Congenital heart defects are the most common birth defect (about 1 in 100 births) and globally there
are about 50 million people living with a CHD. About 1 in 4 defects are "critical" which can have
moderate to severe impacts on quality of life. We're super lucky that Ruby is doing so well and we're
extremely grateful for the outstanding medical treatment she's received, so we thought we should give
something back. Please consider joining our walk or donating. Research is expensive....Every small
contribution helps.
Ruby's Story
Diagnosis:
Ruby was born with a combination of two heart defects that partially overlap in symptoms: Pulmonary
Atresia (lack of main pulmonary artery) and Tetrology of Fallot (a combination of four defects).
In a healthy heart:
1. the oxygenated (red) blood is pumped to the brain and body through the Aorta
2. the deoxygenated (blue) blood is pumped to the lungs through the Pulmonary Artery
In a healthy heart, the Aorta and Pulmonary Artery are totally separate vessels coming off opposite
sides of the heart. In Ruby's case, there was no Pulmonary Artery and instead the flow to her lungs came
from several small vessels coming off the Aorta. This means there was only one way for blood to leave
her heart. Luckily, the right and left side of Ruby's heart were not fully separated due to a much more
common heart defect known as a Ventricular Septal Defect (VSD). This allowed the red and blue blood
to mix in her heart and become "purple" blood. When this blood leaves her heart, some goes to her
lungs and some goes to her brain and body. This allowed her circulatory system to function but it's not
very efficient. This means her blood saturation was usually around 80% that of a healthy person. Through the miracles of modern medicine, Ruby's circulation was modified to be much more efficient/normal and now her blood saturation is typically very close to 100%.
Open Heart Surgeries:
- 4 days old: Involved placing a stent to keep open one of the small vessels that supplied her left lung. We spent about a week in the ICU and then about 10 days on the cardiology floor, working on gaining weight and learning to bottle feed. It's a lot of work for Ruby to eat so we add calories to her Mom's breastmilk.
- 4 months old: Goal was to "unifocalize" (combine into one) the several small vessels that went from her Aorta to her lungs. These vessels will eventually become her Pulmonary Artery so they need to be combined into one thing. We only spent a few days in the ICU and then almost 2 weeks on the cardiology floor. This stay was longer than expected and she struggled to gain weight. Her mom and dad resisted the feeding tube at first, because she had done pretty well with the bottle during her first four months. After consistently losing weight, we accepted that she needed the tube. It's been a real struggle with lots of vomiting and replacing the tube (not fun), but it has also been a great help with weight-gain: she went from 3rd to 23rd percentile in weight!
- 11 months old: (PA to RV conduit) This surgery involved running a conduit from her new unifocalized pulmonary artery to the right side of the heart. The surgeon also temporarily closed (i.e. fixed) her VSD but then determined that she wasn't ready for it to be closed and actually reopened it because the pressure on the right side was too high (since the VSD allows blood to flow from right to left, it acts to decrease pressure.) As a result, Ruby was "on bypass" for around 10 hours which made for a very tough recovery. She was intubated for nearly a week and had a hard time weening from the vent. Slowly but surely she made progress though and we were able to return home after around 3 weeks in the hospital.
- 15 months old: ("emergency" surgery.) During a cath procedure, her doctors noticed that the conduit placed during surgery #3 had developed a psuedo-anuerysm which means that it had inflated to nearly twice its original diameter. For her other surgeries, we had been mentally preparing for months in advance. This time, we had expected to stay in the hospital one night for the minor procedure that was planned. However, once they found the pseudo-aneurysm, they wanted to operate the next day to replace the conduit with something stronger and less likely to dilate in the future. Additionally Ruby's previous surgeon was on vacation, so we had to meet (and learn to trust) a new surgeon with our baby. Despite all the chaos, we were so grateful they could diagnose this problem when they did because it possibly saved her life.
- 23 months old: (complete repair) Everything else was in place and by measuring her pressure, they determined she was ready to the VSD to be closed. Her surgeon said the replaced conduit looked excellent so he was able to close her VSD and she was only on bypass for about an hour. They had told us to expect about a 2 week hospital stay but Ruby was ready to recover much more quickly. (The day after her surgery she wanted to get up and walk around despite all the tubes and wires still connected to her.) After just 3 nights in the hospital, we were discharged to come home.
After 5 open heaart surgeries, her repair is complete and Ruby's life is surprisingly normal. During these last few years Ruby has received an array of therapies for her developmental delays. She was prescribed to physical therapy for gross motor skills, graduated fairly quickly, and is now on track. Her vocal chords were slightly damaged during her 3rd and continues to receive speech therapy through University of Michigan and through Ann Arbor public school systems. Our biggest struggle post surgeries was getting Ruby to eat. She had strong oral aversion, endured a nasal-gastric feeding tube for 15 months then had a gastrostomy tube placed to help with her swallowing issues. Today Ruby rarely has problems finishing a meal or staying hydrated. Ruby’s even been known to finish off three pieces of pizza at dinnertime.
For the past three years Ruby has had a clean bill of health from her pediatrician and cardiologists. She continues to have yearly check ups and as she grows Ruby will eventually need interventions in the cath lab and ultimately have her conduit replaced. A few years ago, it was hard to imagine Ruby being in the place she is now. Words can’t describe how incredible it feels to look back on those difficult times now as a closed chapter of her life. Ruby will turn six in a few months and is about to start Kindergarten at our neighborhood school. Ruby is an incredibly talented artist. She’s quirky, empathetic, silly and has an imagination that takes her and her sister on some Wilde adventures. Our Ruby is an inspiration and true testament to what it means to be a warrior.