Erik Quinn: The Heart of a Family: February 2009

Saturday, February 28, 2009

Rare Disease Day

Thursday, February 26, 2009

Mi Sonador

Brian walked me through the front door of the church last weekend instead of sneaking through the side door to find Marla, Erik's aide. Brian carried Erik in his arms past the long rope connected to the happily clanging bell above our heads. Our pastor saw us coming, and his eyes met mine for an almost uncomfortable amount of time. I could tell he was going to say something to me but wasn't finding the words in time. He took my hand and thanked me for my letter, telling me that he was in need of a little inspiration. He admitted to me that it was nice to have something to occasionally remind him why he is doing the job that he does. I really didn't know what to say in return except thanks. I then smiled and placed the palm of my hand on the scratchy, brown tweed of his suit jacket as I passed by. Later on in the service, he showed this video to the congregation. It's amazing to me what is possible and frightening how easy it is to put limits on ourselves and our children. It's even easier to just give up and quit dreaming.

Erik danced into hippotherapy like Mikhail Baryshnikov this week. He was up on his toes all the way from the Jeep to waiting area in the barn. His therapist studied him with her head cocked quizzically and then asked me if he was going through a growth spurt. That doesn't begin to describe how he seems to have shot up lately. I attempted to measure him at home the other day, but the process frightened him to death. He loves to snack, and he seems to be eating constantly these days. I believe that his Achilles tendons just aren't keeping up with the length of his legs lately. This used to scare me, but I have noticed it happens from time to time. All we can do is keep him stretched out and hope for an outcome that doesn't require surgery. Sometimes when he wakes up from his nap I work on his legs before he is conscious enough to escape. I have made a promise to myself that I will cram his orthotics on his legs more diligently this week in order to keep his feet flat.

His home blood pressure cuff arrived this week, but I have not been brave enough to attempt to take a reading without a bag of chocolate candy nearby to bribe him. His blood work to check his hormones and kidney function has been sent, and I am waiting for the results. He is savvy enough about the medical field to understand what happens at the laboratory. I thought he was clueless as he calmly played with the toys in the waiting room, but when they called his name, he burst into tears and pleaded, "I don't want to!" I prepared to hold him down in the chair in the back room and assured the phlebotomists he doesn't hold a grudge. At least not against them.

Right now I am sitting at my desk at this ungodly hour with an amused smile playing over my face. Using the Internet, I just successfully identified the word Erik said when I asked if he wanted a cracker and placed a whole wheat Ritz in his hand yesterday.

After he studied it, he seemed slightly annoyed and hissed, "Galleta."

I see that's Spanish for hardtack. Or cracker.

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Thursday, February 19, 2009

The Secret Song

This is a beautiful article about Jeremy Vest. I think a lot of us can see our children in Jeremy, which is why I find him so incredibly fascinating.


The Secret Song

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Wednesday, February 18, 2009

A Tale of Two Kidneys

Erik and I headed to the children's heart center today for a blood pressure check before his renal ultrasound at the hospital. When Erik approached the nurses' station carrying his filthy stuffed dog, the woman behind the counter went into the next room and announced, "Stinky Dog is here for his blood pressure check." Today his blood pressure had come down substantially into about the 70th percentile.

The blood pressure cuff is extremely upsetting to Erik, and while he was being calmed, one of the staff members motioned for me to talk quietly with her in the hall. Erik's photo from a prior visit is tacked to the bulletin board above the nurses' station. She told me that a young woman with Williams syndrome came in recently and pointed Erik's photo out to her mother, asking if she thought Erik had WS, too. After they studied his face, they apparently both came to the conclusion that he did. Once again, I have likely crossed paths with this other family I know of but have never met. It's strange. I can never confirm this is the same woman, as privacy laws do not permit the release of her identity, but it is strangely comforting to know she has been in the same exam rooms and waiting rooms we have. Maybe someday we'll meet.

From there, Brian joined us, and we took the stairs leading to the long hallways of the hospital. After we told another woman in floral scrubs working in the imaging department who Erik was, we sat in comfortable faux leather chairs and attempted to get Erik to use his "inside voice" to let the man across from us continue to sleep. I panicked when I realized Stinky Dog had been abandoned somewhere in the building and made a note to myself to locate him after the study. Thankfully, Erik did not notice his absence.

We were finally led to a tiny waiting area separated from a glossy hallway by a saltwater fish tank. I made myself comfortable in the same chair I sat in while I was miscarrying my first baby. The same chair I sat in after I lost my second baby. And the same chair I sat in a handful of times when the doctor wanted to take a look at Erik inside of me to make sure he wasn't leaving my body like the others did. I felt a twinge of anxiety when I sat down, which was quickly replaced by a horrible craving for Chinese food. That tank with all of the fish bobbing in it has the same effect on me. Every time.

A boy of about 12 sat in the corner quietly playing a handheld video game. His mother held a computer on her lap and played solitaire. They talked about another family member who was apparently hospitalized. Erik approached them and said hello. He asked them both if they vacuumed, and the woman laughed and said that she did but that she wished her son did it more often. She introduced her son and asked Erik's name. After a few minutes of conversation, she looked at me with the very same slightly confused, intrigued look on her face I have seen many times on faces after Erik has gently held a stranger's hand and looked deep into their eyes.

She asked, "Is he always this happy?"

I smiled and told her that he was most of the time. I enjoy the reaction Erik gets from strangers. He's different, but at this point, people can't seem to put their finger on exactly why.

A friend or family member came into the small area with an update on their loved one, who apparently was hungry and wanted to sit up and eat with them in his room. Erik approached the newcomer and held her hand gently. She smiled at him and answered his questions about vacuum cleaners, looking to us periodically for a translation. They then all left us to eat lunch and wished us a nice day.

An ultrasound technician came out to get us and lead us around the corner into the small, dark room I am so familiar with. I hopped up onto the table by the machine and invited Erik to join me. He immediately began to cry. When the technician's attempts to calm him down failed miserably, I explained he was missing the gene that controls anxiety. I then gave him a crash course on elastin and renal artery stenosis in Williams, and he nodded his understanding. He mentioned another connective tissue disease, and I felt comfortable with his response. I finally was able to hold Erik still while his back was smeared with clear, warm jelly and the ultrasound wand was held closely to his body. Brian made faces at Erik, and I pressed my face into Erik's hair. Images were successfully obtained of the inside of Erik's lower abdomen. Erik was obviously very anxious, but the tears stopped. He repeatedly asked if we were all done. I told him no but promised him chocolate when the study was finished. When the technician saw the bladder and asked Erik if he needed to go to the bathroom, Erik was obviously deeply insulted. I giggled. Any reference to using the potty makes Erik very irritated.

The technician left us and let the radiologist examine the images while I went into the little dressing room and fixed the wild hairdo I had been afflicted with from holding Erik down on my side for so long. The tech eventually returned and reported that everything looked healthy with no evidence of stenosis or stones. Erik was asked if he wanted a sticker, and he said that he did not. He was already talking about locating the car. We left the department and found our way back down the hall. Stinky Dog was sprawled atop the registration desk waiting for us. On our way back out of the building, I ran back into the heart center to pick up Erik's heart-shaped foil balloon, which was still tethered to a chair in the nurses' station. I gave the staff a thumbs up and said I would be talking to them soon.

We exited the place out into the crisp air and sunshine, knowing all was very well.

At least for now.

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Tuesday, February 17, 2009

Imaging Study

Erik is scheduled for another blood pressure check with the cardiologist and a renal ultrasound at the hospital tomorrow around noon. Not only that, we are getting some blood work for the kidneys. Amazingly, I didn't have to suggest either of these things. I also did a little research and purchased a home blood pressure cuff (for the whole family to enjoy at this point).

For those of you who are curious, his systolic reading yesterday was 142, which I agree is "pretty high," as it is not even close to being listed on my chart for a boy his age and height. His diastolic reading was 70, which falls into the 95th percentile and is also a little high. His cardiologist says his pressure has increased over time.

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Monday, February 16, 2009

Hypertension Part Deux

I took Erik in for a blood pressure recheck today, and it was once again too high. When I asked how high, I was told, "Not extremely high, but it's pretty high."


Pretty high?

Please be more specific. Like, explode your eyeballs high or experiencing mild road rage high?

After I was given a very polite brush off and told I could come back in yet another month, I explained that I have watched Erik's WS peers undergo surgery, have strokes, and take antihypertensive medication. I explained my fears that we have not gotten very accurate readings in the past and that I suspect he has been hypertensive for a while. I was told "not to worry too much."

I calmly explained that I wasn't "too worried," but there is certainly a lot I don't know about what's going on in Erik's body, and my alarms have been going off. I explained the issue of renal artery stenosis and the fact I have never seen images of his kidneys. They haven't even done all of the recommended lab tests on him since his diagnosis, despite the fact I gave them a list of them. I recently gave them the list again. Although I adore Erik's pediatrician, I would give my right arm to have someone anywhere on the West Coast who could tell me what the plan should be and reassure me that Erik was in good hands. I'm sick to death of being told I'm the "expert" by physicians who admit they know next to nothing about this syndrome. I have a sneaking suspicion she knows very little about elastin arteriopathy, which 100% of people with WS have, or the high incidence of renal artery stenosis. See, my kid is 4, and his arteries are already hard. Sure, the the last couple of readings could be nothing, but color me a little concerned and in need of more information. I don't believe I am overreacting in the slightest. I was told they would start a referral to the cardiologist. I thought that was a bit over the top but decided I would at least like to speak on the phone with a specialist. Just for a little friendly advice.

I called the children's heart clinic here and identified myself to the woman who answered the phone. She brightened when she heard Erik's name, and I found myself smiling. The cardiologist is going to call me tomorrow, and I hope she can tell me what the best next step should be. I can certainly wait another month for another reading. I can schedule him immediately for a scan. I can stand on my head and sing "Sweet Home Alabama" in my brassiere and panties. I don't care what the plan is. I would just feel better being told what to do for once by someone who uses an authoritative tone, not the faint, buoyant whine of question marks and a pause for my response.

I just want someone else to take the reins for once.

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Sunday, February 15, 2009

Party Cleanup

"Don't forget to laugh!"

-- Sue Vest (Mother of Jeremy of HYN)

So the most amazing thing happened.

I woke up in so much pain I wanted to chop my throbbing noggin off, but my headache completely subsided after its brutal reign of three and a half days. I was suddenly able to clean the house and cook some appetizers for the "How's Your News" party. After I was done, I slipped on my brand new blue and yellow HYN football jersey, which somehow made it all the way from the bowels of MTV in New York to my mailbox in just three days. I primped myself until I looked fairly lifelike, and most of the people I invited showed up at our door right on cue. We sipped bubbly champagne, feasted on delicious food, and gathered around the television to watch last week's season premiere of "How's Your News." I was quite pleased to be surrounded by friends, family members, a member of my moms' group, and Erik's therapist from early intervention who is now a permanent part of our family.

And the show? I loved every second of it.

I tend to analyze everything to death, but I was instantly entranced. I didn't analyze the content or the format while I watched it at all. I immediately fell head over heels in love with the HYN interviewers. I didn't think about Williams syndrome, Down syndrome, or any neurogenetic condition. I didn't even focus on the wonderful, rare opportunity I had to gaze into the strangely familiar faces of strangers marked with the elfin features I have learned to recognize as I have studied my son's syndrome over the years. I simply found myself immediately comfortable, and I laughed loudly along with the others in the room without a second thought. It was soon clear that the show seemed a heck of a lot less focused on disabilities and more on the experience of being human. About watching for the potential bizarreness that occurs when two very different people happen to collide and communicate. I did end up hungry for more sound from the HYN band and hope to see more music in the next few episodes. Jeremy's enthusiasm sparked by meeting the drummers from various bands and the unexpected gift of a guitar that Brendan, another young man with WS, received from pro skater Ryan Sheckler left me feeling very warm and fuzzy indeed. And that Susan! Well, she's just frickin' FUNNY. I loved them all.

After the show, it was suggested that I play the 60 Minutes video titled "A Very Different Brain" about Williams syndrome, so I popped it into the DVD player. I was anxious to see it again, knowing we would recognize some of the people we met at the convention. We all had a great discussion about WS, "How's Your News," disabilities, our culture, and how far our family has come. We pointed out people we had met or read about. We laughed a lot. Some of us even cried a little.

I remember feeling confused and a little sick to my stomach when I heard the term "Williams pride" for the first time at the convention last summer. I wondered if anything so damned devastating could ever spark a sense of happiness or pride in me. Sure, it sounded good, but I was skeptical. Packing for the convention felt a little like preparing for a funeral in a sense to me. It meant I had to accept something I didn't want to and officially let go of the old hopes and dreams I had forever. Attending the seminars on WS meant shedding the soft scraps of denial I clung to for comfort. In reality, it scared the hell out of me. However, as the convention progressed, I was transformed by the entire experience. Walking through the front door of the hotel for the first time and watching the young people with WS lovingly envelop my son in their arms brought tears to my eyes. It was the most amazing, unexpected sight to see. He held each of their hands and laughed along with them, like they shared some sort of secret joke and had known each other forever. As time went on, I took a really good look at the people around me and realized we were indeed celebrating WS. In a big way. There were T-shirts for sale to prove it. I witnessed gallons of grief-heavy tears shed around me by parents a lot like me over a few days, but when they dried, there were a lot of grins. In the end, I came back a different person. My own tears finally ceased, and I came home wearing a smile.

Today was a real celebration for me. I didn't realize it, but it ended up being another milestone. I was really excited and proud to be the mother of someone who just happens to have Williams syndrome, a devastating but amazing genetic twist of fate that has brought so many wonderful people into my life and reinforced the relationships with the people who were a part of my life before the diagnosis. These are the people who sit on my couch and drink champagne with me, helping me celebrate the beauty in something that racked my whole soul with grief three years ago. These are also the people who are always with me, even if they are miles away. I certainly wouldn't want to be on this roller coaster alone, and they have willingly strapped themselves in next to me, ready for the crazy ride. Once in a while I put my arms in the air and scream loudly, sounding halfway terrified but oddly triumphant. It's exhilarating. I felt like that today.

Three years ago I thought I would never laugh again.

I couldn't have been more wrong.

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Party Day

My "How's Your News" party is today at 3 p.m. I wish my head didn't hurt so much.

Friday, February 13, 2009

Wear and Tear

I'm getting to the point in my illness where I am at least going through the motions. I work out. I put on makeup. I wear real clothing that isn't made with fleece or Spandex. But I continue to feel like the walking dead. My head has been pounding for two days straight.

Erik seemed to be limping around the kitchen island yesterday. I told him to stop, and I sat him down on the floor. I turned the bottoms of his bare feet up to examine them and gasped. Not only is the skin on his toes so thin that it wears away on a regular basis, the skin covering his soles had worn out from simply running around the house. This has never happened before. In fact, it was so thin that his feet were reddish-purple and almost hot where they had been in contact with the floor. His soles looked like they were about to burst, and I hurt just looking at them. He hadn't given me one word of complaint, but he didn't protest when I covered him in shoes and socks. I have to keep shoes on him as much as possible to avoid the grisly bloodbath that occurs when his skin does disintegrate. Although it wears quickly, it heals just as fast underneath a layer of protection. On top of everything, he remains covered in eczema where the skin is a little thicker. His arms and legs feel like the surface of a freshly-plucked chicken, bumpy and dry, no matter what preparation I use or dietary change I make. Neither condition has improved in the slightest with time. Although I continue to go through the motions with greasy balms and lotions, I have really given up fighting it, as it doesn't seem to bother him or his doctor.

His skin just seems so fragile. And he seems so darned tough.

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Monday, February 09, 2009

Out Sick

The whole town seems to have a bug of some sort. Erik has recovered sufficiently to go to daycare, and Brian and I are fighting it now. I was in bed all weekend. I am itching to write SOMETHING, but unless I describe my wacky febrile hallucinations, I don't have much to say. While this is tempting, as it might prove entertaining for some, I fear that exercise may reveal a bit too much about myself for my own comfort.

I'll be back soon. I promise.

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Tuesday, February 03, 2009

The Sound of Mucus

Random Thought of the Day

Scent of Eucalyptus = Yummy

Scent of Eucalyptus + Poopy Diaper = Nasty Stench

Erik is sick with his first chest cold. I'm not feeling so hot, either, and have been up since 1 a.m. with my usual anxious, angry insomnia. We both smell like Vicks VapoRub. I did manage to indulge in two independent films while the guys were asleep during the dead of night, and that was almost fun. Erik and I eventually went out to drop off work and go to the store, and I bought some Ben & Jerry's Cherry Garcia frozen yogurt, which is something I used to do years ago when I was in a foul humor. I wandered down the cleaning aisle and hoped to find a scented candle that struck my fancy but was too picky/olfactorily challenged to select one. I ended up with a very bizarre collection of items in our cart and couldn't remember exactly why we were there. Erik said hello to another family while we were out. They seemed to know him, but I had no earthly idea who they were. Being accustomed to his celebrity status, I let him do the talking. Later on he asked the very quiet, cranky man checking our groceries to give him a high five, which he did, much to Erik's delight and my surprise.

We did get to enjoy a family Superbowl gathering last weekend. The neighbors also joined us for some cocktails, burgers, chicken wings, and friendly gambling. Erik stood motionless in front of the television screen while Jennifer Hudson sang the National Anthem. Every once in a while a song will really seem to touch his soul. I find him in front of the stereo from time to time in a trance. Each muscle in his body seems to freeze. It is extremely unusual for Erik to be still while he is not unconscious or being held down. When the song was finished, he turned around smiling and said, "Again!"

I might need to purchase the Dreamgirls soundtrack.

Life drags on. I don't have a lot to report. My friend with ALS has been receiving IV antibiotics at the hospital for yet another lung infection, and he has horrible allergic reactions to the drugs. Erik has learned to say, "Watch this, Mama!" My feral cat and I spent Erik's nap time cuddled on the love seat this afternoon, but sleep never came for me. I'm off to drug my body into resting, and I hope tomorrow is better. I'm guessing Erik will take advantage of a rare sick day and stay home from school and therapy. We'll do this all over again. We are grouchy but really enjoy each other's company, in sickness and in health.

Erik and Brian, Ready for the Big Game

Rob, Lisa, Me, Mom, Bob, & Susan Enjoying Things in 3-D

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