Every parent of a child who suffers from a life threatening disease such as Congenital Heart Defect (CHD) has pain – but a story of pure courage to tell. As a Foundation where with Heart Disorders, children from lesser privileged families have no means for surgery to survive. We strive to create awareness of the disease and the journey parents and children face while overcoming CHD.
She has written a book called ‘LAMBY: A mother’s journey through a twisted Medical system to save her son” which is available on Amazon.com. which explores the struggle she has faced.
Nikki has a BSN degree from Georgetown School of Nursing and a Master’s Degree in Nursing from NYU. She continues to offer advice for friends and family who have battled with the sometimes confusing medical system. Currently, the family of six
live in Stonington, CT.
Dylan was born full term on December 9, 2005. From the moment he came into
the world, things just didn’t seem as “perfect” as with my first child. It took a bit
for him to receive a high APGAR score. Then the coughing started…a barking
cough that, to me, was not normal for a newborn. Then there was the difficulty
feeding and choking…all before we left the hospital. But the staff kept telling me
he was just clearing mucus. SO we go home and start a journey I never imagined.
The short version is this: Dylan struggled with eating, breathing, crying, and
vomiting multiple times a day for months. At least once a week, we were seeing a
doctor and often they said nothing was wrong; “You just have a fussy baby”. I was
kicked out of the ER twice. He was misdiagnosed three times. They once told me
it was reflux. By two months old, Dylan was on reflux medication, a prescription
formula which also had to be thickened to reduce the amount of choking that
occurred with feedings. Every night he slept on my chest because otherwise he
would vomit and choke and stop breathing. I knew from my medical background
that this wasn’t just reflux. So I went to new doctors and got other opinions, none
of which satisfied me or relieved his symptoms.
At three months old, a GI specialist doing an upper endoscopy, caused my child to
desaturate. It took stopping the procedure and 2 shots of epinephrine to get him
back. Little did I know this would be the beginning of another set of issues… we
spent the next ten days in and out of the ER and doctors office. I had an extremely
hostile 3 month old who wouldn’t stop crying, didn’t eat, just basically laid there.
His breathing was awful…and no respiratory meds were working. Years later, I
found out he had a stroke, caused by the negligence of the physician.
At five months, after switching hospitals and providers, we finally received a
diagnosis of a Vascular Ring (double aortic Arch). He went to surgery 10 days later
and the ring was divided. But the OR nurse pulled me aside and said, “please watch
his breathing. His trachea was pretty crushed and didn’t recover like we had hoped
once the ring was released”. And so we monitored him…for 3.5 years with
repeated pneumonias, doctor appts, specialists, bronchoscopies and lung lavages.
Antibiotics and steroids were his normal medications. It got to a point where he
had aspirated so much in 4 years, his lungs had started to form scar tissue.
Something needed to be done! I researched and interviewed 4 different surgeons.
Everyone had a different suggestion, including trachea reconstruction;…we were
not doing that unless totally necessary!
I found a doctor at Texas Children’s Hospital, who had actually devised a surgical
technique to correct what the problem was with such heart disorders, children like
Dylan could benefit from.
Dylan had mirror image branching; he had extra vessels and they were still choking
him and crushing his esophagus. This surgery called “Translocation of the
Descending Aorta” relieved the pressure on the trachea and esophagus. The
esophagus (because it is a muscle) bounced back/open to normal. The trachea,
though, was crushed 80% in 3 spots and because the cartilage rings had been
damaged by the exterior pressure from the extra vessels, it caused the airway to be
“floppy”; otherwise known as Trachealmalacia. The surgery was a success and we
saw mass improvements with eating and lack of illness. He has been monitored by
every specialty, every year since.
At the age of ten, I started noticing he was having a problem breathing again and
the barking cough was back.
We pursued a thorough pulmonary work up which showed that the lung damage was
not the cause of his current breathing issue. What it did reveal was a complete
collapse of the trachea upon inhalation and coughing. A cardiac MRI showed there
was still vessel compression on the trachea as well. We consulted with the
cardiothoracic surgery team at Boston Children’s who decided on holding off on
moving anymore vessels but that a tracheopexy would be a valuable option. This
requires the surgeon to move the esophagus out of the way and pin the posterior
portion of the trachea to the spine to hold it open and create better airflow. Dylan
underwent this surgery in the spring of 2018. He required 2 dilations of the
esophagus because of narrowing and scar tissue pushing on it but the trachea
improved to only a 20-30% collapse, which for Dylan was life changing.
It took 6 different hospitals, close to 100 doctor appointments, 26 procedures
requiring anesthesia, and 3 major surgeries, including cardiopulmonary by-pass, to
get us to where we are today…
At the moment we are grateful that he is doing very well.