It’s now been over 4 years since my daughter Iris’ diagnosis with GM1 Gangliosidosis, a progressive neurological disease which is always fatal in children. At the time of diagnosis, she was five and half years old and she is now ten years old.
Doctors recently started using the words “progressed” and “advanced” to describe the stage of her disease. We’ve gathered statistics which indicate that the average age of death for a child suffering from juvenile GM1 is age 14. We push this statistic out of our minds. Its simply too much to bear to think she might have only four years left. We simply don’t know what the future holds.
I’ve been struggling for awhile now with what to say. A lot of people ask a very simple question relatively frequently “How is Iris?” I wish the answer was simple, but it’s no longer simple. I want to convey hope, but I also want to be realistic and truthful. My response is usually something along the lines of “She’s doing the best that she can.” Other standard responses include “She’s ok.” or “Relatively speaking, it could be much worse.”
The infantile and late infantile forms of GM1 Gangliosidosis are more severe. We’ve seen dozens of children die from GM1 over the years. In the case of babies with infantile GM1, the majority do not see age 3. For an infantile GM1 baby, age 5 is considered an extremely long life. However, make no mistake. No child has ever survived GM1 Gangliosidosis regardless of whether it’s the infantile form, the late infantile, or the juvenile form.
Recently, just a few days ago someone asked me “How is Iris?” I replied candidly and the person’s reply was “I’m so grateful my children are healthy.” The person who asked seemed to be expressing relief to me. Please never reply this way when receiving a vulnerable admission from a parent whose child is dying. That sort of comparison is simply a dagger to the heart. Nonetheless, the disease is a fact of life for us. We live watching our daughter slowly slip away from us.
At the time of her diagnosis, she could read, write, speak, and play on the jungle gym. Years ago, even shortly after diagnosis, we were told, it was already too late, and basically hopeless. Regardless, we chose to fight to fund medical research and we continue to do so. That was 4.5 years ago. Imagine the words “advanced” and “progressed” ringing in your ears. Know that life does not stop. Time and the disease march on. Know that we’re fighting for something bigger than ourselves.
I’ve decided to say it to myself and to say it here. Iris is not the same and never will be. My heart and my life will never be the same either. Her gorgeous smile and her ever so innocent and sweet spirit have forever changed me. She is loved beyond measure and always will be.
Perhaps, are you wondering now if we’ve given up on our sweet girl? The answer is that we would never give up on our girl. We don’t take even a single millisecond for granted. We’re still fighting to maintain her quality of life to the best of our abilities. We’re still fighting for all those suffering from this awful disease.