We're Home!

Rebekah was such a big girl during her time in the hospital! We were so proud of her and the way she responded to all the doctors and nurses.  She was so sweet, and so cooperative, even when she wasn't feeling great after the cath.  Going in to the hospital, Rebekah was very excited and couldn't wait to see her doctor and nurse friends. Rebekah sailed through her pre-cath assessments and waited very patiently until Dr. Baker was set up and ready in the cath lab.  The child life specialist even brought a box of hats like the doctors and nurses wear in the OR and let Rebekah pick one out! :)

The cath started around 9:00 and Rebekah was done around 12:30.  Thankfully they were able to gain IV access after Rebekah was sedated, so she was not in any pain from that.  They were able to get cath access in Rebekah's right groin area, and measure pressures in her heart. The best news we had from the cath is that her pulmonary pressures are almost normal!!!  She has not had "normal" pulmonary pressures in her entire life!  We are so excited for that piece of good news!  Rebekah's heart function also looks good, and she just has a trivial to mild (less than 10%) leak in her pulmonary valve.  

Dr. Rebekah with Carter and Ellie. 

All set to get in the elevator to head to the cath lab!

The one thing that Dr. Baker did find were two moderate-sized collaterals that were routing blood away from her heart uselessly.  Dr. Bradley was consulted and because of the location of the collaterals, they would not be able to be unifocalized in the future (tied in with her right pulmonary artery to supply more blood to her right lung). So, Dr. Baker coiled off those two collaterals. Time will tell if those collaterals were causing Rebekah's fatigue or not, but we are hopeful that this was indeed the cause of her tiredness.

The recovery from this cath has been harder on Rebekah than previous ones. She's been in a lot of pain in both her groin and her lower back. She also had a lot of trouble with the adhesive this time - the leads for her monitor, the pressure bandage around her upper leg, the tape used for her IV's - all left multiple red areas that look irritated. The other strange thing is that Rebekah's right foot (same leg as her cath site) is purple-splotched, almost like little tiny bruises all over the bottom of her foot.  It doesn't seem to hurt, but it definitely looks strange!

So we are back home and taking it easy. Rebekah is not walking much at all and asks to be carried most of the time.  She slept a good part of today, but is eating and drinking well, and I am thankful for that!  We have a low-key weekend planned to let Rebekah rest and recover.  I hope she will be back to her normal self soon!

~Nancy

Another Trip to MUSC

Just a quick note. We have all been very busy with work, homeschool, soccer, and life in general. Bekah has been growing and developing with steady progression. She is looking forward to turning four soon. She has been enjoying life for the most part and not too affected by O2 tubes and medical stuff. She has been trying to keep up with her brothers the best she can. The last couple of months have been a little up and down for Bekah's energy levels. She has periods when she struggles with a lot of fatigue. The doctors have run lots of tests, and she looks great from everything they can see. Her sleep apnea has dropped from severe to moderate, and all of her bloodwork came back normal (electrolytes, thyroid, CBC, etc).  From the heart side, Bek looks great on echo, but the doctors still can't explain the fatigue. So Miss Bekah is headed to MUSC again to have an internal look at her heart. She is scheduled for a heart cath on May 14th. They will specifically be looking for any collaterals that might be shunting blood away from the heart, any scar tissue building up on her conduit or shunt or valve, and looking at her pulmonary pressures.  Please pray for her and the family as we tackle another heart cath. Pray we can figure out how best to help her with her fatigue.

 -D

Sleep Study

A year ago Rebekah went for her first sleep study and we discovered that she was having both obstructive and central apnea spells during her sleep.  The number of occurrences  per hour put her in the severe apnea range.  Two weeks ago Rebekah had a follow up sleep study.  Today we had an appointment to get the results of that sleep study.

I am so excited that the number of apnea events that Rebekah has during her sleep has dropped from the severe to moderate range!! For the last year Rebekah has been taking Clonidine to help her sleep through the night, and she has been sleeping with 2 liters of oxygen.  She hasn't used the Clonidine in the last two weeks, and we were told today that she doesn't need to take it any more at all!  Dr. G also decreased her oxygen requirement while sleeping from 2 liters to 1/2 liter when sleeping.  We are very pleased with how well she's doing!

 She's so cute!!

Since Christmas Rebekah has been sleeping more and overall more fatigued than she had been.  She also has been more clumsy - walking into walls and doors, falling out of chairs, tripping and falling for no obvious reason. So we've been trying to figure out what is going on.  The bottom line is that we haven't found an easy answer.  Rebekah had an echo and cardiology check up that looked absolutely perfect.  A panel of bloodwork also came back looking perfect - not even borderline readings, but absolutely 100% perfect!  We were really hoping that the sleep study would turn up an answer for the fatigue, but it didn't.  So we are now waiting for Rebekah's cardiologist to call us with a new plan.

Peek-a-boo!

~ Nancy

December Update

Hi again! Although it has been a while since our last update, I am happy to report that no news is good news!  :)  Other than a couple of minor colds and a particularly benign stomach bug, Rebekah (and our family) has so far remained healthy!

In an effort to catch everyone up on exactly how Rebekah is doing right now, I will attempt to give a doctor-by-doctor update and hopefully not leave anything out!

Cardiology:  Rebekah had a follow-up visit and echocardiogram with her local cardiologist just after Thanksgiving.  We are praising the Lord that her heart function continues to look better and better.  In fact, at our next visit (not until March!!), he is going to consider reducing and/or eliminating one of the medications that Rebekah takes.  Wow!

Pulmonology:  Rebekah's lung(s) are continuing to remain stable and for that we are very grateful!  She has only had a couple of colds, but thankfully she has not had any major complications so far this winter.  I think the one thing that we see from a common cold is that it does take Rebekah longer to recover than her brothers.  All the more reason to continue with good hand washing and staying away from anyone who might be sick!

Endocrinology:  All of Rebekah's systems are functioning completely normally!  She has not struggled with any calcium deficiencies and her thyroid is at a normal level.  We have been told that her Endocrine system might take a bigger hit when she goes through puberty, but hopefully we have a looonnngg time before that happens!

Immunology:  Rebekah's  primary immunologist moved to another practice during the last year, so we are now seeing a new immunologist,who is very sweet.  After a traumatic blood draw, we got the happy news that Rebekah's immune system is functioning within normal limits.  Rebekah will continue to see her immunologist on a yearly basis, and we are thankful for each winter we get through without serious illnesses!

Christmas 2012

Gastroenterology:  Well, this is the one where it gets a little sticky....Rebekah has had quite a few GI issues, although thankfully they have been relatively minor.  Over the last few months, Rebekah has complained off and on about stomach pain, with no real answers.  During a recent office visit, Rebekah's GI doctor ordered some labwork and abdominal x-rays.  The labwork didn't show us anything since, unfortunately, it clotted before the tests could be run on it.  The x-rays, however, showed that Rebekah had a significant amount of gas in her abdomen.  One of the medications Rebekah takes can cause that, so the doctor decided to switch that medication for one that is gentler on the stomach.  So far Rebekah has had fewer complaints of stomach pain, although she does occasionally still complain.  

Sleep Medicine:  In general, Rebekah is sleeping fairly well.  She still needs the Clonidine to sleep, but most nights she does sleep peacefully.  Rebekah has a follow-up visit with her sleep doctor in February and he will schedule her for a sleep study after that.  

Audiology:  Rebekah got her hearing aide in November!!  We are so excited about her hearing aide!  Rebekah has mild-to-moderate hearing loss in her right ear and normal hearing in her left ear, so she only has one hearing aide.  And it's a good thing that it is pretty child-proof.  It's already had one dunking in the bath tub!  :)  Seriously, we have noticed such a big difference in her speech already.  Everyone from friends to Sunday school teachers to therapists has noticed how much more Rebekah is able to hear and how much better her speech is.  It is amazing what a difference it has made!  And even Rebekah notices - she reminds me to put her special ear on every morning, often as soon as she wakes up!

Genetics/ENT:  And here comes the other sticky part....velocardiofacial syndrome, a.k.a. 22q.11 deletion, a.k.a. DiGeorge Syndrome.  We've know since Rebekah was very young that she has an unusual mouth/throat/trachea anatomy. Her palate is unusually high, the back of her throat is set back farther than normal, and her airway is positioned forward of what is typical.  So now that Rebekah is medically stable from a life-threatening standpoint, we are going to start working on a few life-enhancing issues.  The first, and probably easiest, was the hearing aide.  Next we are going to try to give Rebekah some voice quality back. If you remember, Rebekah's left vocal chord was paralyzed during her open heart surgery last December.  There is no way to repair the vocal chord, but there is a surgery that can work around it to make it somewhat normal again.  However, it is a complicated surgery and may or may not be completely successful.  We have been told that the longer we can wait to do the surgery, the better.  There is another surgical possibility that would involve crossing the muscles in the back of Rebekah's throat to bring her throat closer to her palate.  It would not "fix" her vocal chord, but it would eliminate some of the breathy, nasal sounds in Rebekah's speech.  Right now we are tentatively planning to take Rebekah to Charleston in January for an evaluation to see if she would be a good candidate for the surgery.

I think that covers all of the recent medical updates.  :)  We have been very blessed with Rebekah's good health and thank you all for your prayers for our sweet girl!

 Zachary, Justin, Aaron, Caleb,

Rebekah and Bradley

Nancy

We're Outta Here!

Hi!  I am so excited to say that Rebekah's cath went so well and so quickly yesterday that I never had a chance to get my laptop out and post an update! :)

Rebekah was second case yesterday which meant that she didn't get taken back to the cath lab until about 12:30.  Since she couldn't have anything to eat or drink after midnight, we were a little nervous about how the morning would go.  I have to say, Rebekah couldn't have been more pleasant!  She asked for food and a drink a few times and told us that she was hungry, but she never cried or got upset.  We just told her she could have something to eat or drink after Dr. Baker finished taking pictures of her heart.

So her morning started out with vital signs, and EKG and an echocardiogram.  Rebekah sailed through all of that like the pro she is!  :)  Then we just hung out in the same day unit and waited for the cath lab to be ready.  Rebekah did some coloring, some sticker crafts, made a bead necklace, played with her baby brother and watched a movie.  She also did lots of talking with the nurses and giving hugs. :)

Once Rebekah headed to the cath lab, we went to get some lunch and a bit of fresh air and sunshine outside.  Two hours later Rebekah's nurse called to say that she was done and heading back to the recovery room.

We had a chance to talk to Dr. Baker and Dr. Do late yesterday afternoon.  Both of them were very pleased with their findings in the cath lab.  Rebekah's heart had no scar tissue buildup and no narrowing of any areas that needed ballooning or stenting.  Even more exciting, Rebekah's heart pressures are the best they have ever looked!

Of course, we are so very thankful and pleased with yesterday's findings.  The current plan is to continue the medications that Rebekah is currently on and come back to Charleston in about a year for a CT angiogram just to make sure that scar tissue hasn't developed around any of her conduits or shunts.  If Rebekah's cardiologist feels that she can wean off of the Viagra that she takes, they would do another heart cath at that time to determine her pressures.  But that's a big "IF" and not something we would consider for at least a year.

So Rebekah was discharged around 7:00 last night and we left the hospital, got some supper and crashed in our hotel room.  Rebekah was somewhat restless through the night, but she woke up this morning and has been playing quietly.  About the only lingering effect I have seen are a little bit of a hoarse voice from the breathing tube and a persistent bloody nose, presumably from the oxygen.

The plan for today is to get packed up and have some breakfast, then head for home shortly after that.  We'll probably take a couple of breaks just to let Rebekah out of her car seat to stretch her legs a little.  Tonight her pressure bandages can come off and I'm sure she'll feel much better after that.

Thank you all for your prayers and a huge thank you to our friends who have been keeping our boys.  We really appreciate your willingness to help out with the munchkins.  :)

Nancy