Rebekah's Heart

It's finally here!  The diagram, with explanations, of Rebekah's heart!  :)  First, though, a diagram of a "normal" heart to give you a little perspective (thanks to Wikipedia for this easy-to-understand heart diagram!).

Okay, now this is the diagram of Rebekah's heart.  If you notice the cute little lacy, scalloped edges around the inside of Rebekah's right ventricle, it's not that way cause she's a cute and frilly little girl. :)  Actually, that means the wall of her heart is thickening due to her heart having to work extra hard to push against the high pressure in her pulmonary artery and lungs.  And I forgot to explain that the right sub-clavian artery is made of a dashed line because it actually travels behind the aorta and the superior vena cava.  We definitely have a beautifully unique little girl!

(If you are having a hard time reading all of my notes, click on the diagram and it should open larger in a new window.)

Nancy

Making History!

Ahhh. That's my sigh of relief at finally being back in the land of electronic communication! :) Although, I am borrowing Drew's laptop at the moment, as he is trying to resurrect mine. Something about not reading the hard drive.... We do not have an internet connection at the CrossBridge house, so this is the first time we have been able to connect and send an update, other than texts from our phones.

So, we made it to Charleston late Wednesday afternoon and got settled in at the CrossBridge house. It is so comfortable, and we are so thankful that it was available to us. Rebekah did well during her pre-op procedures yesterday (Thursday) and even suffered through a second blood draw after part of the first blood draw clotted.

Dr. Bradley, Rebekah's surgeon, came to talk with us yesterday afternoon and really surprised us! We had been prepared to discuss the removal of Rebekah's right lung, and that was not his plan at all! Because Rebekah has not had any issues with infections or pneumonia in her lung(s), Dr. Bradley is content to just leave her lung intact and watch it. If in the future Rebekah's collateral arteries grow, or if technology develops to allow the use of that lung, he would be in favor of attempting to connect it to her pulmonary artery. If Rebekah begins to develop chronic lung infections or other lung problems, Dr. Bradley would reconsider removing her lung at that time.

Drew and I are very satisfied with this approach. Of course, we want whatever is best for Rebekah, but we were disappointed with all the talk of removing the lung. That would have left Rebekah with no chance at all to ever use that lung. While Rebekah may never use the lung, at least she will have some kind of a chance if the lung is not removed.

Now for the long explanation of Rebekah's heart. This information was all brand new to us, so we were a little overwhelmed with the complete turn around after our meeting with Dr. Bradley. The short story is that Rebekah is completely and totally unique. There is not (or has not been) another documented person with Rebekah's unique anatomy combination of Truncus and a missing pulmonary artery. Even a visiting professor from London, who only studies the anatomy of children with heart defects has never seen or heard of this type of combination. SO....all of that leads to a lot of uncertainty as to what might or might not happen when Rebekah's heart is repaired and whether or not her body will tolerate the repair.

So let me tell you what we know for sure that Dr. Bradley will be doing. At this time, Rebekah should be intubated and prepped and surgery should be starting at any time. Dr. Bradley said that it will take him about an hour to work his way through the scar tissue left from Rebekah's first surgery. After that, Rebekah will be placed on ECMO (the heart/lung bypass machine) while Dr. Bradley works on creating a pulmonary artery from the right side of Rebekah's heart to her left lung. He will be using a tube-graft, which is made from a Gore-tex material and a human donor valve. This conduit will allow the blood from the right side of Rebekah's heart (unoxygenated) to flow into her working lung, receive oxygen, and then will return to the left side of her heart to be pumped out through what will become her aorta (the "trunk" that originally supplied both her body and her lung with blood).

Now comes the tricky part. See, in a straightforward Truncus repair, the next step would be to close the large VSD (ventricular septal defect), which is the hole Rebekah has between the right and left sides of her heart. BUT, because Rebekah's conduit will only be supplying blood to her left lung, the concern is that too much pressure will build on the right side of her heart. Imagine a balloon in a small box with a straw in one side. The straw represents the pulmonary artery that is only going to Rebekah's left lung (instead of splitting into both lungs). Now as you begin to inflate the balloon (pumping blood into the right side of her heart), about half of it (or more) will flow through the straw, but after a while the balloon will begin to expand and have nowhere to go. Over time, it would create too much pressure for the balloon and eventually it would burst. Now, imagine that a hole was in the side of the box. There is now an escape vent for that extra pressure to flow into and relieve the stress on the balloon. That, in a nutshell, is a good picture of the concerns Dr. Bradley has about Rebekah's heart.

So, Dr. Bradley will create the conduit and then run a Flow Test to check the pressure inside the right half of Rebekah's heart. If the pressures are high, he will be leaving the VSD open, or at least partially open, in order to allow blood to flow into the left side of Rebekah's heart and relieve some of the pressure. The last thing anyone wants to do is create high pressures (essentially high blood pressure) in her heart. Over time, that would certainly lead to heart failure. On the other hand, leaving the VSD open will result in Rebekah's oxygen saturations remaining low and will increase the likelihood of her remaining on oxygen after surgery.

Now, you may be thinking of the biggest question that we had after Dr. Bradley explained all of this: What will happen with Rebekah long-term? Well, the answer is that no one knows. :) As Rebekah grows, so will her lung (obviously), and as her lung grows larger, it will have the capacity to hold a greater volume of blood. If (when) that happens, Dr. Bradley would be able to close the VSD, possibly even in the cath lab and not during open heart surgery, because her left lung would be able to hold the full volume of blood from the right side of her heart. Of course, if at any time there were collateral arteries that formed to the right lung, they would be used to form a pulmonary artery and would give Rebekah the use of both lungs.

In the short-term, the main concerns are for Rebekah's recovery after surgery and what kind of stress this surgery will put on her heart and lung. No one has wanted to make any kind of speculations about the recovery time, although we are all hoping that it will go smoothly and quickly! Dr. Bradley called in his best team for this surgery - seriously! We were told by several people this morning that the team Dr. Bradley put together for Rebekah is the best there is. Apparently he has different people that he calls depending on the complexity of the surgery, and we got the "A-list."

Just a few minutes ago, we were paged with our first update. The surgery began on time, and Rebekah was stable. Everything is going as planned. The earliest we can hope for this surgery to be completed is around 1:30 this afternoon, but could be as late as 3:30, depending on how things go.

Our biggest prayer today is for wisdom. There is no cut-and-dried technique for Dr. Bradley to follow in Rebekah's heart surgery. A lot of his work today will be trial-and-error, and just plain "gut feeling." He has been working miracles for a long time, and we have been told he just has a sense of how things are going to work when he is in the OR. Please pray for wisdom and divine guidance as he determines the best course of repair for Rebekah's heart. Also, pray for Dr. Bradley's assistants, Cathy and Zeh (pronounced like "hay" with a z), the anesthesia team, the nurses, and the rest of the OR staff as they monitor Rebekah.

Thank you so much for caring and praying for our little girl. You have no idea what an encouragement it was to check facebook today and see all of the posts and requests for prayer that have gone out for Rebekah. Also, thank you for the prayer vigils that are going on today. I know of at least two churches whose members have signed up for around the clock prayer times during today. Thank you all so much for your prayers!

Nancy

Standing.....Finally!!

We were so excited that Rebekah's stander finally arrived!! We picked it up on Friday and Rebekah has been in it several times this weekend.

After all of last week's appointments and running around, we are looking forward to a (hopefully) calm week. As of right now, Rebekah only has her regular therapy this week on Monday, Wednesday and Thursday. Yippeee!!!

Just me being cute!

Uh-oh! Look what I found!!

Since we moved into this house in 2006, Drew has taught each of the crawlers to master the stairs. Aaron was the first and Caleb had his lesson last year. Looks like it's almost Rebekah's turn for stair training!

Melanie and Rebekah in the pool

Rebekah had her pool therapy evaluation on Wednesday with Melanie. Rebekah and Melanie will be working on standing, sitting 90/90 (sitting on her bottom with her feet on the floor and balancing), squatting, standing from a sitting position, and balancing on hands and knees. Rebekah will be in the pool every other week on Wednesdays instead of her regular physical therapy. This week's pool therapy went well for the first thirty minutes.....until Melanie put Rebekah on her hands and knees. Rebekah let us all know how unhappy she was about that, and that was the end of pool therapy! Melanie has learned Rebekah's tricks; next week, hands and knees will be the last thing we work on at the end of the hour. :)

Rebekah in the stander watching her brothers play the Wii.

Look at me standing!

The stander is very heavy - much heavier than we were expecting! It is on wheels, so it is very easy to maneuver Rebekah around. There are brackets for Rebekah's feet and velcro straps to keep her feet in place. There is a pad for Rebekah's knees to fit into and velcro straps around her shins. The yellow bar around her back has a large pad on the front of it to hold Rebekah's bottom in place. Finally, the wide velcro strap goes around the middle of Rebekah's back to keep her from falling backward from the waist. There is also a head/neck support, but since Rebekah has good head and neck control, she does not need it. I will need to take the stander back on Wednesday for her therapist to fine tune a few areas, but overall it is working well. Rebekah is just on the edge of small for this stander, but it is the smallest one made. The pad for Rebekah's shins is just a bit big, so we may have to make some adjustments for it to fit Rebekah correctly. It also seems that Rebekah is not putting full weight on her feet; she seems to be resting/sitting more on the pad on her bottom. I would like DeAnn to take a look at Rebekah in the stander to make sure everything is positioned correctly.

The back of the stander.

Rebekah has been tolerating her stander quite well. Because of church today, we only had one session in the stander, but Rebekah stood for 30 minutes today in that one session. DeAnn would like to see Rebekah standing for 30 minutes twice a day by Wednesday when we go back for therapy. The long-term goal is three sessions a day for 45 minutes each time. I think in two or three weeks Rebekah will be able to do that, providing we don't have surgery in that time frame.

My awesomely cool, new sneakers - size 2!!

Rebekah and I spent a couple of hours on Saturday trying to find some appropriate sneakers for her standing. DeAnn requested that we get hard-soled sneakers with good arch support so her feet get the correct support. Has anyone tried shopping for that type of sneaker in a size 2?? Almost impossible!! We tried Stride Rite, Kid's Foot Locker, Journey Kidz, JCPenney, Gymboree, and Children's Place. The closest we came to finding what Rebekah needed was a size 3 at Stride Rite. We finally left the mall and went to Babies 'R Us and found exactly what we needed. And at less than half the price of Stride Rite! :)

I'm liking this new gadget!

Caleb, Aaron, and Rebekah

The boy are not about to be outdone in the picture-taking department! When I've gotten the camera out this week, they have been begging to have pictures with their sister. Of course, I quickly oblige!

Caleb and Rebekah

Does it count that they are just in the same vicinity at the same time? Rebekah kept trying to scoot toward the camera and Caleb kept chasing after her. I couldn't get Caleb to understand that if he sat still in one place, I could put Rebekah next to him and snap a quick picture. Out of a dozen or so, this is the best I got. :)

Excuse the bed-head; this was after my nap!!

Notice the blue-ish feet in the above picture? It's a look we're seeing a little more these days. This evening when we got home from church, we noticed the blue feet and "gills," as Drew likes to refer to the area around Rebekah's mouth. When we connected Rebekah's sat monitor, she was hanging out around 71-72 on 1/2 liter of oxygen. We bumped the oxygen up to 3/4 liter, but I am anticipating turning it down when we go to bed. Rebekah tends to have much higher sats while she is sleeping since her body is relaxed and her heart is not having to work as hard to pump the blood through her body. We'll see how she looks tomorrow when she is up and more active. Dr. Lucas had mentioned last week at Rebekah's appointment that if she needed as much as a full liter of oxygen, that he would not be comfortable with her being at home. I'm not sure how I feel about that. I agree that the potential for Rebekah to go into respiratory distress is greatly intensified by the amount of oxygen required to keep her stable, but I'm also cautious about going in to the hospital when she is not really "sick." I don't want her to pick up something else that we didn't go in with! Anyway, we're praying that Rebekah has her heart repair before we get to the point of needing a liter of oxygen!

I'm off to check on my little people and get into bed.....nights tend to be very short around here with the new school schedule. Thank you to all who have prayed for the boys as they started school. They both love their teachers and are making some new friends. The adjustment of getting back into a school schedule has really gone smoothly!

Nancy

We're Home!

Thank you so much for all of the prayers, phone calls, texts and Facebook comments today! We are so blessed by all of our friends and family who care for our Rebekah!

Rebekah is standing!! She loves the pool!

Another favorite...music!

I wish that I could say Rebekah is doing much better, but she isn't. On a good note, she's not any worse, either! :) Rebekah continues to be on 1/2 liter of oxygen, and is resting comfortably with oxygen sats in the 70's. As Drew posted earlier, Rebekah's sats dropped rather quickly this morning into the lower 60's, and within an hour she went from happy and playing to very tired and lethargic. Several phone calls with the on-call cardiologist, and we were on our way to the Children's ER. Once we arrived at the ER, Rebekah had chest and abdominal x-rays, an EKG, and a panel of bloodwork. Thankfully, everything looks normal, meaning there is no infection or viral illness. It is the general consensus that Rebekah is outgrowing the band on her left pulmonary artery, and she is close to needing surgery to repair her heart. Rebekah's cardiology team had originally predicted that the surgery would not be necessary until next spring or summer, but Rebekah has grown rapidly (for Rebekah, anyway!) in the last three months. The growth is putting increased demands for oxygen on her heart and lungs, and it is becoming harder for her heart to pump an adequate amount of blood through that pulmonary band. We will get final confirmation of that diagnosis on Tuesday, but all indications are pointing toward surgery at this time.

Rebekah all dressed up for church before we had to leave for the ER.

Smiling for the camera! :)

That's the most recent news....now, let me try to tie up some loose ends from the last month. Rebekah had her final hearing evaluation done, and the result is no hearing loss!!! We were very excited to hear that! She will continue to be evaluated once every six months for the next year or two to make sure that fluid is not building up in her ears, but we should have no worries about her hearing. Rebekah's most recent visits to both her endocrinologist and her immunologist went very well. All of her labwork looked great, and the doctors were very pleased. We are certainly not out of the woods with the complications of DiGeorge Syndrome, but things are looking very well right now. This is a huge relief to us, especially if we are heading into surgery soon!

Great-grandma sent Rebekah a sock monkey and hand-made mittens.

Rebekah loved the monkey.....

.....and promptly used the mittens for a teething ring!

I promise she will actually wear them someday, Grandma!!

Rebekah loves to swing!

Someone has some cute dimples! :)

A perfect pouty face. Must have learned that one from Daddy......

Thankfully this is the face we see most often!

In developmental news, Rebekah is progressing in leaps and bounds! She has temporarily "graduated" from speech therapy for now. She will see her speech therapist on a consultative basis until she is closer to the age of two, when formal speech therapy can begin. As far as her feeding skills go, Rebekah has shown enough progress to not need therapy any more!! In occupational therapy, Rebekah is working on stacking blocks, coloring and pushing/pulling small levers, knobs and beads to strengthen her hands and arms. And she has also had some fun time with Play-Doh! Believe it or not, we still have not received Rebekah's stander, but I was promised that it shipped last week. We'll see if it comes in this week! Rebekah is doing well standing with someone supporting her. She is also crawling in her own way; we like to call it bunny hopping! :) This week we are adding a new therapy - aquatics!! The therapy center we go to has a pool for aquatic therapy, and Rebekah's physical therapist believes she will make great progress in the pool. So we will switch between regular "dry" physical therapy one week and aquatic physical therapy the next week. Rebekah loves the water, so I'm sure she will enjoy the pool.

All five monkeys! Beth, that is the little wagon you bought for Rebekah that came with sand toys. She actually rides around in it with her brothers pulling her. :)

Guess what?!!! I'm standing....with a little help! ;)

This movie shows off a little of Rebekah's "bunny hop" crawling. Caleb was watching the screen with me and keeps chattering about "Bekah" and "pictures." At the end, you can see Rebekah squinting and blinking. That's her "camera face." Anytime I pull out the camera, she does this cute squint/blink thing.....in anticipation of the flash, maybe? I think this girl has been photographed a lot.... :)

Thank you all again for your prayers for Rebekah and our family. Please pray for wisdom for Drew and I this week as we watch Rebekah closely, and for the doctors as they evaluate her heart on Tuesday. Also, please keep our boys (especially Zac and Justin) in prayer as they begin a new school year tomorrow.

Nancy

Sunday adventures

They have run lots of blood tests and x-rays. At this point they can not find a reason for Bekah's needing oxygen support outside of her heart issue. They are sending her home this afternoon on 1/2 liter of o2 and we are to take her back if anything changes. Bekah has an appointment scheduled for Tuesday with Dr. Lucas. I am not sure if he will see her tomorrow or if we will wait until Tuesday. They will be doing a complete echo and looking at her heart function along with her pulmonary band. We suspect that the echo will show us the Bekah has progressed closer to needing her heart repair. She has done a lot of growing in the last couple months. Which will cause her band to get small quicker. So we will await what Dr. Lucas has to tell us and see how we will proceed towards Bekah's next hurdle.

Please continue to pray for Rebekah and all the details surrounding her and her care.

-DE

The Plan of the Day

This morning started out eventful, if nothing else! Dr. Gwinn, the pulmonologist paid us a visit shortly before 9:00 this morning. After checking Rebekah out, she felt like we needed to have a consultation with cardiology. Her reasoning was that we have been treating Rebekah's lungs for basically a month now (counting her last hospital stay and our time at home), yet Rebekah still continues to need an ever-increasing amount of oxygen to keep her sats in an acceptable range.

While we understood that Rebekah's oxygen dependency could be cardiology related, we have heard for weeks now that the reason was respiratory related. So this morning, we hear from respiratory that it's cardiac. We hear from cardiology that it's respiratory. Can I scream now?????

Rebekah watching her brothers on their Sunday visit. We actually got to take Rebekah downstairs so her brothers could see her for about an hour.

So after a phone call from Dr. Darby (pediatrician) to Dr. Lucas (cardiology), it was determined that we are likely looking at a cardiac-related issue with Rebekah's breathing. Yes, she does still have a slight lingering respiratory illness, but nothing that would cause a need for a liter of oxygen. Her x-rays from Saturday looked good, and all of the blood work that was done also looked good.

The fish tank downstairs in a small waiting room. The boys love to watch the creatures inside, particularly the sea urchins like the one Justin is pointing to.

Dr. Lucas believes that the band on Rebekah's left pulmonary artery may be getting too small, not allowing enough blood to get to her left lung. This would result in needing more oxygen to compensate for the lower function of her left lung. On the flip side, we were hoping that one (or more) collateral artery would begin to take over the function of a right pulmonary artery to the right lung and make up for the reduced flow to the left lung. We were warned that it did not appear that a collateral artery had formed, and this scenario would back that theory up. Of course, all of this is just speculation until we get to Charleston in March for Rebekah's heart catheterization.

Rebekah hanging out with Daddy.

Rebekah and Aaron

Rebekah and Zachary

Justin and Rebekah

The other complication that has arisen is that Rebekah has begun to have diarrhea again as of yesterday. She has had two negative c. diff tests, so it is a bit of a puzzle as to what caused the diarrhea to begin again. Dr. Darby did have stool samples sent to the lab to check for a variety of other possibilities. We should find out about that tomorrow.

Rebekah was happy after seeing her Daddy and brothers.

After all of the consultation and talks back and forth, it will be interesting to see what tomorrow brings!

Nancy