Contrary to the traditionally believed „vegetative state“ where the mind is absent while the body lives, children with „Locked-in Syndrome“ are unable to move or speak, are awake, and completely aware of their surroundings.
Researchers from the University of Texas Health Science Center in San Antonio (UT Health San Antonio) were the first to report in the peer-reviewed medical literature that children become locked-in after non-fatal drowning. Led by Peter T. Fox, MD, Professor of Radiology and Neurology and Director of the Research Imaging Institute at UT Health San Antonio, the team described a series of features in brain imaging studies that identified the cause and explanation of Locked-in Syndrome.
The first 11 pediatric survivors of drowning were photographed at UT Health San Antonio in the 2010s. Now, in a subsequent study published in the Pediatric Neurology, the team reports the largest study to date on this issue. The recently published analysis of 154 children confirms that the predominant outcome of non-fatal drowning in children is Locked-in Syndrome.
In the UT Health San Antonio survey, 60% of children who survived drowning events (93 out of 154) were classified as locked-in by caretakers.
„The spectrum of disabilities among the children was wide. About a quarter were mildly affected, nearly normal in all functions. Half were moderately motor-impaired, but cognitive abilities and perception were preserved. A quarter were severely motor-impaired, with cognitive abilities less spared. Of the moderate and severe groups, almost 80% were locked-in.“
– Peter T. Fox, MD, Professor of Radiology and Neurology and Director of the Research Imaging Institute at UT Health San Antonio
Carers in Alignment with the Children
Caregivers were identified and interviewed between 2018 and 2021 after Fox and colleagues published the first brain imaging studies of non-fatal drowning in children in 2016 and 2017. Fox said the data obtained from the survey was clear and meaningful.
„The findings from it are that the parents and other caregivers of locked-in children really understand the signals they are seeing and are doing an excellent job at recognizing whether the children are responsive or not,“ Fox said.
„Physicians and the health team should find more ways to involve and trust in the observations of parents and carers,“ he added. „Carers are a vastly underused resource. We need to be more respectful and more conscious of the quality of their observations.“
In Memory of Conrad
The first surviving child photographed at UT Health San Antonio was Conrad Tullis from San Antonio.
Conrad, born in 2002, survived drowning in 2004 and lived to be 20 years old. He inspired many and attended all 12 grades and graduated from the Alamo Heights school district in San Antonio.
„Conrad’s locked-in state and that of the other children were first recognized by the parents, which is very important,“ Fox said. „I was made aware of this condition by Liz Tullis, Conrad’s mother, who noticed that her son was responsive, even though she was told he couldn’t understand. Tullis, along with other parents from a support group for parents of children who survived drowning, helped the team in recruiting participants for the studies.
„As parents, we know our children and can recognize the difference between children who are responsive and those who are not,“ Tullis said. „While the doctors told me my child would never lead a meaningful life, I knew he was capable of understanding and communicating with us, even though he was nonverbal.“
Imaging Provides Answers
The parents had read about the Research Imaging Institute, a highly specialized center at UT Health San Antonio that utilizes multiple imaging modalities (MRI, fMRI, PET, and more) to answer research questions. „They asked if imaging could tell whether the doctors were right in telling them the children were not conscious, or if they, the caretakers, were right,“ Fox said.
The imaging studies by the Research Imaging Institute revealed that cognitive and sensory networks in the brains of these children remained intact. The injury did not affect the entire brain, as previously thought, but was confined to a small area. The results supported the parents‘ belief that their children experience emotions, learn new ideas, and develop personalities. The research also raised hopes that the brain injury could one day be treatable.
The new research did not include imaging but added experiences and observations from a much larger group of affected individuals. „I hope this new publication makes it possible to conduct more investigations in this larger cohort of children, using both imaging and other tests,“ Fox said.
The researchers asked Pediatric Neurology editors to dedicate the article to Conrad Tullis and to endorse the name „Conrad Syndrome“ for „Locked-in State“. The journal agreed.
Children admitted to the hospital after non-fatal drowning had better outcomes when their hospitalization did not require medical intervention such as intubation, the survey found. When the children were responsive at both admission and discharge and not in a coma, their outcomes were better.
In the future, it might be possible to use portable imaging systems to test patients at home for the Locked-in Syndrome, Fox said.
University of Texas Health Science Center in San Antonio
Razaqyar, MS, et al. (2024). Long-term neurocognitive outcomes in non-fatal drowning in children: Results of a survey among family caregivers. Pediatric Neurology. doi.org/10.1016/j.pediatrneurol.2023.11.001.