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!!

A Great Week!

This is another post to let you know that there isn't much to update on! Rebekah had a really good week, and we are so thankful that the rest of the family also seems to be over all of the sickness we have had lately.

Rebekah had her nine-month well visit on Thursday, and it was a relatively quick one! There were no major problems or issues to discuss, which is just fine with me. The only thing we really had to talk about was scheduling both a hearing and vision evaluation sometime in the next couple of months. DiGeorge Syndrome can cause problems with both vision and hearing, so we are making sure that if there are any problems we can correct them quickly.

This week should also be a fairly quiet week. On Tuesday, Rebekah will see the pediatric surgeon here in Greenville to have her g-tube changed to a little larger one. It should be a five-minute procedure done in the office. The reason for the change is that Rebekah has grown some from when she had the last g-tube inserted in January, and this one is just snug enough that it is rubbing and causing some irritation around the g-tube. It should be a little more comfortable for Rebekah once we get it changed.

On Wednesday, Rebekah will meet with a new speech therapist for an evaluation. If you remember, Rebekah was receiving speech therapy at home in the fall, but we discontinued the therapy because the therapist we had was not trained to work with infants. I felt like the therapy sessions were wasting our time and the therapist's time as well. Our Early Interventionist has not been able to find any speech therapist trained to work with Rebekah who will come to the house, so we will go to them! During Wednesday's evaluation the therapist will mainly be watching Rebekah's mouth and tongue as she eats and drinks. That is a good indicator of how her oral motor skills are developing in relation to her age and other developmental skills. We already know from Rebekah's OT (Hi, Beth!) that Rebekah is not moving her tongue from side to side like she needs to be in order to move food around in her mouth to chew it. Right now, anything that goes into Rebekah's mouth gets pushed straight to the back of her mouth, and she either swallows it (pureed foods) or chokes on it (anything solid). We'll see what the speech therapists have to say on Wednesday!

That about wraps up this update. Hope you all have a great week!

Nancy

Wednesday

Rebekah finally got her bath around 9:30 last night. She was not really pleased by our efforts to clean her up, either! :) It is so fun to see her whole tummy now that everything has been removed. The larger wound at the bottom of her incision is where the chest tube was, and the two smaller wounds on either side of that are where the pacemaker wires went into her heart. All of those are now gone until our next surgery!!

Rebekah was not appreciating bath time as much as Mommy was!

I actually was able to speak to Dr. Ringwold this morning about several questions we had. First, Rebekah's calcium looks great - the best it has ever been in her little life! They will recheck it again tomorrow (more for my peace of mind than anything) just to make sure that it is continuing to remain at acceptable levels. So for now it looks like she will not need a calcium supplement, although that could change and fluctuate throughout her life.

Now to discuss Rebekah's feeding......She is scheduled for a follow-up swallow test on Friday. We are not sure yet of the exact time. Dr. Ringwold was very honest with me this morning, and his feeling is that Rebekah will need to go home with a feeding tube. She has so many factors that she is working against in order to eat, and the goal for the next few months is growth. At this point, the doctors are more concerned with her growth than they are with how she is eating. So, given the fact that babies with DiGeorge syndrome are typically small and have a hard time growing, and the fact that our babies tend to be small anyway, and the fact that Rebekah is aspirating both her feeds and reflux, and the fact that she only has one truly working lung and doesn't need it infected from aspirations, and the fact that she is refluxing significantly and add to all of that the fact that she is a heart baby and recovering from open heart surgery, the doctors feel it is in her best interest to move ahead with the feeding tube. (Anyone want to diagram that sentence???? :)

All clean and cozy and ready for a nap

There has not been a final decision made yet, but it is likely that Rebekah will have surgery again by the beginning of next week to put a permanent feeding tube (called a G-tube) in her stomach. I won't give a lot of details now because there are several different options the doctors could choose with the feeding tube surgery, but we will be sure to let you know more information about it when we know for sure what is happening.

What we do know for sure is that most babies with DiGeorge do have a feeding tube for the first year or so of their lives. During that year, Rebekah will continue to receive OT so that when she is able to eat by mouth, she will have built up the muscle tone and coordination of everything involved in the eating process. Rebekah is also having trouble with her feedings compressed to one hour. About half of the way through the feeding, she becomes very agitated and cries a lot. It is likely that compressing the feeds over a shorter period of time is probably going to distress her even more. If Rebekah does have the G-tube put in, she will go home with a small pump that we will be able to put her milk into and we will be able to run it over a time that is comfortable for her.

Other than the feeding issue, Rebekah is having a great day! She is sleeping lots and looks so peaceful snuggled up with her blanket and teddy bear. :) Drew is probably coming back down tomorrow, and I know that Rebekah will be happy to have her Daddy back - and so will I!! :)

Nancy

Onward and Forward!

Pictures!!!! Rebekah had a really great day today. After having the ventilator removed this morning, she held her stats all day (oxygen, heartrate, blood pressure) exactly where the doctors hoped to see them. Based on that progress, Dr. Hsia feels confident that he applied the correct amount of pressure to the band around Rebekah's left pulmonary artery.

Rebekah tried out the pacifier again today.

One of the things Rebekah will be working on now that the ventilator is out is learning to eat by mouth. To help with that, we are encouraging Rebekah to suck on the pacifier. She gets tired easily, but she kept it in for about 10 minutes this afternoon.

One of my favorites!! :)

It was so much fun to see Rebekah's face today! She has this cute little habit of sucking on her bottom lip just like her brother Caleb did when he was little. You can really see the dimple in her chin when she does it.

Yesterday Rebekah had quite a bit of fluid still making her puffy and swollen, especially her face and hands. Today we were pleased that almost all of it is gone. The doctors even decreased the amount of lasix that she is receiving each day (the medication given to help decrease her fluid).

According to Rebekah's lab work today, her calcium levels are dropping off. We have been expecting this to happen as it is commonly seen with DiGeorge syndrome. Rebekah has received some calcium supplements off and on over the last week, and the doctors are waiting to see what she will do when she is receiving full feeds. Right now she has just been receiving IV fluids, but she was started back on a little milk this evening right before we left. As long as her body tolerates the milk, they will be increasing it in small amounts. Once she has proven that she can tolerate the feeding, the doctors will remove more of her lines and IV's.

Hopefully tomorrow will bring more positive steps toward home!

Nancy

First Day Post-Op

Sorry for the lack of pictures.....Rebekah asked us to hold the flash photography today. :) Seriously, she seemed to be struggling with the brightness of the lights in the unit, and I really didn't want to add to that. We'll be taking more pictures of her beautiful little face tomorrow.

Aside from having a hard time waking up from yesterday's anesthesia and pain medications, Rebekah has had a great day. Her heart rate, blood pressure and oxygen saturation were within the ranges that the doctor was targeting. During the day, the respiratory therapists worked on turning down the ventilator settings and Rebekah sailed through each of the trials. When we left the PCICU for the day, Rebekah was more awake and the plan was to take her off the ventilator during the night shift.

Rebekah had a small dose of morphine this morning and otherwise has just been on Tylenol throughout the day. She has seemed to be resting very comfortably during the day - so comfortably that it was hard to wake her up!

The results from the genetic testing on Rebekah came back positive for DiGeorge Syndrome. We met with a geneticist this morning to discuss a bit about the syndrome and what possibilities may come from it. There are many different potential problems including: cleft lip and/or palate, cranial-facial deformities, auditory problems, endocrine system problems (including calcium deficiency and thyroid problems), immunology deficiency (weak immune system), kidney deficiency, mid-line heart defects, and developmental delays and/or learning disabilities. Rebekah has not exhibited symptoms of most of these things, but obviously some are too early to tell. So far we have ruled out cleft lip/palate, cranial-facial deformities, kidney deficiency, and we already know about the mid-line heart defect (obviously!). As Rebekah grows, we will be monitoring her endocrine and immune systems closely and watching her developmental milestones. She will have a few more tests done while she is in the hospital to check the function of her immune system and she will have a hearing test done. Ultimately, no matter what the doctors say is or isn't "wrong" with our little girl, we know that she is perfectly the way that God has made her, and she is perfect for our family.

We are excited about Rebekah's progress. Tomorrow some of the lines and tubes she came back from surgery with may be removed if she continues to do so well. Rebekah will also be started back on some small amounts of milk tomorrow if she tolerates it well.

That's about it for today. More updates and pictures to come tomorrow!

Nancy

The Long-Awaited Update!

For our first trip to Charleston, I thought we did fairly well - we didn't get lost even once! :) There are more one-way streets in Charleston than I have ever seen in one place before, but we found that it is a very friendly place, especially the people we met at the hospital.

Mommy and Bekah at 30 weeks

Mommy and Rebekah's first trip to Folley Beach! We didn't stay too long, but we did get a couple of pictures of Rebekah's first beach trip, and I got my feet wet. :) The water was actually nice, although as you can see from the picture, it was very windy.

Rebekah's first smile for the "camera"!

Before we left for Charleston on Wednesday, I had a visit with Dr. Greig to measure my fluid and check Rebekah's growth. My fluid is still hanging out on the high side - it was 24 (8-25 is normal) on Wednesday and 20 on Thursday when it was checked by the doctors in Charleston. No problems with fluid this pregnancy!! :)

Rebekah continues to grow well. Overall, she is a small baby (then again, all of my children are fairly small!) - she is measuring in the 22nd percentile for overall growth. Her head, arms and legs are all about average and her abdomen is measuring about a week small. She is only in the 11th percentile for her abdominal growth, but her growth has been steady and has not dropped off, so the doctors are not at all concerned about it at this time. Rebekah is now 2 lbs. and 15 ozs.!!

Every technician commented on how much hair Rebekah has! It's the kind of bumpy-looking stuff on her head in the ultrasound pictures.

We arrived in Charleston around dinner time Wednesday and enjoyed dinner at Olive Garden (Mommy's favorite!!). We checked into our room around 7:00 and after getting settled, we spent about an hour walking in and around the hospital trying to familiarize ourselves with where we were going. The hotel we stayed in was very close to the hospital - only a 10 minute walk. The Ronald McDonald house is even closer - just two blocks and we were pleased to find that it is very well-lit at night.

We started Thursday morning with an 8:30 appointment at Pediatric Cardiology. The ultrasound technician (Probably she has a much fancier title than that, but I don't know what it is. Sorry, Karen!) had someone in that took priority over us, so we waited until a little after 9:00 to get in. Karen began the scan, and took around 150 pictures of various angles of Rebekah's heart. Rebekah certainly put on a show for us - rolls, turns, arching her back, making funny faces. We were having fun watching her! After the scan was done, we were told that Dr. Shirali (cardiologist) was called out and we could go wander the hospital (or check out the cafeteria!) for a while and come back to finish the visit. We had been in the cafeteria just looking around for less than 10 minutes when Karen and Donna (not sure what Donna's title is, either, but she is our contact at Ped. Cardiology and super helpful!) came to the cafeteria to find us. Turns out that our prenatal wellness appointment scheduled for 10:00 could not be bumped later because there were not going to be any available doctors during the afternoon. So, we were given directions to Prenatal Wellness and sent on our way!

I love baby toes!! :)

When we arrived at Prenatal Wellness, we were called back by yet another ultrasound tech, Melissa. Since this was the one and only chance for us to meet the doctors there, they wanted to do a complete ultrasound themselves before we deliver. Rebekah obliged and treated us to a few smiles and even waved her hand at us!

During the ultrasound, Melissa found a cyst on one of Rebekah's kidneys. Dr. Chang came in to discuss it with us, and reassured us that it was not anything of concern. It is something that will be checked further after Rebekah is born and may be monitored as she grows, but for now it is very insignificant.

After the ultrasound, we met with Dr. Goetzel to go over the plan for induction and review my previous delivery history. Dr. Goetzel was very pleased to see that I have never gone into pre-term labor, and we were very pleased that she is not a proponent of Cesarean sections! In fact, in Dr. Goetzel's words, I am a very low-risk for c-section! :) That made me very happy!

After our appointment at Prenatal Wellness, we headed back to the Children's Hospital and sneaked into the cafeteria for a quick lunch before we went back to the Cardiology department. Once we arrived back in Cardiology, we were able to meet with Dr. Shirali. First of all, Dr. Shirali confirmed that Rebekah does indeed have Truncus Arteriosus. After reviewing our records and the pictures and information from the scan that Karen did, Dr. Shirali also told us that they were unable to detect the branch of the Pulmonary Artery leading to Rebekah's right lung. Dr. Shirali believes that there is blood flow to the lung based on the fact that it is growing at the same rate as the left lung, but he is not sure where or how the blood is flowing to it. This will probably mean a more complicated heart surgery as the surgeons will have to trace the pulmonary artery and its branches to see exactly where the blood flow enters the right lung. It is possible that the artery will have to be moved, reattached in a new location, or replaced if it is not supplying adequate blood flow to the right lung after birth.

Dr. Shirali also discussed with us the possibility that Rebekah may have DiGeorge Syndrome, which we had briefly discussed with Dr. Lucas a few weeks ago. DiGeorge Syndrome is not life threatening, but there are a few minor things that Dr. Shirali felt that we needed to be aware of if Rebekah does, in fact, have DiGeorge Syndrome. DiGeorge Syndrome can cause cleft lip and/or palate (We know that Rebekah does not have cleft lip based on her pictures!), lack of calcium absorption (would require calcium supplements and vitamin D supplements), low immune system (This would be outgrown as she gets older, but may require extra precautions early on and possibly delay some vaccinations.), speech delays, learning disabilities, some facial differences, and mid-line heart defects. Usually all of these symptoms are not found in any one person with DiGeorge; this is just a list of possibilities that may occur in a person with DiGeorge Syndrome. They all occur with a range of severity from mild to very severe. Because of Rebekah's Truncus, her cord blood will be tested for DiGeorge after birth. To read more about DiGeorge Syndrome, you can check out the information from the Mayo Clinic website here. (Just don't get too overwhelmed reading it all!)

Sweet little face!

All in all, we had a very good experience in Charleston and feel like Rebekah will have a terrific team of doctors and nurses taking care of her. We are looking forward to Rebekah's birth day and getting to meet her and hold her. :)

Nancy

P. S. Anyone want to join us in Charleston July 10th?? :)