Walk for Williams Syndrome

On May 12, we are walking as a family in the 2012 Annual Walk for Williams to benefit the Williams Syndrome Association (WSA). We are walking for our own Clare Bear as well as the thousands of other children and adults affected by Williams syndrome in our country.

         

When Clare was diagnosed at 6 weeks old, we were immediately connected with the WSA. The WSA is the most comprehensive resource for people and families living with Williams syndrome as well as doctors, researchers and educators. The WSA provides resources, support and the latest medical information to help families throughout their child's life. By sponsoring us in our walk, you will help the largest organization dedicated to improving the lives of people living with WS. By helping the WSA, you are helping Clare.

Support Clare in the Walk for Williams   Please click this link to help us raise money!

Cath Results

Clare did great during her catheterization on Wednesday. We are breathing easier having it behind us as we move forward in what's next for Clare. I always have this feeling that we've cleanly jumped over another hurdle and are gearing up for the next one. But we do get some recuperation time in between!

Clare was such a good sport Wednesday morning waiting for her cath to begin. She was scheduled for 9:30am, so she was cut off food at 11pm and water at 7:30am. However, Clare was scheduled second case and the first case ran late. So Clare did not end up entering the cath lab until noon. She never once complained about being hungry or thirsty and was in fairly good spirits. Since the cath did not begin on time, the anesthesiologist started Clare on IV fluids (at our insistence) while she was still awake. (We have learned from experience that anesthesiologists do not like being told how to do their job, but as long as they keep our child safe, I don't need to like them personally!) They gave Clare a Valium before inserting the IV because she was still traumatized by her lung scan injections from the day before. I don't like seeing Clare so upset and wished she could have already been under anesthesia when they started the IV, but dehydration during a cath while under anesthesia is a huge risk for children with Williams syndrome. So having fluids on board since her start time was being delayed was the safest option for Clare.

As parents, the nerves will never go away, and we will never get used to that horrible moment when you simply have to walk away from your child and literally leave their life in someone else's hands. It's been over four years since Clare has been in the cath lab, but all the old familiar feelings came rushing back. Shawn and I walked Clare down to the double doors before the cath lab, gave her kisses, then went back to the waiting room.

The cath itself was fairly short compared to other caths Clare has undergone. Coming out of the cath, Clare had a comparatively "easy" recovery. In the past, she's had high fevers, vomiting, blood transfusions, trouble with the pulses in her legs, excess fluid and puffiness, high blood pressures, you name it. This time, she looked great, her blood count levels were good so she did not need a blood transfusion (first time that's happened - a sign she's growing up!), and she had no fever or vomiting. She was grumpy the night after and complained of ear pain (she had an ear infection going into the cath), but her ears actually looked fairly clear. Clare had an IV line inserted into a vein in her neck so her ear pain could have been from that as well. Coming home, she had a slight fever and seeping from one of her groin wounds, but those are slowly resolving as well. Today, she is pretty active and feeling good. She will be able to return to school on Monday with restricted activity.
The cath was both diagnostic and interventional. Clare's pulmonary stents were dilated open some more (there was some tissue build-up from the past four years), and the stents still have some growing room. Which means we probably have a couple more years before we have to discuss options for stent replacement. The cardiologist also dilated two of her branch pulmonary arteries, but that was the extent of the dilations. Four dilations is a huge improvement over Clare's last caths in 2007. During those two caths, she had over 15 dilations combined. Her lung scan results post-cath showed improvement in the balance of flow between the two lungs and her systemic pressures in her heart were improved as well. That's the fantastic news!

The not-so-fantastic news is that Clare's ascending aorta and aortic arch has narrowed again. Her echo results over the last four years have shown a zero gradient across the aorta, which is perfect. However, the cath showed that was not the case. Her gradient is actually a 25mm gradient. Without going into the complicated details about gradients and pressures, the basic news is that when Clare had her aorta repaired via open heart surgery as a baby, the gradient in her aorta was 50-60mm. So she is halfway to that point again. The problem is that, unlike the pulmonary arteries, unless the aorta has a distinct "pinch" somewhere, the cardiologist will not balloon-dilate it in the cath lab. And Clare's aorta is narrowing all the way up and through the arch again. It will require another open heart surgery to repair it. Unfortunately, we have no way of knowing how long Clare's aorta has been narrowing again. The last time it was precisely measured was during her September 2007 cath, so it could have been slowly narrowing over the past four and a half years or it could have started six months ago. So we do not know how quickly this is progressing.

We have not gone over these results with Clare's personal cardiologist yet, so we will have lots of questions for her at that visit! Thank you to all for your prayers and supports.

Cath is On

After a stressful couple of weeks with illness in the house among various children (ear infections, coughs, colds, RSV, vomiting), we are finally in Boston and ready for Clare's cardiac catheterization tomorrow. We will not be 100% positive until tomorrow that the cath is actually happening, but we're ready to go.

With Violet and Eliza being sick the last week, we have been so careful in keeping Clare away from them, diligently washing hands, purell everywhere! We didn't want to take any chances with Clare coming down with an illness that would force us to postpone her cath. We thought we were doing good until Clare presented with a slight fever and runny nose over the weekend. I took her to the pediatrician's on Sunday and, sure enough, she had a double ear infection. I thought for sure the cath was off. But after a few days on antibiotics, Clare is definitely not feeling sick in any way (more energy than her exhausted mom, that's for sure!). The team at the hospital said an ear infection alone would not be cause for postponement, so we came on down to Children's Hospital today for her pre-op workup. After a long day of tests and labs and meeting with doctors and nurses, she is cleared for her cath tomorrow morning. One ear is still slightly red, and she does have a small cough, but as long as she is fever- and congestion-free tomorrow, she is good to go!

The plan during the cath is primarily to take a look around Clare's heart and see what's been going on in there over the last four years. The doctors are fairly sure she will need some stent and vessel dilations done while they are in there. The same interventional cardiologist (who is the cardiologist-in-chief here) personally does Clare's caths, so he knows her, knows her vasculature, knows Williams syndrome, and we know she is in the best hands. Depending on the extent of work done will determine what Clare's post-op plan is (one night or two nights in the hospital). We pray that all goes smoothly, and she is discharged on Thursday.

Clare was such a brave, patient girl during the six hours of pre-op today. The worst moment was when she needed her injections for the lung scan and the blood draw. Now we're resting at the hotel for the evening since we've all been up since before 5am. One of the good things about Clare being older is that she is the second case tomorrow which means we can be a little more leisurely in the morning before heading over to the hospital. Right now, her cath is set for 9:30am. Thank you everyone for your prayers!

Happy 9th Birthday, Jamie!

Happy 9th Birthday, Jamie! You light up our lives in so many ways - from your infectious smile to your hard work and achievements in school and soccer to your love of jokes to your concentration and competitive nature playing games and sports to your consideration of your younger siblings and helping out Mom and Dad. We are so proud of the young man you have become! We love you!

Happy Birthday, Violet!

Dear Violet,

Happy 3rd Birthday!

Over the past year, you have transformed from a toddler into a big girl.
From a crib to a bed.
From two-word phrases to conversations that have no end.
From diapers to Tinkerbell underwear.
From Mommy doing everything for you to assertions of independence and insistence on doing it all yourself.
From dancing in princess dresses in the living room to beginning ballet.
From being my baby to being the big sister.

We love you, Violet Grace! Happy Birthday to our big 3-year old girl!

The New Year Begins with a Bang

We started out the New Year with a cardiology appointment. Clare has turned into such a great patient for these appointments. She lays quietly for her blood pressure readings, EKG, and echocardiogram and does everything the techs ask her to do. The boys were at school and Violet was at a friend's house, so Clare loved having the solo time with Mom, Dad, and baby Eliza.

The time has come for Clare to have another cardiac catheterization. I knew the cardiologist was going to utter those words (especially since she and the Boston cardiologist have been warning us of this for about a year now). The cardiologist could not get great readings from the echocardiogram as to exactly how Clare's pulmonary arteries and stents are functioning, so the safest route now is to obtain more accurate date by doing a cath. I do feel blessed that Clare has been able to go FOUR YEARS without a cardiac intervention, but I am also getting the butterflies. During the last cath, Clare went into atrial tachyarrhythmia and had to be cardioverted back to a normal rhythm. And the cath before that, she had a pulmonary stent replaced and ended up in the ICU. But I am a dweller on the positive (for the most part!). Clare is four years older and stronger. Her risk of dehydration and blood loss requiring a transfusion are lower. Her blood pressures have been great for over a year. All in all, she has been fairly healthy, so we are hopeful for a successful outcome to this cath, even if it does involve balloon dilations or additional stent placement.

The catheterization is set for February 1, so we'll be at Children's Hospital for a minimum of three days. Clare is starting to show some anxiety about the upcoming hospital stay. She has some memories of her hospital stay a year ago when she underwent dental surgery (such as she remembers the mask with the "sleepy medicine"), but has lots of questions and comments about "fixing her broken heart," as she puts it. We're already seeing that an older Clare will be a whole new experience at the hospital.

The Journey

Happy New Year!

Being on the other side of the preparing-for-Christmas season (otherwise known as Advent), I finally have a chance to sit, catch my breath, and reflect on the last few weeks. We had a wonderful Christmas (celebrating over three days in three different states) and have enjoyed the last week simply hanging out at home enjoying each other's company (and, yes, the zillion Christmas presents the kids received). It has also been a prime time to finish Violet's potty training. Now I have to get her trained to wear pants again because we have been letting her run around in just her underwear all week. At least it was a success!

Advent is always part-joyful/part-stressful for me. Like most Christian parents, I struggle over that balance between the trappings of the outward exuberance and secular fanfare of Christmas and the inward joy and reflection of celebrating Christ's birth. I think Shawn and I did an okay job this year with the Advent season. I always believe we can do better (as in less parties, less pre-Christmas gratification, and less snapping at children because the cookies aren't turning out the way they should), but I also know we are leading our children down the right path when it comes to the true meaning of Christmas. The "Jesus stocking" was full of good deeds, the kids looked forward to the lighting of the Advent candles and the singing of carols and prayers each evening, and we gave of ourselves both in time and materials (singing at a nursing home, creating prayer cards to hand out, buying gifts for giving trees at school and church, donating food to the soup kitchen). Christmas is probably the #1 season of the year when I am so aware of the blessings in my life and all the goodness that God has given us as a family.

A week before Christmas, I accompanied Simon to his preschool Advent party. As we worked on crafts together, we found ourselves seated at a table with a particular classmate of his who has a . . . shall we say . . . overbearing mother. After nagging at her preschooler over his sloppy handling of the glue and foam angel wings, she snapped at him, "Oh, just let me do it." He then spent the next ten minutes fidgeting on his chair, staring off into space, and experimenting with how far he could bring his foot over his head while she focused intently on gluing each piece exactly perfect, straightening her son's photo on the ornament, then carefully applying glitter decoration. When completed, she exclaimed over her flawless ornament, "Now I can always remember this year at preschool!"

Following this incident, Simon and I took our "very obviously made by a 4-year-old" ornament and moved to another craft table, where we were seated with his main teacher. She was telling me how Simon told her about our upcoming Make-A-Wish trip, and she was interested in hearing more about Clare. I shared a bit, and we moved onto discussing Simon and his love of letters. I told her that Simon desperately wanted to learn how to read and has asked me frequently if I could teach him. And how I would love to teach him and would be so proud of him if he learned how to read at four years old, but that it would also not bother me or worry me whether he learned now or not. I ended with, "If there's one thing Clare has taught me, it's that children will do things when they're ready, and I just have to be there to support them and guide them in whatever way they need me to."

These two encounters back-to-back really got me thinking about how I live my life with my children. Am I always looking to what's next? What we've accomplished? Where we're going? I felt bad for that mom who had her perfect angel ornament which was really no reflection on what her child was like at four years old. I felt bad that she missed the point of doing the craft with your child, not for your child. We have learned so much in these past almost-nine years of parenthood. One of the things that Clare in particular has showed us is that the journey is just as important as the destination. Over the final weeks of Advent and Christmas, and now as we begin a new year, my Number One Resolution is to remember that - live the journey. Be there in the moment with the ups and downs. Focus on what I am doing right now in my life. What's going on in my home with my husband and children. What we are experiencing, whether it's good, bad, or in between. We'll get to our destination, some way or another. Live the journey.