Thanks for praying!

Nancy

Thankful for Colds!

Yes, you read that right - we are thankful for head colds around here! For one, I'd take a head cold any day over some of the stuff our friends have had. Yucky!! Second, this weekend we were very concerned about Rebekah because she began to show a dramatic decrease in both appetite and activity level. While in most kids that is not cause for great alarm (for a day or two), those can be warning signs of early heart failure. So on Sunday morning we decided to call cardiology just to let them know what was going on. We were reassured (somewhat) by the on-call cardiologist that heart failure usually does not present that suddenly (but we are talking about Rebekah, here. When does she ever do things the usual way??). He gave us a few other things to watch for, but basically we ended the call hoping that by mid-week Rebekah would start showing signs of her brother's cold, and we would have an answer for her lack of appetite and energy level.

Aaron, Rebekah, and Caleb playing with homemade maracas.

Well, this morning Rebekah was gracious enough to wake up with a very congested nose and cough. While we certainly wouldn't wish her to be sick, we are very thankful that we can blame all of these symptoms on a cold and not on her heart. Now we are praying that she can fight this cold off in a couple of days and avoid a hospital trip. :)

Zac and Rebekah, our oldest and youngest.
When did they get so big??

Rebekah was a little couch-potato over the weekend. She wanted to be held, cuddled, and loved on, and we were all happy to comply. :) In the above picture, Rebekah snuggled on the couch with Zachary for a long time while all the kids watched Snow White for the first time. They loved it! Rebekah was especially cute; she kept pointing to the TV saying, "Look! Look!"

Happy Valentine's Day!!

The kids and I made cookies for Valentine's Day on Saturday. We all had one after dinner on Saturday night. Rebekah wasn't sure what to do with hers. She kept pointing at it, poking it, pinching frosting off, and talking about it, but she never did pick it up and eat it. Oh, well, she had lots of fun with it! :)

Rebekah is "so big!" She also has frosting everywhere.

In other news, we did hear back from MUSC last week regarding the schedule for Rebekah's next heart catheterization. Dr. Bradley (Rebekah's surgeon) does not see any benefit to moving the heart cath any sooner than it is already scheduled (March 7). He really feels that the high pressure is due to the one-lung situation and is not something that will be resolved in the cath lab. So, his expectation is to get a general "lay-of-the-land" so to speak, of how Rebekah's heart looks now in preparation for another open heart surgery. I'm not sure exactly when that surgery (if needed) will be, but according to Dr. Lucas, he does not anticipate it immediately following the heart catheterization (as in the same week). He said what will probably happen is that we will have the cath, come home, and wait for a phone call from MUSC after all the cardiologists, cath doctor and surgeon have had time to review Rebekah's case and get it on the surgery schedule.

Even covered in frosting, I'm still cute!

I guess that about wraps up the latest developments here. Continue to pray that Rebekah recovers from this cold quickly and that she stays healthy between now and time for her heart catheterization. As I recall, last year around this time we were also praying for Rebekah to stay well prior to a heart cath. Same song, different verse. :)

I just have to quickly share a huge praise for our little buddy, Owen. He had major open-heart surgery last week Tuesday to repair or replace his tricuspid valve. The surgeon was able to repair Owen's valve, so there was no need for replacement! This means that he will be a candidate for the third surgery of the three HLHS surgeries, and will be scheduled sometime later this year for that surgery. His recovery is going wonderfully, and there is talk that he might be going home as early as tomorrow!!! Way to go, Owen!!

Rebekah says hi to Grammy and Nana!

Ummm, yes, she can talk! Matter of fact,

we have a hard time getting her to be quiet sometimes!!

Nancy

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!!