Mys­tery’s answer is a life­saver

After 17 years, a spe­cial­ist’s ques­tions fi­nally re­vealed what was ail­ing a school prin­ci­pal.

The Washington Post - - FRONT PAGE - BY SAN­DRA G. BOODMAN

His ane­mia was fol­lowed by sear­ing foot pain. Seven­teen years later, a stel­lar ath­lete learned what was wrong.

Jeff Si­pos wasn’t used to feel­ing wiped out. ¶ At 31, the Cal­i­for­nia el­e­men­tary school prin­ci­pal was an en­durance ath­lete in ex­cep­tional con­di­tion. Even though he had asthma, Si­pos had climbed Mount Whit­ney, the tallest moun­tain in the con­tigu­ous United States, com­pleted a rim-to-rim hike in the Grand Canyon in a mere 10 hours — at least two hours less than av­er­age — and thought noth­ing of clock­ing 100-mile bike rides on week­ends. ¶ But in May 2001, his en­ergy was flag­ging. Si­pos, who lived in River­side, con­sulted his long­time gen­eral prac­ti­tioner, who or­dered blood tests that re­vealed an ab­nor­mally low iron level. ¶ Sev­eral years later when he de­vel­oped foot pain, Si­pos saw a po­di­a­trist who told him he had os­teoarthri­tis, typ­i­cally caused by wear and tear on joints. A few years later, a spe­cial­ist di­ag­nosed his painful wrists and an­kles as rheuma­toid arthri­tis, a se­ri­ous au­toim­mune dis­ease. That was fol­lowed by the di­ag­no­sis of a rare blood con­di­tion that a hema­tol­o­gist mon­i­tored, but did not treat. ¶ It wasn’t un­til 2018 — nearly 17 years to the day after he first saw a doc­tor for fa­tigue — that Si­pos learned he had none of those ill­nesses. ¶ The news was de­liv­ered by a spe­cial­ist whose gen­tle, mea­sured tone be­lied the shock­ing mes­sage that gal­va­nized Si­pos: With­out treat­ment, which was long over­due, he could die.

“We both started cry­ing,” Si­pos re­called of his re­ac­tion and that of his wife, Tami. “It felt like the oxy­gen had been sucked out of the room.”

He cred­its his sur­vival to the ex­per­tise and com­pas­sion of that spe­cial­ist, who continues to over­see his treat­ment.

En­ergy boost

Si­pos trusted the fam­ily physi­cian, who had de­liv­ered his sons. The doc­tor told Si­pos his fa­tigue was the re­sult of ane­mia and could be eas­ily treated with an over-the-counter iron sup­ple­ment that he should take when he felt un­usu­ally tired.

The pills seemed to work. “For the next 10 years I would pop iron pills ev­ery so of­ten and no­tice a bump” in en­ergy, Si­pos said.

In 2007 a new prob­lem arose: The soles of his feet ached and burned. He con­sulted a po­di­a­trist who told Si­pos he had “fallen arches” — flat feet that de­velop in adult­hood. The doc­tor pre­scribed or­thotics. For a while, they quelled the pain.

By 2015, the foot pain was con­sid­er­ably worse and had en­gulfed his an­kles, putting an end to his par­tic­i­pa­tion in ex­treme sports. Si­pos’s hands felt tingly and numb and he was re­ferred to a neu­rol­o­gist and a rheuma­tol­o­gist.

The neu­rol­o­gist told Si­pos he was suf­fer­ing from carpal tun­nel syn­drome — numb­ness or tin­gling in the hands caused by a com­pressed nerve — as well as pe­riph­eral neu­ropa­thy, dam­age to the nerves out­side the brain and spinal cord.

“He said I should have it looked at be­cause some­one 46 years old should not have neu­ropa­thy,” Si­pos re­mem­bers be­ing told. Pe­riph­eral neu­ropa­thy can be caused by a va­ri­ety of ail­ments, among them di­a­betes, liver dis­ease and bone marrow dis­or­ders.

The rheuma­tol­o­gist or­dered blood tests and di­ag­nosed “seroneg­a­tive” rheuma­toid arthri­tis. Al­though test­ing did not show an­ti­bod­ies to the dis­ease, his joint pain, the doc­tor said, strongly sug­gested it.

The rheuma­tol­o­gist pre­scribed two drugs: methotrex­ate, a chemo­ther­apy drug used to treat rheuma­toid arthri­tis, and when that didn’t help, hy­drox­y­chlo­raquine, an anti-malaria drug used to treat au­toim­mune dis­or­ders. Nei­ther worked.

At that point, Si­pos re­called, the pain was so in­tense “the weight of the [bed] sheets would feel like my feet were be­ing crushed.” He wore braces on his wrists and an­kles in a mostly failed at­tempt to deaden the pain, and took the opi­oid pain killer hy­drocodone to make it through the day.

In 2016, Si­pos con­sulted a sec­ond rheuma­tol­o­gist. She agreed it was odd that nei­ther drug had been ef­fec­tive and pre­scribed other med­i­ca­tions. When they failed, she or­dered a test for im­munoglob­u­lin M (IGM) an­ti­bod­ies, which can as­sess im­mune func­tion. The test showed an “m spike,” an in­di­ca­tion that Si­pos might have a pre­can­cer­ous con­di­tion or pos­si­bly mul­ti­ple myeloma, an un­com­mon cancer.

In early 2017 she re­ferred Si­pos, who by then was suf­fer­ing from fre­quent night sweats, to a spe­cial­ist who treats blood dis­or­ders.

A pre­cur­sor?

The hema­tol­o­gist im­me­di­ately or­dered urine and blood tests. On the ba­sis of those, Si­pos said, she as­sured him he did not have cancer, but rather a pre­can­cer­ous con­di­tion called MGUS, short for mon­o­clonal gam­mopa­thy of un­de­ter­mined sig­nif­i­cance.

MGUS is of­ten found in­ci­den­tally, when doc­tors are look­ing for other things. It fre­quently causes no symp­toms but must be watched closely be­cause in about 1 per­cent of pa­tients an­nu­ally it can progress to mul­ti­ple myeloma, a cancer of plasma cells, or an­other ma­lig­nancy. If there is no pro­gres­sion, treat­ment is usu­ally not re­quired.

“She said the stan­dard of care was watch and wait,” Si­pos re­called. That meant ap­point­ments ev­ery three months to check his blood and urine.

Si­pos said the doc­tor also per­formed sev­eral bone marrow biop­sies but the re­sults were in­con­clu­sive.

When he asked the doc­tor about the un­re­lent­ing burn­ing pain in his hands and feet, she of­fered no ex­pla­na­tion.

In early 2018, he said, he con­fronted her. “I’m get­ting worse,” he re­mem­bered say­ing. Her re­ply was stark. “She said, ‘ Yes, you may be in a wheel­chair, but we would never treat this.’ ” His IGM level, she told him, was not suf­fi­ciently el­e­vated to war­rant the risks of treat­ment, which would in­volve chemo­ther­apy. Watch­ful wait­ing was the safest course.

The hema­tol­o­gist told Si­pos he was free to seek a sec­ond opin­ion. He was ini­tially hes­i­tant. Even though she was rou­tinely an hour or two late for sched­uled ap­point­ments, the doc­tor “seemed so knowl­edge­able and wise and would speak with such author­ity,” he said. But at his wife’s urg­ing, Si­pos de­cided it was time for an­other ex­pert to weigh in.

What he didn’t know was that get­ting a sec­ond opin­ion would pos­si­bly save his life.

A dra­matic re­ver­sal

Si­pos and his wife waited six weeks for the con­sul­ta­tion with Muham­mad Omair Ka­mal, an as­sis­tant pro­fes­sor of med­i­cal on­col­ogy at Loma Linda Univer­sity Cancer Cen­ter.

Trans­fer­ring his records to Ka­mal in prepa­ra­tion for the ap­point­ment was a strug­gle, Si­pos said, re­quir­ing mul­ti­ple phone calls by him as well as Ka­mal’s staff.

Both Jeff and Tami Si­pos say that de­tails of the May 14, 2018, meet­ing are seared into their mem­o­ries.

“I re­mem­ber Dr. Ka­mal leaned for­ward and in his soft, sweet voice full of con­cern said, ‘Mr. Si­pos, has any­one told you you have cancer? How come you’re not get­ting treated for this?’ ” Si­pos re­called.

The stunned cou­ple re­peated what the hema­tol­o­gist had been say­ing for more than a year: that Si­pos did not have cancer and that mon­i­tor­ing was the best course of ac­tion.

Ka­mal stren­u­ously dis­agreed. “You have cancer,” Si­pos re­mem­bers him say­ing. “If you were my pa­tient, you’d be started on chemo­ther­apy to­mor­row.” Then he handed the cou­ple treat­ment guide­lines from the Na­tional Cancer In­sti­tute.

Al­though he still didn’t have all of Si­pos’s records, Ka­mal said he strongly sus­pected the prin­ci­pal had a rare, slow-grow­ing form of B cell lym­phoma called Walden­strom macroglob­u­line­mia, also known as Walden­strom’s or lym­pho­plas­ma­cytic lym­phoma. Ka­mal had seen four other cases in his ca­reer. MGUS can be a pre­cur­sor of mul­ti­ple myeloma or Walden­strom’s. Si­pos later learned that a Jan­uary 2017 pathol­ogy re­port men­tioned it as a pos­si­ble cause of his symp­toms.

About 1,500 cases are di­ag­nosed an­nu­ally in the United States, com­pared to about 32,000 cases of mul­ti­ple myeloma.

Walden­strom’s oc­curs mostly in men over 60 when lym­phoma cells in bone marrow pro­lif­er­ate, crowd­ing out nor­mal red and white blood cells. Ane­mia is com­mon and symp­toms in­clude fa­tigue, neu­ropa­thy and night sweats. There is no cure. Treat­ment typ­i­cally con­sists of chemo­ther­apy and tar­geted treat­ments that kill only cancer cells. The dis­ease is di­ag­nosed through blood tests and a bone marrow biopsy and can be con­firmed by a test for a ge­netic mu­ta­tion.

Ka­mal said he can­not fathom why Si­pos went un­di­ag­nosed for so long. “He was see­ing the right doc­tor,” Ka­mal noted, re­fer­ring to the proper spe­cialty.

When he re­ceived Si­pos’s com­plete records, Ka­mal said, “lab after lab after lab” showed re­sults that war­ranted treat­ment. “There are not many dis­eases [other than Walden­strom’s] where you see high IGM and neu­ropa­thy. Most pa­tients are di­ag­nosed within a year or two.”

Si­pos said that when he told the first hema­tol­o­gist what Ka­mal had rec­om­mended, she balked and in­sisted that her di­ag­no­sis was cor­rect. Switch­ing doc­tors was prob­lem­atic; Ka­mal was out­side his in­sur­ance net­work.

So an­other month elapsed while Si­pos, un­sure about what to do, ob­tained an­other opin­ion. The third cancer spe­cial­ist strongly sided with Ka­mal. “If you don’t have chemo, you’re go­ing to ex­pire,” Si­pos re­mem­bers her say­ing.

Si­pos called his in­sur­ance com­pany and re­quested a trans­fer to Ka­mal; it was quickly ap­proved.

He then un­der­went an­other bone marrow biopsy and test­ing to rule out amy­loi­do­sis, a rare dis­ease caused by the ac­cu­mu­la­tion of a pro­tein that can re­sult in or­gan fail­ure. A pos­i­tive test for the MYD88 L265P mu­ta­tion linked to Walden­strom’s clinched the di­ag­no­sis. (The mu­ta­tion is not passed on to off­spring.)

In mid-au­gust 2018, Si­pos be­gan a gru­el­ing chemo­ther­apy reg­i­men to treat Walden­strom’s. He wore a Su­per­man T-shirt that one of his kinder­garten stu­dents had given him to ev­ery ses­sion as a sort of tal­is­man. By Jan­uary 2019, his cancer was in re­mis­sion.

But the ag­o­niz­ing nerve dam­age to his hands and feet continues to plague him. Ka­mal said the dam­age is prob­a­bly ir­re­versible and may have oc­curred be­cause his cancer went un­treated for so long.

Si­pos, who sees a pain spe­cial­ist, said he has been pre­scribed methadone, which “takes the zing out of it.”

He said he has learned through bit­ter ex­pe­ri­ence to ask ques­tions.

“No healthy adult male should be ane­mic,” he said. “I wish I’d had the fore­sight to ask, ‘ What would cause this?’ ”

Si­pos also wishes he had sought a sec­ond opin­ion sooner. “I was afraid I was go­ing to of­fend her,” he said, re­fer­ring to the first hema­tol­o­gist.

To Ka­mal, Si­pos’s case il­lus­trates the ne­ces­sity for doc­tors to keep look­ing and not brush off what pa­tients tell them. “Don’t just ig­nore neu­ropa­thy, or any symp­toms, in young pa­tients. Even if they’re young, they can have cancer.”

“Don’t just ig­nore neu­ropa­thy, or any symp­toms, in young pa­tients.” Muham­mad Omair Ka­mal, a spe­cial­ist at Loma Linda Univer­sity Med­i­cal Cen­ter

FAM­ILY PHOTO

Jeff Si­pos, an el­e­men­tary school prin­ci­pal, in his kitchen. Si­pos wishes he had sought a sec­ond opin­ion sooner. “I was afraid I was go­ing to of­fend her,” he says, re­fer­ring to the first hema­tol­o­gist.

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