Emily

Emily is an energetic seven year old with Glanzmann's Thrombasthenia. When I talked with her about writing this story to tell people what it is like living with GT she said, "It's really not that bad." I am amazed by her acceptance of it. We have always explained it to her as having "special" blood. I think I have a harder time dealing with it than she does, then I realize if she can be strong so can I. A few hours after she was born they noticed "purple marks" had developed all over her body. They called her the "blueberry muffin baby."

They kept her isolated in the hospital for eight days. Numerous tests were done on her, including a biopsy of one of the purple marks, but still did not come up with a diagnosis. Her bleeding times were prolonged, but the counts were normal. Finally they sent her home, but our pediatrician said she would keep a close watch on her because she knew "something wasn't quite right."

When Emily was one month old her ear started bleeding. I couldn't get it to stop so I called the pediatrician. She examined her, tried to stop it and sent her home. Two hours later it was still bleeding so Emily was admitted to the hospital. When we arrived at the hospital they needed to take a blood sample. The nurse put the rubber tourniquet around Emily's arm and it immediately broke out in purple marks, called petechia, down to her fingers.

They tried the other arm and the same thing happened. I will always remember the shocked look on the nurses' faces. After about 8 hours they stopped the bleeding, but still could not diagnose the problem. They referred her to Children's Hospital of Philadelphia (about and hour and a half away from our house) where the Hematology Department finally diagnosed her with Glanzmann's Thrombasthenia. The scariest part of this was even though we now knew what she had, they gave us little information on what to expect or how to deal with it.

We learn as we go. Each new experience teaches us how to handle it if it happens again. I have learned not to panic at the first sight of blood and that a small amount of blood looks like a gallon when it's coming from your child. Our first "scary" experience came when Emily was about 6 months old. I was playing with her and she fell against me and cut her lip on my necklace. It started bleeding. I thought I had gotten it to stop before putting her to bed. When I went to wake her up the next morning I was horrified to see the ring of blood on the sheet around her

head, her hair matted down with blood and her face and hands covered in dried blood. I panicked and yelled for my husband. He picked her up and she smiled at us! What a sight that was.

Emily gets terrible nosebleeds. The blood flows out of her nose and down her throat at the same time. At times to the point where it causes her to choke. These were scary at first, but like everything else we have developed a system on what to do, stay calm and get through it. Every time a bleeding episode begins you wonder if this is the one that isn't going to stop. Emily has been hospitalized three times for bleeding in the throat. These were stopped with the use of Amicar. She was hospitalized once for bleeding in the soft tissue behind the knee. Emily has been hospitalized twice for nosebleeds. These were so far back that the blood wasn't coming out of her nose, but going down the back causing her to spit out blood every 2-5 minutes. This lasted at least 24 hours both times. She needed to receive one unit of platelets the first time and two units of platelets for the second episode. Emily had a reaction to the transfusions. Her blood pressure dropped very low, her heart rate went up and her body shivered uncontrollably. We now know to tell them they must pre-medicate Emily with Benadryl and Tylenol before giving her blood products. She needed to receive a red blood cell transfusion for the first nosebleed as her hemoglobin dropped to 5.0.

There are always bruises. It upsets me to see her body covered in bruises. Emily accepts them as part of life, even the sore ones. We can usually count on her having a black eye or some bruise on her face for important events or photos. I see people stare at her bruises, luckily Emily usually doesn't notice. I hope as she gets older this won't bother her too much!

If you ask Emily what the worst part of having GT is for her she will tell you its NEEDLES!" This is her biggest fear when she has to go to the hospital or for a checkup, which are every four months at CHOP. Like everything else she manages to get through them. We tell her it's okay to cry. She has earned that much!

In the beginning we didn't know what to expect. Some of it has been better and some of it has been worse than what we thought. So far it hasn't restricted her from doing too many things that she would like to do, she just has to be a little more careful and a little more protected than the other kids. I worry about things Emily will have to face as she grows up. I am extremely grateful for the research that is being done and hopeful for her future!