Genetics Care…for Children and Adults (Hurler-Scheie)

Our son Esteban was born in 1980. At first, he seemed like any other “normal” baby. He crawled in an army type fashion, lying down and using his arms to drag himself. He walked by 11 1/2 months. He was speaking clearly at an early age. It wasn’t until he was about 1 1/2 years old that we started to notice some “problems”. First, the baby fat on his hands was not going away. Then, his hands began to show signs of stiffness. After this, he started to struggle with his fine motor skills – he couldn’t pick up Cheerios as easily as before. Holding on to cups and glasses became a problem – the cups and glasses would, many times, slip from his hands and fall to floor. Esteban also began to have frequent ear infections that eventually caused hearing problems – thank God his speech was well developed before this occurred and he was able to communicate clearly in Spanish. Because of these struggles, we sought the advice of our pediatrician who recommended that Esteban be seen at a genetics specialty clinic located in Children’s Hospital of Wisconsin.

Here we met Ms. Lu Ann Weik, a genetics counselor. She became and still is our link to many of the services and opportunities that Esteban has been fortunate to receive. At our initial visit, it was suspected that Esteban might have some form of arthritis. Esteban was then seen by Dr. Herrmann, a geneticist, who first expressed to us that Esteban might have a rare genetic condition. After many tests and a visit with another geneticist, we were finally told that Esteban does have a rare genetic condition called Hurler – Scheie Syndrome. As I sat in the doctor’s office with Esteban in my arms and was being told of his condition and the expected prognosis, I felt devastated and crushed. My worst fears were confirmed as the doctor explained that Esteban most likely would not be able to do any physical work and possibly could be in a wheelchair by the 8th grade. He continued by saying that there was nothing that could be done to stop the progression of the illness. My child’s health, I believed at the time, was about to deteriorate quickly. I also believed that there was nothing I would be able to do to stop or even improve what was expected to happen. I felt absolutely alone and numb with pain. Even after all these years it is difficult to relive.

However, we were and continue to be fortunate to be connected with the Genetics Department at Children’s Hospital of Wisconsin. Because of people like Ms. Weik and Dr. Lubinsky (Esteban’s pediatric geneticist for several years), Esteban and our family have not been alone and we have been able to fight this condition. Early on, Ms. Weik took it upon herself to serve as an educator, an advocate, and a friend for Esteban and our family. She helped us understand what Hurler-Scheie is, she connected us with helpful resources, she answered our questions, and she has continually provided support and encouragement. We also met annually with Ms. Weik, Dr. Lubinsky, and a team of other health care providers (including other specialists and physical and occupational therapists) to plan out a strategy for Esteban’s health; this continued without much persistence from my part from the time Esteban was diagnosed at the age of four till he turned 18. More recently, Ms. Weik and the Genetics Department are assisting us while Esteban is involved with a research study that presently requires us to travel weekly to New York so Esteban can receive special treatments.

If not for the ongoing, consistent efforts and advocacy of people like Ms. Weik, Children’s Hospital of Wisconsin’s Genetics Department, and Dr. Waters (Esteban’s pediatrician), Esteban would not be where he is today. Their assistance, guidance, support and encouragement over the past 20 years have helped improve Esteban’s health and quality of life. They have helped Esteban and our family through at least 14 different operations and procedures to help correct different problems such as preventing stiffness and improving flexibility in his hands, reducing pain in his wrists, and reducing occurrences of chronic otitis media. They also have given us hope and encouragement for the future. In spite of his “condition” and the current weekly trips to New York, Esteban is active and continuing his education at the University of Wisconsin-Milwaukee. We feel confident that the assistance, guidance and advocacy already put forth from the Genetics Department at Children’s will continue and that Esteban and our family will have access to this wealth of resources for many more years to come.