Diesel hesitated. He seemed as uncertain as she about how he felt like behaving next. “Got any tissues?”
“Right here.” Susan patted the floor beside the piled tissues. “Come join me.”
Diesel turned slowly, studying the situation as if he expected her to leap up and net him. Finally, he came over, picked up the tissues, and clamped them to his nose. Tears and blood streaked every part of his face.
Susan’s training rushed to the fore. She should be wearing a gown and gloves and staying as far as possible from a biohazardous patient. It helped that she knew from his chart he did not carry hepatitis, HIV, or CIV; and she was legally vaccinated against everything contagious and known to science. “Sit,” she said, not expecting him to obey. “Please.”
To her surprise, Diesel sat. He kept the tissues clamped to his face with one hand, the other flat on the floor. He also sat cross-legged.
For several moments, they sat quietly on the floor together, neither saying a word. Susan kept her gaze from the window. Whether or not Diesel knew they were being watched, he did not need to be reminded of it. She let him speak first, knowing instinctively anything she said would only inflame him. Through the years, he had faced the inquisition whenever he acted in an irrational manner: “Why did you do this? Are you insane? What were you thinking?” She wondered what he might say if he had the opportunity to get in the first word.
“I’m a monster,” Diesel said softly. “I hate myself, and I want to die.”
And there’s the depression part. Susan wished she had had more training. Her immediate thought was to disabuse him of such a terrible notion, to assure him he was a sweet boy with a problem, not a monster, that killing himself would only create more problems for all the many people who loved him. But she was sure he had heard it all before, and anything she said in this vein would only shut down a conversation that had just begun, one she suspected had the possibility of yielding important information. “Why do you say such a thing?”
“Because I am. And I do.” Diesel hid behind the wad of tissue, not bothering to look at Susan.
That was useful. Now, Susan worried she had not only shut down the conversation, she had also left him believing she thought him a monster who ought to kill himself. What made me think I could handle this? Susan tried again. “Tell me more. What kind of monster are you?”
Diesel rolled an eye in Susan’s general direction. “I’m a food-hogging monster. If I could, I’d eat Tokyo.”
Susan could not help laughing. “What part of Tokyo?”
Diesel slipped into baby talk. “All of it. Da cakes. Da pies. Da can-dee.” He paused. “Da bui-dings, da people, da can-dee.”
“You said ‘candy’ twice.”
Diesel managed a smile around the tissues. “That’s my favorite.”
Susan could not resist. She slipped a Slookie into his hand and whispered conspiratorially, “Careful how you put that in your mouth. And don’t tell anyone I gave it to you.”
Apparently, Diesel did know about the nurse observing because he peeked sideways at the object, then made a casual motion of moving the tissues and sneaking it into his mouth. “I’m hungry all the time, Dr. Susan. I’m hungry right after dinner. I’m hungry in my sleep. I’m hungry while I’m still eating.”
Susan thought of all the psychiatry she knew about hunger. Most believed it a substitute for something missing, usually love. Nothing in Diesel’s chart suggested inadequate parenting. He had a married mother and father, at least one of whom visited him at every opportunity. They had cooperated in every way with his therapy. They had two other children, an older boy and a younger girl, who seemed normal in every way. From his dress and vocabulary, it was apparent he did not lack for attention, money, or education.
“If I ever threw up, I think I’d be hungry doing it.”
“Gross.” Susan managed to chat even as she mulled the pertinent.
“You’ve never thrown up?”
“I once broke into the freezer and ate three boxes of Popsicles, two things of ice cream, a cake, a loaf of French bread, and a package of shredded cheese in less than five minutes. They said I should be puking all over the place, but they couldn’t even make me.” Diesel seemed almost proud of the accomplishment. “I don’t throw up.”
Susan suspected if she had eaten all that, she would be in a coma. “Wow.” She could think of nothing else to say.
Diesel loosed a raw, honest belly laugh so fun and contagious Susan could not help joining him.
She finally managed, “I’ll bet you could eat Tokyo.”
Diesel laughed again, and Susan realized she loved that sound. She bet people did silly things just to elicit it.
Having stopped his nosebleed, Diesel turned to wiping blood, snot, and tears from his face.
Susan could not help wondering if someone took pleasure in Diesel’s overeating. Perhaps he or she encouraged it, either intentionally or subconsciously. More than one person might be to blame. Maybe even me. She felt a sudden pang of guilt at having slipped him a candy. She wondered how many people had tried to win his trust that way and vowed she would never do so again. “So, how’d you get the nickname Diesel?”
Diesel actually smiled. “Football. I plow through the other line like an old-fashioned diesel truck or train.”
“You’re a lineman?” Susan guessed. She hoped her ignorance of football did not show too much. When she took her pediatrics rotation, she remembered one of the residents telling her the smartest thing a pediatrician could do was to keep up with the trends in gaming, music, and play. Nothing impressed a child more than a doctor who knew the hip shows, the names of the newest characters, or could keep up with him in a game of I-Star.
“Nose guard,” Diesel said. “And center on offense. Right smack dab in the middle of the line.”
Susan looked him over. Though notably short for his age, he was built like a tank. “So, I bet you love the Giants.”
Diesel wrinkled his nose. “I like college. Longhorns.”
“Texas?” Susan asked.
Diesel looked at her as if she had gone mad. “No, the Pennsylvania Longhorns.”
“Pennsylvania?” Susan realized he was teasing her. “Funny.” Of course Texas, you moron. “What do you like about them?”
“For one thing, they’re good. Ten and oh last season. For another, they send a lot of guys to the pros. Especially quarterbacks.”
Susan made a mental note to study up on football, especially the Longhorns. “You think you’re calm enough to go back out there?”
Diesel sighed. “And apologize. Yeah, yeah. I know the drill.”
“Good, because I have to get back to rounds.” Susan looked around the room at the blood-splashed, padded walls. “And someone is going to have to clean up in here.”
Diesel followed Susan’s gaze as if noticing the mess for the first time. Only then, he studied himself. He looked as if he had gone ten rounds in an ultimate fighting ring.
Susan rose, walked to the door, and knocked politely.
It swung open immediately.
“We’re ready to come out,” she announced to the waiting nurse, who gave her a gaze that spoke volumes. Susan had a feeling she was about to face a punishment worse than Diesel’s own.
To her surprise, she did not care.
Chapter 5
When rounds restarted, they naturally focused on Diesel. More confident after their conversation, Susan started again. “Dallas ‘Diesel’ Moore is a ten-year-old black male who has been diagnosed with ADHD, oppositional defiant disorder, severe depression, obsessive-compulsive tendencies, and morbid obesity.” As Susan glanced around the office, she could see the other residents looked more relaxed than they previously did. The hour off had given them more time to meet their patients and review the charts, while she had entombed herself with Diesel. Stony had changed into the blue corridor scrubs that served two purposes. First, they announced their lack of sterility, that the wearer was not headed for the operat
ing room. Second, their bright color was a reminder not to wear them outside of the hospital.
Dr. Bainbridge studied Susan with a bemused expression. “You’re about to tell us you don’t agree with those diagnoses, aren’t you, Susan?” He already had her pegged from her presentation of Starling Woodruff. Worse, he was right.
“Well, actually, sir . . .” Susan paused, uncertain how to continue. She had not expected to get to this point so quickly and had not fully organized her thoughts on the matter. “I think it’s possible he has an undiagnosed syndrome, yes.”
Stony leaned toward her. “What are you thinking?” Less jaded than Bainbridge, he actually seemed eager to hear her theories.
“Well . . .” Susan tried to work through her thoughts as she presented them. A well-reasoned argument would speak volumes over an educated guess. “Obesity has three main causes: familial, psychiatric, and physiological. The first one is the most common, and also the most treatable. The first and second types cover some ninety-eight percent of all cases of obesity.”
“You think Diesel has the third,” Bainbridge said predictably.
Susan did not wish to couch her ideas as speculation or intuition. Those would not fly in a scientific institution like Manhattan Hasbro. “I think there’s a reasonable amount of evidence to come to that conclusion.”
“Evidence.” Bainbridge made a “come here” motion Susan took to mean he wanted more information, not for her to stand beside him.
“Neither his parents nor his siblings are obese. While there is some history of a maternal uncle who was, that’s not strong evidence for familial obesity.” Susan supposed she had not needed to present those details. That Diesel resided in the PIPU proved his previous physicians believed in a psychiatric basis for his problems, not familial or physiological. “Diesel hasn’t responded to any of the standard antiobesity drugs.” A plethora of those had emerged in the early part of the current decade, when medical research dollars had been restored after nearly two decades of attempts to balance the national and state budgets by channeling that money into medical care for the indigent. The antiobesity drugs worked for anyone with even a modicum of self-control and the desire to attain a healthy weight.
Bainbridge made a more severe gesture, as if to get across to Susan they had a limited amount of time.
“Diesel was underweight until about age two and a half, when he developed hyperphagia and put on enormous amounts of weight quite suddenly. That’s remarkably early for nearly all the psychiatric causes. He packed on the weight despite having ADHD, with an emphasis on the hyperactivity part in his case. He started walking very late. Intelligence testing reveals an enormous backward split between performance and verbal aptitude. Most children with learning problems do worse on the verbal parts, but Diesel has a profound vocabulary and the ability to use it. He has much more trouble with dexterity: drawing, writing, shoe tying. Also, he’s short for both his age and his family history, with surprisingly long arms.”
Monk Peterson blurted out, “Prader-Willi syndrome.”
It was the obvious diagnosis. Though rare, it was still the most common obesity syndrome. Even at the turn of the twenty-first century, most children with Prader-Willi died before they reached adulthood. Driven to eat, they would choke to death, poison themselves with spoiled or uncooked foods, or rupture their stomachs. Those who survived all of those things frequently still died young of complications of obesity.
In the last twenty-five years, doctors discovered treating these children with human growth hormone, consistently locking up all food storage areas, and training the families and children from infancy could help delay the development of the deadly hyperphagia and even prevent the obesity in many cases.
“Is he intellectually disabled?” Clayton asked. “All children with Prader-Willi syndrome are.”
Behind Bainbridge’s back, Monk Peterson poked furiously at his Vox. “Not all. Ten to twenty percent have normal IQs, with severe learning disabilities.”
Susan envied him the ability to use the Vox. With Bainbridge’s attention on her, she did not have the same luxury. “And a ten-point split between verbal and performance portions of the IQ test defines nonverbal learning disability. Diesel has a thirty-five point split. Severe.”
Susan could see nurses whispering behind the other residents and knew they had something to say but did not have the temerity to interrupt an attending’s rounds. She addressed them directly. “Has Diesel been tested?”
One of the nurses answered quickly. “Negative.”
Monk punched more buttons. “Methylation or FISH?”
Everyone turned Monk a blank stare, and he casually hid his wrist behind his back.
“Methylation testing is a lot more accurate.”
“It was the more accurate one,” the nurse inserted. “The one that definitely ruled out the syndrome.”
“Methylation, then,” Monk asserted. “It’s reportedly nearly ninety-six percent accurate.”
That put Susan off a bit, though not entirely. She still believed she had read Diesel correctly. “There are other obesity syndromes and states. Prader-Willi is not the only one.”
Directly behind Bainbridge, Monk still worked his Vox. “There’s post-craniotomy syndrome, also known as secondary or acquired Prader-Willi syndrome. It occurs after removal of a craniopharyngioma from the brain.”
As Diesel had not had brain surgery, Susan discarded the possibility. “There are also other congenital obesity syndromes.” She desperately wished she had Monk’s freedom, stuck with racking her brain from genetics class. “There’s Bardet-Biedl syndrome, for instance.” She hoped no one asked for specifics, as she could not recall them. “Hypothyroidism, hypercortisolism, leptin deficiency.” Another thought filled her mind, a brief memory from a single class about rare syndromes. “I’d like permission to explore some of those possibilities.”
Bainbridge stroked his chin. “I see no harm in that. So long as you keep cost in mind. Start with the most inexpensive tests likely to yield the most results.”
Susan had the perfect answer. “He’s had borderline thyroid studies in the past, but it’s only TSH and T4. I’d like to add free T4. Also, how about a vision screening? With a dark room, dilating drops, a cooperative patient, and a decent ophthalmoscope, I can do it myself.”
Now, all attention in the room went to Susan.
Misunderstanding the staff’s sudden interest in her, Susan added, “I believe he’ll cooperate with me. He’s not always in volcano mode.”
Stony explained, “Susan, we’re just wondering what an eye exam has to do with physiological obesity.”
Susan had thought the connection obvious. “In Prader-Willi, the obesity is hypothalamic. The hypothalamus regulates food satiety and hunger, as well as body temperature, mood, thirst, vomiting, pain sensation, hormonal balance and secretion, and some other things I’m probably forgetting. Diesel has six obvious features of hypothalamic dysfunction.” Susan ticked them off on her fingers. “Obesity, hyperphagia, serious mood disorder, short stature, inability to vomit, and high pain tolerance.” She had learned about the last two just minutes earlier. He had directly told her about the vomiting. The high pain tolerance she deduced by his love of football and his positions, despite his squat figure.
“I’m with you so far,” Stony said. “But still not making the vision connection.”
Susan cleared her throat and looked around. Monk still furiously consulted his Vox, but he clearly had not yet found the thread of her logic. “Diesel has no history of head surgery or trauma to indicate he had damage to the hypothalamus. Monk mentioned craniopharyngioma.”
As Susan drew attention to him, Monk swiftly dropped his arm.
“But, as he said, the tumor itself rarely causes these kinds of problems. It’s when the tumor gets irradiated or surgically removed that the hypothalamus becomes damaged. Since craniopharyngiomas are nearly always benign, they have no cancer markers to attack with immunotherapy; and we stil
l have to remove them the old-fashioned, surgical way. So, we can obviously see what causes many of the problems in Prader-Willi syndrome, and post-craniotomy syndrome, is damage to the hypothalamus.”
As Susan explained her theory, she began to realize she had previously jumped several steps. As she filled them in, she saw how she had lost her companions along the way. “So, if I assume Diesel has hypothalamic damage causing his symptoms, and it didn’t come from surgery or head trauma, it has to be something he was born with, right?” She looked around at her fellow residents, the nurses, and Bainbridge. They all still seemed attentive to her. No one wore the “aha” expression of someone who had just figured out something that had previously eluded him.
“The hypothalamus forms during weeks four to six of embryonic development, the exact same time and place as the optic nerve.”
Now, Susan saw a few heads begin to bob.
“Unlike the hypothalamus, which is deep inside the skull, we can actually view the optic nerve directly. We don’t need an MRI or anything fancy. If he has abnormal optic nerves, it would indicate some sort of mishap in weeks four to six of fetal development and would help substantiate my hypothalamic obesity argument.” The coup de grâce delivered, she looked around at her fellow residents.
Stony spoke first. “But if Diesel has abnormal development of the optic nerves, wouldn’t he be blind? Or, at least, wear glasses?”
“I’m not sure,” Susan admitted.
Monk launched into his Vox. Having edged behind Bainbridge, Nevaeh did the same.
Diesel’s nurse, a large man with brown hair and a thick mustache, said, “I’ve often wondered if he didn’t need glasses, but he won’t cooperate with chart vision testing.”
Susan still believed she could get Diesel to allow her to examine him.
Monk finally came back with an answer. “Congenital abnormalities of the optic nerve can result in blindness, decreased vision, loss of snippets of visual field, or even normal or near-normal vision.”
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