My story is long, but since I am one of the oldest diagnosed Glanzmann Thrombasthenia patients in the United States, I must be upfront that my history is not a nice one. I have learned that everyone’s life is not all peaches and cream. This is also taken from a couple of emails that I sent to Helen in August 2001, when we first contacted each other through the Internet.
My name is Karen Tucker (Kaytee to my friends). I am a 43-year-old Glanzmann Thrombasthenia patient. I am from Louisiana.
I was diagnosed in 1971. To begin my history, I should be dead. When I was born, I was bleeding intestinally. A couple of months before I was born, a child was born in New Orleans (where I grew up) with a bleeding ulcer, and the doctors had to perform surgery. Well, needless to say, that is what the doctors want to do. WRONG. By the grace of God, my parents decided not to do the surgery. My stepgrandfather was a doctor and a good friend of my Mom’s OB. They told my parents to wait. This was in 1958. I received blood, still of the scars from the cutdowns. I have pictures of my christening in the nursery (no ICU for babies at the time), and I also received the Sacrament of the Sick at this time. My bleeding did stop. ROUND 1 over and won.
A very young Hematologist told my parents the only test he knew of at that time was to put my finger under a microscope and watch the blood flow through the capillaries. M,y, how times have changed.
I have had bruises, nosebleeds, and gum bleeds all my life. From talking to the Docs, I understand that each patient has their type of bleeding. Some tend to have gum bleeds, others nosebleeds. I have had them all. I remember one birthday, I stayed home from school because I lost a tooth and just continued to bleed. Teabags were my Mom’s secret weapon for gum bleeds. I will still use that today. Also, silver nitrate works well; I learned that one from an emergency room doctor.
You name it, and I have bled from it, but my joints. My parents did not have problems with child abuse. I grew up in the ’60s and ’70s; people did not worry about abuse then. Lucky for my parents. I am short, for 4 years I was the same height in grade and junior high. All my class photos I am wearing makeup. You see I was the same height as doorknobs and guess who either walked into them or had the door opened into me. I always had a black eye. I would have to get stitches for minor cuts; I once got my foot caught in the spokes of a bicycle while riding on the back of it with my cousin. I remember having a drain in my foot, not walking, and staying with my Grandparents. I loved the last part. WI wasnot supposed to be on the bike, Cousin iis n big trouble. But I survived. I have had a nail in a palm of my hand from walking up stairs without planks, just nails (all the other kids were doing it), I got to stay with my Grandparents again (hey maybe a pattern here I never thought of that). I was not allowed to own a bike or skates, but I played kickball, football, and other sports with the neighbors. Everyone I grew up with knew I bruised easily, and I was never teased (what a Blessing that was). My family doctor knew I was a “bleeder” but not a Hemophiliac.
I was in and out with tonsil, ear, and throat problems. He told my parents, “I don’t know why she bleeds, but we are not going to take out her tonsils.” That saved my life. FI fell in love with horses, which is still my passion. I survived childhood. ROUND TWO WON.
Everything went to pot when menstruation hit (12 years old—bledd for ten days. I could not go to school on the first day. I insisted I go the second day (new school Junior High). When Mom got home, I told her I had bled on my dress. She took one look at me and went off to the doctor. In the hospital, Hgb is 4, and there is blood, blood, and more. I received 75 units of blood, platelets, Fresh frozen, Cryo, Factor 8, albumin you name it I got it. My parents had to replace 150 units of blood from that stay. I was so critical that they could not move me into ICU. I still bled. Mom said I was passing clots as big as bab
es. I remember all of it, even though I was semi-comatose. I had an out-of-body experience, and I believe there is a better place than where I am (not ready to leave here, though). On the eighth day, my Hgb drop below 3, the doctors said there was nothing else they could do, they had tried everything. My parents called in the priest. Sacrament of the Sick was given to me at that time. I remember my doctor’s hand pumping blood into me with a pot-a-dotted tie and striped shirt. Mom said that was true, but I was in and out of conscience then. Within two hours after receiving the Sacrament, my bleeding slowed down and then stopped. I was home by the tenth day. Doctors are excellent, and God gave them the power and knowledge to heal, but only God can save a person. That is why I say I am here by the Grace of God. ROUND THREE WON!!!!!!! But the war goes on.
When I got out of the hospital after my first menstrual cycle, I started going to lots of doctors. The hematologist I started seeing was the same hematologist that consulted at my birth. I guess we were lucky. After doing a lot of blood workups, he thought I had GT, but at that time, no one in the area had a platelet aggregation instrument. The doctors put me on regular birth control pills (I did not know what else to do). I returned to school four weeks to the day (well, almost. Kids were asking me about my yellow eyes. Guess what I had. Hepatitis B almost killed me. Every time I started to menstruate, the docs upped the birth control, and the hepatitis got worse. My doctor started calling all over. Finally, three places responded. My parents decided NIH was the best. I spent 3 months in 1971 up
in Washington, D.C. Dr. Shulman did the final diagnosis. I was put on Provera (progesterone) by mouth. It is supposed to stop ovulation. Well, I continued to have breakthrough bleeding. It was tough for a 12-year-old not to run or play (if I did, I would start to bleed). But it was the only solution. I got over hepatitis. A new program from the military (guys coming home with hepatitis from Vietnam) was to let them eat anything they
could keep down. Once they let me eat what I wanted, I was over hepatitis by the time I left Washington. But to this day, I get sick when I smell Portraits cooking.
There are lots of little stories of bleeds and getting blood. The main thing about the NIH experience was that I was allergic to platelets. I have both HLA (white cell) and platelet antibodies. So, platelets are out. I once was going to have laser surgery on my uterus to stop the bleeding. I went to Nashville, T, N to have it done. The HLA matched my platelets. I had several donors ready for me. The hematologist knew Dr. Shulman and called him. They gave me a plateletpheresis to see how I responded and to know
how many to have on hand for me. Well, my platelet count dropped. I destroyed not only the donated platelet but also some of my own. The doctor said to go home. If I continued the procedure, it would be like jumping out of a plane without a parachute.
Here is what the doctors told my parents when I was twelve: No known GT had survived to adulthood. Let me be a child, ride bikes, skate, have a horse, etc.c. I would not survive anyway. Well, my parents did that. I got my first bike and horse when I turned 13.
The NIH researched me for years, but nothing came out of it. I have been told that my platelets were the most researched. Doctors would find out when I was in, see me, and ask questions. The last time I saw Dr. Shulman before his death (the government closed down GT research after his death), he said he was most proud of my accomplishments. I finished high school, graduated from college, and led an everyday life. He did say he wished he never told my parents I could have a horse. Though the research that was done in the 70’s and 80’s did not lead
to anything for GT, my platelets were used to develop a new heart drug. Did you know a drug induces GT in everyday people to prevent atherosclerosis (sp. plaque build-up due to platelet aggregation)? That was developed from early studies using my platelets. So, I am told I should not get heart disease. In the early 90’s a doctor from Japan was over here at NIH and was doing some other research with platelets and they ran out of mine. They used mine as an abnormal control for other experiments. Anyway, he was fascinated with the way mine worked and wanted to do DNA sequencing. Well, mine did not match any other known GT DNA pattern. This got the ball rolling again. But he left to return to Japan, taking some of my platelets with him. Dr Shulman died in 1995, and the government shut down all his research. It’s such a waste. But once, before the government was cutting programs, I was having heavy bleeding, and my doctor could not get in touch with Dr. Shulman. I saw on Tthat V that the government was cutting funds to N, IH, and I wrote President Nixon then. I got a letter from the White House stating that my doctor would receive a call from Dr. Shulman within the week. I was back at NIH by the summer. Dr. Shulman asked me what I did because his programs were slated to be cut. I told him, and he laughed and said Thank you.
In 1979, I was at Tulane going to school; I had a GI bleed Hgb down again to 4 and received only four units of blood this time. But again, I was given the Sacrament of the Sick. Another ROUND WON!!!!!!! I was presented at grand rounds. I got to talk to all these doctors and student doctors from LSU and Tulane. Some doctors had not gone to Grand Rounds for years but were interested in my case. I told them my entire medical history and then about my life. I was nervous and excited at the same time. I was a 19-year-old in front of these doctors, but they were cut, and I asked many questions about my everyday life and how I controlled small bleeds.
In 1994, I fell off my horse and broke my arm. Well, I had to have surgery to repair it. It was a terrifying time. All my life, I was told I would not survive the surgery. My best friend is a nurse anesthesiologist. It was at her house I fell off my horse. Anyway, to make a long story short. I received six units of blood, survived the surgery, and now have a titanium rod in
my left arm. At least it does not go off at airports. Another round was won.
Well, from all this, I was eventually placed on Depo-Provera (shot). It worked great for several years until my thirties. I would go this route again if I had to do it over. Please stay away from platelets unless it is a significant life-or-death situation, but I have faced that several times over the years and did not get platelets due to the allergy. I will not deny getting platelets if I had to, but they would have to overload my system with medication and platelets.
Because of all the blood products I received, I got Hepatitis B, Platelet antibodies, and HLA antibodies. I was diagnosed with Hepatitis C in 1999 and with an Alloantibody (red cells) just last year. I am not too concerned with all the antibodies since cross-matching blood and blood filters are more sophisticated today. Today, my Hepatitis C is in remission. No drugs were taken. Another Round Won. Lots of prayers on this.
I had a flow cell cytometry done in the fall of 2001. A baby in New Orleans was being worked up for Glanzmann’s. My blood was sent off as a positive control. Dr Coker’s (Pathologist) tech called me later to get some info and said she was playing around with my platelets and doing some aggregation studies for fun. She said I was flat-lined—no aggregation whatsoever. Well, I guess I still have Glanzmann’s. The Flow cytometry test confirmed that I have no glycoprotein IIb/IIIa on my platelets. Thus, I have the most severe form. Like, I did not know that already. But I guess it is good to know I was not misdiagnosed as so many other people were.
The bleeding has gotten better as I have aged. I don’t know if it is the disorder or just growing up. Childhood, adolescence, and young adulthood are hard physically and emotionally. You don’t realize it at the time. I did want to be like others (standard). I tried my best and was lucky that my parents let me be.
I do believe patients are being misdiagnosed. More so in the past. I went to Tuscan in high school to visit my grandfather mid 70’s. He was a professor at the Medical School. I started to bleed there (not bad), so he wanted me to see the head of Hematology. Well, when my Mom told him that I had Glanzmann Thrombasthenia, he said there was no such disease. He asked Mom who diagnosed me, and Mom said Dr. Shulman was at NIH. He said well if ifRaphaell diagnosed that disease, then she has it. I am glad that more doctors know it, but not enough.
I know this is long, but I wanted people with Glanzmann’s, parents, doctors, and just laypeople to know that Glanzmann’s is a disease that one can live with. It can be challenging, but there are new drugs now that were not there when I was growing up, such as Amicar for mucosal bleeds, Nova 7, and better and safer blood products.
