Ukuthi yini i-POEMS Syndrome? Izimpawu, Izimbangela, & Ukwelashwa

I-POEMS syndrome iyisifo esingavamile segazi elithinta izingxenye eziningi zomzimba wakho ngesikhathi esisodwa. Igama layo livela ezintweni eziyisihlanu eziyinhloko eziyibangela: I-Polyneuropathy (ukulimaza kwama-nerve), i-Organomegaly (izitho ezinkulu), i-Endocrinopathy (izinkinga ze-hormone), i-Monoclonal protein (amaproteni angavamile), kanye nezinguquko zesikhumba.

Lesi simo senzeka lapho amaseli egazi athile abizwa ngama-plasma cells eqala ukusebenza ngokungavamile. La maseli akhiqiza okuningi kwe-protein okungalimaza ama-nerve akho, izitho, namanye amasimu omzimba. Nakuba i-POEMS syndrome iyingozi, ukuyiqonda kangcono kungakusiza ukusebenza neqembu lakho lokunakekelwa kwezempilo ukuyilawula ngokuphumelelayo.

Yini i-POEMS syndrome?

I-POEMS syndrome iyisifo esithinta izici eziningi zomzimba esibangelwa ama-plasma cells angavamile e-bone marrow yakho. La maseli akhiqiza inani elikhulu le-protein elihamba egazini lakho futhi libangele ukulimala okubanzi kulo lonke umzimba wakho.

Lesi simo sithinta abantu abangaphansi kuka-1 ku-100,000, okwenza kube yinto engavamile. Ngokuvamile iqala kubantu abadala abaneminyaka engama-40 kuya kwengu-60, yize ingase ibonakale kunoma iyiphi iminyaka. Amadoda anethuba elikhulu lokuba ne-POEMS syndrome kunabesifazane.

Okwenza lesi simo sibe inselele ngokukhethekile ukuthi asithinti nje kuphela uhlelo olulodwa lwezitho. Kunalokho, kubangela izinkinga ohlelweni lwakho lwezinzwa, uhlelo lwe-endocrine, igazi, isikhumba, nezinye izitho ngesikhathi esisodwa.

Ziyini izimpawu ze-POEMS syndrome?

Izimpawu ze-POEMS syndrome zingase zizizwe zikhulu kakhulu ngoba zithinta izingxenye eziningi zomzimba wakho. Ake sihambe ngalokho ongakubona, sikhumbula ukuthi akuwona wonke umuntu ozoba nazo zonke lezi zimpawu.

Isimpawu esivamile futhi esivame ukuba yisibonakaliso sokuqala oyobona yilimaza lezinzwa, ikakhulukazi ezandleni nasezinzwani zakho:

• Ukuqina nokuthamba okuqala ezicupheleni nasezinzwani zakho
• Ubuthakathaka ezandleni nasezinzwani zakho okwenza kube nzima ukubamba izinto noma ukuhamba
• Ukuthoba noma ubuhlungu obukhulu ezandleni nasezinzwani zakho
• Ubuthakathaka bemisipha obuya kancane kancane buya ezingalweni zakho
• Ukulahlekelwa yi-reflexes, ikakhulukazi ezinkinini nasemadolweni akho

Lezi zinkinga zezinzwa ngokuvamile ziya ziba zimbi ngokuhamba kwesikhathi futhi zingathinta kakhulu imisebenzi yakho yansuku zonke. Ubuthakathaka ngokuvamile buqala buthambile kodwa bungathuthuka kuze kube yilapho ukuhamba kuya kuba nzima.

Isikumba sakho singase futhi sibonise izinguquko eziningi ezihlukile:

• Ukukhanya kwesikhumba sakho, ikakhulukazi ezindaweni eziphendukayo njengentamo yakho nasesandleni
• Ukuqina kwesikhumba sakho okubukeka njengolwe-leather
• Ukukhula okukhulu kwezinwele ebusweni bakho, ezandleni, noma ezinyaweni
• Amachaphazi amancane abomvu esikhumbeni sakho abangelwa yizitsha zegazi ezaphukile
• Ukukhanya kwamakhokhonkwane akho noma ukuguquka kwamakhokhonkwane

Abantu abaningi abane-POEMS syndrome nabo baye baba nezimpawu ezihlobene ne-hormone. Uhlelo lwakho lwe-endocrine luphethe imisebenzi eminingi ebalulekile, ngakho uma luthintekile, ungase uqaphele izinguquko kwamandla akho, isimo sengqondo, nokuzizwa kwakho konke.

Ezinye izimpawu zingafaka ukukhathala okuqhubekayo, ukuphefumula kancane, ukukhula kwezinyawo noma esiswini, kanye nezinguquko embonweni wakho. Abanye abantu nabo baye baba nokulahlekelwa isisindo, ukubila ebusuku, noma ubuhlungu emathanjeni.

Yini ebangela i-POEMS syndrome?

I-POEMS syndrome iqala lapho ama-plasma cells e-bone marrow yakho eba angavamile futhi eqala ukukhiqiza okuningi kwe-protein ethile. La ma-plasma cells ngokuvamile ingxenye yohlelo lwakho lokulwa nezifo futhi asisize ukulwa nezifo ngokwenza ama-antibodies.

Ku-POEMS syndrome, la maseli eqala ukwenza inani elikhulu le-monoclonal protein. Le protein ihamba egazini lakho futhi ibangele ukuvuvukala nokulimala kwama-nerve akho, izitho, nezinye izicubu. Cabanga ngakho njengokuba nefuthi elikhulu elikufanele ukwenze imikhiqizo ewusizo kodwa kunalokho liqale ukukhiqiza into eyingozi.

Isizathu esiqondile sokuthi kungani la ma-plasma cells eba angavamile asiqondakali ngokuphelele. Akuyona into oyithola kubazali bakho, futhi ayibangelwa yilutho owalwenza noma awungenzanga. Lesi simo sibonakala sithuthuka ngokungahleliwe, yize ucwaningo oluthile luphakamisa ukuthi lungase luhlobene nezinguquko ezithile ze-gene ngaphakathi kwama-plasma cells ngokwawo.

Okwaziyo ukuthi le nto yokukhiqiza i-protein engavamile iqala uchungechunge lwezinkinga kulo lonke umzimba wakho. Ingabangela izitsha zakho zegazi ukuba zivuze, okuholela ekukhuleni nasekukhuleni kwezitho. Ingase futhi iphazamise ukukhiqizwa kwe-hormone yakho futhi yonakalise isikhumba esivikelayo esizungezile ama-nerve akho.

Nini okufanele ubheke udokotela nge-POEMS syndrome?

Kufanele ubheke udokotela uma uhlushwa ukuqina okuqhubekayo, ukuthamba, noma ubuthakathaka ezandleni nasezinzwani zakho, ikakhulukazi uma lezi zimpawu ziya ziba zimbi ngokuhamba kwesikhathi. Lezi zinkinga zezinzwa ngokuvamile yisibonakaliso sokuqala se-POEMS syndrome futhi akufanele zidedelwe.

Kubalulekile futhi ukufuna ukunakekelwa kwezokwelapha uma uqaphela izinguquko zesikhumba ezingachazwanga, ikakhulukazi ukukhanya noma ukuqina kwesikhumba sakho. Nakuba lezi zinguquko zingaba nezizathu eziningi, uma zivele kanye nezimpawu zezinzwa, zidinga ukuhlolwa.

Ungalindi ukubona udokotela uma uhlushwa ukuphefumula kancane, ukukhula okukhulu kwezinyawo noma esiswini, noma ukukhathala okuqhubekayo okuthinta imisebenzi yakho yansuku zonke. Lezi zimpawu zingase zibonise ukuthi lesi simo sithinta inhliziyo yakho, amaphaphu, noma ezinye izitho ezibalulekile.

Uma unezimpawu eziningi ezibukeka zingenalo ubudlelwane kodwa zonke zenzeka ngesikhathi esisodwa, kufanele uxoxe nodokotela wakho. I-POEMS syndrome ingase ibe nzima ukuyiqonda ngoba izimpawu zayo zingafana nezinye izimo, kodwa ukuhlolwa okuphelele kungasiza ekuqondeni ukuthi kwenzekani.

Ziyini izici ezingozi ze-POEMS syndrome?

Ngokungafani nezinye izimo eziningi, i-POEMS syndrome ayinazo izici ezingozi ezicacile ongaziqondisa noma uzigweme. Lesi simo sibonakala sithuthuka ngokungahleliwe, futhi abacwaningi abazange baqonde izici ezithile zokuphila noma ukudalulwa komkhathi okwandisa ingozi yakho.

Iminyaka idlala indima, njengoba i-POEMS syndrome ngokuvamile iqala kubantu abaneminyaka engama-40 kuya kwengu-60. Noma kunjalo, ingase ibonakale kunoma iyiphi iminyaka, kufaka phakathi abantu abadala abancane nabantu abadala.

Amadoda anethuba elikhulu kunabesifazane, kodwa umehluko awukukhulu. Lesi simo sithinta abantu bazo zonke izizwe futhi kubonakala kungavamile kunoma iyiphi indawo ethile.

Ukuba nezinye izifo zegazi noma izimo ze-autoimmune kubonakala kungawandisi ingozi yakho yokuthuthukisa i-POEMS syndrome. Akukhuthazeki, ngakho awukwazi ukuyithola komunye umuntu noma uyidlulisele kwabanye.

Ziyini izinhlanganisela ezingase zenzeke ze-POEMS syndrome?

I-POEMS syndrome ingabangela izinhlanganisela ezingathi sína uma ingalashwa, kodwa ukuqonda lezi zinto ezingase zenzeke kungakusiza ukusebenza neqembu lakho lokunakekelwa kwezempilo ukuzivimbela noma ukuzilawula ngokuphumelelayo.

Ukulimala kwezinzwa okuhlobene ne-POEMS syndrome kungathuthuka kube ukukhubazeka okukhulu. Ngaphandle kokwelashwa, ubuthakathaka ezandleni nasezinzwani zakho bungathuthuka kuze kube yilapho uba nezinkinga zokuhamba noma ukwenza imisebenzi yansuku zonke njengokubopha izingubo noma ukubamba izinto.

Izinhlanganisela zenhliziyo namaphaphu zingase futhi zithuthuke:

• Ukukhula kwamanzi eduze kwenhliziyo yakho noma amaphaphu
• Uxinzelelo lwegazi oluphakeme emaphaphwini akho
• Izinkinga zokuthinta kwenhliziyo
• Ukuphefumula kancane, ikakhulukazi uma ulele phansi

Lezi zinhlanganisela zingaba yingozi futhi zidinga ukunakekelwa kwezokwelapha ngokushesha. Izindaba ezinhle ukuthi ngokwelashwa okufanele, izinkinga eziningi zingavikelwa noma zilungiswe.

Izinkinga zezinso zingase zenzeke kwabanye abantu abane-POEMS syndrome. Amaproteni angavamile egazini lakho angalimaza izinso zakho ngokuhamba kwesikhathi, okungenzeka kube ukwehluleka kwezinso uma kungalashwa ngokushesha.

Izinkinga emathanjeni, kufaka phakathi ukuphuka kwamathanjeni ne-bone lesions, zingase futhi zithuthuke. Abanye abantu abane-POEMS syndrome bathuthukisa izindawo ezibuthakathaka emathanjeni ezivame ukuphuka kalula noma zibangele ubuhlungu.

Nakuba lezi zinhlanganisela zizwa zesabekisa, kubalulekile ukukhumbula ukuthi ukuqonda nokwelashwa kwasekuqaleni kunganciphisa kakhulu ingozi yakho yokuthuthukisa izinkinga ezingathi sína. Ukuqapha okuqhubekayo nokwelashwa okufanele kungasiza ekuvikeleni izitho zakho nokugcina ikhwalithi yokuphila kwakho.

I-POEMS syndrome ihlonishwa kanjani?

Ukuhlonishwa kwe-POEMS syndrome kudinga ukuhlolwa okuphelele ngoba lesi simo sithinta izici eziningi zomzimba. Udokotela wakho uzodinga ukuqinisekisa ukuthi unezinye zezici eziyinhloko ngaphambi kokwenza leli gciwane.

Inqubo yokuhlola ngokuvamile iqala ngomlando wezokwelapha ophelele nokuhlolwa komzimba. Udokotela wakho uzokubuza ngezimpawu zakho, lapho zaqala khona, nokuthi ziguquke kanjani ngokuhamba kwesikhathi. Bazophinde bahlole isikhumba sakho, bahlole i-reflexes yakho, futhi bahlole izitho ezinkulu.

Izivivinyo zegazi kubalulekile ekuxilongeni futhi zizofaka:

• Izivivinyo zokuthola i-monoclonal protein engavamile
• Ukulinganiswa kwezinga le-hormone
• Izivivinyo zokuhlola umsebenzi wezinso nezibindi zakho
• Ukubalwa kwegazi okuphelele ukuhlola amaseli egazi akho

Udokotela wakho uzophinde a-ode izivivinyo zokuphequlula izinzwa ukuze alinganise ukuthi izinzwa zakho zisebenza kahle kangakanani. Lezi zivivinyo zihlanganisa izimpikiswano ezincane zamandla okusiza ekuqondeni ubukhulu bokulimala kwezinzwa.

I-bone marrow biopsy ingase idingeke ukuhlola ama-plasma cells akho ngokuqondile. Le nqubo ihlanganisa ukuthatha isampula encane ye-bone marrow, ngokuvamile emathanjeni akho, ukubuka amaseli angavamile ngaphansi kwesikrini.

Izivivinyo zokuthola izithombe njenge-CT scans noma i-MRIs zingase zenziwe ukuhlola izitho ezinkulu noma i-bone lesions. Lezi zivivinyo zisiza udokotela wakho ukuqonda ukuthi lesi simo sithinta kanjani izingxenye eziningi zomzimba wakho.

Yini ukwelashwa kwe-POEMS syndrome?

Ukwelashwa kwe-POEMS syndrome kugxile ekuthuthukiseni ama-plasma cells angavamile nokulawula izimpawu ezibangelwa yiwo. Izindaba ezinhle ukuthi ngokwelashwa okufanele, abantu abaningi baye baba nokuthuthuka okukhulu ezimpawu zabo.

Indlela eyinhloko yokwelashwa ihlanganisa izindlela zokwelapha ezibhekiswe kuma-plasma cells angavamile e-bone marrow yakho. Lezi zindlela zokwelapha zifana nalezo ezisetshenziswa kwezinye izifo zegazi futhi kuye kwabonakala zisebenza kahle kakhulu kwi-POEMS syndrome.

Imithi ye-chemotherapy ngokuvamile isetshenziswa ekuthuthukiseni inani lama-plasma cells angavamile. Le mithi isebenza ngokubhekisisa amaseli ahlukanisayo ngokushesha, kufaka phakathi ama-plasma cells abangela izimpawu zakho. Nakuba igama elithi "chemotherapy" lingase lizwakale liyesabekisa, imithi esetshenziswa kwi-POEMS syndrome ngokuvamile iyamukeleka kahle.

I-radiation therapy ingase iphakanyiswe uma une-bone lesions ezihlukile. Lokhu ukwelashwa kusebenzisa imisebe yamandla aphezulu ukubhubhisa amaseli angavamile ezindaweni ezithile zomzimba wakho.

Kwabanye abantu, i-stem cell transplant ingase ibe yindlela engcono kakhulu. Le nqubo ihlanganisa ukufaka indawo ye-bone marrow yakho engavamile ngama-stem cells aphilile, noma ngawe noma umnikelo. Kuyindlela yokwelapha enkulu, kodwa ingasebenza kakhulu kubantu abancane, abaphilile.

Ngaphezu kwalokhu ukwelashwa okuyinhloko, udokotela wakho uzogxila futhi ekulawuleni izimpawu zakho. Lokhu kungase kuhlanganise imithi yokusiza ubuhlungu bezinzwa, izindlela zokwelapha ukungalingani kwe-hormone, kanye nezindlela zokwelapha zokunciphisa ukukhula kwamanzi.

Indlela yokuphatha i-POEMS syndrome ekhaya?

Nakuba ukwelashwa kwezokwelapha kubalulekile kwi-POEMS syndrome, kunezinto eziningi ongazenza ekhaya ukuze usize ukulawula izimpawu zakho nokuthuthukisa ikhwalithi yokuphila kwakho.

Ukukhathalela izinzwa zakho ezithintekile kubaluleke kakhulu. Ukuzivocavoca okuthambile ne-physical therapy kungasiza ekugcineni amandla emisipha nokuguquguquka. Udokotela wakho noma i-physical therapist bangakubonisa ukuzivocavoca okuthile okuphephile nokusebenzayo esimweni sakho.

Ukuvikela isikhumba sakho kubalulekile futhi, ikakhulukazi uma unezindawo zesikhumba esiqinile noma esikhanyayo. Sebenzisa amasoseji athambile, afakazayo futhi ufake i-lotion njalo ukuvimbela ukumomotheka nokuqhekeka. Gwema amakhemikhali aqinile noma ukudalulwa okukhulu kwelanga.

Ukulawula ukukhathala ngokuvamile kuyinselele enkulu nge-POEMS syndrome. Zama ukuzilawula usuku lonke futhi ubeke phambili imisebenzi ebaluleke kakhulu kuwe. Izikhathi ezimfushane zokuphumula zingakusiza ukulondoloza amandla ezintweni ozidingayo noma ozifuna ukuzenza.

Ukudla ukudla okulinganiselayo kungasiza ekusekweni impilo yakho yonke futhi kunike umzimba wakho izondlo ezidingekayo ukuze ululame. Gxila ekudleni okunamavithamini naminerali, futhi uhlale uthambile ngaphandle uma udokotela wakho engakwelulekisile ngenye indlela.

Kubalulekile futhi ukuqapha izimpawu zakho nokuthumela noma yiziphi izinguquko eqenjini lakho lokunakekelwa kwezempilo. Gcina idayari elula yokuthi uzizwa kanjani, kufaka phakathi noma yiziphi izimpawu ezintsha noma izinguquko ezisekhona.

Kufanele ulungiselele kanjani i-appointment yakho yodokotela?

Ukulungiselela i-appointment yakho yodokotela kungasiza ekuqinisekiseni ukuthi uthola okuningi kakhulu ekuhambeni kwakho futhi ungakhohlwa ukuxoxa ngezinkathazo noma imibuzo ebalulekile.

Ngaphambi kwe-appointment yakho, bhala zonke izimpawu zakho, kufaka phakathi lapho zaqala khona nokuthi ziguquke kanjani ngokuhamba kwesikhathi. Yiba ocacile ngezinto ezinjengendawo yokuqina noma ubuhlungu, ubukhulu bobuthakathaka, nanoma yiziphi izinguquko zesikhumba oziqaphelayo.

Letha uhlu lwemithi yonke oyithathayo, kufaka phakathi imithi yokwelapha, imithi yokuthengwa ngaphandle kwelayisensi, kanye nezinye izinto. Faka futhi noma yiziphi izifo zokuzalwa onazo emithini.

Lungiselela uhlu lwemibuzo ofuna ukuyibuza udokotela wakho. Lezi zingase zihlanganise imibuzo mayelana nezindlela zakho zokwelashwa, okumele ukulindele ngokuqondene nokuthuthuka kwesimpawu, nendlela yokuphatha imiphumela emibi yokwelashwa.

Uma kungenzeka, letha ilungu lomndeni noma umngani e-appointment yakho. Bangakusiza ukukhumbula ulwazi olubalulekile nokukunikeza ukwesekwa ngokomzwelo ngesikhathi esingase sibe yisikhathi esicindezelayo.

Butha noma yiziphi izincwadi zezokwelapha ezifanele, ikakhulukazi uma ubona udokotela omusha noma ochwepheshe. Lokhu kuhlanganisa imiphumela yezivivinyo zangaphambili, izivivinyo zokuthola izithombe, kanye nezincwadi ezivela kwabanye abahlinzeki bezempilo.

Yini into ebalulekile mayelana ne-POEMS syndrome?

I-POEMS syndrome iyisimo esingavamile kodwa esingathi sína esithinta izici eziningi zomzimba ngesikhathi esisodwa. Nakuba ingabangela izimpawu nezinhlanganisela ezinkulu, umbono wabantu abanesimo esinjalo uthuthukile kakhulu ngokwelashwa kwamanje.

Into ebaluleke kakhulu okumele uyikhumbule ukuthi ukuqonda nokwelashwa kwasekuqaleni kungenza umehluko omkhulu empumelelweni yakho. Abantu abaningi abane-POEMS syndrome baye baba nokuthuthuka okukhulu ezimpawu zabo ngokwelashwa okufanele, futhi abanye baye bafinyelela ekuphulukeni okuphelele.

Ukuphila ne-POEMS syndrome kudinga isineke nokuzimisela, kokubili kuwe neqembu lakho lokunakekelwa kwezempilo. Lesi simo singase sibe nzima ukusilaula, kodwa ngendlela efanele yokwelashwa nokuxhumana okuhle nodokotela bakho, ungasebenza ekuthuthukiseni impilo nekhwalithi yokuphila.

Unganqikazi ukufuna ukwesekwa emindenini, ebangqongeni, noma emaqenjini wokusekela abantu abanezifo ezingavamile. Ukuba neqembu eliqinile lokusekela kungenza umehluko omkhulu endleleni obhekana ngayo nezinselele zalesi simo.

Imibuzo evamile ebuzwa mayelana ne-POEMS syndrome

Ingabe i-POEMS syndrome yikanseri?

I-POEMS syndrome ayisiyona ikanseri ngomqondo ovamile, kodwa ihlobene nama-plasma cells angavamile e-bone marrow yakho. La maseli akhiqiza amaproteni ayingozi kodwa ngokuvamile awasabeli kwezinye izingxenye zomzimba njengoba amaseli ekanseri enza. Izindlela zokwelapha ezisetshenziswayo zifana nalezo zezinye izifo zegazi ngoba zibhekisisa ama-plasma cells angavamile.

Ingabe i-POEMS syndrome ingaphulile?

Nakuba ayikho indlela ephilile yokwelapha i-POEMS syndrome, abantu abaningi baye bafinyelela ekuphulukeni okuphelele ngokwelashwa okufanele. Lokhu kusho ukuthi izimpawu zabo ziya zithuthuka kakhulu noma ziyaphela ngokuphelele. Okubalulekile ukuthola ukwelashwa okufanele kwasekuqaleni nokulandela izeluleko zikadokotela wakho ngokucophelela.

Kuthatha isikhathi esingakanani ukwelashwa ukusebenza?

Isikhathi sokuthuthuka sihlukahluka kumuntu ngamunye, kodwa abantu abaningi baqala ukubona ukuthuthuka kwezimpawu zabo phakathi nezinyanga ezimbalwa beqala ukwelashwa. Izimpawu zezinzwa zingase zithathe isikhathi eside ukuthuthuka, ngezinye izikhathi kuze kube ngonyaka noma ngaphezulu. Udokotela wakho uzolandelela intuthuko yakho njalo futhi alungise ukwelashwa kwakho njengoba kudingeka.

Ingabe ngizodinga ukwelashwa impilo yonke?

Ubude bokuwelashwa kuncike ekutheni uphendula kahle kangakanani ekwelapheni nasekutheni isimo sakho sihlala siqondile. Abanye abantu badinga ukwelashwa okuqhubekayo ukuze bagcine izimpawu zabo zilawulwa, kanti abanye bangase bakwazi ukuyeka ukwelashwa uma befinyelela ekuphulukeni okuphelele. Udokotela wakho uzosebenza nawe ukuze unqume indlela engcono kakhulu yesikhathi eside yesimo sakho esithile.

Ngikwazi yini ukuba nezingane uma ngine-POEMS syndrome?

Ukuba ne-POEMS syndrome akukuvimbeli ngokuqondile ukuba nezingane, kodwa kubalulekile ukuxoxa lokhu nodokotela wakho ngaphambi kokuzimisela ukukhulelwa. Ezinye izindlela zokwelapha i-POEMS syndrome zingathinta ukukhulelwa noma zingase zibe yingozi ngesikhathi sokukhulelwa. Iqembu lakho lokunakekelwa kwezempilo lingakusiza ukuhlela ukukhulelwa okuphephile uma lokho kuyinto oyifunayo.