VACTERL: What It Is Exactly

I, along with my husband, was born with. condition called VACTERL. Back in the late 80s, it was called VATER. Sometime in the late 90s/early 2000s, more letters were added.

Each letter stands for something. You have to have 3–4 out of 7 to qualify, I have all 7. The VACTERL community calls that “winning the lottery”.

Breakdown

V: Vertebrae: I have scoliosis. It’s made me really short — 4′ 5″. When I get asked if I’m really that short (I wear heels 90% of the time and I do my hair like Dolly Parton), I say yes, I’ve escaped from the Lollipop Guild and I’m a wanted woman in Oz.

A: Anal: This can be a range of colon deformities, but it usually is an imperforated anus — basically we’re usually born without buttholes and they have to be surgically created. Some of us have extensive bowel regimens. I’m very lucky that although I had to have my anus reconstructed, my bowel function is pretty normal.

C: Cardiac. I was born with a series of defects — ASD, VSD, Pulmonary Stenosis and a form of tetralogy of fallot. All were corrected when I was 13 months old, and although I require regular checkups, my heart is doing fine.

T/E: Tracheal/esophageal. Basically, my trachea and esophagus were born wrapped around each other. When they tried to feed me, I aspirated into my lungs. My breathing is okay, but eating was a challenge as a child and I had a feeding tube in my stomach until I was about 9. In the past five years, I’ve had regular esophagus dilations done because my esophagus narrows at the bottom, causing food to collect and me to choke. It’s annoying, but manageable.

R: Renal. I was born with one really big kidney on my left side and no kidney on my right. The left one is a form of a horseshoe kidney, and it has two collecting systems. It’s unique and some doctors call it one kidney, some call it two. I catheterize through my bellybutton and have since 1999, when an amazing doctor named Doug Coplen created a bladder made out of colon for me. Before then, I had an ostomy, where I peed in a bag on my side. I definitely prefer the catheter route — I can pee anywhere. Definitely a plus.

L: Limb. My right arm is shorter than the left by 3–4 inches because it’s missing it’s radial bone. I am missing my thumb as well, although I can type just fine. (I can’t knit though, that really does require two opposable thumbs.)

There have been some other complications that don’t strictly fall into a letter category. My reproductive system was unusual, and I had a double uterus. That’s been taken out. My ovaries have given me some issues, and as of December 2020, I’m down to one functioning one. I’m good with that. I also have a vein deficiency issue — because of all the IVs I’ve had since my birth in 1986, my veins are pretty much gone. From the age of 17 to 29 I had three ports (permanent IVs implanted in your body, usually used for cancer patients) and then took a five year break, getting my latest one in May 2021. I’m hoping I can keep this one for the long haul. I get frequent bladder infections due to the cathing and the way my system is built, so I frequently need IV antibiotics. The port is the best way to go about that, otherwise it can take many tries to get an IV. The record is March 2017, 34 sticks in 4 hours. Never again.

Genetic or not?

We don’t know if it is genetic, but studies are being done. Some say yes, some say no. I’m on the yes train, because I had a great aunt that died when she was three of issues that — on her death certificate — sound like a mild form of VACTERL. Others say that it was a fluke, an environmental factor, or something involving the mother’s health (I do not blame my mother, nor my father, for this, for the record. While I think it’s possibly genetic, I don’t blame them.)

The benefits:

Believe it or not, having VACTERL is actually okay. I mean, I’ve had over 150 surgeries and I’m on a first name basis with five doctors, but on the upside, I have a tremendously supportive community. Most of my closest friends have it — I met them online or at conferences. My husband has it — I met him in 2005 at a conference in Denver, and we married in August 2011. His is a much lesser form, but he still completely understands and is the best husband in the world. Also, when I get sick and I don’t want to go to the hospital, he’s been known to carry me to the car. I still live a full life. It’s not a “normal” one by any means, but it’s still full and rich and perfect.

Originally published at http://emilyhendricksjensen.com on August 10, 2021.