Susan paused to make sure she still had the parents on board. They stared at her with rapt attention, so she continued. “The hypothalamus is a control center of the brain. One of the many things it handles is hunger and satiety. People with Prader-Willi syndrome almost never feel full; and, even when they do, it’s short-lived. Given free access to food, children with Prader-Willi syndrome will, quite literally, eat themselves to death.”
The parents nodded. Mr. Moore cleared his throat. “Why are you telling us this? Dallas doesn’t have Prader-Willi syndrome, does he?”
Susan nodded agreement. “Dallas has a far rarer syndrome. It’s called the syndrome of optic nerve hypoplasia. For short, it’s ONH.” Such a diagnosis might devastate most parents, but Susan saw a hint of relief soften the tightness of Mr. Moore’s features. Mrs. Moore almost smiled. Having a name for Diesel’s behavior, a medical reason for the odd things he did, had to be liberating after the hell he must have put them through.
“Doctor,” Mr. Moore said, “what does that mean?”
Susan tested a theory. “Was Dallas born in Texas? Perhaps Harris County?”
Both parents stared. Mrs. Moore finally stuttered out, “H-how did you know that?”
“I didn’t,” Susan admitted. “I just put the pieces together. It’s not terribly uncommon to name a child after his place of birth. Also, of the cases of ONH, a high proportion of mothers spent the early weeks of pregnancy in Harris County, Texas.”
“Harris County.” Mrs. Moore was amazed. “It was Harris County, wasn’t it, Jamal?”
“Houston,” Mr. Moore replied. “That’s in Harris County, all right. By the time he was born, we’d moved to Dallas.” He frowned. “Why do they think Harris County has this problem? And what does it mean for our son?”
Susan had more information than answers. “The syndrome’s so rare, no one has done any significant studies of it. I just happened to tap into one ophthalmologist in California who performed a survey, looking for a cause. He never actually found one, but he did find the uptick in patients who spent their early embryonic lives in Harris County. He believes something in the environment explains the defect. Mind you, most of the kids with ONH don’t come from Texas. It’s just a statistical thing.
“The upshot is Dallas has congenital abnormalities of his hypothalamus and optic nerves.” Susan did not get deeper into the brain technicalities. Some people with the syndrome also had certain missing septa in the brain, but Dallas did not.
Mrs. Moore guessed, “He has trouble . . . seeing?”
“That’s how it’s usually detected,” Susan said. “Hypoplasia is when things in the body don’t develop to their full potential. Kids with ONH are often blind. Most have poorer than normal vision; but a few, like Dallas, have normal or nearly normal vision. His optic nerves are smaller and thinner than usual, but they work just fine. The best part is the damage is done before birth. So, we have no reason to believe Dallas’ vision will get any worse.”
Susan gave the Moores time to digest this information.
After several moments of dense silence, Mr. Moore finally spoke again, “So, how does this apply to Dallas?”
Susan anticipated the question. “As I said, ONH is nearly always diagnosed by ophthalmologists because of vision problems. Ophthalmologists know children with ONH often also have problems with the hypothalamus. The lucky ones with normal vision, like Dallas, fall through the cracks. They present with the hypothalamic symptoms first, and they become extremely difficult to diagnose.” She hoped this would help the Moores understand why medical science had, thus far, failed them.
Mr. Moore still worked to grasp the greater significance of the syndrome. “So . . . Dallas’ obsession with food is not obsessive-compulsive or oppositional. And his depression?”
Susan thought she had a reasonable understanding of the situation based on talking to Diesel and reading the charts and the nursing notes throughout his hospital stay. “Oh, he’s depressed, all right. Deeply and severely. Among many other things, the hypothalamus regulates mood, and people with disturbances in hypothalamic function have Moods, with a capital M. Their highs are higher, their lows subbasement, their anger —”
“Volcanic,” Mrs. Moore supplied.
“Yes.” Susan felt certain they had experienced many of Diesel’s explosions, probably since infancy. “Now, imagine you’re hungry all the time, day and night. You’re driven to eat. Tens of thousands of calories every day don’t satisfy your belly because, no matter how full your gut, your brain keeps telling you to eat or die.”
Mrs. Moore put her hands to her face. “Dallas feels like that?”
Susan did not answer, needing to continue her scenario until she made a specific point. “But you’re constantly being told that taking food is greedy and disgusting, and that fat people are undisciplined slobs. But you need that food every bit as much as you need air. So you start to sneak it, to steal it, to hide it, which only brings down more anger and humiliation. Any moral child would come to believe himself” — she used Diesel’s own words — “a monster. And hating oneself is the very definition of depression.”
Mr. Moore sat in thoughtful silence, rocking ever so slightly. Tears formed in Mrs. Moore’s eyes. “They told us he was just being . . . oppositional. Defiant. Fighting us for control.”
Mrs. Moore’s voice quavered. “One doctor said we didn’t love him enough. That he ate to fill the void.”
“Nonsense,” Susan reassured them.
Mr. Moore turned entirely practical. “So, what do we do?”
Susan had a plan all worked out for them. “First, we get you hooked up with an endocrinologist. I’m willing to bet Dallas would benefit from growth hormone and thyroid hormone replacement. He may need testosterone to go through puberty; and, even then, I should warn you he will probably be infertile.” Susan gave them time to process that information. Eventually, they would likely realize it did not matter. Given his severe lifelong problems with food, Diesel would never have the wherewithal to handle children of his own.
“Next, we hook you up with a security company that can go through your entire house and figure out how to secure any and all food-containing areas. You will need dependable locks on the freezer, refrigerator, garbage cans, and cabinets. Better yet, if you have an enclosed kitchen, lock the whole thing. We will have to work with the school as well and realize children with hypothalamic forms of obesity will take food wherever they can get it: stores, vending machines, other people’s plates, floor sweepings, dog food bowls.”
The Moores started talking at once, comparing memories of times when Diesel had raided places they never expected and had eaten items most people would never touch.
Susan let them converse. She had given them a whole new perspective on their son, and it would require them to turn their lives, and those of his siblings, upside down. Most people did not realize just how important and central a role food played in every social aspect of life until it became a problem.
When the Moores seemed ready, Susan continued. “Dallas will feel most secure when he has no personal access to food, when people he trusts fully control his access to it. If he tests the locks and finds them wanting, he will continue to work on them anxiously. If he tries and fails, he will become a much calmer person knowing his life, and his appetite, are controlled by a higher authority: his parents and doctors. We will make him up a diet and stick by it rigidly. Dallas must know he will be fed, at regular times and in predictable and consistent amounts. Food can never be used as reward or punishment, nor can it be present anywhere he is expected to concentrate.”