Daniel

My name is Roger. My wife's name is Kay. We have two children. Daniel is 12, and Sarah is 9. Only Daniel has Glanzmann's. He was diagnosed by Dr. James White at the University of Minnesota at the age of 15 months. We knew he had a bleeding problem at the age of 6 months. An immunization took a very long time to stop bleeding. We also noticed purple rings around his ankles from socks that were too tight. Then he started bleeding in his ear after scratching himself with a fingernail. It took us another 9 months to get a definite diagnosis. Daniel's Glanzmann's is classified as Type 1 or severe.

Multiple types of tests confirmed he is totally missing the glycoprotein IIb/IIIa complex. Dr. White told us when we he confirmed Glanzmann's that even though it is described as severe, it may manifest itself in ways different from other Glanzmann's patients.

Daniel has had the bruising which is typical of other Glanzmann's patients. We were called by the police department in our community when Daniel was about two years old. Someone suspecting child abuse because of his bruises, saw Daniel at a garage sale and wrote down the license plate of the car that Daniel had been in. We've heard similar stories from parents of children with hemophilia, so that part is not unique to Glanzmann's.

He has had many nosebleeds. When he was younger, they were more severe, that is, we would pinch his nose for up to 45 minutes in order to stop the bleeding. While pinching his nose, often in the early morning hours just after midnight, he would want to listen to a record with songs from the 50s and 60s. We called it our nose pinching record. For very severe nosebleeds, we sometimes tried something called a rhino rocket. It is designed specifically for nosebleeds, so that the nose does not need to be packed. It is basically gauze on a string and is put in the nose for about 1 day. It is uncomfortable for Daniel to use, but it worked. We did not have to do that very often, but it was good to know there was something there that we could do to help control severe nosebleeds. Daniel pinches his own nose now, and tells us when he needs Amicar. Just recently, we just switched to Amicar tablets. He greatly prefers them to the syrup (blech!). We didn't use the pills sooner because the pills are rather large.

Daniel was hospitalized at 13 months for bleeding from the mouth/lip. He fell while learning to walk, and at that time we knew next to nothing about controlling bleeding. We had Amicar, but thought it would stop the bleeding. We didn't know we had to get a clot to form first before the Amicar would work. His hemoglobin level was 5.7. He was hospitalized for 3 days and received platelets and packed red blood cells. At 17 months, he had a mystery throat/nasal bleed (or maybe new teeth?), which put him in the hospital. His hemoglobin was at 5.2. That put him in the hospital for two days. He received directed donor platelets from me and packed red blood cells in that case.

Daniel was nearly three years old as we anticipated the birth of our daughter Sarah. We considered an umbilical cord stem cell transplant (essentially the same as a bone marrow transplant). We even harvested the umbilical cord blood at Sarah's birth. After Sarah was born, she was tested immediately and found to not have Glanzmann's. Our hematologist consulted with other hematologists around the country. Even though the stem cell cord blood was a close match, they all agreed that they could not recommend that Daniel get the transplant. They felt the risks associated with the transplant outweighed the benefits.

When he was 5 years old (in 1996), he received platelets again for a fairly serious injury he received after slipping while climbing unto something at a relative's house. For those of you who use NovoSeven, remember that NovoSeven was not introduced for use in the U.S. until March of 1999.

He has not needed platelets since then, but we sure thought that we might need them again (or NovoSeven) while going through 4 days of continuous, serious uncontrolled bleeding which was caused by the installation of metal bands for braces in February of 2003. Our dentist used novocaine with epinephrine around the tooth to finally stop the bleeding. We think Daniel may have had antibiotic resistant strep last year (2002) while we were on vacation. Ever since then he seems to be susceptible to blood oozing in his throat when he gets a sore throat.

Daniel is OK now with letting his classmates know about his Glanzmann's. This last year in Daniel’s class (2003), each student had to do a report or a poster on a genetic disorder. Daniel chose Glanzmann's and told his class about his disorder. He even gave them a quiz at the end of his presentation. The bleeding because of the braces got Daniel thinking about a time when some lifeguards at a State Park were suspicious of Daniel's bruises, but never approached us about them. On his own, with no prompting from us, he recently wrote our U.S. Senator (Norm Coleman - we are from Minnesota), and suggested that Congress pass a law requiring lifeguards, medical staff, police, and daycare workers learn about the difference between children with bleeding disorders and abused children. Senator Coleman's office even called him back from Washington about his letter.

When we are dealing with a major bleeding episode, we need to remember that Sarah needs our attention, too. The University of Minnesota Hemophilia Center hosts periodic bleeding disorder support group meetings. At the suggestion of my wife, Kay, one of the meetings dealt with how siblings feel about growing up in a house where a brother or sister has a bleeding disorder. The brothers and sisters of the children with bleeding disorders got to have the grownups ask them questions about how they felt when their sibling was "getting all the attention".

We have learned that it is important to never stop asking questions about medical issues, that the parents often need to advocate for their children, and that parents often need to educate the professional people in the child's life (teachers, dentists, even doctors) about how this disorder is affecting the life of the child.