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Ed Harris – My Story Updated: June 21, 2016 [email protected] My Medical History Diagnosis and Early Symptoms IwasdiagnosedwithCRESTsyndrome(oldnameforlimitedsystemicscleroderma)inJanuaryof 1990,buthadinitialsymptomsforaboutfiveyearsbeforemydiagnosis.Myapproachfromthe onsethasbeentoreadeverythingIcouldfindonthediseaseanddecideonmyowncourseof treatment.(IhadtochangephysiciansbeforeIcouldfindonewhowaswillingtoletmetake chargeofmyowntreatments!) Theconventionalmedicalapproachfortreatingthisdiseaseistowaituntilsymptomsdevelopand thentrytotreatthesymptoms.Thismadenosensetomeatthetimeofdiagnosisandstilldoesnot. EvenbeforeIunderstoodwhatInowdoaboutthedisease,itwasobvioustomethattryingto preventthediseasefromprogressingwouldpotentiallybe“easier”thandealingwithsymptoms aftertheyarose. In1991,Ibegantohavesevereheartburn,whichischaracteristicofCRESTsyndrome.Thisis becausetheloweresophagealsphincterlosesmuscletoneaspartofthedisease,resultinginacid reflux,i.e.,thebackupofstomachacidintotheesophagus,whichiswhatheartburnis.AtthetimeI wastakingProcardia(nifedipine)totreatmyRaynaud’ssymptoms.Myphysicianhadeithernot knownorfailedtomentiontomethatProcardiaandallothersimilarmedicationshaveasideeffect ofreducingtheloweresophagealsphincterpressure,thusincreasingheartburn!Idroppedthe Procardia,whichimprovedtheheartburn,butmyRaynaud’sgotworse.So,backtotheresearch literature...Iquicklydiscoveredthatsomeresearcherswereexperimentingwithadrugcalled YohimbinefortreatingRaynaud’s(thedrughasbeenusedforcenturiesasanaturalaphrodisiac andwasthenmostlyusedtotreatcertainformsofimpotence.).ItturnsoutthatYohimbinedoes nothavethesideeffectproblemthatProcardiadoes,andin1992,Ibegantouseitinsteadof nifedipinewithalmostasgoodaresultsforhelpingmyRaynaud’ssymptoms.Meanwhile,my heartburncontinuedtogetworseandwasnotfullycontrolledbylargedosesofPrilosec (omeprazole).Also,bylate1992Ihadbecomechronicallychilled,tothepointwhereIwaswearing sweatersinthesummerandraisingtheheatinmycarsomuchmyfamilywasconstantly complainingaboutsuffocatingfromtheheat! Search for a Potential Treatment Bylate1992,Iwasstartingtofeelincreasinglyhopelessasmysymptomscontinuedtoprogress. MywifeandIwenttoseethemovie“Lorenzo’sOil”andIleftthemovieinspiredtotrytofinda treatmentapproachthatperhapsmyphysiciansdidn’tknowabout.So,inearly1993,Ibegana 1 muchmorethoroughliteraturesearchtoseeifIcouldgetabetterunderstandingofthedisease processandperhapsdiscoverawaytoeitherstopordelaythenormaldiseaseprogression. However,youhavetothinkabouthowchallengingthiswasin1993.TherewasnoInternetand whileIhadaccesstoamedicalschoollibrarywhereIlived,doingaliteraturesearchmeantstarting withcardcatalogsandultimatelygoingthroughmanyboundvolumesofmultiplerheumatology journalsasafirststep.ThistaskwasveryslowanddifficultandIwasincreasinglyfeelingthatit wasaninsurmountabletask. Then,throughanamazing“payitforward”story,everythingchanged.IwasthefounderandCEOof afairlylargesoftwarecompanybackthenandwasonanairplanetriptoatradeshowthatwewere exhibitingat.Idon’trecallhowthiscameupbutIendeduptalkingwiththepersonsittingnextto meaboutmymedicalsituationandwhatIwastryingtoaccomplishinmyreviewofthemedical literature.ItturnsoutthatthisgentlemanwasalsotheCEOofasoftwarecompany,butinhiscase, hiscompany(SilverPlatter)soldMedline–themedicalsearchcatalog–tomedicallibrariesand researchfacilitiesonCDRomforseveralthousanddollars.Hesaid“tellyouwhat,whenIgetback totheofficenextweek,IwillsendyouafreecopyofMedlinesoyoucanbetterdotheresearchyou aretryingtodo”.HefollowedupandabouttwoweekslaterIreceivedhisincrediblygenerousgift, whichliterallychanged(andprobablysaved)mylife!(Asasidenote,in2014,Itrackedhimdown withgreatdifficultyandtoldhimeverythingthathastranspiredsincebecauseofhisgenerousgift. Hetoldmelaterthathewasreallymovedafterreadingmyemail.) Armedwithavastlyimprovedwaytodomedicalresearch,Ilocatedsomelittleknownresearch studiesfromtheNetherlandsthatshowedaverysignificantfindingaboutsclerodermathatappears tohavebeenmissedbyvirtuallyallsclerodermaresearchersatthattime.Itturnsoutthatin sclerodermapatientswithRaynaud’s,theseresearchershaddiscoveredthattheredbloodcells clumptogetherabnormallyascomparedtoeithernormalpeopleorevenpeoplewithprimary Raynaud’s(samesymptoms,butnounderlyingseriousdisease).Theywereusingatreatment calledplasmapheresis(alsocalledtherapeuticplasmaexchangeorTPE)totreattheRaynaud’s symptoms,andhaddiscoveredthatithadsignificantlyreducedsclerodermarelatedRaynaud’sbut noeffectonprimaryRaynaud’s.Moreimportantly,theTPEtreatmentsresultedinareturnto normalredbloodcellaggregationthatlastedforasignificantamountoftime(3to9months) followingasingleseriesofjustfourweeklyTPEtreatments.Icalledtheresearchersaswellasa clinicianintheNetherlandsandlearnedthattheyhadbeenusingitclinicallythereforawhilewith goodsuccesswithCRESTpatients,butnotasgoodresultswithdiffusesclerodermapatients.With diffusepatients,thistreatmentapproachdidappeartoworkwellinitiallyevenforthemoresevere patients,butonlyiftheTPEtreatmentsweredoneonaweeklybasis.Unfortunately,aweekly treatmentfrequencycannotbesustainedoverthelongtermwithoutsignificantproblemsfrom suppressionoftheimmunesystem.(Thissameproblemisseenincurrenttreatmentapproachesto sclerodermathatdependmostlyontheuseofimmunosuppressantdrugs.)Incontrast,thereduced frequencyoftreatmentsappearedtoworkwellinpatientswithlimitedsclerodermawithoutanyof theimmunosuppressionproblemsseenwithlong-termweeklyTPEtreatments. Thisgavemeaclueastowhatmightbegoingoninscleroderma.Bydoingsignificantlymore literatureresearch,Iwasabletoconvincemyselfthatmost,ifnotall,sclerodermasymptomscould beaccountedforsimplybythepotentiallong-termeffectsofabnormalredbloodcellaggregation. Ironically,atthetimeIdidthis,theideaofprogressivevasculardamageasatriggerforthe developmentofsclerodermasymptomswasnotwidelydiscussedintheresearchliterature,butin thepastfewyears,Ihavenoticedthatthisisnowthegenerallyacceptedtheoryastothe mechanismofdamageinsystemicscleroderma.However,Ihavetothisdatestillnotseenany mentionoftheabnormalredbloodcellaggregationasapotential“firstcause”. 2 Plasmapheresis,bytheway,isasimplebuteffectiveprocedure.Whatisdoneistoinsertone needleintoaveinononearm,andanotherneedleintoaveinontheotherarm.Acontinuousflow machineremovesbloodfromonearm,separatesouttheredandwhitebloodcellsandplatelets fromtheplasmausingacentrifugalseparationmechanism,discardstheplasma,re-mixesthe red/whitebloodcells/plateletswitheithersterilizedalbuminordonatedplasma,andputsthenew mixturebackintheotherarm.Ittakesabouttwohours,ismildlyuncomfortableoncetheygetthe needlesin,andleavesmefeelingtiredforafewhoursaftertreatment.Theusualrationaleforusing plasmapheresistotreatandautoimmunediseaseisthatittemporarilyreducescirculating antibodiesandotherpossiblesubstancesthatmighthavearoleinthedevelopmentofdisease symptoms.However,whilethismaybeafactorintheimprovementsseenfromusingTPEwith sclerodermapatients,Inowbelievethattheresearchsupportsadifferentmechanismofactionthat mayaccountformostoftheseimprovements. Iputtogetheratreatmentplanthatincludedannotatedresearchcitations,submittedittomy physicians,andaftermonthsofeffortactuallymanagedtoconvincemyinsurancecompanytofund aone-yeartrialofthistreatmentapproach.Itisworthnotingthatwhilemyteamofphysicians agreedtotrythissincetheyhadnothingelsetoofferthattheyfeltwouldbeeffectiveinslowing downdiseaseprogression,theydidnotexpectittohaveanybeneficialeffects. IbegantreatmentsinNovember1993.Basedontheresearchresultsfromtheoriginalstudiesand adiscussionwiththeclinicianstryingthisintheNetherlands,Idecidedonaninitialtreatmentcycle thatconsistedofonetreatmentperweekforfourweeks,followedbyatwo-monthlayoff,thenthe cyclerepeats,foratotalof16treatmentsduringthisfirstyear. Initial Results AfteroneyearoftreatmentsmyrefluxwassignificantlyimprovedandwhileIwasstillsomewhat chronicallychilleditwasnotasbadasithadbeenbeforestartingthetreatments.Aftertwoyears oftreatmentsmyrefluxwasundercompletecontrolwithaverylowdoseofPrilosec.And,Iwasno longerchronicallycoldatall.Theseareniceresults,buttheyaresubjective.Ofmuchmore significanceisthefactthatmyhematocritvalue(aquantitativemeasureofthepercentageofred bloodcells),whichhadbeenabnormallylowfortheprevious8years,returnedtoanormalrange followingthefirstroundoffourplasmapheresistreatments.Thiswascompletelyunexpectedsince normallywithplasmapheresistreatmentsyoumayseeaslightdropinhematocritlevelsbecauseof minorbloodlossfromtheprocedure.WhenIfirstsawthissuddenincreaseofhematocritafterthe firstroundoftreatments,Ispeculatedthatthereasonforthismightbebecausetheautomated equipmentusedfordoingbloodcountsmightbecountingsmallclumpsofredbloodcellsasa singlelargeredbloodcellandthatoncetheclumpingwasbrokenup,theredbloodcellcount wouldbemoreaccurate.Iwasfinallyabletoconfirmin2014thatthisisexactlywhathappenswith clumpedredbloodcellsbytalkingwithanengineeratthecompanythatmakesthecellcounting equipmentthatwasinusebackin1993(Beckman-Coulter). Oneothersignificantquantitativemeasurethatsupportedthetheorythattheplasmapheresis treatmentswerestoppingtheprogressionofthediseaseistheresultsofrepeatedpulmonary functiontests(PFT).InJune1994,IhadaninitialPFTthatshowedthatwhilemostmeasures,e.g., lungcapacity,werenormalorabovenormal(Iexercisealot),thegasexchangeabilityofmylungs haddeclinedtoabout68%oftheexpectedvalue.Thisiscommonwithlimitedsclerodermaandifit continuestoreduceovertime,thiscanbeveryserious,ultimatelyleadingtopulmonary hypertension,whichisevennowverydifficulttotreat.Aboutoneyearlater,inMay1995,I repeatedthePFT.Theresultsofthiscriticalmeasurewereunchanged.Atthattime,Iconsidered thistobethebestpossibleresultsincethesechangestothelungsarenormallyirreversibleand 3 progressive.InAugust1997IrepeatedthePFTagain.Again,allothermeasuresexceptgas exchangewerenormalorabovenormal.However,thistimemygasexchangemeasurehad increasedsignificantly,uptoabout76%ofexpected.Thiswasanunexpectedresultandcertainly verygoodnews.Itsuggestedthatmylungswereslowlyhealing.MymostrecentPFTwasdonein December2000.Thistimemygasexchangemeasurehadincreasedto81%oftheexpectedvalue, whichisinthenormalrangeof80%to120%. Evidence for Effectiveness of Treatments - Part 1 In1997Iwasforcedtostopmyplasmapheresistreatmentsagainstmywishes.Whilemyinternist andrheumatologistwerethenbecomingincreasinglyconvincedthatthetreatmentswereeffective atstoppingoratleastdelayingtheprogressionofmylimitedscleroderma,oneofthephysicians whowasinchargeofthetransfusionservicesunitwheremytreatmentswerebeingdonehad alwaysbeenhostiletothetreatments,consideringthemawasteoftimeandthatallofmyso-called improvementswereplaceboeffect.Heconvincedmyphysicianstostopthetreatmentstoseewhat wouldhappen.Theresultswereverysignificant.Aboutsixmonthsaftermytreatmentswere suspended,myheartburnsymptomsstartedtoreturnforthefirsttimeinyears.Thisgavemymain physiciansenough“ammunition”tojustifyputtingmebackonthetreatments.Asbefore,ittook aboutoneyearoftreatmentsfortherefluxtogetunderfullcontrolagain.Whilethisforced vacationfromthetreatmentswasnottomyliking,itactuallyhadamajorimpactonhowmy physiciansviewedtheeffectivenessofthetreatments. [Technicaldigression…Inadditiontobeingacomputerexpert,Iamtrainedasaresearchclinical psychologist.Duringmyresearchtrainingwelearnedhowtocorrectlydesignresearch experiments.Obviouslyasinglecasestudyisneveranymorethansuggestivethatatreatmentmay beeffectiveinthegeneralpopulation.However,thereisasinglesubjectresearchdesignthatcan beusedtoestablishthatanyeffectsseenafteratreatmentisinitiatedislikelytobefromthe treatmentandnotjustcoincidence.Basically,ifyoutakeabaselinemeasureofacondition,adda treatmentandgetachangeinthatmeasure,thenremovethetreatmentandseeareturntothe baselinelevel,andthenre-introducethetreatmentandgetthesamechangeinthemeasure,thereis averysignificantlikelihoodthatthetreatmentcausedthechange.ThisiscalledanABABReversal designandiswellestablishedasavalidresearchtechniqueinsingle-subjectresearchdesign.And thisisexactlywhathappenedwhenmytreatmentswereremovedandthenreintroduced.] Evidence for Effectiveness of Treatments - Part 2 In2003,thingswerecontinuingtogoextremelywell.Ihadnoresidualsymptomsotherthanvery mildRaynaud'sandslightrefluxcompletelycontrolledbylowdosesofPrilosec(seenotebelow). SoIdecidedtoseeifIcouldcutbackthefrequencyoftreatmentsabit.Ihadhistoricallyfolloweda treatmentcycleofoneonetreatmentperweekforfourweeks,followedbyalayoffalternating between8and9weeks,soastoaverageatwo-monthgapbetweentreatmentrounds.Idecidedto graduallyincreasetheintervalbetweentreatmentrounds.Istartedbydoingtwotreatmentrounds witha9-weekgapwithnoproblems.Thiswasfollowedbytwotreatmentroundswitha10-week gap.ThisalsoseemedtogowellsoIincreasedthegapagain,to11weeks.However,thistimemy heartburnsymptomsbegantore-occuragain.Iimmediatelywentbacktotheoriginaltreatment cycle,andaftertwotreatmentcycleswithan8-weekgap,myheartburnsymptomsagain disappeared.SincethenIhavestayedattheoriginaltwo-monthtreatmentgapwithnoother problems. Onimportantsidenoteonprotonpumpinhibitors(PPIs)suchasPrilosec(omeprazole):althoughI hadnoheartburnsymptomswithfairlylowdosesofPrilosec,wheneverIstoppedthemedicineI startedtohaveheartburnsymptomsagain.Iassumedthattherewasstillsomeresidualweakening 4 oftheloweresophagealsphincterevenwiththecontinuedplasmapheresistreatments.However,it turnsoutthatwasnotthecaseatall.Mywife,whoisaphysician,developedanulcerandhadtogo onPrilosecaswell.AsitisnowknownthatmostcasesofulcersarecausedbyanH.Pyloriinfection andthatappropriateantibiotictreatmentscancompletelycuretheunderlyingcauseoftheulcers, shereceivedacourseoftreatmentsthatcompletelycuredherulcers.However,shewasalso unabletostopPrilosecwithoutherheartburnsymptomsreturning!Shedidsomeresearchand discoveredthatmedicinessuchasPrilosecactuallycreatetheirownneed!Apparently,ifyouput healthyvolunteerswithnoheartburnsymptomsonPrilosecandthenstopit,theparticipantsinthe studyactuallydevelopreboundheartburnsymptoms!Armedwiththisknowledge,weboth decidedtotaperoffPrilosecVERYslowlyoverathree-monthperiodbackin2006.Thisworked andneitherofushaveeverneededtogobackonPrilosecagain.Iamcompletelyfreeofany heartburnproblemswithnomedications.ItisverylikelythatIcouldhavestoppedthePrilosec backin1996hadIknownthis. The Present Day… AsIeditthisarticle(February2016),Iamnow68andinexcellenthealthwiththeonlyremaining scleroderma-relatedsymptombeingverymildRaynaud’s(whichissayingsomethingbecauseIlive inMadison,WIanddealwithverycoldwinters)!Accordingtomyinternist,Iamthehealthiest patientinmyagebracketinhisentirepractice,inspiteofthefactthatIstillhaveaformaldiagnosis oflimitedsystemicscleroderma.Iamveryactive(Iplaytenniseverydayandalsoworkoutonmy ellipticaleverydayaswell).Medicarecontinuestopayformymedicaltreatmentswithoutany problemsandthecurrentplanistostayonatreatmentfrequencyof16treatmentsperyear indefinitely,oruntilamoreeffectivetreatmentbecomesavailable.Todate,Ihavehadmorethan 350plasmapheresistreatmentswithveryfewissuesorproblems.Theonlyexceptionwasone incidentthatarosefromdefectivetubingthatleadtosomebloodlossthatwaseasilydealtwith. ThisisnowaroutineprocedurethatIgothrough16timesayear.Iconsiderthisaverysmallprice topayforbeinginexcellenthealthmorethan30yearsafterfirstdevelopingsymptomsoflimited systemicscleroderma. The Rest of My Story The Scleroderma Education Project In1995,astheInternetwasinitsinfancy,Isearchedanddiscoveredthattherewasessentiallyno informationavailableonlineforsclerodermapatientsthatcouldbereadilyunderstoodbysomeone withoutamedicalbackground.IwasabletoobtainanupdatetotheMedlineCDROMlibrarythatI hadoriginallyreceivedin1993,reviewedallnewscleroderma-relatedstudiessince1993,and wroteandpublishedonlinethefirsteditionofapatienteducationdocumentonsclerodermatitled theSclerodermaFAQ.ItwontwopatienteducationawardsandwastranslatedintoSpanishover thenextfewyears.ThelastmajorupdatetotheSclerodermaFAQwasdonein2009sincegood patienteducationinformationwasfinallybecomingavailablefromanumberofsourcesincluding theSclerodermaFoundation,theMayoClinic,andJohnsHopkinsUniversity. InOctober2013,IdecidedtodoanupdatetotheSclerodermaFAQasaprequeltoupdatingthe Wikipediaarticleonscleroderma,whichisnotverygood.Afterreviewingthemajorscleroderma educationalwebsites,Irealizedthattherewasahugeamountofimportantinformationthatwas notbeingincludedonanyexistingwebsite.Alotofthisinformationwasfairlytechnicalbutalso importantforpeoplethatwantedtolearnasmuchaboutthediseaseaspossibleinordertowork 5 moreeffectivelywiththeirdoctors.IdecidedtocompletelyrewritetheSclerodermaFAQinorder totrytoproduceacomprehensivedocumentthatwouldbridgethegapbetweentechnicaland medicaljargonfoundinresearchpapersbyexplainingeverythinginawaythatpatientswithouta medicalbackgroundcouldunderstand. ThefirstdraftofthecompletelyrewrittenSclerodermaFAQwascompletedinlate2013afterabout 200hoursofresearchandwriting.ThisinitialdraftoftheupdatedFAQwas34pageslong(the existingversionwasabout8pageslong).OverthenextsevenmonthstheFAQwentthroughmany revisionsbasedonexcellentfeedbackfromtheSclerodermaFoundationandseveralphysicians, includingonewell-respectedsclerodermaexpert/researcher. InApril2014IresearchedandwroteacompaniondocumenttotheFAQtitledtheGuideforNew andFuturePatients.Ialsorealizedatthispointthatinadditiontotheseinitialtwodocuments, therewereanumberofotherarticlesthatIwantedtowrite,includingadditionalpatienteducation documentsbutalsoaseriesofTechnicalArticlesthatwouldcovercertaintopicsinmoredetailand includeselectiveresearchcitations.Someofthesetechnicalarticleswouldbetargetedat physiciansandresearchersinadditiontomoresophisticatedpatients.Asaresult,inMay2014,I madethedecisiontolaunchacompletenewwebsitecalledtheSclerodermaEducationProject whichwouldhouseallofthesedocuments.Thenewwebsite,locatedatSclerodermaInfo.org,was launchedinJuly2014. TheSclerodermaEducationProjectwebsiteisnotmeanttoreplaceorcompetewithexcellent websitesprovidedbytheSclerodermaFoundation,theMayoClinic,JohnsHopkins,etc.Itprovides additionalandmoredetailedinformationaboutavarietyoftopicsthatareusuallynotcoveredon thesewebsites,forexample,detaileddiscussionsonANAandantibodytesting,informationonthe new2013Guidelinesforsclerodermadiagnosis,etc.,alwayswrittenatalevelthatpeoplewithout medicaltrainingcanunderstand.Mybeliefisthateducatedpatientsarebetterabletoworkwith theirteamofphysicianstomakethebestpossibledecisionsabouttheirownhealthcare. Update3/10/16:TheSclerodermaEducationProjectLtdisnowa501(c)(3)taxexempt organization.ThismeansthatanydonationsarenowtaxdeductibleintheUS. Scleroderma Patient Advocate OncetheoriginalversionoftheSclerodermaFAQwaspublishedonlinein1996,Ialmost immediatelybegantoreceiveoccasionalemailswithfollow-upquestionsaboutsclerodermafrom patientsaroundtheworld(myemailaddresswasincludedintheFAQ).Forthemostpart,these typesofquerieswerelimitedtoafewtimesamonthpriortothelaunchofthenewwebsite. AfterthenewSclerodermaEducationProjectwebsitewaslaunchedinJuly2014,Ijoinedand becameaveryactivememberofabout12scleroderma-relatedpatientsupportgroupsonFacebook aswellastheInspiregrouprunbytheSclerodermaFoundation.Asaresult,overthelastyearand ahalfmypatientsupportactivitieshaveincreasedsignificantlyandInowspendatleastacoupleof hourseachdayworkingwithpatientsboththroughsupportforumsaswellasonanindividual basis.IalwaysmakesurethatpatientsknowthatIamnotaphysicianandcannotoffermedical advice.However,Iamoftenabletoprovideinformationtosclerodermapatientsthatcanbeuseful inhelpingthemtoworkmoreeffectivelywiththeirteamofphysiciansingettingquicklyand accuratelydiagnosedaswellastomakebetter,moreinformeddecisionsontheirownindividual healthcare. 6 Scleroderma Researcher Inearly1993,afterreviewingalloftheresearchliteratureonsclerodermathatIcouldfind,I developedanewhypothesisthatsclerodermasymptomsmaydevelopbeginningwithdamageto thevascularsystemthatisadirectconsequenceofredbloodcellsbeingabnormallyclumped together(hyperaggregation).Ifthishypothesisiscorrect(anditcaneasilybetestedbyresearch), thenANYtreatmentthatcanbreakaparttheclumpedredbloodcellsandkeepthemdisaggregated shouldpreventanyfuturesymptomdevelopmentandallowthebodytopartiallyorfullyhealover time,totheextentthatthisispossible.Obviously,ifstartedlateinthediseaseprocess,complete reversalofdamagewillnotbepossible.Plasmapheresistreatmentshavebeenshowninanumber ofresearchstudiestobeeffectiveindisaggregatingredbloodcellsandreturningoverallblood viscositytonormallevelsinsclerodermapatients.Therearealsoseveraldrugsthatmayhave potentialaswell,buthavenotyetbeentestedasatreatmentforscleroderma.IntheAdditional ArticlessectionoftheSclerodermaEducationProjectwebsite,thereisatechnicalarticletitled “SclerodermaHyperviscosity:ImplicationsforTreatmentandResearch”thatexploressomeofthe researchbehindthisproposednewdiseasemodel. Inanefforttoadvancetheresearchtotestoutthispotentialnewdiseasemodelandtreatment approach,Iamnowworkingwithseveralresearchersonaseriesofresearchpapersand presentations.Thefirstofthese,areportonmyownverylongterm(22plusyear)experienceasa sclerodermapatientreceivingregularplasmapheresistreatments,waspresentedasaposterata medicalconferenceinOctober2015(Ididthepresentation)andtheabstractispublishedinthe September2015specialissueoftheresearchjournalTransfusion.Thetitleofthatabstractis “BenefitofLong-TermTherapeuticPlasmaExchangeTreatmentinaPatientwithCRESTSyndrome (LimitedSystemicScleroderma):A21-yearSuccessStory”.ThenewsorganizationScleroderma Newsdidagreatstoryonthiscasereport: http://sclerodermanews.com/2015/12/03/scleroderma-patient-long-term-remission-tpetreatment/. Update5/27/16:Anexpandedversionofthecasereporttitled“SuccessfulLong-Term(22Year) TreatmentofLimitedSclerodermaUsingTherapeuticPlasmaExchange:IsBloodRheologythe Key?”wasjustacceptedforpublicationintheresearchjournalClinicalHemorheologyand Microcirculation.Themanuscriptversionofthiscasereportcanbefreelydownloadedhere: http://www.sclerodermainfo.org/pdf/CHM-Case-Report-Manuscript-US.pdf. Ipresentedasecondposter,titled“TherapeuticPlasmaExchangefortheTreatmentofSystemic Scleroderma:AComprehensiveReviewandAnalysis”,attheAmericanSocietyforApheresis2016 annualconferenceinMay2016.TheabstractofthisposterispublishedintheJournalofClinical Apheresis.Thisisacomprehensivereviewarticlethatexaminesalloftheresearch(whichis actuallyquiteextensive)donetodateontheuseofplasmapheresis/therapeuticplasmaexchange totreatpatientswithsystemicscleroderma.Hereisalinktotheanextendedversionoftheposter thatwashandedoutattheASFAconference:http://sclerodermainfo.org/pdf/ASFA-Handout.pdf.A fullversionofthisposteriscurrentlyinpreparationforjournalsubmissionthissummer. Ijustsubmittedanabstracttitled"TherapeuticPlasmaExchangefortheTreatmentofSystemic Scleroderma:AComprehensiveReviewandAnalysis"totheAmericanCollegeofRheumatology meeinttobeheldNovember2016inWashington,DC. Thefourthpaper,tentativelytitled“SclerodermaBloodRheology:ImplicationsforTreatmentand Research”,willbeanexpandedandupdatedversionofthehyperviscositytechnicalarticleonmy 7 website.Itwillexaminealloftheresearchthatdiscussesabnormalbloodrheologyinscleroderma patients,includingredbloodcellhyperaggregation,andlookathowthismightleadtothe developmentofsclerodermasymptomsaswellasexploringpotentialoptionsfornewtreatmentsif thisdiseasemodelisproventobecorrectbysuitableresearch. Asanon-physician,ithasbeenamajorchallengeformetogetacceptedassomeonewhomay actuallybeabletohelptoadvancesclerodermaeducationandresearch.Iamnowfortunate enoughtobeworkingcloselywithseveralresearchersthatnowagreethatmyproposednew diseasemodelmaywellbecorrectanddefinitelyneedstobestudiedthroughappropriateresearch. Thebottomlineisthis:ifmyproposednewdiseasemodelsturnsouttobewrong,thensome researchmoneywillhavebeenwasted(whichhappensallthetime);however,ifitturnsouttobe correct,itmayquicklyleadtonewtreatmentapproachesforsclerodermathatcouldbemore effectiveandlesstoxicthancurrent,largelyineffective,treatmentapproachesthatareheavily focusedonusingimmunosuppressantdrugstosuppresstheimmunesystem,frequentlyleadingto majorsideeffectsandcomplicationsfromlong-termusage. About Me AfewyearsagoI“retired”fromalongcareerasacomputersoftwaredeveloper(since1964)and successfulserialentrepreneur.Iamalsotrainedasaresearchclinicalpsychologist.Iammarried toaphysician(whichhasbeencriticalinmyjourneyfrompatienttoresearcher).Iamaskilled amateurmusician(clarinetandbassoon)andplayinalocalcommunitybandandorchestra. Directlyrelatedtomyinterestinmusic,Iamalsointhemiddleoftrainingtobeacomposerof classicalmusicfororchestra,band,andchambergroups.(Ihavecompletedthreecompositionsand wouldbehappytosendlinksifanyonewouldliketohearthem.J).Mymainhobbiesaretennis(I playatleastonceaday)andcooking. ©Copyright2000–2016.EdwardS.HarrisAllRightsReserved 8