The Power of Self-Advocacy

Mark A. Lewis, M.D. @marklewismd

by Mark Lewis, MD

Hypochondriasis was the diagnosis that first sprang to the doctor’s mind as he surveyed his new patient.

The 30-year-old man had just moved from out of state to begin a high-pressure job. Compounding those stressors, he had steeped himself in medical textbooks and become convinced—on the basis of a single abnormal lab test—that he carried a genetic defect predisposing him to cancer.

Surely, then, there was a documented mutation in the preceding generations? But when pushed to fill in the details of his family history, the patient demurred that he lacked any formal records for his relatives.

Still, he insisted that his father, paternal uncle, and paternal grandfather must have shared the same hereditary flaw, now passed down to him. Further inquiry revealed that these three older men had died in different decades of life and of entirely different causes, weakening the argument for a common, mortal bond. Indeed, if this was the court of law, the witness would have crumbled under cross-examination, his testimony exposed as a shoddy amalgam of circumstantial evidence.

But medicine is not jurisprudence. Instead, the physician serves as both judge and jury in assessing what’s faulty and determining the proper course of corrective action.

The doctor was tempted to render a judgment of severe anxiety and send the man away with a prescription for calming anti-anxiety medication. And yet he also had a sinking feeling that this new patient would not be so easily dismissed. For one thing, they shared a workplace, and it seemed inevitable that they would remain in awkward proximity for years to come. And then there was the unsettling fact that the patient was an M.D. himself. Not only do doctors legendarily make the most insufferable patients, this young man was— worst of all—a budding oncologist with a fervid imagination. No wonder he was susceptible to seeing seeds of cancer sown in his own pedigree! Perhaps, then, it was best to accede to his demands and simply order the genetic test he had asked for by name. The elder physician looked forward to calling him in a few weeks to soothe his nerves when the result returned negative.


An endoscopic ultrasound showing the dominant mass in the head of Dr. Lewis’ pancreas.
An endoscopic ultrasound showing the dominant mass in the head of Dr. Lewis’ pancreas.

Such reassurance never arrived. Rather, the laboratory confirmed the presence of a disease-causing mutation for multiple endocrine neoplasia type 1 (MEN1). MEN1 is passed down, even when only one parent possesses the mutation,  matching the pattern the patient described. MEN1 also explained why the 30-year-old had developed osteoporosis at a shockingly young age, as his parathyroid glands had become constitutively overactive and were leaching calcium from his bones. Other manifestations of this endocrine disorder were found on an MRI showing a small growth on his pituitary gland and on an endoscopic ultrasound showing multiple tumors on his pancreas.

 Hypochondriasis had been disproven by a chilling reality.

I know all this because I am the patient in question. I was just starting my fellowship in oncology at the Mayo Clinic when I developed terrible abdominal pain and was found to have an elevated level of calcium in my bloodstream.  My father had suffered from the same problem, and there are only a few conditions in which hypercalcemia runs in families. This metabolic disturbance was a clue to a larger pattern of misfortune.

My dad, paternal uncle, and paternal grandfather were all Presbyterian ministers, men of faith united by a love of God, and a streak of especially bad luck. My father died at 49 from a “lung cancer” that was, in fact, an aggressive neuroendocrine tumor, which arose in his thymus, just behind his breastbone. His brother died in his early 50s, mere days after a neurosurgeon attempted to remove a large benign pituitary tumor. My grandfather, a towering figure in the Protestant community of Belfast, survived the sectarian violence of The Troubles, but was forced to leave the pulpit in his 60s as his voice faltered; his strength sapped by a mysterious growth in his upper chest.

The 3 Ps of MEN1: the pituitary, parathyroids, and pancreas

MEN1 is most easily remembered as the 3 Ps of the affected organs: the pituitary, parathyroids, and pancreas. This alliterative triad is tidily arranged along the midline of the body. Thus the disease is readily learned for tests in medical school and later board examinations, but, due to its rarity, will never graduate beyond trivia for most doctors.

A deeper meaning lurked for me, awaiting discovery after years of inexplicable tragedy. Somewhere between my hippocampus and my heart, between the esoterica I’d memorized as a student and the sorrowful life lessons of repeated loss, I connected the dots and named my family curse. Simply put, I was in the right place at the right time to identify the glitch in my DNA and then disentangle my fate from that of my forefathers.

I realize how tremendously privileged I’ve been as a patient-physician. I’ve been given a finely ground lens through which to interpret the anomalous signals from my own body and then the professional persuasion to cajole other doctors into helping me. Here, I feel the utmost sympathy for my fellow patients who lack such leverage. There is virtually no aspect of the carcinoid syndrome, for instance, that cannot be misinterpreted as a more benign condition. Flushing can be misconstrued as hot flashes. Wheezing can be incorrectly attributed to late-onset asthma. Diarrhea can be ascribed to any number of gastrointestinal ailments. Most distressingly, the very nomenclature of neuroendocrine tumors is a minefield of misnomers, none more ripe for underestimation than the term ‘carcinoid’ itself.  How many well-intentioned doctors have told patients there was “nothing to worry about,” that their neuroendocrine tumor was only “cancer-like,” even as it placed them at fatal risk for liver or heart failure, not to mention posing an enormous detriment to their quality of life?

I am two-faced to be sure—hopefully in the best possible sense of that term—as I occupy both sides of healthcare: benefactor and beneficiary. But either way, I know that I am the only person to inhabit my body 24/7 and am therefore best suited to explain its aberrancies to my own physicians. We should trust our instincts and be persistent when we sense a disturbance in our equilibrium.

Through it all, through the initial doubts of my somatic complaints and then the bittersweet vindication of my deepest suspicions, I have learned this one undeniable truth: for all the studied intellect of doctors, and the incredible array of diagnostic testing by which they can refine their hypotheses, there is no force in medicine more positive and powerful than the self-advocating patient.

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2 years ago

What a fine man. I read your story and I was rocked to the core. I had a neuroendrine tumour (atypical) in my right lower lung. The diagnosis took a long time then after two throcatomys they removed my right lower lobe lung.
I was very ill there for a time, and looking back it took me over two years to “feel better”

That was back in 2008, now I have had a cyst removed from the brain, thyroid removed and now osteoporosis has set in.
I don’t think I will feel ever the same again. I honestly think all these problems have been the result from neuroendrine disease.
I am sure the doctors I visited probably thought it was all in my mind. But there you go.
Please keep up your wonderful work and someone is looking down on you and your family.

All the best
Kind Regards.

Lesley Barkmeyer Perth Australia

2 years ago

Mark A. Lewis and NET Tumor Research Foundation thank you so much for this article. I imagine many lives have been and will be soothed and energized by reading it.

I sincerely thank you.

Another NET Patient,
Deborah DeNail, Ohio, USA

2 years ago

You are the answer to many prayers! Press on doctor! My husband was also a patient at Mayo Clinic AFTER I had advocated heavily for more scanning. He was 32 and diagnosed with a neuroendocrine tumor in the tail of his pancreas. Too many times we have heard, ‘its psychological’ in regard to His symptoms. Now, instead of becoming angered, I calmly hold my ground, explain my opinion of not agreeing with their opinion and let them know it’s okay to say ‘I don’t know to a patient.’ The physicians that acknowledge they are stumped but are willing to look further or refer, etc have my utmost respect.

Susan Krell
2 years ago

I have had to advocate for myself since I was 16. That is when the diarrhea began, puberty. I had grown 6 inches in 3 months. I was told I had spastic colon, then colitis, my doctors would not believe I could poo as often as I said.
At 5’11” and 107 pounds I was starving all the time. The doctors decided I must be taking laxatives to stay thin, it was the 60’s. I wasn’t, but they had decided
my upper & lower GI tests were fine, I was a Hypochondriac! I learned to not eat until after 4 pm so I could work without staying in the bathroom. Not until age 50 was I able to make an appointment for a “routine” colonoscopy. When the doctor said, Who referred you? And I responded, I did, I am 50, have a history of colon cancer (grandmother, Norwegian decent) so I qualify to have a colon screening. He agreed it was covered. He found a tennis ball sized mass in my illeosecal valve, I had felt it, but my gynecologist said that there was nothing there, & would not refer me. When my cancer was found, I was already stage 4, I have been stage 4 for 12 years now. People do not believe I have cancer because, I don’t look bad. My eldest son is now type 1 immunodeficiency diabetic, his pancreas shut down. I hate to think I passed this on to my two sons.

E.J. Kornfeld
2 years ago

For close to two years, twelve doctors have told me my “hot flashes” were due to menopause.

I went through menopause five years. I did not have one “hot flash” before, during, or directly after it started. Despite two positive Chromogranin A tests, they continued to say menopause. Hormone replacement, an SSRI, and Brisdelle did not work. The Gastroenterologist said I do not have symptoms of Carcinoid Syndrome.

I did not accept the diagnosis of menopause related. I finally persuaded my doctor to send me to an allergist. She said “no allergies, probably menopause related. ” After two months of an abnormal WBC, she referred me to a Hematologist.

He did another WBC – normal. But, for the first time in two years – he said “no way, this sweating is not due to menopause.”

I knew something was wrong. If I were a man with my symptoms and blood tests, more tests would have ordered.

I feel relief that someone finally believed, panic that I am diagnosed with cardinoid syndrome. He said we will figure this out together. I will be referred to other specialists, but he will remain my primary contact.