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Have you ever heard of Thrombotic Thrombocytopenic Purpura? I didn't think so! It's okay though, most people haven't. Thrombotic Thrombocytopencic Purpura is a very rare and fatal disease. Only three to for people out of one million people will be diagnosed with it and one of those people were my aunt, Rachael.
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Rachael's Story
My life finally seemed to be on track after years of struggling with my parents' divorce, my mother's new marriage, a battle with depression, low self esteem, and a teenage pregnancy that landed me a new job as a single mom. My son was four years old, and the light of my life. The demons in my past were slowly fading into the background and I was moving on. I graduated high school early and we finally had our own place. I had my first brand new car, I was paying my own bills, and I had a great job working for a wonderful company. I was planning on enrolling in college the following fall to study to be an ultrasound tech like my mom. Little did I know of what was lurking in my body and how it would change my life forever.
It started with a headache. It wasn't out of the ordinary...I had suffered with headaches since puberty. I wrote it off to being overtired and a little stressed. It wasn't extremely painful...just a very irritating and strong ache. I mentioned it to several people but didn't think much else about it. That was a Saturday. On Wednesday, things started to get a little worse. I noticed several large bruises in odd places...on my hand...my foot...my forearm. Again I just dismissed it and started to get ready for work. I was really dizzy and lightheaded and decided that maybe I needed to go to the doctor. Next thing I knew, I was waking up on the floor. I was confused...”how in the world did I end up down here?”. I called my mom and told her what happened and she took me to see a PA at my primary care office. He didn't do much...asked me about where the pain was and said that I was having a migraine. He gave me a shot of stadol in the office and sent me home. Looking back, I should have mentioned the bruising and the “pinprick” rash on my ankles, but I just wasn't very in touch with my body and didn't think it was related. If I had mentioned it, maybe he would have recognized the petichae and he would have known it was serious and requested blood work. Talk about hindsight being 20/20!
I went home and slept for several hours...when I woke up, the headache was still there. I was really aggravated...after several days of dealing with a headache, I was so hoping I would wake up and feel better! I decided that I would just have to suffer with it...maybe it would go away on it's own. No such luck.
I got progressively worse...I started blacking out at work, and I was exhausted 24/7. On Saturday, after I had a meltdown over a bowl of soup, I decided I was going to stay the night at my mother's. I don't remember putting my son in the car, or driving across town to her house. Thankfully someone was watching over me and got myself and my son there safely. My mom put me to bed and told me I would feel better in the morning. I hoped she was right, but in my gut I knew she wasn't.
When I woke up the next morning, the headache was still there. After 9 days of stressing and feeling like I was never going to be better...I was suddenly at peace. It was surreal...I remember vividly the color of the bedspread on my old bed...I remember when I walked outside the feeling of fall. I remember seeing my niece sitting at the kitchen table eating toast and smiling and talking about Maggie and the “Verocious” Beast. I remember being so grateful that I had been blessed to spend 4 years with my son, and I wondered if he would remember me when I was gone. It was as if someone slapped me...I suddenly realized that I was going to die. And I was ok with that. I walked out on the back porch where my mom was sitting and I sat down. When she asked me how I felt, I told her I didn't feel any better. She told me to go back upstairs and lay down and get some rest and she would take me to the doctor the following day. I will never forget my response, or the look on her face, or the feeling of peace that I had when I said it...”If I close my eyes, I'm not going to wake up. I'm going to die.” My mom immediately picked up the phone and called the urgent care center, and we loaded up in the car and headed to the doctor. On the way there the peace that I had felt before seemed further and further away, and was replaced by something new...panic. It crept up slowly. I felt a feeling in my stomach that started as a quiet hum, and then begin to grow rapidly...it bubbled over and began to take hold of my whole body. My mind was racing...who would take care of my son? Who would take care of my mother when she was old? Would someone tell my son how much I loved him and how he saved me from the person I was becoming? Would he remember the lullabies I sang to him before bed, or the plans that I made for our life? Would he tell his children about their grandmother? Or would he just forget me and struggle without a mother to comfort him? The feeling was terrible...it was as if every fear I had was crushing down on my heart all at once, and I felt completely powerless to stop it.
Meanwhile, my mother pulled into the parking lot of urgent care. Suddenly it was as if I had been hit by a train. My left arm and leg went completely numb. I begin to panic outwardly...screaming to my mother that I couldn't feel my arm. She put the car in reverse and headed to the ER which was right across the street. I could see my mom physically shaking and I felt so bad for her...I remember feeling so guilty for making her so scared.
We walked into the ER and I sat down while my mom checked me in. They didn't react...just told her to sit down and they would call us shortly. As the time ticked by, I began to lose feeling in the side of my face. I remember crying and wishing that they would hurry up. Finally they called us back. To fill out insurance papers. I remember feeling so confused...I'm dying, and they're asking for my insurance card!? As I was trying my best to answer the girl's question about my employer and my social security number, my tongue went numb. I began crying hysterically telling my mom that I couldn't talk, I couldn't feel my left side or my mouth. I've never seen her so afraid or so helpless. If the terror in my eyes wasn't apparent, I know the insurance girl could see it on my mother's face. She kept apologizing to us and telling us that it would only be a few minutes. Finally after what seemed like 10 years, a nurse came back and stuck us in a room. Again we waited...and when she finally returned she asked me a million questions that I didn't understand. Was I sexually active? Could I be pregnant? Did I have a history of depression or anxiety? The list went on and on. I remember looking at my mom and wondering why she was asking me these things. I remember looking in the corner of the room and seeing a dead baby hanging from the curtain, dressed in red...a halo around my mother's head...a woman in purple standing below the dead baby with a cane...a red “W” written across the forehead of the nurse asking the seemingly pointless questions...all things that I have never told a soul, until now. The images were terrifying, but for some reason I knew they weren't real and I did my best to hide the fact that I felt as though I was losing touch with reality. The nurse was emotionless...too busy today for this girl acting like something was wrong with no visible signs of trauma. She told me that I might need to be admitted to the psych ward for an evaluation and it looked as though I was having a panic attack. I looked at my mom and said “I am NOT crazy! Something is wrong...why is she acting like I'm crazy?” My mom shook her head and said she didn't know. Again we waited...they asked me to give a urine sample so they could determine whether or not I was pregnant. I went to the bathroom and saw snakes crawling the walls and butterflies in the toilet. I looked in the mirror and wondered if maybe she was right...maybe I was going insane. I tried to shake the mental images of slimy snakes and drowning butterflies, but I couldn't bring myself to give the sample. A nurse stood outside the door and kept knocking telling me to hurry up. I just couldn't do it. I walked out with an empty cup and she rolled her eyes and told me that they would have to do a catheter. They did...and they drew some blood. Later, they came in and drew more blood. The nurse seemed a little more concerned and came in several times to tell us they were working on it and the doctor would be in shortly. I remember an older doctor coming in, asking a few questions and then leaving. And then I remember my knight in shining armor...a young doctor with dark hair and a nice smile. He came in and calmly asked me some questions. He looked at my legs where the rash was and told me it was called petichae...he was the first person that had taken the time to look me over from head to toe, and the first doctor to notice the bruising. I remember being told that they were planning on giving me blood because my counts were so low, and if they had it would have killed me. The angel doctor just happened to be in the right place at the right time...he had done a little bit of study on a disease known as TTP while he was in med school. It was a rare disease with only one known effective treatment, and the hospital wasn't equipped to do it. The older doctor had never heard of TTP...but after a quick briefing by the younger doctor and a look in a medical journal he told my mom that they would be transferring me to a larger hospital nearby to begin treatment immediately. When my mom asked if I would be ok, he said “some people survive”. I wasn't around when he said that, but I can only imagine how my mother felt.
The next few days are a blur...I remember being loaded into the ambulance and crying that I wanted to go home. The nurse walking beside the stretcher cried with me. I remember the girl in the ambulance asking me about my son and if I had any special plans for him for Christmas. She was nice.
I spent a few days in ICU...I remember a wonderful nurse who, oddly enough, was named Hope. She was very kind and reassuring. When the doctor came in to put in my line in my leg, she held my hand and shed tears with me when he struggled to get it in. I remember my father coming in and brushing the hair off my forehead. I met “my angel” as I call her when I was in ICU. She was the apheresis nurse who gave me my first treatment. It was the middle of the night when she walked in...the rest of the ICU was quiet and the lights were dimmed. I was wide awake...scared out of my mind because my mom wasn't allowed in the ICU but for minutes at a time. She walked in and the whole feeling of the room changed...it went from stark and cold to warm and somewhat comfortable. She explained to me that she was going to start my plasma pheresis treatment...and then she sang to me. I will never forget how her voice soothed me.
I spent a total of 10 days in the hospital. My mother rarely left my side. Without her, I don't know how I would have gotten through it. She was, and still is, my rock. While I was there, I met some amazing people who touched my life forever. While I hate the circumstances under which we met, I feel very blessed to know them and I attribute the fact that I am still here to their dedication to their patients and their never ending research. I continued to get treatment on an outpatient basis, and eventually had my portacath removed. The side effects from the prednisone were almost unbearable...I had terrible hot flashes and night sweats, and the intense mood swings were hard to control. I gained 40 pounds from the steroids, and my face was enormous. My family and I love looking back at pictures from that time and laughing at how fat my face got. :) (Just a word of encouragement...the “moon face”, as my nurses called it, goes away eventually.)
It's been 7 years this September since I was diagnosed with TTP. I have since married the love of my life and given birth to two more beautiful children...a boy and a girl...and I have one more baby who is in heaven. I have been lucky enough to have avoided a relapse. In 2011 my doctors decided that since my levels were low during my last pregnancy that I would benefit from a rituxan treatment. They were right...my levels are higher than they have ever been and I continue to stay in remission.
My life is much different since TTP...I constantly worry when I get a headache or a bruise, wondering if this awful disease is creeping back up on me, but I've been lucky so far and it's kept it's ugly head down. My energy level is low...I tire easily and I feel as though it takes me longer to recover from daily activities. I have a lot of joint pain...but no one seems to think it's related to the TTP. I also have extreme anxiety issues and mood swings, but I have been working with my PCP to get these issues under control. In the past 2 years I have been diagnosed with ADD, OCD, and post traumatic stress disorder which she believes stems from my parent's divorce and my TTP experience. All of these issues are silent and unrecognizable to everyone else but me, which makes dealing with TTP all the more difficult. With such little research on this disease, we just don't know what is “normal” for those of us in remission. My doctors tell me that my low energy and joint pain aren't related, but when I speak to others who have been through this journey as well, they all claim to have the same issues. It's my belief that TTP does more damage to our bodies than it is given credit for.
In spite of TTP and all of the issues that come along with it...I am ALIVE. I am here to raise my beautiful children, be a wife to my amazing husband, be a daughter to my saint of a mother and my dear father, be an aunt to my beautiful niece, be a sister to my incredible brothers, be a friend to my wonderful sister-in-laws...all of which are the most wonderful jobs I could have been blessed with. I am a survivor. While TTP is a huge part of my life, I won't let it define me, and I am here to share my story in the hopes that it will encourage and inspire those on this journey. You CAN beat TTP...you can become a survivor and share your stories too...and hopefully one day our stories will help raise awareness and fund the research needed to help others afflicted. Good luck, best wishes, and high platelets to all of you fighters!! xoxo, Rachael
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I was very young when Rachael got TTP and I don't remember much other than seeing Rachael in the front seat of the car on the way to the hospital telling my nana that something was really wrong. I don't remember much but I do know that I am thankful for my aunts "Knight is Shining Armor". If it weren't for him I wouldn't know, remember, or have the best aunt in the world. (: My mom also wrote a short story of what it was like for her, because, like me, she is very close to Rachael.
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My Mom's Story
It's been almost 7 years since the day I almost lost my sister and best friend to a blood disease called TTP. I'm thankful every day for that one person who had just enough knowledge of this puzzling disease and happened to be in the right place at the right time. In the end, it took many people working together to make her well again, however if it were not for Rachael's "Knight in Shining Armor", as she calls him (I just call him HERO), she would not be here today.
Thrombotic Thrombocytopenic Purpura is a rare autoimmune blood disorder. TTP is diagnosed at a rate of 3-4 in 1 million people per year. Potentially fatal complications can result from internal blood clotting with damage to critical organs such as the brain, heart and kidneys. Without treatment, TTP has a 95% fatality rate. On the other hand, with treatment 80-90% of TTP patients achieve remission. Of these, about 30% will relapse.
It was knowledge that saved my Sister's life. It is also knowledge that continues to keep her in remission. Knowledge is power…so they say! I'd like for you to consider Donating to the Answering TTP Foundation in Rachael's name…by doing so, you can give the gift of knowledge. In turn, you could help to save the lives of other people who suffer or will suffer from TTP. Whether or not you are able to make a donation, you CAN help to raise awareness. Join us for the Walk to Answer TTP on September 22nd. Rachael's Team members will receive a free TTP Awareness T-shirt so that we can walk in style. If you plan to walk with us, let me or Rachael Dolan Everhart know so that we can get you registered as a part of her team!
Any donation that you are able to make and/or having you there to walk with us on September 22nd will mean the world to Rachael, our Family and the many other people who will benefit from your generosity and desire to raise awareness about this disease.
***Follow the link to read Rachael's story, find out more about TTP, say "You'll be there" and get all the details about the walk and to find out how you can make a donation to the Answering TTP foundation in Rachael's name.***
Thank you so much for your support!!
P.S. If you can't walk with us and you are unable to make a financial contribution, YOU CAN STILL HELP!! Visit your local RED CROSS for information on Plasma donation.
TTP Patients can LIVE with the PLASMA you can live WITHOUT.
