What is I-cell anyways?

Let's just say we have been learning lots and I feel like I have only scratched the surface as to it's complexities.  Before Gabrielle was born I had no idea what a storage disease was.  Now, I do and I am trying to learn as much as I can about it.  There are many different lysosomal storage diseases.  Unfortunately, I-cell is one of the nastier and more horrible diseases with no cure, and these poor children who live with this disease don't have it easy.
You see the tricky thing with this disease is that there is no way of knowing exactly how it will affect Gabrielle.  It could progress really fast and her health could deteriorate quickly and be quite devastating.  Or it could be much more mild and Gabrielle could reach some developmental milestones all on her own.  This has made the BMT decision 'impossible'.
Our hope for Gabrielle is that the stem cell transplant, which will take place at BC Children's hospital (dates have been changed again: line insertion March 11th and chemotherapy to begin March 14th) will significantly improve her quality of life.
Every bit of my heart goes out to my daughter Gabrielle and to all the babies born with this disease.  They teach us so much about life, put everything into perspective, and remind us that we are only human.

Some other words used in place of "I-cell disease" are Mucolipidosis II, or ML II.

The first clip is done by another ML II family who have twin boys with the disease.  Thank you Mercedes for sharing your experiences with others in hopes of bringing awareness and educating others about the disease our children have.
The second clip is one of the first clips I ever saw teaching me about ML II.  It is short and simple and makes it really easy to understand.