Elizabeth Lang tearfully spoke about the pain she’s felt since the summer, pain that she knows she has to manage, and that she also knows will never fully go away.
“I know everyone says their child is beautiful or perfect … but he really was the perfect little boy,” Lang said of her infant son Raymond, who tragically passed away in August. “He was so cute and he never cried … always smiling. He was always smiling.”
Lang and her family took on incredible loss on Aug.15, when Raymond passed after almost two months of battling an extremely rare genetic disorder known as Barth Syndrome. The situation was compounded by dilated cardiomyopathy and an infection.
Barth Syndrome primarily affects boys and impacts the heart, muscles and immune system. Symptoms can also include low white blood cell counts, which can lead to increased risk of infection. X-linked recessive genetic disorder, meaning it is more common in males who have one X and one Y chromosome. Males with a single mutated copy of the gene are affected, while females with two X chromosomes would need mutations in both to develop the condition. There is no known cure.
It affects approximately 1 in 400,000 live births worldwide. The condition is life-threatening, in many cases as it often necessitates a heart transplant.
Raymond passed away in his mother’s arms, after an extended battle that began from Raymond’s birth on June 20.
“There’s a 50-50 chance of the child being born perfectly healthy,” said Lang of children suffering from Barth Syndrome. “Some are lucky enough to go on to not be diagnosed with it until later in life. But there’s limited treatment for symptoms and there isn’t a cure,” Lang said. “They said that Raymond was the only baby boy to ever be diagnosed with it in Louisiana since (the illness) got its name in 1983.”
The Hahnville mother said Raymond began having respiratory issues following a difficult delivery process, and was put on oxygen in the NICU. Attempts to test whether he could come off of oxygen were not successful and he underwent an EKG.
Lang was told Raymond would need to be transferred to Ochsner’s main campus and that he had been diagnosed with an enlarged heart.
After her son’s arrival and examination, Lang received an alarming update.
“A woman walked in and introduced herself as part of the heart failure team,” Lang said.
Raymond proved to be a fighter. He was in danger of passing away in July – on Lang’s birthday – after a situation arose, but doctors were able to stabilize him. He continued to receive treatment and Lang and her husband received regular updates on his heart following weekly echocardiograms.
Ultimately, however, there was nothing that could be done to realistically save his life.
She recalled what her grandmother-in-law told her after Lang learned she was pregnant with Raymond.
“We had just been married in August of last year, and we found out in December. She told me, ‘See, God was just waiting for you to get married to give you a baby,’” Lang said.
“It’s so hard … he was so loved. I’d do anything to have him back with me,” Lang said. “I think there’s solace in that he’s not suffering.”
Lang’s grandmother knew this pain. Her son passed away at just two weeks old in 1959.
“We think it may have been this,” Lang said of Barth Syndrome. “But, at the time, genetics weren’t widely studied. (The illness) didn’t have a title until 1983, and none of the females in my family (gave birth to) a male in that time, until now.”
On top of their grief, the family has had to incur mounting medical expenses for Raymond’s care during the situation. A GoFundMe (Help with Ray’s PICU Medical Costs) has brought $1,500 in donations.
“Our support system has been incredible. And then there’s people I’ve never met, people who I haven’t talked to in years, who have wanted to help and have offered to in different ways. And our friends and family have been stepping up for us in any way they can.”
