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Shah's Spleen 2011

Shah's Spleen 2011

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02/04/2013

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HISTORY
The Shah’s Spleen: Its Impact on History
Leon Morgenstern,
MD
,
FACS
The spleen had never before played a major role in shapinghuman history. Failing hearts, damaged brains, or organspiercedbyassassinsbulletshadalloccupiedcenterstageintheir time. In 1979, a diseased spleen provoked a seriousconflict between The United States and the newly formedIslamicRepublicofIran,reverberationsofwhicharestillinplay today. In the dramatic evolution of that conflict many other countries played lesser but significant roles. Medicalprofessionalism was challenged in a multinational arena,involving dozens of doctors. In the central role of a haplesspatient in need of urgent surgical treatment was the re-cently deposed Shah of Iran, Mohammad Reza ShahPahlavi.Mohammad Reza Pahlavi became the Shah of Iran inSeptember 1941 at the age of 22, after the forced abdica-tion of his father, Reza Pahlavi, founder of the Pahlavidynasty. Unlike his father, who had a strong, despotic de-meanor, the new Shah assumed the role of a benevolentdespot, whose goals were to modernize the country andguide it into the 20th century. Within 10 years into hisreign, the oil industry was nationalized, education was up-graded by building schools, libraries, and institutions of higher learning, and women’s suffrage was instituted. Thecountry’s infrastructure was strengthened and the plight of the Iranian people was unquestionably improved.
1
Politically the Shah was decidedly pro-Western in inter-national relations. Physically he was handsome, urbane,andoutwardlyahealthyandactiveindividual,twiceescap-ing assassination attempts and twice surviving crashes inaircrafthewaspiloting.Earlyinhisreignitseemedthathismonarchy would fare well under him. But the Middle Eastis noted for its constantly seething discontent, and Islamicextremists continuously challenged the stability of hisreign. In October 1971, undeterred by the gatheringstorm, the Shah put on a lavish celebration at the ruins of ancient Persepolis, marking the 2500
th
anniversary of thePersianEmpire.Ifanything,thepompousscaleandextrav-agant cost of the event did nothing but exacerbate thegrowing threat to his rule.In early 1974, The Shah’s spleen entered the picture. WhilevacationingontheisleofKishinthePersianGulfhenoticed an unusual swelling in his left upper abdomen. Hehad been water-skiing (a favorite activity) and the swelling was probably rendered more prominent in the posture as-sociated with that sport. Back in Tehran, his physiciansdetermined that the swelling was an enlarged spleen andsummoned urgent consultation with the French hematol-ogist Dr Jean Bernard, well known for his expertise in he-matologic disorders, particularly lymphoma. Dr Bernardchose to take with him his young pupil, Dr GeorgesFlandrin. So began the keeping of a secret that remained well guarded for the next several years. Even the invitation wasdeceptive.Theywereinvitedostensiblynottoexaminethe Shah, but one of his aides. On their arrival it was madeclear that the patient was the Shah himself.The French doctors examined the Shah and confirmedthepresenceofsplenomegaly.Theyalsodetectedsomecer-vical lymph node enlargement. Blood studies, includingbone marrow, established the diagnosis of chronic lym-phatic leukemia. Although the doctors recommended acervical node biopsy for a tissue diagnosis as well, the Shahbalked at the idea of a surgical procedure. Anxious to sparethe Shah from the anxiety likely to be induced by the word
cancer 
,theShah’spersonalphysicianconvincedthevisitingFrench doctors to tell the Shah that he had Waldenstrom’sdisease.” The Shah accepted this diagnosis and onllearned of his true diagnosis several years later. The Shah,his doctors, and his personal aide all agreed that there wasto be no disclosure of any information even suggestingmalignancytoanyone.TheShah’sconditionwastobekepta well-guarded secret from everyone, even his wife, theQueen Farah. She was to learn of the diagnosis, to herdismay, several years later.The French consultants initially recommended observa-tiononly.Severalmonthslatertheyprescribedaregimenof chlorambucil to keep the disease in check. Although headhered to the chemotherapeutic regimen initially, theShah proved to be an inconsistent and noncompliant pa-tient. On one occasion he even took the wrong medicationasaresultofaprescriptionrefillerror,alsoaconsequenceof the conspiracy to keep the diagnosis secret. In 1975, thedoctorswerecalledagaintomeettheShahinZurich,Swit-zerland.Theretheyfoundthatthespleenwasenlargingandrecommended that the dosage of chlorambucil be in-creased. They also advised that the Shah desist from phys-icalactivitiessuchasskiing,asporthewasinveteratelyfond
Disclosure Information: Nothing to disclose.
Received September 2, 2010; Accepted October 27, 2010.From the Department of Surgery, David Geffen School of Medicine at Uni-versity of California, Los Angeles, Los Angeles, CA.Correspondenceaddress:LeonMorgenstern,MD,FACS,Cedars-SinaiMed-ical Center, 8700 Becker Blvd, Becker Building - Suite 216, Los Angeles, CA 90048.
260
© 2011 by the American College of Surgeons ISSN 1072-7515/11/$36.00Published by Elsevier Inc. doi:10.1016/j.jamcollsurg.2010.10.014
 
of.They insisted that his condition be monitored by morefrequentbloodcountsandexaminations.DrBernard,thenaged 67, designated this task to his younger colleague, DrFlandrin.SobeganatravelingroutineforDrFlandrin,wellcloaked in secrecy, which would involve his seeing andexamining the Shah every 5 or 6 weeks as well as collectingblood samples for examination in France. By the time theShahleftTehranin1979,Flandrinhadmade35tripstoseehispatient,aroundtripdistanceofmorethan5,000miles,equivalent to traveling 7½ times around the world. It wasonly the beginning of his later transcontinental journeys tofollow the Shah in the turbulent odyssey that was to ensue.In 1977, Queen Farah was informed by Drs Bernard andFlandrin of the true diagnosis of chronic lymphatic leuke-mia, but the Shah himself still thought he had Walden-strom’s “disease.”The political situation in Iran had begun to deteriorateseriously in 1978 with increasing popular sentimentagainst the monarchy, fueled by the rise of Islamic extrem-ism.TheShah’sbenevolentdespotismhadalsobecomelessbenevolent and his secret police force, “Savak,” was fearedand despised. As the position of the Shah weakened, heostensiblyreceivedassurancesfromUSofficialsourcesthathewouldbewelcomedintheUnitedStatesifhehadtofleeIran. In 1979 he did indeed have to flee, in the wake of aviolentwaveofstrikesandriots,againstwhichherefusedtouse military force. The year 1979 was then to be a calami-tous year in which he would seek refuge in 7 countries,suffer constantly declining health, undergo 4 operations,and experience rejection by both governments and doctorsinamaelstromofcomplexpoliticalandmedicaldecisions.
2
On January 16, 1979, encouraged by the United Statesgovernment, the Shah and his entourage departed TehraninaBoeing707piloted bytheShah himself.At thetimeof their departure the Shah clung to 2 unrealistic assump-tions. One was that the departure was for a temporary “vacation” and that he would be recalled by popular senti-mentashehadbeenafterasimilarcoupin1959.Theother was that his invitation to come to the United States was afirm commitment that he could rely on. Delaying his tripto the United States, he chose instead to accept invitationsfrom President Anwar Sadat of Egypt and King Hassan IIof Morocco to visit their countries. He first stopped in Aswan, Egypt, where he received a hospitable and fittingly royal welcome from Anwar Sadat and his wife Jehan. In Aswan, Dr Flandrin visited the Shah for his regular exam-ination and laboratory studies. The visit to Egypt was ashort one and was followed by a visit to Marrakech, Mo-rocco, where he enjoyed the hospitality of King Hassan II.There he was again visited by Dr Flandrin, faithfully keep-ing vigil on his peripatetic patient.This idyllic stay in Mo-roccoendedsomewhatabruptlyandunpleasantlyaftersev-eral weeks, when the government of Morocco decided(with encouragement from the government of France) torequest the Shah to leave.The sudden need to depart Morocco posed an urgentand unexpected problem.
3
Events in Iran had gone frombad to worse. On February 14, 1979, Valentine’s Day, amobhadbrokenintotheAmericanEmbassyinTehranandcommitted mayhem. By the time they were dispersed, an American marine sergeant was missing and the embassy staff was severely shaken. This development changed theattitude of the United States government, then under Pres-ident Jimmy Carter. The Shah was told, to his great con-sternation,thathisplannedsettlementintheUnitedStates was not “convenient” at the time, considering the unstablesituation inTehran. So the Shah was left as a man withouta country. He was refused safe haven in Switzerland,France, Italy, England, Jordan, and other countries thatmight have been suitable choices for him. Only Paraguay and South Africa indicated their willingness to take him,but they were not acceptable to the Shah. Declining health was also an issue. Photographs of the Shah in Egypt andMorocco showed him appearing increasingly gaunt.The Shah’s twin sister, Princess Ashraf, came up with atenable solution by suggesting a temporary haven in theBahamas, a Caribbean island nation with a salubrious cli-mate and a relatively safe environment. To facilitate thismove, the Shah’s friend, Henry Kissinger, the former USSecretary of State, flew to the Bahamas to negotiate withthe Prime Minister there. The Bahamian governmentagreedtoissueavisatotheShah,butonlyontheconditionthat he stay on Paradise Island, a small island adjacent toNassau,whereasmallbeachhousewasavailable.Whenthenegotiations were complete, The Shah left Morocco onMarch 30, 1979, for his next refuge, a very small beachcottage on a tiny island in the Atlantic, lodging decidedly unfit for a king.The 10-week stay in the Bahamas was an ordeal for theShah and his wife Farah. The quarters were cramped, thehumid climate uncomfortable, and the isolation boring.The Shah was depressed by the increasingly horrible newsfrom Iran, the unaccustomed nonregal existence, and per-haps most important of all, his worsening health. He haddiscovered an enlarged lymph node in his neck that occa-sioned another visit by Flandrin, who aspirated the nodeand sampled the Shah’s bone marrow. The news was notgood. The presence of immunoblasts indicated a transfor-mation of the leukemia from a chronic, less threateningstage to an acute form that required more aggressive treat-ment, including radiation and possibly surgery for thesplenomegaly. The chemotherapy was changed from
261
Vol. 212, No. 2, February 2011
Morgenstern
The Shah’s Spleen: Its Impact on History
 
chlorambucil to the stronger drug combination of vincris-tine, procarbazine, nitrogen mustard, and prednisone. Be-cause this was given by weekly infusion, more visits by Flandrin were necessary. He advised the Shah that it wastime for him to be hospitalized for more intensive diagnos-tic and therapeutic measures. That would have meant re-vealing the long kept secret of the Shah’s illness. Althoughthe Shah agreed that it might eventually be necessary, hebelievedthatitwasneitherthetimenortheplaceforthattobe done.Meanwhile,intenseeffortswerebeingmadeinWashing-tontofindthenexthavenforthePahlavisbecausetheirstay in the Bahamas was limited by their expiring visa and thelackofsuitablemedicalfacilities.InvolvedintheseeffortswereHenry Kissinger, the former secretary of state, Zbigniew Brzezinski, the national security advisor, President Jimmy Carter, Hamilton Jordan, the President’s chief of staff, andDavid Rockefeller, a long time friend of the Shah. TheUnited States was still out of the question considering thestate of affairs in Iran and the precarious status of the re-maining Americans there. Kissinger finally made a crucialpersonalcalltothePresidentofMexicowithapleatoadmitthe Shah to that country. Kissinger’s request was receivedfavorably and it was a solution that the Shah found accept-able. With only 2 days remaining before the expiration of the Bahamian visa, Mexico granted him a 6-month touristvisa.In Mexico, a suitable lodging was found in Cuernavaca,acity30mileswestofthecapital,knownforitsbeautyandpleasant climate. A house called the Villa of Roses, moresuitable to the royal taste, was available there and the Shah,Queen Farah, their Great Dane dogs, and their retinue of servants settled in comfortably for a several-month stay. In Washington the debate continued about the feasibility of allowing the Shah’s entry into the US, a promise that hadbeen graciously made previously but then rudely broken.That debate came to a head only 4 weeks after their arrivalin Mexico, when the Shah’s health took a drastic turn for the worse.Flandrinvisitedhimseveraltimesandadjustedthedoseof chemotherapy.Mexicandoctors,calledinfortheShah’sonsetof fever and jaundice, diagnosed malaria. At this point thedecision was made to call in an American consultant,given the increasing complexity of the medical problems. With the advice of the David Rockefeller staff, the physi-cian chosen was a New York internist, Dr Benjamin HKean, well known for his prominent Park Avenue practiceand his subspecialty in tropical diseases.
4
In late September1979, Kean arrived to visit and examine the Shah. He wasastounded by what he found. As he put it, “the Shah hadbecome a sad, shrunken figure.” He was deeply jaundicedand obviously cachectic. Kean quickly excluded the diag-nosisofmalariaandinsteadsuspectedobstructivejaundice,possibly due to bile duct obstruction, but when he wishedtohavesomebloodsamplestakentheShahrefusedwithouttheconcurrenceof his “French doctors.” “Flabbergasted”at the mention of “French” doctors that no one had evermentionedtohimbefore,DrKeanwithdrewfromthecaseas diplomatically as he could and flew back to New York. Again the Queen called Dr Flandrin to come to Mexico. When Flandrin arrived, one glance at the Shah told himthat the Shah had to be hospitalized. At the time, the Shah wasadamantinhisrefusaltoconsidertheUnitedStates,sopiquedwasheatAmericanbehaviortowardhim.AgainDrKeanwassummonedtoCuernavaca,thistimetomeetwithDr Flandrin. Dr Flandrin disclosed to him that the Shahhad been known to have lymphatic cancer for 5 years buthadconsistentlyrefusedaproperworkup.ThiswasthefirsttimethatKeanlearnedofthediagnosisoflymphoma.Bothhe and Flandrin agreed on the need for the proper imagingstudies that were available in nearby Mexico City, but theShah balked at such a move because of the language diffi-cultiesandsomedoubtsabouthospitalarrangementsthere.DrKeansaidthathefavoredanAmericanhospitalandthatthere were at least 7 cities in the US with world-class med-ical centers where such studies could be done.Though theShah was embittered by what he considered his betrayal by the United States he reluctantly agreed. Dr Kean’s choice wasNewYorkHospitalinNewYorkCity,wherehewasonstaff, knew the doctors, and was well acquainted with thefacilities. He suggested that the process for moving theShah to New York begin immediately. Dr Flandrin, origi-nally in favor of hospitalizing the Shah in Mexico, agreedthat the facilities in the US would be better. He then with-drew from any further care of the Shah, a duty he hadperformed faithfully and at great effort for the past 5 years.Dr Kean now assumed the role of the Shah’s personal phy-sician. He made the request through government channelsthat the Shah be admitted without delay to the US forhospitalization at New York Hospital and awaited permis-sion to see this accomplished.The news that the Shah was suffering from cancer, thathe needed urgent treatment, and that Kean had recom-mended his immediate admission to NewYork Hospital inthe United States hit Washington like a bombshell. ThesituationinTehranhaddeterioratedevenfurther.ThePres-ident and key members of his staff were still divided on theadvisability of admitting the Shah to the US for fear of provoking even more anti-American violence in Iran. Pres-ident Carter was especially fearful of such a consequence,but finally conceded that for humanitarian reasons the UScould no longer refuse the Shah admission. Permission wasgrantedforadmissiontoNewYorkHospital.Whateverthe
262
Morgenstern
The Shah’s Spleen: Its Impact on History
J Am Coll Surg 

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