Happy Birthday, Clare!

Where have the past seven years gone? They flew by with days of smiles, days of tears, days of joy. You are so beloved, our sweet firstborn daughter. You have proven from the moment you were born that you are a fighter (you were so tiny in your daddy's hands!) and can accomplish anything you set your mind to and your heart on. Happy 7th Birthday, Clare Bear! We love you so much!

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.

Six Months of Sweetness

Eliza is six months old today!

It has been a whirlwind of a six months, and I cannot believe my baby is halfway through her first year. Eliza is such a bundle of sweetness in our lives. I have had a couple "high-maintenance" babies, and Eliza is anything but. She is so mellow. She rarely cries, so when she does, we all sort of freak out! She is such an easy-going girl and goes along with whatever we have planned for our day. All babies are a blessing, but I admit I am glad to have a baby who is low-key at this phase in our life.

All the children are as infatuated with her as the day she was born. The boys fight over who gets to push Eliza's stroller up the hill when we pick Clare up from school. Simon and Clare ask first thing in the morning to sit and hold her. Jamie's favorite job is to put Eliza into her pajamas at bedtime (he even changes her diaper, if it's not dirty!). And Violet exclaims "Hello, baby!" when she sees Eliza.

It does not matter how many kids you have - it is always thrilling to watch them grow and achieve their milestones. Eliza is working very hard on sitting up for longer periods of time (I still hold out hope that she'll be sitting at Christmas), and I am eager to watch her over the next six months as she learns new skills.

Happy six-month birthday, Eliza Marie!

Looking Up

Things are slowly getting back to normal in our house. Our kitchen is 100% functional again. There are some minor details we need the contractor to take care of (such as my kitchen sprayer hose only comes out about a foot), but I will not complain. I WILL NOT COMPLAIN! After being upside-down and inside-out for nine weeks, I WILL NOT COMPLAIN! Our basement playroom is almost there. I am still looking forward to the day when there are no more people in my house making messes, though! Hopefully in the next few days everything will get wrapped up, I will be able to unpack the boxes from my kitchen cabinets, and the contractors will move their tools and garbage out of the garage. (The contractors have been using my garage bay to store everything. For weeks it held our Corian countertop, side cabinets, and other items. I was using Shawn's bay for our van, but after last week's incident, I determined all would be better if I just parked the rental van in the driveway. It's a little tricky getting in and out of Shawn's bay with the van because of the curve of our retaining wall. However, after scraping ice off my parked-in-the-driveway car this morning and having to bundle everyone up just to walk to the car to drop Jamie off at school, I pushed - because it was all too big to lift - everything from my bay of the garage to Shawn's. I mean everything - bags of garbage, garbage cans, boxes, air scrubber, table saw, snowblower, humongous box holding our old dishwasher, everything. I was desperate! After one winter of having a garage, I am spoiled by it now.)

My van is still in the shop, and it hasn't even been started on yet. The big ice storm at Halloween put such a backlog on all the repair shops (at least this is the story they gave me!) that it will probably be another couple of weeks before we get our van back. This is irritating and inconvenient (our family of seven doesn't quite fit the greatest in our rental van, and I hope we don't have to make any of our Christmas trips in it), but this is a time where I feel like I have to swallow the pill I've been given without complaining because it was me who put us in this position.

Our house is decorated for Christmas - the tree is up, lights twinkling in and out, and we set up our New England village throughout the first floor. It is such a peaceful time at the end of the day to sit in the darkened living room and enjoy the beauty of the Christmas lights. It is one of my favorite things about the Advent season. Our shopping is done, so our evenings and weekends can be spent enjoying Christmas concerts, school parties, Jamie's futsal games (form of indoor soccer), our Advent wreath, outdoor Nativity walk, cookie swaps, Williams syndrome Christmas party, Make-A-Wish Christmas party (where the photo was taken - they had a fun photo booth Clare and I tried out), Clare's ballet recital, drinking eggnog, and driving around looking at lights.

And I finally met a kindred spirit at Clare's school! I have felt so much like I was on-the-sidelines for the year-plus Clare has been at her school. Never quite fitting in with the other parents because the path I walk with my child is so different from theirs. However, when you're standing around after school watching your children play on the school playground with an AED (which is another story for another day!) at your feet, you're bound to draw someone's attention. Which I did, and I am so glad for it!

Focus

I ripped the slider door off my minivan this morning coming out of the garage. Yup, ripped it right off, throwing the van door back into the garage where Jamie, Simon, and Clare were standing, shattering the back window, and showering Violet with glass. Our guardian angels were definitely watching out for us this morning because the huge, heavy door landed about a foot away from the kids, and Violet was scared, but unharmed. We were running late, I was fuming at the bickering kids who were all very unhelpful this morning in getting out of the house on time (Shawn being away on business), and I (obviously!) misjudged my clearance pulling out of the garage with the sliding door still open. It was not a pretty sight.

Almost four hours post-incident, I am still irked at myself that it happened at all. I am NOT that kind of person - distracted, irrational, fly-by-the-seat-of-my-pants, making rash judgements. But I was that kind of person this morning, and now I have my mess to clean up for it. It really put my life at this moment in focus. We're in a crazy place right now with our life. (Wait, you have five kids - isn't it all crazy?) Actually, it's not always crazy. Like every family, we have our moments of chaos, meltdowns, temper tantrums, and toys everywhere. But we also have our structure, routine, priorities, and our focus on what's important. And that keeps us running smoothly. Usually.

We had a dishwasher leak eight weeks ago that led to a huge mold problem in our kitchen and basement. Since then, we've had remediation companies come in to clean the mold, our kitchen and half the basement playroom gutted, and now are on the tail end of everything being reconstructed. My kitchen is either packed away in boxes in the garage or in my dining room (which is where my stove, dishwasher, and fridge currently reside). We have nowhere to eat, I cannot access my mudroom or garage (which means dirty shoes in and out of the living room), and my house is filled with strangers making messes. When my house is in chaos, I am in chaos. I want things organized, in their place, and clean. And, right now, my house is anything but so I am anything but!

Add to that, I also feel as if we go through cycles when it comes to Clare and what's going on with her. Right now, there seems to be so much going on. We are dealing with issues with her kidneys, muscle pain in her legs and feet, exercises for her mouth because her tongue is weak, home stretching program, limiting certain items in her diet while adding increased fluids. And that doesn't even touch on what it takes to help her through her school assignments. In addition to the services she receives in school, she has three private therapies after school. So three days a week, I pick the kids up from school, and we all go to some sort of therapy and arrive home at dinnertime. Last year, Clare did her one private therapy in the early afternoon since she had morning kindergarten. I (naively) thought this year would be easier for so many reasons (two kids in full-day school, Simon in a preschool closer to home, two little ones that nap in the afternoon), but, in many ways, I feel as if we are on overload as a family. And I am at a loss what needs to be done about this. Clare needs her therapies. There are times when I wish we were doing more for her. As it is, there are so many different exercises, stretches, lessons I should be doing daily that sometimes don't get done. Where do we draw the line? I don't want to cut out things that she needs, but I have four other children and my sanity to think of. This morning's incident was a wake-up call that I am so much on overload most of the time, I am not making good decisions. I am not taking the time to process and think things through (such as let's pull out of the garage first with the sliding door open, then I will get Simon's car seat and put it in the car, then load up the kids). That didn't work out so well!

This morning's trauma also made me realize how blessed we are with such good friends. We do not live near any of our family, so I can't call my mom or sister to come help me out in a pinch. I have never been the type of person to have a wealth of friends (being slightly on the introverted side at times). There are limited amounts of people that I can ask for help. But, this morning, I saw that there is just enough. That God always provides. Simon, Clare, and Jamie each go to a different school, and three good friends were able to rearrange their schedules and put up with some inconvenience (like crying 2-year olds who didn't want to be in the car!) to pick up each of my children and bring them to and from school today. That made my day so much more manageable to deal with my mess with only the two little one who still nap.

As we approach Thanksgiving and enter the Advent season this weekend, my focus is on my family, preparing my heart for Jesus' birth, and the blessings in my life. I am going to try to let go of the craziness and busy-ness that has consumed me lately. Even though I do not have a kitchen, I do not have my car, and I have a lot on my mind when it comes to Clare, I want to focus on the three things that are truly important to our lives - faith, family, and friends. May this Advent season keep me in that spirit.

(So this is from Monday, but I couldn't get Blogger to publish until today!)

Make-A-Wish

A few weeks ago, the Wish Granters from Make-A-Wish came to our home to meet Clare and talk to her about making a wish.

Make-A-Wish is something I have thought about on and off for a couple years. I was unsure whether Clare even qualified for Make-A-Wish, but, if she did, I wondered whether that was something we should nominate her for. Part of me felt like this was not right for us because Clare does not have cancer (which is what I am sure most people associate Make-A-Wish with, as I did), she is currently stable heart-wise, and I just didn't want to "take advantage of the system." Then we heard about WS-friends of ours getting ready to have their wish fulfilled and others being nominated for Make-A-Wish. The little voice in my head would wonder again about it. Then her cardiologist told us that Clare needs another catheterization in the next few months. Then the nephrologist diagnosed her with calcifications in her kidneys and hypercalcemia, and we discussed other scary topics such as renal failure. And over the past several months, Clare has been suffering pain in her lower legs and feet. So we've been doing x-rays, visits with the orthopedic surgeon, private physical therapy, and fittings for foot orthotics. If any child deserves having a wish granted, I truly believe Clare is one of those children.

Clare was nominated, she met all the qualifications, and the Wish Granters paid us a visit. Clare was eager for their visit and knew immediately what her wish would be. Ahead of time, they informed us that they would need some back-up wishes in case they could not fulfill Clare's top wish, so we did talk to Clare about it.

Top three wishes:
1 - To meet a princess.
2 - To meet Jesus.
3 - To visit Planet Earth.

The Wish Granters assured us that they will be granting Wish #1! A trip to Disney for our entire family is now in the works. It was very important to Clare that she goes on her trip when she can go swimming, so we won't be going to Orlando until sometime next spring. But we're obviously all so excited. For a couple years now, Shawn and I have wanted to take Clare to Disney to see a "real" princess (and give that experience to all our children), but we just have not been able to do it financially. It is still surreal that we will actually be going and seeing our princess' wish fulfilled.

Eliza at Four Months Old

Eliza is now four months old! She really is the easiest baby I've ever had. (Finally got it figured out by Number Five!) She is very mellow, rarely cries (so when she does, everyone goes on super-alert), and sleeps like a champ at night. She has the same big blue eyes as her older sister Violet, but her hair is darker like Clare's. At her four-month pediatrician appointment, she weighed over 15 pounds - more than what Clare weighed at a year old - and Eliza has all the delicious rolls to prove it. We all are so in love with our sweet baby girl!

Under Our Belt

We now have one month of school under our belt. Jamie is enjoying third grade so far, although the amount of homework has increased, which is an adjustment for all involved. I am definitely of the opinion that school and homework come first, but I admit it is tough to squeeze it in on those days we are also juggling one of Clare's after-school therapies and Jamie's evening soccer practices. There are a couple days during the week where homework is done somewhere other than home! But Jamie is an avid student and a hard worker. He landed a speaking role in the school play (Pinocchio), and Shawn is going to help out with some set-building this year.

Simon started at a new preschool this year. It is a school that is closer to home, and he entered school knowing two children (from our church) in his class. So he is very happy with the change and loves his school. Simon would go to school every day if he could! One day a week, he stays at school for a full-day and has such a great time. He asks me every morning if this is the day he "gets to eat lunch at school." I know most young kids love school (it's only when they get older that starts to wane!), but I always loved school. (Yes, even high school.) I hope my children always love school, too. There will be good days and bad days, but I want them to always wake up eager to see what the day will bring.

Clare has taken to her new school schedule better than we expected. There are days she is tired, and, by the end of the week, she is exhausted, but we all are! But there has only been one day where she came home exhausted to the point of almost not being able to function, and that was on fire drill day. She was so set off from the fire drill all day that both her kindergarten and first grade teachers told me it was a "rough day." Other than that, Clare has adapted very well to going to school all day. She loves eating lunch at school and now orders hot lunch a few times a month, which is even more exciting to her! So exciting that she ordered it on a day she was not supposed to because I knew she would not like it and I had packed her a lunch. She ended up not eating lunch at all because she didn't bring her lunchbox to the cafeteria and didn't like the hot lunch. (Told you so!) Now we have a simple note-system worked out with her aide, so Clare only orders hot lunch on the days I can guarantee she will eat it. But she loves eating with her friends and her favorite part of the school day is that she often gets two recesses (one in the morning with kindergarten and then the after-lunch recess with first grade). The best of both worlds!

Clare had her nephrology follow-up last week - kidney ultrasound and visit with the pediatric nephrologist. The calcifications in her kidneys looked the same (not increased, which is good). The nephrologist is still hopeful that Clare's kidneys will grow without the calcifications growing, so she will eventually have more good tissue than bad tissue. We continue to monitor her calcium and sodium intake, but now are under orders to increase her water consumption to 2 liters (or 60 ounces) a day! That is a lot to get into a 6-year old who does not drink very often. Her recent calcium levels in her urine were high as well, so we are prepping for some follow-up tests (including a 24-hour urine study) to determine whether Clare needs to go on medication as well. The doctor again stressed the importance of water, water, water to filter through those kidneys. Clare's aide in school has been great in getting Clare to drink more water at school (and patient with the increased bathroom trips), and we have upped our efforts at home, too. Hopefully we'll see some improvement in her numbers when she has her 24-hour study done.

Our last appointment on the checklist is orthopedics next month. Then we're set (hopefully) until her next nail-biting cardiology work-up (in which we will discuss scheduling her cath). We have some other exciting things in the works for Clare (such as planning another trip to Kentucky to see Dr. Mervis in February). I know it's going to be a great school year!

Fashionista

Violet is a mystery to me. At the ripe old age of 2 1/2, she loves to accessorize. And this girl can accessorize! I am not an accessory girl (quite the opposite!). I wouldn't call myself a tomboy exactly, but I don't like dressing up or wearing make-up or jewelry. We went to a pool party last month, and took Violet from her nap directly into the car. After the 40-minute drive to the party, when I went to get Violet out of the car, she was wearing a pearl necklace, pearl bracelet, and a crown. I am not sure where she had those stashed in the car, but she was ready for the party. It took a little convincing that pearls were not exactly swimwear!

From the time Violet could walk (and have free access to the house), she has adorned herself with baubles and bling. She wants to wear a dress or skirt every day (calls it her "princess"). I think we'll be in trouble the day she discovers make-up! (Or Clare introduces Violet to it.) In the age-old debate of nature versus nurture, Violet is proof that little girls are made this way. I certainly did not teach her this or model this behavior! Violet loves her little bit of glamor, and I love seeing what she comes up with next.

One Day Down, 179 To Go

Clare had her first day of school yesterday. The day before, we went to the playground at Clare's school with friends. The teachers were busy getting their classrooms ready. While we were there, Clare's special education teacher from last year saw us sitting by the playground, so she came over. She shared with me the news that she was no longer the special education teacher for the school, but had her own second grade classroom instead. I was a little shocked. (This doesn't warrant a phone call over the summer to introduce us to the new special ed teacher? Apparently not.) So now the night before Clare begins school, I am worried that the new special ed teacher does not know Clare, does not know the IEP, does not know what is going on. And this is another big year for Clare since she is doing a transition year - morning kindergarten and afternoon first grade. Add in lunch, new IEP goals, and a new schedule. A schedule developed just for Clare, a rare occurrence in the school, and not something that will necessarily go smoothly in the beginning since there are so many people involved. And now a new special education teacher? So I was a little freaking out (to put it mildly).

That afternoon, I took Clare to her first grade orientation. I had 30 minutes in which I planned to meet her first grade teacher, chat with the new special education teacher, introduce myself to the new nurse (yet another new person on Clare's team since the old school nurse transferred), walk Clare through the lunchtime routine, and get information on the pick-up procedure. We met her new first grade teacher (super nice, very young, and enthusiastic), Clare saw some of her former classmates, and that was it. The 30 minutes were done, orientation was over, and we had to leave to pick up Jamie from school. So now I was a little more than just freaking out.

Clare, on the other hand, was counting down the days to her first day of school, especially since Jamie had started school the previous week. She was so excited the night before school started (and cried when we left her orientation because she wanted to stay). We packed her new pink backpack, planned her snack and lunch menu (and went over again and again that snack was for snack and lunch for lunch and never the two shall meet!), and gathered all her school supplies. She helped lay out her clothes for the morning, and Clare was ready. I am so grateful that Clare did not seem to have half the trepidation that I had about this new school year. I believe she was a little nervous when we first arrived at orientation because she wanted to hold my hand the whole time (not something she usually wants to do in a crowd - she usually wants to be out socializing, especially since she saw lots of friends). But she quickly overcame her nervousness once she saw her first grade classroom and got to sit at a desk. And now that the night before school starting was finally here, Clare was a bundle of eagerness for it all to begin.

Clare woke up at 5am (and I convinced her to lay down in the guest room for another hour before I was functional). Jamie did not have school yesterday, so the entire family was able to bring Clare to school. The joy on her sweet face was contagious. She just could not wait. Before school started, we were able to meet the new special education teacher, which eased a lot of my anxiety. We found out that Clare will have the same aide all day. I still want to meet with the nurse, but we're planning on setting up a team meeting in a couple weeks to review what's working and what's not. Even though we've been over the plan with Clare multiple times, I think she was a little confused that she was lining up with kindergarten again (especially since she saw her friends from last year going somewhere else), but she had a big hug for her kindergarten teacher. She waved eagerly at us from the line, and off she went.

The rest of our day was chock-full (it is always good to keep busy on days like this!). Simon had his preschool orientation that morning as well. I left Violet with Shawn (who was working from home), and took Simon to his school, along with Jamie and Eliza. Simon went home from orientation with a friend, so Jamie, Eliza, and I went out to lunch. I never get to go out with just Jamie (and Eliza slept the whole time, so she was a quiet third wheel), and we had a great time together. Then we came home, played Clue, woke Violet up from her nap, and it was time to pick up Clare.

The nerves came back walking up the sidewalk to the pick-up area. I know it was silly because what was done was done, but I just wanted my girl to have had a great first day. She had been looking forward to this day for weeks. Her class was one of the last classes out of the building, so after what seemed like forever, we finally saw the class. Clare was so tiny next to her classmates, and she did look tired. But she had a big smile on her face when she saw us, and we were greeted with huge hugs. We lingered to talk with the first grade teacher while Clare and her siblings went on the playground. Miss R said Clare had a fantastic day. She came into the first grade during circle time and transitioned beautifully, sitting down quietly and joining right in. Lunch went well, and Clare loved going to the library in the afternoon (which was the first grade special that day). Clare told us all about the library ("I even went to the bathroom in the library!"), but that was the extent of the information we got out of her. She was tired (and had a little cry when we told her it was time to leave the playground), but not the meltdown, non-functioning exhaustion like I had prepared myself for. She enjoyed seeing her brothers and sisters again ("I missed you, Jamie!" she proclaimed). Once Simon came home from his friend's house, Clare helped Shawn make pizzas and we had a pizza-movie family night.

So the first day went better than I expected. I will never be satisfied with the amount of information that Clare is able to impart to us, but I know she had a good day, her teachers were enthusiastic about her day, and Clare was disappointed when she woke up this morning to find out it was Saturday. I pray next week is just as wonderful!

Back to School

My favorite time of year - fall! (Oh, and back to school!)

I have enjoyed the laziness of (most) summer mornings and all that we have crammed into eight short weeks. But I always look forward to getting into our routine again. And I will say it - having a break from the frequent chaos, sibling bickering, and craziness. With the school schedule, we have a predictable pattern of frantic mornings, calmer mid-mornings, quiet early afternoons, and then back to a loud, boisterous household for the late afternoon/evening. I like the predictable. I like the routine. I like the calm before the storm!

Jamie started the third grade last week at his Catholic school. Clare has her first day of her transition kindergarten/first grade year on Friday, and Simon starts at his new preschool next Wednesday. So we are slowly being eased into our school routine. Which is good for me because with a new baby, I am still figuring out how to be showered and dressed by 9am, never mind getting five children dressed, feeding them breakfast, and packing lunch boxes and backpacks! (And before he starts huffing and puffing - yes, Shawn, does a lot of the morning grunt work as well.)

In most families, back to school means going out for new school clothes, buying school supplies, and getting haircuts. We did get some new uniforms for Jamie (but that's so easy that I passed along Jamie's sizes to a friend who was already going to the uniform store that I didn't actually have to shop!). We did buy some school supplies. No haircuts, though (we like them buzzed). In our family, back to school also means I try to fit in all the doctor's appointments for Clare that I can. So far, she's gone to the endocrinologist, cardiologist, and dentist. I have her new schedule for private speech therapy and hippotherapy lined up. She has upcoming appointments scheduled with nephrology and orthopedics. Nothing shocking or unexpected from these appointments so far, but I feel better having them behind us so we can focus on Clare's academic needs.

I am taking Clare to her orientation on Thursday for first grade. (They go over pick-ups, drop-offs, navigating the lunch room, and I start freaking out.) We went over to the school today to find out which first grade classroom she is assigned to, and we were able to see who her classmates were. Then she and Simon played on the school playground for a few minutes. Clare was thrilled to show Simon "her" playground, and she cannot wait for school to start. She asks me every morning if she has school today. She is excited about having her kindergarten teacher again. She is eager to bring "shells and cheddar" (her favorite all-time food) in her new thermos container for lunch. I know Clare cannot wait for Friday to come. I will be glad when Friday is successfully over! I cannot believe Clare will be in full-day school. Where has the time gone?

The World of Simon

With the advent of Facebook, it is so easy for me to simply post the cute, funny sayings that go on in this house on Facebook when, in the past, I would collect them for the blog. There are so many blog readers who are not on Facebook, though. And I would hate for you to miss the little joys in my life!

Like my sweet, precocious, four-year old Simon. He has always been extremely verbal and super-smart (for example, he knew all his letters and numbers, both in recitation and recognition, before the age of two). I never know what's going to come out of his mouth, and there are so many daily occurrences where he cracks me up. So here are some quotes direct from the World of Simon:

Simon: We are going downstairs to play secret spy.
Me: Okay, see you later, alligator.
Simon: You don't need to call me alligator.
Me: Sorry - I thought that was your spy code name. [trying to be funny]
Simon: [in all seriousness] No, my code name is Sparkles.


Simon: Jamie told me when you kiss a girl, that's how you get a baby. I just kissed Eliza and I kissed you, so maybe I'll get a baby.
Me: That's not exactly how it happens. You won't get a baby from kissing Eliza.
Simon: Oh, you're right. Jamie said you have to kiss a girl you don't know. [Gotta love older brothers and their wisdom!]


Simon [checking every stall in the girls' bathroom]: "Awwww, man, where are the urinals?!?!"

Cardiology Merry-Go-Round

Once again, we have a new pediatric cardiologist for Clare. This is our fourth cardiologist in six years. We loved #1 (who we met when Clare was barely 24 hours old), but she moved to Virginia. #2 was nice personally, but really disappointed us professionally. #3 was incredible personally and professionally, but having Clare's primary cardio in Boston did not end up working for our family and Clare's various medical needs. So we're on #4. We switched back to Children's Hospital at Dartmouth, but went with a new doctor practicing there.

We met Dr. G for the first time at Clare's six-month visit on Friday - EKG, echo, and examination. Dr. G is young, vibrant, thorough, and wonderful with Clare. A good fit for us so far. Her office is literally seven minutes from our house, we know from experience that we can pop in for a quick peek or blood pressure check if the occasion arises, and she practices in the same larger group as Clare's pediatrician, endocrinologist, and nephrologist, so they have access to each other's notes and Clare's medical records. That all works for us, so I pray that Dr. G works out! We've never really been that picky or unhappy with any of our providers, so it's been weird to feel as if we have not really had a cardiologist on board over the past two years.

Clare's visit went as well as expected. She remains clinically stable, which is the good news. Her gradients remain about the same, and her blood vessels are growing, but still not at a great rate (this is not unexpected with the severity of Clare's stenosis). The echo could not get a really good view of the pulmonary stents, so it's unknown how they're holding up. (Four years ago, one of Clare's stents was actually crushed in her artery and the echo did not pick that up. The doctors found out when they were in there during a cath.) Her ascending aorta and arch continues to look fabulous (this is the portion that was surgically repaired six years ago). Her descending aorta continues to also look stenosis-free. Her EKG was solid, and based on that, her coronary arteries seem to be good as well. Her blood pressures were fabulous, so she can continue off any hypertension meds. We have been monitoring her weekly for over a year now off meds, and the pressures are usually very good for Clare's condition. Her pulses were good, and all-in-all, a wonderful examination.

However... (and here it is, the not-so-good news)... Dr. G agrees with the previous cardiologist's assessment that Clare should undergo a cardiac catheterization in the near future. Prior to Clare's appointment, Dr. G had already spoken with Dr. B, the liaison cardiologist in Boston (Dr. B is a different cardio than the one we had been seeing as a primary in Boston, and he has been involved with Clare's case since her first catheterization at three months old - Dr. B is our cardiologist whenever Clare is inpatient at Children's - I know, it gets confusing!), and she reviewed the Boston echos on paper (Shawn is going to get her a digital copy of the actual echo video). Since it has been an amazing four years since Clare last underwent a cath, all agree that it is time to look at what's going on inside her vessels, other than just doing echos. Right now, the plan is to do another full work-up with Dr. G here in Manchester in six months, then schedule the cath a few weeks after that.

Dr. G cannot say right now what the plan will be during the cath, but we have been through this six times before and are quite familiar with the nature of this beast. I am 99.9% sure that Clare will have her pulmonary stents and smaller pulmonary vessels dilated. At the very least, the doctors will shoot a lot of dye and take a lot of pictures! They will be able to get better views on how her stents are holding up, what her coronary arteries look like, and maybe even exactly what her renal arteries look like (we've had those scanned recently, but a cath is always more accurate). So we'll hold tight for six months and take it from there.

Baby Blues

Eliza turned six weeks old yesterday. Unbelievable! I feel as if we are finally settled into some sort of pattern in our day. Eliza sleeps in her cradle, wakes 2-3 times at night to eat and goes right back to sleep, and is more alert during the day. She will sleep in the car seat and is a great passenger, but hates the bouncy seat and swing - so much so that I put the swing away completely and only put her in the bouncy seat for 20 minutes for my morning toilette. She usually naps in the playpen instead. In those first couple weeks, I never thought we would get there (I always feel this way at first - must be those hormones!). Part of me loves having the baby in bed with me, but the part that wakes up with a sore neck and back from falling asleep while nursing the baby is not so fond of it. But here we are, six weeks later, with a baby who predictably will spend the night in her cradle, and is "easy" enough overnight that Shawn at least gets a full night's sleep. (I am not bitter - this works in my favor for sleeping longer in the morning and letting him start the mad breakfast rush with the older children.)

Our bedtime is not as predictable, though. Right now, my sweet husband is doing his nightly duty of baby rocking since I have declared a nursing freeze for the next hour. Eliza will struggle to fall asleep for hours at night. Once she is down for the night, it's golden. But getting her there is frustrating. She fusses, needs constant position changes, wants to nurse, nurse, nurse until I want to scream, scream, scream. Shawn and I alternate between me nursing and him rocking. Some nights, we do this for a few hours. And, again, I know we will get there (since I have four older children currently asleep!), but the getting there part is so hard sometimes. This time with our tiny baby is so short and I don't want to wish it away. But it would be nice to get a little more sleep!