Saving Henry (3 page)

The name, it turns out, is Fanconi anemia.

“
F
anconi anemia.”

“I'm sorry?” I heard Allen say into the phone two weeks later. We were lying in bed together late on a Friday afternoon in early November, catching a quick respite between feedings, diaper changes, laundry, and doctor' appointments. Henry was peacefully sleeping in the bassinet next to our bed. “Can you please repeat that?”

I didn't know this at the time, having never heard of Fanconi anemia before, but those two little words were about to wipe out all the dreams I'd ever held for my family in a matter of seconds. Later, when I thought about that moment, it's not the hearing of the
words that I remember as much as the moment right before it. The moment when it was only his thumb and heart. Those problems, I'd learn, were easy to fix. Like many new moms, I had a stack of books on my night table that promised to help me navigate my way through parenthood, but none of them prepared me for Fanconi anemia.

Henry tries to swallow me whole
The Strongin Goldberg Family

G
rowing up outside of Washington, DC, the mantra in my home was “There's always room for one more.” Our house was a meeting place where neighborhood kids, family, and friends of all ages gathered for food, fun, and conversation. Our pantry was stocked with cookie-making ingredients, and our garage with balls, skis, bikes, stilts, and other suburban accoutrements. Our days were filled with public school, visits to parks and museums, and hikes on
the C & O Canal. Our nights initially featured Red Rover and Kick the Can and, eventually, late-night excursions to the Tastee Diner in Bethesda, Maryland, in my 1947 Willy's Jeep or my friends' Duster or Pinto. The rules in my family were simple: Be honest, treat others the way you wanted to be treated, give back to the community, work hard, be prepared, and enjoy. When, from time to time, things didn't go our way and Abby, Andrew, or I would exclaim, “It's not fair,” my dad, Sy Strongin, a labor arbitrator by trade, would answer, “Life's not fair.” I listened, but the truth is, I didn't believe him.

I'm not sure whether it was out of fear of being average or just my nature, but as far back as I can remember, I had always hovered around the extremes, at least for a suburban good girl. I would run ten miles in a stretch and then eat a pound of M&M's. One summer I won honor camper and after another, I was asked not to return to sleep-away camp due to a series of episodes involving capsized boats and bras up the flagpole. I was captain of the Bethesda–Chevy Chase High School field hockey team and on the homecoming court. I held the esteemed position of being one of two women to participate in the University of Michigan's first-ever Nude Mile in 1986 but took comfort in the predictability of living an adult life that mirrored the one I had as a child: a life based around family, trust, and love.

For Allen and me, it wasn't love at first sight. The first time we met, Allen Goldberg and I were each out on a date with someone else. But by the end of the night as we drove out of the Tower Records parking lot in downtown Washington, listening to the words of U2's
Rattle and Hum
as loud as Allen's VW Golf stereo would allow, a friendship was born.

It was 1988. Allen was twenty-six, Jewish, good-looking in an unintentional way, smart, employed, and fun. He was tall, redheaded, and had a pair of dimples I could sink my fingers into. Having grown up in the Washington area and never straying too far from
home, Allen knew all its secrets, like the intimate and romantic wine cellar at the now-closed Dolce Finale in Woodley Park that sat about four people, and Joe's Record Paradise in Rockville, which stocked bootlegged albums and featured little-known local artists. Allen loved music, and his record collection rivaled mine.

He's never been a paint-the-face kind of fan, but like many Washingtonians, Allen rooted for the Redskins. For him, fall and winter Sundays were game-focused, and the general mood was determined by wins or losses. He threw parties that strained the square footage of his Northwest DC apartment, as well as his neighbors' nerves. Allen's ability to enjoy everything was contagious. Even McDonald's recognized this, featuring him in a national advertising campaign in which he exclaimed, “Excellent!” in response to a question about what he'd say if they told him their burgers were only seventy-nine cents. “Excellent” was what Allen would say about most things, and that made him the kind of guy I wanted to be around.

At the time, Allen worked at a DC-based national trade association, and I was a communications consultant. Allen hired me to plan a tribute dinner for his mentor and retiring boss, George W. Koch, CEO of the Grocery Manufacturers of America, whose daughter was afflicted with colitis. The event was a fund-raiser for what is now the Crohn's and Colitis Foundation of America. I'm still not sure if Allen hired me based on my credentials, my girl-next-door appeal, or some combination of the two, but the event was a tremendous personal and professional success for us. It also set the standard for what would become a lifetime partnership built on loyalty, the importance of family, good deeds, and good parties.

There was something comfortable and familiar about Allen. Maybe it was the fact that we grew up five miles from each other in the Chevy Chase and Potomac, Maryland, suburbs of Washington. We were both children of professionals and grandchildren of immigrants, coming of age insulated by the suburbs. We were reluctant
dog walkers; got shuttled to and fro in station wagons; camped in West Virginia; and spent summers at the Delaware shore. Growing up, we were both extremely fortunate to have dodged the trend and trauma of parental divorce, the worries of financial instability, and the horror of significant health problems.

I was twenty-three, and Allen was my best friend. For four years, we shared beach-house rentals; went to rock concerts at the Bayou, Grog and Tankard, Wolf Trap, and other venues; took road trips to New York City and New Orleans; danced to reggae at Kilimanjaro in Washington, rock and funk at the Bottle & Cork in Dewey Beach, Delaware, and zydeco at the New Orleans Jazz Fest. We dated other people, but every six months or so, Allen would point out the obvious: We were made for each other. But I couldn't see why we would let romance ruin what we had.

One morning in February 1992, I woke up with a start and realized that Allen was right. I called him and asked him on a date that transformed us from friends into everything he's since become to me. He was as good a boyfriend as he was a friend. He listened to what I had to say. He paid attention to my interests. He took care of me while respecting and admiring my independence. He made me feel special.

Four years after we met, I beat Allen at Battleship. It was pouring and cold, and we were at my parents' house on the Chesapeake Bay outside Annapolis, Maryland. I played the trick where you cluster the ships and make it nearly impossible for your opponent to figure out where one ship ends and the other begins. Allen protested that after Pearl Harbor no one does that in real life, but I did. After the game, Allen suggested we walk down to the water. Despite my insistence that warm soup and a hot fire held greater appeal, I agreed.

We went for a walk and came home engaged.

 

A
s I was preparing to get married in the spring of 1993, my friends were busy preparing a proper bachelorette party for me. In high school, my friends Erica Antonelli, Laura Subrin, and Becca Knox and I had started our own little clique, which we called BOA (Bitches of America). We had business cards printed and thought it was all very cool. Of course, “cool” for us meant that we were good students who engaged in innocent fun, like streaking naked along the Beltway on the afternoon of our SATs. For our sixteenth birthdays, we had sweatshirts made up: black ones with BOA printed in white capital letters on the front. We'd wear them to meet up with our boyfriends at the all-night Tastee Diner. We had a blast.

After college, most of us returned to Washington. Erica, Becca, and I—as well as other women we'd met in college and through friends—began to meet once a month for dinner at the Pines of Rome restaurant in downtown Bethesda, not far from where our teenage antics had occurred, and where one of us had had her sweet-sixteen party. It was a classic Italian place with red-and-white tablecloths and candles stuck in large wine bottles on the tables. We'd drink house red out of small juice glasses and begin every meal with a large white pizza for the table. We called ourselves the Ladies of the Pines.

It was here that my friends met to organize my bachelorette party, and a few weeks before my wedding day, the Ladies of the Pines arrived at my parents' house to pick me up. On the hood of the minivan they rented was a huge banner:
DESTINATION ANYWHERE
. We blasted Bruce Springsteen, Elvis Costello, Billy Bragg, and U2 and drove to Atlantic City, where we spent most of the night laughing, reminiscing, and dancing in our hotel room.

 

O
n May 15, 1993, I married my best friend. It was a perfect day, unseasonably warm and sunny at Washington Harbour on the
Potomac River. When we broke the ceremonial glass and kissed as husband and wife, the 230 guests, as well as the hundreds of other diners and boaters, all cheered.

We never talked about whether we would have kids, because it was a given that we would. The only question was when. Like many Ashkenazi Jews, we underwent genetic testing to determine whether we were carriers of Tay Sachs, a fatal genetic disease in the Jewish population, and the only one for which testing was available at the time. We both tested negative. Since neither of us had any history of genetic disease in our families, we were unconcerned about passing along anything deadly to our children. We just wondered if they would be redheads with green eyes, and tall and thin like Allen; or brown-haired with brown eyes, and short and olive-skinned like me. We were pretty clear on one fact: They'd probably be good dancers.

“I'm sorry, can you spell it?” Allen was now saying as I lay beside him, reading the notes he was jotting on a scrap of paper.
Fanconi anemia. Rare. Fatal? Henry
.

The first things I learned about Fanconi anemia were that it causes a bunch of birth defects and it has the impossible-to-accept label “fatal.” Over time, I came to know the truth about Fanconi anemia. It's shit and piss and blood and agonizing pain. It's twenty-one or twenty-four medications a day, anesthesia, surgery, infection, malnutrition. It's hoping your child will be lucky enough to make it to the first day of kindergarten. It's interminable days and sleepless nights without a break; fear; loneliness; desperation. It's the possibility of death, each day. It's longing for an escape, and the awful, horrible realization that the only escape is death. It's knowing that death would be far, far worse. It's thoughts of throwing dirt on your boy's coffin. It's not knowing how you are going to live without him. It's having to live without him.

I'm glad I didn't know all that at the time. It was way too much for a new mom to comprehend.

 

A
llen hung up the phone, and I leaned over and scooped Henry out of the bassinet and into my arms. There are three bedrooms upstairs in our rowhouse, and Henry's room was in the back. It had a wall of windows that filled the room with sunlight. Everything was a pretty, comforting blue: his crib and dresser, and the walls, to which we had affixed a sweet wallpaper border of a beach scene. For the first few weeks after his birth, though, he slept near us. I held Henry tight to my chest to shield him from those menacing words that threatened to steal our happiness. Allen, meanwhile, went to the computer and typed the foreign name into an Internet search engine.

Named for the Swiss physician who first identified it in 1927, Fanconi anemia (FA) is an inherited anemia, passed along from parents to their children. It is a recessive disorder, meaning that both parents have to carry and then pass along a defect, or mutation, in the same FA gene in order to have a child afflicted with FA. In other words, Allen and I had unknowingly given this disease to Henry at the same time we had given him brown hair, brown eyes, and the absolute cutest dimples I had ever seen. Researchers estimate that one out of every three hundred people is an FA carrier; but among Ashkenazi Jews like Allen and me, the carrier frequency is approximately one out of every ninety. By the year Henry was born, approximately one thousand people had been diagnosed with FA worldwide, and Allen and I didn't know a single one of them.

Some of the websites we visited used the word “fatal” to describe FA. Others used “often fatal,” and some even “usually fatal.” I would have given everything I had for a “rarely fatal” or “seldom fatal.” But no matter how hard or how often we searched, those words never appeared.

The first few weeks at home, I tried to figure out how to breast-
feed and change Henry's diaper, and get his little arms into his undershirt, while recovering from my C-section. I struggled to absorb all of the news we were being forced to understand. He might turn blue any moment. He would definitely need open-heart surgery in the next few months.

He could die before kindergarten?

My mom called a friend and got the name of a pediatric hematologist, whom Allen and I called that very night. In addition to giving us the names of experts, the hematologist planted a critical seed of hope. Although Fanconi anemia was still considered a fatal genetic disease, she explained that the dismal death rates reported were out-of-date, as science was advancing and with it, the chance that Henry could survive.

The day after Henry was diagnosed with FA, we visited Dr. Rosenbaum at Children's National Medical Center, who opened his office on the weekend to explain everything he knew about FA and to give us the names and phone numbers of the very best specialists. He also gave us a quick genetics course, explaining that Allen and I were each carriers because at least one of each of our parents was a carrier and had passed it along to us at birth. Through blood tests we soon learned that in our case, each of our mothers was a carrier. Being a carrier isn't a concern as long as your spouse isn't a carrier. Since neither my father nor Allen's was a carrier, our parents would never have a child with Fanconi anemia. However, when one carrier (Allen) marries another (me), with each pregnancy, we had a 25 percent chance of having a baby with the disease; a 25 percent chance of having a baby who is healthy and not a carrier; and a 50 percent chance that the baby would be healthy, but a carrier like us. In other words, Allen and I were unknowingly playing Russian roulette with our children's lives.

There are at least thirteen FA genes or complementation groups. FA-A, FA-C (Henry's type), and FA-G account for nearly 85 percent
of all cases. The disease occurs equally in males and females, and is found in all ethnic groups, although the percentage of Jews who have FA is higher than the percentage of Jews in the general population, making it a disproportionately Jewish disease. The median age for the onset of bone-marrow failure is seven, and the average life expectancy is twenty-two years, but the actual life span of any one individual can be quite different from the average. Kids like Henry with FA-C tend to have the most severe birth defects and earlier onset of bone-marrow failure. They also have the poorest bone-marrow-transplant survival rates—or the highest death rate—following a transplant.