Friday, June 13, 2014


Life is unpredictable. That much I knew. I just never expected there would be these many sharp turns. Rett Syndrome is relentless, throwing one obstacle after another. And just as we start to catch our breath, a new challenge creeps up around the corner. I'm exhausted.

Caroline started having seizures last fall. We were desperately hoping she would never join that club. Sadly, an EEG test in December revealed that Caroline suffered from partial complex seizures several times a day. Of all the challenging symptoms associated with Rett Syndrome, seizures are among the worst. Seizures are dangerous and unpredictable. The International Rett Syndrome Foundation website reports that "factors most strongly associated with an increased risk of sudden unexplained death in RTT are uncontrolled seizures, swallowing difficulties and lack of mobility."  It destroys me that Caroline now suffers from all three complications.

Caroline's seizures were dangerously long and uncontrollable until about two months ago when, after trying a number of treatments, we found that a combination of two different medications diminished the frequency of her seizures. They are mostly under control at this point, but it's still hard for me to accept that seizures are part of our life now. These episodes are incredibly difficult to watch and I always feel the same: disbelief; fear; sorrow; and complete and utter helplessness. Crushing helplessness. There is nothing we can do but hold her unresponsive convulsing body while we wait for this scary thing to release its grip.

I feel as though something has been lost this past year. Caroline is not the same little girl she was last summer, before seizures began. Regression seems to have picked up its pace: her motor skills are steadily declining; she fatigues easily; her body is increasingly stiff; her ability to swallow is worsening so she chokes and vomits a lot; her appetite is low; and her tremors are more frequent and intense. I hate even writing this, but we also noticed that a little bit of her spark has dimmed. Something has also shifted in me that I can't quite articulate yet. I still smile and laugh and look forward to each day. I feel incredibly lucky for having support systems that give Caroline a healthy and happy life and also allow us to retain some normalcy despite living under chronic stress. Maybe what's changed is knowing for sure that my heart is irreparably broken.

This is not the life I thought we would have when we became parents, but Caroline is and will always be the best thing that has ever happened to me. She is my heart. She is my life.

Caroline getting hooked up to the EEG in December 

Her 24-hour video EEG was conducted at home

Saturday, January 4, 2014

It doesn't have to be weird

For many (though few would ever admit to it), interacting with someone who has severe disabilities can be awkward. I get it. A conversation with a non-verbal person can feel one-sided and uncomfortable.The fear of saying the wrong thing or not knowing what to say often results in not saying anything at all, leading to a situation where Caroline (and people like her) are often ignored or forgotten.

I'm not here to judge. In fact, I can absolutely relate. Before I had Caroline, I was uncomfortable in those situations as well--not because people with disabilities made me uncomfortable, but because of my own fear of doing or saying the wrong thing. What I didn't know was how much I would learn about myself, relationships, and love through those interactions. I would like to help take some of that weirdness away by offering a little bit of guidance that will hopefully make your interaction with Caroline a more meaningful one.

Approach spending time with Caroline as you would any other child. Caroline is like most little girls her age; she just can't express herself in the same way. She loves one-on-one interactions so she'll be very grateful that you talking to her. You can talk about yourself or mention her interests to her (she gets super excited to hear that people know about Belle or Yo Gabba Gabba!). And it's totally okay to ask her questions. I used to struggle (feeling silly and even guilty) when asking her questions she couldn't answer, but her bright eyes and smiles showed me that she appreciated my efforts. I understand that it's easy to question the cognitive level of someone who is unable to show their true intellect, but I encourage you to assume complete understanding even if you aren't getting typical feedback. 

She may look away or appear disinterested, but she's still listening. It is difficult for Caroline to focus her eyes on anything for too long. Rett Syndrome prevents her brain from sending the right signals to her body, resulting in the inability to control movement. This is manifested in the constant involuntarily motion involving her hands, as well as the various spastic/jerky movements in the rest of her body. She may close her eyes or look away, but we believe it's easier for her to process information that way. It may also take her around 30 seconds to respond. I should note that 30 seconds of silence and waiting can seem like an eternity when you are talking to someone.

It's okay to kiss and hug her. People often ask me if they can touch her. Yes, absolutely! Caroline is constantly smothered in kisses and hugs. We spend a lot of our time together cuddling. She LOVES it. She's used to my style of affection which is loud and intense! If you find that you don't know how to talk to her (which happens to me too sometimes), please feel free to hold her on your lap, or simply smile and make eye contact with her. I spend a lot of time doing just that. I'm always amazed at how much can be communicated without words. 

Please feel free to ask me questions. This goes without saying, but we've very open about Caroline's condition. I believe that talking about "difficult" issues in an honest and respectful way can reduce the stigma, fear, and discomfort around those issues. If Caroline's around, I'm happy to talk about how she communicates, how she uses her adaptive equipment, or how she goes about doing various activities. I'm also willing to talk about the hard stuff as long as it's not right in front of Caroline.

My message here is that any type of interaction is greatly appreciated (with or without the use of words). I believe that you will gain something profound out of it as well.