Our take on Hurler's Syndrome
Hurler's Syndrome, also refered to as MPS I, is a very rare disease that affects every 1 in 100,000 infants. While there are numerous types of MPS, Hurler's disease is one of the worst. A child with Hurler's will have noticeable symptoms within the first year of life whereas other forms of MPS are noticeable as the child grows.

In a nutshell, Hurler's affects all parts of the body. The child stops developing after the third year of age and will most likely be mentally retarted. Hearing loss and Corneal clouding are common for kids with Hurlers. The corneal clouding gets to the point where the child will need glasses and will have night blindness. They have the possibility of having and enlarged liver, spleen and/or kidneys. Their bone structure will not develop properly: their ribs will not form correctly, their hand motions will resemble that of a claw and they would most likely have stunted growth, or dwarfism. They would easily get upper respiratory infections and would suffer from breathing difficulties and sleep apnea. If that weren't enough, there would be a decent chance that their teeth also would not come in normally. Finally, and worst of all, the child would most likely die between the ages of 5 and 10 due to heart failure.

In lamans terms ... children with Hurler's are missing a "cleansing enzyme." Normally, garbage will collect on our cells but the "cleansing enzyme" would go through the body and clean the cells. Since kids with Hurler's lack that enzyme, the collection of the garbage continues and eventually the garbage on the cells would damage their bone structure and organs.

There is no official cure for MPS. Treatments are available though. First is ERT, or Enzyme Replacement Therapy, which is basically like being given medication that has the enzyme which would clean out the organs. However, it doesn't fix any neurological damage, so if the MPS that a child has will affect their brain, ERT will not prevent their mental retardation. Another option is a Bone Marrow Transplant, but with kids with Hurler's it is almost always rejected and could actually kill them.

Another treatment that isn't really mentioned is an Umbilical Cord Blood Transplant where the child receives cord blood from a unrelated match. What will happen is that the child will get chemotherapy to reset their immune system and then they will receive a blood transplant that will contain that missing enzyme. The hope is that the new blood will take and that the body will actually start to produce that enzyme. If that happens, then 95% of the damage that the Hurler's would cause will stop. In some cases, the enzymes will actually go back and repair any damage that may have been caused. The only thing this treatment doesn't fix is any damage done to the skeletal system. (In our case, Logan's pelvis is a little deformed.)

The road to the transplant is not a quick and easy one. Our doctor told us that there is a 10% chance that a child receiving this transplant may run into fatal complications. The entire process can take up to a year. Two weeks for evaluations, nine days of chemotherapy with the blood transplant on the 10th day, then about seven months of monitoring to make sure that his immune system is picking up steam and, most of all, the missing enzyme is being reproduced. The child will spend the first two months post-transplant in the clean room for daily monitoring then they will be taken out of the hospital but still monitored as their immune system continues to strengthen. If it's successful, then yearly checkups are needed after the transplant is considered a success.