It wasn’t until the Martins’ fifth pregnancy that the couple heard a heartbeat. Yadi Martin experienced four miscarriages and baby number five was their “miracle.” As the baby grew, they became excited as many first-time parents do. Then during an anatomy scan, everything changed.
“The doctor said, ‘He has very large lungs for this far into this pregnancy. It’s abnormally large. And I think this is something called CHAOS,’” Yadi Martin, 39, of Brooklyn, told TODAY. “Jamaal said, ‘That is one hell of an acronym.’ And the doctor said, ‘Yes it stands for congenital high airway obstruction syndrome.’”The Martins had never heard of it. When they tried searching the internet, they struggled to find anything about it. They met with high-risk maternal fetal medicine doctors, pediatric surgeons, pediatric otolaryngologists and others to understand what CHAOS is and what this meant for their baby.
“The doctor said to us, ‘You know this is a very serious situation. This is a very serious diagnosis. It is very rare and so early in the pregnancy, we don’t really know what that means for your baby,’” Yadi Martin explained. “We’ve been through hell and back and here we are with this baby and all I kept thinking was, ‘God, please don’t let this be another one.’”
What is CHAOS syndrome?
While doctors have known about CHAOS since the late 1900s, it’s unclear how often it happens or what causes a baby to develop an obstructed airway, according to a BJR Case Reports article.
“Sometimes these can be cysts. Sometimes these can be full blockages. Sometimes they can be partial blockages. But they’re not always at the same spot,” Dr. Scott Rickert, chief of pediatric otolaryngology at Hassenfeld Children’s Hospital at NYU Langone, told TODAY. “Frequently, they’re in the most narrow part of your airway, which is where your vocal cords and just below are.”
If it’s not diagnosed prior to birth, CHAOS is fatal.
“It becomes a very big deal the minute the baby’s born when the baby’s separated from the placenta because the only way the baby can get oxygen is by taking its first breath,” Dr. Ashley Roman, vice chair of clinical affairs, obstetrics, director of the division of maternal fetal medicine in the department of obstetrics & gynecology at NYU Langone Health, told TODAY. “If there’s an obstruction in the airway that the baby can’t cry in its first breath. It’s deprived of oxygen.”
Roman had seen a CHAOS diagnosis once while in training and she confirmed the diagnosis.
“It’s so rare there are even high-risk OB providers who do ultrasound every day (have) never seen cases like this,” Roman said. “It’s the kind of thing where you see it once, you never forget it because it’s so serious and the consequences are so severe.”
After that, Roman reached out to her colleagues, Rickert and Dr. Jason Fisher to plan how to deliver the baby and help him breathe.
“The delivery planning typically involves doing an EXIT procedure,” Roman said. “That’s a complicated way of saying basically we keep the baby on placental bypass until an airway can be established.”
At about 28 weeks, the Martins learned what the birth would involve. Even with the plans and back-up plans they felt stressed. Jamaal Martin said their baby wouldn’t be viable until 31 weeks, instead of 25, which made the pregnancy feel even scarier.
“Once we met the team we felt more at ease because they seemed like they knew what they were doing — even though it was rare,” Jamaal Martin, 43, told TODAY.
Still, the couple felt isolated. Internet searches provided little information and there weren’t any support groups or families talking about their experiences with CHAOS.
“We don’t know what we’re really getting into,” Yadi Martin said. “There were all of these questions without answers.”
Yadi Martin also experienced hyperemesis gravidarum, which causes severe nausea and vomiting during pregnancy. During the first two trimesters, she could barely keep anything, including water, down and needed to be on a pump with medications to ease her symptoms. Despite the stress of the unknown, the couple experienced joy and excitement.
“They would print out the sonogram pictures and just to see his little face (was amazing). And he had a little personality — they kept trying to see him from the front and every time he would just turn around and show his butt,” Yadi Martin said. “It gave us hope.”
Yadi and Jamaal Martin encouraged each other when they felt scared, reminding themselves they had each other and a great medical team. Doctors scheduled delivery for when Yadi Martin was 36 weeks pregnant. They’d use the EXIT procedure, which stands for ex utero intrapartum treatment. Still, baby Aydin’s birth came with scary and unforeseen complications.
“It was chaos, pun intended,” Jamaal Martin said. “Just about everything that could have gone wrong went wrong.”
What is an EXIT procedure?
After Yadi Martin was sedated and given medications to relax the uterus so the placenta wouldn’t separate early, Roman delivered Aydin’s head, neck and shoulders. He was still connected to the placenta, which helped him breathe until Rickert could create an airway. Rickert first evaluated Aydin to understand the blockage.
“I even tried to see if I could sneak my camera past anything and (the blockage) was really hard, almost like cartilage or like a plug,” Rickert said. “We proceeded to do the tracheotomy, which bypasses the airway obstruction.”
After about four minutes, Aydin could breathe and Roman finished delivering him. Then his heart stopped.
“Part of the challenge with CHAOS is when you have an airway obstruction, the lungs tend to get quite big and full and it clearly seemed to be pushing on the heart,” Rickert said. “The heart was not working as well as we suspected.”
Doctors tried resuscitating him and Fisher placed Aydin on ECMO, a heart-lung bypass machine.
“Our initial thought was that the baby’s heart was being basically compressed. The lungs were taking up too much space,” Fisher, director of the children’s surgical services and chief of the division of pediatric surgery at Hassenfeld Children’s Hospital at NYU Langone, told TODAY. “Thankfully he was only on ECMO for four, six hours.”
For much of that time, doctors struggle to stabilize him.
“We were having trouble supporting him, which is weird. When they're on ECMO … you’ve taken full control,” Fisher said. “Part of the reason we were having trouble was because the baby was getting CPR for such a long period of time — while we were getting him on ECMO — that the belly started to get big because the intestines were getting swollen. In addition, the chest compressions had created some bleeding around the liver.”
Fisher and his colleagues opened Aydin’s belly to relieve the pressure and drain the air from outside the lung.
“He was leaking all the fluid in his bloodstream because he was so sick. Not from bleeding per se,” Fisher said. “We were constantly having to give him more and more fluid in order to make the ECMO machine run to support his heart.”
At some point, though, the doctors knew they couldn’t pump him full of fluid because he’d turn into “a big marshmallow” and his kidneys would stop working well enough to help. When they saw a “small window” where Aydin was stable, they took him off ECMO.
“He ended up being able to oxygenate and get off carbon dioxide,” Fisher said. “That’s when I had a sense that he might actually survive.”
Meanwhile, doctors updated the Martins.
“When they finally came upstairs and said, ‘We’re still working on him. He’s in critical condition. We don’t really know what’s going to happen in the next eight hours’” Yadi Martin said. “They weren’t even saying 24 hours.”
But Jamaal Martin felt hopeful because Aydin had survived.
“I just heard he was alive. Everything else they said was irrelevant,” he said. “I can’t tell you anything they said after I heard he was alive.”
Hours later, they saw him in the neonatal intensive care unit (NICU) hooked up to ECMO with wires and tubes snaking from his body.
“There was so much happening with his little body that we were just clinging onto the fact that he was alive,” Yadi Martin said. “Although he was on ECMO, he was breathing.”
Jamaal Martin felt stunned by how good Aydin looked.
“I’m thinking I’m going to walk in there and see something that looks like ‘The Walking Dead,’” he said. “He’s beautiful. He’s got a head full of hair — he looks like his sonogram pictures.”
Doctors also discovered Aydin had a congenital septal heart defect and an anorectal malformation, a birth defect leaving him with no rectum. Fisher had given him a colostomy and a G-tube during surgery soon after birth. Later, the infant underwent open heart surgery to repair his heart defect and in another surgery, Fisher created a rectum for Aydin.
“He gets fed by his G-tube and he poops like any other baby, which is nice,” Fisher said.
After being in the NICU at Hassenfeld Children’s Hospital since his birth on August 3, 2021, Aydin moved to a rehabilitation hospital on January 19, 2022, Yadi Martin’s birthday. Three weeks before his first birthday, he returned home. While they were excited to celebrate his first birthday at home, he returned to the hospital because of an infection in his trachea.
“We had some presents,” Yadi Martin said. “We got to the hospital and he was all able to see all of his uncles, grandmas, girlfriends from the NICU at NYU. So it worked out. He was happy like nothing happened.”
Life at home
Aydin has speech, physical and occupational therapy and will soon work with someone who will teach “him how to be a child.” He can’t speak because of how his vocal cords developed and he will need to have his airway reconstructed when he is older. This fall, he’ll also undergo a procedure to fix an undescended testicle. Still, his parents continue to be amazed with Aydin.
“It’s so awesome to see he’s getting stronger. He fights to sit up and just stay sitting up. He can stay sitting up by himself approximately three minutes,” Yadi Martin said. “It’s so incredible to say this is our baby. He’s growing and when it’s all said and done he will be absolutely perfect.”
The Martins never talked about how sick he was in front of him because they didn’t want him to feel bad that he has health challenges. Despite the grueling past year, the couple says their marriage became stronger.
“We were head over heels in love already,” Jamaal Martin said. “It reaffirmed it.”
Even with his early health problems, doctors think Aydin will thrive.
“I think he’s going to developmentally be pretty close to on target, which is the biggest miracle of all,” Fisher said. “The happiest moment I had …. we sent him for a MRI of the brain, which came back completely normal.”
The Martins wanted to share their story so if any others learn their baby has CHAOS they won’t feel as alone — and they’ll know there are options.
“There’s a light at the end of the tunnel,” Jamaal Martin said.
Relying on each other and their faith helped them navigate the difficult birth and Aydin’s continued health difficulties. They have a GoFundMe to help with some of the medical costs associated with Aydin's experience with CHAOS. They added that their hope continues to sustain them.
“God will not give us this blessing without getting us through this. We’re going to be fine,” Yadi Martin said. “He’s going to be fine.”
This article was originally published on TODAY.com