Arq Neuropsiquiat r 2005;63(3-B):741-744
CEREM Lit oral Paulist a, DIR XIX, Depart ment of Healt h f or t he St at e of São Paulo; Depart ment of Neuro l o g y, M edical Facul t y of Universidade M et ropolit ana de Santos (UNIM ES), Sant os SP, Brazil: 1M D, M Sc, PhD; Lect urer in Neuro l o g y, M edical School UNIM ES; 2BSc, Head Nurse; Lect urer of Nursing School UNIM ES
Received 16 December 2004, received in f inal f orm 28 M arch 2005. Accept ed 12 M ay 2005
Dra. Yara D. Fragoso - Rua da Constituição 374 - 11015-470 Santos SP - Brasil. E-mail: yara@bsnet.com.br
DESCRIPTION AND CHARACTERISTICS OF 81
PATIENTS ATTENDING THE REFERENCE CENTER
FOR MULTIPLE SCLEROSIS OF THE COASTAL
REGION OF THE STATE OF SÃO PAULO - BRAZIL
Yara Dadalti Fragoso
1, Ana Patricia Perez Fiore
2ABSTRACT - O b j e c t i v e :t o regist er mult iple sclerosis (MS) pat ient s residing in t he coast al region of the St at e of São Paulo (CEREM Litoral Paulist a). Method: Individual int erviews wit h ident if ied cases of MS. R e s u l t s :
81 individuals w it h diagnosis of MS agreed to come f or regist rat ion (62 f emales [76.5% ], 19 males [23.5% ]). 65% of all pat ient s were resident s of t he cit y of Sant os. The mean age of these patient s was 43 years (14 t o 74 years), and t he Expanded Disabilit y St at us Scale (EDSS) w as ≤5.5 in 76.5% of t he cases. 82.7% of t he assessed pat ient s present ed t he re l a p s i n g / remit t ing f orm of M S. 81.5% of all pat ient s w ere underg o i n g t reat ment w it h immunomodulat ors. Conclusion: Due t o t heir clinical prof ile, pat ient s seem t o come t o CEREM Lit oral Paulist a f or prescript ion of immunomodulat ors. There is a clear need t o ident if y ot her cas-es in t he region and t o allow ot her f orms of t reat ment t o be put int o pract ice.
KEY WORDS: mult iple sclerosis, immunomodulat ors, cit y of Sant os.
Descrição e características dos 81 pacientes em seguimento no Centro de Referência em Esclero s e Múltipla do litoral paulista.
RESUM O - Objetivo: Regist rar os pacient es port adores de esclerose múlt ipla (EM ) resident es na região do lit oral paulist a (CEREM Lit oral Paulist a). Método: Ent revist as individuais com casos ident if icados de EM .
Resultados: 81 indivíduos com diagnóst ico de EM (62 mulheres [76,5% ] e 19 homens [23,5% ]) concord a r a m em comparecer para re g i s t ro no centro. 65% do grupo total de pacientes eram residentes da cidade de S a n t o s . A idade média dest es pacient es era 43 anos (14 a 74 anos), e o Expanded Disabilit y St at us Scale (EDSS) f oi < 5,5 em 76,5% dos casos. 82,7% dos pacientes regist rados apresentava a forma re m i t e n t e - re c o rrent e de E M . 81,5% de t odos os pacient es estava recebendo t rat ament o com imunomoduladore s . Conclusão: Pelo per-f il clínico, os pacientes parecem vir ao CEREM Lit oral Paulist a para prescrição de imunomoduladores. Exist e uma clara necessidade de ident if icar out ros casos na região e permit ir que out ras f ormas de t rat ament o sejam implant adas.
PALAVRAS-CHAVE: esclerose múlt ipla, imunomoduladores, cidade de Sant os.
Pat ient s w it h suspect ed mult iple sclerosis (M S) a re increasingly being re f e rred t o specialized cen-t ers f or diagnosis, cen-t reacen-t mencen-t and f ollow -up1. The
c h ronic and disabling charact eristics of M S are de-t e rminande-t s of subsde-tande-t ial cosde-ts2 , 3, since young
adu-lt s are oft en obliged t o rest rict their economic and social act ivit ies, eit her t emporarily or on a perm a-nent basis. The direct and indirect cost s of M S in-c rease w it h t he severity of t he disease. The advent of immunomodulatory drugs t hat may modif y t he course of t he disease may also have creat ed an ex-tra burden on the budget s of governmental
depar-t mendepar-t s dealing w idepar-th public healdepar-th4. The Consensus
and Guidelines f or prescript ion of t hese immuno-modulators are strict and complex5 , 6, there f o re
cre-ating the need for specialized centers to w hich pa-t ienpa-t s need pa-to be re f e rred for prescrippa-t ions of such drugs.
Specialized centers for M S care have been cre a-t ed in Brazil in a-t he lasa-t decade, and daa-ta re g a rdi n g cli nical charact eri st ics o f p at ient s f rom som e o f t h ese cent ers have b een p ubl ished7 - 1 4. W it h t h e
742 Arq Neuropsiquiat r 2005;63(3-B)
now have descript ive dat a on our pat ient s, w hich w e present here. Our aim is t o add t o our dat a t o t hat of ot her cent ers, in an at t empt t o charact eri-ze M S in Brazil.
Up t o January 2002, all p at ient s suspect ed o f , or diagnosed w it h M S, w ho resided in t he coast al region of t he State of São Paulo, w ere being ref e r-red t o dist ant tert i a ry centers for evaluation. Once t hey w ere evalu at ed at t hese specialized cent ers and immunomodulat ors w ere prescribed, t he pa-t ienpa-t s could conpa-t inue pa-t heir pa-treapa-t menpa-t in pa-the cipa-t y o f their residence, receiving t heir medication from the cit y of Sant os branch of t he St at e Healt h Depart -ment Pharmacy.
In Janu ary 2002, b y req u est f r om t h e St at e Health Depart ment of São Paulo, a new cent er for M S w as creat ed in t he cit y of Sant os, at t ending a t ot al populat ion of 1,500,000 inhabitant s of more t han 20 t ow ns in t he region. This cent er w as set up t hrough an agreement bet w een t he St at e gov-ernment and t he M edical School of Universidade M e t ropolit ana de Sant os (UNIM ES). In M ay 2003 our center was acknowledged as a “ Reference Cen-t er” for M S, in an off icial publicaCen-t ion f rom Cen-t he SCen-ta- Sta-te government. We then starSta-ted the process of i d e n-t if yin g and regisn-t ering all cases of M S, ob serv i n g t heir clinical charact eristics, and det ermining pati-ent s’ needs.
METHOD
By request from the St at e Health Depart ment of São Paulo , all pat ien t s dig an osed as M S w ere re q u i r ed t o regist er in our Ref erence Cent er. Such regist rat ion w as p e rf o rmed by means of a consultat ion w ith clinical eval-uat ion of the pat ient (hist ory taking, clinical and neuro-logical assessment ) and review of exams requested by t h e neurologist s in charge of t hese pat ient s.
We st art ed t h e reg ist r at i o n by callin g all p at i en t s w ho w ere alread y receivin g m ed icat i o n f or M S, fro m t he Sant os branch of the Stat e Health Depart ment Phar-macy. They w ere request ed t o come f or a consult at ion at a set dat e and t ime. All neurolog ist s and neuro s u r-geons of t he region w ere inf orm ed of our cent er, and encouraged t o ref er t heir pat ient s f or regist rat ion. The invit at ion t o pat ient s w as made by t elephone, and t he reasons for this request w ere clearly st at ed t o them and t o t hei r doct ors. Any o t her M S p at ien t s w ho cou ld be ident if ied in t h e reg ion w er e also requ est ed t o co m e f o rw a rd f or regist rat ion. All int erview s t ook place bet -w een August 2003 and April 2004. An associat ion of pa-t ien pa-t s w ipa-t h M S, creapa-t ed apa-t pa-t he sam e pa-t ime, encouraged pat ient s t o come f orw ard f or regist rat ion w it h us.
Up on co llect ing d at a f ro m all cases t hat cam e f o r registrat ion with us, w e perf o rmed a re t rospect ive evalu-at ion of clinical records of t hese pevalu-at ient s.
RESULTS
A t ot al of 83 individuals w ere ident if ied as M S pat ient s (63 females, 20 males). Except f or tw o cas-es, all patients readily agreed to come for the inter-view and r egist rat ion at t he cent er. Of t he t ot al g roup of 83 in divid uals, 65% o f t hem w ere r e s i-dents of the city of Sant os (w hich accounts f or less t han 30% of t he population of t he region). It is in Sant os t hat more t han 90% o f neurologist s and n e u ro s u rgeons of the region live and practice. The f u rther aw ay from Sant os that w e looked, the few -er t he numb-ers of diagnosed cases t h-ere w -ere. A s u m m a ry of the distribut ion of the population and t he d iagnosis o f M S i n t h e coast al r egi on o f t he St ate of São Paulo is show n in the Table. It is possi-ble t hat, despite diagnosis, some patients from t he region may not be regist ered w it h us, t hough all e ff o rt s w ere m ade t o lo cat e all d iagnosed cases during t his period of regist rat ion.
The 81 patient s w ho re g i s t e red w it h us consist -ed o f 62 f em ales (76.5% ) and 19 males (23.5% ). Tw o pat ient s (2.4% ) w ere dark-skinned of Af rican origin, t w o (2.4% ) w ere mulat t os and one (1.2% ) w as of oriental origin. All remaining pat ients w ere Caucasians (94% ). The mean age of t hese 81 pat i-ents w as 43 years (range = 14 to 74 years), and t he Expanded Disabilit ay St at us Scale (EDSS) w as ≤ 5 in 76.5% of cases. 82.7% of all pat ient s pre s e n t e d the re l a p s i n g / remitting form of M S (RRMS). On a v e r-age, p at ient s had had t he d iagnosis o f M S f or 9 years and t he f irst sympt oms appeared, on avera-ge, at t he age of 32 years (range = 14 t o 61). The most f requent and disabling p ersist ent sympt om
Table. Number of inhabitants in the cities of the coastal region of São Paulo, according to the latest census, in comparison to the total number of diagnosed patients with MS in each of these cities.
Cit y Number of Number of “ Prevalence” of inhabit ant s cases of M S M S per 100,000 inhabit ant s
Sant os 417,000 52 12.5
São Vicent e 303,000 10 3.3
Guarujá 265,000 4 1.5
Praia Grande 193,000 9 4.6
Cubat ão 108,000 2 1.9
It anhaém 72,000 2 2.8
Regist ro 54,000 -
-M ongaguá 35,000 1 2.9
Bert ioga 30,000 1 3.3
Jacupiranga 17,000 -
-Ot hers 206,000 -
Arq Neuropsiquiat r 2005;63(3-B) 743
w as f at igue bot h f or men (40% ) an d f or w o men (37.7% ). Current ly, 84.3% of all 83 pat ient s diga-nosed w it h M S in t he coast al region of São Paulo are undergoing t reat ment w it h immunomodula-t ors, immunomodula-t hough only 81 o f immunomodula-t hem are re g i s immunomodula-t e red w iimmunomodula-t h us and t heir clinical dat a can be discussed. The re-maining t w o patients w ho live in t he area but did not com e f or regist rat ion w it h us cont inue t heir t reatment in t ert i a ry specialized cent ers in t he cit y of São Paulo.
The tw o pat ient s w ho ref used t o come f or re g-ist rat ion had t heir immunomodulat ory drugs pre-scr ib ed in an ot her Ref erence Cen t er, and t heir com pliance and sat isf act ion w it h t he t re a t m e n t could not be assessed. Out of t he subgroup of 81 f ully re g i s t e red patient s, w e identif ied 68 individu-als in use of immunomodulators (83.9% of our re g-i s t e red populatg-ion of 81 pat g-ient s). Det ag-iled assess-ment of t his group is present ed below.
Patients in use of immunomodulators – This sub-g roup of M S pat ient s consist ed of 53 w omen and 15 men. The average age w as 41 (range = 14 to 6 5 ) . The total number of consultations with neuro l og i s t s re g a rding initial symptoms that resulted in no diag-nosis of M S had been 103 f or w omen (1.9 c o n s u l-t al-t ions per pal-t ienl-t w il-t houl-t diagnosis) and 19 f or men (1.2 consultations per patient w ithout diagno-sis). A m uch larger number of consult at ions w it h ot her specialt ies that did not result in diagnosis of M S w as identified, but the patients could not specf y the total number ospecf such consultations. At t he t i-me of t he consultation t hat conf iri-med t he diagno-sis, all of t hem received a prescription f or immuno-modulat ors by t heir privat e neurologist s.
The median EDSS score was 3.0 for t his group of patients undergoing treat ment (range = zero t o 8). Alt hough all pat ients in use of immunomodulators had M S di ag nosed accordi ng t o t he crit eria of P o s e r1 5, not all of them had clear indication f or t he
use of immunomodulatory therapy. Regarding gui-delines and recommendations for the use of immu-n o m o d u l a t o r s5, we informed the doctors of the four
pat ient s w ho d id no t f ulf il l t he cri t eri a f or such t reat ment (t w o cases of EDSS ≥7, and t w o cases of elderly patients w ho had not presented any re l a p s-es of M S f or many years). In all f our cass-es, t he doc-t ors agreed doc-t o suspen doc-t he prescripdoc-tion, using only sympt omat ic medicat ion and rehabilit ation.
R e g a rding t heir expect at ions f rom t re a t m e n t w it h im munomodulat ors, o nly 18.9% o f w om en and 33.3% of men expect ed simply a decrease in
t he f requency o f b out s. A much h igher perc e n t-age (47% of all 68 patient s) expected the medicati-ons to complet ely suppress bouts, or to allow them to re t u rn to normal life, to be free of symptoms a n d even to be cured. Possibly as a result of their expec-t aexpec-t ions, only 10.3% of our paexpec-t ienexpec-t s declared expec-t hey w ere t ot ally sat isf ied w it h t he t reat ment .
37.7% of t he w omen and 53.3% of men w ere not part icularly dissat isfied w it h t he f act that they h ad t o b e t reat ed w i t h f requent inject ions. Ho-w e v e r, 47.2% of Ho-w omen and 40% of men claimed t he t reat ment w as painf ul or t roublesome, w hile t he remainder of dissat isf ied pat ient s claimed that t he inject ions w ere f right ening or complicat ed t o use. All pat ients were re q u i red t o inject themselves in t he presence of t he nurse, and 54% of them did not perf o rm t he injections corre c t l y. A similar num-ber o f pat ient s admit t ed t o “ t aking holidays” or “ days off” occasionally, so that t hey could “ have a rest ” f rom t he inject ions.
DISCUSSION
Regist rat ion of our pat ient s show ed t hat diag-nosis of M S w as clearly relat ed to the number and availability of neurologists in the cities of the re g i o n , as w ell as t he presence of healt h-related f acult ies. The cit y of Sant os is t he largest in the area, and it is in Santos t hat Universities are concentrat ed. There is a clear need t o identif y other cases in t he re g i o n and, from early 2005, a special educational pro g r a m is to take place in t his area of the State.
The higher prevalence of t he disease in w omen (rat io f emale: male = 3:1) is similar t o t hat re p o rt -ed by ot her Brazilian aut hors7 - 1 4. The pre d o m i n a
n-ce of Caucasians affected by the disease in our gro-up seems t o be higher t han t hat report ed in ot h-er Ref h-erence Cent h-ers in Brazil7-14.
744 Arq Neuropsiquiat r 2005;63(3-B)
w it h us pref er t o cont inue exclusively f or t he pre-script ion of t he inject ions of immunomodulat ors. Patients from privat e clinics and other hospitals are equally interested in medication, giving little atten-t ion atten-t o rehabiliatten-t aatten-t ion. A special educaatten-t ional p ro-gram is n ow st art ing in order t o m ake t his p oint clear: t reat ment of M S is not exclusively d one by means of inject ions of immunomodulat ors.
Possibly d ue t o unrealist ic exp ect at ions of im-m u n o im-m o d u l a t o r y t reat im-m ent , 89.7% o f pat ient s u n d e rgoing such therapy were not part icularly sat -i sf -ied w -i t h t h e r esul t s. A l t hou gh t hese pat -i ent s may have f ew er or no new relapses of t he disease w it h such t reat ment , t hey may st ill be dissat isf ied because t hey had expect ed much more. Simply by using t he inject ions (and of t en using t hem inade-q uat ely), 53% o f t h e p at i ent s expect ed cure o r complet e supression of relapses.
Resu lt s f ro m ou r Ref eren ce Cen t er, speciall y o rganized to at tend patients residing in the coastal region of t he St at e of São Paulo have brought t o l ight a vari et y o f probl em s amo ng M S pat i ent s. Rather than being exclusively descriptive, re g i s t r a-t ion of paa-t iena-ts has poina-t ed oua-t a-t he difficula-ties w e face in the region and has lead t o the implementa-tion of educaimplementa-tional programs for patients and h e a l t h prof essionals.
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