Doctor's Office

For the last few days, Erik has not been himself. At first, I thought it was the cold he seemed to be coming down with last Thursday. Then I noticed his skin was irritated and possibly infected in places. He has been naturally rashy from day one. I have every cream, dandruff shampoo, and special soap known to man and am always trying to keep one step ahead of his skin problems without much luck. His face is ruddy, blotchy, and peeling. His legs are alarmingly bumpy and dry. He had cradle cap for over a year with large, orange flakes (thank goodness for blond hair). On top of everything, his tear ducts are clogged, and he always has a little tear on one side or the other. My red, blotchy, tear-streaked boy always gets sympathy from strangers because they think he is sad. Now the perpetually happy boy who doesn’t particularly notice my absence or presence has been fussy, clingy, cuddly, and whiny. Erik and I headed to the pediatric office to see the doctor on call yesterday morning. He diagnosed Erik with a unilateral ear infection and eczema all over his body (even his manly bits). Being opposed to antibiotics unless absolutely necessary, with the doctor’s blessing, I decided to see if Erik can fight the ear infection on his own before filling the prescription for amoxicillin. I did go to the pharmacy to pick up hydrocortisone cream for his face and swollen sensitive areas. I can already see the difference in his face. The doctor told me that the redness will go away with the cream, but his face will always be rough. That makes me a little sad, but I suppose I can live with it. He cried himself to sleep last night but slept and seems just a little cranky but consolable today. The other day Brian and I tried to remember all of the parts of the ear but ended up in fits of laughter because all we could come up with were flower parts like “stamen” and words that sounded suspiciously like parts of a 1979 Dodge Dart. Time to review my ear, nose, and throat terminology, I guess.

Happy Memorial Day Weekend!

Little darling, it's been a long cold lonely winter
Little darling, it feels like years since it's been here
Here comes the sun
Here comes the sun
And I say it's all right

--Beatles ("Here Comes The Sun")

We are having a relaxing weekend thus far. Brian is cleaning up the colossal mess I made in the kitchen making eggs, home fries, biscuits, and gravy for breakfast. Erik ate his Malt-O-Meal using his very own spoon and now has crunchy, maple-scented granules in his hair and behind his ears, but at least he is dressed (unlike the rest of us). Here is Erik playing with his learning table in his room this morning.

Looking back on the week, it was emotionally draining for a variety of reasons, but Thursday we were lucky enough to have dinner with Alan, Kathy, Dominick, Lisa, Cory, Baby Owen...and Ed, our friend from our high school days who was here from Portland on business but took the time to spend quality time with our families. I contracted a couple cases of the giggles, which is commonplace when Ed and I get together, and that felt fantastic. Erik was absolutely amazing. He broke out of his shell, even around the other kids, and worked the crowd, making sure to touch everyone and make them feel acknowledged in his quiet way. It is wonderful to have the friends and family we have, even the ones we don't see as often as I would like. You are all a great support!

I will teach Erik Monday how to put the flag in its holder on our house. We wish you a wonderful, safe Memorial Day weekend!

Echocardiogram

Yesterday morning Erik and I drove to the clinic for his followup echocardiogram. He was understandably surly from being denied breakfast. Brian met us in the waiting room, where Erik entertained the staff and other patients by walking around in his Squeakers, tennis shoes that sound like a doggie chew toy with every step. It feels strange being a patient’s mother in the place I used to work for nearly a decade. For years, I used to transcribe echocardiograms directly into the cardiology department’s archaic computer system in a cramped, dark room early in the morning while I had my first cup of coffee. I frequently typed reports on kids without thinking much about their tiny bodies or their fearful parents faking smiles in exam rooms. I realize now that on the other side of the medical report there is a world of anxiety and bargaining with God. Our little family was led into an exam room, where Erik was weighed and measured. He was then stripped down to his diaper, and we signed consent forms for the medication that would make him sleep. I hate watching Erik run around in his diaper because he looks painfully skinny, stiff-limbed, and awkward. I watched every muscle in his body contract and relax as he moved about, his ribs and spine cruelly showing through his pale skin. Erik was not hugely impressed with their very wheel-less toy selection, a collection of likely germ-infested plush animals. At that point, the tech presented Erik with the exact same fish puppet that sent him into orbit last time. I cringed visibly as she pushed a hidden button and the seemingly innocent toy emitted a horrible bubbling sound like Jimmy Hoffa drowning in the Hudson River. I am sick to death of explaining the whole Williams/hypersensitivity to sound thing to people and have decided Erik has to learn to cope with this noisy planet at some point anyway. He turned his face into me but remained tear-free. After signing the papers, we then settled in the echo room, a cramped space with a gurney-like bed and chairs surrounding it. The ultrasound machine, roughly the size of a Geo Metro, hummed ominously in the corner of the room. The techs filled a needleless syringe with chloral hydrate, a foul-tasting, pink liquid, and we held our screaming, crying child down so they could force it down his throat. Even after all we have been through, I continue to fight back tears myself. The lights were turned off, and we were instructed to soothe Erik to sleep. Erik and I sat in the strangely out of place looking glider rocker (Mother Hubbard meets Jonas Salk). We rocked for some time, but Erik fought the medication and tweaked his lips to make the satisfying “bibble” sound he loves so much. When he slowed down a bit, we laid him on the gurney. Each time we thought he had fallen asleep, his eyes would pop open, and we would get a bibble-bibble and a sleepy, drunken wave. The techs gave him a smaller second dose, which Erik didn’t protest this time, and he finally slipped into an alarmingly soundless, un-Erik-like sleep. The techs attached three leads to Erik’s chest, a blood pressure cuff, and a vital sign monitor that glowed like a red-hot ember on his toe. The tech at the lighted keypad and monitor gooped up the ultrasound wand and ran it over the contours of his chest. This is how the entire procedure went from this point on for what seemed like an eternity. We watched the valves and chambers of Erik’s heart convulse through a pie-shaped area on the computer screen. The tech would snap still photos of some areas with the computer, and the machine then spit them out through a slot on the side. Brian and I sat quietly to the side of the gurney while the techs talked and did their jobs. I wished I was back on their side of things, not watching my own little boy lying motionless under wire cobwebs looking suddenly very grown up. The pressure in my head seemed unbearable, and I felt like screaming. I was filled with frustration knowing that we will spend the next years waiting like this, at times steeped in anxiety. Waiting to see if Erik’s health will hold. Waiting to see what his abilities are. Waiting to see how bad his syndrome will be as he ages. Waiting to see if he will be teased at school. Waiting to see if he will be a desperately lonely, perpetually unmarried man as his friends around him start their own families. Waiting in rooms designed just for waiting and filled with disgustingly chipper parenting magazines. Waiting for the other shoe to drop when things might turn out just fine. I closed my eyes and could not escape the sound of his heart, translated by computer to sterile, electronic beeping. I tried to will myself into the mural on the wall, a cartoonish cutaway view of the beach and ocean at twilight with a giant whale leaping up and piercing the surface of the sea. It made for kind of a pleasant effect in the amber glow of the machines under the pink, plastic, glow-in-the-dark stars. After what seemed like a lifetime, the echo leads were disconnected, an ECG machine was wheeled in, and another tech hooked up yet more wires with clips that bit into new stickers placed in strategic places on Erik’s chest. This machine ejected another paper strip printed with the rhythm of Erik’s heart. After the goop, equipment, and wires were removed from our son, the moment of truth had arrived. Dr. T. stepped into the room and informed us that everything looked normal, although she did not guarantee things would remain normal as he grew. We would be sitting here yet again in one to two years. We are deeply thankful for this news and the relief it provides, although for some reason we don't talk about it much or feel like celebrating. To me, it seems unnatural to react at all to what physicians tell us anymore, which must look strange, but we always say the standard "that's greats" and "thank yous" we carry with us for such an occasion. The pressure in my head began to subside. Erik woke up long enough to have a little water from a paper cup, and they allowed us to go home. Brian placed Erik in his car seat and said goodbye to us before we went our separate ways for the day. When we arrived home, I tucked Erik in his crib, smoothed his hair, and gave him his binky. He looked at his surroundings briefly, seemed to nod his approval, and shut his eyes.

Changes

Okay, I admit it. The last 19 months have been challenging. Never in my life have I struggled so hard to make it through each day and come out smiling. I do not always succeed. I can actually look back at the calendar at this point and map out each stage we have survived. When we decided to become parents, I had no idea what it would entail. My old life and the person I used to be are long gone. I wake up in the same house with the same husband/cat, and I am thankful for that, but everything else has changed. During pregnancy I experienced "baby brain," which I formerly believed was a ridiculous old wives' tale. Silly girl! I always paid most of the bills, and on two occasions the bank notified us I had written completely random, mystical numbers on our mortgage checks that did not seem to correspond with anything anywhere. I finally had to hand over my bill-paying pen until Erik was born. Brian could have ribbed me mercilessly about this, but he (a) knew the pen would be plunged through his eye socket or (b) he was being kind to his swollen, mentally challenged wife. In addition, I was obsessive-compulsive woman, returning to the house three times before work to grab forgotten items or checking to see if I extinguished a candle/the stove/my curling iron. After Erik's arrival, the hormones subsided, and I found I could think again, only to realize Erik was not going to cease crying. Brian and I began the next stage without sleep. I wondered if I would ever get my brain back and my sluggish synapses firing again. I wondered if being a mother was as mind-numbing for other women and if it was normal that I was not bonding with my baby or enjoying myself at all for months at a time. I admitted this to my husband, but it was a terrible, dark secret I carried for months. Truthfully, this would have gone to my grave with me if Erik had not been diagnosed. Thankfully, Dr. G., Erik's pediatrician, gave Erik medicine to quell the acid that was burning his throat and face raw, and life began anew. She also told me to discontinue breast feeding, which stopped my infections/fevers/joint pain, Ebola virus-like bleeding, and the unbearable, shooting pain I endured for three months. No more cold sweats before latch on! During my time off with Erik, the job I had for 10 years changed dramatically without warning, and I decided it was time to move on. I will never forget the feeling of being escorted by the human resources manager to my car with my little box of belongings without even saying goodbye to anyone. Honestly, this was like a death to me, as it was a large part of who I felt I was and how I achieved my independence before I met Brian. I was devastated. I tried working part-time in another office, thinking the interaction with others would be fun. As it turns out, breast feeding was more fun. I was horribly unhappy and found myself sitting jobless at home again. Thankfully, a very wonderful ex-coworker of mine called out of the blue and needed help with her own accounts, and I am doing medical transcription from home now. It was a giant adjustment to go from sitting at a desk in a busy office with a line of people waiting to talk to me and a constantly ringing phone to sitting in a quiet room in my house with a cat staring at me. It took time to realize I was not a failure and was just as important as I was before to different people. It took a year to fully adjust to being a mother and even longer to figure out who I am and what I am supposed to be doing. Once I had a handle on that, we were told something was very wrong with Erik. While it was a relief to get a diagnosis and discover why everything had been so hard for us, we had to get up and dust ourselves off yet again. I had to take Unisom nightly for a month after the visit to the geneticist to get any sleep at all. Now that I am sleeping better, I am constantly coming down with something. Thankfully, Erik has the immune system of an elephant (ever hear of a sick elephant?) and has been sick only twice. We shared his last illness, and he did much better than his poor old mother. I have learned that being a parent means contracting exciting new illnesses that make death look extremely attractive. Add stress to the mix, and they hit extremely hard. I begged Brian to hasten my demise, but he refused and took care of me instead. Now that I am over the latest bird flu/malaria/Hantavirus thing, I seem to be coming down with yet another *&%$#@! sore throat.

I know that I will someday be able to look back on this particular stage and see that we trudged through it successfully. I know now that life means constant change, as stagnant as things seem to be at any given time. Although change can be painful, it can mean moving on to something better. For the time being, though, please pass the cough drops.

Picture Perfect

I find it almost impossible to get suitably decent video or photos of Erik, as he doesn't stay still for long or quickly ventures toward the camera. There is also the issue of his smile instantly evaporating before the photo is taken (why does it take so bloody long after one pushes the button?). I have probably hundreds of Erik photos that look like textbook pictures of Williams syndrome taken by researchers and geneticists in clinical settings (mouths open, drool stringing down, glassy eyes). I have seen those horrible freak show photos on line, and I hate them. I now have a hard drive full of them of my own son. I want to scream, "That's not my kid!" It's similar to when he goes to his school or is around a lot of people and noise. For the most part, he shuts down, although this is improving with time and age. I have seen him in the midst of happy, animated, playing kids, just sitting there expressionless or on the verge of tears and shaking violently. I used to cry after that happened and avoided taking him to noisy places to be around other kids, especially when he was tired or I didn't feel like I was up to it emotionally. It was isolating for both of us. We now go everywhere together and just hold on to each other for support. He is doing great and getting less upset as time goes on. I need him as much as he needs me. Many people are now impressed with a couple little signs he gives to indicate he is in there somewhere or little things he does which are old hat to him but seem impressive in that setting. At this point, Brian and I just smile and say nothing, as we are now accustomed to this phenomenon. We both know that he is completely different at home when nobody is watching. He is a busy, chatty, smiling, belly laughing, sometimes rambunctious little boy in his natural habitat. I believe this photo was taken last week after his in-home visit. We were playing and relaxed. I want more than anything for my friends and family to see what is inside this very wonderful little person, as his true personality is sometimes hidden at this point. I know that once he breaks out of his shell, there will be no stopping him, but for now he is holding so much back. This week will be crazy with Erik's school, his home visit, and the echocardiogram. We are starting off on the right foot, as our boat leaves the property today with an excited new owner. I am a very happy girl. Brian and I both agree it is better to know people with boats than to own one! I will post the news on Erik as it comes. I hope everybody has a good week. Thanks for stopping by.

Washington University School of Medicine Study

Public release date: 19-May-2006
Contact: Michael C. Purdypurdym@wustl.edu 314-286-0122
Washington University School of Medicine

Computerized atlas highlights 'plethora' of changes in brain disorder May 19, 2006

A computerized atlas has brought unprecedented sensitivity to the search for brain structure changes in a genetic condition known as Williams syndrome, revealing 33 abnormalities in the folding of the brain's surface. The disorder, which occurs in 1 in every 20,000 births, impairs visual and spatial skills but preserves musical ability and sociability. The findings, published in The Journal of Neuroscience this week, suggest the same technique may produce insights into more common brain development disorders such as autism, according to the researcher who developed the brain atlas at Washington University School of Medicine in St. Louis. "We already have a study of autism well along in the pipeline with colleagues at the University of California-Davis," says lead author David Van Essen, Ph.D., the Edison Professor of Neurobiology and head of the Department of Anatomy and Neurobiology. "We think that study will also highlight several previously unrecognized abnormalities in the folding of the cerebral cortex." A more detailed inventory of changes in brain folding and its connections to changes in cognitive function should enable researchers to better understand the origins of developmental brain disorders and begin devising new approaches to treat them. Van Essen announced the creation of the brain atlas, known as the Population-Average, Landmark and Surface-based (PALS) Atlas, in summer 2005, when it was made available online. PALS is the first atlas that accurately portrays the complex folds of the cerebral cortex not just from a single individual but from a group of individuals. This is important because the folding of cerebral cortex varies dramatically from one person to the next, similar to the variability of human fingerprints. Van Essen and colleagues from Washington University, Stanford University and Cedars-Sinai Medical Center in Los Angeles used data from brain scans of 16 individuals with Williams syndrome for the study. For the analysis, they aligned or "registered" each individual brain to the PALS Atlas. This allowed them to identify 33 changes in the folds of the cerebral cortex, the surface layer of the brain credited with many higher cognitive functions. "We already knew that there are structural abnormalities in the brains of individuals with Williams syndrome," notes Van Essen. "What is interesting and new is that we found a plethora of changes discernible on a background of normal variability in folding patterns, and the fact that the changes are strikingly symmetric." Scientists found 16 changes on the left side of the brain and identified 16 changes in corresponding regions on the right side of the brain. Another abnormality was present only on the right side of the brain. Any one person with Williams syndrome would be unlikely to have all 33 changes, Van Essen explains, and the degree of change present can also vary. For example, they found that the olfactory sulcus, a groove or furrow-like structure just above the olfactory tract, tends to be shallower on average in those with Williams syndrome. Genetic and environmental differences, reactions to injury, and inherited disorders can all change the topography of the brain in minor and major ways. According to Van Essen, the new study shows that PALS can help scientists look beyond such individual variations to quantify brain structure trends in ways that may provide important insights. Williams syndrome results from deletion of genetic material on a region of chromosome 7, but the size of this deletion varies across individuals. The new inventory of structural changes may one day enable scientists to more closely associate genetic alterations with the development of different brain structures, or allow them to more precisely link alterations in specific structures to changes in cognitive functions. Although interventions to alter brain development in Williams syndrome are likely still a long way off, identifying the connections between genetic changes, alterations in brain structure and changes in brain function may help clinicians and teachers develop customized approaches to education that allow children with Williams syndrome to take full advantage of their unique capabilities, according to Van Essen.

View online: http://mednews.wustl.edu/news/page/normal/7254.html?emailID=9409

By Michael Purdy Van Essen D, Dierker D, Snyder A, Raichle ME, Reiss A, Korenberg J. Symmetry of cortical folding abnormalities in Williams syndrome revealed by surface-based analyses. The Journal of Neuroscience, May 17, 2006. Funding from the National Institutes of Health, the National Institute of Mental Health, the National Institute for Biomedical Imaging and Engineering, and the National Science Foundation supported this research. Washington University School of Medicine's full-time and volunteer faculty physicians also are the medical staff of Barnes-Jewish and St. Louis Children's hospitals. The School of Medicine is one of the leading medical research, teaching and patient care institutions in the nation, currently ranked fourth in the nation by U.S. News & World Report. Through its affiliations with Barnes-Jewish and St. Louis Children's hospitals, the School of Medicine is linked to BJC HealthCare.

Embraces

I went to France for the first time in the early 1990s. I remember realizing that all of the words I had mindlessly memorized during my years of French classes allowed me to communicate there. The first time I spoke this seemingly meaningless language and people understood me seemed like absolute magic. In my opinion, there is nothing more frustrating and sad than not being able to communicate with another human being and nothing better than understanding each other. Every day I repeat words over and over for Erik: duck, dog, bath, water, milk, cup, mama, dada (you get the idea). Most of the time he will repeat one sound in that word (dog = guh) and does not generally use words spontaneously. He is beginning to look toward the objects we name (book, light, fan, etc.). For the most part, though, he mimics the sound without seeming to absorb the word or its meaning. After his in-home visit yesterday, I asked the kid to give me a hug. Not only did he say "hug," he gave me one! I almost fell over. I did not teach him that word. I have talked with other WS parents. Many of us seem to be skeptical about what our kids understand, and we scrutinize their words and actions to see if the lights are on and anybody's home. It's strange, but it is almost a relief when I can prove Erik doesn't understand to those around me. Like many of us, I am still fighting that fear of being hurt/disappointed and building a wall, attributing his actions and behavior to a lucky accident or misinterpretation. I'm afraid to believe because I might be wrong. This, however, was crystal clear. When his little arms wrapped around me and I heard that word, I was suddenly 20 years old and in Paris again, feeling like I could accomplish anything without any of the fear that comes with age and experience.

Merci beaucoup, Erik. Je t'aime.

Malaise

I have a moment to myself before Erik wakes up this morning. Because it was so hot yesterday, Erik cried and cried last night until we finally gave up and got him out of his crib. When he was tiny, I used to sit on the couch with my feet up on the coffee table, position him on his side along my legs, and rock him back and forth. I spent hours/days/months/a year doing that to ease his discomfort (I have seen EVERY episode of Dawson's Creek). I still do that sometimes, although he has to be folded in half at this point. That did the trick last night. We blasted the air conditioner to cool his room off, changed him into cooler PJs, and finally got him to go to bed. I am not looking forward to working upstairs today. Brian bought a swamp cooler for my office, but it has a drip, so I must wait until we locate some plumber's tape. It sounds like a 747 taking off, and I am hoping I can hear what I am transcribing. However, after working in sweltering heat yesterday, I am thankful for it. Sometimes I think of the people baking in the sun outside harvesting strawberries or putting up houses and think I am the biggest wuss. This morning I am failing to find my sense of humor already. There are days when it is hard to see the bright side of anything, and this is one of them. What I have to tell myself on days like today is that maybe tomorrow I'll feel more like myself. In fact, I probably will. Sometimes I'm surprised to find that I feel better even in a couple of hours. We'll see. the trick is to hang on for just a little while longer. Erik has an in-home visit today from EIP (Early Intervention Program) at 3:30.

The Beauty of Holland
by Emily Pearl Kingsley

I am often asked to describe the experience of raising a child with a disability -- to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this...

When you're going to have a baby, it's like planning a fabulous vacation trip to Italy. You buy a bunch of guidebooks and make your wonderful vacation plans. The coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very, very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The flight attendant comes in and says, "Welcome to Holland."

"Holland?!?", you say." What do you mean, Holland? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy. "

But there's been a change in the flight plan. They landed in Holland and there you must stay.
The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine, and disease. It's just a different place.

So you must go out and buy new guidebooks. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for awhile and you catch your breath, you look around, and you begin to notice that Holland has windmills. Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy, and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say, 'Yes, that's where I was supposed to go. That's what I had planned.'

And the pain of that will never, ever, ever go away, because the loss of that dream is a very significant loss.

But if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things about Holland.

Poetry

I am not fond of Tuesdays. They are becoming my emotional nadir each and every week. I take Erik to therapy on Tuesdays, which goes wonderfully, but I crash and burn afterwards. I am not sure why. What is a girl to do? I crack myself up. Those of you who know me will not be surprised. I know a lot of you who are reading this blog are hurting, and I am so sorry. I am hurting, too, and would not wish this kind of pain upon anybody. However, please don't forget to laugh here and there. It gets easier with time. Here is something I wrote today to chase the blues.

Ode to Erik’s Binky
by Nancy P.

Oh wondrous pacifier, blue and round
Well known to us as “Binky”
Your rubb’ry plumpness stuck with hair
Each second much more stinky!

I see it’s true that you’re made
For just months one through three
As you’re bobbing in his face --
A rowboat lost at sea!

He does not care that long ago
We ceased our scrubbing you
Months ago we brushed you off
And said, “Now that will do.”

For ev’ry lintball, ev’ry germ
Is added flavoring
For beloved Binky you are made
For bedtime savoring!

Dare to Cheer

Today a woman posted a message on the Yahoo WS board that stuck with me all afternoon. She said that she was excited but “afraid to mention” how well her granddaughter was doing at 5 months. This little girl had finally made it to 10 pounds and was smiling. I remember how long we had to wait for Erik’s first smile. One of the gifts that Erik has given us is brand new appreciation of life’s little things. The things that a lot of parents might think are small potatoes in their children are momentous accomplishments to our family that warrant celebration! I have learned that if our fears prevent us from cheering our child on, we will miss the moment, and the moment will be gone. We have earned the profound joy that comes with each small accomplishment and must open ourselves up to it. It takes the sting away, even for just a moment. My heart swells each time Erik tries something new, and I will try harder than ever to believe in him and cheer him along every step of the way, even if I am scared out of my mind (which I am most of the time). Today Erik brought his grandfather a book and repeated that feat at home for Brian—TWICE! Brian yelled the news from the nursery, and I cheered.

Thursday, May 25th is looming ahead on the calendar. Erik has his followup echocardiogram scheduled. He will be sedated, and Brian and I are both nervous about that. We did not allow sedation last time, as we did not know what was going on in his heart and were afraid of losing him. We now know that his heart is healthy, so we are going ahead with the process. The main concern is the supra-aortic area. This area is commonly narrowed in children with WS and sometimes needs stenting. The velocity of the blood moving through this area in Erik was slightly elevated on the last study, so the doctor wanted to check it again.

Mother's Day 2006

Mother's Day this year felt different. I felt a little off all day. I think perhaps Brian and I are still processing Erik's diagnosis in our own ways, but the process is by no means close to being complete, at least in myself. I have never had a classic panic attack in my life but the other morning began feeling claustrophobic in my own body. The knowledge that Erik has Williams syndrome is like a brick in the back of my brain, and I can feel the weight and mass of it every second of the day, even when I am not consciously thinking about it. I carry it with me everywhere. I assumed that this heaviness would dissolve by now, but it hasn't. When I realized that this was quite possibly a permanent fixture in me in almost a physical sense, I couldn't breathe for a moment. I cannot escape this heaviness no matter what I do or where I go. On the other hand, today I have thought a lot about being Erik's mother and what that means. It is a great responsibility and honor. I have never loved anyone so much. Erik's syndrome is an intrinsic part of him, although it is only a part of him. Comparing him to other kids with Williams syndrome, I realize that we are lucky he lived at all, as many kids with WS die during infancy and toddlerhood of heart-related problems. The ones who live often have shiny, purple scars running down their baby-perfect chests from surgery. During my pregnancy, Erik was so still in my womb but let me know occasionally he was hanging on with taps and kicks. My two previous pregnancies before him ended without warning, and a sign here and there was good enough for me, although I now know that there were differences right from the beginning. I believe he was saving his strength to survive. I remember hearing his heartbeat for the first time in the doctor's office. I recorded the sound on a little tape recorder and have it to this day. At the time, I had no idea how important that sound would be. Erik's heart is perfect, and he is perfect. I cannot describe how thankful I am for that. It is a Mother's Day I will not forget.