Friday, February 25, 2011

Quick Update

Clare is home from the hospital. The surgery went well, and the dentists were able to save her tooth. She had a bunch of work done and is doing well mouth-wise. However, she is having a reaction to the anesthesia (which happened after her last surgery in July 2009 as well). Nothing major, just uncomfortable. I was fortunate to get an appointment with the allergist yesterday evening, so now we are going to work on finding out what exactly sets Clare off. Since she is due for a cath within the next year, the cardiologists want us to get to the bottom of this before she goes under anesthesia again.

One more procedure behind us. Thank you for all the prayers!

Tuesday, February 22, 2011

Flying In, Driving Out

I am waiting for Shawn and Clare to return home from their trip to Louisville, Kentucky to see Dr. Mervis. Being that they have a layover in Philadelphia, their flight (of course) is delayed, so I am still waiting for their arrival at home! (No insult to my peeps in Philly, but the airport stinks.)

Dr. Mervis was kind enough to let me conference call in on her chat with Shawn this morning, so I got to hear firsthand the scoop on Clare. Overall, she is doing well and actually scored in the average range as compared to typical children in her verbal and nonverbal reasoning skills (which means she scored quite well as compared to other children with Williams syndrome). When it comes to her spatial skills, however, not so good. Which we expected, knowing Clare as we do. She also is having more trouble than is typical for kids with WS with some language skills. The fact that she is having trouble comes as no surprise since we, her private speech therapist, and her team at school have all noticed that there are some challenges when it comes to language development with Clare. However, it was a surprise to us that this is NOT typical in WS and that Clare is behind in these skills when compared to other children with WS. Definitely something to work on, keep an eye on, and be sure the school knows that it cannot be blown off "just because she has WS." (Not that the school is overlooking it right now, just something to keep in mind when discussing Clare's progress at our next team meeting.) The actual term for Clare's problem is "specific language impairment." Unfortunately, she is about a year too young to administer the actual test for this impairment, but it is certainly something we can work on over the next year and have Clare tested when she visits Dr. Mervis again next February. In the meantime, Dr. Mervis provided some recommendations for what Clare should be working on at home and in school and emphasized that Clare should definitely repeat kindergarten. All in all, I think the visit with Dr. Mervis was successful, but I know I am going to have two very tired people arriving home (hopefully any minute).

We leave at 5:30am tomorrow morning to bring Clare to Children's Hospital Boston for her dental surgery. If all goes as planned, Clare is going to have a tooth extraction, some fillings, x-rays, and a cleaning (what else can they squeeze in dental-wise while she's under anesthesia??). So she will be good to go for another six months. Then Clare and Shawn will stay overnight on the cardiac floor so they can keep an eye out as she recovers from anesthesia. Her surgery is scheduled for 8:30am, so prayers are appreciated!

Saturday, February 12, 2011

Surgery Scheduled

Clare's dental surgery is scheduled for February 23, which is perfect. It gives me more than a week to get all my ducks in a row plus it's over school vacation week, so Clare will not miss any school. Clare is only in a half-day morning program so between the Williams syndrome clinic, snow days, and snow delays (kindergarten is cancelled completely when there is a delay), she has already missed so much school over the past few weeks. Yet the surgery is not so far out that my days of freaking out over whether the "bubble" in her tooth has popped and is releasing dangerous bacteria into her bloodstream and potentially her heart is down to only a *short* 11 days.

I will take Clare down to Boston on February 16 to do her pre-op day. This will give us the opportunity to meet with the team involved in the surgery and go over everything. I have been impressed so far with how on top of everything they are (as they should be, but you never know!). The surgical coordinator and I talked yesterday on the phone (about an hour after she called me with the surgery date), and she had already spoken with Clare's cardiologist, pediatrician, and nephrologist to get the approval from them to proceed with surgery. She only needed me to sign a release with the endocrinologist before they would release any information. (Which took me about three minutes thanks to fax machines!) Everyone has given the thumbs up and is on board, so we are ready to go. I will get to talk with cardiac anesthesia on the 16th, but we have dealt with them (too) many times over the past six years, so I know they are usually well-familiar with WS and the associated anesthesia risks.

Clare will have to stay overnight on the cardiac floor following the procedure just to be on the safe side. She has had issues with anesthesia in the past (abnormal rhythms, cardioversions, blood pressure swings), but she was undergoing a catheterization each time, so the doctors were actually in her blood vessels and heart. When she had her fistula surgery eighteen months ago, she did great during the six-hour surgery with anesthesia. She had a lot of trouble post-surgery from the effects of so much anesthesia, though - high fever, vomiting, general yuckiness. The dentist assured me that this was a straightforward procedure, however, and Clare should not be under anesthesia that long. Hopefully an hour tops. So her recovery from the anesthesia should not be as bad. But I am glad they are keeping her overnight so the cardiac nurses and cardiologists can keep an eye on Clare post-surgery. If all goes as planned, she will be discharged the next morning.

I have our childcare lined up, and we are ready to go! Thankfully Clare is only in pain when we go near the tooth, so we are avoiding brushing that part of her mouth until after the surgery. Shawn and Clare are heading down to Kentucky on the Monday and Tuesday prior to her surgery to meet with Dr. Mervis, one of the WS experts in this country. So it's going to be a busy couple of weeks for Clare. Unfortunately, school break is not going to be so relaxing for her!

Thursday, February 10, 2011

The Perfect Storm

There are some days when it's just one "problem" after another, and I am on overload at the end of the day and feel like I don't even know if I am coming or going. When Clare was younger, these days happened more often since she was in a more critical condition, so I am out of practice with dealing with them now!

Over the weekend, I noticed that one of Clare's back molars looked funny. Clare has had some cavities for almost a year now that we have been monitoring with our local pediatric dentist (who specializes in children with special needs) and the pediatric dentist at Children's Hospital (who would perform any necessary procedures in an OR setting at the hospital). Since anesthesia is very risky for individuals with Williams syndrome, we have been doing all we can to avoid having any dental work done. In November, Clare had a set of x-rays taken down at Children's, and we were cleared to just continue her care with our local dentist. The cavities had sealed themselves and none of the nerves were in danger. So when I saw that her molar looked black, at first, I thought it was just chocolate (shocker!). But Clare said it hurt when we brushed it, and the "chocolate" did not come off, so I made an appointment to have our dentist take a look. Which was today. And, sure enough, the pulp of the tooth is gone. So she needs a pulpectomy (kids' version of a root canal) and crown. As in yesterday (as the dentist put it). Clare is now at a dangerous point where bacteria getting into the bloodstream is a very real risk.

In addition to our normal running around, today was also allergy clinic day for Jamie (if you're a regular blog reader, you know what hell that is for me!), and we are at the end of Shawn being away for the week at a sales meeting. So my patience is thinner, my tolerance is lower, and my legs and back are killing me from shoveling slush and ice this week. And then I added phone calls to Clare's cardiologist and Boston dentist to my to-do list. We still had not heard back about Clare's echo results, so I wasn't even sure she was cleared to undergo anesthesia right now.

Clare's cardiologist, Dr. S, (finally!) called me back tonight. Right at dinner time. On my cell phone (we get almost no service at our house). So I left four kids at the table with hamburgers and pasta and raced out to answer the call and freeze my tushie, standing on the driveway (neglected to grab a coat or gloves in my rush to pick up before it went to voicemail) while I talked to the cardiologist. But I am not complaining. Really I am not because I was so glad to hear her voice.

The good news is that Clare is cleared for her dental surgery from a cardiac standpoint. She is still clinically stable, and her heart looks good compared to what it used to look like. Dr. S feels that Clare will tolerate the anesthesia well and, as long as cardiac anesthesiology is on board, she gives the thumbs up for the dental procedure. However, the bad news is that her pressures are creeping up again. Her aorta gradient and one of her pulmonary gradients are higher as well. Not dangerously so in that she needs to have something done right away, but we are at the point where her cardiologist is talking "cath." Yuck. Dr. S consulted with the cardiologist who handled Clare's care when she was cathed as a baby, and they agree that within the next 12 months or so, Clare really should undergo a cath. Primarily to get a more exact picture of what is going on in Clare's heart, but, most likely, to do a little work while they are in there. The plan right now is to do another echo in 6-9 months and then schedule a cath from there.

So there's my perfect storm. Too many things piled on top of each other in a chaotic day after a long week for me to think about. I am still waiting to hear back from the dentist at Children's to actually schedule a date. I really do not think the procedure itself is going to be a big deal. It's just more the trepidation of putting Clare under anesthesia combined with the fear that bacteria could get into Clare's bloodstream if this is not taken care of soon. Hopefully I'll hear something before the weekend.

Monday, February 07, 2011

Feeling Sorry For Myself Never Gets Me Anywhere

Clare and I went to see Disney on Ice: Princess Wishes last week. I took Clare last year, and she loved it. Every time we pass the hockey arena, she asks if the princesses are there. So when they came around again this year, I knew we were definitely going. Clare insisted on dressing up as Cinderella that night (she remembered seeing all the other little girls in their princess dresses last year, and she was not wearing one). She was so over-the-top with excitement to go. We had seats with friends of ours, so Clare was thrilled to be with her friends as well.

For me, watching Clare during the show was better than watching the show. She gasped, exclaimed, grinned, was enthralled by the show. Until the big, evil, fire-breathing dragon from Sleeping Beauty appeared on the ice. Fireworks exploded from its mouth and a line of fire blazed on the ice (actually pretty cool from a special effects viewpoint). In one instant, the enchantment was over. It was a hundred times worse than when Cinderella's magic wore off at midnight and her gorgeous carriage was just an old pumpkin again. All of a sudden, my princess was a quivering mess. Sitting on my lap, crying, hands over her ears, which eventually erupted into a full-blown hysterical fit. The evening ended with me carrying Clare out of the arena and into the icy night while she screamed bloody murder.

Clare fell asleep in the car almost immediately, and I cried the whole way home. The horrible part is that I was not crying for Clare. I did feel bad that the dragon scared the you-know-what out of her and put a sour note on the end of our otherwise-wonderful princess-y evening. And I do hope that this does not ruin her anticipation of going again next year. But I admit I was crying for me. For me. For poor, poor me who had to have a child who could not, at the age of almost-six, understand that the dragon was not real. A child whose hyperacussis made those fireworks sound like a thousand times louder than they really were. A child whose body is programmed in such a way that the adrenaline and cortisol rush of the "fight or flight" response lasts four hours in her little body. Four hours of stress hormones racing around her bloodstream, often causing her to become aggressive, hypervigilant, and over-reactive. Sometimes paralyzing her so that she cannot move. It really was a blessing that Clare exhausted herself out and fell asleep because once she is "triggered," it can take awhile for her body to maintain stability again.

So I cried the whole way home. Shawn came out to the garage, carried Clare in, and put her in her pajamas and into bed. He asked if I wanted to talk about it, but I was still so wrapped up in my self-misery and pity-party that I just wanted to go to bed myself.

Waking up to a new day usually helps me gain perspective. And having my little curly-haired girl crawl into bed, put her arms around me, and say, "I'm so sorry, Mommy," made my heart ache. I have challenges in my life. We all do. And, yes, sometimes I think my challenges are greater than other parents because Clare does have so many medical, developmental, and emotional issues and that puts me into my "poor me" state of mind. But then I realize that feeling sorry for myself never gets me anywhere. I cannot wallow in self-pity and spend the day in bed, which would be my preference some days. Something or someone will snap me out of my funk and make me focus, not on the challenges in my life, but on all the blessings. I am not a touchy-feely, mushy kind of girl. I loathe self-help books, Dr. Phil types, and other such devices. But I am finding these days that a dose of perspective, a thought to meditate on, and prayer can get me through. And all the hugs my children will give me!