Connect Issues Fall 2020

By Hannah Moore Huck was born early in the morning on April 6, 2020. The first thing everyone noticed was just how long his fingers and toes were. We joked about how he had such a “mean old man face” as he snuggled his older brother shortly after birth. From the moment Huck was born, he fought. Hard. Huck was placed on ECMO (a machine that replaces the function of the heart and lungs) at birth as he was suffering from severe respiratory distress that led to intubation and a feeding tube. He was diagnosed with neonatal Marfan syndrome and remained on support for his first eleven days. When he was finally discharged, it was the proudest moment for me. “He made it! He fought through and now I can take my baby home and spoil him like I have my others.” Over the three-and-a-half weeks he spent at home, Huck’s heart was fighting harder and harder to keep up, unbeknownst to us. This depleted his heart function and, when he was readmitted to the hospital, he was diagnosed with congestive heart failure. Huck fought through every minute, every procedure, every new line placed, medicine, and even dialysis. His heart held on, until it just couldn’t anymore. His strength still amazes me. It gave me the strength to be his backbone and advocate through it all. It gives me the strength now to ensure his life had a purpose, and a good one. Neonatal Marfan syndrome is what took Huck’s life. As a parent, nurse, and sonographer, I believe education is everything. I know that it leads to better healthcare, more informed decision making, and, ultimately, healthier outcomes. My goal is to provide neonatal Marfan education to as many people as I can, for Huck. Neonatal Marfan syndrome is the worst end of the spectrum of Marfan and, even within the neonatal Marfan babies, there is a range in severity, depending HUCK’S LEGACY “… I know that [education] leads to better healthcare, more informed decision making, and, ultimately, healthier outcomes. My goal is to provide neonatal Marfan education to as many people as I can, for Huck.”

p Huck was the son of Phillip and Hannah Moore, and the little brother to Maddox, Lucas, and Phillip. He is missed and loved abundantly every day.

on the combination of features and the severity of the individual components. Neonatal Marfan syndrome is a different entity than Marfan syndrome. It has early onset and rapidly progressive features, most seriously related to the heart, lungs, and airways. Being diagnosed prenatally (which is very difficult) or at birth can meaningfully benefit the outcome of the patient. It can be diagnosed at birth due to certain visual characteristics, as well as significant cardiovascular/pulmonary involvement immediately after birth. The most detrimental feature is severe regurgitation (or leakage) of the mitral and tricuspid valves in the heart, which leads to pulmonary hypertension (too much pressure in the lungs), which progresses to poor squeeze of the heart, and ends in congestive heart failure. Unfortunately, the prognosis for neonatal Marfan prognosis is poor –95% pass away within the first year of life. Because it is extremely rare, there are limited resources and interventions as of now. My hope is that I can raise awareness, and ask anyone who can to also help raise awareness. One shared speech could save one child's life, and I hope Huck's life will do that.

Hannah Moore is a nurse, OB/GYN sonographer, mama to four boys, and wife. She will always strive to be Huck’s continuing advocate and Marfan Foundation supporter.

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Fall 2020