I have a disease called Juvenile Recurring Respiratory Papillomatosis, also known as RRP. RRP is a severe breathing condition where papilloma’s grow on the vocal cords/airway and block it. It can also cause narrowing of the airway.
Here is my story:
When I was born, I contracted RSV which is a virus/cold that is theorized to have caused the RRP virus to become non-dormant or active. After contracting it, my cry was never quite right, very soft. At 6 months old, I was at the Outer Banks in North Carolina with my family. My great grandma noticed that I had dropped my pacifier/binky and visibly I was crying but no sound came out. Silent Screams. Can you imagine your child crying and no sound coming out?
I was evaluated at 6 months and referred to multiple ENT doctors. At 9 months old, I was dx with RRP and immediately scheduled for my first surgery as 90% of my airway was obstructed with papilloma’s. I had to have surgery every 4-6 weeks, sometimes every 2 weeks. They utilized a laser that burned off the growths. This might not seem important currently, but it comes into play later in life.
My parents tried multiple homeopathic, cabbage juice, etc. At age 2 I was placed on alpha interferon which allowed me to spread my surgeries out on avg 8-10 weeks.
Around age 5, I started to have trouble walking. I was dx w/ discoid meniscus and had surgery although, my mobility never returned to normal. I was dx with mild CP, later it was dx as spastic diplegia meaning that my muscles are contracting when they shouldn’t be. Very stiff movements. This was from the interferon therapy. My parents tried to take me off the interferon but I experienced a drastic relapsed of papilloma’s and doctors told my parents it was either give her a trach or put her back on interferon. They made the difficult decision to put me back on the medication. When I was 8 years old, they discovered I had been receiving an overdose of the medication for 1-2 years. My legs worsened and I had to do PT and Botox in my legs, wear night braces.
My parents brought me to Pittsburg for surgery and cidofovir treatment, but it ended up causing scarring on the vocal cords along with the usage of the laser and I was never able to talk above a whisper again.
At age 9.5 I was brought back to National Children’s, because there was no improvement with the drug. I was put back on interferon. I got frequent headaches, so badly that I could not function at points. Once removed from the interferon, the headaches stopped.
On 2.20.02 I had my 76th surgery (11 years old). There were more than 20 growths found. They were afraid to remove them from the trachea. More clinical trials happened, I went to Norfolk. They put me on a cancer drug that had showed improvement in others. Methotrexate.
I missed school, came down with mono, stomach aches, headaches.
In 2009-2010, I experienced a cold that would not go away. I remember waking up and still having this cold after 3 weeks. I got in the shower to clear out some congestion, I had to drive my brother to school. I was running late already. I stepped out of the shower and put my robe on, it has green frogs all over it. And suddenly, I was gasping for air. I started to get dizzy and weak. I could no longer stand. Now, my room was located by the stairs. I crawled from my bathroom (at the back of my room) to the stairs. I remember my brother, Spencer a freshman in high School was about 13 or 14 at the time. He is walking back and forth in front of the stairs jamming to his music with his headphones in. I am like SPENCER gasp gasp gasp. He can’t hear me. He can’t see me. My heart sinks I try to not cry for that will make it even harder to breathe. With the little strength I had, I started to bang my hand on the wood stair. I keep smacking and smacking it until he heard it. After about 5 smacks, he flipped his headphones out and I said 911 911 911 please 911. And I watched my brother trembling running up the stairs to me to call 911 and sit me up. I watched the fear consume his body and mind as he tried to process what was happening. He said “my sister she cant breathe, here is our address, she has a condition, send help please she cant breathe” As he was on the phone, my nanny came in yelling at him “you ALWAYS call me first!!!!” And I smacked the floor again and gave her a look and managed to gasp out he did the right thing and she immediately realized how scared he was. She was scared, she wasn’t in control. I was scared, silently, trying to tame the demons in my mind for what could happen. I remember Debbie, my nanny, put me in possibly the tightest clothing that I owned, it felt sticky from the sweat but I was just thankful to be dressed when the paramedics arrived. I refused the hospital and they gave me an epinephrine shot in the arm and left, but said if I came back he would have to take me. I said ok. Leading up to this point, I had been in the ED approximately every other night for the past 2 weeks. It usually helped for a while and then would flair back up. Well, after 30 minutes of sitting up straight at a 90-degree angle in bed. Panic washed over my body, cold yet hot and sticky panic. It wasn’t getting better. It felt worse. I started to gasp again. I held onto my Teddy for life. I gripped the bed sheets as if they could tether me to life. 911 was called again. The medic looked terrified and said, I had a feeling I would be back after I googled and started reading what the disease was. They took me to the hospital in the next county over and I felt ok for a minute or 2 and then the sweat came again. My body overwhelmed to survive and becoming sweaty, everything hurt. I spiked a fever. Quarantine came next due to the swine flu being present even though I never tested positive. The doctor, older man, tried to make conversation saying his son had a teddy bear too. I remember trying to smile but I was scared, I thought, this man has no idea how to treat me. I was air lifted to Johns Hopkins, my father was having surgery there that day. My grandma ended up taking my brother to school and driving to the hospital to see me. I remember it was fall, I had turned 18 just days prior. They asked if I wanted to look at the Ravens stadium, fear consumed me, I felt out of control. I couldn’t look. When I got to Hopkins, a lot became a blur. I remember trying to be strong waiting for my parents. For anyone to not be alone. I held my Teddy, I gasped trying to make sense and keep breathing. I remember telling Teddy, that I was so grateful for holding all my pain. I talked to teddy and said I’m going to make it. I have to make it for Connor for spencer for all my family-they need me. After a month, I was stabilized and went through surgeries. The doctor would not take my case, but I was lucky enough that the fellow on my case knew Dr. Rutter at Cincinnati Children’s and I was referred to him for airway reconstruction surgery. My airway was so scarred and narrow that the scar tissue from that laser had webbed together and prevented enlarging of the airway and breathing. I had everything possibly go wrong with the reconstruction surgery, I coughed out the keel holding open my airway – Dr had never seen that before. I contracted strep and landed up in the ICU 2 times thinking I’m not going to make it. Finally, after 7 surgeries 1 of them being an emergency and nearly an entire month in the hospital, I was released. I started community college. I transferred to Catholic University, ended up not liking it and having some more heath issues. I went to ECU and graduated with my Bachelors. I got married in 2015 to John who is training to be a nurse, to work at children’s, to provide others a life. He is more than I could ask for. I still continued to have keel surgeries, failing each time. Each time, Dr. Rutter redesigned and reengineered it to work better. I got into graduate school at University of Louisville making my way closer to Cincinnati Children’s, for I knew I needed a job with in network health insurance by the time I was 26. I had another keel surgery in 2015 in my first semester of grad school, I thought for sure it would fail out. It didn’t. I worked extra hard; I worked 40 hours a week -maybe more and was a full time graduate student with a full time graduate degree. I graduated with my MPH, which while I wasn’t top of the class or anything, I had made it so far. Knowledge was my control when I wasn’t in control. I have anxiety disorder that I struggle with that has come out of just this life that is a long-term struggle. I got a job at St Elizabeth. I had another failed Keel surgery when I started my job, but it could be fixed and after that emergency surgery I finally got some good news the scar tissue was not there. The scar tissue is back now unfortunately and it is a constant battle.
My advice would be to just be a warrior and ask questions. If something doesn’t feel right let your doctors know. Educate yourself and be your own best advocate because only you know how you feel. I was able to grow up and get my education and obtain a good job in healthcare providing behind the scenes care for others to make sure the quality of their care is good. Be patient as hard as that seems and never give up the fight.