“Yeah, the ambulance . . .” He looked at me curiously. “Say, have you heard this one before?”
“I used to belong to an HMO. Look, Mr. Rollins, I’ll consider your request if you can send me some info on this research project of yours. Diverting already scarce resources for research is a gamble. A worthy gamble, but a gamble nonetheless. Before I roll the dice on an expectant mother hemorrhaging in the delivery room, I want a sense of the stakes for future lives.”
“You think we’re playing God, Sam?”
“One way or another, we’re all playing God, Lou. Most of us just won’t own up to it.”
A thin wail pierced the conversation and I noticed that we had ambled into the maternity wing. The neonatal unit was to my left and I caught sight of a dozen tiny beds and four closed incubators beyond a large glass window. Five babies rested or squirmed in their hospital cradles while a sixth shrieked its pain or anger from the back of the room.
“Well, I’m sure I can get the company to send you some information,” Lou was saying, “but I gotta warn you—it’ll probably be pretty technical.”
“That’s okay,” I said absently, “I’ve been reading a lot of medical research papers of late.”
A nurse was carrying the screaming infant against her shoulder, walking back and forth, trying to soothe it into restfulness. Not anger, I decided, the pitch and tone are pure misery. Acute discomfort, if not actual suffering.
“Well, let me set you up with a tour of the BioWeb facilities,” Lou was saying as he pressed some cardstock into my hands. “And here’s a couple of free passes for our ‘Death Sucks’ blood drive and Halloween dance! Bring a friend. Hell, here’s two more: bring friends!”
I nodded absently as if I had friends. Instead, I concentrated on the baby’s wails, trying to clarify the pattern. Obvious—blatant even—once I figured it out but Rollins had distracted me, preventing me from seeing it sooner. Every time the nurse brought the child in close proximity to the window on the far side of the nursery, it cried all the harder. It might not be obvious to the untrained human ear or a nurse nearing the end of a thirty-six-hour rotation but Lupé says I have the ears of a wolf.
And she should know.
“Excuse me, Lou; did you say you had a meeting in Pediatrics?”
Startled and derailed, it took him a moment to shuffle through his mental scripts. “Actually, I’ve arranged a sit-down with the neonatal supervisor. . . .”
“Great! Can I get an introduction?”
“You want to sit in on the meeting?” Caught between company pitches, he hadn’t regained his balance. Or maybe he didn’t fancy any third parties at the next deal cutting.
“No. I just need a few minutes of whoever’s in charge here’s time.”
“That would be me.” The voice belonged to a short, round woman in pale blue scrubs, cap, and booties. “I imagine one of you is Mr. Rollins.”
“That’s me!” Outstretched hand and thousand-watt smile, Lou Rollins was back on track. “And you must be Anita!”
“Nurse Jensen,” she said with a mild smile. “You’ll forgive me if I don’t shake your hand. I just scrubbed and I have to get back to my babies in a few minutes.”
“Ma’am, what’s the matter with the baby that’s crying in there?” I asked politely. I learned early on that it never pays to be rude to those in authority.
She frowned at me anyway. “I’m a nurse, not a ‘ma’am.’ Are you family?”
“Maybe,” I said. And gave her a mental nudge.
The frown lines deepened but she nodded and answered anyway. “We don’t know, yet.”
“What do you know?” I pushed a little harder.
“Baby Helen has an enlarged liver and spleen. She’s anemic and her bilirubin is elevated.” Her frown deepened. “Doctor suspects EB.”
“EB?” I asked, trying to blend a “please tell me more” tone with a subvocal command to never mind my stranger status and keep talking.
“Epidermolysis bullosa. It’s an inherited disorder that causes blisters to form on the skin at sites of trauma to the body.”
“The child has trauma injuries?”
“She has blisters,” Jensen snapped, eyes narrowing as she shifted her attention away from the what of my question and more toward the why. “Infants with EB are sometimes born with blisters.”
“Sounds as if you need a genetics consult,” I suggested, preparing to mentally cram that request down her throat if necessary. I was spared having to mind-wrestle Nurse Jensen into placing the call by the arrival of a familiar face.
“Mr. Haim?”
I turned and looked at my newest client. “Ms. Delacroix?”
“Dr. Chalice,” Nurse Jensen said, “I was just explaining the infant’s symptoms to this gentleman.”
“Really?” Chalice Delacroix nodded briefly to Lou Rollins, who nodded back. “Why? Is he family?”
Jensen looked a little confused; her frown lines squirmed.
“In a manner of speaking,” I answered. “I think this baby and I have something in common.”
“Oh my,” my client said with a good-natured smile. “I don’t know how I can resist passing up such a wonderful set-up line, Mr. Haim.” She turned and nodded at Lou. “Mr. Rollins. What brings you to neonatal?”
He smiled but leaned forward and lowered his voice to answer: “Umbilical cords.”
I mentally grabbed my eyebrows before they could rise.
“What’s that?” Nurse Jensen’s frown deepened.
Rollins turned back to her and upped the wattage on his smile. “Is there someplace we can sit down and talk?”
“Let me just collect those blood samples and I’ll be on my way,” Delacroix said, placing an insulated carrier on the counter of the nurses’ station. “Then you can palaver to your heart’s content.”
Jensen turned to the small refrigerator at the back of the station and stooped to open the door.
“What makes you think EB?” I asked Chalice.
“I don’t think EB. Doctor thinks EB. I run the samples on the parents and child and screen for a variety of genetic disorders and see what pops up.”
“I thought you worked the night shift.”
“Ditto.”
“An emergency came up,” I answered.
“Ditto,” she repeated with a smile. “Here’s mine. Where’s yours?”
“Downstairs.” I was spared a longer answer by Nurse Jensen’s return with four vials of blood.
“I should have an answer in forty-eight to seventy-two hours,” Delacroix told her.
“Will you screen for Xeroderma pigmentosum?” I asked.
“What?” Jensen asked.
“Why?” Delacroix seconded.
I nodded toward the nursery. “The child shows signs of increasing distress every time she’s carried close to the window.” Another thought occurred and I turned to Jensen. “You said her bilirubin was elevated. Has she had photo-therapy?”
“I don’t know,” Jensen replied, “I just came on duty. Let me check.”
As she went off in search of the chart I looked back at Rollins and Delacroix.
“Billy who?” Rollins asked with a half-smile.
“Bilirubin is a byproduct of red blood cell destruction,” Delacroix explained before I could open my mouth. “Hemoglobin is broken down to heme and globin. Heme is then converted to bilirubin and carried to the liver by albumin in the bloodstream, where it’s further processed and then excreted in the bile.”
The expression on Rollins’ face wavered between “huh?” and “so?”
“The problem,” I said, continuing the explanation, “is that a newborn’s liver isn’t as efficient as an adult’s—it’s just started working for one thing. It takes a few days for an infant’s system to gear up the entire process for breaking down red blood cells and eliminating the byproducts. Sometimes there’s a brief period where the bilirubin builds up in their systems, causing their skin and the whites of their eyes to appear jaund
iced.”
I noticed that Chalice Delacroix eyed me with the same look that most zoo-goers gave the duck-billed platypus.
“Most of the time this is a temporary condition but there are occasions when the bilirubin levels can get dangerously high. If too much accumulates for too long, it can find its way into the central nervous system and cause brain damage.”
“Kericterus,” Chalice said, nodding.
“So I’m wondering if the hospital has tried photo-therapy.”
“Photo-therapy,” Rollins repeated. If he had been following me up to this point I’d clearly lost him now.
“It’s the most common treatment for reducing bilirubin levels in infants,” Chalice explained. “By positioning special fluorescent lights over a newborn, a chemical reaction can be stimulated that speeds up bilirubin breakdown in the bloodstream.”
“So,” Rollins pondered, “you think they ought to try it on the baby in there?”
“No,” I said a little too sharply. I softened my tone. “If this baby is photo-sensitive, it would be harmful—possibly fatal—if she’s exposed to excessive light!”
“What makes you think the child is photo-sensitive?” Chalice wanted to know.
Nurse Jensen returned with the chart and spared me the necessity for elaboration. “Yes. She’s had photo-therapy. In fact we have two blood samples on her, one taken before and the other after, to see if there’s been any changes in the blood chemistry.”
“We need to see the baby,” I said.
“What?” Jensen shook her head. “No. I’m afraid that’s out of the question.”
Chalice glanced at me before giving the charge nurse her full attention. “I really think it might be a good idea, Nurse,” she said.
Jensen’s mouth was set in a tight line. “You have no jurisdiction here, Dr. Delacroix.”
“Let us take a quick look,” I said reasonably. You won’t regret it! “You won’t regret it.”
“I—I shouldn’t—”
“It’s all right,” I said. Really! “Please?”
“Perhaps . . .” Jensen was wavering. Rollins just stared at us, bug-eyed.
“Think of it as getting a second opinion.” Let us in! I pushed, finally out of patience as the infant continued to squall in the next room.
Jensen opened the half door that permitted egress into the nurse’s station and led us into the adjoining nursery. The nurse who had been carrying Baby Helen had placed her back in her isolette and was tending to another infant now. She looked up at our approach. “I couldn’t get her to stop crying, Anita.”
“It’s all right,” Jensen said. “We’ll—I’ll take over for now.”
“I think her blisters are worse,” the other nurse said as she made notations on another chart. “When is our consult coming?”
“I’m here,” Chalice answered. “I’ve already logged the tests as high priority but I’ll camp in the lab and try to push to the front of the line if I can.”
While they were talking I reached into the isolette and retrieved Baby Helen. “Oh dear God!” I whispered as I drew her close to me. The child was covered with vesicles or bullae—quarter-sized blisters. I touched one and, as it gave under the light pressure of my finger, it oozed clear fluid. It could still be XP but I had another idea. “I need a Wood’s lamp.”
“Put that child down,” Jensen said, reaching for Baby Helen.
“Get me a Wood’s lamp now!” I barked, applying enough pressure to jumpstart my own headache.
Pain and confusion in her eyes, Jensen turned and hurried away.
Chalice eased her hands between mine. “May I?” she asked carefully. Just as carefully she eased the infant up and over just enough to get a good look at her back. “See the bullae here? The vesicles are smaller and more newly formed.”
“The blisters on her front are probably from the bili-lights,” I reasoned. “Her back would have picked up indirect sunlight while she was being carried close to the window.”
Chalice nodded. “A difference in time and light intensity. Let’s check the diaper.”
I laid the baby down on the changing table. Jensen returned with a Wood’s light as we unfastened the diaper and folded it down. Stains, as though someone had spilled a small amount of red wine, marked the inner layer. Also telling was the absence of blisters on the skin that had been shielded by the diapers.
“See this?” I said to the group as they gathered around closely. “The reddish color in the urine? Someone douse the lights and plug in that lamp.”
While Chalice plugged in the Wood’s lamp and positioned it, Jensen turned off the room’s lights and the other nurse lowered the shades over the observation windows to block the light from the hallways.
As the Wood’s lamp flickered into an eerie purplish fluorescence the stained area of the diaper began to glow an unearthly pink.
“What you’re seeing now,” I continued, “are the abnormal proteins that have been excreted through Baby Helen’s renal system.”
“Porphyrins,” said Chalice.
“Right. And I think the proper tests will confirm one of the porphyries—probably CEP.”
“CEP?” Jensen’s licorice frown was even scarier in the violet murk of the Wood’s light.
I nodded. “Congenital erythropoietic porphyria.”
“It’s pretty rare,” Chalice observed. “Even among the known porphyrias.”
“Less than a couple of hundred known cases worldwide,” I agreed. I looked over at the head nurse. “You’re going to need a serious genetics consult. I can give you a list of experts in the field of porphyrias and photo-sensitive disorders such as XP if the hospital doesn’t have ready access.” I switched off the Wood’s lamp, and the purple and pink luminescences faded. Now immersed in darkness, the room’s only illumination came from a Christmas-y constellation of red and green LEDs on the natal monitors.
“They’ll recommend more specific treatments but, starting right now, you’ve got to keep this infant away from direct light sources. No windows. No bili-lights. In fact, she will be safer around incandescent bulbs than fluorescent lamps as they emit less porphyrin-exciting wavelengths. But any light at all is a hazard. Keep her in the dark as much as possible until you’ve got a doctor on the case that knows CEP!”
“How serious is it?” Jensen asked as she picked up a blanket and began to drape Baby Helen’s isolette.
“Very serious. Although a lot better now than once upon a time.” I explained as best I could how the absence of the enzyme uroporphyrin in the body’s cells created two serious problems. First there was the issue of heme, an essential ingredient of hemoglobin that victims of CEP couldn’t manufacture. Transfusions and bone-marrow transplants could help, assuming the right genetic donors could be found, but that was a trickier business than one might assume. The second problem echoed the bilirubin issue: the uroporphyrin enzyme deficiency prevented the breakdown of heme’s toxic protein precursors—porphyrins. The buildup of toxic levels of these porphyrins in the bloodstream and urine produced a number of unpleasant side effects. The photosensitivity not only produced blisters on the epidural surfaces but also caused scarring and even patches of hair to sprout where it might not normally grow. Porphyrin deposits on teeth and bones produced a reddish discoloration and made them brittle. Even so, with proper treatment and careful avoidance of sunlight, most patients with CEP could now anticipate a life expectancy of forty to sixty years.
“Once upon a time they would have lived short, painful lives,” I concluded. “Shorter, if the locals decided they were vampires.”
The other nurse gasped as she turned on a small lamp at the far end of the room. “Vampires?”
“Receding gums giving the teeth an elongated appearance, already stained red from the porphyrin deposits . . . reddish urine . . . extreme pallor and a nocturnal lifestyle.” I omitted the fact that garlic was also a no-no, due to the fact that it painfully stimulated heme production.
Jensen made a call as we l
eft, demanding an immediate genetics consult and would someone please page the attending Pedes physician. Stat!
“You’re extremely well-read on the porphyrias,” Chalice said as we rode down on the elevator together, “for a private investigator.”
“I have a wide range of interests.”
“You work the night shift, wear a hat, sunglasses, protective clothing . . .” her nostrils flared, “ . . . sunscreen . . . pardon my nosiness, Mr. Haim, but do you have something like CEP?”
“No,” I said as the doors slid open on the second floor. “Nothing like, at all.”
Unfortunately.
I stepped out and walked away as the doors closed behind me.
Chapter Six
I found Olive and her sister camped out in the visitors’ waiting area just down the hall.
“I just don’t understand it,” Claire said, staring dully at the floor. “He was as healthy as a horse yesterday. Wakes up this morning with a cough—just like Mr. Lloyd.”
Olive slipped her arm around her sister’s shoulders. “Jamal’s had the flu before.”
“Not this flu. This flu be killing people.” Claire shook her head. “They won’t even let family in to see him.”
The first hint of distress crept into my secretary’s voice. “They won’t let you see him?” The receptors in the vomeronasal region of my nose caught a faint odor of Olive’s stress pheromones behind the miasma of fear surrounding her sister like a clammy fog.
“They let me stand outside a special room and look at him through a window. They say they have to keep him in isolation.”
“It’s for your protection as much as your son’s,” said a new voice.
We all looked up at a man who had seemingly appeared out of nowhere. With broad shouders and blond hair in a crew cut, he looked like someone had locked Drew Carey in a gym and taken away his glasses. His three-piece suit fit oddly, as if tailored for someone else. Or maybe it was that the man, himself, was proportioned just a bit strangely. “May I speak with you about your son’s situation?”
Olive’s eyes gave him the quick once-over. “Are you a doctor?”
Dead on my Feet - The Halflife Trilogy Book II Page 9
