OUR JOURNEYS

Patient story

Nicole Shirey

"Being a healthy patient was something I was proud of. I still feel that I am a healthy patient, but in my own way."

- Nicole Shirey

My whole life I have considered myself to be a healthy person. I didn't always have the best diet, but I've always been an active person and hard worker. It was so nice to tell doctors that I wasn't on any medications and that I didn't have any past surgeries or medical conditions. Being a healthy patient was something I was proud of. I still feel that I am a healthy patient, but in my own way.

After being a stay-at-home mother for six years, I decided to return to work. I was a part time maid, so I was always in and out of the heat or cold, depending on the time of year. In the winter I would get really cold and my fingers would turn white and be numb. It was hard to drive and it took a while inside homes to warm back up and be able to use my hands. During the summer I would get rashes on my arms and legs from the sun, but I thought it was just a reaction to my birth control pills. At first, I didn’t think much of these symptoms. We all have little quirks and these were mine. I could handle those annoyances, and never got them checked out.

"At first, I didn’t think much of these symptoms. We all have little quirks and these were mine. I could handle those annoyances, and never got them checked out."

- Nicole Shirey

I left that job after a couple years to start working full time as a receptionist for a chiropractor. It was a great job and he knew a lot about everything in the medical field. When he noticed my rashes one day he called a personal friend of his who was a PCP, in Internal Medicine. He came to our office and checked out my arms and then referred me to a dermatologist. I went to the dermatologist, thinking I would walk out with a cream. They tested me for Lupus (an Auto-Immune disease). You have to pass 4/7 tests to confirm that you have it. I did, and was diagnosed with SLE (Systemic Lupus Erythematosus) at the age of 26. My husband was devastated. I on the other hand, thought it's no big deal; this is something I can live with. I was sent to a Rheumatologist and she put me on Plaquenil. I really didn't have symptoms besides skin rashes, increasing fatigue and Raynauds Syndrome (a condition where your body doesn't send enough blood to your hands or feet, so they feel very cold or numb). I had been told that since the SLE had invaded my bloodstream that there was a possibility of it attacking my organs. The liver and kidneys are the most common to be affected. I wasn't too worried about that happening and in the meantime got pregnant and had my second child. I also started working in another medical office. I had an episode where I went to the hospital and was diagnosed with Pericarditis (a condition in which the sac-like covering around the heart becomes inflamed), which happens often with SLE. I took off work for a week and started seeing a Cardiologist. I was then told I had a slight murmur and small hole in my heart called Atrial Septal Defect (a heart defect in which the wall that separates the upper heart chambers does not close completely). I was told that I would need to be monitored yearly, but otherwise it wasn't much of a problem at the time. Again I thought that it was something I could deal with and didn't change much about my life.

"It wasn't that I wasn't physically able, but I couldn't breathe. How could this be?"

- Nicole Shirey

A year later I was with my friends at the Sand Dunes and was unable to walk up them. It wasn't that I wasn't physically able, but I couldn't breathe. How could this be? I was always fairly healthy and had been working out regularly. I went to the Doctor and he said I had bronchitis. I was given an inhaler and put on an antibiotic. I started noticing that I would get short of breath anytime I bent down, went up a flight of stairs or did anything active. Since I have SLE, when you get sick it breaks down your immune system, so I thought that was all it was. I went back to the doctor at least 3 times for check-ups and kept complaining of my problems. He put me on different medication and inhaler and said if those didn't help that he would refer me to a Pulmonologist. My body had different ideas. I got up for work one day and felt especially bad. I was short of breath (this was usual at this point) and also having chest pains. I thought that it might be Pericarditis again. I asked the nurse at our office for her opinion and she said to call my cardiologist. I was instructed to go straight to the hospital, but I wasn't in terrible pain and decided to continue my day.

I went to the local ER after I left work. They couldn't find any issues and were getting ready to send me home, but let me eat while I was waiting to be discharged. The cereal I had didn't sit well and I began throwing up. My oxygen dropped to 78%.

I went from getting ready to go home to being transferred up to the ICU with a full oxygen mask on. I was there for a couple days but was told I needed to go somewhere that dealt with these symptoms more often and would be transferred U of Miami. I was glad to be going somewhere to figure this out so I could get back home and back to work. After a lot of testing was done, they found out I had a flare of SLE which attacked my heart and lungs.

I was diagnosed with Pulmonary Hypertension at age 30. I was told that when they take your blood pressure, they only measure the pressures of the left side, and that PH wasn’t detected because it is a problem in the right. While I was there one of the doctors pulled my mother aside and told her that I wasn't taking any of this illness seriously and I was in denial about it. After a week in that ICU I was finally sent home on a lot of new meds (Revatio, Aldactazide, Digoxin, and Coumadin) and Oxygen 24/7. A month later I was also started on Letairis.

"My life would never be the same. I had to stop working and wasn't able to take care of my kids on my own. I couldn't even take care of myself. It has been the hardest thing in my life thus far—letting someone else take care of me."

- Nicole Shirey

My life would never be the same. I had to stop working and wasn't able to take care of my kids on my own. I couldn't even take care of myself. It has been the hardest thing in my life thus far—letting someone else take care of me. My husband, mother and step-father have all been incredibly helpful and supportive. I've had to let go of a lot things I used to enjoy doing, and my kids will never know the energetic and active person I used to be. I still hope for a normal life again someday.

After a year on these new drugs, I was still having trouble with my oxygen levels and activity, so I decided to try Ventavis. I had horrible trouble with coughing during the treatments, so we had to stop that. I was then told about Remodulin Sub Q. I was very scared initially, but it's been the best thing for me. Since then I have made a huge improvement and was able to drop the oxygen to just wearing it overnight. I also had trouble with my sinuses and traded the Revatio for Adcirca. My PH is stable with the meds I'm on, although the SLE has been giving me issues now. I've added Imuran, Cellcept and just recently started Benlysta, an Infusion therapy. I still have a hard time walking uphill or carrying anything heavy for any period of time, but I'm finally able to do chores around the house on my own and grocery shop by myself.

Those little things may seem trivial to some but I was so glad to be able to do them again. I've always told myself that things could be worse and I'm lucky for the problems I have. It's been a long road, but I'm thankful everyday that I'm still here to be with my family and watch my kids grow up. I've never believed I was in total denial, but I was in partial denial. I know I'm sick and I deal with it every day. I don't like to dwell on it, heck I don't even like talking about it. I decided to share my story because I do want other people to hear it and maybe it will help them with their own journey.

 

 

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The National Organization for Rare Disorders (NORD) awarded PHA the Abbey S. Meyers Leadership Award in 2012 for outstanding service to PHA members in advocacy, education and other key areas.