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 SLID. 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 bonemarrow 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 humangenetherapy work that Mulligan, Grosveld and the others are working on. They're having some real success in building some ADAdelivering 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 giantcell pneumonia, cytomegalovirus or adenovirus infections, or our old buddy Pneumocystosis carinii . . . one good head cold and the child is gone. Their own proteinlosing 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 genetherapy 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. “Autoimmunological reconstruction? WBCs rebuilt from zero? T and Bcell levels up? Gammaglobulin levels returned to normal? From a SCID child with three hundred lymphs/muone 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, autoimmune reconstruction doesn't happen outside the comic strips. Two, any live vaccines or nonirradiated 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, Katefatal graftversushost disease, progressive generalized vacciniahell, you know what the result would be. There's something wrong with this picture . . . either a misdiagnosis on the Romanian end or a total screwup 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 modem 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 wasalthough 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 Underhillone of the world's top men on hereditary nospherocytic hemolytic anemiaand his secretary Calvin on a search for some antiseptic and a BandAid. 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 sixhundred-milliondollar research center containing stateoftheart medical laboratories and diagnostic equipment . . . and no Mercurochrome or BandAid.
Finally they found some sprayon antiseptic and adhesive strips in the chief administrator's officehe was a fanatic jogger who tended to fall down a lotCalvin 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. Mauberlychief administrator and a PhD doctor in epidemiology, not a medical doctorhad 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 MRimaging, 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 RMCDC'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 ClassVI 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 corpsesboth human and animalthat were the raison d'etre of Pathology and Biolab's daily toil. Mauberly had agreed.