She resisted the urge to sigh. “I’m afraid so. The child shows severe adenosine deaminase deficiency.”
“ADA too?” interrupted the doctor on the other end of the line. She heard his teeth click on the stem of his pipe and imagined the pained expression on his face. “The poor little bastard has all four types of SCID. The symptoms usually manifest themselves between the third and sixth month. How old did you say he was?”
“Almost nine months.” Kate thought of the “birthday cake” Julie would be shopping for at King Soopers. They celebrated Joshua’s “birthday” every month. She wished she had taken time to shop for the cake herself.
“Nine months,” came Paul’s voice, obviously musing to himself. “I don’t know how the little guy got that far…he won’t be getting much older.”
Kate winced. “That’s your prognosis, Paul?”
She heard fumbling sounds at the other end of the line and could almost see the rumpled researcher sitting up, setting his pipe on the desk. “You know I wouldn’t make a prognosis without seeing the patient and the tests in person, Kate. But…my God…signs of all four of the SCID variations. I mean, if it were just the ADA it would be bad enough… Has there been a haploidentical bone-marrow transplant?”
“There’s no twin,” Kate said softly. “No siblings at all. The orphanage couldn’t find even the parents. Obviously no histocompatibility is possible.”
There was silence for a moment. “Well, you could still try ADA injections to restore some of the immune function. Also shots of transfer factor and thymic extracts. And there’s the human-gene-therapy work that Mulligan, Grosveld and the others are working on. They’re having some real success in building some ADA-delivering retroviruses…” His voice trailed off.
Kate said what her friend would not. “But with all four types of SCID present, the chance of avoiding a killer germ while the gene therapy was building resistance would be…what, Paul? Too small to count?”
“My God, Kate,” said the researcher, “you know as well as I do that all it takes to bushwhack a SCID’s kid is one infection…generalized chicken pox, measles with Hecht’s giant-cell pneumonia, cytomegalovirus or adenovirus infections, or our old buddy Pneumocystosis carinii…one good head cold and the child is gone. Their own protein-losing enteropathy adds to the problem. It’s like greasing a slide and then going down it on waxed paper.” He paused for breath, obviously upset.
Kate spoke softly. “I know, Paul. And I used to do that too.”
“Do what?”
“Grease the slide on the playground and go down it on waxed paper.”
She heard him chewing on his pipe again. “Kate, are you working with this child…personally, I mean?”
“Yes.”
“Well, I’d put my hope on the gene-therapy work being done and hope for the best. There’s a lot of energy going into solving the ADA problem these days, and if you lick that, the Swiss type, B lymph, and reticular dysgenesis malfunctions can be attacked with more conventional immunological reconstructive techniques. I’ll fax you everything we have on Mulligan’s work.”
“Thank you, Paul,” said Kate. The deer had gone back into the pine forest when she was not looking. “Paul, what would you say if I told you that this child’s symptoms were periodic?”
“Periodic? You mean varying in severity?”
“No, I mean literally periodic. That they appeared, grew critical, and then were beaten back by the child’s own rebuilt system?”
This time the silence extended for almost a minute. “Auto-immunological reconstruction? WBCs rebuilt from zero? T- and B-cell levels up? Gammaglobulin levels returned to normal? From a SCID child with three hundred lymphs/mu-one as a starting point? With no histocompatible marrow transfusions, no ADA retrovirus gene’ therapy?”
“Correct,” said Kate. She took a breath. “With nothing but blood transfusions.”
“Blood transfusions?” His voice was almost shrill. “Before or after diagnosis?”
“Before.”
“Bullshit,” said the researcher. Kate had never heard him use a curse word or vulgarity before. “Absolute bullshit. One, auto-immune reconstruction doesn’t happen outside the comic strips. Two, any live vaccines or non-irradiated transfusions for this child prior to diagnosis would have almost certainly killed him…not brought about some miracle cure. You know the problems an allogenic transfusion would cause, Kate—fatal graft-versus-host disease, progressive generalized vaccinia—hell, you know what the result would be. There’s something wrong with this picture…either a misdiagnosis on the Romanian end or a total screw-up in the T-cell study or something.”
“Yes,” agreed Kate, knowing that the data was valid. “I’m sorry to take up your time on this, Paul. It’s just that things seem a bit muddled.”
“That’s an understatement,” came her friend’s voice. “But if anyone can straighten it out, you can, Kate.”
“Thank you, Paul. I’ll talk to you soon.” She set the phone in its cradle and stared out at the empty meadow.
She was still staring two hours later when her secretary buzzed to tell her that Julie was there with the baby.
Even after fifteen years as a physician, Kate thought that the saddest sight on earth was a small child surrounded by modern medical equipment. Now, as a mother watching her own child submit to sharp needles and frightening equipment, she found it twice as sad.
Julie had shown up weeping and apologizing. It took several minutes for Kate to understand that the girl had set Joshua loose in his baby seat for a moment in the front seat of the Miata—“just while I put his birthday cake in that dinky little trunk”—and the child had tumbled out, hitting his forehead against the center console. There had been little bleeding, Joshua had already stopped crying, but Julie was still upset.
Kate had calmed her, shown her how slight the abrasion was—although there was going to be a serious goose egg—and then led a small parade of Josh, Julie, Kate’s secretary Arleen, her office neighbor Bob Underhill—one of the world’s top men on hereditary nospherocytic hemolytic anemia—and his secretary Calvin on a search for some antiseptic and a Band-Aid. Kate found it amusing, and even Julie began chuckling through her tears that there they were in the Rocky Mountain Region Center for Disease Control, a six-hundred-million-dollar research center containing state-of-the-art medical laboratories and diagnostic equipment…and no Mercurochrome or Band-Aid.
Finally they found some spray-on antiseptic and adhesive strips in the chief administrator’s office—he was a fanatic jogger who tended to fall down a lot—Calvin brought a lollipop for Joshua, Julie left in a better mood, and Kate brought her baby down to the basement Imaging Center.
When the Center was being moved to the NCAR complex, Dr. Mauberly—chief administrator and a PhD doctor in epidemiology, not a medical doctor—had opposed the presence of the magnetic resonance imagers in the same complex as CDC’s pride and joy, the twin Cray computers on the second floor. Mauberly and the others knew that in the early days of MR-imaging, faults in shielding had ruined wristwatches and stopped automobiles on the street outside. Or so the tales went. Dr. Mauberly wanted to take no chances with the Crays that represented a sizable chunk of RM-CDC’s budget.
Alan Stevens and the other technicians had convinced the administrator that the Crays’ brains and cojones were in no danger from the MR and CT scanners; Alan had shown how the basement imaging complex would be electromagnetically isolated from the rest of the world, literally a room within a room. When Dr. Mauberly had still hesitated, Alan brought in the pathologists and Class-VI Biolab glamour boys. The MR and CT equipment might not be necessary for living patients, they pointed out, but it was absolutely vital for the corpses—both human and animal—that were the raison d’être of Pathology and Biolab’s daily toil. Mauberly had agreed.
Alan met Kate in the basement hallway between the imaging and sealed lab centers. Joshua had been here before and was not afraid of it, although this time there was a
nasty surprise as RN Teri Halloway was waiting in the imaging room with an i.v. tube and needle. Joshua wailed as the needle was inserted on the inside of his skinny arm. Kate tried not to wince. She would have handled the transfusion herself, but Teri had a gentler touch. Sure enough, Joshua quit crying after only a pro forma protest and lay back blinking. Alan and Kate helped set him onto the imaging palette, setting his head firmly in place with pillows and strips of broad tape, also taping his wrists to the pillows. It was disturbing to see, but they could not run the risk of the baby turning and moving during the imaging sequence. Not only would it ruin the CT pictures, but it would dislodge the bio-sensors Teri was setting in place so they could monitor real-time physiological changes. Kate leaned over and cooed to Joshua during the entire preparation, having his favorite stuffed animal—a Pooh bear with one eye missing—talk and play with him. He barely seemed to notice when Teri pricked his finger for the first of many blood tests. The nurse nodded at Kate, smiled at Joshua, and hurried off to the adjoining lab.
Finally Kate tucked Pooh in next to her son and left the room. Airlock-style doors slid shut behind her. She joined Alan at the bank of video monitors.
“Is his runny nose just from crying or have the flu symptoms returned?” asked Alan.
“The last three or four days,” said Kate. “The diarrhea’s back, too.”
Alan nodded and pointed toward the bio-sensor readout. “His temperature is closing in on a hundred. And look at the results of the first test Teri took.”
Kate was looking. Data from the lab was fed directly through to the MR/CT control room. According to the first test, Joshua was showing the characteristic SCID shortage of white blood cells—the WBC count was at 930 lymphocyte/µl—as well as the classic dropoff in T-cell, B-cell, and gammaglobulin levels. More than that, liver enzymes were elevated and there were indications of an electrolyte imbalance.
“Looks like GVH problems to me,” said Alan.
Kate tapped a pencil against her teeth. “Yes, except it’s been almost a month since the last transfusion, and he showed no graft-versus-host rejection then. It’s not the new blood his body has trouble with…it’s his own system he seems to want to reject.” She glanced at the monitor. Joshua seemed frail and insignificant strapped into his imaging cradle. She could see his mouth move as he cried, but there was no sound. Kate switched on the audio pickup and keyed the microphone so that he could hear her. “It’s all right… Mama’s right here… It’s all right.”
She nodded at Alan. “Let’s do it and get him out of there.”
Alan’s fingers played the console as if it were a Wurlitzer keyboard. Joshua’s imaging palette slid him into the CT torus, and Kate had the surreal sense that he was a tiny, human artillery shell being loaded into the breech of a plastic cannon. She watched as the display showed that the i.v. drip had been opened to the whole blood, then as the bio-sensors began to relay Joshua’s body’s response to it. Three-dimensional images of his liver, spleen, and abdominal lymph nodes began to build up on the monitors.
“To do this right,” said Alan, his eyes moving from monitor to monitor, “we should scan his spleen using 99mTc colloid or heat-damaged red blood cells so we could get a detailed fix on any functioning splenic tissue.”
“Too invasive,” snapped Kate. Her eyes stayed glued to the bio-sensor columns. “We’ll stick to the CT, MR, and ultrasound,” she added, her voice softer. “I don’t want him to go through any more than he absolutely has to.”
Alan nodded agreement. “OK,” he said, “coming up to the scan of stomach wall…right…here.”
Kate leaned over, stared at the central monitor, and frowned. “I don’t see the abnormality we found last time.”
“The CT can’t pick up anything less than two centimeters,” said Alan. “At this point we’re dealing with a slightly fibrous mass, smaller and less dense than most tumors. Ultrasound isotopic imaging with 67Ga- and 111In-labeled leukocytes would show it was something worth worrying about, but the CT just gives us the slightest indication of an abscess…there, see that shadow?”
Kate did, but only because Alan’s finger tapped the monitor at the precise spot. It was the shadow of a shadow. She looked back at the bio-sensor columns.
“My God,” she whispered, “his temperature is at a hundred and three and rising. Stop the sequence, I’ve got to get in there.”
Alan grasped her forearm. “No, wait… I’ve got a hunch on this, Kate. We weren’t monitoring his temperature last time, just taking pictures. My guess is that whatever’s going on with the redistribution of blood to that shadow organ in his stomach wall, it’s burning up lots of energy.”
“It’s burning him up,” said Kate. “Abort the sequence.”
Alan set his hand above the red master switch, but then raised it and pointed. “Look.”
Joshua’s temperature now hovered at 103.5, but the other sensors showed near chaos. His blood pressure spiked, normalized, then spiked again. His heart rate was fifty percent over normal. Skin resistance traced a mountain range of changes.
Kate leaned over the console, her mouth open. “What’s happening?”
Alan pushed his glasses higher on his snub nose and pointed to the primary monitor.
The shadow on Joshua’s stomach wall had materialized into a vein-and-capillary-rich mass. The CT scan showed a nexus of nerves that was almost three centimeters across and growing.
“He’s stabilizing,” said Alan, voice tense.
Kate saw that he was right. Temperature, blood pressure, heart rate, and the other vitals were dropping back into the normal range.
“We’re finished with the first sequence,” said Alan. The monitor showed the palette sliding out. Joshua was squirming a bit within his restraints, but showed no signs of trauma or discomfort. He was not crying. Alan looked at Kate over the tops of his glasses. “Do you want to go in with Teri for the next round of blood and pictures or shall we scrub this right now?”
Kate hesitated only a second. The mother in her wanted to lift her son out of that torture device now…take him home now. The doctor in her wanted to find out what was trying to kill him, and find out now.
“Call Teri,” she said, already heading for the airlock. “Tell her I’ll help her draw the next blood sample.”
The three imaging sequences took less than fifty minutes. Joshua had wet his diaper—they’d had a catheter rigged for urine samples but it had overflowed—but other than that and a lot of rage at being restrained for so long, the baby seemed fine as Kate lifted him out and rocked him while Teri and Alan helped detach the bio-sensors. Teri took the last blood sample, pricking Joshua’s big toe again, and the small room echoed to his wails.
As they left the imaging complex, Alan said, “I’ll program the entire sequence to deal with different variables and have the enhanced videotapes ready to roll by eight A.M. Shall I start with T-cell rate or the adenosine deaminase curve?”
“Do the AD,” said Kate. “But I want it visually all cross-referenced.”
Alan nodded and made a note on a small pad.
“Well have all the lab data back by six P.M.,” said Teri. “I’ll make sure McPherson handles it personally.”
Kate used her free hand to pat the nurse’s shoulder.
“Oops,” said Teri. Joshua’s Band-Aid had been loosened from rubbing against his head restraints. Teri pulled it free. “Well, I guess we don’t need this old thing, do we, sweetums?”
Alan caught Kate’s hesitation and sudden attention. “What?” he said, some concern in his voice.
Kate made sure that her voice was calm and level. “Nothing. I was just hoping that this doesn’t screw up his nap schedule.”
Swinging him around slightly, bringing out the first smile of the afternoon, Kate brought her son’s forehead closer to the overhead light. She leaned closer and kissed him, her eyes only inches away from the sweet-smelling skin of his scalp.
The nasty bruise and abrasion he’d received less than tw
o hours before were gone. No pooled blood under the skin, no sign of swelling or lingering hematoma, not the slightest sign of the raspberry rash that should have taken a week or two to fade.
The wound was gone. As if it never existed.
“This should be fascinating stuff,” said Alan, returning to his console. “I can hardly wait.”
“Me too,” said Kate, looking into the baby’s eyes and realizing that her heart was pounding wildly against her rib cage. “Me too.”
Chapter Fifteen
ON Saturday morning Tom drove his Land Rover to Kate’s house, she loaded picnic things in her backpack, Tom stowed Joshua in the backpack carrier, and they hiked the easy mile to Bald Mountain. Technically, Bald Mountain was part of the Boulder city park system, but it was far enough away from the town not to get too many hikers and picnickers. Kate had always loved it for its view; it was just that much higher than her home to open up a wider vista of high peaks and plains.
The July sun was hot and they paused several times while climbing the hill to let the breeze cool them. At one of these times Kate had the out-of-body view of the three of them, Joshua well and happy on Tom’s broad back, her ex-husband grinning and not the least out of breath, the breeze ruffling her own hair and the sunlight hot on her bare legs. She could not help but feel a pang of loss at this snapshot of their family that could have been.
The summit of Bald Mountain was almost devoid of trees, which made the view that much more impressive. Kate spread the blanket she had packed up, they set Joshua down to play, and Tom began setting out picnic things. The sky was a faultless arch of blue. Heat shimmered on the plains to the east and Kate could see sunlight glimmering off windshields on the narrow ribbon of the Boulder Turnpike to Denver. Only the smallest pockets of snow remained on the Indian Peaks to the west.
“Deviled eggs,” said Tom. “You sweet thing.”
Kate hated deviled eggs, but she had remembered Tom’s fondness for them. She cut a slice from the roll of French bread and folded it around some turkey. Joshua ignored the food and crawled over Tom’s knee to get off the blanket and onto the grass.