My husband, Keith, and I have a little girl named Jenna Marie who was born April 24, 1997. She was born with a heart problem known as cardiomyopathy but the doctors
were confident it would clear up. It never did.
After 5 months, the cardiologist (Dr. Matt Wienecke) decided to do a test on Jenna for a genetic condition known as Mucopolysaccharidosis (MPS). He was doing the test mainly to eliminate it. However, the test came back positive. Dr. Wienecke had the unfortunate responsibility to tell us what this condition meant. (Mucopolysacchrides are long chains of sugar molecules used in the building of connective tissues in the body.) Jenna was missing an enzyme which is essential in cutting up certain mucopolsaccharides. The incompletely broken down mucopolysaccharides remain stored in cells in the body causing progressive damage. The four organs mainly effected by this disease are the liver, spleen, brain and heart. Thus, the reason
for Jenna's heart condition.
There are several types of MPS, but the type it is believed Jenna has is Hurler's, one of the most severe. One thing about MPS is that one cannotlook at a child with the disease and assume another will have the same problems. These problems range from stiffening of joints, loss of hearing, blindness, and chronic respiratory problems. There are also various defects
in bones, cartilage and connective tissue, leading to short stature. Children with MPS look very much alike because of these various defects...their heads tend to be "too large for their bodies", their eyes are far apart and they have "flat noses". Many of them have "enlarged" tongues and because of this may have limited speech. Mental retardation is also a product of
this disorder. As if all this weren't enough, children with Hurler's usually do not live past the age of 10.
The only successful procedure for potentially helping children with this disease is a bone marrow transplant. When Dr. Wienecke first told us what Jenna had, he felt that because of her weak heart, she most likely would not survive a bone marrow transplant. The reason is because of the chemotherapy and radiation used to destroy the bone marrow and immune system. However, since that first meeting, Dr. Wienecke has met with a hemotologist (Dr. Gretchen Eames) at Cook Childrens Hospital in Ft. Worth, TX and because of a different type of chemo that can be used, now feels there is a chance for Jenna.
I am telling you all this to ask if you will forward my information on to other Christians so they may pray on our behalf. We have already seen what the power of prayer can do...when the hospital searched the bone marrow donor bank (we have to go with an unrelated donor since Jenna is our only
child and therefore cannot have a sibling match), without doing a complete search, they came up with twenty "six out of six" HLA type matches. We also had to make a choice of where we wanted to have the transplant. When we finally decided to have it done at Cook-Childrens in Ft. Worth, which is local for us, we had such a peace about our decision. The only other option was Minneapolis, MN, which is where the experts on MPS are located.
If we had chosen to go there, they would have used the chemo that is known to be toxic to the heart. And, by doing the transplant here we will have family and friends nearby to give us the support we will need to get through this.
Jenna will enter the hospital on May 6th (two weeks after her first birthday), and the chemo will begin Saturday, May 15th. The transplant will take place on May 18th and from that point it will be a "wait and see" situation. The hospital length
of stay could be as short as 4 to 5 weeks if all goes well or as long as 4 to 5 months if there are any complications. I
will be taking a 3 month leave of absence from my job as of May 4th and am unsure of what I will do once my leave is over since Jenna will need to remain isolated for the next year.
