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For how long have you been suffering from hypoplastic right heart syndrome?
Hypoplastic right heart syndrome is a birth defect known as “congenital heart disease,” which causes abnormalities in the heart’s structures.
In my case, the right side of my heart didn’t develop completely. The right ventricle is responsible for pumping blood to your lungs. Since I don’t have a right ventricle, I needed open-heart surgery as a baby and then as a teenager to survive.
I’ve also taken heart medications throughout my entire life to prevent heart failure.
How was it diagnosed?
Today, newborn babies get checked for heart defects right after birth with a test called “pulse oximetry” or while in utero when the mother gets their 20-week anatomy scan and baby’s heart can be scanned.
Unfortunately, when I was born in 1987, these tests didn’t exist and I was sent home without getting my heart checked.
As I grew, my mom and her friends noticed a bluish tint on my skin when I cried. This is known as “cyanosis,” which occurs due to a lack of oxygen in the body, often during an outburst. This puts increased pressure on the heart, making it harder to pump oxygenated blood. (3)
My mom was concerned already but her friends’ comments convinced her that something was wrong with me.
When did your family consult the doctor?
After noticing worrisome signs when I was 3 months old, my mom took me to a pediatrician to get a thorough checkup.
After doing an echocardiogram, the doctor said something was wrong with my heart. However, he couldn’t make a diagnosis, and so he referred us to a pediatric cardiologist.
The cardiologist ran more tests, and I was finally diagnosed with seven different congenital heart defects in the right ventricle that together make up hypoplastic right heart syndrome.
What causes or increases the risk of hypoplastic right heart syndrome?
The statistics on congenital heart defects (CHD) indicate that 85% of heart defects have no known cause. The remaining 15% are caused by genetics, which means they run in the family. (1)
In my case, I was born with heart disease because both my mom and dad were born with heart defects as well.
Although a heart-healthy mother can do everything in her power to carry a healthy child, CHD is random and even the healthiest families can give birth to a child with CHD. It doesn’t discriminate and can happen to anyone.
This is one reason I’m so passionate about advocating for CHD to increase awareness about this condition because an undetected heart defect can result in death.
I fight for awareness every day, so babies won’t go home undetected like I did. I was surprised I lived with a single ventricle for 3 months after birth without any kind of treatments!
What treatment were you given after your diagnosis?
In order for me to survive with “half a heart” as they call it, I needed to get a series of open-heart surgeries to reconstruct my heart’s circulation so it can pump blood to my lungs.
Usually, patients with a single ventricle like me get three open-heart surgeries, namely, the BT shunt, Glenn shunt, and Fontan procedure. (4)
These heart surgeries involve stitching artificial tubes and conduits to the heart, specifically to the main coronary arteries, which are then connected to the pulmonary artery to send blood to the lungs.
These are “palliative” surgeries, meaning they’re not permanent. These parts tend to wear out as the patient grows into adulthood, and they may need a heart transplant in the future.
I’ve taken heart medications since I was 3 months old. I will take heart medications for the rest of my life because there is no cure for congenital heart disease and it requires lifelong care and treatment. (5)
How has it been living with hypoplastic right heart syndrome?
I am not going to lie or sugarcoat and say that living with hypoplastic right heart syndrome is easy because it’s not. It’s very challenging honestly.
My journey with heart disease has been quite long, hard, painful, and most of all, full of uncertainty, but I have always tried to remain positive and hopeful through it all, no matter what the doctors say.
What was your biggest challenge throughout the treatment?
My biggest challenge throughout my journey is accepting that I was born sick. I don’t know how it feels to be healthy.
During my childhood, I didn’t really understand what was happening to me, but I knew I was different from the kids around me because I couldn’t keep up with them physically and that made me feel alone.
Growing up with a chronic illness can be isolating and can lead to depression, which I’ve struggled in the past.
How would you describe your whole journey and what kept you motivated?
Even though my life with hypoplastic right heart syndrome had been devastating at first, there’s light at the end of this dark tunnel. I’ve learned to use my strength and struggles to empower others who struggle like me.
I may live with half a heart, but I live my life to the fullest. There will never be enough words to express how grateful I am that I’m still alive at the age of 33 years, considering my heart doctors told my mom when I was first diagnosed that I’d never make it past 5 years old.
What would you say to those who are struggling with the same syndrome?
I would say to never give up and to stand strong, remain hopeful, focus on the possibilities, and stay positive no matter what the doctors are telling you.
When you have faith and the will to live, nothing is impossible. Doctors are not God and they are not always right.
You can definitely have success and lead a normal life with hypoplastic right heart syndrome. Just don’t let this condition take over your life. You control your life!
How can others help people who are diagnosed with hypoplastic right heart syndrome?
I would say to show more compassion for ill people even if they don’t look sick but say they are. I personally don’t look sick but I’ve been through a lot, and I struggle with nasty judgmental remarks from strangers that don’t understand my illness.
Take a step back and think about how the simple daily tasks you do – such as showering, carrying groceries, getting out of bed, or even breathing without being short of breath most of the time – may seem unimportant to you but they can be a huge feat for someone like me who wishes he could do them as easily. The simplest, most mundane activities are big challenges for me daily.
Know that being sick is not about playing the victim or being lazy. For affected individuals, it’s about constantly being on top of what their body needs to feel well enough and muster the energy to take a simple shower.
When a person is born sick, their body is fighting to stay alive 24/7, and that’s exhausting if you really think about it. As a person with an invisible illness, I can say that all we want is acceptance without judgment.