A Decade of Devotion
By Scott Borinstein, MD, PhD, Director, Pediatric Sarcoma Program and Adolescent and Young Adult Oncology Program
I can halt any conversation at a party by answering a single question, “What do you do?” and if I reply honestly that I am a pediatric oncologist, the awkward pause follows. “Oh, that must be hard,” or “How can you do that?” are common replies. My response is always the same: “I love my job! I get paid to take care of the best patients in the world.”
Among them is Kayla, who was 20 years old when we met. She was in college, thriving, when she developed hip pain. An X-ray revealed an abnormality in her pelvis, resulting in an MRI, followed by a biopsy. She was diagnosed with Ewing sarcoma, a highly malignant and aggressive tumor of bone that, unfortunately, had spread to her spine as well.
I was a junior attending oncologist at the time, just a year out of training, and was possibly more nervous than she was when we sat down to discuss the treatment of her cancer. However, Kayla put me at ease with her infectious smile and witty sarcasm interspersed with frequent bouts of laughter. We talked as partners together, working toward a common goal, instead of doctor and patient. The treatment plan was outlined: a brutal marathon combination of surgery, radiation and chemotherapy. School would have to be put on hold requiring full commitment to treatment. Kayla jumped in with both feet.
Treatment was hard. She needed to be hospitalized, transfused and monitored. Emotionally, it was difficult — away from her friends and family. Instead of doing the things college students should be doing, she was hooked up to an IV pole in a cancer clinic. However, she endured, always with a smile on her face and a snarky comment ready for me. Her treatment went well, and she was in remission.
I saw her for scheduled clinic visits to monitor for recurrence, and the banter continued, chock-full of grins and chuckles. She got involved in cancer awareness and spoke at events inspiring many with her courage and determination. Soon she graduated college and matriculated into dental school. We were all so proud of her. She met Nathan and brought him to her clinic visits for moral support. He is a kind, thoughtful young man, and I was so happy that they had found each other. Life was going so well for them and I couldn’t have been happier.
Then Kayla found a “bump.” I wanted to dismiss it, tell her it was nothing to worry about, but the bump got bigger and despite being nearly five years off therapy, Kayla indeed had a recurrence of Ewing sarcoma. I didn’t want to believe it; I was truly heartbroken, dreading the conversation that we soon would be having. I shared the news, and as before, we developed another plan of attack. She withdrew from dental school so she could remain in Nashville for treatment. More surgery and more chemotherapy ensued. Kayla tolerated therapy extremely well, and again remission was achieved. Her professional life pivoted and she changed careers. Not at all surprising to us, she has thrived in her new professional path, charming clients with her 1,000-watt glowing personality.
Recently, Kayla visited me in clinic. It was a hectic clinic day, with anxious patients waiting. After going through the routine clinic details, she showed me a book she had brought — her wedding album. I sat down and savored every picture, every photo of two amazing people celebrating their union together. Kayla has been my patient for nearly a decade, and I have watched her grow up into a mature and talented woman, married to an incredible guy, living her life despite unbelievable challenges. That moment, flipping through the pages of glossy pictures, is one that I will never forget. This is why I come to work every day, why I love my job, and why I am so proud to be an oncologist.
The Care and Keeping of a Colleague
By Mohana Karlekar, MD, medical director of Palliative Care Medicine
I don’t exactly remember when I met Art Wheeler, MD, but I remember almost every detail of the day he told me he had pancreatic cancer.
Art was, and will always be, one of the most intelligent people I will ever know: incredibly compassionate, well respected and an all-around impeccable clinician. He was a professor of Medicine and the longtime director of Vanderbilt University Medical Center’s Adult Medical Intensive Care Unit (MICU). Our professional paths crossed often.
So, when he asked me to be his palliative care physician, I was both honored and stunned. I was honored because he trusted me, but truthfully both shocked and terrified that he asked me. All I could think was, “What if I get this wrong?” I truly did not understand why someone so intelligent, capable and clear about what he wanted in terms of his health care and end-of-life wishes would need a palliative care physician on his team.
After Art met with his oncologist, I met with him and his wife, Lisa, to talk through their thoughts about treatment. We discussed all the options, and he ultimately made his choice to forego cancer-directed treatment with the full support of Lisa. He and I talked about what was important, knowing he had an incurable disease that would likely result in a life expectancy of months. He was clear about what he wanted at the end of life. Not surprisingly, it was exactly what I think most people want: the best quality of life possible, time with the people you love and the ability to die in your home, in your bed.
Although I knew Art well before he received his cancer diagnosis, our relationship grew much closer after I became his palliative care physician. It became a part of my normal routine to check on him weekly in some manner by text, call or visit. Sometimes we would talk about the medical stuff, like how on earth I was going to be able to manage his symptoms when he confessed to me that he would not swallow pills. Other times we talked about the joys in his life, his worries about the future and what it was like to be a “dead man walking” — his words, not mine. When he was unable to come to work due to progression of his disease, I visited with him in his house and helped care for him there along with his MICU family. All of us who cared for Art, I think, formed this incredible bond, as we saw each other often daily, spending hours together. It was intimate, intense and exhausting, but gratifying. It felt right to give back to someone who had given so much of himself to others his entire professional life.
Why did Art make such an indelible mark on me? So many reasons. He reminded me that the smartest, most compassionate and capable of physicians are human and vulnerable like the rest of us. That dying is scary and the loss of control in what physically happens to us is unsettling. He taught me that if we are intentional about what we want, we can successfully shape how the end of our lives looks like. He showed all of us how to truly live life while he was nearing the end of his.
Editor’s note: Art Wheeler, MD, died Dec. 17, 2015. He was 58.
By Jay Wellons, MD, MSPH, chief, Pediatric Neurological Surgery
“Here you go, smart guy. Take this patient,” said the resident, as he handed me an index card and walked hurriedly on down the hall, his white coat flaring out.
Over his shoulder, “Remember, he’s your patient…to talk to, to examine…yours.”
“Got it!” I said to no one in particular while standing up on my tiptoes as the resident sped around the corner, hand flashing to his pager as he disappeared.
On my first day of medicine at the VA as a third-year medical student, I had just enough training over the prior year to know that I could really hurt people if I didn’t do my job. We were students, but there was no doubt that we had real responsibility with our patients. Residents and interns circled us like sheepdogs, keeping us in check. It was our job to take a history, examine our patients, present to the attending physicians offering diagnoses when we could, and connect with the patient and the family. All of it was to prepare us for our residency and new life as physicians. Our successes briefly bolstered us, and our failures came home with us and lingered around the edges.
My patient was Anthony, a mid-20s Army cook who was a recovering IV drug abuser. He was also more recently recovering from an urgent median sternotomy after a Ross Switch, where the chest is opened down the middle through the breastbone and the diseased aortic valve is removed under cardiopulmonary bypass and replaced with the patient’s own healthy pulmonary valve. A donor valve is used to replace the “switched” pulmonary valve. He also was midway through a long course of antibiotics and had passed through the storms of heroin withdrawal and was out on the calmer seas of the other side. Regardless, he was in no way interested in talking to me, never responding to my questions about his background or his time in the military. Only silence and a distant stare.
That was until I put my stethoscope on his chest.
His heart sounds nearly leapt out. WHOOSH came the crescendo-decrescendo mid-systolic murmur of aortic stenosis, loud and clear, a four out of six. Then quickly during diastole the low-pitched rumbling of mitral stenosis building just at the end, the results of scarring from infected vegetations on his mitral valve seen on echocardiogram. A cacophony of sound coming from the chest of a man otherwise so silent in life. Every morning they were there, for three weeks on my rotation. Imagine opening the door to a symphony hall and hearing the sounds inside, then closing it and silence again. I heard the sounds of his heart well, more than that of his voice. Indeed, I began to feel that this was the way we were intended to communicate.
“Good morning, Anthony.”
He would nod.
“Anthony, you OK with me sitting here for a while, listening?” I would ask every morning and every evening.
Often, I would arrive a little earlier and sit on the side of his bed, just to understand the sounds sent up through the bell and tube, trying to sear them into my memory. Over time, I could identify where each sound was louder, high on the opposite chest for one, apex for the other. Aortic stenosis. Mitral stenosis. Every day. Listening to what Anthony could tell me, through the sounds of his heart. Hypnotic. Constant.
Until the morning it was not.
One early morning near the end of my rotation, I settled into my familiar position on the side of Anthony’s bed. In the darkness of his room, I warmed my hands by rubbing them together, and slid the stethoscope under his standard issue VA pajamas.
The cacophony was there, but right at the beginning of diastole, right after the brass of aortic stenosis sounded the heart cycle and just as the rumbling base of mitral stenosis warmed up, there was an ever-so-slight, but present “rrrrrr.’ Again, the same, “rrrrrr.” Barely a whisper. I removed my stethoscope, silence. Darkness. I replaced it to his chest. Right there, the interloper! Like a novice crashing the symphony stage. Aortic regurgitation, was it possible, but ever so slight?
On house staff rounds a few minutes later, I passed on my finding. The resident, somewhat disgruntled initially, listened and said he would run it by the attending. Soon afterwards, there was general agreement all around. Anthony spiked a fever to 102.4 F during the echocardiogram that showed a perivalvular abscess had formed around his aortic valve. The new murmur was the sound of the blood rushing back around the sides of the valve, regurgitating back into the ventricle, his heart dangerously dilating as it struggled to pump blood around the body. Anthony’s breathing had become labored. He stared up at me from his bed, his eyes moist around the edges, his breath raspy and wet with fluid building up in his lungs. We started to move in the familiar rhythm of resuscitation around him.
He was taken back to the cardiac operating room emergently for a valve revision. I heard later from the attending that had we not listened to his chest that morning and started the work up early, Anthony would not have survived. I never saw or interacted with him again after that fateful morning. Postoperatively, he was whisked away directly to the cardiac ICU, impenetrable to even the most enterprising and responsible medical student. My own training took me on to the next rotation and Anthony’s recovery ultimately took him home to his parents. When I think of him now, I can just make out his face turned away staring, but I can vividly hear his heart and remember those mornings in the darkness.
A Way Forward, Together
By E. Wesley Ely, MD, MPH, Grant W. Liddle Professor of Medicine
“But can’t you help me die, Doc?”
My 54-year-old patient was alone, with no family or friends in his life. He slumped in his Intensive Care Unit (ICU) bed gasping for breath, staring up at me. Admitted with lung fibrosis and pneumonia, he had scars and infection aggressively replacing his airways, despite our best treatments.
I pulled a chair next to his bed so we could talk at eye level. His face was blank. “I want euthanasia. I’m going to die soon, so what’s the point of living longer. I’m just wasted space.”
The illegality of euthanasia was not what ran through my mind. Instead I thought about how important it would be to help Paul recover his sense of personhood.
This was about two people finding their way forward, together, and we both knew it.
My response came in parts over my days with him. First, I explained that, as his physician, I wanted to guide him through the dying process and that I considered us to be in a mutual covenant. That meant that I would never intentionally harm him, and that we both had a degree of autonomy that had to be respected. “Paul, our covenant includes my limiting your suffering. You are the best judge of when you need more meds for pain, anxiety and breathing. All of us will work day and night to end your distress but we won’t deliberately end your life.”
Finally, with our eyes locked, came Paul’s instruction, “Just don’t abandon me.” And we sat there, partners, nodding.
In fact, Paul was receiving excellent palliative care for his physical symptoms, but that is never enough. When faced with emotional isolation, despair can cast a shadow darker than disease. Physicians fail our patients on a human level. We forget that our patients are more than the sum of their medical conditions, so I asked Paul about other types of therapy — art, music, spirituality, pets, sunshine — that might help him.
“I want my music. Can you get me the soundtrack for Lord of the Rings?” Within minutes we had the songs playing in his room, and his demeanor changed from desolate to alert and engaged. “I love music. It’s always been a motivator, but now…I don’t know.” He shifted in his bed. “My goals are gone.”
Nietzsche’s words came to my mind, “He who has a ‘why’ to live can bear almost any ‘how.’”
Viktor Frankl, as a physician-survivor of Auschwitz, used Nietzsche’s quote four times in his crucial analysis, “Man’s Search for Meaning.” I think this book should be required reading for medical students.
As with many patients, Paul’s story needed unpacking. “Paul, what did you do in life?”
He smiled and shrugged. “I’m a rare beast: a plumber who acts. I do Shakespeare festivals. You know, ‘The quality of mercy is not strained.’ I’ve been mulling over that line. Years ago I didn’t understand it. I do now. Mercy should not be forced. It’s better as a natural infusion, one person to another.”
I described for Paul the many years I spent alongside my single-mom-turned-Shakespeare-director, helping her cue actors. As we talked, I watched his erratic oxygen levels fall and then partially recover, obliging us to take breaks. Sweat would build up on his forehead, and, more than once, I worried that our conversation was too much of a strain. But we were unpacking, and it was essential.
That night I asked Christine, a nurse who loves theatre, to sit and talk with Paul. Soon their lives were interwoven, too.
“I think I helped Christine,” Paul said the next day. “My own illness made me forget that one of my ‘things’ is seeing when someone is hurting. And Christine is. She came to help me, but it turns out some really personal things in our background are weirdly similar. I told her my way through the worst of it.”
It seemed like a good time to return to his previous request. “Paul, you’ve asked for euthanasia, and you brought up Portia’s line from Merchant of Venice about mercy. Can we talk about these things?” He nodded, and closed his eyes.
“We all want to be here with you in your suffering. I want your opinion. I don’t think injecting you with a lethal drug would be truly merciful, but it would, in every sense of the word, be ‘strained.’ It would be a forced and unnatural ‘false’ mercy. Whenever possible, mercy must also be lifting and healing. I don’t want to abandon those key elements in serving you.”
His eyes opened wide. “Doc, talking with you and Christine about life’s best and worst times has helped. She said she’s coming back tonight, and that means the world to me. When we met, I was afraid of being a burden and not mattering. Now I feel different. I’m not a believer like others, but I guess I remembered the ‘why’ to live.”
Short of a war zone, there are few settings as raw as an ICU. Yet treasured moments of human transformation come when people like Paul re-discover their ‘why,’ even if just for an hour or a day.
I see many paths toward death in the ICU: sudden, prolonged, stuttering, reluctant, stoical…and these paths are dynamic, not static. It is a rare patient who asks me for euthanasia, but it is nearly universal that dying patients seek help with suffering. Sitting on their beds brings me right up against their fears, the greatest of which is usually not intolerable pain.
Paul reminded me that the best remedy for such angst is human relationship and community. The problem with assisted suicide and euthanasia for Paul — and for others — is that it presented him with an illusion of ‘cure,’ when in reality it would have left him devoid of the healing he received.
Obviously, this approach won’t be an answer for everyone, yet it is too often left untried. Some will say Paul’s story is merely an uplifting anecdote. Nevertheless, I hope to embrace the lessons I learned at his bedside for most of my patients, and I hope my colleagues do the same.
We are sent to heal even when cure is not possible. What we should be trying to heal is often not physical disease or clinical depression, but a person’s sense of insignificance and hopelessness, which we cannot heal by eliminating the person.
Paul died a week later. By then he realized his life was of value even with a terminal disease. In fact, the last thing he said to me, between gasps, was: “I’m glad I didn’t miss this time. I never saw it coming, Doc…In losing my breath, I gained it.”
Born Without Bones
By Jill Simmons, MD, director, Pediatric Program for Metabolic Bone Disorders
“What do you see when you look at this X-ray?” I asked the pediatric resident who was looking carefully at the patient’s lung fields, evaluating for evidence of an infiltrate. She shook her head and replied, “I’m not sure.” She did not notice the most unusual aspect of the film, as it was too incredible to consider. The film demonstrated a complete absence of mineralization. The patient — a baby girl named Janelly — literally had no bones.
As a new faculty fresh-faced from fellowship completion, I was fortunate to meet baby Janelly, who has an ultra-rare disease called hypophosphatasia (HPP). Patients with HPP are deficient in tissue-nonspecific alkaline phosphatase, which is critical for bone development. Without the ability to build new bone, remodeling leads to an apparent melting away of bones. Historically, the infantile form of this disease was fatal in 73% of patients by age 5. Death was typically due to respiratory failure, as the absence of ribs makes it impossible to breathe without assistance. Janelly required a feeding tube and mechanical ventilation, and she was unable to even move her limbs against gravity. Much of her life was spent in the pediatric ICU with recurrent infections. Her parents, who spoke minimal English, were told her life was nearly over, so they brought her finest clothing to Monroe Carell Jr. Children’s Hospital to take what was presumed to be her final photos. Other than supportive care, there was no treatment for this horrifying disease.
Janelly was one of only 11 infants enrolled worldwide for a clinical trial of a synthetic version of alkaline phosphatase, a bone-targeted enzyme replacement therapy. After a prolonged stay in the pediatric ICU, she was transitioned to home for subcutaneous dosing. And then we waited and waited and waited. It took three years before we began to see evidence of rib mineralization. Once that occurred, Paul Moore, MD, (her pediatric pulmonologist, who had also provided care for her since early infancy) was able to wean her ventilator settings. Amazingly, we watched as she mineralized her skeleton with no inappropriately located mineralization. After five years on a ventilator, she was able to breathe without assistance.
The trial lasted seven years, which is an extremely long time for a life-saving medication, and it must have felt like forever to families who had children with this disease who did not qualify for the clinical trial. Amazingly, the five-year survival rate for infants born with HPP now is 89% on enzyme replacement therapy, and most children with HPP never require mechanical ventilation.
Janelly’s story truly is a magnificent one, and it has been told around the world, including by BBC and PBS. Even prior to the clinical trial, her survival was incredible, and is due largely to excellent care from the multidisciplinary team of providers at Children’s Hospital as well as meticulous care from her parents. What I learned from my experience with this young girl and her family was that dramatic life-saving medical advancement still occurs. I believed from my training that our ability to change disease outcomes would only happen in small increments.
Instead, a disease that almost always killed those who had it during early childhood now can be treated, and bones can literally grow seemingly from nothing. It gives me hope that we can continue to have impactful scientific advancements that can radically alter the course of severe disease. And, I remember every day how thankful I am that I got to play a role in this for Janelly. Most importantly, I am thankful to know a young child and her family who took a risk on a medication that had never been given to a human. Her family’s courage means that parents of children with HPP no longer need to bring their children’s finest clothing to the hospital for final photos.