Princess Sophia

Princess Sophia
My name is Sophia Grace Crawford and I am one year old. I have been diagnosed with Batten Disease, a rare and incurable genetic disease that will cause me to lose the little sight that I have, worsening seizures, loss of the ability to move my arms and legs, and will ultimately leave me bed ridden. And it is always fatal. I will leave this world within the next two years but I don't know exactly when. Until then, my Mommy and Daddy want to help me bring awareness to Batten Disease. Please come on this journey with me so that, together, we can celebrate each day that I have left!

A Simple Question

A simple question, asked of my husband, without any real concern in my voice, would ultimately begin our journey into the world of epilepsy. I had noticed our daughter, Sophia Grace, then two months old, having odd jerking movements in the form of a head twitch and an arm lift. I asked my husband, Jacob, if he had noticed Sophia making odd movements. Sophia is my first and only child and Jacob has two sons so when he said something along the lines of “you are driving me crazy with all your concerns”, I let it go. About two weeks later, my husband noticed similar movements, and called me at work. I received a voice mail from my husband on my work line, telling me that he believed Sophia was having seizures and he was taking her to the pediatrician. I remember telling my supervisor that I had to leave work immediately and when he asked why, I began crying when I said that Sophia may be having seizures. And thinking back on it now, I was scared but I had no idea where this would take me. I was scared about my child having seizures of course, a diagnosis I did not have much knowledge of, but really I thought, okay, so Sophia will need medication. No big deal, right? Everybody takes medication.

My dream of becoming a mother began long before I even understood the concept of motherhood. And even when I became an adult and thought that I understood the concept of motherhood, of course as other mothers can attest, I had no idea. But one thing I knew for sure, I wanted to be a mother more than I have ever wanted anything in my life. My husband, having two children from a previous relationship, was not all that thrilled about having another child. He remembered the sleepless nights, expensive diapers and formula, and the general worry that comes along when a little one depends on you. I thought of fat baby cheeks, walks with a stroller, and hearing a child call me “mommy”. My husband and I never tried to prevent having a child and at one point, I did become pregnant only to have a miscarriage about 5 weeks into the pregnancy. We went to the obstetricians office for the first ultrasound and the technician said nonchalantly, “there is no heartbeat”. She said it like it was no big deal but to me, it was the end of my world. I cried for days on end; I just wanted my baby back.

After years of no pregnancy prevention, and believing truly that pregnancy was not in the cards for me, a coworker suggested a fertility medication called Clomid. I told Jacob that I wanted to try for a baby, really try, I was surprised that he was agreeable. He was nervous about using a fertility drug but I went to the doctor, received further information, and started the medication. The first month, nothing. The second month, success! I was so scared to take that pregnancy test but Jacob said “either you are or you aren’t” and so I took the test. I was so happy and so scared at the same time.

We had our first appointment with a new obstetrician, and we requested to have an ultrasound earlier than normal because of our previous miscarriage. That first ultrasound was terrifying for me; I gripped Jacob’s hand so hard but then I heard the most beautiful sound: Sophia’s heartbeat. Of course, she wasn’t yet Sophia back then, but just the thought of having two hearts beating inside me, was enough to make me smile.

My pregnancy and delivery were completely normal with no complications. I was induced three days past my due date and Sophia Grace Crawford was born on November 17, 2011, weighing 7 pounds and 9 ounces, and 19 inches long. For two wonderful months, she was nothing but normal. And then the seizures began.

Sophia has been admitted to the hospital seven times total, with no less than three days each time. The first hospital stays were optimistic, with us leaving thinking that everything was under control. Each hospital stay brought more reality into our lives. We have had issues with doctors, one telling us that “babies move in funny ways” and “there were no seizures found on the EEG”, and our favorite, “Sophia is fine and will be discharged”. We have had issues with nurses, from listening to Katy Perry playing at the nurse’s station with about ten nurses standing around laughing while our baby girl was seizing uncontrollably in the next room, to a nurse saying that she didn’t mind “making babies cry” when giving Sophia her first IV. We have had issues with hospitals, having to share a room with another family with a child recovering from meningitis, to having bed accommodations for one so my husband and I had to sleep in shifts. We have tried numerous medications, each one costing a little more each time, only to be told that the medication is not working and to go and get yet another one filled at the pharmacy. We have learned to give intramuscular injections which our daughter had to endure each day for a month. Imagine making your child scream because of the pain you are causing, on top of everything else she is going through! We have willingly given medication that is known to cause vision problems, most likely eliminating periphereal vision, because the pros outweighed the cons. We have given medication that the hospital cannot even carry because it is not approved for use. Sophia has been poked so many times by nurses that want to “just try” to get an IV to work even though we have expressed that she is a hard stick and only the specicalized teams have been able to do this; it’s almost like an honor badge to the nurses to be the one to get the IV to work.

The pain that came with the knowledge that my child will never walk or talk was unspeakable. Now, sitting in the PICU with my child on oxygen and the doctors speaking of introducing a medication induced coma, the walking and talking thing doesn’t seem to matter much anymore. Amazing how God can put things into perspective with each passing minute. If she didn’t need me so much right now, I think I would comjpletely shut down. There are times when I go sit in the bathroom floor, lights off, just to cry in my own little dark world. I think that I cannot possibly go on. But Sophia needs me and I need her. She is a little me, lying in the bed just patiently waiting for the doctors to finally get it right. And I’m sitting in a chair next to her waiting on the same exact thing. It’s just that I am not quite as patient as she is.

So we learned that the phrase “one day at a time” really means just that. I don’t look ahead to the future, I look to getting through each day. I don’t google medications and dignoses and diseases anymore. I don’t ask about the statistics because I refuse to put limitations on my daughter or to allow anyone else to tell me what she can or can’t do. I still want my daughter to be President, a doctor, a teacher. But in the meantime, I am happy to just see her eyes open and for her to breathe on her own. It really is the small things.


  1. I believe that this is the most beautiful thing I have ever read. No matter what she is lucky to have a mom like you! God truly blesses with the children we are thr perfect for for! I have two children and can't imagine what your going through! God Bless!

    1. Sophia is my everything, the light of my life, and I wouldnt trade her for the world! She makes me happy everyday. :) Thank you so much for taking the time to read Sophia's blog and for your comment.