EA: Ebsteins Anomaly (Rare But Real)
I am very familiar with EA, which is the abbreviation for Ebstein’s Anomaly. My knowledge on EA is the result of having my son being born with it.
It is not something that can develop later, it is congenital, which means that a person is either born with it or not. EA occurs during the formation period of the heart.
In many cases, it isn’t diagnosed at birth and are most likely a mild malformation. The definition in layman’s terms is that it is a malformation in the tricuspid valve, which separates the right sides’ atrium and ventricle/top and bottom.
Ebstein’s Anomaly is diagnosed on a scale of mild to severe, depending on degree of malformation. Below is a diagram of a “normal” heart, showing correct blood flow.
As you can see, the right side of the heart is designed to take the used blood from the body to the lungs to be oxygenated. If the valve is malformed, it cannot fully handle the job.
Some EA patients don’t even know they have it until later in life and that would indicate that it is a mild malformation. For many of these EA patients, it will require only medication. In a moderate form of EA, the valve can be repaired by the cone procedure or can be replaced with a mechanical valve.
In severe EA cases, reconstruction and/or replacement are not options. This is the case for my son, Kyle. He was born just over 20 years ago and it has been one hell of a roller coaster ride. Below is a diagram of my sons’ heart after the Fontan procedure.
As you can see now, the blood is re-routed to bypass the right side of the heart. It comes from the body and is shot straight to the lungs via the pulmonary artery. It then comes back to the left side of the heart and it is pumped to the body.
The left side is doing all the work from this moment on, which can have a profound effect on the whole body. My son developed WPW, which is the abbreviation for Wolff-Parkinson White Syndrome. WPW is an electrical abnormality and can cause the patient to go into SVT, which is tachycardia.
Kyle has experienced heart rates above 200 bpm and, in couple of cases, given medication to stop his heart in hopes that it would reset itself. Crazy sounding but it has worked a couple of times now. It is scary for the both of us and Kyle describes it as just feeling weird. Sadly to say, there’s more. For instance, my son developed a DVT (blood clot in left leg) while on blood thinners.
It is a rough road but, well worth it all to have my son in my life everyday! I write this not only to inform, but also give hope to the many families that have to live with such a rare heart defect.
You will see many graphic things, things no one should ever see let alone endure. You will feel guilty, trying to find reasoning. You will hear others’ stories and, depending on the amount of time in the hospitals, empathize with them. Leaving you to feel guilty as they lose their child, or in my case, children.
The Intensive care Unit are pods of about 8 families. I say families because a couple behind me and my son lost their triplets one by one. It was awful. Didn’t want to leave my son, but felt so guilty because my baby is in serious but stable condition.
One thing that you will find hard to see is the children there alone. Ones you never see anyone visit. I personally wouldn’t leave Kyle, so afraid that I wouldn’t be there and him not make it.
He has surpassed the doctors expectations and he is still teaching them. Faith and technology combine are mind blowing!