The Human Genre Project

The Maple Syrup Urine Disease

The week after the baby’s funeral, Carla’s mother made her breakfast every morning, but after the fourth day, told her that she wasn’t going to bring it to her in bed anymore.

“You have to keep living your life, and that means eating like a normal person at the table.”

Carla didn’t want to argue, so each day at 7:30am, she arrived downstairs wearing a spare robe of her mother’s, her bare feet slapping against cold linoleum tiles.

“Put some slippers on, will you?” her mother called from the kitchen, raising her voice above the dull hum of the electric mixer. “You’ll catch your death of pneumonia if you keep walking around like that.”

Carla’s slippers, along with her socks, her own robe, and all her clothes, were back at her apartment. The only things to wear that were currently with her were a black dress, black stockings, and black shoes, along with matching black underwear.

Trust me, it’s important,” her mother had said when helping her pick them out. “Maybe no one’s seeing them, but you’ll feel better knowing your panties and bra go together.”

After the burial, Carla had too much to drink at the reception, and after finishing an entire bottle of wine by herself, she’d fallen asleep in a chair in a back corner of the church hall. No one had noticed she was missing until the end, when her Uncle Daniel realized her car was still parked out front. They took her back to her mother’s house, and Doctor Schutt, who’d come to the funeral and who she’d taken the baby to after it was too late, told them not to worry, it was just too much alcohol, to let her sleep it off and she’d be fine. She’d been there ever since, wearing whatever spare underwear her mother could find for her.

It had been her mother who told her not to worry in the first place, that the baby didn’t need to see a doctor.

“He’s just being fussy,” she said, when Carla would bring it up, before changing the conversation and talking about Cousin Annette’s upcoming wedding. “You were fussy too.” Over the phone line, Carla could hear the sound of pages turning as her mother thumbed through bridal magazines. “Did Annette tell you she’s thinking of going with chartreuse? That’s going to look horrible on you if you don’t get some sun soon.”

A few weeks later, when Carla called her from the hospital, telling her the baby was sick, that it was Maple Syrup Urine Disease, and that it was bad, her mother laughed.

“I’m sorry, I’m sorry,” she said. “It’s not funny, I know. But come on, it just sounds a little funny. Admit it. Where’d your sense of humor go?” Carla just clutched the black phone at the nurse’s station so tightly that her knuckles turned white. A doctor was being paged by the intercom that blared overhead, and she pressed the receiver into her ear harder. The plastic frame of her glasses dug into the side of her face; in her rush, she hadn’t had time to put in her contacts. When her mother arrived in the NICU a few hours later, she sniffed and commented that she couldn’t believe Carla left the house looking like that.

It was the same sniff her mother had given her when Carla first told her that she was pregnant, even though she and Bill had split up weeks before.

“Well, you’re not going to keep it are you?” her mother had said. “I mean, I know the church goes on and on and on about things, but I think God would make an exception for you this once.”

When the baby died, after Doctor Schutt had put a hand on Carla’s shoulder and squeezed it gently while using words that she didn’t quite understand like amino acids, genetic mutations, autosomes, and encephalopathy, before finally saying how sorry he was, her mother sniffed again.

“Honey, you know maybe this isn’t the worst thing that could have happened. Sometimes the universe just has ways of righting its wrongs if we make a bad decision.”

It was lucky that they were in a hospital. When Carla slapped her, her palm came into contact with her mother’s nose. Her mother screamed and covered the lower half of her face, but blood dripped from between the cracks in her fingers, landing on her new, silk blouse. The nurses were there instantly to apply ice-packs and make sure nothing was broken, but she still had the dark bruises under her eyes a few days later at the funeral.

Now, Carla hadn’t left her mother’s house since, sleeping in her old childhood bed, spending her days in front of the television watching talk shows and soap operas. Her mother, who since the hospital hadn’t talked about the baby, had taken to making elaborate meals to start the day. Things like omelets, French toast, bacon, sausage, and Eggs Benedict. The type of foods that she never would previously have made for Carla as a teenager, saying it would just go to her hips.

“Eat up,” she said to Carla on the fifth day after the funeral, setting down a plate of hot food in front of her. “I want to take you shopping today for some clothes.”

Carla stared down at the pancakes, and ignoring the condiments that were sitting in the middle of the table, picked up her fork and began to eat.

“Oh, honey, don’t you want to put something on those?” her mother said, reaching for the imitation butter spread. Carla just shook her head, but her mother pushed the glass bottle and plastic tub towards her. “They’ll be all dry if you don’t. And come on now, you know there’s nothing worse than dry pancakes.”

Note: Maple Syrup Urine Disease (MSUD) affects infants and is attributed to a deficiency in the branched-chain alpha-keto acid dehydrogenase complex, located on Chromosome 19. While prognosis is good when caught early, if left untreated, it can lead to brain damage and death. It is so named because the ailing infant’s urine, quite literally, has a sweet smell, reminiscent of maple syrup.

Genevieve Schrier