You may have noticed that a few weeks ago, the Lariviere-Cayer clan became local celebrities. If your definition of "local celebrity" includes a 25 second segment on the NH news channel wherein the news anchor gave Andrew my last name AND called me Justin, that is.
You can view the longer piece that the Boston Channel aired here. It's definitely worth a look, mostly because Gracie steals the show, as usual.
It was considered a "human interest" piece on our families experience with VHL. What is VHL?
VHL is a rare, genetic disease. In normal people, the VHL gene is a tumor suppressing gene. In people with VHL, this gene is mutated or incomplete in some way, causing the body to form tumors in various parts of the body. These tumors are not cancerous, but can cause various symptoms & complications depending on where they are located.
VHL has been featured on medical dramas such as House (of course), Private Practice and most recently, Grey's Anatomy. Which is how we ended up on the news. The VHL family alliance sent out an email, asking its members to write letter to their ABC affiliated stations regarding the misrepresentation of VHL on Grey's Anatomy. So I did. Within a week, I had been contacted by assignment editors from both WMUR and WCVB.
(Side note- If you are an avid Grey's watcher, it appears that ABC chose to remove the part of their story in which the character suffering from VHL flies into a violent rage due to his adrenal gland tumor, known as a "pheo". This negative, inaccurate portrayal was what the family alliance specifically took issue with)
When I first met Andrew, he spoke of his diagnosis as non-chalantly as someone might talk about having the flu. He had brain tumors, he discovered them after he had to have life-saving, emergency brain surgery at 20 years old. He will probably always have brain tumors & will have to have brain surgery when the tumors create cysts. He could potentially get tumors in other parts of his body and had to adhere to a rigorous screening schedule involving MRIs, CAT Scans and ear & eye exams. No big deal. He handled the disease with a self-depricating sense of humor that was both reassuring and alarming.
When I was about 6 months pregnant, Andrew started to have symptoms of another cyst. He was losing his balance frequently. Dry heaving in the shower every morning. Although we were living together, he hid his symptoms. If he bumped into me while we were walking, he made it look like it was on purpose. I distinctly remember him almost falling down an entire flight of stairs at a friends house and laughing about it, thinking he was being foolish. Finally, he knew he couldn’t ignore it anymore & made an appointment for a MRI.
I went with him to Boston and sat alone in the waiting room during the procedure. It was the first time I truly contemplated this disease becoming my reality. With one hand on my rounded belly, feeling the cartwheels and somersaults, I wondered how I would feel about sitting in a waiting room like this not for Andrew, but for our child. I am about as anxious as they come, but for some reason, I felt an eerie sense of calm. I just knew, as I know today, that things would be ok. That there was nothing for me to worry about. When I relayed this to Andrew, he took it to mean I believed that our child wouldn’t inherit VHL. For me, it meant that I knew I would simply handle whatever came. I wouldn’t have another choice.
Andrew did indeed, have a cyst the size of a golf ball in his cerebellum. When we went to visit with his surgeon, I watched in horror as he failed every neurological test. He couldn’t touch his nose with his eyes closed. He couldn’t walk in a straight line. Surgery was not only necessary, it needed to happen the following day.
8 hours in the surgical waiting room crawled by. I forced myself to eat & my friend Robyn tried everything she could think of, including a game of Yahtzee that almost got us evicted, to keep me occupied. Later that evening, when it was time to go see Andrew in the Neuro ICU, I thought I would be fine. My mom is a nurse, I grew up around hospitals, I was prepared for the tubes and the bandages and the IVs and the monitors.
Wrong. My knees buckled. Robyn says I paled instantly. Andrew’s sister encouraged me to come close to him, kiss him and let him know I was there. I couldn’t move and uttering coherent words were out of the question. Robyn told her we needed to leave (and later confessed to me she was certain I was seconds away from hitting the floor & winding up in a hospital bed myself) and once we hit the hallway, I dissolved into her arms, sobbing. Andrew’s family, veterans of this brain surgery business, assured me he looked so much better than last time he was here. But I barely heard them. The man I loved- my best friend- looked broken. Here was the man that was strong & brave and could make me laugh no matter the circumstance, looking small and weak and somehow shrunken in a giant hospital bed.
I was staying with Andrew’s cousin in Cambridge, who was nice enough to have me while he was at Mass General. When I got into bed, I thought I wouldn’t be able to banish that image of him from my mind, that I would never be able to sleep. Instead,for the first time I felt the full weight of my exhaustion and instantly fell into a dreamless sleep. By the next morning, Andrew was teasing me, joking with nurses, and holding my hand. I was able to almost completely banish the fear I felt only a few hours before.
The day we found out Grace had VHL, that fear returned.
Andrew came home to find Grace and I snuggling and giggling, rolling around on our bed. It seems that moments of pure, unadulterated happiness always precede bad news. He walked into the room, picked Grace up and held her close to him. And whispered “I’m so sorry”.
I knew I couldn’t cry then. Instinctively, I knew my brave face was important. I hugged them both, reminded him that it wasn’t his fault. Tried to joke that my “good” gene was a wimp & couldn’t overpower his. Andrew hadn’t know if he even wanted children, because of this moment. When we found out I was pregnant, I promised him it would be ok. I was wrong. I walked out the door to go to class.
I’m not sure I even made it out of the driveway before I crumbled. I called my mother at work, who hearing the terror & sorrow in my voice, started to cry herself. We talked each other down from sadness for the majority of my ride into campus. I parked my car, but found myself frozen. I called my dad, who thankfully, remained calm. I voiced my biggest fear out loud, in one breath. That I would lose them both. That this disease would steal the two people who take up this massive space in my heart. I was able to be calmed & comforted enough to walk to class, but my tears were constant. Even though I felt relatively collected, I simply couldn’t stop crying. For almost 45 minutes of my class, I sat, typing out notes on my computer, crying silent, continuous tears. Finally, I grabbed my things and just walked out. Robyn found me pacing in front of the building and we walked together, sometimes talking, sometimes crying, until I felt..ok.
When I returned home, I felt oddly refreshed. I knew we could handle it. As a mother, I was saddened. I felt (and still do) the dull, aching pain of the pull and tug of guilt and grief. Of wanting to take it away from this innocent, beautiful child and place it on myself. I would take those ominous three letters away from both of them, if I could. We chose not to tell many people right away, I think mostly so we could have time to digest it ourselves, without constantly ripping the band-aid off a fresh wound. Things unfolded naturally, and many people did not find out about Grace’s diagnosis until we were on the news. I worry that this hurt people, but truly, it was just the best way we knew to handle it.
The “good” news? People with VHL are rarely symptomatic before adolescence. Some people live their whole lives without even knowing they have the disease. Grace is, right now, perfectly healthy. But her childhood will not be entirely typical. It will be filled with trips to Boston for screening and yearly appointments with the geneticist who is following her case. I will have to try and explain to her that she & daddy share this backwards gene, that might make her sick someday, but that we are doing everything we can to keep her healthy.
We took her to Boston Childrens for her first screening when she turned 1. It was a fairly simple eye test, but involved a lot of down time. In that waiting room, I looked around at the children who were very sick, children with multiple disabilities and challenges. Here I was, trying to reign in this beautiful, energetic toddler who looked, on the outside, to be the picture of health. I felt a very different guilt. Guilt for being so scared. Guilt for mourning this for so long. Grace will laugh. She will play. She will roll her eyes at me & slam her bedroom door. She will make mistakes, fall in love & grow up so fast she will surely break her mother's heart. How could we think of ourselves as anything but lucky?
It is my greatest hope that my daughter will live a life symptom free, or that by the time she reaches adolescence, the diligent efforts of the VHL family alliance and their researchers will have resulted in a cure. Short of that however, I know that my daughter will live a fulfilling, rich and rewarding life, regardless of her diagnosis. She will not be defined by the label of disease, but rather by the infectious nature of her laugh, the way her big blue eyes shine and by countless other beautiful, wonderful attributes that have yet to be discovered. She will be stronger because of this.This will help her to respect the fragile balance of life and love and savoring every minute. I know she will have a special bond with her father, that goes beyond shared genes- something that I will feel lucky to stand at the edge of and marvel at for a while.
I can't begin to know the challenges we will face or the roads we will have to travel. But I know we will do it together.
Mostly, I know that in this life we are not given things we can’t handle. I find great peace in that.
Want to help? Spread the word. Educate people about VHL. Donate whatever you can afford..even just $1..to the medical research being done by the VHL family alliance. Added bonus? With a cure for VHL also comes a cure, or better management, for many kinds of cancer since the diseases function in such a similar way. It is on my list-of-things-to-accomplish-when-I-FINALLY-finish-grad-school to organize some sort of annual fundraiser for VHL (suggestions and helpful hints would be much appreciated!)
This wasn't easy for me to write about. This is our reality, mostly private & sometimes painful. However, there are often days and weeks that go by where I don't even give VHL a passing thought. Lately, it has been weighing heavy on my mind and my heart.
But see how easy it is to get distracted around here?