There is still much unknown about adult Congenital Heart Disease Patients and the long term effects of their treatment in childhood. This week, Marissa, a single ventricle patient about to complete her nursing degree, shares her story of an unexpected complication, which almost cost her future in nursing.
I call myself a “heart patient”, but why? Sure, I was born with six complex congenital heart defects, but my heart disease effects more than just one body system. As a 21-year- old Single Ventricle Fontan, I’ve always had my routine EKGs, echocardiograms, stress tests, halter monitors- you name it. Because I am a fontan, I’ve been made aware of the high likelihood of developing liver cirrhosis, so I have liver ultrasounds each summer. There can be issues with pregnancy, exercise tolerance, mental health disorders, and an overall a deterioration of the Fontan circulation. I’ve always been very realistic and knowledgeable with my own health condition, which is why I was so surprised when I heard my doctor say the words “you have T-cell Lymphopenia related to your Fontan circulation”.
In November of 2016, I underwent a diagnostic endoscopy that yielded abnormal findings. A week later, I found out I had an infection lining my esophagus. I presented with no symptoms and had been completely unaware of the infection. The treatment was simple, a two-week course of oral antibiotics, but my doctor felt I needed to undergo further immunology testing, considering this was an opportunistic infection. After three separate lab studies showing low immune cell counts, I was referred to an Immunologist in Philadelphia. Since then, I’ve seen three separate immunology specialists.
T-Cell Lymphopenia is a secondary immunodeficiency that results in decreased levels of T cells (infection fighting cells) within the blood. The high pressures within my circulation do not allow my lymphatic system to release the proper amount of T-cells needed within a healthy individual. For me, my case is mild and was caught early, however; the newfound diagnosis almost ended my career as a nurse before it even began.
When I finally received my official diagnosis, I was in my third year of nursing school. I was attending clinical rotations twice a week, and I was already working in a hospital setting. Many specialists were unfamiliar with my diagnosis in relation to my childhood heart surgeries. Before I began my senior year of school, I visited a new doctor who advised I avoid patients with any form of a transmittable illness. I was advised to wear a mask in crowded spaces, to avoid friends or family members who were ill, and I was given immunizations intended for those with a weak immune system. Having these new restrictions was difficult, and it was a daily reminder that I will always fight this chronic illness; I’ll never be considered
When starting my last year of nursing school, I was told I couldn’t complete the program with the accommodations my doctor required. I was devastated to find that I may not be able to graduate with my nursing degree, and I may not be able to fulfill my passion to work as a nurse. I had spent three years studying and preparing to succeed in my future career, and it all came to a halt on a warm August day. I’ve always tried to treat myself as normal as possible; I’ve never wanted my congenital heart defects to define me as an individual. I knew this career would be tough, especially for someone with a single ventricle. Nursing school is the most challenging thing I’ve ever done, but hearing that I wouldn’t be able to graduate because of my heart was the worst part. No matter how hard I studied, how well I performed in clinical, and how well I prepared for my future, that didn’t matter anymore. With this crushing reality, I requested a third opinion from a pediatric specialist, hoping this physician would have more experience considering Congenital Heart Defects are from birth.
Thankfully, after visiting a third immunologist, I now have no restrictions related to my T-Cell Lymphopenia. I will require follow-up care to monitor my T-cell and vaccination levels, however, I have NO restrictions in the clinical setting. I’m allowed to finish my degree to fulfill my dreams and work as a nurse. I’m incredibly grateful to have been able to see a specialist who was familiar with my diagnosis. Apparently, T-Cell Lymphopenia is present in a small percentage of Fontans by the time they reach their early 20’s.
CHD does go beyond the heart, and affects the body in various ways. It’s important that we as patients receive lifelong care, but this can be challenging when complications arise outside of the heart. Although it took over a year, I’m thankful to have received the proper diagnosis and plan of care. I want to inspire others to be an advocate for themselves and to not give up on their dreams. Life can get difficult sometimes, but through determination we can reach our goals,even when taking an unconventional path.
Marissa Mendoza lives in New Jersey and was born with six complex congenital heart defects. She was diagnosed with a Ventricular Septal Defect, Congenitally Corrected Transposition of the Great Arteries with Ventricular Inversion, Pulmonary Atresia, Bilateral Superior Vena Cavas, and Dextracardia. She underwent three open heart surgeries before the age of two, now classifying her as a Single Ventricle Fontan. She is a senior year nursing student graduating in May, 2018 and has a passion for working in pediatric cardiology. Marissa has a lifelong goal to increase awareness of Congenital Heart Defects and serve as an advocate for not only herself, but for the millions of others facing this chronic condition.