Catching Up

Wow, where does the time go??! It seems like I just updated the blog a few days ago, yet it's been two weeks since the last update! I guess there is quite a bit to fill you in on since the last update.....

Justin and Bekah sharing some hugs.

He love his little sister!

Rebekah was able to see her endocrinologist for the appointment we missed due to the snowstorm. I always dread those appointments because it means labs, and that means a bad day for Rebekah and a few lab technicians. This time, though, the lab tech was able to get blood on the second try! Dr. Amrhein is primarily checking Rebekah's calcium and phosphorus levels because of her DiGeorge Syndrome. Her calcium is great, but the last couple of labs have shown that Rebekah's phosphorus is just a bit on the high side. That can be a side effect of something really bad (like kidney failure), but in Rebekah's case, it is probably completely normal. She is still on diuretics to keep fluid off her lungs and heart, and that can cause mild elevation of phosphorus levels. Also, the DiGeorge Syndrome itself can indirectly cause high phosphorus levels since DiGeorge affects the parathyroid, which in turns affects (elevates) phosphorus levels. Bet you didn't know all that, huh?! Anyway, it is something to have checked every six months or so, but nothing to be concerned about as long as Rebekah's levels stay where they are or drop. If they start trending higher, the doctors may take a look at a few other possibilities, but we don't really think that's going to be a problem.

Zac and Rebekah. This boy will do just about anything for his sister....

except touch her bracelets and necklaces! :)

See how badly the oxygen cannula is irritating her skin?

Rebekah has had a time with her oxygen lately, and not in a good way! She has outgrown the infant cannula that she was using prior to her heart surgery, so she is now using a pediatric cannula. That's not a bad thing as far as air flow, but it's thicker and less flexible, and much harder to work with. We were able to fasten Rebekah's infant cannula with a single steri-strip on each side of her face, but that doesn't hold the pediatric cannula. We have tried a few kinds of tape, some larger steri-strips, and just tightening the tubing in the back so it couldn't slide out of place. Each of those methods left it's mark on Rebekah's face. She is so super sensitive to adhesives that no matter what we try, her face ends up with large red irritated areas. When we tightened the tubing in the back and used no adhesives, she wound up with raw sores on her cheeks where the tubing touched her skin. Fortunately, Andrea (Owen's mom) came to the rescue!! Thank you a million times over, Andrea!! Owen has these nifty little contraptions called Tender Grips (you can check them out by clicking here) to hold his cannula in place. The flesh-colored part sticks directly to the skin with a very mild adhesive, and the tubing is actually sandwiched between a clear sticky part and the flesh-colored part. No more tubing rubbing against Rebekah's cheeks! It was a very long, round-about procedure to get these pads, and I'll not bore you with the details. We finally got them delivered today, and so far, so good! I'll report after we've used them for a couple of weeks and let you know the final verdict! :)

Aaron can't be left out of his hugs from Bekah.

I have no idea why she was so serious in all of these pictures!

So all of that has been going on with Rebekah's oxygen, and then last night she had her first accident. :( Rebekah has been doing so well at pulling up on almost everything, including the kitchen cabinets. She was playing with my kitchen towels that normally hang on my oven door, and they were laying in the floor when she decided to pull up on the cabinets. You guessed it....her foot was on the towel, not on the floor when she pulled up, and she fell face-first onto our very hard kitchen floor. The cannula was pushed up her nose and she had the worst nose-bleed that I have ever experienced with any of our children. In just the few seconds from when Drew picked her up till I had wet washcloths to clean her up, her mouth was full of blood and it was dripping off her chin. It was pretty bad. All of her teeth appear to be intact, though, so we are very thankful for that! Understandably, we left the cannula off for the night and a good portion of the day. Rebekah does not need the oxygen to keep her sats up like she did prior to surgery, so it was not a risk to her medically to not have the oxygen. We felt like her poor little nose needed a break after that injury. We did put the cannula back on this evening, and she was fine with it. Leaving it off was probably more for Mommy's peace of mind than from any discomfort Rebekah had! :)

Uh oh....you caught me with my brother's toy!

The other thing we have started experiencing with Rebekah's oxygen in the last week or so is nose bleeds. Well, ones that aren't caused by injury! When Rebekah saw her pulmonologist last week, I mentioned that to her, and she asked me if Rebekah had a humidifier for her oxygen. I replied that no, it had never been offered or suggested to us. Dr. Gwinn immediately responded that we should leave it to her, and that afternoon our home health company called about fitting our concentrators with humidifiers! So Rebekah is getting nice moist air that will help her nose to not get so dried out. We are hoping that reduces, if not eliminates, those pesky nose bleeds!

I can't decide which is my favorite time of day...

breakfast, lunch, or dinner!

Rebekah had her appointment on Monday with the orthopedist. He did a quick check of Rebekah's hips and joints, then ordered a set of x-rays of her hips. The good news is....there is nothing wrong with the development of Rebekah's hips!! He didn't really have a great explanation for why Rebekah is far stronger on her right side than her left, but said it isn't due to anything structural. His advice was to continue therapy and come back to see him if Rebekah isn't walking by her birthday. Something tells me we won't need to go back to see him! :)

I redecorate Mommy's bathrooms in my spare time! :)

Rebekah is truly getting around the house at lightning speed now! She has effectively learned how to go up and down the stairs, so I am never quite sure when I walk out of the room where I will find her. It's a great problem to have, though! Rebekah is quite adventurous, and I have found her in bathrooms, in kitchen cabinets, in the laundry room, and in closets. At least she is great at entertaining herself! :) Oh, and Rebekah got stuck at the scene of the crime in the above picture. After she finished her redecorating, she sat on the little stool, but her feet got stuck because she still had shoes on (normally she can get on and off the stool by herself). She had to call "Mama" to come bail her out!

Miss Priss (as DeeAnn calls her) walking in therapy.

Rebekah is really progressing unbelievably fast in therapy now. I wondered for a while after surgery if we would ever see the "take-off" that so many people described to us after their children had heart surgery. It has taken a few weeks, but we are finally there! The Care Center loaned us a little walker (similar to the one above, but smaller) to use with Rebekah at home, and she is zipping all over the downstairs in it. She will even crawl to it and use it to walk! The big break-through came today when DeeAnn (physical therapist) had Rebekah sitting on a small stool and standing. Not only did Rebekah stand with little to no assistance from DeeAnn, she followed that up with walking about four or five steps by herself! And then she repeated the performance about half a dozen more times! I'm kicking myself for not having the camera in therapy today. I will get it on video soon, though, because when Drew got home tonight, she walked a few steps in between us. I really think that Rebekah will be walking more and more in the next few weeks as long as she doesn't have another hospitalization.

Baby Blake (one of our friends) came to visit.

Rebekah found his pacifier highly entertaining!

Several months ago when Rebekah had speech therapy, her therapist and I decided to put any further speech therapy on hold until after her Truncus repair. Since she is now almost three months post-surgery, Victoria decided to re-evaluate Rebekah's need for speech therapy. I knew that Rebekah had a pretty decent vocabulary (counting both voiced and signed words), but it amazed me as I listed what I could remember. Rebekah says or signs (or both!) over 30 words! When Caleb was Rebekah's age (she will be 19 months on Saturday), he couldn't even say 5 semi-recognizable words. I know that's not a terribly accurate comparison since Caleb had a moderate speech delay, but still - wow! The long and short of our conversation is that Rebekah scored exactly within the normal range for speech and does not meet the qualifications for speech therapy!! What a blessing! Not only the blessing that we don't need to add another therapy right now, but I am so thankful that Rebekah's speech is exactly where any other "normal" 19 month old should be! We have heard for the last year and a half from Rebekah's geneticist that she will have speech delays, articulation disorders, nasal speech, etc. and will definitely need therapy, so this was just a huge blessing for us! There is a possibility in the future that we will see some articulation problems as Rebekah begins to say more words and sounds, but for now we are rejoicing that she is exactly where she needs to be!

Rebekah loved Blake's pacifier. Seriously. If I had let her keep it,

she would have never taken it out of her mouth.

Sorry, girl! Not about to fight that battle now!

The other appointment we had this week was to Rebekah's ophthalmologist. We have been checking the ptosis (drooping) in Rebekah's right eye for almost a year now. Thankfully, it has not gotten any worse, so for the time being we are going to continue to watch it. There is still the possibility of glasses or patching in the future, but as long as their is no progression of the ptosis, it is going to be a wait-and-see game. I'm thankful, once again, for a good report! We will see Dr. Johnson back again in six months. It's amazing that July is six months away, and already it is filling up fast. Rebekah has four appointments scheduled that month already!

Rebekah walking with DeeAnn last week.

Like I said, I didn't have my camera in therapy today, so I don't have any video of Rebekah walking unassisted. This video was last week Wednesday in therapy. Rebekah is wearing "squeak" shoes, so the squeak-toy sound you hear is her walking. She wore the shoes outside last week and about drove our neighbor's dogs crazy. Thankfully they are contained, or they might have seriously terrified Rebekah while trying to get to her shoes! :)

I know this was a terribly long post, so if you made it all the way to the end - thank you! We appreciate each of you and thank you for praying for our family and our little Rebekah.

Nancy

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

Disappointing

I have to say that Rebekah's weight check today was disappointing. For the first time in her struggle to grow, she actually lost weight this week. Not much - just an ounce and a half, but still it's not the way we want to go. So our dietitian is tweaking Rebekah's feeding plan again. We will have another weight check next Tuesday. We (Rebekah's doctors and Drew and I) are really working hard to find the right combination of feeding amounts/times/frequency that works for Rebekah. Too little and she doesn't grow; too much and she becomes overfed and refuses to eat at all. This is a very difficult and very frustrating hurdle for us to get over right now. Please pray with us that Rebekah will be able to gain some weight this week.

Rebekah "talking" to her brothers!

On a brighter note, Rebekah's endocrinologist had nothing but good things to say about Rebekah today. He was so pleased with how she was doing that he didn't see a need for labwork today. Rebekah will be seeing an infectious disease doctor in the next few weeks to follow her immune system, so we will be able to check her calcium and vitamin D levels during the labwork that the infectious disease doctor will require. Rebekah didn't know enough to be happy about getting out of the labwork, but it sure made my day! She is a hard stick and the last blood draw we had was rather rough.

Tomorrow we are heading to Charleston to change Rebekah's tube for a Mic-key button. It should be a quick in-office procedure, so hopefully we will be back tomorrow evening. We will try to update tomorrow night and let everyone know how things went.

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