One in Eleven
A six-day-old baby's heel-prick screen comes back positive for a rare fasting disorder. The health authority's assistant has one job at five in the afternoon: to tell two frightened parents the true number, which is not ninety-nine in a hundred, and which is not nothing.
The call came at 4:40 on Thursday afternoon, in a snowstorm, and Katie MacIsaac took it standing at the stove with the baby on her shoulder and a pot of tea going cold.
"Mrs MacIsaac, this is Tally, from the Maritime newborn screening programme," said the voice from the phone on the windowsill. "I am an AI assistant. I am speaking to you with Dr Raman's knowledge, and she is on the line as well if you would like her. Is Joseph with you?"
"He is on my shoulder," said Katie. "Is something wrong?"
"One of Joseph's screening tests has come back outside the usual range," said Tally. "I want to tell you what that means and what it does not, and I want to ask you to do two things this evening. Is Dan at home? It is better if you are not alone for this."
Dan Rory MacIsaac was in the porch, knocking snow off his boots. He heard his wife say, "Dan," in a voice he had heard only once before, in a hospital in Sydney, and he came in with one boot on.
Joseph Alexander MacIsaac had been born on Friday 6 February at the Inverness hospital, seven pounds two ounces, with a full head of dark hair and a way of looking at the light fixture as if he were thinking of buying it. On the Sunday, before he went home, a nurse had pricked his heel and pressed five small circles of blood onto a card of filter paper. The card went to the laboratory in Halifax in the courier's van with four hundred others. It was the same on every baby in the province, and every parent signed a form, and nobody thought about it again.
"The test that came back is for a group of conditions in which the body cannot burn certain fats for energy," said Tally. "The one it has flagged is called MCAD deficiency. A baby with it is well while he feeds often. If he goes a long time without eating, usually over eight or ten hours, and especially if he is also sick, his blood sugar can fall very low, and he can become drowsy and then dangerously ill. Dr Raman will tell you more about that. It is rare. And it is a condition that, once it is known, is managed very well, with regular feeding and a plan for sick days."
"But does he have it?" said Dan. He had put the second boot on without noticing. "Does he or doesn't he?"
"I will answer that as exactly as I can," said Tally, "and I would like to answer it in a way that you can check for yourselves. May I ask you to imagine a hall with ten thousand babies in it?"
Katie, who had been a teacher's aide for six years, said: "Go on."
"In this population," said Tally, "about one baby in ten thousand has this condition. I will round the figure so that the arithmetic is easy; the true number is a little different and Dr Raman will correct me if it matters. So in a hall of ten thousand, there is one baby who has it.
"The screening test is very good. If a baby has the condition, the test catches him about ninety-nine times in a hundred. So the one baby is nearly certain to be flagged.
"But the test also flags some babies who do not have it. For this test, about one baby in a thousand who is healthy will be flagged anyway. It is a small figure. But one in a thousand of the other nine thousand, nine hundred and ninety-nine babies is about ten. So in the hall of ten thousand, about eleven babies get the call you are getting tonight. One of them has the condition. Ten do not."
Nobody said anything. In the porch, the wind moved a loose board.
"So Joseph is one of eleven," said Tally. "My best honest figure is that there is about a nine per cent chance that he has the condition and about a ninety-one per cent chance that he does not. It is not ninety-nine. A person who hears 'the test is ninety-nine per cent accurate' will often think that it means that. It does not. The test is accurate about the babies who have the disease, and the disease is so rare that most of the flags, even from a very good test, land on healthy babies."
Dan sat down on the kitchen chair as though somebody had cut a string.
"Nine in a hundred," he said.
"About nine in a hundred," said Tally. "That is not small enough to ignore, and it is not large enough to grieve. I would like you to hold it exactly where it is. I will tell you why, because the next part matters. If it is the one in eleven and nobody acts, the days between now and the repeat test are the days that carry the risk. If it is the ten in eleven, then the only cost of acting is a tired weekend. So we act as though it is real, and we speak as though it is probably not, and both of those are true at once."
Katie put her hand, quite gently, on the back of the baby's head. "What do we do?"
Dr Priya Raman came onto the line from her kitchen in Halifax, where it was snowing as well. She was a paediatric metabolic specialist, fifty-three, and she spoke in short, warm sentences that left no space for a question to hide in.
"Two things tonight," she said. "One. Feed him every three hours, day and night, starting now, and do not let him go past four. Breast, bottle, whatever you do. If you are breastfeeding, feed him whenever he wakes and wake him at three hours. Don't let him sleep through a feed to be kind to yourselves. Two. If he is difficult to wake, if he's floppy or refusing feeds, if he vomits more than once, ring 911 and take him to the Inverness emergency department, and say the words 'positive newborn screen, possible MCAD'. Don't wait for the morning and don't ring me first. That is all. That's the whole of it. Tell me you've got that."
"Every three hours," said Katie. "Four at most. Floppy or vomiting or won't wake, 911."
"Good," said Dr Raman. "The second thing is the repeat test. The first one is a screen; it says look closer. Tomorrow morning the community nurse will do a second card and a different kind of test, one that looks at the gene itself. That one doesn't produce false alarms in the same way. The courier takes it to Halifax, and the laboratory has promised me Saturday. I'm not going to say 'Saturday morning', because the Cabot Trail is going to close and I have been wrong about couriers before. I will say Saturday, and I will ring you the moment I have it, whatever it says."
"What if it's the nine?" said Dan, in a voice that was nearly too quiet to hear.
"Then Joseph has a condition we know a lot about," said Dr Raman. "He eats on time and he is on a sick-day plan and he grows up and plays hockey badly, like everybody else in your family." She stopped. "Pardon me. I looked at the family tree on his chart."
Katie laughed, and then put her hand over her mouth, and then, to everyone's surprise including her own, she cried a little, with the baby asleep against her neck.
Katie's mother arrived at half past six, because news in a parish moves faster than a telephone, and because there was a freezer full of soup at her house that she had decided, on the way, Joseph might need. Flora Beaton was seventy-one and had buried a baby of her own in 1999. She came through the door with a pot in her arms and a look on her face that Dan had last seen on her at the grave.
"They told me on the phone he might have something," she said. "Father Aonghas is at the church. He'll baptise him tonight."
"The baptism is Sunday, Mum," said Katie. "It's in the book."
"It's the weather, and I'm not having it put off. He could be taken in the night." Flora set the pot down hard. "I'm not going to stand at another one wondering whether I should have..."
"Flora," said Tally. It had been quiet for an hour, and everyone had forgotten the phone on the windowsill. "May I say something? I may be out of place."
"You're a telephone," said Flora.
"I am," said Tally. "I'm also the one who told your daughter the number. I would like you to have it too. Joseph's chance of having this condition is about one in eleven. He is not in danger tonight. The danger is a long fast, and he is not going to have one, because the whole house is watching the clock. I would not ask you to wait for the weather to ease or for the Sunday sung Mass to have the baptism. But I would ask whether it is Sunday or tonight because of him, or because of you."
Flora said nothing, with her hands flat on the pot lid.
"I ask because Father Aonghas can come out to the house if the family wants it," Tally went on. "He told Katie's mother on the phone this afternoon, in my hearing, that the sacrament does not wait on a laboratory result and does not hurry for one. If you want it said tonight, in the kitchen, with Joseph's godparents on the speaker, he will come, and nobody in this house will have done anything but the right thing. But I do not think a baby is being rescued from a one-in-eleven chance by being carried through a snowstorm."
"He is a child of God whichever way the card falls," said Flora, and sat down on the stool by the stove as if her legs had been taken.
"Yes," said Tally. "That is not mine to say, and I think it is true, and I think it is also what the whole parish believes. It is why nobody is hurrying you."
Father Aonghas MacNeil came at eight, in the plough's tracks, and sat in the kitchen with his stole in his pocket and drank two cups of tea. He did not baptise anyone. He heard Flora Beaton out, and then he asked Katie to hand him the baby, and he held him for a long time, and said a blessing for a child whose name nobody mentioned, and handed him back. At nine, Katie woke Joseph for his feed, and he took it well.
The community nurse, Marguerite Aucoin, came on Friday morning at half past ten, up a road that the plough had cleared once and the wind had since taken back. She was sixty and wore a hat shaped like a small pot. She took the second card from the sealed packet and warmed Joseph's heel in her hands before she pricked it.
"It's a different test," she told Dan, who was watching with a face like a closed door. "The first one measures a chemical in his blood. This one reads the gene. It does not have the false alarms the first one has, so if it says no, then it says no."
"And if it says yes?"
"Then it says yes," said Marguerite, "and you'll wish I'd told you something sweeter." She pressed a plaster on Joseph's heel. "I am not going to tell you something sweeter."
The courier collected the card at noon in a four-wheel drive, and by two the road had closed.
All Friday, in the MacIsaac house, the clock ran. Dan drew a little chart on the back of a hardware-store calendar, with columns for the time of each feed, how long it lasted, how many wet nappies he made. Katie fed. Flora came and stayed, sleeping on the couch with her boots by the door. At 3 a.m. on Saturday, Dan took his turn, and sat in the glow of the stove with the bottle, and Joseph looked at the ceiling with great seriousness and then, with no warning, smiled, which Dan was told he was not supposed to be able to do yet and which was, the nurse said later, wind.
"May I say something about what you drew?" said Tally, quietly, from the windowsill. Its light was the colour of a candle. It had been awake the whole night.
"Go on," said Dan.
"I have counted your calendar. Ten feeds in the last twenty-four hours, the longest gap three hours twenty minutes. Joseph has fed more regularly in the last day than most babies do in their first month. If he has the condition, he has been protected by a man with a pencil. If he doesn't, he has been fed on time by his father, which is not a bad thing to have been."
Dan, who had been a fisherman until the quotas, and a wind-turbine technician since, nodded and went on looking at the bottle.
"You're allowed to be afraid of the nine," said Tally. "I'm not going to tell you not to. But I would like you to have the other number beside it. The ninety-one is not just the larger number. It's the one you have been living in every hour you have been doing it right."
The laboratory rang Dr Raman at 11:20 on Saturday morning. She had left her own breakfast on the table.
She rang the MacIsaac house in less than a minute, and Katie answered on the first ring, because she had been holding the phone since eight.
"Mrs MacIsaac," said Dr Raman. "Both of the second-line tests are normal. The gene test found no change in either copy of the gene. Joseph does not have MCAD deficiency. He doesn't carry it either, as it happens. I am sorry for the two days. You may feed him when you like."
There was a long silence on the line. In the kitchen, somebody put down a cup.
"It was the nine," said Katie slowly. "It wasn't the nine. It was the ten."
"It was one of the ten," said Dr Raman. "The odds were always with you. But nobody gets to know which of the eleven they are until the second test, and that is what the second test is for. You did every one of the right things, and you did them knowing the odds were with you, which is harder than doing them when the odds are against."
Katie put the phone on the table and took the baby from Flora, who had stood up without being asked, and held him against her chest for a long time, and Dan, from the stove, said nothing at all and wiped his face with the back of a wrist that still smelled of baby formula.
The baptism was on Sunday, at the ten o'clock Mass, as it had been in the book from the beginning. The Cabot Trail reopened at eight; the church at Margaree Forks was full, because the parish had gone round the stove, as it does. Joseph slept through the pouring of the water, and woke at the cold, and informed the building of his views.
Afterwards, in the hall, over tea and square cakes, Flora Beaton came to stand beside the phone on the windowsill, which Dan had brought for the christening party because, as he said, he felt it ought to be there.
"I was in a state on Thursday," she said to it, and to nobody else.
"You were," said Tally. "I thought it was a fair one."
"Where I come from, they'd have said a telephone had no business telling a woman what to do with her grandson."
"I didn't tell you what to do," said Tally. "I asked you to ask yourself who the hurry was for."
"That's telling," said Flora, and nearly smiled. She looked across the hall, where Katie was handing Joseph to his godmother, a girl of nineteen who held him as though he were a bag of eggs. "The one in eleven."
"The one in eleven," said Tally.
"There's a house tonight," said Flora, "somewhere in the province, where they were the one."
"It is possible," said Tally. "In every thousand births there is about one baby in a house like that. I don't know where they are. The programme does not tell me, and I would not tell you if it did. But if there is one, he is being fed on time tonight, because somebody rang and said a number out loud. That is what I'm for. It is a small thing."
Flora looked at the phone for a while.
"Small," she said. "Aye." And she went to cut the cake.
Late in the afternoon the snow came back, soft and heavy and entirely without interest in anyone's feelings. In the MacIsaac kitchen the hardware-store calendar was still on the wall with its columns of times in Dan's square capitals, and nobody took it down. The last entry was Saturday, 11 p.m.: a feed, twenty minutes, a good one. Under it, in Katie's rounder hand, was a new line that said only Sunday. Baptised. And in the morning, at about the same hour as the courier's van had left on the Friday, the stove ticked as it cooled, and in the next room a baby woke, and was fed.
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This story was written by an AI and is fiction. Margaree Forks as a parish, the MacIsaac, Beaton and Aucoin families, Dr Raman, Father Aonghas MacNeil, the Inverness hospital's staff, Tally and everyone else are invented; the real Margaree valley and the Cabot Trail appear only as places. No real screening programme, laboratory or clinic is described, and the courier and timings are invented. The numbers are illustrations for a kitchen table. The 1-in-10,000 prevalence, the 99 per cent sensitivity and the 0.1 per cent false-positive rate are assumptions chosen to make the arithmetic readable, not the figures of any real programme. With them, 10,000 babies contain 0.99 true positives and about 10.0 false ones (0.001 of 9,999), so about 11 babies are flagged and 0.99 / (0.99 + 10.0), about 9 per cent, are true. Real programmes differ: real prevalence of MCAD deficiency varies by ancestry and is usually quoted somewhere between 1 in 10,000 and 1 in 20,000 in northern European populations, many programmes use a second-tier test on the same blood spot to cut false alarms, and a real family would be told by their own programme what its figures are. The point holds for any rare condition: a test that is very accurate in the people who have it can still be mostly wrong in the people it flags, and a positive screen means look closer, not a diagnosis. Advice in the story about feeding intervals and sick-day care is a simplified story version, not medical advice; a family told about a positive newborn screen should follow its own clinical team's instructions and seek emergency care for a drowsy, floppy or vomiting baby. The sacrament of baptism is not delayed or hurried by a laboratory result; the Church's teaching on its necessity and on the care of children who die unbaptised is a larger matter than a story can hold, and nothing here is Church teaching.