WITH APOLOGIES TO JOHN STEINBECK

There is a terrible pun in the title of this site, Of Tumors And M.E.N. But I accept full responsibility for the bad joke because I've also embedded an important, under-recognized acronym, standing for Multiple Endocrine Neoplasia.

M.E.N. syndromes predispose those affected to lifelong tumor development in various endocrine glands. In M.E.N. type 1 (often abbreviated as MEN1), the at-risk glands are the pituitary, the parathyroids, and the pancreas. Known by the mnemonic of the 3Ps (alliterative organs tidily arranged along the midline of the body) the disease is readily learned for tests in medical school and later board examinations, but will never graduate beyond trivia for most doctors. 

My memory was stirred in a different way when, at the age of 30, I was diagnosed with high calcium levels. 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, my paternal uncle, and my 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 pituitary adenoma. 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 as his voice faltered, his strength sapped by a mysterious growth in his upper chest.

Somewhere between my hippocampus and my heart, between the esoterica I'd memorized in med school and the sorrowful life lessons of repeated loss, I connected the dots and named my family curse: M.E.N. type 1.

The clergymen went to their graves unaware of the shared flaw in their genetic code. Only through their tragic ends was I given the awareness to see my own tumors coming. In my case, forewarned is forearmed, but the knowledge of my germline mutation was hard-earned by the generations above me. I owe it to them to learn from our familial mistake, and to publicize the glitch hidden in our DNA, braiding us together even as I try to disentangle our fates.

Mark Lewis5 Comments