Lucas's CHD Story

JackieMac979
JackieMac979 Posts: 189 Member
edited November 8 in Social Groups
Hi everyone! Welcome to the new CHD group. I hope it helps increase CHD awareness! I thought I'd share my son's story with you. His name is Lucas and he turned 4 in December. Lucas's anatomy is very complex and not a textbook case of any particular heart defect... but in essence, he is a single ventricle hypoplast. Here are some of the major ones in his medical records... usually I just say the first one or two when someone asks me about his heart or summarize it that he has complex cyanotic CHD's.

Transposition of the great arteries (TGA)
Hypoplastic right ventricle (HRV)
Double-inlet left ventricle (DILV)
Ventricular septal defect (VSD)
Atrial septal defect (ASD)
Straddling tricuspid valve
Tetralogy of Fallot (ToF) - on paperwork, but it's not a classic case
Patent ductus arteriosus (typical PDA - this was repaired a week after birth)

Surgical Procedures:
Fontan (Extracardiac fenestrated 4mm) April 2010
Post Damus-Kaye-Stansel (DKS) Anastomosis (modified Norwood) and Bidirectional Glenn (BDG) July 2008. PA Band removed.
Pulmonary Artery (PA) Band and Patent Ductus Arteriosus (PDA) Ligation a week after birth
Balloon Septostomy by cardiac cath two weeks after his first surgery, as an emergency.

He has also had multiple cardiac catheteriations and sedated echocardiograms. He puts up with echos now that he's older.

Symptom: Cyanosis (dusky blue-ness) occurred until after the fenestration created during the Fontan operation closed. He also is very fair skinned and kind of looks like he is tired or has dark circles often. He bruises easily due to aspirin therapy (81mg daily.) Unfortunately he also had IV infiltration once when he was about 6 months old and we had to postpone his second stage surgery until the bad stuff was out of his system and the site healed. (Took a month, ugh!)

All surgeries are "palliative," not curative repairs.

When he was younger he saw more specialists including a pediatric gastroenterologist for reflux/GERD. Feeding has been a challenge his entire life... now that he is 4, it's mainly issues with volume, picky eating, and avoidance of certain textures!

As an infant, we did trials of pumped breastmilk, Similac Advance, and Similac Isomil before settling on hypoallergenic baby formula... ready-to-feed Similac Alimentum as a base, fortified with powder Alimentum and Duocal. I was not allowed to breast feed because it was too much work for him. One GI wanted to put a feeding tube in but I fought that and instead fed Lucas about every 45 minutes. I don't miss that. It was awful. I also kept a pile of tshirts for myself nearby and used full on bath towels as burp cloths. Some days, the reflux was more stressful to manage than his cardiac stuff.

Lucas was put through the Upper GI series (barium swallow) and a milk scan. His anatomy and gastric emptying rate was normal. Eventually, skin testing for allergies was done and he was no longer reactive for milk and soy protein. He also had an endoscopy where biopsies were taken from his esophagus, stomach, and small intestine. He had no inflammation, esophagitis or allergy signs, due to the Zantac EFFERdose and Prevacid I fought for the GI to prescribe. We are no stranger to Furosemide (Lasix) but he only took that as a preoperative diuretic. He was on it for quite a while after the Fontan to help prevent Pleural Effusions.

After he outgrew fortified Alimentum with Duocal, he remained on Pediasure until he was about 3 and a half. I'd still give it to him, but he prefers other fluids and will drink actual milk now. He says Pediasure was for his baby bottles! lol

Current Medications - just 81 mg baby Aspirin daily and tons of vitamins (multi, omegas, and calcium.)

That's a bit about my little guy! He is awesome and I can't imagine choosing any other way, back when I was FAR along in my pregnancy and received the news about his CHDs. My Mom was with me and Lucas's Daddy was overseas. The doctor said... "you have 3 options. Compassionate care/hospice, which means he will be comfortable for a few hours before passing away, transplant (which only lasts 7 years when done for an infant, typically), or three staged surgeries." Obviously the surgical option was the best choice. We have been lucky to not have major complications, but they are always in the back of my mind (in particular, sudden death from sicknesses, or plastic bronchitis.)

We consented to have our DNA studied (as did two grandparents.) The defects have no genetic tie whatsoever, it just happened due to bad luck. The doctor ironically said it's almost like double bad luck because even in the realm of heart defects, Lucas's configuration is something they don't 'see.' I have yet to see a child with these exact anomalies.

I had a full cardiac workup and all is well with me... wish I could say the same for him one day. I did not drink or smoke or do anything unhealthy while I was pregnant, aside from being overweight, which the doctors said did NOT cause this.

If anyone has any questions, please feel free to ask me. I did a lot of reading and became a novice-wannabe-Pediatric Cardiologist so I tamed down some of the medical terms here as to not bore anyone reading. I remember what it's like to get that news and be scared about it. I can give plenty of advice if needed!

Here's a photo from when Lucas was about 1 year old - a little more "blue" back then (around his lips.) Around this time, he was receiving developmental therapy, occupational therapy, physical therapy, seeing the Feeding Team and a behavioral feeding psychologist, family behavior therapy, and later he received speech and in-home feeding therapy. I also do not miss having up to 5 appointments at my home daily, but I learned a lot. I am glad that it is behind us and he is doing so well. So parents of children with speech delay... never fear - despite expressive language speech delay, my son now says the most complex sentences! Sometimes he won't stop talking! lol
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Some Fontan recovery pics of me and my little guy:
(please excuse my HAGGARD appearance - I had probably been crying before these were taken and I wasn't putting on makeup for the hospital stay!) Age 2 years, 3.5 months.
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He was happily watching Cars on DVD when this was taken, and the Easter bunny had just visited him. This was only 2 days after open heart surgery and the morphine had worn off. Kids are AMAZING. I vividly remember crying out in the hall probably 20 minutes before this was taken due to a family issue going on at the time. Moms have to be strong for their little ones. I normally never cry after some of the stuff I have been through in my life, but this day was an exception. I can be a ham for the camera just like Lucas, I guess!
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To end on a positive note, people who meet him now have NO idea he has this medical history, unless they see his chest. His sternotomy / incision site has faded to a pale white, but the area where the bulb drain was is pretty prominent. If anyone wants to see that, I'm happy to share. I also have plenty of graphic surgery photos, which if anyone wants/needs to see that, I'm willing to share. I remember I wanted to know what to expect - the good and the bad.

Here's my cutie pie having fun this past summer.
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I remember the doctor saying "he's PINK!" (We don't like angry red-purple-blue cyanosis.)
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Living it up with Santa, informing him of Super Mario Bros. and Hot Wheels requests... shockingly he was only 3 years old when this was taken. He is very tall for his age (off the percentile charts!)
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Heart hugs,
Jackie
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