by Sami Kennedy
I first encountered Emily in a photograph. In the photo she is smiling in her bedroom, a large penguin sticker attached to her right cheek. The penguin holds up a thin tube. At the time, the penguin vaguely reminded me of the black stickers that athletes wear on their cheekbones to keep the sun out of their eyes. As a child, I had heard these called war paint, a presumable reference to the fortitude asked of the players throughout the game. Only later, when I had become well acquainted with Emily’s story, did I recognize just how appropriate my analogy had been.
In January 2010, Emily thought she had caught a particularly stubborn strain of the stomach flu. Her abdominal pain, frequent and urgent bathroom trips, and bloody stool fluctuated in severity but persisted for weeks. The high school freshman was young and active, and thought she had no reason to worry. She held her silence with more confidence, despite unrelenting discomfort, her sole focus a long-awaited school trip to Costa Rica. She would either get better or she wouldn’t. Either way, she decided, she would go to Costa Rica. In order not to jeopardize this plan, she hid her symptoms as best she could. In one photo from that trip, Emily poses outside a Costa Rican café. She bends backward in a moment of endearing spontaneity. In another, she grins as she wraps her arms around her younger host sisters. She looks happy and well.
When she returned home from Costa Rica, Emily’s condition had worsened considerably. At night, she began to wake up because of severe and sudden heartburn. “Like waking up screaming, thinking I was having a heart attack,” she clarifies.
Three years later, when I arrive on Emily’s doorstep in a charming neighborhood in western New Jersey, she doesn’t look much different than she did in those photos. At eighteen, her wavy brown hair still hits just below her shoulders. She leads me through the front door decorated with a bright, seasonal garland, past her white rabbit Theodore in the front parlor, and upstairs into her bedroom. Light pours through three large windows that look out onto the street blanketed with snow.
There is a cadence to how she tells the story of her illness, phrases that come back in every iteration. She knows it all too well from multiple tellings but still pauses frequently to concentrate on its sequencing. Her joints felt stiff and painful. She lost weight as her appetite gradually fell. She noticed light red streaks in her stool and suffered fevers as high as a hundred and three degrees. After she returned from Costa Rica, Emily could no longer keep her symptoms a secret. She and her parents sought help. Her stumped pediatrician referred her to the infectious disease team at The Children’s Hospital of Philadelphia. At fifteen, Emily found that her voice seemed to matter far less than those of the adults surrounding her. “Everyone was insisting that my symptoms hadn’t started before Costa Rica and that I had parasites.” She contended in vain that her symptoms had begun long before she’d approached her parents and doctor in March. Costa Rica had been her motivation to hide her symptoms, not their root cause. It took nearly a month for her voice to resonate loudly enough to have an effect—or for the medical team to reach the same conclusion independently.
Emily’s case was passed on to the gastroenterology team at Children’s in May. Over four months had passed since the onset of her symptoms. “I really didn’t think it was anything that bad,” she admits. “I don’t remember being scared.” Her gastroenterologist initiated several tests in mid-June, including upper and lower endoscopies. Both procedures use a camera and tiny pincer attached to a thin, flexible tube, allowing a doctor to see the digestive tract and take small tissue samples called biopsies for testing, the results of which typically take about a week to come in.
Emily’s doctor called to explain her biopsy results on June 29th. Sick and homebound, as had become the norm, Emily heard the news immediately. “My mom was crying,” she remembers, “and I just didn’t get it.”
The biopsies had tested positive for Crohn’s disease.
Crohn’s disease was named after the doctor who first described it in medical literature in 1932. A type of inflammatory bowel disease, it is part of a class of illnesses characterized by inflammation in the digestive tract. Crohn’s and ulcerative colitis are the most common of its recognized forms. Unlike in ulcerative colitis, which more selectively attacks the colon, Crohn’s inflammation can occur anywhere from mouth to rectum. A host of extraintestinal complications, including arthritis, liver disease, eye and skin disorders, and nutrient deficiencies, are also possible with either illness. The cause of inflammatory bowel disease is unknown, but as with other autoimmune diseases, an inappropriate immune response is commonly held responsible. Although often stigmatized as inconvenient “bathroom diseases,” inflammatory bowel diseases are in fact dangerous and pose serious health risks to their victims. Significantly, Crohn’s disease has no cure. It and other inflammatory bowel diseases are considered chronic, and currently available treatments focus on improving quality of life and driving the disease into a symptomless remission. The battle is long-fought for many patients; the Center for Disease Control estimates that around ten percent of those diagnosed with inflammatory bowel disease each year are younger than eighteen.
Crohn’s disease is rarely fatal, but the prognosis for any individual patient cannot be accurately predicted even in patients with a family history, as is the case with Emily. Two decades before Emily began showing the symptoms of her Crohn’s, her mother was diagnosed with ulcerative colitis. Inflammatory bowel disease can run in families, with an estimated incidence rate of ten to twenty-five percent. The hereditary links are often disease-specific: the child of a parent with ulcerative colitis is more likely to have ulcerative colitis than Crohn’s disease. A child and parent with different diagnoses, as with Emily and her mother, is more unique though not extraordinary.
Emily was prescribed a colonic anti-inflammatory, a series of antibiotics thought to help control Crohn’s symptoms (each with its own subsequent adverse reaction), and medicine to ease the nightly reflux causing her chest pain. “I was told to start taking meds at my appointment which was maybe a few days later and assumed it would just go away after that.” She laughs and grins a knowing smile. “Little did I know.”
Slowly, Emily recovered her weight and strength. After two months, doctors confirmed that she had hit her first remission, a period characterized by little to no active disease. The opposite of remission is known as a flare. The time between remission and flare can vary drastically among patients and even within a single patient. Emily’s disease had been flaring when she was diagnosed, but the medicine had brought it under control. In September 2010, she entered her sophomore year firmly in remission. In November, her disease was flaring once more.
Her family was presented with the treatment options of either powerful steroids or enteral nutrition, which would entail inserting a feeding tube through Emily’s nose to fulfill her caloric needs and allow her bowel to rest. The former would come with multiple short- and long-term side effects, including an increased likelihood of osteoporosis, glaucoma, and diabetes, among others. The latter would require a significant lifestyle change. In the end, Emily was uncomfortable with the long-term risks associated with steroid use, and she elected for the feeding tube. In December 2010, eleven months after the onset of her original symptoms, doctors inserted a nasogastric, or NG, tube into Emily, and combined it with a milder, less risky steroid. Even so, Emily’s weight and energy level stayed low. “The problem,” she explains, “was that when I actually needed to do the tube feedings was when I was very sick, but they made me so sick [presumably from the extra irritation associated with inserting the tube] that I couldn’t do them during that time.” Despite the precarious balance, she made do. “It was helping me because I wasn’t eating,” she admits. “I lived off Boost and whatever feeds I could do.”
The enteral nutrition helped minimize Emily’s weight loss, but her underlying disease continued to flare. Her doctors suggested Remicade, a different type of drug first approved for the treatment of Crohn’s by the FDA in 1998. Unlike her previous medications, Remicade would attack the disease at its source by regulating the production of a protein thought to be associated with the inflammation in Emily’s intestines. Many patients praise this monthly or bimonthly IV infusion as a miracle drug, but it comes with extensive risks. Frequent and intensified infections. Liver disease. Allergic reactions. A rare and especially deadly cancer. Before her treatment could begin, she needed to undergo extensive precautionary testing, including a liver biopsy.
The results were shockingly unfavorable. Emily tested positive for a rare combination of liver diseases: autoimmune hepatitis and primary sclerosing cholangitis overlap syndrome. Estimates on the prevalence of the overlap of these two diseases vary from study to study, ranging anywhere from only seven to around fifty-three percent. The new diagnosis indicated her liver and bile ducts, like her intestines, were inflamed. In time, her condition could progress to liver cirrhosis and failure. The long-term prognosis was frightening, especially in combination with her already aggressive Crohn’s. Although close to ninety percent of patients with primary sclerosing cholangitis also have inflammatory bowel diseases, most have ulcerative colitis. Autoimmune hepatitis is less often correlated with inflammatory bowel disease—up to sixteen percent of autoimmune hepatitis patients have both diseases—but still affects ulcerative colitis patients more commonly than those with Crohn’s. “For now, I guess it’s best to just move on with my life,” she would later write in her CaringBridge journal, a free blog hosting service for patients with chronic or life-threatening health conditions and their supporters. “Even in the most serious of cases, which mine isn’t at this point, it takes years to become a debilitating condition. And who knows? I could get lucky.”
Luck seems to have a lot to do with inflammatory bowel disease, an idea especially prevalent particularly in the language of patients with the illness. Some remissions last for decades, others for mere weeks. Crohn’s disease and ulcerative colitis, like other autoimmune diseases, are generally unpredictable and resist comparison between individuals. I, too, have inflammatory bowel disease. Like Emily, I was diagnosed in my first year of high school, but my immune system has been kept at bay by an immunosuppressant and other drugs for three years now. From my perspective, I have been lucky. If you ask Emily, she’ll tell you the luck has been hers. “It could be so much worse,” she insists when I challenge her.
The primary treatment for Emily’s liver diseases are either steroids or immunosuppressants. Either could be effective against both her Crohn’s and her liver conditions, but immunosuppressants take months to work and side-effect-heavy steroids are ill-recommended as a long-term treatment. Emily couldn’t wait, so her doctors took a gamble. The liver diseases progress slowly. If they could wrangle her Crohn’s into remission, treatment for her liver could come later. In February 2011, she began monthly Remicade infusions as initially planned, despite the risk to her sensitive liver.
To her surprise and delight, Emily felt relief from the Remicade within two days. Her pain decreased and, in a twist her doctors couldn’t explain, the condition of her liver actually improved temporarily. Still, she struggled with intense daily fatigue. She conservatively estimates she missed nearly fifty days of school. On March 10, 2011, she lamented in her CaringBridge journal, “The fatigue is really getting to me. I have so much to do to get caught up at school, and just with life in general, after it being on hold for so long. I’ve been trying to do work, but I’m so exhausted.” As her energy faded, so, too, did her social life, adding to the distress she felt on school days. Moreover, Emily continued to struggle with her NG tube. The physical difficulty of using the tube, coupled with the social stigma she faced when she was able to attend school and sporting events, encouraged the young self-advocate to take a brave step. She asked her doctors to trade her NG tube for a G tube.
A gastrostomy tube, or G tube, is a more permanent alternative to a NG tube for patients who, like Emily, need enteral nutrition. A thin tube is surgically inserted through the abdomen to provide nutrition directly into the stomach. The tube is held in place by a balloon that rests just inside the stomach’s lining. A small white cap about three inches away from Emily’s belly button allows access to the tube. G tubes are rarely offered to pediatric Crohn’s patients because they often remain on enteral nutrition only temporarily. Emily had been flaring nearly continuously for over a year. In her doctors’ eyes, it was a rare and questionable move. In Emily’s, it was the right one. “I didn’t want to be the sick kid forever,” she reflects. “That’s hard to get away from with a tube taped to your face.” To convince her doctors, she needed to summon the courage to challenge them.
Emily is modest, but she is no stranger to raising her voice. She’s been an Honored Hero for one of the Crohn’s and Colitis Foundation of America’s major fundraising efforts for three consecutive years. “I never thought I should accept the position because I know so many people who have much worse Crohn’s than me,” she confides, but she relented once she realized she could use her voice to advocate for and inspire other patients. “It doesn’t so much have to do with ‘I’m the sickest person with Crohn’s ever, so you should listen to my story and donate money,’” she explains. “That’s not what it’s about because that’s not true.” Despite her courage in public, she faced a learning curve in the hospital. Before Crohn’s, she trusted that doctors always knew best. These days, she considers herself an equally valuable member of her care team. Her once little voice has grown strong. The Crohn’s and Colitis Foundation of Canada’s Youth Advisory Council has an excellent blog dedicated to young IBD patients, called “The Gutsy Generation.” The title, more than just a pun on the nature of bowel diseases, speaks to a characteristic particularly valued in the young IBD community. Gutsy: showing courage, determination, and spirit.
Slowly, Emily began to have influence on her doctors. On March 16, 2011, Emily underwent laparoscopic surgery to place her new G tube. It allowed her to return to enteral nutrition and still attend school on a more regular basis, but not without sacrifices. “I was supposed to do the feeds all overnight,” she explains, “but I could never do that because the feeds themselves made me so sick.” Her body couldn’t tolerate a high volume influx of formula within a short time span. Emily says, “It would leave me dry heaving all night, so I would sleep in the bathtub every night when it was bad.” So once again, Emily had to resort to using her feeding tube during the school day. She insists she doesn’t mind now that her initial disappointment has worn off. “Most people don’t know,” she gushes, relieved at her newfound privacy. “It’s not like I’m embarrassed about it, but it’s not everybody’s business.”
For several weeks after her diagnosis, Emily did grapple with the question of whom to tell and when. I ask her if she’s ever regretted telling anyone. She denies regret but admits, “One of my friends told my Spanish class, which led to everyone finding out about it—and googling it, which is the worst possible thing you could ever do because you’re going to come up with all this weird stuff.” To resolve the gossip, Emily made a formal announcement to her school and encouraged supporters to join her at fundraising walks sponsored by the Crohn’s and Colitis Foundation of America that fundraise for a cure. These days, she shares her disease on a need-to-know basis. “My ideal would be everybody being okay with talking about it,” she reflects, “but just as a part of normal life, not sad or pitying.” As a girl who considers herself emotionally stronger because of her continuing ordeal, she struggles to accept pity. “Everyone has their stuff to deal with,” she says simply.
It’s the morning after my arrival at Emily’s house, and she’s preparing us waffles. She interrupts our conversation to ask if I’d prefer blueberries or blackberries in mine. The kitchen walls are painted bright yellow. We talk about “transition,” which is the common name for the transfer of a patient’s care from a pediatric to an adult practice. Several children’s hospitals in the country are just now beginning to implement formal transition programs to guide patients from pediatric to adult care. Previously, patients were often left to navigate the system solo, or with some guidance from their pediatric care provider. The Children’s Hospital of Philadelphia has a detailed program to pair patients with adult providers at a respected nearby hospital. Emily has not yet enrolled in the program (patients typically transition between the ages of seventeen to twenty-three, and the hospital will treat patients up to age twenty-six), but in many ways she already acts like an adult patient would, as an active member of her care team. She tries to take as much of the responsibility as she can when it comes to managing her disease. “If I really liked relying on my parents or if I did, then I probably wouldn’t know how to do it on my own,” she says. Still, she admits she’ll miss the comfort of Children’s. “I’ve seen a few adult doctors, and…” She pauses. “I don’t really like it. You go from being with babies to really old people. I think I would adjust to it, but Children’s is all colorful and everybody is so friendly.” I point out that she has many of the traits that lend a patient well to adult care. She understands her disease and treatment, she actively monitors her symptoms and expresses concern when necessary, and she is strongly opinionated. She credits her age at diagnosis for her maturity. “I never had to go through that whole stage of being a little kid and my parents taking care of my disease for me. I’ve always taken care of it.” It occurs to me that Emily may not be giving herself enough credit. Perhaps the transition between childhood and adulthood, much like the transition between pediatric and adult chronic illness care, doesn’t happen at a standardized time – nor is the timing always a matter of choice. Emily became an adult long before she turned eighteen.
Emily’s G tube operation was a success, but she soon faced another obstacle in her treatment. Remicade began to lose effectiveness, presumably because her body developed antibodies to the drug. Open to the idea of holistic therapies, she tried a special diet popular among inflammatory bowel disease patients. Emily discovered that eating certain foods seemed to intensify her abdominal pain, but, in general, the diet did not alleviate any of her other symptoms. Two new drugs also failed to provide relief. She had a severe allergic reaction to one. The other caused a less immediate reaction that left her extremely weak and unable to walk for days. Struggling to figure out what medicine to take next, she was faced with another tough decision. She had missed close to half of her junior year of high school as she fought to control her illness and, in early 2012, she announced to her class that she would be taking a leave of absence and then repeating her junior year. “It just reminds me of what I’m losing in all of this,” she wrote in her CaringBridge journal a month later. “I’m so sad about it, but I know that it’s the right decision.”
Emily pulls out her computer to show me a video clip of her sharing the story of her illness. Her desktop background is her most recent softball team photo. In it, she kneels on the turf in her uniform with her arms around her teammates’ shoulders. She shares a special bond with the team’s coach, who allowed her to play even when her illness forced her to miss crucial practices. She endeavors to remember the good amidst the bad, particularly in light of her time “Away.”
“Away” is Emily’s word for a twenty-week gap in which her health took another turn for the worse. “My whole life, I’ve had anxiety,” she explains. “I struggled with it, but it didn’t keep me from doing anything – just made it harder.” But it really “all started to fall down” with the physical and emotional isolation triggered by her leave of absence from high school. She struggled with depression, heightened anxiety, and suicidal thoughts. “But it was never something I thought I couldn’t stop,” she emphasizes. In mid-2012, though, a classmate committed suicide, and “that was when everything exploded.” She wasn’t personally close to the deceased student, but she began to fear her suicidal thoughts. “I think once that happened with him, it was like my brain was all—oh my god—if he could do it, then I could do it. I could really do this. And once it got started, it took over.”
There is a candidness in her voice that implies she doesn’t fear the stigmas associated with mental illness in the United States. This assumption is far from true. I ask her why she didn’t seek out therapy early on. “I didn’t want people to think about me like that. I didn’t want to be seen as being sad all the time. I don’t ever want people to think I’m not fine even if I’m not.” Emily is a person with dreams, hopes, and fears, something easy to forget in the conversation about mental illness. She didn’t want to be associated with the modern social stigma that often dehumanizes mental illness patients.
Emily was admitted by choice to a local psychiatric hospital in June of 2012. Most of the hospital’s patients stay an average of a week to stabilize and begin medications. Emily stayed eight. The program was heavily medication-based. Personalized therapy was not offered, only group sessions covering general concerns usually not directly applicable to Emily. She saw a doctor for little more than five minutes at a time and spent most of the rest of each day struggling to find a safe place. The young woman so accustomed to having a voice in her medical care felt stripped of her power. Although she was initially placed with other adolescents, she was soon moved to the adult ward due to renovations, despite only being seventeen at the time. One roommate had a granddaughter her age. Another made sexual advances. (“I felt bad because obviously it wasn’t her fault,” Emily says sympathetically. “She’d been living with sexual abuse her whole life.”) Most of the time, the threat of her own disease preoccupied her and prevented her from considering her surroundings, but she made several memorable bonds with other patients. “The people that were on the unit were people I probably would have crossed the street to avoid in my normal life,” she confesses, “but many became my friends when I was in there. I definitely felt like my situation was not as bad as what all these other people were going through.”
Despite her inherent optimism, Emily’s condition continued to worsen. By week eight, she was addicted to several of her prescribed medications, including Xanax and Klonopin, with little to no effect on her symptoms. Although her gastrointestinal symptoms had improved (which was surprising as stress is known to exacerbate Crohn’s flares), her appetite bottomed out. Desperate for relief, she turned to self-harm, particularly cutting. Her psychiatrist took notice and, for an uncomfortable stretch, placed her under one-on-one supervision, after which she took care to conceal her wounds. Although she regrets the self-harm, she wishes more people would understand the realities of mental illness. She draws an analogy with her physical disease. “With Crohn’s, I know there’s likely nothing I did to make myself sick. With mental illness, others will often blame patients for their actions as if we should be able to control what’s in our heads without help when we’re sick.” She speaks with conviction, well aware of the social stereotypes surrounding mental illness, particularly for young adults. “We can’t.”
Most discouragingly, after eight weeks in the hospital, Emily still felt bound by and terrified of her suicidal thoughts. “They were in my head all the time,” she emphasizes. “All the different scenarios and ways I could do it.” Emily realized that while she couldn’t leave, she couldn’t stay either. “I literally did not see the light of day for two months other than toward the very end when I got to go to the cafeteria for dinner—and even that was a ten second walk outside.” As she is quick to point out, “that’s not very good for anyone’s mental health.” She felt as if the system was giving up on her. “My psychiatrist told me a couple weeks before I left, ‘We just don’t have any more ideas left for you.’” Even in crisis, Emily knew she had to start advocating for herself again. She began looking into residential programs for adolescents with moderate to severe anxiety and related disorders. The particular program that interested her is nestled in the mountains of Vermont and hosts a maximum of twenty residents between the ages of thirteen and twenty, with a typical length of stay between thirty and ninety days. “I just knew the residential program was what I needed to do.”
Her insurance refused to facilitate her move. The company agreed to cover a year’s worth of inpatient monitoring and her prescribed medications, but would not cover the cost of residential therapy. “It’s ridiculous because it’s the same price,” she says, “and when you go to residential for two or three months, you’re a lot better and then you don’t need to be inpatient.” Her doctor, dismayed by her lack of progress, officially discharged Emily to the residential program in Vermont. As expected, her insurance company refused to cover any of the cost. For Emily, it was worth the price tag. “It was small and super active and outdoors.” She smiles. “I just loved it there. I wanted to stay.”
In August, she moved onto the program’s quaint campus, which resembles a quietly rugged outdoors summer camp, and stayed for three months. Within three days, her new psychiatrist had a diagnosis—one that felt right to Emily: Obsessive Compulsive Disorder. OCD is a hard illness to define, characterized by uncontrollable thoughts and actions. In the same way that irritable bowel syndrome is less severe than inflammatory bowel disease, even if it can seem equally troublesome, obsessive or quirky tendencies are not equivalent to true OCD. Emily’s suicidal thoughts were more than just obsessive, they were all-consuming. She now hopes to advocate for OCD and anxiety patients in the same way she has advocated for Crohn’s patients.
Even though she was transitioning to the treatment program of her choice, the summer was not easy. “It was obviously very different and scary because I’d been inside for two months and then thrown into being expected to be outside much of the day doing active stuff without breaks. I felt like I was in a strange world.” After the first couple days, though, Emily fell in love with the program and staff. Although trying at first, the results were immediate. “We did exposure therapy, which is doing the things that cause you the most anxiety repeatedly on purpose. It’s hard and horrible, but it’s very effective.” Moreover, the active program—a typical day could include equine therapy, yoga, and rock climbing, in addition to private tutoring to help residents stay on track with their education – and the connections she made there gave her a feeling of normalcy she had missed since taking her medical leave of absence from school. Although she still struggled with Crohn’s and needed to be driven off campus several times for appointments with a gastroenterologist at a nearby medical center, her symptoms had settled to the point where she could participate in the outdoor activities with her peers. She believes the friendships she made in the program helped her even more than therapy. “We could really joke about my OCD and people would kid about it, but in a good way. It was exposure therapy in the real world, and they felt comfortable doing it because they had similar issues.” For the first time in months, Emily felt at home.
In retrospect, Emily understands that the medications prescribed to her in the hospital failed to alleviate and may have even worsened her symptoms because she had been misdiagnosed. “My doctor there kept acknowledging that there was something obsessive about my thoughts,” she points out, “so he kind of knew, but didn’t take the time to look into it.” The Vermont program does not rely only on medication regimens for treatment. “They take more of a holistic approach,” she explains. “They care about treating the person, not diagnosis by diagnosis, but as a matter of figuring out the most important issues.” Just as she had known parasites weren’t the cause of her Crohn’s symptoms, she had felt strongly that depression was not her primary mental ailment. Once again, she had been right.
On November 11, 2012, after five months “Away,” Emily came home. The adjustment was bittersweet. “There were a lot of things that got better and then got worse again when I came home,” she confesses, “so I was initially upset about that because I felt like I worked too hard to go backwards.” Her diagnoses continue to pose challenges to her on a daily basis, but she has hope. She has found a local psychiatrist whom she loves. She has clear and attainable goals – to graduate from high school and work toward her dream of becoming a pediatric gastroenterologist. She and her medical team have high hopes that a new treatment will finally drive her Crohn’s disease into a long-lasting remission. She was recently granted a wish from the Make-A-Wish Foundation, a non-profit organization that grants wishes to seriously ill children and teenagers, a surprise given that she didn’t know she had been nominated. She has returned to high school and is playing softball again.
In Emily’s own words, the future is bright:
I’ve said this before but dealing with health problems is both a blessing and a curse. I’ve felt all the pain and isolation of being different and missing out on things. Like tonight being sick at home instead of out with my friends.
But then there are the times in life where I feel really good. And I truly get to enjoy that time that many people may take for granted. I certainly did when I felt healthy. And I know that I am such a lucky girl because I have the best and most loving support system anyone could ever ask for. Even when I am at my darkest most lonely moments I don’t forget that. I am ready to start being thankful for all of you. Most of all I’m ready to start living my life. I’m done waiting for the perfect time when I’m happy and I’m not sick, because that day may never come. But you know what? That doesn’t matter so much anymore. Because it’s the people in my life that make it worth living. So thank you all for giving me something to hold onto. Goodbye 2012 and good riddance. Here’s to a happy and healthy 2013 and to a year surrounded with just as much love.