Elie Rogot

First post: Nov 10, 2017 Latest post: Feb 22, 2023
Elie (rhymes with "jelly") was born Nov. 3, 2007, in Frederick, MD.  A couple of hours after her birth, it was discovered that she had a major heart defect. She was quickly flown to Children's National Medical Center in Washington, DC, where it was determined that she had Truncus Arteriosus (type III-B) with right pulmonary atresia, along with a genetic syndrome often called DiGeorge (but which is really a "22q11 microdeletion" syndrome known as Velo-Cardio-Facial syndrome, or VCFS).  Truncus is an exceedingly rare congenital heart defect, accounting for only 1-2% of all heart defects.

Elie underwent an extensive open-heart surgery on Nov. 12 (although her chest was left open for four days to allow the swelling to go down), which was performed by Dr. Richard Jonas, a rock star in the field of pediatric cardiovascular surgery. Elie's repair included closing a hole between her ventricles (a VSD, or ventricular septal defect), detaching her aorta from the pulmonary artery trunk, and attaching an artificial conduit (a donor homograft) to act as her new pulmonary artery trunk and bridge to her nearly nonexistent right pulmonary artery ("pulmonary atresia"). Elie was in the OR for nearly six hours, but the surgery was a total success.

After that, Elie spent two weeks in the cardiac intensive care unit (CICU) at Children's; she was kept in an induced coma for nearly a week, complete with induced hypothermia to keep her blood pressure under control. (Yes, it was as scary and horrific as it sounds.) She was also in something called JET (junctional ectopic tachycardia) for four days following surgery, meaning that her heart's electrical system was misfiring because of the stress of the procedure. THANK GOD it resolved itself.

After her time in the CICU (whose phenomenal staff was incredibly knowledgeable, compassionate, and competent), Elie graduated to the Heart-Kidney Unit (HKU), where she spent another ten days learning to become a regular baby. Finally, FINALLY, on Dec. 5, Elie came home!!

While Elie will need more cardiac procedures, her doctors expect her to live a relatively normal, active life. As one doc put it, "She'll never be an Olympic athlete." To which Mom and Dad replied: "Not even in the luge?"

We also don't know what the future holds as far as Elie's VCFS issues. We DO know that, so far, she has dodged nearly all of its bullets. 

In early June 2008, Elie was diagnosed with pulmonary hypertension (high blood pressure in the lungs), a serious condition that *used* to be considered fatal, but which is now seen more as a chronic illness. She now takes bosentan (aka Tracleer), Digoxin, Lasix, sildenafil (aka Revatio or Viagra), and aspirin, and she's responding wonderfully to all of them. Also, in something of a miracle, her PH appears to be unilateral -- affecting only her left lung. (Update on 10/15/17: She *seems* to have beaten PH. We're wary of saying it out loud, but that seems to be the case.)

(Update #1: Elie had her conduit replaced for the first time on 2/26/10. She sailed through the procedure and was back home after four nights at Children's -- only two of them in the CICU. She was also able to go off the Lasix soon after.)

(Update #2: After an aggressive, bold cath, Elie now has a larger RPA but has lost -- forever -- the use of the upper lobe of her right lung. See the CarePage update from 8/31/11 for the details; reading it still makes Mom and Dad want to puke from nerves.)

(Update #3: Elie had a mechanical heart valve -- a Melody valve -- inserted via cardiac cath on 2/26/16. It was her 11th cardiac cath/angioplasty, and we're hopeful it will buy her a few years before her next open-heart surgery. Elie's 22q-related cognitive issues are also becoming more apparent as she gets older (she's in second grade now and quickly falling behind in math), but she's still the same wonderful kid she's always been. We look at her nearly every day and think, "She's the best kid in the world.")

Overall, Elie is a happy, well-adjusted, compassionate kid, despite all the drama surrounding her entrance into the world. Mom and Dad have aged in dog years since her arrival. She's the light of our lives and makes us laugh every single day.

(To get a sense of how gut-wrenching it is to be a "heart parent" -- or the doctors and nurses who take care of heart kids -- read "Walk on Water" by Michael Ruhlman. It's about the world of pediatric CV surgery, and it's outstanding -- even Dr. Jonas gets a few shout-outs in it.)

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