They were dealt a ‘death sentence.’ Here’s how 2 sisters with cystic fibrosis have persevered – Press & Sun-Bulletin

Sisters Bethany and Emily Brown were both diagnosed with cystic fibrosis as children and overcame life-threatening conditions to make it to adulthood.

Kevin Stevens, Binghamton Press & Sun-Bulletin

Sandy Brown watches graduation candidates file in and settle down, respectfully tolerating bad-hair caps, too many photos and suspended texting privileges. It’s a Friday night in June at Downsville Central School, and Mom is doing just fine.

Then the slide show begins — photographs of these high school seniors as children — and up pops the submission from Sandy’s daughter.

There’s 3 ½-year-old Emily, inhaling medicine through a plastic mask as an inflatable vest delivers therapy through high-frequency vibration. By her side in the family’s East Branch home: Em’s 5-year-old sister Bethany, grinning for the camera after completing treatment of her own and handing over the vest.

Oh, my God, Sandy thinks.

We made it.

“And not just one, but there’s the two of them in that picture together,” Sandy says later. “It was that moment where we were just grateful to God that we made it!’”

Sharing a childhood and diagnosis

Sandy and her husband, Yancey, once wondered whether their two eldest daughters would live to see graduation day. See, cystic fibrosis doesn’t give a tinker’s damn about visions, dreams or prayers.

And yet, here they are, not just grads but standouts: Emily, class salutatorian, record-setting, champion runner and soon-to-be University at Albany scholarship athlete, celebrated her 18th birthday the day before commencement. Bethany, Presidential Scholarship recipient a year into accounting studies at SUNY Delhi, turned 19 in February.

The sisters share vibrancy and courage, feistiness, bluntness, inherited bullheadedness and toughness galore.

Too, they share a death sentence.

Graduation for both sisters is momentous, but bittersweet: As fate and genetics have cruelly conspired to determine, Emily and Bethany are essentially middle-aged women.

Cystic fibrosis, over time, limits the ability to breathe. Mucus in organs becomes thick and sticky. In the lungs, mucus clogs airways and traps germs, prompting infections, inflammation and respiratory failure. It builds in the pancreas and inhibits absorption of nutrients. Mucus can block the bile duct and cause liver disease.

The sisters are fighters. But CF is unbeatable. Those with the disease are assigned a life expectancy of roughly 37 years.

A ‘horrific’ discovery and navigating a dark path

Rallying in Emily and Bethany’s corner, though, are eminently invested parents who’ve dedicated their existences to improving their daughters’ quality of life. With their parents’ support — as well as a team of others — and their own determination, the Brown sisters have thrived.

The family’s journey has followed a twisting trail from devastation to celebration. They’ve negotiated the fear, pain and uncertainty that accompanies the disease and the huge amount of time, work and sacrifice it takes to fight it. 

Together, the family has guided the teens through innumerable medications, odometer-taxing physician appointments, incomprehensible dietary needs and the myriad additional challenges of an exhausting daily routine.

They’ve also battled CF’s progressive psychological strain, which can ache with an intensity that rivals the physical suffering. And they’ve tested the boundaries as the girls have emerged into young adulthood.

With two kids with cystic fibrosis in one family, Sandy says simply: “We’ve navigated a lot of water.” 

Years later, Sandy calls it “the dignity of diagnosis.”

It came on April 14, 2004, at Geisinger Medical Center in Danville, Pennsylvania, when the girls were 4½ and 3. Emily had experienced frightening symptoms and several emergency-room visits before being transported there. Bethany was brought to the hospital to visit her sister — only to be subsequently admitted and assigned a bed in the same room as Emily, suffering from the final stages of malnutrition.

► What is cystic fibrosis?: Learn more about the disease

As Sandy sees it, Emily’s diagnosis saved her sister’s life.

“They diagnosed me at 4½ and said I wouldn’t make it past my fifth birthday,” Bethany said. “I think I weighed 20-some pounds — and I was taller than the average kid.”

As if the initial 1-2 wasn’t sufficiently cruel?

Distal Intestinal Obstructive Syndrome, if not diagnosed and tended to, could have cut Emily’s life short at age 7. It took 28 procedures before doctors discovered the root of her severe belly bloating and knifing pain.

“They found that in her intestines, the fecal matter had solidified like cement. She would have died in probably the next two months,” Sandy said. “It was horrific, so disturbing.”

Bethany was diagnosed with DIOS within two years.

Emily’s hospitalization required insertion of a nasogastric tube through the nose, past the throat and into the stomach. This didn’t sit so well with rambunctious young Em, who twice removed the intrusive plastic with her hands, prompting her arms to be secured at her sides for reinsertion. As resourceful and spunky then as she is today, Emily yanked out the tube once more — using her toes.

Through it all, Emily has kept her sense of humor.

For instance, numerous sinus surgeries, Emily said, have required scraping of her brain cavities to head off the excruciating sensation of a jackhammer against her skull. Em finds a way to chuckle at the circumstances.

“They tape my nose back like a pig when they do surgery,” she said. “How could you operate on somebody who looks like a pig? I’m just saying. It’s too funny.”

Humor may be a critical coping mechanism for the Browns. Consider this peek into Emily and Bethany’s daily routine:

•  Swallowing 50 to 60 pills.
•  Taking in a minimum of 6,000 calories, at least double the daily norm.
•  Twice-a-day 20- to 30-minute sessions of high-frequency chest wall oscillation, or, “The Jiggle Machine,” an inflatable vest that vibrates the chest to loosen and thin mucus. Those are in concert with inhalation of medicines to open airways or to thin mucus.
•  Ingesting pancreatic enzyme supplements unfailingly with every meal and snack to improve absorption of nutrients.

Emily has coped with those six sinus surgeries, this restriction and that instruction, abnormal bathroom habits, brutal ear infections, too much travel for too many doctor’s appointments and unavoidable trepidation regarding her future. Bethany shares most, as well as significant bone problems that limit the activities of a former multi-sport athlete.

Why do they endure? It’s in their family DNA. The sisters are in a race against time, determined to outrun the statistics. They’ve been fighting all their lives, and they’re not about to stop now.

Run Em, run!

It’s May 2018, and Emily is getting ready to run.

If you want to see the dividends of the Brown sisters’ support network, here’s the place to do it. It has taken parents, family and physicians, coaches, teachers, administrators — and one exceptional school nurse — to make Emily’s and Bethany’s extraordinary feats possible.

In school, Bethany’s fancy was softball, and to this day Yancey talks up her prowess in the batter’s box and on the base paths. She also played basketball and ran track. Emily’s played basketball and soccer.

But track is her forte.

On that mid-May day in 2018, Emily ran in the highly competitive William Eddy Track & Field Invitational at Schenectady High. Chilly conditions worked in her favor, and she maximized with a second-place 58.29-second zip through the 400-meter race. It remains her fastest time.

She came back with a sporting 59.46 to top Section 4 and concluded her junior season by running 58.76 for eighth place among Division II runners in the state championship meet at Cicero-North Syracuse. She holds school records in four individual events and five relays.

All high-quality stuff by any standard. Higher yet given all she faces. Still, It nagged at her that she did not crack 61 seconds as a senior.

At the end of each race, her breath comes in short bursts. She struggles to breathe deeply.

Air in.

Air out.

It takes a long time. And for someone born with this disease, she has no point of reference.

“I don’t know personally how it feels to breathe like an average person — I don’t know what that’s like,” Emily said. “All I know is what I feel right now, day-to-day. Whenever I wake up in the morning I breathe and I’m like, OK, that’s what it feels like to breathe. But for you guys it could be different. I just don’t know.”

Luccia Martucci and Emily have shared space on tracks, soccer fields and basketball courts. Luccia has seen the good days and bad, the successes and the shortcomings, the challenges her friend sizes up and knocks down with regularity.

“She’s very disciplined, I don’t know how she does it,” she said. “Going from home to school to track to home, doing all her meds, her jiggle machine, getting everything done— then waking up for school the next morning and having to do it all over again.

“Then coming from meets, don’t get home until 9, 10 o’clock, and she still has to do all her homework, her meds and everything else. But she gets it done and come the next day, she’s full of energy. It’s the same Em.”

Rich Feeney, physical education and health teacher and track and field coach at Downsville, located about 70 miles east of Binghamton, has coached, observed — and admired.

“Emily’s never showed being afraid of what’s going to happen,” he said. “But she knows, she’s aware she may not have as long as other people but she’s going to do what she can do in that time and make the best of it and try to make a difference in the world because that’s what she’s starting to do already.”

Team Brown and the goal of ‘Cure Found’

Team Brown formed when Yancey and Sandy wed on May 7, 2004, just maybe with a toddler’s assist.

The two were on their second “date” — if bedside in a hospital room counts as such — when Sandy detected Emily fidgeting.

“Yancey had taken a phone call and Emily was playing with the cord, swinging it, tugging it, pulling it,” Sandy said. “She kept staring at him. I finally asked, ‘What are you doing?’ She said, ‘I’m fishing for my Daddy.’

“After he heard that, Yancey said, ‘How can I not ask you to marry me?’”

Yancey, Beth and Em’s adoptive Dad, met Sandy on a dating site after she’d divorced the girls’ biological father. The two rounded out the squad with daughter McKenzy (born Feb. 26, 2005) and son Riley (Nov. 13, 2006), neither of whom have CF. 

“Yeah, all end in ‘Y.’ Cute, huh?” Emily observed.

And get this, the inspiration behind the younger kids’ names. McKenzy was named in honor of Dr. Robert Kinsey, who finally diagnosed the girls, and Riley was named for a child the girls befriended during their Make-A-Wish trip to Disney World.

The elder sisters share a remarkably close bond, joined by a disease foreign to 99 percent of the world. They share, advise, commiserate and boost one another.

“Kind of like that twin-like connection,” Feeney said. “I know when Beth went to college it was hard for both of them because they’re used to being right next to one another, they did everything together.”

But they are teenage siblings, too, equally headstrong and steadfast.

“We fight a lot,” Bethany admits. “We used to get in the dumbest fights. We’re both very bullheaded and like to be right. And my Mom is bullheaded as well, so we get it from her.”

But the unyielding support they also get from Mom — and Dad — is just as defining.

“They would do anything for them. And it’s not just Emily and Beth, it’s Riley and McKenzy, too,” Feeney said. “They all do stuff and they make an effort to be at everything. They don’t make excuses for the kids, they hold them accountable and they tell them kind of how it is.”

Support is Yancey and Emily sitting in the family living room sorting hundreds upon hundreds of pills. Support is Yancey coming to Em’s aid after she was dealt a splash of disappointment.

She’d applied for a scholarship through the Boomer Esiason Foundation. The former NFL quarterback’s son, Gunnar, has cystic fibrosis. Emily was not granted the scholarship. Enter Dad. He nominated Emily for Team Boomer’s CF Workout Warrior of the Month, and indeed, she was chosen its March recipient.

In accordance with that distinction, Emily submitted her end of a Q&A that concluded with her hopes to one day contribute to raising funds and awareness to find a cure for CF “and to help make CF stand for ‘Cure Found!’”

And support has been Sandy’s lifelong oversight, guidance and insistence.

“Honestly, I don’t know if I could give you an explanation why I might be healthier than other CF kids other than that my mom, after we were diagnosed, was so diligent with our care,” Emily said. “She never let us skip anything. For me it’s like, ‘I have the disease, why are you stepping on me? Let me figure it out.’ But for my mom, it’s almost as if she were diagnosed with the disease.”

Meals, a shake and a large dose of medication

Emily the high school athlete — all of 130 pounds — caps off morning meds and a jiggle session with something like two or three eggs, three pancakes and four bacon slices. She crams a bevvy of snacks into her backpack before embarking on those 16 snaking miles of at times craggy road on a northeasterly path across State Highway 30 to school.

A primary go-to? Pretzels, which are about ideal.

“They’re salty, and CF kids need salt. It’s very important to have salt, especially when you’re in athletic training or any form of exercise,” she said, kindly explaining that her body produces protein, but the wrong kind of protein, so it does not properly process salt. “So, when I’m running? I’m sweating, you can see salt grains on my skin and that’s just because I don’t absorb it.”

Pretzels, Cheetos, Doritos — it is not uncommon for her to wolf a family-sized bag. Beef Jerky is another welcome staple. She’ll snack from pudding cups as well, and of course needs fruits and vegetables. However, those apple slices are best slathered in peanut butter or whatever to fall in line with her high-fat requirements.

Following three post-breakfast snacks comes lunch, perhaps three sandwiches with a plate of fries. More snacks through supper, then three more before lights-out.

She has to eat three or four times as much as the average person. Oh, and there’s something else.

“I have to take these enzyme pills — we call them pancreas in a bottle — and I have to take them every time before I eat,” she said. “Even if I pick up a candy bar or something, I have to take enzymes, otherwise I don’t absorb it, it just goes right through me.”

Ordinary supper portions might be two cups of lasagna or goulash, but for Emily, up that to five cups. A normal helping of green beans is more like five servings piled on her plate.

“Once she gets her enzymes in her, she can eat and eat and eat, eat you under the table,” Sandy said. “She eats what we would at a buffet and does it three times a day.”

Mom has long since developed tricks in the kitchen to help the girls meet those crazy caloric needs.

Over that sauce for lasagna she’ll generously sprinkle sugar, add butter to the girls’ noodles, assure their garlic bread and spaghetti come with healthy toppings of cheese. And the saltier, the better. Chicken with gravy and stuffing and all the fixings has been a regular feature.

There was a treat the Brown family came to know as filet mignon — a.k.a., cuts of steak ($4 per two-pack at Aldi’s) — which, when chopped and properly seasoned with fresh garlic, garlic salt and a bit of olive oil, made for one four-star after-school snack.

For perspective’s sake, Emily shared: “For me, I absorb one-fifth of what I eat. I eat five burgers, I absorb what you would eat if you ate one burger.”

Imagine the grocery bills.

Sandy works for the Callicoon Post Office and is a substitute teacher in Downsville. Yancey is an EMT, church pastor and drives bus in the Downsville school district. He operates a lawn-care business in summer months, its profits earmarked for feeding the family.

The ‘jiggle’ that’s helping keeping the sisters alive

Beginning and ending food-filled days are those 20- to 30-minute sessions in the vest, or as the sisters have dubbed it, “the jiggle machine.”

Rapid inflation and deflation of the vest creates pressure on the chest. The vibrations separate mucus from airway walls and move it up into the large airways. Typically, a person uses the vest for five minutes and then coughs or huff coughs to clear the mucus.

“An average person would not want to take 20-30 minutes out of their day to go sit on the couch and do something that is prescribed to them, they’re supposed to do it to keep themselves alive,” Emily said.

“It sounds really easy and really basic when I’m saying it keeps you alive, but when you’re keeping up with all the pills in a day, all the percussions, making sure you have your inhaler for track meets, making sure you have enzymes in your car and your purse, anytime you go to a friend’s house, at school, it really adds up over time.

“So, it sucks to have to wake up and jiggle but for me, when I wake up, what keeps me in a good mood is I sit there and say to myself, ‘Everybody is jiggling right now, everybody’s getting a massage.’ That just makes it feel normal to me.”

Martucci, fellow member of Downsville’s Class of ’19 and good buddy since the two were kindergartners, is a member of the exclusive Jiggle Club: She’s had a test ride or two in the device.

“It’s literally like somebody is hugging you and you’re just jiggling back and forth,” she said.

For those with CF, there are lasting repercussions for bypassing time in the vest.

“Sure, sometimes I take it for granted, sometimes I’ll skip because it’s hard to keep up with it,” Emily said. “Sometimes you just don’t want to do it on weekends. But every time I miss a percussion, it’s five days off my life. That’s the average.”

Adulthood brings new challenges for cystic fibrosis patients

The Brown family has spent years preparing Emily and Bethany for this moment in their lives: managing the complexities of cystic fibrosis as independent young women. 

What Emily will face when she heads to University at Albany next month, Bethany experienced in her first year at Delhi. Emily, like her sister, has secured off-campus housing, as dormitory living would not suit all that goes into fighting CF.

Beth is no morning person, but her days must began at 5 or 5:30. Time for a cup of tea, a bit of breakfast, treatments, then more food before classes. And like her sister, she must prepare food/snacks to bring along, to head off the shakes that accompany too little intake.

Thankfully, the 12,000 or so steps required to get around campus daily gets her breathing up. Bone and joint issues largely prohibit full-scale workouts, though yoga has proven something of a substitute. Issues with bone health are common for those with CF, as poor food digestion robs the body of needed vitamins and minerals. Vitamin D and calcium are vital to bone health.

Beth held a couple part-time jobs her first year in college, one in Delhi and another in Oneonta, more hours to be juggled with studies and treatments, appointments, fetching prescriptions, bill-paying and caring for her clingy Shih Tzu, Rose.

“The medicine, it sucks taking that much. It’s so hard, it’s like having a full-time job on top of college and having two part-time jobs,” she said. “I’m always going to doctor’s appointments, I have to wake up extra early to do medicine, I have to do medicine before I go to sleep. It takes up a lot of time doing my homework because I can’t write when I do my treatments because it makes my writing really shaky, almost illegible.”

Emily worries about what it will be like to be on her own. 

“It’s a hard thing to deal with mentally,” she said. “My mom is teaching me what I’m going to have to eat when I’m in college, refill all my prescriptions from the pharmacy, getting a pharmacy that’s close to my apartment, making sure that every three months I have to go to the CF Care facility and get a check-up.”

Bethany’s guidance in this transitional period has been invaluable — if on occasion difficult to hear.

“My sister has helped me so much, especially with college in general,” Emily said. “Honestly, I didn’t even know what the word ‘undergraduate’ meant until I asked her.

“She’s gotten the whole first-hand experience, from CF to being a teenager in an adult world. And my sister does not hold back the truth, you know what you’re going to get and I love that about her. Sometimes I want to say, ‘Beth, shut up!’ But at the same time, she has a year more experience than me, so … ”

Like any young adults, Bethany and Emily have tested the waters of independence.

Last summer, Bethany was free of Mom’s continuous instructions and reminders and, from a teen’s perspective, nagging. She was a grown-up now, no longer in need of supervision regarding meds, treatments and the like.

“I figured when I moved out I wouldn’t have to deal with her telling me to do that stuff all the time, I’d just do it,” she said. “But that was not the case.”

She stuck to her medical routine for three months or so, then stopped for several months.

“I noticed I was getting really sick, having a lot of problems,” Bethany said. “I was on antibiotics all the time. I’d have to call my doctor because I was having coughing fits and I didn’t know what I was supposed to do, so they’d have to prescribe me another antibiotic.

“By December or January, I was like, ‘This is not worth my time repeatedly getting myself sick.’”

Beth reached out to CF patients she’d met via social media and discovered she was hardly alone: It’s common for teenagers to hop off the adherence wagon. One patient’s story of lapsing into serious sickness smacked Bethany straight.

“It was kind of like a wake-up call to start doing my stuff and stop acting like it’s a joke,” Beth said.

“Kids rebel, it’s a common thing,” said Sandy, who is pleased to report Emily has been “on the straight-and-narrow” in recent months.

“She has an attitude just like mine,” Sandy said. “Her strong-willed personality, not taking no for an answer. Like with her treatment when she got older, ‘OK Mom! I know, Mom! How many times do you have to tell me, Mom?’”

The fear of living with something ‘inevitable’

But for all those typical teen-parent dynamics, the Brown family must also cope with the intense emotional burden that comes with not just one cystic fibrosis diagnosis, but two.

The certainty of its long-term truth hovers. Reminders come when they come, at times simply when head hits pillow.

That number, 37, weighs increasingly. And it means grappling with hypotheticals that no teenager should have to consider.

“I’m so stressed out that I have a disease where I could be getting a lung transplant in 5-6 years, or I could have a family at 32 and five to six years later pass away from a disease that … It’s something that’s inevitable,” Emily said. “It’s something that I have, I can’t get rid of it, there’s no cure.”

She wants to have children, but she worries about the medical choices she’d have to make, about the timing, about time running out.

“ … One of the big mental things for me is the depression of the illness. Some nights it’s like, I can’t wait to have kids. But at the same time, in order for a CF kid to get pregnant you have to go to your doctor, you have to get off certain pills so that the baby won’t have certain deformities and stuff like that.

“At 17 I shouldn’t have to be thinking about that,” she said. “I don’t want to have kids until I’m almost 30. But at the same time, I have to think, if 37 is the average life expectancy … And sometimes when you’re 18 your condition can go down dramatically, your condition can just change.”

Bethany’s greatest fear? Or source of anger? “Dying early. Not getting a full life.”

She wisely, if begrudgingly, sought every-other-week visits to a psychologist.

“I have been severely depressed since I was 12 and I chose never to tell anybody because I like to be seen as strong and indestructible,” Beth said.  “There is a lot of stuff we have to deal with. I never thought that we would get depressed because we’ve always been so strong with it, we’ve never had as severe cystic fibrosis as other CF patients, our doctors have always been surprised because we come out with some of the best results they’ve ever seen.

“So when I realized I was getting depressed I didn’t want to believe it because I was always really strong on the outside and people saw me as somebody who was really strong — ‘There’s no way she could be depressed, she’s too strong and powerful and carries herself at such a high manner.’ ”

How mom keeps everything together

When her girls got sick, Sandy Brown left a well-paying position as secretary to the warden at the Federal Correctional Institute in Ashland, Kentucky, and relocated to Houtzdale, Pennsylvania, the Clearfield County borough where she was raised. Her daughters’ needs became priorities 1 through 10.

After the diagnosis, she made a vow: Every pill and treatment, appointment, meal, snack and whatever other provision would be overseen. Bethany and Emily were going to do this.

There’s a maxim Sandy abides by and shares with anyone who could use a boost: If you can conceive it and believe it, you can achieve it.

The disease has stretched the limits of her will. Nevertheless, it is her nature to pry open the jaws of negativity and not only find positive but apply it to the next trying situation. Never mind thorns on that rose bush, Sandy would prefer to savor the soft, colorful petals and soothing fragrance of the flower up top.

Along the way, she’s found strength in her faith. When Emily was 7 and suffering, she remembers holding on to a sense of hope.

“There was a hope in God to say, He’s got this, He didn’t bring us this far to bring us down now,” she said. “So, I sat and I cried, I prayed with my head on my husband’s shoulder, and then I regrouped and went on.”

Sandy experienced a spiritual, almost magical sense of awareness and gratitude around the time Bethany went off to school.

“I remember standing here looking out the window at the trees rushing around in the wind, and I went, ‘Oh my God, we made it.’ It was like complete exhilaration,” she said. “Because you’re handed a death sentence when they were diagnosed, ‘Sorry, your kids have this terrible illness.’

When the girls were 5 and 6 years old, the family was gifted a backyard playground and a Florida getaway from Make-A-Wish Foundation, which exists to put smiles on the faces of children with critical illnesses.

“Now fast-forward 12 years, and I stand here and look out there and I go, ‘Oh my God, it’s like a wish come true.’  It’s just total thanks, to God, to the advances made in cystic fibrosis, doctors, three medical centers, people at the top of their profession gave us every answer we ever needed from every angle.”

In the whirlwind of graduation night, Sandy finds herself reflecting on years of transporting her girls to this doctor and that in Danville or Syracuse or Binghamton, to stirring meds into applesauce that little girls would more readily ingest, to that Make-A-Wish excursion.

“They were 6 and 5 — you know what I’m saying?” she says. “I got to thinking, that’s not good when you’re granted a Make-A-Wish trip that suddenly.”

And now: one daughter in college, another poised to enter the world of young adulthood. It’s a momentous transition for any family; for the Browns, it’s more.

“It’s a lifetime wish you’ve waited for, you’re going to see your kids walk across the stage, grab their diploma and there they go,” Sandy says. “They conceived it, I conceived it, believed it and now they’re achieving it. It’s just awesome.”

When Sandy looks at that slideshow photo, she sees her pint-sized daughters all those years ago, connected then by the bonds of sisterhood and the disease they share. What’s invisible — but there just the same — are the threads of the community that loves them, of family and friends and medical professionals and school staff that have supported them through the years.

“I look at the nurse and the principal— it was just gratitude to all of them because it really took a community to do this, to keep them in the best health possible. I just felt so much gratitude to the staff, too— thank you for helping us get to this day,” Sandy says.

Take school nurse Sherry Becker, a staunch advocate for the girls who put off her retirement until Emily got through high school. On this night, Sandy recalls, the two embrace and have a cry. Then Sherry turns to Emily.  

“We did it, kid,” she says. “We made it.’”

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