Need to Stay Healthy

Clare is almost back to normal. Her rash is 95% gone (one week later!) and the itching is almost gone, too. She had moments when she scratched herself so much she would bleed. Our fabulous cardiac nurse at Children's is on the hunt for the anesthesia records to find out exactly what medications Clare received, so we can red flag those for the future. I think the rash was the worst part of recovery!

Clare's left wrist is still very bruised from the angio-embolization, as is her groin. In her wrist, she has a hard lump where the glue is in her vein. The Big Cahuna said that, over time, the glue will dissolve. By then, the vein will have shut down because of the occlusion. Clare goes back next Friday for a follow-up with the Big Cahuna to make sure that the glue is working. You can't feel the thrill in her pulse anymore, so I have to think it is working. Other than that, the only other noticeable recovery is that Clare is still working the anesthesia out of her body. Her muscles are floppier than usual, especially her upper body. When I pick her up under the arms, there is almost no resistance. Many times, my hands just slide right up her arms and I almost drop her - there is so little resistance, she cannot even hold her trunk muscles tight enough (like a 30-pound newborn!). I have been doing a funny "under-the-butt-first" pick-up, if she needs to be carried up the stairs or in and out of her car seat or dinner chair. I am afraid I am going to pull her arms out of her sockets! The increased floppiness means that Clare has to work harder to do anything, so she is exhausted by lunchtime. Especially since she is back in school four days a week. She had two therapies in school today and we got the brunt of that for the rest of the day. I wish she would nap! The best I can do is have her settle down for a movie, read books on the couch, and do other quiet things, like painting race cars that we can assemble tomorrow. It is very frustrating because I know she needs to nap, but try reasoning with a four-year old over that.

Since Clare is on the mend, I now have some sort of kidney obstruction. (Maybe that's one of the rules about a large family - all of us cannot be healthy at the same time.) After a few days of a new pain in my lower back and other symptoms, I had an ultrasound done this week that showed my right kidney is distended, most likely due to some blockage or infection. I am on antibiotics, but it is becoming increasingly painful, so I am going in for a CT scan tomorrow. I am praying that they find something on the scan. If not, I will be encouraged to undergo a more invasive test that involves an isotope injection. If I do this test, I will not be able to breastfeed Violet for 24 hours. I also cannot take any of the medications the urologist wanted to prescribe to soothe my enlarged kidney because of breastfeeding. So it's been a tough few days and I am trying to figure out what to do. Right now, I am pumping for the first time in six months to bank some breastmilk in case the urologist does want me to do the second test. This is where breastfeeding gets tricky - trying to balance my own health and comfort against my baby's. Right now, I can handle the pain, but I am a little worried about what comes next.

Home Again

We arrived home with Clare this afternoon a little after 2pm. She has been in great spirits, but still has the rash all over her body which is now itching. She is like a little bear all over the house - rubbing her back up and down on the carpet, shifting from side to side in her chair at dinner, sticking her hand down her pull-up - all in the attempt to calm that furious itch! Before we left Children's today, I talked with a doctor from the anesthesiology department. They are going to flag Clare's file and compare the medications used in her anesthesia from this visit against her past visits and determine if they used anything new. The consensus is that Clare is having an allergic reaction to one of the anesthesia meds. She also spiked another fever this afternoon, which the cardiologist feels is just a reaction to the glue, being an artificial substance in her body. So we filled her up with Tylenol and Benadryl and put her to bed. We are all ready for a good night's sleep. Thank you again to everyone for all their prayers and well-wishes! We all made it through another one.

A New Day

I just got off the phone with Shawn, and Clare Bear is feeling much better. She woke him up at 4:30 this morning with, "Daddy, I feel better!" Her fever is gone and her heart rate is back in the 120s. She is still a little puffy and has the rash, but the doctors are pretty convinced it is a reaction to something in the anesthesia and not a fluid build-up. Shawn said her rash looks more pink now than flaming red, so it is improving. Clare was eating a little breakfast when I called, so if she keeps that down, she will probably be discharged today.

I am going to take Jamie to his allergy shots this morning then head back to Children's. Hopefully to bring Clare back home with me! (Oh, yeah, and Shawn, too!)

Successful but Sick

The best news is that the procedures were considered successful. However, they were also incredibly long and Clare ended up under anesthesia for six hours. Other than her open heart surgery, I think this is her longest time under anesthesia so far. So now she is pretty sick from post-anesthesia "complications." Fever, high heart rate, vomiting, unexplained rash over her face, trunk, and back, and fluid build-up. When I left the hospital at 8pm, she was starting to fall asleep. She is still receiving IV fluids since she is unable to keep any water down. She is being treated for her fever, and the nurse was waiting for the pharmacy to send up Benadryl (for the rash) and Lasix (for the fluid). The plan tonight is to treat her various symptoms, keep her as comfortable as possible, and see how she is feeling in the morning. Everyone expects Clare to be much-improved by then and, if she is, she can come home tomorrow.

I am not going to go into tons of details because it is probably boring to most people and I am exhausted, but the frenulectomy was successful in that it was done quickly and with no bleeding. We will not know if it makes a difference until Clare is back in school and working with her speech therapists. The embolization was also successful and the fistula is now closed. The interventional radiologist was able to save her radial artery and just use glue to close off the vein (the vein is not as important as the artery since there are only two arteries in the arm, the ulna artery and the radial artery, but there are numerous veins - here is your anatomy lesson for the day!). The aneurysm was not repaired - because of its placing, it would be very complicated to get to it and, right now, it is not making an impact on her hemodynamically (my dad was impressed I knew that big word - basically the aneurysm is not interfering with blood flow or causing added stress on Clare's heart, like the fistula was). Clare will return to IR in two weeks for another ultrasound scan to be sure the fistula is still closed and blood flow has returned to normal. Then we will just have to keep an eye on her aneurysm periodically to see if it ever develops into something more serious.

All in all, we are very happy with the outcome of today, but pray that Clare gets over the anesthesia effects quickly and can come home tomorrow. We all miss our sweet little girl!

The Waiting Game

I dislike the waiting part of the day almost as much as I dislike soothing the increasingly cranky 4-year old who at Day 2 has already proclaimed she is "all done" with the hospital.

So far this morning has gone off without a hitch. It was a fight to get Clare to take her normal morning meds, so the anesthesiologist gave her Versed (the mellow drug) through her IV instead of orally. (Amy - I totally understand now when you said Avery would just lose it if anyone came near her.) Clare is definitely at her limit with being poked, prodded, stuck, and wired up. We met with the team of doctors and nurses this morning, and the Big Cahuna went over the procedure and even drew a neat little diagram on the dry erase board for us. Depending on what they find when they actually enter Clare's vessels, the procedure may take anywhere from 3-6 hours. So we have some LONG waiting ahead of us. They are going to access the vessels through her groin and put in an arterial monitoring line as well (which I am not crazy about because that is what originally caused this whole mess in the first place almost four years ago!). Some things are still undecided such as how they are going to close off the vessels, whether they can save the radial artery, whether Clare will need a blood transfusion, yada yada yada. So we're just going to wait, wait, wait, and see. We are not allowed to stay with Clare when she is prepped and intubated, so we said our good-byes as they wheeled her away. She was extremely mellow at that point.

Thank you for all the thoughts and prayers this morning. I keep thinking that this is going to get easier some day, but the huge pit in my stomach always comes back as we prepared to hand our little princess over. In the words of our current hospital-expert Amy, "Prepare to be unprepared."

Going to Bed Now

Last update of the night - General anesthesia and interventional radiology will just see us in the morning. Lovely. The one big downer of this whole thing (not counting the fact that we're here in the first place) is that with four different departments involved, it's a lot of hoopla just to figure something out. Logistically, Clare's caths go so much smoother because only cardiology is involved. Now we're dealing with cardiology, general anesthesia, general surgery, and IR, so it gets very complicated with who's doing what and who's going where.

We met with the cardiologist tonight who went over the brief amount of information he knew regarding what was going on tomorrow. I have to say that he, his top nurse, and the general surgeon have been fabulous in nailing down these IR people to get this thing done. If it wasn't for them, we would still be in fistula-limbo. (Not sure what that means but it does not sound like a pretty place to be.) The doctors are fascinated by Clare's fistula (it's a rare occurrence in a 4-year old) and everyone wants to feel the thrill and listen to it. One of the doctors even let Shawn and I listen to it with her stethoscope. Not as exciting to us as it was to her, but interesting, I guess.

We also found out that Clare is on for the frenulectomy as well. The general surgeon will come in right at the beginning of anesthesia and clip that tongue once and for all! Hooray! That means that, not only will Clare finally have this done after four years of waiting for the right time, but she won't have to undergo anesthesia a second time in the process.

The IV finally went in around 8:30pm and was every bit as traumatic as I expected. They wrapped Clare tightly in a blanket so she wouldn't kick or flail her other arm. She screamed like a banshee and kept begging me, "Help me, Mama!" It breaks my heart, but all I can do is keep calm and stroke her hair and promise to pick her up when it's all done. Thankfully, the IV nurse made it a one-stick deal. A prize of Mickey Mouse checkers brought a tiny smile to Clare's tear-streaked face when it was done. Shawn was going to lay down with Clare in the hospital bed, put on a movie, and hope she falls asleep quickly. At midnight, the plan is to start her IV fluids so she is well-hydrated in the morning.

Violet and I are in the hotel for the night and ready for sleep. (Violet already beat me there.) I am heading back to the hospital at 7am because Clare will be taken in for anesthesia around 7:30. I will try to update as I can, but we really have no idea what to expect tomorrow. We are just praying for the very best!

More Fun Pics

We're still just waiting so there is nothing else to do but take pictures and post them!

(And I am glad that Clare is smiling now because she won't be once the IV goes in.)

Addendum: So I have to explain some of the photos because Clare was a riot today at the hospital. The top photo is her in her princess nightgown riding around the cardiac floor in the evening. She is such the little charmer. Shawn pulled her around the floor, and she waved at everyone. Practicing for when she is Miss America or something!

The photo of Clare in the bed talking on the phone - it is a play cell phone that she insisted on bringing to the hospital. She would "talk" on it frequently throughout the day. The cardiac floor is a strict no cell-phone floor because of all the equipment, so once you hit the elevators, everyone whips out their cell phones and turns them on, including Shawn and I. And including Clare. As soon as we got to the elevators, Clare would open her pink cell phone as well and start talking.

The last photo is Clare putting hospital bracelets on her stuffed animals. The hospital bracelets are printed on a sheet and come with five ID bracelets and a bunch of labels. Clare's nurse Kathy (awesome lady!) let Clare keep the rest of the bracelets, so Clare put one on each of the pink stuffed animals she brought with her. (Pink is the new favorite color in our house.)

Pre-Op Fun

Oh, the hospital life ain't the life for me!

My goodness - we are told to show up at noon for admittance and pre-op. It is now 7:00pm - Clare has been weighed and measured, an EKG done, and we met with the cardiac anesthesiologist. Seven hours and that is all that has been accomplished so far!! We still have not met with the interventional radiologist, cardiologist, or general anesthesia. Plus the dreaded IV and blood work has not been done yet. But, in the meantime, Clare has had a blast - riding the halls in a little car, painting in the activity room, playing on the hidden playground, splashing in the garden fountains, watching movies, and being served pudding, cheese puffs, and chocolate milk while lounging in bed.

Hopefully the IV will be inserted and blood work drawn before it gets too late, so Clare can go to bed. We do know that Clare's case starts at 8:30am, she will be on fluids via IV and off oral food and drink around midnight, and she will be intubated (on a breathing tube) while under anesthesia. We do not know how long the procedure will be (1-3 hours possibly), what kind of recovery Clare is facing, or even how exactly the procedure will go. The cardiac anesthesiologist (who will not be part of the actual case tomorrow, but has to consult on the anesthesia involved because Clare is a cardiac patient) explained that many of the decisions regarding the case will be made tomorrow once she is in interventional radiology - like whether they will use coil or glue to occlude the vessels or how they even gain access to the site of the fistula (through an artery in the wrist or in the groin).

These are photos of Clare enjoying her pre-op day (at least someone did!). She loved the $12 balloon Daddy bought for her... until it broke its string outside and floated up into the trees. So if you're ever in Prouty Garden at Children's Hospital, you may find that balloon stuck at the top of the trees.

Tomorrow

We head down to the hospital tomorrow so Clare can do her pre-op and be admitted for surgery on Wednesday. Not technically surgery - it is a catheter embolization, but surgery is easier to say.

As we get ready for our hospital stay, my thoughts tonight are with two of our friends from our WS-community, Avery and her mom Amy. Avery is one month older than Clare and is undergoing surgery tomorrow morning to have a pacemaker put in. We are sending loads of prayers their way tonight and tomorrow for a successful surgery and that the pacemaker does its work and Avery can get back to being that amazing, sweet, equally-crazy-curly-haired girl that she is!

Weird

Despite...

... being up a good part of the night with a teething Violet
... finding the perfect exercise outfit only to have Violet grab my bowl of granola and milk and spill it all over me
... getting four children fed and dressed and out the door in time to drop Clare off on time at morning summer school
... going to the allergist so Jamie can receive his sixth round of weekly allergy shots and keeping the kids entertained while we wait quietly in the doctor's office for 30 minutes to be sure he does not have a reaction
... having Simon fall off the curb and cut his lip open

... I was so proud of myself that I actually made it to the gym this morning. Only to have the guy next to me jogging on his treadmill BACKWARDS. Come on? Really? Backwards? Hey, it was an accomplishment I was just walking forward.

Familiar Rhythm

As we start our countdown of less than a week until we return to Children's Hospital for a three-day stay, it amazes me how easy it is to slip back into the routine. The routine of mental and physical preparedness for our stay. It has been almost two years since Clare was at Children's for a procedure. That alone amazes me! I feel as if we are finally on the path of Clare outgrowing her pulmonary stenosis. Her cardiologist always told us it would happen one day, but I never truly believed her. It's hard to see that as even a possibility when faced with the harsh reality of cath after cath, as we were in Clare's first year. And just when I had a glimmer of hope and Clare was able to go 15 months between caths - BAM - she had two more back-to-back. But it's been 22 months since her last cath, and she does not return to the cardiologist until... I can't remember now which month she has to go back. That is progress!

The downside to not being in the hospital as frequently is that I do not know what to expect out of this hospital stay. Since I always seem to have a nursing baby these last few years (and that nursing baby is NOT Clare anymore!), Shawn is the parent who will stay with Clare overnight. Violet and I will be with them all day Tuesday, then stay in a hotel down the street from the hospital that night, so we can be there almost all day Wednesday as well. As long as the procedure goes well, though, I will head home Wednesday night to be with Jamie and Simon again. Clare was 2 1/2 years old that last time she was admitted at Children's. Now she is over 4 years old, and I have a feeling that there will be much trauma involved. She has reached the age where she knows what's coming (as in nurses with needles), and the anxiety over any procedure (blood draw, IV stick, x-rays, echo) is worse than the actual procedure. I am already preparing myself that the worst moment in terms of Clare meltdowns will be when they insert her initial IV. And I know the worst moment as a mother will be when the doctors take Clare away from us. That's how prepared I am at the moment. And, of course, I know that you are truly never prepared for any of it.

A Date

We finally have a date for Clare's procedure! She will undergo a catheter embolization of her A-V fistula on July 15. This will be done in Interventional Radiology at Children's Hospital. We were able to easily convince the general surgeon of the seriousness of Clare being well-hydrated before undergoing anesthesia. They are going to admit her the night before so she can have fluids via an IV when she is cut off food or drink. Score a HUGE point for us! Her cardiologist wants her to remain overnight after the procedure on the cardiac floor for monitoring, so we are looking at a minimum of a three-day stay at the hospital. I will just be so happy to have this done with!

Photos Photos Photos

I finally got around to updating Jamie's, Clare's, and Simon's yearly photos last week. (I did take Violet for solo photos last month, so she just got in on the group shot this time.) I tried a new photographer at a new studio and LOVED IT! The only photos I were not 100% thrilled with were Simon's and that was because Simon has declared for the past few months that he does not partake in photo sessions. (I have Easter and cousin group shots to prove it!) But his photos are still awesome. Enjoy!

My Kids Rock!

Love It!

The Runaround

I am going to preface this post by warning one and all that I am not in a good mood. It's most definitely been one of those days. After enduring three weeks with a temporary crown on one of my teeth (read - three weeks of eating on only the right side of my mouth), I finally had my permanent crown put on this afternoon. Unfortunately the crown is a bit of a tight fit, resulting in the dentist and the hygienist attempting to yank yards of floss between my molars with little success. I am not sure why they needed to floss my teeth, but I was not in a position to ask questions (mouth wide open, blood dribbling down my chin). So after breaking floss string after floss string, the dentist warned me that my gums "might be a little sore" tonight. One of the understatements of the year.

Today was the day we met with the general surgeon who was going to repair Clare's fistula. (Note the use of the verb WAS. Was was was was was.) We endured over two hours of driving hell down to Boston (we live about an hour from Boston) with four grumpy children we had to wake up and load into the car, enticing them with promises of Dunkin Donuts. Even though we gave ourselves an extra hour of travel time, due to construction traffic (thank you, President Obama) we still arrived 15 minutes late for our appointment. We had been forewarned that the surgeon was "squeezing" us in today since he does not have an appointment opening until the end of July. Wednesday is his day for meetings, so he agreed to meet with us between meetings. Shawn and I were both stressed about being late (my nightmare was that he would be unable to meet with us after that torturous drive to the hospital), but the surgeon was actually very understanding about us being late. Given that he himself made us wait another 30 minutes past when we arrived. This is now the fifth doctor we have met with concerning Clare's fistula.

So here's the recap for those who cannot remember in detail each of my posts:
April 1 - ER doctor first notices the thrum in Clare's wrist.
April 3 - Clare's cardiologist examines Clare and determines that she does indeed have an AV fistula in her left wrist. Decides on a "wait and see" approach.
April 7 - A second cardiologist disagrees with the first cardiologist's approach and decides to send us down to Children's Hospital to meet with a surgeon about undergoing surgery.
April 14 - Meet with the cardiovascular surgeon and Clare undergoes ultrasound scans on her arms. The surgeon decides this would be better addressed by Interventional Radiology and refers us to them.
April 17 - More scans done at Children's and Clare is seen by the interventional radiologist. He says "yes, indeed, I can fix this!" (Okay, I am paraphrasing.) He does not know, however, what method he will use.
April 23 - IR calls to schedule the "procedure," but that is the only information the woman has. We refuse to schedule anything without knowing what Clare is undergoing, so she promises to get back to us.
April 27 - We find out Clare will undergo a catheter embolization of the blood vessels. The IR schedule is full until July and the July schedule is not available yet.
June 11 - After waiting more than a month and hearing nothing, I track down the cardiology nurse. She finds out that IR is unable to fit Clare in before the fall, so cardiology has decided to send Clare back to a surgeon (this time a general surgeon) so she can have this repair done in July.
And back to today....

The surgeon did not say these words, but Shawn and I had the impression that he thought surgery on Clare's wrist was overkill and unnecessary. According to him, it is a procedure best addressed in Interventional Radiology. He was going to pull his magic strings and get her on the July schedule. He literally told us to "wait right here" and he would go talk to IR. Upon his return, he told us that they could fit Clare in on Friday. FRIDAY???!!!?? She would have to be admitted tomorrow for pre-op as everyone agrees that Clare needs overnight hydration via IV before undergoing anesthesia. After all my complaining about this taking too long, Friday was just not an option. Logistically, we cannot coordinate our life with so little notice without going insane. Obviously, in an emergency situation, we would manage somehow. (And, very important in our family dynamics, is that this weekend is Shawn and Jamie's annual Father-Son Camping trip with our church, which would have to be cancelled.) But the surgeon agreed this is a priority, not an emergency. (He really seemed like a great guy, we are just exhausted from the never-ending doctor shuttle.) Soooooo.... it's back to waiting for IR to get back to us with a date. The surgeon pretty much guaranteed it would be in July, so I am praying he really can pull those strings of his.

A ridiculously long post with very little information. I know, I know, quit my whining. We could be headed back to Boston tomorrow and have this thing done with. In this four-year journey, though, I have learned many, many things. There will be things I cannot change. Things I cannot fight against. Things I just have to accept. And one of those things is that there will be times where I have to choose between two sh#$%tty options. This is definitely one of those times.

A Day With Clare

Shawn took Jamie and Simon to his mom's house for the day. Since Clare had school, she stayed home with Violet and I. Having four children, I understandably have to divide my time and attention between them. There is always compromise on what we do during the day, depending on everyone's desires. Other than sleeping and eating when she wants to, Violet is fairly easy-going right now, so I thought it would be a rare day to let Clare do what she wanted all day when she wanted to (within reason!). It was a fun day and a great opportunity to see what Clare enjoyed doing.

Before school, we ate toaster strudels together for breakfast, then blew bubbles. After soaking the living room carpet with bubbles, we watched Mickey Mouse Clubhouse (and I took a quick shower during the second half), got dressed, and did our hair. Clare played with her bucket of ribbons and barrettes for about 15 minutes. This is something she asks to do almost every morning, but I usually say no because it means there are about a hundred tiny items everywhere that I have to pick up after. But today was a day for saying yes. Then it was off to school. While Clare was at school, I did my housekeeping chores, so I wouldn't have to do it after I picked Clare up.

After school, Clare wanted to go to the grocery store to get a cookie and a piece of cheese (two free things they offer at our grocery store), which worked perfectly in my plans since I needed to go anyway. When we came home, Clare wanted to play outside. She moved firewood from the side of the shed to the side of the porch. In doing this, she discovered one log full of slugs. Not my choice of a girls' day activity, but Clare wanted to play with her new friends. (I just watched - there is a limit to how much sliminess I can touch!) By the time she was done, I am not sure if her friends survived the playdate - it was hard to tell what was dirt and what was slug slime (or body parts?) on her hands.

Time for hand-washing, which Clare stretched into a 20-minute activity. (Again something I rarely allow her to do - play in the bathroom sink to her heart's content with the water, soap, and lotion.) Then a late lunch of ham and cheese and bananas. (Since someone was full earlier from the free stuff she ate at the grocery store - cookie, cheese slice, small cup of Whoppers, and two Hershey Kisses. I did put my foot down on the Ben & Jerry's ice cream - enough sweets! Apparently Friday at noon is THE time for free samples.) During lunch, I received my Tastefully Simple order (and felt good for helping support Williams syndrome by ordering too much yummy food!), so Clare and I delved into the box after lunch and examined each item in detail and made towers out of the boxes and bottles. She loves to open packages, but the majority of our UPS drop-offs are defibrillators for Shawn, so she is not allowed in on that fun.

And now we're off to meet Shawn and the boys at the beach for the rest of the afternoon and evening. The rain looks like it may hold off for a little while and the sun has popped out. You may be bored by our run-down of the day, but I loved every minute of it. It was one of those perfect days. I don't regret having four children in the least and the demands on my time and attention, but it was amazing how much I learned about one child when I spend hours with just them (okay, and the baby, but she was a very cooperative third wheel!).

Ummm, Hello? Anybody There?

We're here, just busy!

Way back in.... I cannot remember now, the plan for repairing Clare's aneurysm/fistula was to do so by interventional radiology (IR), using coil or glue to occlude the vessels. Clare and Shawn met with the radiologist, all the ultrasound scans were done, and we were waiting to hear from Children's Hospital on a date when the July schedule was open for booking. Come June 1, we still had not heard from the scheduling department. So I called our awesome cardiology nurse down there, who promised to track down some information for us. True to her word, the nurse called the next day and explained that she did not see Clare anywhere on the procedure schedule, so she would have the secretary from IR to contact us by the end of the week.

IR never called us. I was going to give them one more day (tomorrow), but then the cardiology nurse called today to let us know that IR was going through some personnel changes and were not sure when Clare's procedure would be booked. We were most likely looking at another few months. Before I could get all "mother bear" on them, the nurse said that Clare's cardiologist at Children's Hospital had already decided that was not going to cut it. His fear is that, by the fall, the aneurysm/fistula could grow to a point where doing the repair in IR would not be successful. Then that procedure would have been for nothing, and Clare would be headed into the OR for a second procedure under anesthesia. So we're not going to wait that long and Clare will undergo surgery on her wrist instead in the next few weeks. The bad news is that surgery is riskier. The good news is that it's going to get done soon plus there is a very good possibility that Clare can have her frenulectomy done at the same time, since she will be in an OR under anesthesia. That would be awesome! We were already planning on setting up her frenulectomy for August once her aneurysm was fixed. But if Clare can have them done at the same time that would be so much easier and less risky for her.

Since Clare will most likely have the two surgeries done together, she is going to be operated on by the senior general surgeon at Children's Hospital instead of a vascular surgeon. We will be meeting with the new doctor in a couple weeks so he can meet Clare, see the site, and go over the procedure with us. Then hopefully we can get on the schedule for July. Other than IR (who Shawn did not like the doctor there anyway, so this is probably a good thing!), everyone else seems to want to get this repaired as soon as they can now.

Quarterly Report

It's been a while since I just chatted on how life is going in our land. With Simon's 2nd birthday over, we are through with the run of birthdays in our family until 2010. Thank goodness! I am quite done with entertaining my family and friends (just kidding... ha ha ha!).

Jamie graduated from kindergarten on Friday. He did his assessment testing for first grade at his new school and sailed through it (no surprise there). Overnight it seems, Jamie is reading. He reads everything he can get his hands on (even things I wish he wouldn't know how to read - such as bumper stickers on other people's cars). He loves to read to Clare and Simon, which is so sweet to see. Shawn and I are both huge readers, so I hope Jamie always loves to read. He gets to go in one day next week to spend the day with his new class (since they are not done with kindergarten yet), and he is so excited to do this! I think it's adorable that he is thrilled about packing a lunch and wearing a uniform. I won't hold my breath that he will be thrilled about this in a couple years! We are sad to say good-bye to St. Mark's Nursery School. It has been a fabulous experience for Jamie and us - a great introduction to the world of education. I am already looking forward to starting Simon there in a couple years. Jamie and Shawn are in the middle of their t-ball season (Shawn is coaching again). This will be Jamie's last year in t-ball before he moves up to the instructional baseball league. I am enjoying being a Saturday morning baseball mom again, which basically means I run after Clare and Simon as they play near the field and only get to actually watch the game whenever Jamie is at bat. It's been a tough couple of weeks medically for Jamie with two tooth extractions and starting allergy shots. Let's get all this out of the way so we can have a good summer!

Clare, being in the public school system, still has a month of school left. We continue to wait for the call from Children's Hospital about scheduling her wrist procedure. The fistula/aneurysm thrums all the time now, and she frequently complains that it hurts. Hopefully it will only be two more months max before it is repaired. Her speech therapist is still "on us" about having her tongue released, so as soon as we hear from Children's, we are going to look into getting that procedure scheduled for the third time! I know the therapist feels the tongue-tie is really impeding Clare's articulation, so she is currently concentrating more on sentence structure, grammar, and cognitive speech (such as recognizing shapes, colors, numbers - Clare can now count to ten and identifies a few colors). I am so proud of how hard Clare works with Judy. I can really see that her attention span and focus are increasing in length. She can make it through the entire one-hour speech therapy session and continue pretty much on task. Of course, we usually have a meltdown at the end from exhaustion! Clare has been asking when she gets to play t-ball. Our city has a league for kids with special needs, which she can begin when she is five. She has sat through many t-ball and soccer games (and is Jamie's number one cheerleader), so we are definitely going to get her involved in something next year. We also have received notices from her preschool about Special Olympics, so I am going to look into that as well. We have hit a "sad" phase where Simon is starting to pass Clare developmentally (sad for me, at least). Clare and Simon are unaware of this, but Jamie has noticed and is starting to question why Clare, at 4, does not know and cannot do many of the things that 2-year old Simon can. I am still formulating my answers to these questions.

Simon is a whirlwind these days! A very, very busy 2-year old. He and Clare have fallen in love with playing outside, and the two of them beg to go out all the time. They enjoy playing on our swingset or just exploring the great outdoors. I find them collecting buckets of rocks to throw into the sewer grates or rearranging Daddy's box of firewood or picking flowers (aka dandelions). Simon has started to develop cognitively by leaps and bounds. Now that Simon has mastered numbers (he can count to twenty and recognizes all his numbers) and letters (seriously, the kid is scary smart), he is working on shapes and colors. Actually, he has shapes fairly down pat as well now. It is such a dichotomy to watch Clare versus Simon develop. It amazes me how easy everything seems to be for Simon. I can read a book to him just a couple of times, and he has it memorized. I catch him multiple times a day sitting on the floor near the bookshelf or on the couch looking at books. He loves to read and be read to (a boy after my own heart!).

Violet turned four months today. In the last couple of weeks, her personality has blossomed. She can roll over, smiles all the time, and we have started hearing little giggles out of her. She always has a bright smile for us. Violet loves to just sit on my lap and watch the entertainment that having two older brothers and an older sister provide. She loves to play with her activity gym, and I started putting her in the exersaucer, which she loves as well. All kids seem to grow so fast, but I find that the more I have, the faster they seem to grow! We recently moved Violet out of our room and into the nursery. That event is always bittersweet because it means I don't have a newborn anymore, but it is nice to have my bedroom back!

You Choose...

which moment of my day was the worst:

1 - Watching Jamie have a tooth extracted and making an appointment for next Friday to do it again.

2 - Having the road crew who is re-paving my road yell at me, "What are you doing, lady???!!!??" as I am driving down my road (on the side that was completed yesterday, mind you) to get into my driveway. (If you're curious, I just yelled back, "What do you want me to do? I live here!!!!")

3 - Facing said road crew four more times as I do my daily school drop-offs and pick-ups.

4 - Comforting Clare during a hysterical meltdown as we are eating ice cream at Mack's Apples because two fire trucks, an ambulance, the fire chief, and two police cars all with sirens blaring scream by us.

5 - Dealing with the woman who said to me, "You have too many children." Right in front of my four beautiful children!

6 - Having the double-stroller tip over backwards with unbuckled Violet in it.

(Yes, I am having a let's-complain-about-everything moment.)

The Things That Make Me Cry

I doubt this will make you cry, too, but I started crying when I read in a Williams syndrome publication that a new collaborative study is being done between scientists in the fields of cellular biology, cardiovascular gene therapy, and pharmacology. As the article states (because I could never paraphrase this properly!): "The study will examine possibilities for 'turning on' the elastin gene, modulating genes in the WS region on chromosome 7, and investigate rapamycin therapy as a way to slow down muscle overgrowth responsible for the stenosis in blood vessels."

People with Williams syndrome are missing a portion of genes on chromosome 7, one of these being the elastin gene. Elastin is used in the construction of blood vessels. Since people with WS do not produce elastin, this is why almost all of them have heart defects (ranging from mild to severe). I know this won't "fix" Williams syndrome, and it may not even have an impact on Clare. But to think that future generations of individuals with Williams syndrome may not have to face the severe cardiovascular issues that we have gone through with Clare overwhelms me with emotion.

Belated Mother's day

S~ Okay, I know what all of you are thinking...Where was this post ON Mother's day? Well I am not going to make any excuses and I will follow the advice of a lawyer and keep my mouth shut. I did however want to post something on just how AWESOME my wife truly is. Anyone who has walked in her shoes (and my Mom and I have done this) knows that the schedule and routine my wife keeps is not an easy one. Shuttling the kids to and from school, scheduling all the doctor's appointments; the feeding, clothing, and keeping after four very active kids. She also makes sure that our house is always warm and inviting. And at the end of the day, she still feels guilty if Violet did not get enough mommy snuggle time. I remember hearing on the radio years ago that if they calculated up the income that a stay at home Mom should make for all the duties they do it would be an annual salary of around 250K, of course the IRS would take about 50% of that, but it is perfectly clear that this stay at home Mom clearly out earns me. However her pay comes in the form of dirty kisses from little boys who have been playing outside, hugs and smiles, from Dinosaur romps throughout the house, from stressed out daddies who have been struggling with a 2 year stop shipment,and lastly from constant complaints on the dinner she slaved over during the kids most pleasant of hours (we term it the witching hour).

So she gets one day all to herself to be pampered and praised and gushed over. However, this year Teresa sacrificed that so I could spend a weekend with my Dad fishing up North. We all met up at a lake in Manchester so the kids could go fishing and we had a picnic with my parents. I knew it was not the perfect day for her, but she smiled and had a good time anyway, and for that I love her. I love her and admire her for all that she does. Happy Belated Mother's Day Post to the most wonderful Mother and Wife!

Sometimes...

I wish my child was normal. That when I turn around in the car to find her smearing herself and the car seat with cream cheese, and I speak to her sternly, she does not launch into a hysterical crying fit. That I didn't then feel horribly guilty about disciplining her and causing her to be so upset. That I didn't worry about what this was doing to her blood pressure, her heart, her aneurysm. That I didn't have to wonder if she truly didn't understand why I was upset and that I was too harsh. That I could just parent her as I would any other 4-year old. That she didn't spend the remainder of the car ride crying in the back, while I did the same up front.

Happy Birthday, Simon!

Happy Birthday to our number-counting, dinosaur-roaring, elephant-stomping, hug-giving, newly-turned 2-year old boy. We love you so much, Simon Joe!

Family

Clare's preschool held a fundraiser at Chuck E. Cheese last week. We were a little apprehensive about doing Chuck E. Cheese with four kids (I haven't been there since Clare was a baby), but it was for a good cause. Everyone had a blast (even Violet enjoyed watching the chaos around her from the safety of the Baby Bjorn). Clare was a little overwhelmed at first by the noise and craziness, but once she scoped out the situation, she enjoyed herself playing games with Shawn and Jamie. I was on Simon patrol and spent my time either watching him play on the toddler climbing gym and slide or putting tokens in the Teletubbies ride. What fun! Clare's teacher snapped some photos and sent us these two great ones. The top photo is our family eating dinner (yes, even Violet is eating!) and the bottom photo is Clare with her teachers, Miss Karin and Miss Pam. Clare loves her teachers and loves school so much. Her teachers are awesome and work so well with the kids. Their love of their job and the kids they teach shine through them. We have been so blessed with Clare's placement in the preschool.

All My Updates

It's been a busy day in our household! And it's 95 degrees today in New Hampshire. Insane! The kids and I played outside for about 20 minutes this morning, then had to call it quits. Way too hot!

My god daughter Faith has been transferred to the NICU at a children's hospital, while her mother remains in a hospital an hour away. We continue to say lots and lots of prayers for this family, who are so dear to us. Having a child with medical needs ourself, we know how tense and stressful this situation is, especially with Faith being only hours old. I cannot imagine not being with my baby right after birth. At least I did not have to be parted from Clare, so my heart goes out to them.

We received two calls from Children's Hospital today about Clare. The first was from the scheduler again. The radiologist's schedule is full until July (and we were told he was "very open" - is that what they mean by that??), so once the July schedule is released, they will call us back to schedule it. She just did not want us waiting around wondering why no one was calling. Clare is on a waiting list if there is a cancellation, though. The second call was from the cardiac nurse who answered three questions for us - 1) Clare will be admitted overnight. 2) Clare will be under general anesthesia. And 3) The radiologist will determine on that day whether he wants to use a coil or glue. If he uses a coil, Clare will have another piece of metal in her for the rest of her life. So now we just wait some more. I love this game!

And finally, Jamie is almost done with his course of steroids and his allergy symptoms have not magically disappeared like they were supposed to. So now we are actually at the point of immunotherapy. I am now doing some more waiting for the nurse who arranges the shots to call me to go over everything. We have to go over Jamie's history again to determine what serum she is to order. Plus Jamie needs to not be actively symptomatic (good luck with that!) before he can start receiving the allergy shots, so it may be a good six weeks or so before that happens (which is usually how long it takes Jamie to clear up). And even more waiting...

Prayers for Faith

Our newest god daughter, Faith Anne, was born this morning. However, she is having trouble breathing and is currently in an oxygen tent. She also may have broken her arm during delivery. We have been eagerly awaiting Faith's arrival, as has her mom and dad and siblings. Please send some prayers Faith's way!

Getting Closer

On the drive home today, I checked our voicemail and we still had not heard back from Children's. With hours in the car ahead of us, I called the nurse who worked with us a couple weeks ago. I was surprised (and thrilled) that I actually reached her and not her voicemail. She instantly knew who I was and, once I explained what I was looking for, she instantly pulled up Clare's information. So we got some answers today!

The interventional radiologist is going to perform a catheter embolization of the blood vessels. The nurse did not know whether the radiologist was going to use a coil (made of either stainless steel or platinum) or liquid glue to repair the vessels, so she is going to get back to us tomorrow with that information. Clare will have a pre-op day the day before the procedure and may have to stay overnight following the embolization to be sure the procedure worked. A cardiac anesthesiologist (versus a general pediatric anesthesiologist) will be on board, given Clare's medical history. We will meet with the anesthesiologist during pre-op, so will find out then exactly what method of anesthesia will be used. The nurse admitted that she did not have all the answers for us as this is a rare case (always lovely to hear those words!). She did say that she believed the anesthesia did not need to be as "deep" as if Clare was undergoing surgery, so hopefully Clare will not need to be intubated for anesthesia. The risks of a catheter embolization are less than those of doing an open surgery on the vessels, so we pray that the radiologist can indeed make this a one-shot deal.

Now we wait for the scheduler to call again, so we can actually get this procedure over with!

This Time I Actually Scream

Clare had a rough night last night. We are still in the hotel in Philadelphia, and she has had a LONG three days. She has had tons of fun, but everyone has been going to bed late and waking up early, so every night, Clare misses out on about 3-4 hours of sleep. She stopped napping over a year ago so she just goes, goes, goes all day and is like a little wilted, droopy flower by the end of the day. (She was the only child who did not sleep at all on the 7-hour car ride down here - even Jamie took a nap.)

The past two days have been in the 90's. We were not prepared for this heat wave and are not at home. Yesterday was my niece's Baptism. During church, Clare's face was flushed and you could tell she was feeling the heat. She has never been good in extremely hot or cold weather. Her body has a hard time adapting to temperatures, so if she becomes overheated, it is very difficult for her to cool down. The heat can also dehydrate Clare quickly, so we always have to be extra-diligent at getting fluids into Clare whenever it is hot. Thankfully, my sister Christina had filled up the kids' sippy cups with ice water prior to going to church, so Clare was able to drink that. Once we arrived at the air-conditioned restaurant for lunch after the Baptism, Clare cooled off and quickly regained her energy and charmed everyone there.

Once we got back to the hotel, Shawn took Jamie and Clare swimming (they had been begging all day), so come bedtime, Clare was exhausted. She also does not eat well when she is out of her home environment. Despite all our coaxing, I think she only ate two slices of cheese and some M&M's all day. Tired and hungry, Clare refused to even eat the offered pop tart at bedtime (one of the only things we had to eat in our hotel room at 9pm that Clare could actually manage) and melted down instead. She finally feel asleep exhausted, but woke up continuously in the night crying. Part of the time, she was not even awake, so Shawn and I alternately rubbed her back until she fell asleep again. One time, she was awake and that is when she told me that her left arm hurt. She had her pajama sleeve pushed up past her elbow on that arm (she does not like her sleeves pushed up, so this is unusual behavior for her) and kept complaining that it hurt. I felt her fistula and it was thrumming rapidly. I did not know what else to do, so I gave her some Tylenol and sat with her for a long time, stroking her head, until she eventually settled down and fell asleep again. I think that was around 2am. Now it is almost 7am, and she is still sleeping.

After that initial phone call on Thursday to schedule Clare's procedure, we have not heard back from Children's Hospital yet with more information on what the "procedure" entails. We are journeying home today, so I am planning on calling the nurse tomorrow who we dealt with while we were down there a couple weeks ago and demand some answers!

I Scream But It Only Comes Out As A Yawn

Children's Hospital called today to schedule Clare's "procedure." However, the woman on the phone had no details beyond that. She had no idea what procedure, what anesthesia process, anything about what the doctors had decided. Shawn explained that we were not scheduling any procedure until we had some information about what exactly was going to happen to Clare. So we're still just waiting some more.

Jamie's allergies, true to form, have now kicked in full-force. He is back to red, swollen, itchy eyes, congestion, cough, headaches, and a general grumpy attitude. The allergist put him on a course of steroids, which Jamie started yesterday. No improvement yet, but the steroids are supposed to wipe out all his allergy symptoms in a day or two.

We are packing up to head down to Philadelphia for a few days for my niece's Baptism. Jamie and Clare are on school break next week, and we're all looking forward to a week free of driving and full of playdates!

Still Need A Plan

Clare saw the interventional radiologist today. He definitely ruled out compression as a means of closing the fistula given the position of the fistula and the number of vessels involved. So that leaves us either surgery by the cardiovascular surgeon or the radiologist can insert a coil to close the opening (in a method similar to the cath procedures). Both require general anesthesia, so the anesthesia risks are the same regardless of which method Clare undergoes. The radiologist is going to conference with the surgeon and anesthesiologist again and determine what is the best plan for Clare. We want to make this a one-shot deal, so Clare does not have to undergo anesthesia twice. Today's visit does not tell us anything new, so we're still just waiting to find out what the plan is and when it is going to happen.

I Love My Kids

Today's words of wisdom:

Simon (as I am changing his diaper): "See you later, poop! Have fun!"

Jamie (after we get in the car from going to the gym): "Whew! I am so glad we went to the gym today. I needed the exercise." (Note - he plays in the play room while I exercise.)

I Need Help

Am I the only person in the world who cleans the inside of her dishwasher?

Easter Photos

My absolute favorite photo from Easter - the pure joy on Clare's face as she goofs off with her Uncle Brian is priceless.

Violet's first Easter - she was adorable in her dress!

Simon's favorite part of Easter dinner was dessert. Grand Dad helps him eat some (not that Simon needs any help in that area!).

Jamie was the king egg hunter again.

Clare finds an egg hidden in the daffodil. (Yes, folks, we only had one daffodil bloom this year.)

This was Simon's first "real" year egg hunting, and he loved it. "More eggs? More eggs?"

Jamie displays his find.

Simon wanted no part of taking Easter photos this year. At least Jamie, Clare, and Violet smiled!

Violet checking out her first Easter basket.

Another Appointment

The next step is Clare has another appointment at Children's Hospital with the interventional radiologist on Friday. He wants to see Clare in person and do another ultrasound of her wrist before proceeding any further with a treatment plan.

As if that isn't enough, Jamie had his six-month dental cleaning this morning (always fun with the gang!). Six months ago, his teeth were beautiful. Now they are riddled with cavities. The hygienist was plying me with questions about Jamie's diet and brushing habits. Other than upping a bunch of his meds, nothing has changed, so I don't know why he suddenly has all these cavities. Leave me alone, lady - I do the best I can! Then she started losing her patience with Jamie because she wanted to get an x-ray and the film was too big for his mouth. The poor kid was trying to follow her directions, but we all know how hard it is to do dental x-rays. How can you expect a 6-year old to not gag when you stuff this huge thing in his mouth that is designed for an adult? I personally like my dentist, but the office is not equipped to handle children. (One of the reasons why we switched Clare to a pediatric dentist, but Jamie had not had an issue with our family dentist. Yet.) So I have an appointment scheduled for him with the pediatric dentist to do an exam. Jamie will most likely need several restorations done under sedation in the near future. Sigh.

And It Just Goes On And On

Now that we are home, and I am lounging in bed with four kids asleep, a piece of chocolate cream pie, and American Idol (even though I am not fond of Quentin Tarentino), the day does not seem quite as long, but it was still a pretty long day.

We spent five hours at Children's Hospital this afternoon for Clare's evaluation of her wrist. She had an ultrasound first. After the tech did her stuff, she wanted to call in a radiologist to take a look at the results. The radiologist informed us that there was indeed an aneurysm in Clare's left wrist. After about an hour and a half of these two women intently studying the ultrasound of Clare's arm, they determined that Clare had an aneurysm and a fistula. They were having some trouble figuring out which veins were involved, but eventually they obtained all the data they needed and sent us on our way.

Next stop was cardiology where we waited for the surgeon to see us. He didn't do anything hands-on with Clare other then feel the site. He explained to us that it was actually rare now to need to do surgery on a fistula. An interventional radiologist (never heard of this occupation before) could use ultrasound and apply compression to the site of the fistula. With the right amount of compression, the fistula would clot on its own and repair itself. Now the fistula would most likely require 1-2 hours of compression for that to happen, so Clare would have to be sedated for the procedure. If that did not work, we would have a plan B, which may be surgery. We asked the surgeon what the fistula meant heart-wise. He said there was definitely increased turbulent blood flow in Clare's left arm as opposed to her right. Arteries are bigger than veins and carry the blood away from the heart. Since there is an opening between the artery and vein, the blood flow is abnormal, which makes Clare's heart work harder. Right now, the fistula is relatively small, but that he wanted this taken care of within the next couple weeks because if the fistula becomes bigger, then Clare's heart will be working that much harder. The surgeon wanted the interventional radiologist to review the ultrasound findings. We met with the nurse practitioner of the cardiologist who oversees Clare's care at Children's. She told us to sit tight in the waiting room while the radiologist looked over the ultrasound, in case they wanted another peek at Clare before we left.

So after more time waiting and waiting, the nurse came back and informed us that one of the interventional radiologists reviewed the ultrasound. He did not feel that the fistula could be repaired by external compression. He wanted the lead interventional radiologist to take a look at the ultrasound, but that doctor was in the middle of a case. They also want a team of doctors on board because the fistula involves one artery and two veins, the position of the fistula in her wrist, and the fact that Clare has Williams syndrome and all the risks associated with that. So the radiologist, cardiologist, anesthesiologist, and the vascular surgeon will discuss the options and what is best for Clare. As the nurse stated to us, this is a unique case. The nurse herself was unsure of what all the options were (surgery versus something else the interventional radiologist could do). Since there were not going to be answers today, she was able to send us on our way. She stated that there should be a plan to go over with us by the end of the week, and that Clare would most likely have something done within the next few weeks. So we have some answers, but still little clue as to what is happening next.

The surgeon did warn us to be mindful of Clare's wrist and that she could do some harm if she fell on that spot. The surgeon said that the area could be painful to Clare because the blood vessels were distended there and there is that thrill you can feel beneath the skin. Shawn is going to have a chat with Clare's teacher and school nurse on Thursday about what's going on (we have been keeping Clare's teacher updated). On a normal day, Clare falls multiple times a day (like she had just fallen at school right before we picked her up this morning and one of her teeth had cut her bottom lip), so we're going to be as extra careful with Clare as we can. (Hard to do with a four-year old sometimes!)

*** After seeing Kerry's comment, I had to add this because, as much as I am whining about all the waiting we have to do with Clare's care, we absolutely are in good hands at Children's. As frustrating as it is to have to wait some more, I am always so thankful to have these amazing doctors so close by who take Williams syndrome seriously and the intricacies it imposes. Clare is not just a normal kid and nothing is routine when it comes to her care.

(And, off subject, the one piece of good news we received today was that all of Violet's thyroid tests came back normal.)

Happy Easter!

From all the little bunnies at our house! Happy Easter!

(Taking photos with four children and having everyone looking at the camera.... yeah, pretty much impossible!)

And Yet More...

I received a call from the cardiovascular surgeon's office today, scheduling an appointment for next week. The cardio from our office here was true to his word and made the call right away to set up an appointment with the surgeon. (Usually things don't happen this fast in the world of medicine, if you've ever been in our shoes!)

The surgeon wants to evaluate Clare next week, so we have an appointment scheduled for Tuesday afternoon at Children's Hospital. She will have an ultrasound done on her wrist and we will meet with the surgeon. That's all the news for now!

Continuation

Over the weekend, Clare's right arm behaved itself, but she frequently complained that her left hand hurt. When we asked where it hurt, she pointed right at the location of her fistula in her left wrist. She was still complaining of the pain on Monday, so I called her cardiologist's office. When the office finally called me back on Tuesday, it was not Clare's cardio but another one from the practice (one we have never met, but spoken with a few times on the phone over the past four years). He apologized for the delay, explaining that Clare's doctor was on vacation, and he had ordered copies of the ER reports to bring him completely up to speed before he talked to us. After quizzing me on various things regarding Clare's fistula, he asked us to come into the office right away so he could check it out himself. He gave me the impression that he did not think Clare's doctor had fully evaluated the fistula and that it needed to be taken care of sooner rather than later (which was what Clare's doctor had suggested - a wait and see approach). Of course, this always happens at 4:30pm - the kids are all hungry and whiny, dinner is in the oven, and Shawn is not home yet. So dinner was turned off, all four children nicely cooperated with getting their shoes and jackets on and back in the car, and Shawn was only 20 minutes away. (We ended up arriving at the doctor's office almost at the same time since herding four kids in and out of the car takes a little time.)

After examining Clare, asking lots of questions, and listening intently to Clare's wrist, the doctor stated that he believes an aneurysm has formed at the site. (The ultrasound at the hospital did not ultrasound past Clare's lower arm, so there is no ultrasound of the site itself.) This is not life-threatening, but definitely should be dealt with. It has only been a week since the vessel problem was diagnosed, and it has progressed fairly quickly over that week. He thanked us for coming right in, explaining that he did not want to start referring Clare out to other doctors when he himself had never even met her. He was going to put in a call to Clare's cardiac surgeon down at Children's and expects we will at least speak with the surgeon's office before the end of the week. Clare will probably have an appointment with the surgeon so he can take a look at the problem himself and discuss where to go from here. The cardiologist believes it would be a fairly simple surgery if Clare has a fistula. I think it would be more complex if it is an aneurysm. Since the vessels are near the skin, the surgeon can most likely make an incision in Clare's wrist and go from there. We now have lots of new questions about what's going on!

Start Walking!

We are participating in the American Heart Walk this year. Eons ago, before I had children and had a job outside the home, Shawn and I participated in the American Heart Walk every year with my company. Little did I know then that, in the future, I would have a personal interest in this event. Over the last few years, we have chosen to walk for Easter Seals since they were providing such outstanding service to Clare, at no expense to us. Since Clare no longer receives any services through Easter Seals, we have chosen to do the Heart Walk instead this year. We have a web page on the Heart Walk website if you are interested in making a donation in honor of Clare. Thank you!

Donate in Honor of Clare Bear

Curiosity

After a long couple of days focused almost solely on Clare and her health, it's nice to muse about the perks of being a mother. One of the things I love about Jamie's age is his curiosity. I don't know if all 6-year olds are like this, but there are days when Jamie's curiosity is insatiable. The other day, we had a deep discussion on the way to school after Jamie asked whether Jesus was around during the time of the dinosaurs. This naturally led into an explanation of the Holy Trinity (awkward and inadequate on my part, inquisitive on his). As in, no, Jesus was not around physically on earth when the dinosaurs were around, but, yes, God was in existence; and how God and Jesus are the same, yet not the same. (Okay, where are Jamie's godparents when you need them? I really need to find a book about explaining the mysteries of our faith to a 6-year old.) I admit I pulled out the old analogy of relating the Trinity to a shamrock - which Jamie grasped fairly well since he had just had his St. Patrick's Day party at school a couple weeks ago.

When he's not asking question after question about the world around him, Jamie also loves his new skills of reading and writing. He carries a notebook and pen around with him to copy anything and everything he sees. He especially loves to do this in the car, so when we get home and I read his notebook, he has quite an eclectic collection of phrases! In school this past month, his class has been learning about outer space. All of their reading, writing, and math activities are focused on the solar system. Every day, we learn new facts about the planets as Jamie learns them. And his memory is incredible. Today Jamie enlightened me about the meaning of the word "year" (as in how many days it takes a planet to complete its orbit around the sun) and which planets had long years and which had short years. He knows how many moons each planet has, what the air is like on Mars, and how no one has ever seen the side of Mercury that faces the sun because it is too hot for anything to go near to take a photo. It is so refreshing to see life through his eyes and what amazement and wonder he has for the world we live in and how it works. I just wish this lovely planet was a little kinder to him sometimes!

Jamie has been fighting more allergy attacks since about January. We finally had our second long-awaited appointment with the allergist at the beginning of the week. I filled the doctor in on how we had taken Jamie off the Zyrtec in November (per the doctor's orders) and tried getting through the winter on just two of his allergy meds. Come January, however, Jamie's allergies kicked back in, so we restarted the Zyrtec. Since where we live was covered in snow and ice during this time, the likely source of Jamie's allergy symptoms were the dust and cats in our house. (And although my housekeeping would not necessarily win any awards, I do dust, vacuum, and clean frequently!) The allergist is a little concerned that Jamie could not survive the winter without being on all his allergy medications since that does not bode well for what will happen when the pollen season is in full force (Jamie being most allergic to trees and grass). Ideally, winter is a time to skate by on minimal meds, so the arsenal can be built up come spring and ready for the attack. Unfortunately, Jamie headed in the opposite direction. We again discussed allergy shots, but the doctor believes we are still at an acceptable level of medication without shots being necessary. The doctor did question me on whether I was okay with the number of doses I had to dispense each day. I did not even crack a smile when I assured him I was on board - after all, what's six more doses when you're already doling out eight to Clare? Sometimes I feel like I'm my own little pharmacy. My kitchen counter has quite a prominent space carved out for our morning meds.

So the plan with Jamie's allergies is to bump up his Zyrtec, add a fourth medication, and use eye drops as well if needed. Sort of like getting ready for battle by storing up extra ammunition. When the pollen season hits, if Jamie's symptoms become out-of-control like they did last spring, the allergist will put Jamie on a short run of steroids to wipe out the allergies. However, if Jamie ends up needing more than 2-3 courses of steroids over the next year, then allergy shots would be the next step. I have been warned that, due to the severity of his allergies at such a young age, allergy shots are most likely sometime in the near future. As long as Jamie is reasonably symptom-free, I want to see how long we can hold that off.

48 Hours

It's been a long two days on pins and needles.

Clare went in for an echo and visit with her cardiologist today. Her echo looked great. There is no change in the narrowing in her pulmonary arteries and aorta. Everything looks beautiful, and she does not need another echo for six more months. We may even be at a point where Clare is outgrowing her stenoses. That is great news but it still does not explain her right arm issues.

So we are at a number of theories right now:

1) Clare has developed an arteriovenous fistula in her left wrist (this is a fact not a theory!). An AV fistula is an abnormal passageway between an artery and a vein. Normally, your blood flows from arteries through capillaries and back to your heart in veins. When an AV fistula is present, blood flows directly from an artery into a vein, bypassing the capillaries. If the volume of diverted blood flow is large, tissues downstream receive less blood supply. Clare most likely developed the fistula as a result of her open heart surgery 3 1/2 years ago, when an arterial line was placed directly into the artery in her left wrist to take blood pressure measurements. Now the fistula is at a point where you can actually feel it vibrating when you hold Clare's left wrist (it is very strange to feel - like a little vibrator was inserted under her skin). One of the theories is that the fistula is "stealing" blood and so there is a decreased blood flow to Clare's other arm - the right arm which was the arm in question the last two nights. Clare's cardio took blood pressure measurements of both Clare's arms and of the fistula itself. All the measurements were consistent (about 99/60 range), so the fistula should not be a problem right now. If it becomes an issue in the future, then it can be surgically repaired.

2) Clare is experiencing Raynaud's Phenomenon (this is a theory). Skin discoloration and decreased temperature occurs because an abnormal spasm of the blood vessels causes a diminished blood supply to the local tissues. It often occurs after being exposed to cold - both nights it happened immediately after Clare returned inside from playing outside in the semi-chilly evening air. This phenomenon can be present by itself or be a symptom of an autoimmune disease. Clare has congenital hypothyroidism, which is considered an autoimmune disorder. There are other disorders as well that can present with this symptom, but there would be more tests and doctor's visits in the future to see what's what. Also, Clare's blood pressure medication, Propranalol, can cause these blood vessel spasms. Our plan for now is to keep an eye on it, and bring it up with her pediatrician at her annual physical in a couple weeks. If it is a symptom of another disorder, then the pediatrician is our route right now, not the cardiologist.

3) I am ecstatic to say that Theory #3, a blood clot, has now been officially ruled out. Clare's cardio looked at her blood draw numbers from Wednesday night at the ER and realized that the ER doc was comparing the numbers to those of an adult patient (gotta love the local ER!). So she wanted to compare them against a pediatric patient (duh) and re-run the tests today. After Clare's traumatic blood draw experience at the ER, she flipped out the minute we walked into the lab at the doctor's office this afternoon. I loathe putting Clare through that (especially since the whole time, she is sitting on my lap, screaming "Help me, Mama"), but her numbers today were normal. So no clots!

The only really bad news today was that Clare's cardio is leaving the practice and moving further south. We have always had a great relationship with Clare's doctor, and I feel like she truly knows Clare inside and out. We did meet the cardiologist who will be taking charge of Clare's care. He is an interventional cardiologist at Children's Hospital at Dartmouth, which means he can actually perform cath procedures. So she is still in good hands, but I am not eager to have to build a new relationship with one of the most important people in Clare's life (health-wise, that is).

A huge weight has been lifted off my chest that Clare's heart is not the cause of what's been going on the last couple of days. We still do not have THE answer, but at least we have some direction now. And peace of mind.

It Must Be April

Yesterday was April 1, and, true to form, Clare landed in the ER.

It started in the morning when she did not want to eat breakfast. She did not have school yesterday (as it is her one day off), but had a dentist appointment in the morning. I gave her the dose of amoxicillin she needs to take prior to any dental work. Antibiotics always give Clare diarrhea, so I knew that was coming. She did great during the appointment (with minimal crying), but refused to eat lunch as well (and it was a treat - Burger King lunch complete with chocolate milk, her favorite). I chalked it up to the fact that maybe she had an upset stomach from the antibiotics, but could not coax her to eat anything. We were at a Burger King with a play place and, towards the end, she was just laying on one of the mats inside the tunnel structure, not moving. Again, I thought maybe she was just tired from a morning of running around, so we dropped Jamie off at school and headed home. Since Simon and Violet were napping, Clare and I laid on my bed and rested as well (as I've said before, the girl refuses to nap!). She kept saying she didn't feel good, and I asked her where her boo boo was. She said her hands hurt, which I thought was funny. She still would not eat anything, but Clare has been up and down with the virus we've all had plus I still thought her stomach might be upset from the morning's medication (excusing the bad mommy who didn't listen to her daughter!). And I am never sure when we ask Clare where it hurts, if she truly is capable of telling us yet.

Come dinner time, Clare still was not eating and complained again that her hand hurt. When I felt her right hand, it was ice cold. The kids had been playing outside prior to dinner (and it was a tad chilly), so I felt her left hand. The left hand and arm were nice and warm. I felt Clare's right hand and arm again, and they were freezing. That's when we started to get worried. We called her cardiologist who did not like the sound of that at all. She instructed us to take Clare to the local ER immediately and tell them to doppler her arm for a blood clot. Of course, that totally freaked us out! Shawn took Clare right over, and I had the fun job of waiting at home with the other kids. I truly don't know what is worse - being the parent uncomfortable (and without dinner) at the ER for hours on end with a sick child or the one waiting at home, comfortable but with no clue what was going on and keeping a brave face on for the sake of the other children.

Shawn and Clare were at the ER past midnight. The ER staff also noted the drastic temperature difference between Clare's arms. The nurse was able to pinpoint in Clare's right arm where the temperature changed. The ultrasound, however, did not find any sign of a clot in her arms, so Clare's cardiologist wanted the ER doc to take blood pressure measurements on all four extremities and run some blood work. The blood pressure measurements were in a good range for Clare, so that was good. However, the blood work did show abnormalities in Clare's blood clotting factors. Even though they found no actual clot on the doppler, something is not right in Clare's body right now. Clare's cardiologist talked to a vascular surgeon about Clare's blood work results. The ER had already run all the diagnostic tests that the vascular surgeon recommended. The cardio wanted to discuss Clare more with her colleagues at Children's Hospital in Lebanon (the children's hospital affiliated with our cardiology practice), so she discharged Clare for the night with the order to call her office this morning.

After a very late night, Clare woke up for about an hour this morning, then went back to sleep for a while. I have spoken with Clare's cardiologist this morning. She is not comfortable with the results of Clare's bloodwork, especially given that, with Williams syndrome, clots can develop easily. Clare's arm and hand feel better this morning, but she still does not feel good and is very tired. Clare also still is not eating or drinking much, but we really have to push the fluids since hydration is extremely important in keeping Clare's blood vessels in good shape. So now we're in the waiting game. Clare's cardio put a call into the cardiologist at Boston Children's Hospital, and we are waiting to hear what the game plan is. I hate the waiting part of this. I just want some answers. It drives me up the wall to sit here outwardly calm, while inside I am so agitated. Shawn is upstairs with Clare and Violet (Clare is awake again, but complaining of being tired), and I am downstairs with Jamie and Simon (who are coloring while I type).

I think the only certain thing today, though, is that it looks like Clare's tongue surgery may be cancelled once again.