Rebekah News

So....it's been a while! :)  So long, in fact, that Blogger has a whole new look and format behind the scenes that I'm having to get used to.  Hmmmm.....guess that'll teach me to not stay away so long!

But in the meantime, Rebekah has been doing fantastic!  She is growing, active, has been relatively healthy, and is so much fun.  I'll try to hit the highlights of the last few weeks......

 Future piano player?

Rebekah's last cardiac check-up was great!  Her pulmonary hypertension is still under control (higher than "normal," but much lower than what it was before surgery).  She continues to take Viagra three times a day and sleep with oxygen to keep that pressure down.  Rebekah's heart function also looks really good; probably the best it has ever been in her life!  The best news is that Rebekah is not going to be scheduled for a heart catheterization this summer like the doctors originally talked about!  As Rebekah's cardiologist said, we don't need to do an invasive procedure to confirm good news.  I'm thrilled about that!

 Rebekah and Great Grammy (Hi, Grandma!!)

We are still struggling a bit with the sleeping issues, but I anticipate that we will have ups and downs in the sleep department for quite a while to come.  Rebekah is currently taking Clonidine nightly to help regulate her sleep patterns.  At one point, right before we began the Clonidine, Rebekah was waking up as many as 20 times in a night!  And at least half of those times she would pull her oxygen off.  :(  We are trying to get her body used to the feel of sleeping through the night.  I have to say that we are improving.  Not perfect, but certainly improving!  Rebekah sleeps through the night, on average 2 or 3 nights a week, and the remaining nights she wakes up probably less than 5 times.

 Someone loves Fudge pops!

Since December (surgery), Rebekah has really grown considerably!  I think she was trying to make up for a year's worth of growth in a few months!  :)  She is more than three inches taller and at least five pounds heavier than she was before surgery.  Thankfully Rebekah has an older cousin who passes down clothes that she outgrows!  :)  For some reason, Rebekah actually went backwards in the last month and lost half a pound.  I'm hoping that we can contribute that to the cold that Rebekah had and that we see her weight bounce back up this month.

Our little fish!

In the last few weeks we have also seen Rebekah make good progress in her strength and endurance.  Rebekah has been working in the pool during her physical therapy time, and it has really paid off!  Rebekah can run and play for much longer periods of time before she gets winded or tired.  She is also doing really well at learning her limitation and knowing when she needs to take a break to rest.  About the only thing that Rebekah is still not doing consistently is stairs.  She still prefers someone to carry her up and down the stairs.  I think it just takes a lot of energy for her to do that.

 Sweet girl

Probably Rebekah's biggest challenge and obstacle right now is her speech.  It's not something I think about a lot because I am with her all the time and usually can understand most of what she says.  However, she is very difficult to understand in a quiet setting, and almost impossible to understand in a crowded environment.  Recently Rebekah was evaluated with our local school system and they found her speech to be significantly delayed.  As of now, Rebekah's left vocal cord is still paralyzed, which is contributing to her speech problems.  She also has articulation and phonemic delays, meaning that more than half of her words are either missing the beginning or ending sounds.  Her understanding of speech is excellent, though, so that is good news!

Rebekah continues to receive private speech therapy once a week.  Tomorrow we will have an IEP meeting with the school system and we anticipate, based on their evaluation and reports, that she will be offered speech therapy in a group setting once or twice a week in a local preschool.

 Silly girl

"The brothers" are all doing well and so happy that school is over for the summer!  They all did very well in their school work this year and are looking forward to a fun summer of swimming, playing, and visiting grandparents.  Friday begins our local library summer reading program, and they are already eager to get started.

Justin, Great Grammy, Rebekah, Zachary (back row),

Aaron and Caleb

Drew and I are also doing well.  I must admit, I was just as eager for summer vacation as the boys! :)  Teachers need a break also, right?!  Drew has an interview sometime next week (not sure of the day yet), and we are praying that it goes well and he is able to start full-time work again.   

And the littlest member of our family, formerly known as T6, is also doing well!  HE is growing, kicking, squirming and all-around being a boy!  :)  We are so excited that Rebekah will have a little brother, and she frequently pats my tummy and says hi to the baby.  At our 20 week ultrasound, everything looked completely normal - including the heart!  We will be having at least one more ultrasound at 29 weeks when he is a little bigger, just to make sure that everything still looks good.  I can't believe we will be meeting this little guy in about three months!

Rebekah "playing" piano.  

She even has to have books on the music rack!

I'll hopefully get another update posted sooner than the last one.  I'm thinking maybe things will get a little less hectic now that we are done with school, but that's probably wishful thinking, I'm sure.  :)

Nancy

Closer to answers?

First off, I have to say thank you all for your prayers for Rebekah's appointment this morning.  She did fantastic!  I was a bit concerned because I wasn't sure how long the appointment would take this morning, but Rebekah was as good as she possibly could have been!

To start with, Dr. L scheduled Rebekah for an echocardiogram.  We are so grateful that everything with Rebekah's heart looks great.  Her pulmonary hypertension remains stable (about where it was after surgery), her heart function looks good, and she is not retaining any fluid. 

Based on my description of Rebekah's symptoms, Dr. L thinks she is having bronchial spasms caused by micro-aspiration secondary to her vocal cord paralysis.  Do you want the English version now?  Dr. L thinks that despite thickening Rebekah's liquids, that she is aspirating very, very small amounts of liquid which is irritating her airways.  The irritation is causing the coughing and choking and breathing abnormalities. Since Dr. L didn't see anything cardiac related, he referred us upstairs to pulmonology.

Unfortunately, Rebekah's pulmonologist was not in the office today, but one of the other partners was able to work Rebekah in immediately.  He completely agreed with Dr. L's theory, so he made a couple of recommendations.  First, he wants us to restrict as much liquid as possible from Rebekah's diet.  That's not going to be fun at all!  Second, he wants Rebekah to have another swallow study done here in Greenville.  Two weeks ago when we discussed another swallow study with Rebekah's pediatrician, she wasn't choking nearly as much as she is now.  It seems like we are going rapidly in the wrong direction.  So he wants to get another look at Rebekah's swallowing and see what (if anything) has changed in the last few weeks.  The nurse was hopeful that we could get it scheduled for next week.  Finally, we left the office with prescriptions and samples for two inhaled medications.  This is our first experience with inhalers.  In the past when Rebekah has needed inhaled medications we have always used the nebulizer.  The inhalers are much faster than nebulizer treatments, so I'm hoping that Rebekah gets used to them pretty quickly.  She has what is called a spacer that holds the medication until she breathes it in.  It's a pretty ingenious little device.  I'll hopefully get a picture of it and how it works with Rebekah in a couple of days after she has had a little time to adjust to it.  She wasn't exactly thrilled with it tonight.  :)

Hopefully Rebekah will only need to use the inhalers for a few weeks until we can get the irritation in her airways calmed down.  She is taking Flovent in the morning and evening, and Xopenex as needed for uncontrollable coughing.  We gave Rebekah the first dose of Flovent after dinner, and she has so far been sleeping fairly well.  She has only woken up coughing twice, which is a definite improvement from last night.  We'll see how the rest of the evening progresses.

I just had to show you this hilarious picture.  Rebekah held her foot out to Aaron and said "thiss," which means "kiss."  So Aaron very sweetly knelt down and kissed her foot.  It was very cute!  And, you can't see it in the picture, but Rebekah is rubbing Aaron's hair on the other side of his head.  :)

I just cannot describe to all of you how much the boys love their sister.  She is absolutely the light of our family, for sure!  We are so blessed to have been given such a special little girl.  Thank you all for praying for her, and please continue to pray that we find some definite reasons for the cough and choking Rebekah has.

Nancy

Rocky Road

Rebekah is struggling again today. We have seen a steady decline in her eating since the first of July. Her fatigue has increased also. She had about 10 mins of energy this morning and then crashed. She had a not so good echo 2 weeks ago. She was pretty upset while they did the echo so we were thinking maybe the results were skewed a little because she was upset. DR. L was going to re-do the echo in month. After talking with him this morning he wants to see Rebekah today and run some tests and another echo. This is to make sure we are not missing something.

Poor girl just does not feel well.

We will post what Dr. L finds later today.

Thanks for keeping Rebekah and our family in your prayers.

D

Catching Up

So I'm finally back with an update...and pictures! :) To say that the last few weeks have been busy would be an understatement, but it is a good kind of busy. Drew is busy finishing the last couple of weeks of school (for those of you who don't know, he is back in school working on a Computer Science degree), I am busy trying to keep up with everyone's crazy schedules, the older boys are busy in their school work, and the younger boys are just plain busy!! And of course, Rebekah has an agenda all her own. :)

No, I didn't just randomly stick in a picture of my cabinet door.

If you look closely, you will see very tiny fingers peeping out of the top.

Those very tiny fingers belong to......

.....this very special little girl!

We were pleased to learn at Rebekah's most recent trip to the pediatrician that she does not have an ear infection! If you read the last post, you will remember that we had some concerns about another ear infection, but not this time! There is still fluid in Rebekah's ears, so we do have some concerns that we will be addressing at Rebekah's audiology appointment in a couple of months, but for now she is infection-free!

Rebekah loves to feed her baby dolls a bottle. :)

Rebekah's most recent cardiology visit was also encouraging. Dr. Lucas was very pleased with Rebekah's echo. It seems that the pressures in her pulmonary arteries have gone down again (remember, they can only estimate using echo, but this is a good sign!). Rebekah's heart function continues to look good also. Yippee!! We had some concerns prior to the appointment about an increase in Rebekah's sleeping, but it appears to be unrelated to her heart. Since then, she has gone back to a normal-for-her sleep routine. Dr. Lucas is targeting late summer or very early fall for the date for Rebekah's next heart catheterization. This will be an important catheterization because it will give us the exact measurement of the pulmonary pressures. We need the pressures to continue to drop between now and then! If the pressures are not down significantly from the last heart cath, then Rebekah will likely need another open heart surgery to re-open a portion of her VSD that was closed in November. We are praying that the pressures are down enough to buy her a little more time before her next surgery!!

Just being cute!

Between now and the end of the summer, we will continue seeing Dr. Lucas (cardiology) once a month. The plan is for Rebekah to grow, grow, grow this summer and to stay healthy and out of the hospital! Rebekah will continue OT at least until June when her therapist goes on maternity leave. We will re-evaluate at that point and determine if Rebekah still needs services.

Showing off a new hat!

Rebekah is making great progress in PT, and we are so very thankful for that! She is an amazing little fighter and has come so far since November. If you remember, she was just starting to pull up on things and stand the week before her heart surgery in November. Now she is walking everywhere! Rebekah's PT is helping Rebekah with stairs and stepping up and down from different platforms. While this is a necessary skill for Rebekah to learn, it is also helping to improve Rebekah's balance and stability while she is walking. The next thing on the schedule is jumping...I know that will be fun! :)

We have noticed in the last few weeks that Rebekah has been walking on the insides of her feet much of the time. Because we are super paranoid about Rebekah's development, it caught our attention. I mentioned it to her PT last week, and it turns out that Rebekah (and all of our boys, as we discovered!) is flat-footed. Not only that but her feet are actually rolling inward as she walks. So, she has earned yet another doctor - an orthotist! Rebekah will be having casts made of both feet so that she can have plastic orthotics made for her shoes. This will help to keep her feet in the correct position when she walks. Hopefully this will help prevent any future leg/foot/hip problems as she grows.

Please continue to keep Rebekah and our family in your prayers. We are so thankful for each of you and your ministry of prayer for our family.

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!!

2011 Snow Storm!

We are enjoying a week of snow days from school thanks to a snow storm that dumped 7.5" of snow followed by 0.25" of ice on us Sunday night and Monday. We are enjoying the time off from school to play together and enjoy being home.

Because of the snow, all of Rebekah's appointments this week have been cancelled. So, we have not seen her cardiologist to have an echo to see if the oxygen therapy is helping her high pressures. As of tonight, it was reported that cardiology will open at noon tomorrow. I am planning to call as soon as they are open to reschedule our missed appointment.

I see you pointing that camera at me!

Last week Rebekah had an audiologist appointment to recheck her hearing. If you read the last post, you may remember that I was going to suggest to the audiologist that we stop checking Rebekah's hearing every six months. However, I learned at Thursday's appointment that the two types of tests that Rebekah have had are different. The "long" test that Rebekah passed tested her auditory nerves. We know that Rebekah's auditory nerves are fine. The short test, however, tests the middle ear; basically, it measures the vibration of the eardrum and how well Rebekah actually receives sound into her ear. So even though Rebekah's auditory nerves are fine, she is not receiving sound into her ear as well as she could. Rebekah's loss is in the slight to mild range right now, with her right ear testing very close to the mild/moderate range. If Rebekah falls into the mild/moderate range, she will be fitted for hearing aids for at least a few years. Rebekah will continue to have her hearing tested every six months.

Daddy's first dance

Rebekah also had a gastroenterology appointment on Thursday last week. The week of Christmas we had reduced Rebekah's overnight feeds from 40 mL's an hour to 35 mL's an hour. Rebekah maintained her weight even with the reduction in feeds, so we were given the green light to reduce by another 5 mL's an hour. It doesn't sound like much, but every time we drop 5 mL's, Rebekah gets 2 ounces less of formula overnight. It has already made a big difference in her appetite, and we are seeing Rebekah eat much more than she was a month ago. The plan is to continue to check Rebekah's weight once a month and reduce her feeds by 5 mL's each month that she maintains her weight or gains. Our GI doctor said a conservative weight gain for Rebekah should be 4-5 ounces each month. So as long as she meets that goal, we continue to drop feeds. We are hoping that Rebekah will be off, or very close to being off, her overnight feeds by her birthday. We will take it one step at a time, though, and go as slowly as Rebekah needs to.

Rebekah loved sledding!

This week Rebekah missed her endocrinology and cardiology appointments, as well as two of her therapies. While it has been nice to have a break from the constant busy schedule, I am a little nervous about fitting in those two appointments into the next two weeks, which were already busy enough! It will work out, though. It always does. :)

After a while, we gave up sledding and just sat in the snow.

Rebekah kept patting the snow with her mittens.

If you would like to see more pictures of the boys and Rebekah playing in the snow, click here to head over to our family blog. We really enjoyed our snow days!

Yes, Rebekah really is in there somewhere! :)

Thank you all for your prayers for Rebekah. So far she has remained well, and we are thankful for that! Please continue to pray that Rebekah stays well and that her pressures will be better when she has her next echocardiogram.

Nancy

Our 200th Post!

Can you believe that Rebekah's birthday is almost here??? We are having a hard time convincing ourselves that it has really been that long! Rebekah has grown and changed so much since then, and we are so very thankful that God has trusted us with her.

Rebekah made her first trip to the pool on Saturday, and she loved every minute of it! She kicked and splashed and played for a while, then she kicked back and relaxed. It was such a fun time! The boys all enjoyed swimming and playing in the pool, as well.

Check out my super cool shades! I'm ready for swimming!

Rebekah is making great progress in her weight gain. She had a doctor's appointment today and is now up to 15 pounds. That's right! 15 pounds!!!! Rebekah has also grown an inch and is now 27" tall. All of that eating Rebekah is doing must be doing some good! We have really seen an increase in the amount of food Rebekah is eating each meal as well as more variety in textures and flavors that she will eat. I am very excited about Rebekah's eating progress!

This is pretty fun!

I am very happy to say that Rebekah had a good report from her cardiologist today. We had been noticing that Rebekah was more fatigued than usual during eating and playing. Sometimes Rebekah lays her head down on the floor or on her high chair in the middle of whatever she is doing. After getting the results of Rebekah's echocardiogram today, the doctor feels that Rebekah's heart is still stable. Indirectly, Rebekah's heart is responsible for her fatigue because it is working so much harder than a normal heart in order to pump blood to her heart and lungs.

Really, why wouldn't I want to put it in my mouth??

So, because Rebekah's heart is working "overtime," she is going to get tired with more activity. That makes us feel a little better knowing that this is going to be normal for Rebekah until her heart repair. The one thing we need to keep an eye on is Rebekah's oxygen saturations. When they begin to trend downward and stay down, then we will be heading toward surgery.

Life doesn't get any better than this! :)

Next week is a big week for Miss Rebekah. In addition to her regular therapies, Rebekah's repeat hearing evaluation is Tuesday. We are going to have her ears checked for fluid at the pediatrician's office on Monday so that we know before the hearing exam whether or not she has any fluid in her ears. Since the audiologist felt that fluid was the cause of not passing her first test, we don't see a reason to retest if there is still fluid present. If the fluid is still persistent, we may end up with an appointment to the ENT to discuss tubes to drain the fluid. Hopefully we won't be having that discussion! :)

Swimming makes me sleepy!

Isn't Rebekah so cute?!!! When we came home from the pool on Saturday, Drew put her on the floor to play for a few minutes while we got the boys started on their showers. When we came back downstairs, Rebekah was fast asleep on the floor.

That's the news for now! We hope to see many of you at Rebekah's birthday party!

Nancy

Hold the train!

The verdict is in and we are not going to Charleston this week. After two days of discussion between our Greenville cardiologists, our pediatricians, and the cardiology group in Charleston, it has been decided that we need to wait and let Rebekah's lungs get back to normal before attempting the heart catheterization. We will know more this week, but the current plan is to try to reschedule in about two weeks.

So sweet!

Rebekah has had a busy two days! Yesterday, she woke me up around 5:00 in the morning with her oxygen sat monitor beeping. She had dropped into the mid 70's and was not able to come back up on her own. So her nurse gradually increased her O2 until Rebekah was at a full liter of oxygen. Then Rebekah had another incident early in the afternoon yesterday when her sats went down again, so we are currently on a liter and a half of oxygen just to maintain sats in the low to mid 80's. Our night nurse was going to try to wean Rebekah down some during the night, but her sats were never stable enough and high enough to wean. That left us with a big question - what is going on to make Rebekah's sats so low and unstable??

I love you!

This morning, Dr. Butler came by (Zac and Justin both had his wife as their K-4 teacher.). He looked at the chest x-rays that Rebekah had done yesterday and said that the lower part of Rebekah's left lung has collapsed most likely due to whatever respiratory bug she has right now. If she is not expanding her lungs fully, they are more likely to collapse. Thankfully, her right lung looks fine. We also ruled out RSV yesterday, so that is encouraging! Dr. Butler ordered some chest PT for Rebekah (remember how much she liked that last time she was in the hospital?) and some nebulizer treatments with Xopenex every 6 hours. Xopenex is a fairly new drug that is supposed to have the result of opening the airways just as well as Albuterol, but is not supposed to elevate the heart rate and blood pressure quite as much as Albuterol does. We'll see how Rebekah does with it.

I like to smile!

Rebekah also had an echocardiogram this morning, just to rule out any possible heart complications. Dr. Raunniker came by to see us this morning, and he was going to read the echo and let us know if there were any problems. We aren't expecting any. This looks like the work of a respiratory illness, not a cardiac problem.

So, that leaves us in the hospital a few more days until we can get Rebekah's lungs back to normal. We have officially been in the hospital for half of 2010 now, and are looking forward to being able to go home. We are so thankful that Rebekah made it without having to be hospitalized until almost Christmas and then again in January. Many other kids and parents are not able to say that. If you think of it, please keep our little friend, Josiah, in your prayers. He had been home for a few days after Christmas, but had to be taken back to MUSC with unexplained high fevers. Now the doctors believe he has an ulcer in his stomach and have put him on some new medications. I'm sure their family would appreciate your prayers.

Cardiology Update

Yesterday was Rebekah's cardiology appointment, and while there, we also popped in to see the dietitian for a weight check. First, the cardiology appointment....

Rebekah's echocardiogram went well, as usual. As much as they can see on the echo, Rebekah's heart function looks good and she does not seem to be in heart failure. However, Dr. Lucas told us that because Rebekah's heart is essentially functioning as a single ventricle (because the blood mixes back and forth on both sides of her heart it is as if she only has one side working), it is almost impossible for them to measure the amount of heart failure, if any, that Rebekah is experiencing by echocardiogram. The only true way to determine if Rebekah's heart is failing is by heart catheterization, which is coming up at the end of January. Dr. Lucas said the cath lab doctor, Dr. Baker, will measure something called QPQS, which is basically the amount of blood flowing to the lungs and the amount of blood flowing to the body. That will give the doctors the information to know how hard Rebekah's heart is working to keep her lungs and body supplied with oxygen. In turn, that information will tell us how close she is to needing her heart repair.

Dr. Lucas has also had some conversations with Dr. Bradley (the head cardio-thoracic surgeon who will be doing Rebekah's next surgery), and Dr. Bradley feels that the collateral arteries that we had hoped were growing are probably not growing. Based on pictures and notes from Bekah's original surgery and information that Dr. Lucas has been able to send to Dr. Bradley, he feels that she really has not shown any signs that those collaterals have grown. We are praying that Dr. Baker will find out differently in the cath lab, but we are preparing ourselves for the difficult decisions that will come if Rebekah does not have large enough collaterals to work with. Basically, in Dr. Lucas' words, we will come to "a fork-in-the-road decision" about what to do. We can possibly do another "temporary" fix if her heart allows, to try to give the collaterals more time to grow, but everyone is pretty much agreed that if they haven't shown signs of growth by now that they probably won't. Or, we can proceed with the heart repair, and Rebekah will only have one working lung. Ever. From what Dr. Lucas said, there really isn't any middle-of-the-road on this one. I'm not really sure what kinds of options are available, but we have been told there are several options for the heart repair if the collaterals have not grown. I think the idea is to keep from stressing her working lung too much in the process of repairing her heart. Of course, the big day for the cath lab is January 26, and those results will answer many of the questions we have right now. We probably will not know much, if anything, when we leave Charleston, but we are having a meeting with Dr. Lucas on February 3 to discuss the results of the cath lab and weigh in on the opinions of all of the cardiology team from Charleston. We won't actually be meeting with the team, but they will send the results and opinions from their big meeting to Dr. Lucas for him to share with us. We will, of course, be meeting with them all before Rebekah's heart repair in Charleston.

Dr. Lucas is a little concerned (understandably) about Rebekah's ongoing congestion and her on-again-off-again fevers. He mentioned that if we come down with something more serious that what she was hospitalized with a couple of weeks ago, or if this turns out to be another lingering illness similar to the last one that required hospitalization, he will be likely to send us to Charleston. Ugh. We, of course, want to do what is best for Rebekah, but Charleston is a logistical nightmare right now! There are two reasons why Dr. Lucas feels that Charleston would be a better choice in those events. First, if Rebekah continues to have lingering, on-going illnesses, there is a good chance that her heart is playing more of a role in her health than we think it is. By being in Charleston in the cardiac unit, they will be able to monitor and test things much more closely and accurately than here in Greenville. Second, Rebekah cannot go into the cath lab while she is fighting a respiratory illness. The results would be too inaccurate to be of any help. So, if Rebekah is in Charleston, they could work to get her over the respiratory illness and then choose the optimum time to go to the cath lab for the best possible results. Please pray with us that Rebekah will fight off any little colds and stay healthy. And please pray that if we do go to Charleston, the logistics will be worked out (care for the boys, transportation, finances, lodging, etc.).

There was a bright spot in our visit yesterday.....Rebekah is gaining weight!! It seems that the continuous feeding through the night is really working, which makes getting up in the night much easier! :) Rebekah now weighs 10 pounds and 1 ounce and is 23.25 inches long! In the last month or so, she has gained a little over a pound (because she had lost some weight she was less than nine pounds) and she grew about 3/4 of an inch! We are so excited!!!

We are off to a rather lengthy pediatrician's visit this morning. Rebekah has her six month well-check and immunizations, and there will be a lot of ground to cover regarding Rebekah's care. We will fill you in on all of the details and try to get some new pictures posted soon!

Nancy

What's Up, Doc?

Rebekah is feeling a little grumpy tonight since she had shots at her well visit this morning. She is letting us know that she isn't really appreciative of the effort we made on her behalf to vaccinate her! :) After a bottle and a few more complaints, she is now sleeping peacefully on Daddy's shoulder.

This has been a busy week of doctor's appointments for Rebekah in addition to her regular therapies. On Mondays, Rebekah's Early Interventionist comes to see her for an hour, followed by an hour of Physical Therapy. Rebekah's Speech Therapist comes on Wednesdays for a half hour, and we have Occupational Therapy on Thursdays for an hour. Of course, that schedule is all subject to change depending on the doctor's appointments that are scheduled for any given week.

All this therapy is hard work and makes Rebekah sleepy!

This week Rebekah saw Dr. Lucas on Tuesday. I mentioned to Dr. Lucas that we had seen a few things in the last couple of weeks that we have not noticed before. Several times Drew and I have noticed that Rebekah's heart rate is elevated significantly and her breathing becomes more rapid. Also, Rebekah has been sweating for no apparent reason. When Rebekah's O2 sats were measured on Tuesday, she was a little higher than her normal. A person with a normally functioning heart would have an oxygen saturation level at 100%. Because of her heart defect, a "normal" oxygen saturation level for Rebekah is in the 85-90% range. On Tuesday, Rebekah's sats were hanging out around 94%. All of that combined to give Dr. Lucas hope that the collaterals are finally starting to grow and that is causing extra work for her heart. The plan for now is to give Rebekah a little more Lasix to rid her heart of a little more fluid and plan for a catheterization sometime in January or February. We are praying that the collaterals are indeed growing and will be large enough for the surgeon to work with to make and artery to connect Rebekah's lung to her heart.

I love chewing on my fingers!

After visiting Dr. Lucas, we went downstairs to see our friends in the GI office. We were a little disappointed to find that Rebekah had only gained an ounce in the past week. So we are increasing her intake again (from 95cc's per feeding to 105cc's per feeding). The doctors are closely watching Rebekah's growth, but they are not necessarily looking for a specific amount of weight gain. What the doctors are looking for is that Rebekah grow following along her growth curve, and right now her curve is a little flat. We are working to give that flat line a little upward curve.

Rebekah has also been chewing on her hands and fingers a lot more in the last week or so. All of the boys cut their first tooth when they were five months old, and Rebekah is 4 and 1/2 months old this week. We'll see if she follows in her brothers footsteps!

Next week's agenda includes a trip back to GI to check Rebekah's weight gain and a trip to visit the endocrinologist. Rebekah will have labs done to check her calcium and vitamin D levels, among other things. On Wednesday we head back to Charleston to get Rebekah's g-tube changed to a Mic-key button. This one will sit flush with her skin and not have any tubing attached to it unless we hook the tubing up to use it. Hopefully, there will be much less of a chance of Rebekah pulling it out, and dressing her will be much easier! They will be able to change the button in the office during a regular office visit, so this trip will just be down and back in the same day. Please pray for traveling safety for Rebekah and I as we travel and for no problems getting the button changed when we are there.

Until next time,

Nancy