Ingxaki engaqhelekanga echaphazela ingqondo: Ngaba uyayazi i-PML? (Progressive Multifocal Leukoencephalopathy - PML)

Ngaba wakha weva ngemeko ebizwa ngokuba yi-PML (Progressive Multifocal Leukoencephalopathy)? Usenokuba awuqhelananga neli gama. Isizathu kukuba i-PML lusulelo olungaqhelekanga oluchaphazela ingqondo yethu. Okwenzekayo koku kukuba iiseli ezivelisa i-myelin, isigqubuthelo esikhuselayo esijikeleze iiseli zemithambo-luvo engqondweni yethu, ziyatshatyalaliswa kancinci kancinci. I-Myelin yinto efana neoyile. Oku kukukhusela ii-neurons kwingqondo nakwi-spinal cord (inkqubo yethu yemithambo-luvo ephakathi). Ngoko ke xa le myelin ilahlekile, into emhlophe engqondweni iyonakala.

Lithetha ukuthini igama elithi PML?

Ngoku makhe sibone ukuba oonobumba abakwigama elithi PML bathetha ukuthini. Ukuqonda oku kuya kukunika umbono ocacileyo ngesi sifo.

• P — Inkqubela phambili: Oku kuthetha ukuba esi sifo siya sisiba mandundu kancinci kancinci. Oku kuthetha ukuba iimpawu zinokwanda ngokuhamba kwexesha.
• M — Multifocal: Oku kuthetha ukuba esi sifo asiphelelanga kwindawo enye yobuchopho, kodwa sichaphazela iindawo ezininzi . Kufana nokuba kukho izilonda ezincinci kwiindawo ezininzi zobuchopho.
• L — I-Leukoencephalopathy: Eli gama livakala linzima kancinci, akunjalo? Ngamafutshane, lithetha ukuba esi sifo sichaphazela kakhulu i-myelin, okanye into emhlophe, engqondweni .

Ngoko ke, i-PML ibangela umonakalo engqondweni. Lo monakalo unokukhokelela kwiingxaki ezahlukeneyo zemithambo-luvo. Umzekelo, ukukhubazeka kwengqondo, iingxaki zokubona, kunye nobunzima bokuhamba. Ngenxa yokuba i-PML sisifo esiqhubekayo, inokuba mandundu ngokuhamba kwexesha. Nangona kunjalo, kwabanye abantu, unyango lwangethuba lunokucothisa ukuqhubela phambili kwesi sifo.

Ziziphi iimpawu zokuqala ze-PML?

Kwabaninzi abantu, iimpawu ze-PML ziqala kancinci kakhulu. Kwakhona, iimpawu zinokwahluka ngokuxhomekeke ekubeni leliphi icala lobuchopho elichaphazelekileyo sisifo. Cinga nje xa isebe lomthi lisifa, yonke eminye imisebenzi ekwelo sebe iyayeka ukusebenza.

Iimpawu zokuqala ze-PML zingabandakanya:

• Ukungakhathali okanye ukungabikho kolungelelwaniso
• Ubunzima bokuthetha okanye bokucinga
• Ukuziva ubuthathaka emzimbeni

Njengoko usulelo luqhubeka, unokufumana iimpawu ezifana nezi:

• I-Dementia, ukulahleka kwenkumbulo
• Ukulahleka kwentetho
• Ukulahleka kombono
• Utshintsho kwindlela yokuziphatha kunye nobuntu

Abanye abantu banokuba neentloko ezibuhlungu okanye ukuxhuzula, kodwa oku kunqabile. Ngelishwa, i-PML idla ngokuba mandundu ngokuhamba kwexesha, kwayeIdla ngokuba yingozi. Nangona kunjalo, iziphumo zinokwahluka ngokuxhomekeke kwiimeko zonyango zesigulana kunye nendlela esisabela ngayo kunyango.

Yintoni ebangela i-PML?

I-PML ibangelwa yintsholongwane ebizwa ngokuba yi-JC virus (JCV) . Le ntsholongwane ibizwa ngegama lonobumba wokuqala wegama lomntu owaqala ukosulelwa yintsholongwane (uJohn Cunningham). Le ntsholongwane ikwabizwa ngokuba yi-human polyomavirus 2.

Okumangalisayo kukuba, malunga nama-85% abantu abadala bane-JC virus emizimbeni yabo. Kodwa iingcali azikaqiniseki ukuba i-virus idluliselwa njani ebantwini. Kukho ubungqina bokuba abantwana banokuyifumana ngokutya okanye ngamanzi. Le ntsholongwane ikwafunyenwe nakwiimvavanyo zomchamo. Ngoko ke, kunokwenzeka ukuba inokudluliselwa ngongcoliseko lomchamo nomlomo.

Nangona kunjalo, nangona abantu abaninzi bene-JC virus emizimbeni yabo, ayisebenzi, ilala nje ingasebenzi. Ngenxa yoko, akukho zimpawu zibonakalayo. Nangona kunjalo, kumntu one-immune system ebuthathaka, le virus inokuba isebenza emzimbeni ize ikhule ibe yi-PML. Ifana notshaba oluleleyo oluvukayo luze lusihlasele xa sibuthathaka.

Ngubani osengozini enkulu yokuhlaselwa sesi sifo?

I-PML idla ngokuvela kuphela kubantu abaneenkqubo zomzimba ezibuthathaka kakhulu. Usenokuba sengozini yokufumana esi sifo ukuba unenye yezi meko zilandelayo:

• Usulelo lwe-HIV/AIDS
• Umhlaza wegazi kunye nomongo wethambo, umzekelo, i-leukemia
• Umhlaza wenkqubo ye-lymphatic, umzekelo, i-lymphoma okanye isifo seHodgkin
• Izifo ezizikhusela ngokwazo emzimbeni, ezifana ne-rheumatoid arthritis, i-multiple sclerosis (MS), okanye i-lupus

Kwakhona, ukuba ungumnikeli wezitho zomzimba kwaye uthatha amayeza okuthintela amasosha omzimba ukuthintela umzimba wakho ukuba ungalamkeli ilungu, usenokuba sengozini.

Oogqirha bayixilonga njani i-PML?

Ugqirha wakho uza kuqala akuhlole aze amamele ngononophelo iimpawu zakho. Emva koko, banokuqinisekisa i-PML ngokwenza iimvavanyo ezininzi ezihlola ubuchopho bakho kunye nomqolo wakho. Kusenokufuneka wenze iimvavanyo ezifana nezi:

• I-Brain MRI scan: Oku kungathatha imifanekiso yezilonda kwi-white matter yengqondo. Kufana nekhamera ejonge ngaphakathi kwengqondo.
• Ukubhoboza umqolo/umqolo: Oku kuquka ukuthatha isampuli encinci yolwelo lwakho lwe-cerebrospinal (CSF), ulwelo olujikeleze ingqondo yakho kunye nomqolo. Oku kunokujonga ukuba ikhona na intsholongwane ye-JC.
• I-biopsy yobuchopho: Oku kwenziwa rhoqo. Kwenziwa kuphela xa kunzima ukuqinisekisa ukuxilongwa.

Inyangwa njani i-PML?

Eyona nto iphambili ekunyangweni kwe-PML kukuqinisa amajoni akho omzimba. Khumbula, izifo zenzeka xa amajoni ethu omzimba ebuthathaka. Ngoko ke, eyona nto ibalulekileyo kukuqinisa olo khuselo kwakhona.

• Umzekelo, ukuba une-AIDS, unganikwa unyango lwe-antiretroviral (ART) . La mayeza asebenza ngokunciphisa inani le-HIV emzimbeni wakho. Olu nyango lunokunceda inkqubo yakho yomzimba yokuzikhusela isebenze ukuya kuthi ga kwinqanaba elithile.
• Abanye abantu banokungenelwa kunyango olubizwa ngokuba yi-plasma exchange . Olu nyango lususa amanye amayeza okunciphisa umzimba egazini lakho. Olu nyango lunokunceda inkqubo yakho yomzimba yokulwa nentsholongwane ye-JC ngokunyusa ukusebenza kwayo.

Oososayensi okwangoku bavavanya amayeza amatsha amaninzi okunyanga i-PML. I-US Food and Drug Administration (FDA) inike imvume ekhethekileyo kwabanye abantu ukuba basebenzise la mayeza, kodwa iziphumo zixubene.

Ndifanele ndimbone nini ugqirha?

Ukuba uyazi ukuba inkqubo yakho yomzimba yokuzikhusela ibuthathaka kwaye uziva ngathi kancinci kancinci uqala ukuba neempawu ocinga ukuba zinxulumene neProgressive Multifocal Leukoencephalopathy (PML), kubaluleke kakhulu ukubona ugqirha ngoko nangoko uze uhlolwe.

Ndingayibuza yiphi imibuzo ugqirha wam?

Xa ubona ugqirha wakho, ungambuza imibuzo efana nale:

• Iyintoni ingozi yam yokuba ne-PML?
• Ngaba kukho into endinokuyenza ukunciphisa lo mngcipheko?
• Zeziphi iimpawu ekufuneka ndizijonge kwi-PML?
• Zithini iingozi zokuthatha amayeza okunciphisa umzimba?
• Ngaba zikho ezinye iindlela zonyango zengxaki yam?
• Zithini iingozi kunye neenzuzo zonyango lwangoku?

Ngokubuza le mibuzo, ungafumana ukuqonda okungcono ngesi sifo kunye nonyango lwaso.

Ingakanani iminyaka yobomi bomntu one-PML?

Le yingongoma enzima kakhulu ukuthetha ngayo. I-PML sisifo esiqhubekayo. Oku kuthetha ukuba iimpawu ziya zisiba mandundu ngokuhamba kwexesha. Ulindelo lobomi bomntu one-PML luxhomekeke kubukhali bosulelo kunye nonyango alufumanayo. Phakathi kwama-30% nama-50% abantu bayafa kwiinyanga ezimbalwa zokuqala emva kokufunyaniswa ukuba banaso. Abantu abaphila ixesha elide bayaqhubeka nokuba neempawu, kwaye ezo mpawu ziya zisiba mandundu. Ezi mpawu zinokukhokelela kwiingxaki ezinkulu, ezifana nokulahlekelwa kukuthetha kunye nokulahlekelwa kukubona.

Nangona kunjalo, izifundo ezintsha zibonise ukuba inani elincinci labantu abane-PML eyosulelwe yi-HIV banokuzinzisa isifo sabo baze baphile ixesha elide. Ngaphambi kokuba kufike unyango lwe-antiretroviral (ART) lokunyanga usulelo lwe-HIV, yi-10% kuphela yabantu abane-PML abaphila ixesha elingaphezu konyaka. Kodwa ngoku, nge-ART, izinga lokusinda lonyaka omnye linyuke laya kwi-50%. Kwezinye iimeko, i-ART inokunceda inkqubo yomzimba yokulwa nentsholongwane ye-JC.

Ngaba kunokwenzeka ukuba uphile ngokupheleleyo kwi-PML?

Abantu abafumana i-PML ngelixa besebenzisa amayeza okunciphisa umzimba banokuchacha emva kokuyeka amayeza. Abantu abaye banyangwa ngempumelelo nge-ART banokuchacha kwi-PML. Nangona kunjalo, ama-80% abantu abasinda kwi-PML ababuyi zimpawu. Oku kungenxa yokuba i-myelin entsha ayiveliswa kwiindawo ezonakeleyo. Umonakalo ongapheliyo wemithambo-luvo uqhelekile. Nangona kunjalo, abanye abantu banokuphila ukuya kuthi ga kwiminyaka eli-15 emva kokuba i-PML iphelile.

Zithini izinto ezilindelweyo kwabo batshintshana ngeplasma?

Ukuba iimpawu zakho ze-PML ziqale ngenxa yeyeza elithintela amasosha omzimba, iimpawu zakho zinokuphela xa uyeka ukulisebenzisa elo yeza. Ungafumana notshintsho lwe-plasma ukuze ususe amanye amayeza okunciphisa amasosha omzimba emzimbeni wakho. Injongo yotshintsho lwe-plasma kukukhulisa amasosha omzimba wakho. Kwabanye abantu, utshintsho lwe-plasma lunokucothisa ukuqhubela phambili kwe-PML kwaye lwandise ubomi. Nangona kunjalo, iingenelo zoku azikaqondwa ngokupheleleyo.

Abantu abaninzi banokuqhubeka beba neempawu ze-PML emva kokuyeka ukusebenzisa amayeza okanye emva kokufumana utshintsho lwe-plasma. Abanye banokuba neengxaki zemithambo-luvo. Kukwakho nezinye iingozi zokuyeka ukusebenzisa amayeza. Ngoko ke, kubalulekile ukuthetha nogqirha wakho xa usenza izigqibo malunga namayeza akho.

Ndingazi njani ukuba ndisemngciphekweni wokufumana i-Progressive Multifocal Leukoencephalopathy (PML)?

Ukuba unesifo esibangela ukuba amajoni akho omzimba abe buthathaka, buza ugqirha wakho malunga nomngcipheko wakho wokuba ne-PML.

Ngaba ezinye iimeko zonyango ziyandisa umngcipheko wam wokuba ne-PML?

Abantu abanezifo ezifana ne-multiple sclerosis (MS) okanye isifo sikaCrohn kufuneka bahlolwe igazi ukuze kuhlolwe iimeko ezifana ne-HIV ezonyusa umngcipheko wokosulelwa yi-JC virus.

Oogqirha badla ngokunika iyeza elibizwa ngokuba yiNatalizumab (Tysabri®) ukunyanga i-MS okanye isifo sikaCrohn. Eli yeza linokunyusa umngcipheko wokuba ne-PML.

Nokuba une-JC virus engasebenziyo kwaye ungenazo iimpawu, unokukwazi ukuthatha i-natalizumab. Nangona kunjalo, kubalulekile ukuthetha nogqirha wakho malunga neengozi kunye neenzuzo.

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

I-Progressive Multifocal Leukoencephalopathy (PML) lusulelo olungaqhelekanga olubangelwa kukuvuselelwa kwentsholongwane ye-JC. Uninzi lwabantu lunentsholongwane ye-JC elele emizimbeni yabo. Ayibangeli monakalo kubantu abangenayo inkqubo yomzimba yokuzikhusela ebuthathaka.

Ukuba unenkqubo yomzimba yokuzikhusela ebuthathaka, kubalulekile ukuba uqaphele iimpawu ze-PML kwaye usebenzisane nogqirha wakho ukuze wenze njalo.

Esi sifo sidla ngokuba sibi ngokuhamba kwexesha. Nangona kunjalo, kukho unyango olunokunceda ekunciphiseni ukuqhubela phambili kwesi sifo kwaye lwandise ubomi bakho. Ngoko ke, musa ukuphelelwa lithemba. Eyona nto ibalulekileyo kukufuna iingcebiso zonyango ngokukhawuleza.

I- PML, i-Progressive Multifocal Leukoencephalopathy, intsholongwane ye-JC, usulelo lobuchopho, inkqubo yomzimba yokuzikhusela, izifo zemithambo-luvo, i-myelin