Thank God for Perspective!

We woke up Christmas morning this year to the stomach flu at our house. Before the Bible could be read (we start every Christmas morning cuddled in bed reading the story of Jesus' birth) or the presents from Santa could be opened, this first "gift" was upon us. Much to the 5-year-olds frustration she had to wait for Mommy and Giraffeboy to feel well enough to start the morning's activities, and as soon as the last present was unwrapped Mommy went back to bed. There was no going to the big family party at my Aunt's house. I barely registered how cute Giraffegirl and Giraffeboy were opening their presents because I was trying to just make it through without being sick, and really it didn't feel like Chirstmas to me. But I believe that as in so many other times in my life, God blessed me with the gift of perspective, and left me grateful for the Christmas we had. A few days before this Christmas we got an email from our pastor asking us to pray for a family she knew who had just lost their wife/ Mother. This dad was left to raise two young kids, ages 4 and 8, on his own. In addition, 5 days before Christmas I attended the funeral of a close friend's husband, who lost his battle with cancer. My cousin celebrated her first Christmas since her son, age 15, died. So sick or not, as I looked around my room as we curled together watching a movie that afternoon, I couldn't help but realize that I was surrounded by everything I needed on Christmas, and that I was blessed. We are still working on getting better over here, but we are on our way, and we've had plenty of bonding time to enjoy each other as we've been homebound.

This isn't the first time that I've been blessed with perspective. My sister has a close friend whose little girl, Maryah, was diagnosed with ewing-sarcoma bone cancer at age 9. Maryah was amazing- strong, brave, inspirational, and her battle with cancer, including multiple remissions, went on for over 6 years before she died at age 16. Just watching someone so young fight such a hard battle gives you perspective over the things going on in your own life, but in our case, it gave us an even more important perspective and was a blessing. During the years of her fight, my son was born and diagnosed with marfan syndrome, followed by my daughter being born and diagnosed. It seemed that with every new marfan "issue" that came up in my family, my sister would call with the news that something worse was going on with Maryah. I remember vividly the day we got the news that my daughter had marfan syndrome. I was shocked, heavily grieving her perfect health, and I called and cried with my sister on the phone. It wasn't a half hour later that the phone rang. My sister, in her tear-filled voice, told me that Maryah's cancer was back. Suddenly I wasn't grieving my daughters perfect health, but was instead thanking God that she could LIVE with what she had. That wasn't the first time that happened with Maryah and our situation, and it surely wasn't the last, but it lives in my head as a reminder. And don't get me wrong, I would gladly give back all the perspective for Maryah to never have had cancer, for her to be alive and healthy. But I recognize this gift she left us all with.

 Maryah

There are other ways that the gift of perspective has been a blessing in my life. I mentioned earlier that my friend's husband died this year when he lost his battle with cancer. I've watched as he went through the ups and downs of fighting cancer for nearly 10 years, including the periods of remission. I've seen the struggles of dealing with insurance, medical appointments, and so much else. I have another good friend who is fighting cancer too, and I see the struggles she and her family face. And because of our own experience with marfan syndrome I can empathize with the financial strain they go through, and with the insurance struggles and other things. And because of so many situations I've been through with my extended family and friends, I have seen how quickly someone can die, how we aren't guaranteed to live to old age, and how each day is a blessing. When I see these friends fighting these battles I truly realize that it could be me or my family instead. I think of the quote "there but for the grace of God go I", and I am grateful for that grace. It's one more way the gift of perspective has blessed me.

Taking Things Away

I was ok until his chin quivered. Up until then I had rehearsed it in my head. What I needed to say. Why I needed to say it. How he would react. But that quivering chin as he tried to hold in his emotions did me in.
Parenting is hard, whether your kids have special needs or not. There are times in every parents life when you have to be the bad guy, when you tell your kids "no" to something that they think every other kid gets to do. We've been through this many times with both our kids, and though it is sometimes hard, it's a parenting right of passage that we all face. It's just a part of life. When your kids have a medical condition the number of times you have to say no is increased, and there are things you have to say no to that most parents don't need to think about. We plan around these potential "No's", avoid starting things that we may later need to take away, but that isn't always possible. My little marfs didn't come with a handbook, and there is no official list of forbidden activities they must avoid.
The past few weeks have been really hard around here, so much so that it has taken me awhile to process it and come to a place I could write about it. I've talked in my blog before about my son being diagnosed with aspergers syndrome, and as such he has a hard time making friends, and often spends his recess time alone. We found out he was playing gaga at recess. It is the first real activity outside that he has interacted with the other kids. For most kids, it wouldn't matter that they played gaga, a modified form of dodge ball where the ball is supposed to stay on the ground and is batted around with the hand instead of thrown. For my son, whose lenses are already partially dislocated, and whose aorta is enlarged it represented a threat, a serious potential injury, a risk too high to take. So Giraffedaddy and I talked and planned and sat down to have the conversation with Giraffeboy that he could no longer play. We didn't even really make it through our first attempt at the conversation. We outlined the risks, told him we thought he should stop, and when he asked to leave the table we let him. There were no tears from him that day. That was the weekend.
He came home Monday after school and mentioned playing at recess again that day. We couldn't put it off any longer. We needed to know he would no longer do it, that he really understood it wasn't ok. So before bedtime stories we started the talk again. Same outlined dangers, same information, but this time it came with a definite "you can no longer play". And partway through the conversation I saw his chin start to quiver. This little boy, 8 years old, rarely very emotional, so easy going and brave, and I lost it. As I saw the first silent tears rolling down his face, I no longer saw my 8 year old in front of me. Instead I saw that tiny baby, so hoped for, prayed for, wanted, with the same quivering chin when he would start to cry. The tiny boy that from the moment I knew I was carrying him I had sworn to protect and love. It's no wonder I couldn't stop myself from crying. And when that little boy, the brave 8 year old with his silent tears and quivering chin said "I'm sorry mom. I didn't mean to make you cry." I reassured him that I was crying with him, not because of him. I was mourning with him the loss of something that I understood he loved. They were tears of sadness for the opportunities he would never have that most people take for granted. For the unfairness of it all. And for my own failure to protect him from this disappointment.  
In the midst of this there have been numerous blessings. Giraffeboy played for more than a week before we were aware that it was happening, and although there have been multiple injuries daily in the nurses office, he never got hurt. I fully trust that he now understands he can't play, and he has always been responsible enough to follow the activity rules we set up (the ball was supposed to stay on the ground, so in the mind of an 8 year old gaga wasn't off limits). I have no doubt he will follow this too. The number of people who have reached out to us as we have filtered through the aftermath, who have offered advice on how to proceed with making sure the school would understand the seriousness and come up with a way to keep him better protected in the future. The friends who called to offer an ear, or whose shoulder I cried on as I struggled with finding a way to keep him safe. My special needs Moms bible study ladies, whose experience dealing with IEPs for their own kids offered so much help. And our faith, on which we relied on for guidance through the whole situation. The truth is that in the end we aren't willing to keep him in a plastic bubble, so there isn't a foul proof way to keep him safe. But after IEP meetings and new plans, we are relatively confident that this won't happen again.
In the end, none of this was about me or my feelings. It's about the baby boy, with a quivering chin, who has grown up way too fast and is an amazing little boy, who holds my heart in his hands. This Mommy couldn't be prouder of who he is becoming.

 

 

We need a new Role Model

It's that time of year again, when the season is changing from summer to fall, when the kids are getting back into organized sports, and when they play Kenny Chesney's "The Boys of Fall" on the radio every five minutes. It's my favorite time of year to be outside, but it is also the time of the year when I need to mentally pull myself out of the "what might have beens" into the "be grateful for what you haves".
There is something about seeing the facebook posts of friends at their kids' saturday morning soccer games and sunday morning football games that feels a little like a stab at my heart. It's not a heartbreak that my kids don't have enough options, that they will miss out exactly, although there is some of that too. You want your kids to have every choice in life. But there is also a camaraderie that happens between parents at the sports field that you miss out on if you have kids who can't play sports. I see it in the weekly class update letter from giraffeboy's teacher, where she references being like the rest of us heading to our kids' weekend sports events, and in the faded relationships my kids and I have with families of kids from preschool that have gone on to other elementary schools, that have been easier kept up with other sports families. There is an extra effort that needs to be made by us sportless families that I admit I'm not often good at making.
Then there is the final straw for me, the thing that makes my blood boil and drives the point home each year, the Farmington Homecoming week, complete with autograph sessions during elementary lunch with the football team and cheerleaders. I realize it's tradition, a rite of passage that happens for these high schoolers that has been done for longer then people remember here, but I hate it. I hate that my son comes home excited, and that instead of sharing that excitement I can only think "that will never be you". I hate that we celebrate these athletes in our town, but ignore the academic all-stars. I wish we'd relook at what it means to be a hero, and what is worth "stardom".
I wish instead of athletes we'd have the kids come to give autographs that have succeeded in high school against all odds. Bring me the kid who grew up without parent involvement that is excelling, the kid with a mental disability that is beating all the odds. Bring me the kid with aspergers, who despite a natural lack of understanding of basic social skills is thriving. Bring me anybody, really, for my kid to admire that they can relate to. Because no matter how much they might want to, or I might want them to be able to, they will never be that high school athlete. And it doesn't matter. They are perfect just the way they are. I just wish the rest of the world would learn to appreciate them and their fellow "sports-challenged" classmates a little more often.

GiraffeGirl goes to Kindergarten

I'll preface this with the fact I wrote a similar letter to giraffeboy the night before he started kindergarten. I decided to share the letter to Giraffegirl with everyone, since I know every parent feels the same mix of excitement and dread as their babies start school. I hope everyone has a great first day of school!

 

9-03-2012

Dear Giraffegirl-
Tomorrow after lunch I will lead you onto a bus and watch it drive away as you head for your first day of kindergarten. And once again another huge milestone passes as you start your adventure of your school age years. You could not be more excited, talking about seeing your teacher and making new friends, but I find myself more nervous, and a little sad, and worried about how it will go. There are so many things I have no control over, and I wonder if Daddy and I have prepared you enough to tackle this new adventure. School can be a scary place, full of bullies and cliques and everything else, and there is no way to completely protect you from all of it.

There are so many hopes I have for you as you start school. I hope the excitement you feel about learning today continues on throughout your life, and that the smart, silly, sensitive, and creative young girl you are now grows up to be a smart, silly, sensitive, and creative young woman. I hope you continue to think of everyone as your friend, that you continue to see the best in everyone, as you do today. I hope you continue to view the world as a lifelong lesson in learning and that the journey never ends for you. And I hope that on this learning journey you find something that you are so excited about, so passionate about, that brings you great joy, and that you are one of the lucky ones that can turn this passion into a career. I hope you see success as a part of the journey, and that you measure it by how you feel, what you learn, and how you treat others, not by any monetary standard or by the possessions you do or don't have. That is the true measure of success.

One of the things I love most about you at this age is your individuality. I love that you'll mix patterns with stripes, that more color is better (matching or not), and that you don't notice or care what others think about it. I love that you already admire your big brother, and that the two of you have such a great bond and love. I feel like you will be there to protect each other in school, that you each already have a built in friend. I hope as you get into school you continue to follow your beliefs, that you are less concerned about whats "cool" than what's right. I hope you keep the belief that it is ok to be different, and that you are able to realize that the kids who would tease you about it are the ones with the problem. I hope that you do find a group of friends who understand and accept you for who you are unconditionally. Friends will change throughout your life, but I would love it if you found a lifelong friend this year that you could carry with you through all the changes that lie ahead for you.

I hope that as you venture out into the adventure of the school years that you keep the childlike optimism you have now. I hope that as you learn how cruel the world can be, that you continue to view it as hopeful, that you look for the positives in life, and that you appreciate the beauty in the simple things around you. Happiness lies as much in attitude as in circumstance, and so I hope that when life gives you lemons you'll make lemonade.

I hope that you continue to build your relationship with God, that you aren't afraid to share your beliefs, and that you show your classmates God's love through your words and actions. I love listening to you pray each day, and hearing you sing to God, and I hope that you continue to build that relationship and to trust in him.

One of my biggest fears as I send you off is how you will deal with your marfan syndrome, and with your challenges when it comes to physical education. I worry about you being teased for not being able to play contact sports, for not being able to shoot hoops or play floor hockey with your classmates. But I recognize that the kids who would tease would find something to make fun of regardless, and that being teased is a universal experience everyone goes through while growing up. I hope that when faced with the choice to make fun of someone to fit in or to walk away, you'll choose to walk away. Real friends will respect you for this decision, and anyone else isn't worth your time.

The most important thing you need to know in this new adventure, as in everything in your life, is that your Daddy and I are so proud you. We know that great things lie ahead for you, and we believe that you can do anything you set your mind to do. When we wished for a little girl 6 years ago, we had no idea how much joy and how much personality you would bring to our family. We couldn't have imagined how much we could love you, how silly, sweet, smart, and compassionate you are. I couldn't have imagined that a second child would make our family feel so complete, and I had no idea how it would feel to have pieces of your heart walking around in two separate little bodies. But I know now, and it's amazing being your mom. I will always be able to close my eyes and see you as that tiny newborn attached to all those wires in the NICU, so when I want to hold you tighter to keep you safe, you might have to remind me it's ok to let go. You've turned out to be the strongest little girl I've ever met. No matter what you become, what adventures you take on in this journey called life, we will never love you less that we do right now, with all our heart & soul & mind. We can't wait to watch you conquer the world and to travel this journey by your side. Learn well baby girl.

I Love you all the way to the moon and back,
Mommy

Our Marfcation- AKA NMF Conference 2012

If this past weekend was a Visa ad it would go something like this:
Conference registration fee for 2 adults- $600 (thanks MN chapter for the scholarships!)
Cost of 3 nights at the Pennisula Hotel (conference rates) - $620.
Cost of Parking in Downtown Chicago for 3 days- $82.00.
Being surrounded by hundreds of other people who have connective tissue disorders or raise children with connective tissue disorders - PRICELESS!

Many of my friends and family asked me how conference was, or have asked me to describe the experience. I've struggled to find the words to do so, maybe because the experience is so unique, and it is so rare in this world to encounter people as openly accepting as the marfamily. But I think it is more than that. How do you explain to those closest to you how lonely it sometimes feels to raise children with this condition, and how sitting in a room with a hundred + other marf mothers for an hour who are walking this road with you can momentarily make you forget those lonely times? How you don't even have to say your struggles out loud, but when you do you can look around and see the tear stained faces of so many others who have faced them too? How do you explain the experience of spending three days with your heart bared open and your walls down, on emotional overload but not wanting the time to end either? How do you tell them that a complete stranger can understand what you go through better than they who have been there year after year? How do you describe the healing power of being with people who are empathetic to your situation, not just sympathetic? How do you describe the indescribable?

This isn't my first NMF conference I have been privileged to attend, but it is the first one I got to be at fully with no responsibilities. Our first one was in 2009 in Rochester, MN, and as part of the MN chapter it seemed I spent as much time with "behind the scenes" things as I did enjoying the experience. I spent Saturday at that conference with the kids instead of attending general session and the medical workshops. On Sunday I even led (really badly, mind you) the session for unaffected spouses that year. It was a different experience all together for me, and I spent so much time trying to make sure I didn't forget any responsibilities I forgot the most important thing about conference that year- making connections.

I didn't miss the chance this year. Before conference had even begun I had met at least one marf family in our hotel swimming pool, and we had walked the streets of Chicago playing "who's a marf" (be honest, we all did it). Day one, I got a chance to get to know some of the other chapter leaders from around the country. They are an amazing group of men and women, and I left the leadership session inspired to get moving on some new ideas. At dinner that night I got to better know some other parents of marfan kids, as well as a few of the teens and young adults. Day two, I had the chance to learn about some of the newest research, and I was excited to learn that they are studying how marfan affects pregnancy, giving us hope that some of the dangers may be gone before giraffegirl is old enough to consider starting a family. Day two also gave me the chance to meet the famous Dr. Dietz, as well as some of the other top marfan doctors. I also met many other parents, including two moms from our home state of MN who are relatively close by and have kids my children's age! And day three I got to participate in the emotional sessions, and got to talk honestly about the experience of being a marfan mom. It was all amazing! I can't count the number of people I met and the connections made.

It wouldn't be right to blog about conference without talking about the very best part. It happened before the pediatric orthopedic session on Saturday. I was waiting for it to begin when I heard my name. It took a minute to recognize her, but when I did I couldn't believe she was there. The wife I spent the long day with as our husbands had aorta surgery in February of 2010 was there. I hadn't seen her since we had left the hospital, only talked to her via email a handful of times, but the bond made those five days left us family. It was amazing to see her and to finally meet her husband. Even my husband, Mr. Shy Guy, really liked them (I kept forgetting he didn't know them-it just seems so weird because she played such an important part of my life- what was he thinking sleeping through all that?!) It was wonderful to see them, and to reconnect and catch up on their family. We will forever share that special bond, and have that lifelong friendship formed so quickly. It was the best surprise ever!

It's been two days since we returned home from our trip to conference. Two days of the normal routine, but I feel anything but normal. I've been busy, finding people I met at conference on NMF Connect, making sure that the initial connections I've made remain. I still feel a little raw emotionally, but I think there is a part of me that wants that to stay. I don't want to put my emotions about marfan back in the little box I keep them in. I don't want to put on the false smile of everything is OK that I can put on in an instant. I don't want a pity party, but I do want honesty. I don't want to have to smile and celebrate with my friends their children's athletic accomplishments without feeling able to express my sadness when registration time comes around. I don't want to bury feelings because I feel the need to protect those around me.

When my husband first went to conference 3 years ago, at the age of 33, the experience of being among so many other marfs healed a part of him that was still suffering from the teenage years experience of feeling different. We vowed at that conference we would do whatever it took to get our kids to conference during their teenage years, whatever the cost, wherever the location. Seeing the magic that happens between the marfan kids is worth it to us, especially the teenagers. Until then we'll go when it is doable, when it isn't too far, when we can afford it. I do think that the teenage years are the most important time for kids to go, but I'd say that for parents, the years now, as we are starting to negotiate our children's school plans and preparing for the future challenges, are the most important time to go. I feel incredibly lucky and blessed to have had this experience.

The MOSKS

I forgot my bag of wonders when we went to giraffeboy's eye doctor appointment the other day. The kids are getting older, and I wrongly assumed they didn't need me to bring so many distractions for these appointments. And as I sat there in the waiting room waiting for the drops to dilate his eyes, I recognized a kindred spirit across the waiting room, who gladly shared some books with my kids, making the 2 1/2 hour appointment more bearable. Once again, I was so appreciative of the bond shared with other moms in my position.

We're the MOSKS, or Moms Of "Sick" Kids. You can recognize us in specialist's waiting rooms across the country. We're the Moms whose kids go to the doctor more often than they go to the mall, whose kids have entire pages in their address books for the numbers of all their various specialists. We're the ones who drive long distances, sometimes even flying across the country, in order to get our kids into the right doctors who know our kids' disorders. Were the ones who can tell you our kids' days off from school next year before the last school year even ends, because we're trying to plan our kids' appointments around their days off, so they miss as little school as possible. We network online with other parents whose kids share the same condition, hoping to give our kids the best life and medical treatment possible. And we are the ones who aren't afraid to tell a complete stranger about our kids' conditions, hoping to spread awareness and also change how the world sometimes sees our children as "different."

I've never met another MOSK at the doctor whose kids had marfan syndrome, but I have learned of so many other challenges a child can face from my conversations with them. Our kids have heart problems, eye problems, orthopedic problems, or a combination of these. They have down syndrome, muscular dystrophy, heart disease, and any number of other medical conditions. Although varied in their challenges they face, they are all amazing. They have learned over the years to be patient, and the waiting rooms we've shared have not been their first, and certainly won't be their last.

You can recognize a fellow MOSK in a waiting room with a quick glance around, especially at our eye doctor. While many of the parents bring their kids sans toys or entertainment, my fellow MOSK's have their bag of wonders in tow. The bag is simple: books, coloring supplies, snacks, our kid's favorite toy. But for the kids who visit these places frequently enough to be bored with the doctor's toys, it is essential. And for the parent whose kids visit once a year or are new around the place, it isn't necessary. But for those of us who have a membership card to the MOSK group, we visit frequently and have learned to adapt to make the trip bearable. And somewhere along this journey to diagnosis and treatment, we've lost the need to hide from each other what our kids face, and have instead learned to let down the walls we so often build and honestly discuss our kids' conditions.

Which brings us back to the other day. I looked over and saw the mom and her daughter, bag of wonders in tow, and struck up a conversation. We talked about her daughter's eye condition she was born with, I shared about the eye issues my kids face from their marfan syndrome, and she generously shared some of the bags contents to entertain my kids. Chances are, we will never see each other again, as I have rarely run into the same MOSK a second time. But I am grateful for the knowledge I've gained from other MOSKS over our many conversations, and the reminder that the challenges my kids face are like those of so many others. I didn't ask for a membership card to this club, but I have enjoyed meeting some of the various other members, and gaining a knowledge and appreciation for the challenges they've faced.

Sandals

My husband wore sandals today. We took a road trip around Lake Pepin, enjoyed the nice weather along the river, and during a stop for cheese, beer and ice cream about half way around I realized he was sockless in sandals. I didn't say anything when I noticed, afraid I would ruin the moment, afraid an innocent comment would send him back into socks and shoes indefinitely. So when he finally acknowledged it as we got nearer to our house. I was overjoyed and proud of him.

So what? I know most of you are asking that question right now. So here's the deal. My husband is 36 years old. I have been with him since we were both 19, and not once have I seen him wear sandals when it didn't involve water. Even then, it was only in the last 5 years that he traded in water shoes that fully covered his feet for a pair of sandals. My husband has hammer toes, a trait that my kids are slowly showing signs of, that leave his toes an intricate weaving, resembling a work of art. They are beautiful, interesting, uniquely his, but to him they have always been something to hide, a source of difference in a world of straight toes. He has always been amazed by my feet, not in a foot fetish type of way, but in the way you marvel at something you covet. So to see his feet exposed to the world is a huge thing.

I can't say I don't get it, that need to hide the parts of you that are different, that people might notice and exclaim over. I have always been overweight, and I'm not talking about a few pounds, and the initial trip into my swimsuit every time I've gone into a pool has always been a dreaded experience for me. Once in the water, I'm able to let it go, but the walk from the safety of a cover-up to the edge of the pool or beach seems like miles to me. But I do it. I love to swim. And more importantly, I won't let my weight stop me from enjoying the water with my kids, who love to swim too. So I get it, I really do.

For my husband, marfan syndrome has also left him with a slightly protruding sternum, and so in addition to his foot sensitivity he doesn't like to wear thin shirts, or things that cling to his chest. Getting him to swim with the kids has been a work in progress, and I'm glad to say he's finally reached a point where he does it without a fight, and enjoys it too. Someday, maybe he'll even reach the point of comfort where he can swim without a shirt, but that doesn't really matter to me. Being in the water with us is enough.

I don't think I truly got the big deal about his chest protrusion until recently. Maybe I'm just used to it after all these years, and it's just another feature that makes him who he is. But a recent experience helped me get it much more. I was with his aunt waiting for a minor surgery, and the anesthesiologist was doing a medical history preparing to do her job. "What's this?" she asked, poking his aunt's protruding sternum. It was so insensitive, so thoughtless, and unnecessary. And yet I realized in that moment, it was not the first time she had been asked that way, that it was probably an experience that many marfs, including my husband had faced. And it suddenly made sense why he was so sensitive about it. How else would you react to being asked that throughout your life? Who can blame a person for hiding the things that make them different from others when that has been their experience?

Which takes us back to today. We talk often about the need for both of us to find acceptance of ourselves, so we can teach our children to love themselves for who they are. Since so many of the things that might visibly differentiate our kids from their friends are related to their marfan syndrome, much of the burden to set that example falls on Giraffedaddy. And today, one more wall was torn down. one more victory in the fight for self acceptance and self confidence was won. And this wife couldn't be more proud of the amazing man she's married to.

A Mother's Intuition

One month ago I sat at a meeting with various members of our school's special education staff and my husband, discussing the social situation for my son at school, and the possibility of testing him for social disorders. The meeting stemmed from my son's spring conference, where his teacher told us that giraffeboy had "no friends". I asked if it was possible to look into this at the conference, but as I sat at the meeting and listened to the school psychiatrist describe his brief observations of my son, I already knew what they would find, and I wondered again about the difficulty of being a mom during the tough times.

To me, "Mother's Intuition" is that ability that we as mother's have to know there is a problem before anyone else does, or to perceive what our child needs before they tell us. It varies from the basic packing snacks and drinks before we leave the house to stave off our kids hunger (and before you argue that this is common sense, when was the last time your husband packed the kids snacks or beverages when you didn't have to tell him to?) to knowing something is wrong in your gut before someone says something or something happens. It is the second kind that is the tough times, the kind that you know will differentiate your child yet again from what is "normal", the kind that will lead to the revelation that something happened to your child that was bad, or the kind that means you will start on another long journey. What makes this kind hardest is that they come with things you can't "fix" for them, problems not easily solved, and they leave you feeling like an inadequate parent. They leave you with the "what if he'd had a better mom?" kind of questions that none if us like to ask ourselves. 

I can distinctly remember the first time that my "Mother's Intuition" resulted in a whirlwind of events and I wondered to myself "what have I done?" My daughter was just over a day old, and as I sat holding her after our last visitors of the day had left the hospital, I remember thinking "something isn't right". The best way to describe it was that she was wheezing.  So I called my nurse, and they took her to the nursery to check her out better. Fast forward less than an hour and my full term daughter had been admitted to the NICU. There they hooked her up to a litany of machines, wires everywhere, and her three days in the NICU had begun. Thankfully for us, when all was said and done it was just fluid left in her lungs that didn't get pushed out during birth because she was a c-section baby, and my daughter was just fine. But those three days of waiting for an answer while she endured various tests were some of the scariest days of my life.

There are times in life too where my "Mother's intuition" has failed me. My daughter's diagnosis of marfan syndrome is the one that stands out the most. I wasn't prepared for her positive genetic test, and it hit me hard when we found out. I really had no idea, and I really thought she didn't have it. Even when it comes to this situation with giraffeboy, I always just assumed he'd be the kid whose happy with only a few close friends. It wasn't until he got into kindergarten that I began to really worry there was more to it than that. And even then it took me more than a year to really acknowledge what I'd begun to suspect and push for the testing. So it's definitely not a foul-proof sense for me, or at least I don't always listen to it.

Which brings us back to yesterday. I sat again at a new meeting table with many of the same staff at our school. The results of the month of testing and survey filling-out were back. And the results were what I suspected all along. My son has an Autism Spectrum Disorder. It doesn't change who he is or how I feel about him. It doesn't change how much I love him. He's still the kid who is so smart, whose smile can make my entire day, who gives spontaneous hugs and kisses, and who will randomly say "I love you mom" to me for no apparent reason. He's high-functioning to be sure, but it's still there. One more challenge he will face. One more road block he'll have to overcome. And I will be there with him every step of the way making sure he's not alone, making sure he knows how much I love him. I don't have all the answers to help him right now, but I will be doing everything I can to get them.

After all, that's what a "mother's intuition"is for. It's the horn that rings in our heads and hearts to warn us that we should prepare for battle. And even if our "enemy" changes every time, being a mother means we'll fight the war no matter how big the problem. We'll fight it until we find a solution, fueled by our best weapon, our unending supply of love.

Marfan Disney World

We just finished the first week back to school after spring break. My friend's Facebook accounts are filled with pictures of their fun vacations taken, and as I've been dreaming of someday being able to afford the trip to Florida or some other exciting place, the bills have come in for our yearly vacation to what I like to call Marfan Disney World.
So what is Marfan Disney World, you wonder? It's a place we visit for a day every February, the four of us trekking the 30 minute or so ride anxious for the days adventures. The whole time spent there is less than 4 hours, but the cost per child is about $750 dollars (this year). The money spent there wouldn't quite cover a week at the real Disney for us, but easily two years worth would.
Marfan Disney World isn't at all like the real theme park. You aren't guaranteed a fun time, and if it "rains" it's hard to remember the good parts of it. But if it's sunny, there isn't a better feeling as a parent than leaving it on a sunny day. On a sunny day, you get to let your guard down a little and just live for a few hours. You almost forget the list of cant's that come with marfan syndrome on the sunny days. You know the ones- the same ones that would make a ride on the roller coasters off limits at the real Disney World if we ever made it.
At Marfan Disney World, there aren't rides, but there is a movie, an incredible, mesmerizing movie, a movie of each of our children's hearts, that I watch each year in nervous anticipation, waiting to see what the results of what this movie tells us are, and wishing I knew how to read the measurements taken myself. So far, the kids are more interested in watching whatever cartoon or other show they can talk the technician into putting on the big screen, but us adults in the room barely notice that show.
The wait after that show for the main attraction is the longest wait of the year for Giraffedaddy and I. We sit in a exam room, reading stories to our children, offering silent prayers to our God that it's a sunny visit.
And then she arrives. She wears a business outfit covered by a lab coat, her stethoscope draped around her neck, her smile radiant as she greets the kids and us, regardless of the type of news she is going to deliver. There is no princess dresses involved here, but she's such a better role model for my girl anyways, and sometimes she shares that coveted stethoscope with the kids. There are no song and dance shows, no special princess teas, and instead of wearing a princess dress Giraffegirl gets to don a child size hospital gown, but she loves it just the same. She checks over each of our kids, listens to their hearts, and then it's time for what we've all been waiting for: The Big Discussion. On a simple white notepad with a picture of a detailed human heart in the background she jots down the measurements and the z-scores for the two kids.
We've been blessed so far. On our worst year Giraffeboy's aorta grew about .3 cm and his z-score topped off at about 2.83. To an untrained eye, these numbers mean nothing, and 3 millimeters seems like a nominal measurement, But if you factor in that a normal adult aorta is less than 3 cm, and that the kids were at around 2 cm when we started these measurements, suddenly millimeters matter. And the most simple way to define a z-score is to just say it is a standard deviation measurement that lets us know how far the kids are away from what a "normal" aorta size is for their height and weight. Anything less than 2 is considered in the normal range. We covet a number less than 2.
This year was the most sunny year yet. Giraffeboy's aorta stayed the same, and for the first time since we started these trips to Marfan Disney World 7 years ago, his z-score was just under 2. Praise God! And Giraffegirl's aorta stayed basically stable too, and her z-score was down from 2.39 to 2.3 this year. So we have so much to be grateful for. It was an exceptional visit this year.
We talk with the doctor a few more minutes and then were headed back home, our yearly trip over. Instead of souvenirs we leave with prescriptions for the coming year, our main weapon in combating aorta growth before next year's visit. We spend the rest of the day together as a family, have a rare meal at a restaurant, and we laugh and love. It's been a good day.
So as these bills come, and as I write the check out of our HSA account to pay for this year's trip, I reflect that somewhere, someone else had a rainy trip today, someone else had no need for the trip because their child died from this syndrome, and still someone else struggles to figure out how to pay this bill each year. And I dream of a day when this visit, a necessary part of a person with marfan syndrome's yearly physical, is covered by insurance. And I say a prayer of thanks to God for this year's sunny day. Some day I hope there is enough money in our budget to pay for the trip to the real Disney World, but for now I'm grateful that the money is there to pay for this trip. For now, it's enough to enjoy the real Disney World through the eyes of my friends, and to reflect on how lucky my family really is.

Two Year Anniversary

     Two years ago today GiraffeDaddy had valve sparing aortic root replacement surgery on his ascending aorta. We are grateful to God everyday for that surgery's success, and for the relatively quick healing that followed. Although there are still times he doesn't have quite the stamina that he did pre-surgery, he is pretty much back to normal.
     Instead of writing in detail about that day, I thought I would copy and paste the series of emails I sent out during our week at the hospital. They describe so much better what that week was really like.

Monday, February 22, 2010 1:07 PM

Thought I would send a quick email and let everyone know E is still in surgery. The actual surgery started about 11:30. We are anticipating they will be finished around 4:30 or so. Thanks so much everyone for your thoughts and prayers!

Monday, February 22, 2010 4:24 PM

Hey everyone. Just heard from the nurse coordinator and E is off the heart/lung machine and they are closing him up! Sounds as if everything went well and it was a success. I'll send an email later tonight after I've talked to the doctor and I've seen him, but he's doing great! Thanks so much for all your thoughts and prayers!

Which brings us to a prayer request....my Mom and I have been in the waiting room with another family who's loved one has Marfan Syndrome and is having his entire descending aorta grafted in today. He is only 33 and has a young child. Please pray that this surgery goes well for all of them! It is a much more severe surgery than Eric has just had. Thanks!

Tuesday, February 23, 2010 7:48 AM

 

Hey everyone-

Sorry I didn't email last night but it was a late night. Quick update. E is doing great! He had a little trouble waking from anesthetic, and so he didn't get the breathing tube out until about 5:40 this morning. I was here with him until about 1:30 this morning, when he sent me out and told me to go sleep. He is in good spirits but is definitely feeling some pain. He really likes his drug button! They will be getting him up into a chair sometime soon, and then he'll move to his permanent room for his hospital stay (he's in the ICU right now.)

As far as the other family. Thank you for praying for them! N, the guy, finished the main part of his surgery last night about 10:30, and it was so fun to see the celebration with his wife M and his parents and siblings! There is a long recovery road for him but the hard part is done!

Please conitinue praying for both these guys as they deal with the pain and recovery!

Thanks so much for all your well wishes and emails! They feel like great big hugs buoying me up during this LONG past day! (We got up yesterday at 4:45, and I am NOT that 20 year old college girl anymore! I don't do all-nighters!)

Tuesday, February 23, 2010 1:30 PM

Hey everyone-

Wanted to let you all know that E is now moved out of the ICU and into the cardiac care unit where he'll remain until they kick him out of here. He is in unit 5C, room 5688 in Mary Brigh Builing at St. Mary's Hospital. I'm not sure how to call him, but at this point he really just needs quiet time to heal anyways, and you all know he is not really a phone guy anyways. At this point, while he is in pain and still getting back on his feet and germs are still a concern we need to minimize visitors, but know that I am giving him all your messages and sharing with him how many people care and are concerned. If you are thinking about visiting, talk to me first and we'll see where he is at and how he's feeling. Just know we are both so thankful for everyone's prayers, thoughts and concerns. There is no way to reply to everyone's email but just know that I am so thankful for them and they have really helped to boost my spirits! I love you all.

Tuesday, February 23, 2010 9:37 PM

 Hey everyone-

Just a quick update before we try to get some sleep. E is doing well, although he is having some anxiety about the pain and how hard this recovery really is. He's also very worried about the kids having to go through this surgery in the future. He is in a lot of pain and he is also scared of what is to come in this recovery process. (let's be real- we are scared!) I have to admit this is way harder and worse than I thought it would be, but I am also really thankful that E is alive and doing well. I can't imagine going through this without all the knowledge we had ahead of time, as so many people who never knew they had Marfan Syndrome until they reach critical aorta dilation do, or without a faith in God that it will all turn out alright. I came back down tonight to stay with him (Thanks for the ride/ car delivery Pastor Pam and Marshall!), and I am probably not heading home for the next few days. Mostly, I just don't want him left here alone. But we are both doing good overall. It's been a rough couple days. Please keep the prayers for less pain and a quick recovery coming!

Wednesday, February 24, 2010 9:29 PM

Hey everyone-

Let me start this by acknowledging that maybe we should have gotten a caringbridge site when it was offered and given everyone a choice about whether to read these updates or not. We chose not to because we really felt those should be more for the families of people suffering though long term illness that would need long term updating ability. I figured I'd send out one quick email saying surgery went fine and he's good and that would be all. I didn't realize how hard the recovery process would really be (I know, so naive of me, but our friends who have had this surgery really made it sound quick before hand, or at least that's what E and I chose to hear, and though I know this is still really soon after surgery I don't think I really GOT it before hand.) At any rate, if any of you are sick of updates feel free to send me an email and I'll take you off the list with no hurt feelings. Or just delete this.

I was so frustrated with my email account before this started because it wouldn't let me make a list, and I had to individually get the emails onto that first email, but I'm also incredibly thankful since it made me add myself to the email and I'll now have all these emails I can print out as sort of a journal of this experience, and maybe it will help the kids in the future. 

I am so thankful for a much better day for both of us. It does seem to get easier each day, and I've heard from many that day 3 and 4 post surgery are the worst so I'm prepared for tomorrow and looking forward to those easier days!

E's pain control was so much better today! He got his chest tubes out this afternoon, which was a very painful (but short!) experience, but they were right in saying the pain levels were reduced almost immediately. He's been up walking 3 times today (our goal was four, but the nurse came in and gave him a back rub right before his planned last one and so he just wanted to sleep then, and who can blame him). He's pushing himself farther each time, and he's been pretty steady and strong once he's on his feet. The getting up and down without his hands is the hard part. With his long lower legs he has to push up even further since his knees are above his waist, so this makes this even harder. But he's making great strides in mastering this. You would think they would have special furniture designed for tall people here since they specialize in aortic surgery for those affected with Marfan Syndrome here, but no such luck. I'm just thankful that E is only 6 feet or so, so that foot to knee length isn't longer.

Again, we are both very thankful for all the prayers and words of support! We love you all!

Thursday, February 25, 2010 10:48 PM

Hey everyone-

    With any luck this will be our last update from the hospital. I'll send out at least one more when Eric goes home tomorrow, as a sort of end to this part of the healing process.

    Today has been a day of celebration for us. E walked 5 times, sat up from the bed to standing twice, got to take a shower, survived on less pain meds (I'm starting to feel like a drug pusher as I encourage him to take some), lost another IV (leaving him with only one) and most importantly, passed stool tonight and was rewarded with actual food! So there have been large strides today and I am no longer scared about bringing him home, as much more of his physical care was done by me today then nurses (they still won't let me give drugs and have weird fascinations with his output).

    There were some emotional highs today too. My Dad was wonderfully kind and drove the kids down today with my Mom so they could see Daddy, and they did great and boosted both mine and E's spirits. It was hard though to watch them leave, and I had a good cry after I watched my babies go. With any luck we'll all be together tomorrow. Then, our good friend T from our Marfan Group came, who had the same surgery about 2 years ago, and he and E were able to compare war stories. I saw the first real spontaneous smile from E since surgery, which was immensely joyful. Both visits were great for our spirits.

    Life in the hospital strips away all dignity from the patients. I remember after having the kids thinking the entire world had now seen my parts, but it was nothing like the lack of privacy E has experienced this week. At least with child birth there is a baby to distract everyone in the end. And the nurses are great at trying to protect his modesty, but there are only so many things they can do with a gown that opens in the back and no undies. There is an intimacy here with E that I have never experienced before, and although I would have spared him the pain of this journey I appreciate the outcome of it. There is something beautiful in caring for the person you love's most basic needs. I have helped E do things this week I would never have thought I'd need to, and yet I would have done a million more things if it meant this same outcome. I helped him shower today, and as I gently washed his scar I thought how it represents one more victory in this war we fight against this disorder that affects our family. I hope he will someday look at that scar and not see it as something to hide, but rather something to celebrate, a badge of bravery in this step he took, experience that may one day help our own kids. This man I married showed me a whole new level of  his bravery, endurance, character, and love, and I know I leave here more in love with him than ever before. This week has been sort of like one big date for us (not sure E would see it this way, but really he takes me on walks and I get hospital ice, what more could a girl want!), a constant opportunity to connect, to let down the guards we so often put up, and to just enjoy the quiet together. I don't think I shared this, but one of the first things E did as he woke up from sugery Monday was sign "I Love You" to me, and though my tired self took awhile to get the message, it represents what this whole week has done for both of us, reminding us to take that extra minute to say those words, to let each other know we appreciate the other, to remind us to lift each other up. In these years of young kids where we sometimes forget to put each other first, it's been a great experience for both of us. In a strange way I'll miss this hospital time.   

    Enough of my ramblings. Thanks again everyone for all your prayers this week and for all your great messages. We are incredibly lucky to have all of you in our lives!

    And just to update today, N is doing fine but still in intensive care- much more serious surgery. He and his wife and M found out today that their son's aorta is also enlarged,  meaning he probably also has Marfan Syndrome. Please pray for them as they grieve the loss of perfect health for their little boy at the same time trying to heal N.

Saturday, February 27, 2010 10:58 PM

Alright everyone-

    I said I'd send out a final email when he came home so here it is. Sorry it didn't get out last night, but by the time the kids went to sleep last night it was all I could do to keep my eyes open long enough to crawl into bed! I just wanted to acknowledge I didn't mean to make everyone cry in the last email, but I wanted to share what this experience had given us, and I wanted it in here for the kids someday when I give them this email record. I hope they find the unconditional love that E and I share.

    Friday morning at the hospital brought a final visit from Dr. Schlott (I think that's his name), the only constant in a stream of otherwise varying doctors on Dearani's (the surgeons) team. He was our daily wake up call, the around 6 am visit with a friendly smile and fabulous accent (think Prof Bomshad from college, Matt, and yes I was smitten with him!) The rest of the morning was filled with a visit from Dearani himself, real food for E for breakfast, a final echo to double check E's heart repair, and at least 3 lectures on recovery after leaving the hospital. We also took one last walk around the unit (without the walker this time!) to make sure E really felt ready. By about 2 pm we finished all the paperwork and lectures and it was down to get drugs and then on our way home. Although Pastor Pam brought us down lots of pillows to cushion E's ride (thanks!), macho man wanted nothing to do with them and by cannon falls it was back down to just the car seat. He swears the bumps didn't bother him, although his heart pillow did cushion the seat belt. The kids were ecstatic to see him, and after hugs he went up to bed and spent much of the night there. When I asked how those first set of steps went, he didn't remember taking them, so they obviously aren't a problem (big surprise, I didn't help him cause I had to pee after the long ride and he was too quick and impatient to wait the 10 seconds!). It was fabulous to come home to a clean house, and I was so thankful for my Mom for how hard she worked while watching the kids all week! E did come back down for dinner and then spent the rest of the night watching tv in our room.

    My first hour home involved building a Lego helicopter (thanks Uncle Sean and Aunt Amber) with giraffeboy and making baby diapers out of sanitary pad wrappers for giraffegirl (you use what you got, I guess!) so it was back to reality quick for me.     

    I'd say today seemed like a normal day, and it pretty much was, but E was tired much of the day and spent a lot of it in our room. The kids were in and out visiting him, and he and giraffeboy spent tonight watching a Lego movie we got at the Library today (I left him home to run there and to Target for groceries, with the cell phone next to him and a promise I'd leave the cart and rush home if he needed anything!). The best part of being home is the change in E's moods, as he is so much happier now that we are home, much more back to his old self. I know this recovery process will take time, but I finally believe the people who said he be almost normal after a week. It's amazing how fast the human body recovers.

    Life isn't fair. E didn't have this surgery due to any decisions he made in life, any choice of lifestyle he had. The genetic syndrome that made it necessary was passed on at birth, in the same circle of life in which we passed it to our kids. But this week has been a constant reminder to me of all I have to be thankful for. In the intensive care unit that E was in Monday night still lives a 10 month old baby who has undergone 3 heart surgeries already for deformities in her heart. Next door to him Monday night was a 3 year old who was recovering from her own heart surgery. N, the other Marfan surgery on Monday, left the ICU finally today, and his recovery will be so much longer than E's is. My Mom watched as a family was taken down from our waiting room that wasn't getting good news, and though I don't know their story I know it could have been us. The girl who comforted me Tuesday night during my waiting room cry, after E opened up about his fears for our kids, was there in the hospital watching both her parents die. Life in the hospital can quickly show you how bad it can get, but it also shows you the triumph of the human spirit, and the best of humanity as we put aside our own troubles to help another in their time of crisis. I have seen more compassion and kindness shown to strangers this week than I had in a long time, and it renews your belief in the goodness in the world we live in. I don't think I will take life for granted nearly as much as I have in the past. Each day is a gift, and I plan to spend it thankful for what I have, not sweating the small stuff, taking the time to appreciate the little things. Thank you for all of your support this week, for all the email that boosted my spirits, for all the offers to help with anything we needed. It's wonderful to feel so loved during all this time. When E is up to it I'll share them all with him. I know he will appreciate them as much as I do. God has truly blessed our lives with all of you and I am very thankful for each of you. I am glad the worst of this journey is over. Unless something major happens, I won't bore you with the details of our life now that we are home. And now I'm off  to bed to listen to the beautiful sound of my husband breathing as he sleeps........  

I just wanted to end this post today with a quick acknowledgement that while we celebrate giraffedaddy's health two years later today, we also mourn the loss of my cousin Christopher, who died this past week at the age of 15. Life is short. We never know when it is our time, so take the time today to tell your loved ones how you feel. We love you and will miss you Christopher!  

The Short Bus

My daughter rides the short bus. And she cries on the days when she doesn't get to, when I drive her to school or a friend picks her up. I cried the day we found out she was eligible to ride it. Although we had fought to get her help with speech and occupational therapy through our school district for over a year, when she finally qualified I was torn between happiness and worry. Happiness that she would get help, and that the strain of the $100+ monthly preschool bill would be gone. But also worry that labeling her as special ed, even at the preschool age, would affect her future years of schooling, possibly negatively. And I was torn about sending her on the bus. Would she be safe? Would she ride with bigger kids? Would she be made fun of for riding the "short bus"?
I grew up in an era where there was much less understanding of differences (I like to think the world has improved in this, that it has grown more open-minded as I have grown), where terms like political correctness weren't yet invented, or at least weren't common. I am ashamed to admit I was as guilty as everyone else of tossing around jokes with my friends about having to ride the short bus. I don't remember even having kids at my school who rode the short bus, they definitely weren't mainstreamed with us at that time, and I don't know how I first learned the term. But at least in Minnesota it was one of those terms growing up in the eighties you just knew.
Fast forward to now. As I said, my daughter rides the short bus. In the end having her ride the bus wasn't really an issue, and she was so excited to ride that how could I say no? My son goes to school with all types of children, and at least for a portion of everyday he has kids with many types of learning difficulties in his class. There are children at his school with down syndrome, autism, aspergers, chromosome deficiencies, ADHD, ADD, and these are just the ones I can name off the top of my head I know about. My children have friends at school with cerebral palsy and cystic fibrosis, and of course, they themselves have marfan syndrome. At  5 & 7 my children have been exposed to a larger variety of people's challenges than I had even into my teens, but my kids don't really see it or see it as anything to pay attention to. That is the beauty of children, their ability to accept differences in appearance and physical abilities without passing judgement or caring. It is through adults and society that they learn to pass judgement, and I can't think of a better way to help them be open minded than to have them in this diverse environment, to answer their curiosities with facts, and to allow it to be their normal.
My son rides the regular bus to school, and he has since the start of Kindergarten. It wasn't until the beginning of first grade that I learned he had the nickname booger boy the whole kindergarten year from a kid at the bus stop who saw him pick his nose. Although he has friends on the bus, just like back in the days we went to school there is a definite pecking order on the bus and you can see it even in elementary school at the bus stop. He's not afraid to take the bus, has been lucky enough to not have encountered much bullying so far, but I know through friends that it still happens too much. I see his bus driver everyday, but she's never made an effort to get to know us parents. It's just business on that big bus.
And that's where the short bus is magical. My daughter's bus driver introduced herself the first day last year when she picked her up. Every day she stops to chat a few seconds as giraffegirl makes her way off. We know about her grand kids down south, funny stories about her husband and family. When she saw us at the State Fair last summer she came running up to give giraffegirl a big hug. It was one of giraffegirl's favorite parts of the fair when we talked later at home. When it is still warm and the windows are down, as the bus drives by each day to turn around to drop her off at our driveway, I can hear them all singing and hear the little kids giggling. It's a beautiful place, that short bus she rides. And the best part- no pecking order. There aren't the cool kids and the dorks, the nerds and the jocks. The kids who ride her short bus this year all face a struggle, whether physical or learning disabilities, and they share that common bond. They stroll past the "regular" kids and their parents who make up half their preschool class, and they excitedly run to their bus with the teachers. And like my daughter before she was special ed, many of those "regular" kids wish they could ride the bus. Heck, I wish both my kids could ride that bus through the rest of their school years. And I can't help but think what a better world we would live in if we could all experience the magic of the short bus once in awhile.

Choices, Part 2

I watched the movie "In My Hands" for the first time about a year ago. The movie is a documentary about marfan syndrome, and it tells bits and pieces of a number of people and families' stories that are affected by marfan syndrome. The first time I watched this movie, I was with the other three officers of the Twin Cities Chapter of the National Marfan Foundation and we were all seeing it for the first time. Through most of the movie, the only sound we heard in the room were each other's tears. Although all of our stories are vastly different this movie was deeply personal for all of us, and touched us all in ways both the same and different. We all finished watching and felt a need to debrief. A counselor would have been handy at that moment. If you've never seen the movie, and have any interest in learning more about marfan syndrome or living with a medical condition I strongly suggest you see it.
Although I don't think there is a boring minute in this movie, there are two parts of this movie that really resonated with me, that haunted me long after the movie finished, and both of them in a sense revolve around the issue of guilt. One of them is when a mom of a marfan child talks about sometimes just wanting to walk away from her child, her husband, everything, just to escape having to deal with marfan syndrome. So here is a secret. I've thought that. I've been that mom. There are days when it seems overwhelming, when the kids are facing something new, when giraffedaddy has some new unexplainable symptom or pain, when I'm faced again with one of the many things we have to tell our kids "no" to in order to keep them safe, and I think wouldn't it be nice to just run away. But I don't, I wouldn't, I couldn't. But even admitting to the thought of it leaves me with a feeling of guilt that I technically could, a freedom none of those I love most have.
The second part of the movie that continued to bug me was a small statement made by a doctor describing the passing on of the marfan gene. I don't remember the exact line or wording, but the gist of the statement was that for most people with marfan syndrome they are able to make a choice to risk passing on the marfan gene, but for the families where spontaneous mutation occurs (25 % of marfan cases)  they have no choice. Technically, there really isn't anything wrong with that statement, but emotionally my mind fought what it said. Did we make a choice to pass this on? Do all those affected by marfan syndrome make that choice? Would anyone faced with that "choice" even call it that? I still disagree with how it is worded. It isn't that simple. I didn't make a choice to pass this on to my kids, and neither did my husband. Yes, we knew the statistics, we knew the odds, we knew the 50/50 chance. But we made a much simpler choice. We made a choice to love whatever child God gave us. Before we ever became pregnant we talked about all the things that could go wrong with our child. We talked about the possibility of down syndrome, about whether or not the muscular dystrophy that his cousins had could happen to our child too. And we decided that no matter what, we would love our child exactly as they were. So yes, we made a choice, but it was a choice to love, not a choice of passing on health issues. I firmly stand by the fact that a person with marfan syndrome having a baby makes no more a choice to pass it on than a mother over forty could be said to choose to have a baby with down syndrome. Is the risk higher? Yes. But deep down you never really believe it will happen, you optimistically believe your baby will be healthy and live a long, happy, uncomplicated life. And so many of the best things in life involve risk, so why is this any different? The truth is, it isn't. Marfan syndrome, no marfan syndrome, my kids are who they are meant to be. And if it was a choice we made to pass it on, then I'd make the choice again in a heartbeat to have the joy these kids have brought to our lives. It far outweighs any challenges that come along with their diagnoses.

Passing It On....

Long before this blog existed, before I knew what marfan syndrome was, I was just a girl in college who met an interesting boy in college and started hanging out and emailing. It was in one of those emails, during our pre-dating friendship, that giraffedaddy first told me he had marfan syndrome. A few minutes with a search engine on the library's computers had me convinced my friend was going to die any minute of this terrible condition, and I was so scared and heartbroken. A phone call later with assurances that his case was nothing like what I had read had me calmed down and laughing at my overreaction. His case was nothing like what I had read, more a pseudo-marfan syndrome. Yes, his aorta was dilated- but only a little, and he didn't have any of those other things that I had read. Perhaps at the time it would have been wise of me to read a bit more of what I had seen on the web, to have done a bit more research on what he had, but I trusted what he'd been told by doctors all his life and never questioned what he told me. I knew he had a family history of this, but no one in his family had ever had any real complications due to marfan syndrome- no surgeries, no bracing, nothing to fear.
So that college girl and college boy dated, got engaged, got married, and got ready to start a family. Giraffedaddy was nervous about having kids, about the potential to pass this on (a 50/50 chance), but I was adamant it wasn't a big deal. What's to fear? I'm ashamed to admit I even told too many family members that if I had to choose between my kids having marfan syndrome or struggling with my weight issues, I'd pick marfan syndrome.  Based on what I knew then, it seemed like the easier choice to a girl who'd been teased for her weight all her life. And for me, having kids was a lifelong dream, and that included carrying them, so it didn't really seem like a choice to me, more like a need.
So after a long time of trying, and a visit to a fertility clinic we were finally waiting for the baby to arrive. Visits to the prenatal geneticist, level two ultrasounds, and then the big day arrived. I have no memories of Giraffeboys' birth, since I was knocked out for an emergency c-section, but I can distinctly remember the first time I saw him, how beautiful he was, and the magnitude of love I felt for him from the start. And one look at his long skinny body, his long skinny fingers and feet, and we knew without a doctors confirmation that we lost the gene lottery, and our boy would have marfan syndrome. And quite frankly, we didn't think it would be a big deal.
Fast forward two and a half years to the birth of giraffegirl. We always knew we'd have a second child, and there was no hesitation to get pregnant again. This time I was awake for the c-section. I remember the first glance I had of her, of thinking she looks so different from her brother, a rounder face and body. She was just as beautiful, I loved her just as much, but there was a relief in me that maybe she wouldn't have to deal with marfan syndrome. Eight months later, after a genetic test done as a precaution by our geneticist, who herself expected the test to be negative, we got the diagnosis that she too had inherited the gene. I grieved that day far more for her than for her brother, probably because I had falsely believed in her health for so long.
It was because the kids had marfan syndrome that we first went to a meeting of the Twin Cities marfan group. I thought it was important to learn more, to meet other parents, to find some support for our kids as they got older and dealt with some of the self-esteem issues that their father had faced because of his marfan syndrome. We walked in that first day and actually said our family had a pseudo-case, nothing to worry about. It's amazing they didn't laugh us out of the room that day. Because we got involved in the end of 2008, as the group was preparing to host the 2009 NMF National Conference at the Mayo, we got drawn in to the conference excitement and attended. It was at that conference, during the Saturday doctor's presentations, that info was distributed about what aortic dilation was needed for preventative surgery. A glance at his measurements from his last echo told us that giraffedaddy was already over that number, and led us to search out a different cardiologist who knew more about marfan syndrome, and who got us scheduled for giraffedaddy's heart surgery 5 months later.
So you could say having kids, passing on this gene, saved my husband's life, or at the very least made for a much less serious surgery. He never would have gone to that meeting if it wasn't for the kids, and we wouldn't have thought to go to conference, or possibly even known about it had we not been involved with the group. We would have gone on naively believing our family's case was mild, with nothing to really worry about. Fear not, we have long quit being those naive people. We now know every case of marfan syndrome is serious. Every person with it needs to have echos and yearly check-ups. We are thankful that so far the kids cases fall somewhere in the middle of the severity spectrum, with no huge concerns thus far, just small challenges. We pray daily for consistency, for no aorta growth, no serious complications. And we take things as they come.
I believe that God played a role in getting us to conference at that exact time, when giraffedaddy's aorta was quickly nearing a critical measurement. I don't think it was an accident or coincidence. Too many things fell into place for us to get there. From the scholarships from the NMF and our local chapter to cover costs, to the nearness of the location, a mere hours drive from our house so we could skip the hotel costs, so many things fell into place to make this work on our limited single-income budget. And I'm grateful everyday for the people we met, the information we learned, and the chance to proactively treat his aorta before a dissection could happen. I know were some of the lucky ones, and our story could easily have a different ending.

Choices

Today I went with my sister to the St. Paul School's Open house at the River Center. Like my youngest, giraffegirl, my nephew will be entering kindergarten this coming fall. Today was an opportunity for us to talk to people from all the elementary schools in one setting, to compare and contrast both the positive and negatives of these 40 or so public school choices. We started the day with a list of five or six schools that were possibilities for her to consider. We talked to the staff that was there from each school, asked questions and spent much of the day overwhelmed. By the time we left the event (over 4 hours after arriving) we had tossed out some schools from her list, added one new school to the list, and narrowed her choices to 4 schools, each with pros and cons. Does she choose the school with the staff that was obviously incredibly passionate about teaching and the principal everyone raves about, but has less perceived parent involvement? Does she send him to the school in which she loves the feel when she has been there, the school he is most familiar with from taking community ed classes, even though the school is predominately white and my nephew is biracial? Does she send him to the sole St. Paul school that has a year-round schedule, which offers science daily in the classroom and lots of diversity, even though it would eliminate some of his summer vacation time with his cousins, and everything she has read says there is no benefit to year round schedules in elementary school? Or does she pick the school with the greatest quoted diversity, but which she is least familiar with and hasn't really felt a draw to before? How do you make that choice? And is there really a bad choice among them? She isn't done with her decision-making process, and has a little over a month to go tour the schools and to possibly attend a PTP meeting at each to see about parent involvement. We've grown up in a family that believes that you can get a good education wherever he goes, that parent involvement and student motivation is more important than the physical building he attends school in, so regardless of where he goes I think he'll end up okay.
The whole experience left me extremely reflective though. I started the day so envious of my sister's choices. We live in one of the farthest out suburbs of the Twin Cities, and we have only one elementary school our kids go to. There isn't a choice for public schools. What a magical opportunity she has to mold her child's educational experience. I expected to be blown away with all the options, and to come home wishing I had stayed in the city where I grew up, instead of following my husband out to his place of origin. And I did see an amazing amount of options, was blown away with all the choices and the difficulty one has making that decision. I don't have a clear idea of what my sister should choose. But somewhere after we left this afternoon, as we over-analyzed her four options over lunch, it dawned on me. Had it been my children I had been searching for a school for, the questions and criterion would have been different. We would have focused less on diversity and how they split up specialist time (gym, music, art, science). Our number one concern would be how they'd be prepared to care for two children with special medical concerns. We'd ask questions like "Have you heard of marfan syndrome?", "What is the break-down of the recess area and will there be a safe place for our children away from balls and impact sports?", and "How flexible will the gym teacher and classroom teacher's be in adapting the curriculum to our children's needs?" Our choices would be less about the options than about our child's safety, less about the dog-and-pony show and more about the general flexibility we felt from the staff and principals. And the repercussions if we would choose wrong seem so much greater. Would they suffer emotionally at a school where differences were less socially accepted, regardless of the flexibility of the staff? And how do you really tell what school would be most open to that, especially when what your children suffer from is rare, when you don't know parents locally who have pioneered this road before you? How do you chance it? How do you live with yourself if the choice you make is wrong? Suddenly I think home schooling may have been a more realistic option for me, that I may have chosen to keep my kids in my own safe cocoon for a long while more.
I'm grateful today for that lack of choice. As I said, we had one school to send our child to if we wanted transportation. So I arrived at that school without another choice, asked those same questions above anyways, and learned that the answer to the first one was "No". Our school hadn't heard about marfan syndrome and had no prior students with it. But then something magical happened anyways. My initial conversations with the nurse happened the spring before my son arrived. For his kindergarten year we met with the teacher and explained his condition, filled in the nurse, and since there was no gym that year, felt safe with the "no balls" rule for kindergarten that they implemented. I felt safe for that year but so fearful of what would happen once gym started. We wrote up his 504 medical plan, with the necessary accommodations for marfan syndrome needed for gym and recess (no high impact sports, shooting hoops, or other things where he had a high chance of getting hit in the head with a ball or getting hit in his body. No forced running, rest when he feels he needs to). And we started the summer wait before first grade started. And here's where the real magic happened. I read about people's school experiences with marfan syndrome on NMF connect and other marfan blogs. I somewhat expected a battle for my son. Instead, we met before this school year began and were greeted by a staff that had not only researched marfan syndrome but had discovered it qualifies as a disability in Minnesota, automatically qualifying him for special education services in our state in only one area (you normally need 2). Pretty soon they were helping to advocate for what is best for our son, and the majority of the fears I had disappeared. Really, what I've found in this school that wasn't our "choice" is more valuable than a million options at a hundred other schools. I've found peace-of-mind, security, a caring staff, and my son flourishing. It doesn't hurt that our school happens to be in the top 40 schools in the state for test scores and other things they compare. But it isn't perfect. I crave the diversity my nephew will get in his school he goes to. But the grass out here is green enough for me, I don't need the other side. Today I was reminded that sometimes having no choice is what I still choose. Sometimes, having choices is overrated.

The Journey Begins

For years I've talked about starting a blog about my family, about the joys and challenges giraffedaddy and I face raising two kids with marfan syndrome while dealing with his health issues too. I've even considered what I would write, who would want to read it, and what was the pull to do this in blog form instead of a personal paper journal? The answer...I don't completely know. But then I had a particularly bad November. Giraffegirl had eye surgery, which was not completely successful. Giraffeboy got foot orthotics for the first time, and suddenly something as simple as boots and snowpants for a 7 year old that he could navigate himself over the orthotics became a gigantic obstacle to surmount. And mostly, I felt really alone in a sea of people with perfectly healthy kids. And then I read a entry from a blog that a friend posted on Facebook from the mother of an autistic boy. Her challenges were nothing like mine, her victories for her child were completely different, and yet the connection to her over raising "different" kids felt so tangible to me. You could say that was the final kick in the pants I needed to start this blog. If in the course of posting our experiences it helps even a single person feel less alone in the world, then it is worth my time. And if while doing so I can help educate even just my friends and family about marfan syndrome, and the things I don't generally talk about, then all the better.

It seems wrong to have a first post and not talk about what marfan syndrome is. Marfan syndrome is a genetic condition that affects about 1 in every 5000 people. It is a connective tissue disorder, and since every part of our body is made of connective tissue it's affect on a person can be widespread. The most dangerous possible symptom for someone with marfan syndrome is an enlarged aorta. Undiagnosed and unmonitored it can lead to an aortic dissection, and then death. To make it even more challenging to diagnose and treat, it affects every person differently, so that no two cases of marfan syndrome are exactly alike. In our family, the kids are more affected then giraffedaddy. Giraffedaddy had a dilated aorta, but had his ascending aorta replaced in February of 2010 at the Mayo Clinic (He was dilated to 4.6 cm when they did the surgery). He suffers from joint pain (especially the hip), and since surgery he has developed temporal lobe epilepsy (more on that a different day). He has pectus carinatum (his sternum protrudes out). He has no eye issues from his marfan syndrome. He has flat feet and hammer toes, typical things in affected people. Giraffeboy was diagnosed by us when he was born, but had this confirmed when he was 18 months old. His aorta is dilated, his lenses are dislocated, and he has incredibly flat feet and is losing flexibility in his heels. He recently started wearing orthotic AFO's to help increase his heel flexibility and to provide ankle support for proper joint placement. We are hoping this helps to minimize pain he may deal with as he gets older. Giraffegirl was our surprise case of this, as she seemed unaffected when she was born. She also has a dilated aorta.She has pectus excavatum (her sternum is sunken in).  She has lazy eye, which she recently had surgery to help fix. Although her ability to pull her eyes back in from wandering has improved, her tendency to wander has not, so the surgery was not completely successful. She also has orthotics, but for her in the daytime she wears SMO's, one of which is trimmed down to the top of her shoe, and the other slightly higher. She has a special orthotic at night she must wear to increase flexibility in her left ankle, which goes up to her mid-thigh. She has had orthotics since before she could walk, and although she doesn't always wear them she has always had a version of them since about 18 months. She also has hammer toes. In addition to all of this she is our slow developer. Every childhood milestone she took her time with, and was the last of her friends to really walk, talk, etc. Although there is no positive proof connecting this to marfans, I share other parents belief that it is related to her condition.
And with these words, let this journey begin. I'm so excited to see where it takes me!