When I perform a liver resection, I start with a so-called exploratory laparotomy. This is a full check under the hood into the abdominal cavity; the stomach, small intestine, colon, pancreas, pelvic organs, and kidneys are inspected and palpated. Despite her normal test results before the operation, during the laparotomy I felt a small area of thickening in the lower portion of her colon where it joined the rectum. I could not see any abnormality on the outside of the colon, but it clearly did not feel normal. I inserted a flexible scope into her rectum and it revealed a one-centimeter tumor at the junction of her rectum and sigmoid colon. It was a flat tumor and had not been identified during her previous colonoscopy. A biopsy confirmed adenocarcinoma. I realized I was not dealing with an intrahepatic cholangiocarcinoma, but a large solitary metastasis from a colorectal cancer. Leaving the colon tumor alone, I proceeded with removing the liver tumor knowing that if it grew only a little more it would extend from the right to the left lobe and become unresectable. During her recovery, I explained to my patient and her mother that we were dealing with stage IV colorectal cancer, and chemotherapy and another operation to remove the primary tumor in the colon would be necessary. My patient asked why I did not remove the colon cancer during the liver operation, and I explained the incision I had made was in the upper abdomen and her colon cancer was at the opposite end of the abdominal cavity, in the pelvis. I also informed her we had not completely cleansed her colon before the operation and it was possible she would have ended up with a temporary colostomy. I described what a colostomy would mean for her, and I admitted I really didn’t want to get my butt kicked by a girl for giving her one. She found this answer completely satisfactory, complimented me for making a wise decision, and told me in resolute fashion, “We will beat this thing.”
This remarkable young woman recovered uneventfully and subsequently received intravenous chemotherapy for six months. I then performed a second operation to remove the section of the colon bearing the malignant tumor and did an intraoperative ultrasound of her liver. To my disappointment, and again despite normal preoperative CT scans, I found two small tumors in the hypertrophied left lobe of her liver that a biopsy confirmed as metastatic adenocarcinoma. I destroyed both of these tumors with radiofrequency ablation, but I suspected that this finding did not portend well for her. My colleagues in pathology and genetics did studies of her normal colon, the primary colon cancer, and the liver metastasis but could not identify any known syndrome or genetic abnormalities that explained her developing colon cancer at a young age.
After her second operation, all was well. The patient returned to her home state and resumed her career, care for her son, and karate with joyous abandonment. She became my leading and favorite source for email jokes and humorous pictures. Rarely did a week go by without at least one hilarious, sometimes inappropriate joke, video clip, or picture from her. She also sent newspaper articles from her small town describing strange-but-true occurrences, and she would bemoan her fate as a diva living among rednecks.
I saw her every three months and all blood tests and CT scans were normal. But at the one-year mark the bubble of optimism burst. There were several new tumors in her liver. Scans did not show tumors at any other location so she received another three months of different chemotherapy drugs and then a third operation to perform a combination of resection and radiofrequency ablation of the remaining liver tumors.
One afternoon while she was in the hospital recovering from this third operation, I walked into her room with an entourage of surgical fellows, residents, and medical students. “I’ve been waiting for you and your team,” she announced. “I want to show them what the mad scientist has created.” Unashamedly, she pulled down the bed sheets and pulled her gown up to her chest to show an impressive combination of surgical scars and a new incision and said, “Ta-da, Frankenbelly!” She was a piece of work.
She agreed to an additional three months of chemotherapy reluctantly, noting that chemotherapy “cramps my style.” Energetic and indefatigable, “Frankenbelly” recovered and returned to her small hometown. The weekly stream of jokes, pictures, and upbeat messages resumed. She also mailed crayon drawings produced by her kindergarten-aged son with the recurrent theme of a surgeon (me) performing operations on his mommy. There were unintelligible scrawl marks in the margins of the artwork, which my patient politely interpreted as her son’s thank-you notes to me. She also told me he included warnings that he, too, was learning karate so I could be in for a double butt kicking.
Great. Double jeopardy.
Every time I saw this young woman she informed me she had a son to raise and her plan was to beat this “cancer thing.” While I desperately wanted to be convinced that she could kick cancer’s butt, her cancer never agreed. Six months after completing her third round of chemotherapy, her cancer roared back with multiple tumors in her lungs and in her peritoneal cavity and pelvis. Though the tumors in her pelvis caused severe pain, incredibly, she found ways to create humor from her own significant physical discomfort. During a clinic visit she described the pain as, “being pulled on my bare ass behind a BMW going seventy miles per hour down a rough asphalt road.” She went on to explain she chose to be pulled by an imaginary BMW because she was a lady of class and distinction and she wanted me to remember that always. I will never forget her description and her toughness.
My colleagues in the pain-management service concocted combinations of medications to help relieve her discomfort. The powerful medications did not eliminate her pain entirely, but after about a month she reported that the BMW pulling her backside down the road had slowed down to twenty miles per hour. These words are severely overused these days, but this young woman was unbelievable and awesome! Her situation also made me feel an abiding sadness. The respect I had for her was complete, as was my admiration for her relentless determination and optimism.
Almost thirty months after I met this black-belt, butt-kicking diva, she developed a cough. A chest X-ray revealed that the tumors in her lungs were growing rapidly, and within days they almost doubled in size. She came in to the emergency room on a Sunday night because of coughing and shortness of breath. She was admitted by one of my colleagues in medical oncology, so I did not learn she was there until the next morning.
I went to her room, quietly knocked on the door, and entered. The room was darkened and the window shades were pulled down. Her admitting diagnosis was “difficulty breathing,” but the reality I witnessed was far more graphic and horrifying. The awful image is indelibly etched in my memory and was incomprehensively brutal. My patient was wearing a large rebreathing oxygen mask and she was using every bit of her breath and energy to say goodbye to her six-year-old son on the phone. She knew she was dying. The only other person in the room was her mother. They were both terrified. Her mother told me that her daughter had been struggling to breathe for the past six hours. She was taking forty to fifty short, quick breaths per minute. Despite the maximum oxygen flowing through the rebreathing mask, she was using all of the muscles in her neck, chest, and belly wall to gasp for air. She tensed and quivered as she made an exhausting effort to breathe. When she hung up the phone after a heart-wrenching conversation with her son, she managed to say she was scared.
I was surprised when she turned to the tear-streaked face of her mother and asked her to leave the room for a moment. Without hesitating, her mother left, and I sat down in the chair next to the bed and grasped her hand. She spoke, but it required more than five minutes for her to say what usually would have taken her fifteen to twenty seconds. She would gasp out a few words, then struggle for interminably long pauses to get enough oxygen, and then wheeze out a few more words. She told me she had said goodbye to her son and to her father. She had told her son that she would be there to watch over him during the course of his life. Then, after an almost minute-long pause, a noticeable sense of calm came over her and she looked into my eyes. “I’ll watch over you, too.” Stunned and stupefied, I stammered out an incredulous
thank-you. She nodded toward the door indicating she was ready for her mother to return to the room. Her mother came in, sat on the side of the bed, and held tightly to her daughter’s right hand while I sat holding her left hand.
My patient had signed a DNR, or Do Not Resuscitate, order the previous night. She did not want to be on a mechanical ventilator or have the medical team do anything to alter her invariable death. The oxygen mask was not sufficient to overcome the volume of cancer replacing her normal lung tissue. Nothing was going to change her outcome.
She glanced toward the window and I opened the blinds to the bright sunshine. As her mother and I watched, her fingernails, then her fingers, and finally her lips turned blue. Her rasping, maximum-effort breathing became slow, irregular, and ineffective. With a final pursed-lip exhalation, she passed from this life.
Benumbed by grief, after a few minutes I went automatically into doctor mode. I rose from the chair and felt for a pulse in her carotid artery. There was none. My eyes welled with tears that rolled down my cheeks. During the agonizing five-minute conversation I had had with her, she told me not to mourn her passing and to keep working to defeat cancer. But I did mourn. I also unprofessionally wiped the tears from my face with the sleeves of my white doctor’s coat, and then turned, walked from the room, and informed the nurses of her time of death.
Grace. I have no other word to describe this woman and the gift I was given. I watched my patient die an ugly death, slowly suffocating. Her last spoken words were directed to me. I initially struggled to understand how I could accept such a gift in the face of her death because I had failed, and she had lost her battle with cancer. I eventually realized it was not my place to question such a gift, but to accept it with the dignity with which it was given.
The day after my patient’s death, her mother telephoned my office and left a phone number for me to call, so I did. I realized it was my patient’s cell phone when I heard her announce, with her usual buoyant, lilting, and teasing tone, that she was unavailable “just now” but to leave a message. I was initially unnerved hearing her voice until I remembered her saying that she would watch over me. I smiled and felt a sense of comfort, and I left a message. Her mother thanked me later for sitting with them in the hospital room during that frightening and difficult time. From my conversation with her mother I understood the source of my patient’s spirit as she exhorted me to keep working to find better ways to treat and beat the disease that took her daughter’s life.
As I mentioned before, this is not a pleasant or pretty story. But like the Taoist concept of yin and yang, it represents the balance of opposites in the universe. Light and dark. Love and hate. Conflict and peace. Beauty and ugliness. Life and death.
I am reminded every day of a basic reality: cancer sucks.
12
Mister Lobster Guy
“We ought to be vigilantes for kindness and consideration.”
Letitia Baldrige
Consideration: Thoughtfulness and sensitivity toward others
“Hey, can you go find all of the medical students, residents, and fellows we have in the clinic?” I stuck my head out of an examination room and asked my nurse to go on a search-and-fetch mission to find trainees. “This is something they may never see again and I want everyone to take a look at this.”
My nurse looked at me quizzically, “You want me to find all of the students and fellows and bring them now?”
“Yes, please go round up everybody you can and bring them in here!” We are in Texas after all, so rounding up people and cattle is something that occurs routinely.
Lest you think me a complete surgical lout, I will inform you I sought and received permission before sending out a Calling-All-Trainees bulletin. As I was standing in the door talking to my nurse, the patient in the room was openly chuckling. He informed me he was becoming accustomed to these requests. He was a new patient, a previously healthy fifty-six-year-old man, referred to me to treat an unusual problem. He had carcinoid syndrome from liver metastases caused by a primary small-bowel carcinoid tumor. I put out the call to all of the trainees in the clinic because as we sat and talked, my patient developed a classic carcinoid flush. As I watched, a blush crept up his neck and onto his face and he turned bright red. I asked him if he was aware of the flush and he said, “Yes, I can always feel it coming on but I can never predict when it will happen.”
He informed me he had been having five or six episodes daily for almost four months, and he reported abdominal bloating along with ten to twelve loose bowel movements every day. He noted, “I always know where the nearest bathroom is located.” He had seen his physician after enduring these symptoms for three months. The inevitable, perfunctory CT scan revealed liver tumors and a biopsy showed metastatic neuroendocrine cancer cells. Further pathology and blood tests confirmed a carcinoid tumor, a type of neuroendocrine cancer. He saw a gastroenterologist followed by a medical oncologist and then ended up in my clinic.
This gentleman was a high school history teacher and his “events” occurred regularly during his classroom teaching sessions. He would turn bright red or quickly excuse himself for a bathroom excursion. He told me a great story. One day, he flushed in front of a class and a student blurted out, “Look, it’s Lobster Guy!” The classroom went completely silent. In a moment of teenage angst that goes along with the sudden self-recognition that the brain-to-mouth filtering system has been disengaged, the offending student himself blushed red and mumbled an apology. My patient, a large, imposing man fixed the student with a stern look and stated, “Let me be very clear, it’s Mister Lobster Guy to you.” The class erupted with laughter and cheered!
A nickname was born.
After collecting half of a dozen trainees from the clinic, I had them examine his bright-red face and touch his skin, which was not warm. This is surprising because people who flush usually do so in the middle of a high fever and their skin is warm or even hot to touch. The students and trainees proclaimed “Oh, wow!” and “That’s amazing!” and then quietly filed out of the room. I thanked my patient for allowing the teaching moment because carcinoid tumor and carcinoid syndrome are relatively rare occurrences in surgical and medical oncology.
Carcinoid tumors can originate almost anywhere in the gastrointestinal tract from the esophagus, stomach, small intestine, pancreas, appendix, or colon and rectum. They can also arise from the lungs as a condition called bronchial carcinoid. These sites contain enterochromaffin cells, and when they begin to grow abnormally they form a tumor. Carcinoid tumors have a predilection to metastasize to lymph nodes, and, for tumors in the gastrointestinal tract, to metastasize to the liver. Occasionally a one-centimeter or smaller carcinoid tumor will be discovered in an appendix that has been removed for appendicitis. In that situation, no further treatment is necessary. Or if a carcinoid tumor of one centimeter or less is removed during a colonoscopy, no further surgery is indicated, but close follow up and a colonoscopy every few years is recommended. However, for larger carcinoid tumors arising in the small intestine or colon, there is a risk of metastasis to lymph nodes in the area, so a more formal cancer operation that removes a segment of the intestine and all of the lymph nodes in the area is standard treatment.
Carcinoid tumors are unusual in that they tend to be very slow-growing, and many patients have them for years, if not decades, before symptoms or manifestations of the disease develop. For example, tumors in the bowel may cause obstruction or bleeding before a patient realizes something is wrong.
Carcinoid tumors are interesting because once they metastasize to the liver, carcinoid syndrome can occur. Serotonin plays numerous roles in regulating normal functions in the intestine and in the brain. But when excess amounts of serotonin are released by carcinoid tumors in the liver, the result is episodes of flushing, diarrhea, heart palpitations, asthma-like symptoms, and high blood pressure. If not addressed and treated, a patient can go on to develop thickening of the pulmonic and tricuspid heart valves and right heart f
ailure.
I have performed liver operations on hundreds of patients with carcinoid tumor metastases and carcinoid syndrome, but I have never cured a single one. That may seem strange because a basic tenet of surgical oncology is to remove all cancer-bearing tissue with clear surgical margins during an operation performed with curative intent. Carcinoid is one of the rare exceptions to that hallowed convention. Patients treated with surgical liver resection or ablation techniques can live for many years, often for a decade or more despite the fact that the surgery performed is not “curative” (defined as removal of all detectable malignant tumors).
Patients with carcinoid syndrome from liver metastases have several treatment options available to them. Because their symptoms are related to release of excess levels of naturally occurring hormones that bind to specific receptors on the surface of normal cells, we can block the effect of the hormones with an intramuscular injection of long-acting octreotide in the gluteus maximus once every twenty-eight days. Octreotide acetate binds to somatostatin receptors on a variety of cell types throughout the body and can remarkably relieve diarrhea, flushing, and other symptoms related to high serotonin levels. My patients like to say, “It really helps the symptoms but it’s a real pain in the butt!” However, this treatment is not effective in all patients. For many we are forced to increase the dose until we reach the maximum, and even still, some patients eventually develop severe symptoms despite the medication. There is now another monthly injection medication available that has helped, but some patients’ symptoms are not totally alleviated.