Ingabe uthola nomkhuhlane njalo ngaphandle kwesizathu? Ingabe unezinye izinkinga kanye nawo? Lokhu kungaba yi-'Familial Mediterranean Fever (FMF)'!

Ingabe uvele uhlaselwe umkhuhlane ngokuzumayo ngaphandle kwesixwayiso? Ingabe ubuye ube nobuhlungu besisu, umuzwa wokuqina esifubeni sakho, noma ubuhlungu bamalunga? Ngezinye izikhathi lezi zimpawu ziyaphela ngemva kwezinsuku ezimbalwa, bese ziphinde zivele ngemva kwesikhashana? Kuvamile ukuzizwa wesaba kakhulu uma lokhu kwenzeka. Namuhla sizokhuluma ngesifo esibangela lezi zimpawu, okuyinto engavamile kancane, kodwa okubaluleke kakhulu ukwazi ngayo.

Kuyini i-'Familial Mediterranean Fever (FMF)'?

Kalula nje, i-Familial Mediterranean Fever, noma i-FMF ngamafuphi, yisimo sokuphila konke esivela ezizakhini zethu zofuzo. Okwenzekayo kulokhu ukuthi, ngaphandle kwesizathu esibonakalayo, sithola umkhuhlane ngokuzumayo, futhi ngesikhathi esifanayo, kukhona ukuvuvukala ezingxenyeni ezahlukene zomzimba. Ukuze kube sobala, lokhu kuyinto eyenzeka ngenxa yenkinga encane ohlelweni lwethu lokuzivikela komzimba. Yingakho ibizwa nangokuthi isifo sokuvuvukala okuzenzakalelayo .

Ithinta kakhulu abantu abahlala emazweni aseMedithera. Ibangela ukuvuvukala kwezicubu ezincane ezibizwa ngokuthi 'izicubu ze-serous' (lezi zimboza izindawo ezifana nomgodi wesifuba kanye nomgodi wesisu), okubizwa ngokuthi 'i-serositis'. Kungabangela nokuvuvukala kwezicubu 'ze-synovial' ezimboza amalunga ethu, okubizwa ngokuthi 'i-synovitis'. Izimpawu zivame ukuhlala izinsuku ezimbalwa bese ziphela.

Ziyini izimpawu ze-FMF?

Izimpawu ze-FMF zivame ukuqala ebuntwaneni, ngaphambi kweminyaka engama-20. Lezi zimpawu zingase zibe zimbi ekuqaleni, kodwa zingase zithuthuke kancane kancane njengoba ukhula. Uma usunalesi sifo, ungase ungabi nazimpawu amasonto, izinyanga, noma ngisho neminyaka. Kodwa-ke , ukutholakala nokwelashwa kusenesikhathi kungasiza ekunciphiseni izimpawu futhi kuvimbele izinkinga zesikhathi eside njenge-amyloidosis.

Manje ake sibone ukuthi lezi zimpawu ziyini:

• Umkhuhlane ophakeme ofinyelela kuma-degree Celsius angu-40.6 (105 Fahrenheit), ngezinye izikhathi ohambisana nokubanda nokuthuthumela.
• Kungase kube nobuhlungu bekhanda kanye nentamo eqinile.
• Ubuhlungu besisu kanye nokuqunjelwa (ukuqunjelwa).
• Imisipha yesisu iyaqina futhi kungase kube nokuqunjelwa .
• Ubuhlungu besifuba , obukhula lapho uphefumula.
• Ubuhlungu nokuvuvukala kwamalunga (ngokuvamile esilungwini esisodwa).
• Ukuvuvukala nobuhlungu emasendeni kwabesilisa.
• Ubuhlungu bemisipha , ikakhulukazi emzimbeni ongezansi.
• Kungase kuvele isilonda sesikhumba esibomvu nesiphakanyisiwe emilenzeni .

Lezi zimpawu zingavela ngokuzumayo phakathi namahora ambalwa. Kodwa-ke, akuwona wonke umuntu ozobhekana nazo zonke lezi zimpawu.. Umkhuhlane uphawu oluvame kakhulu. Ebuntwaneni, umkhuhlane ungase ube uphawu olulodwa kuphela. Ukugula kuvame ukuhlala izinsuku ezimbalwa.

Izimpawu ze-Prodromal ze-FMF

Abanye abantu baqala ukuzwa izimpawu ezincane ezinsukwini ezimbalwa ngaphambi kokuqala kwalomkhuhlane. Njengoba nje abanye abantu bezizwa ngaphambi kokuthi "i-migraine" noma abesifazane bazizwe ngaphambi kokuthi "baye esikhathini". Lokhu sikubiza ngokuthi " izimpawu ze-prodromal". Uma uzizwa lezi zimpawu, uthola umbono wokuthi "Ah... manje leyo nkinga iyeza".

Yiziphi lezi zibonakaliso ezibonisa lokhu?

• Ukukhathazeka, ukungahlaliseki
• Ukucasuka
• Ikhanda elibuhlungu
• Isicanucanu
• Ubuhlungu bomzimba
• Ubuthakathaka ( ukuntuleka kwentshiseko, ukungabi nampilo )

Kungani lokhu 'FMF' kwenzeka?

Lokhu kungenxa yezinguquko ku-gene ye-'MEFV' emizimbeni yethu. Le gene ye-'MEFV' isiza ekwenzeni iphrotheni ebaluleke kakhulu, okungukuthi iphrotheni ebizwa ngokuthi 'i-pyrin'. Isimiso sethu sokuzivikela somzimba esizalwa naso - okungukuthi, ingxenye yesimiso sokuzivikela somzimba esisebenza ngokumelene nezinsongo ezifana nokutheleleka okungazelelwe - sithembele kule phrotheni ye-'pyrin'. Ngakho-ke, lapho i-gene ye-'MEFV' ingakwazi ukwenza 'i-pyrin' kahle, leyo phrotheni ayikwazi ukusebenza kahle ngaphakathi kwesimiso sokuzivikela komzimba. Yilapho-ke kuvela khona le nkinga.

Kutholakale cishe izinguquko zofuzo ezingu-30 (`ukuguquguquka`) okubangela i-`FMF`. Kulezi, ezivame kakhulu yi-`V726A`, `M680I`, `E148Q`, `M694V` kanye ne-`M694I`. Lezi zivame kakhulu kubantu abavela eMedithera naseMpumalanga Ephakathi. Isibonelo:

• Ama-Arabhu
• Ama-Armenia
• AmaGriki
• Ama-Iran
• AmaNtaliyane
• Abantu bamaJuda
• AmaKurd
• AmaTurkey

Izimbangela ze-FMF

Nakuba i-FMF ivame ukubonakala ngokuzumayo futhi ngaphandle kwesizathu esibonakalayo, kwabanye abantu ibangelwa izinto ezithile. Kwabanye abantu, lesi simo singabangelwa ukucindezeleka ngokomzimba noma ngokwengqondo. Abantu bathi i-FMF ingabangelwa izinto ezifana nalezi:

• Ukuchayeka emakhazeni
• Ukuzivocavoca noma ukukhathala ngokweqile
• Ukutheleleka kwamuva nje
• Ukulimala noma ukuhlinzwa kwamuva nje
• Ukucindezeleka okukhulu (njengenkinga enkulu empilweni)
• Isikhathi lapho abesifazane beya esikhathini sabo sanyanga zonke noma beya esikhathini sokuvuthwa kwamaqanda

Izinkinga ezingaba khona ze-FMF

Nakuba umkhuhlane we-FMF uphela ngemva kwezinsuku ezimbalwa, ukuvuvukala okuqhubekayo emzimbeni kungathinta imizimba yethu ngokuhamba kwesikhathi. Yingakho odokotela bencoma ukwelashwa okuqhubekayo kwalokhu. Uma kunenqubo yokuvuvukala okuqhubekayo njengale, amasosha ethu omzimba afakwa ngaphansi kwengcindezi enkulu, futhi nezinye izifo zingavela.

Inkinga enkulu kakhulu yalokhu yisimo esibizwa ngokuthi i-'secondary amyloidosis' . Lokhu kungaholela nasekuhlulekeni kwezinso. Yingakho kubaluleke kakhulu ukuqaphela ngalokhu.

Ezinye izinkinga zingabandakanya:

• I-splenomegaly (i-splenomegaly)
• Ukungazali
• Ezinye izifo zokuzivikela komzimba (`Isifo sesibili sokuzivikela komzimba`)

I-FMF itholakala kanjani?

Udokotela uyixilonga kanjani i-FMF? Benza izinto ezinjengalezi:

• Bazolalela ngokucophelela izimpawu zakho, ukuthi zivela kangaki, nokuthi sezinesikhathi esingakanani zikhona .
• Bazobuza ukuthi ukhona yini emndenini wakho oke waba nalesi sifo nokuthi liyini ifa lakho lezakhi zofuzo.
• Uma ugula, bazokuhlola ukuze babone ukuthi kukhona yini ukuvuvukala noma ukucasuka emzimbeni wakho .
• Ngesikhathi sokugula , kwenziwa ukuhlolwa kwegazi ukuze kubonakale ukuthi kukhona yini izimpawu zokuvuvukala .
• Kunconywa ukuhlolwa kwezakhi zofuzo ukuze kuhlolwe izinguquko ku-MEFV gene, okuvame ukubangela i-FMF.
• Bazobheka nokuthi usabela kanjani ekwelashweni kwe-FMF.

Ukwelashwa kwe-'FMF'

Ukwelashwa okuyinhloko kwe-FMF umuthi wokulwa nokuvuvukala i-colchicine. Lokhu kusebenza ngokunciphisa umsebenzi wohlobo lweseli elimhlophe legazi elibizwa ngokuthi ama-neutrophils, ahilelekile ekuvuvukeni. I-Colchicine ingasiza ekuvimbeleni noma ekunciphiseni ukuvama kokuqubuka kwe-FMF, futhi inciphise ingozi yezinkinga. Abantu abangaphezu kuka-90% abane-FMF bayelapheka . Iningi labantu kufanele lithathe lo muthi nsuku zonke impilo yabo yonke.

Kodwa-ke, inani elincane kakhulu labantu alitholi imiphumela efiselekayo nge-colchicine, noma bangase bangakwazi ukuthatha lo muthi ngenxa yezinye izizathu zempilo. Kubantu abanjalo, odokotela basikisela ezinye izinhlobo zemithi. Ezinye zazo yilezi:

• `Anakinra` (`Anakinra`)
• `I-Rilonacept`
• `I-Canakinumab`

Kwenzekani uma une-`FMF`?

Nakuba kungekho ukwelashwa kwe-FMF, ingaphathwa kahle ngemithi . Kwabaningi, umkhuhlane kanye nobuhlungu bomzimba cishe kuyaphela ngokuphelele lapho beqala ukuthatha imithi. Kwabanye, ukuvama kanye nokuvama kokuqubuka kwesifo kuyancipha. Kodwa-ke, ukuze le mithi isebenze kahle, kumele ithathwe njalo, ngisho noma ingenazo izimpawu.Ukuthatha lo muthi uma usuvele ugula ngeke kumise ukugula ngokushesha. Lo muthi uvimbela kuphela ukugula ukuthi kungenzeki.

Ingabe i-FMF ingavinjelwa?

Abantu abaningi abazi nokuthi bane-gene variant ebangela i-FMF. Ukuze uthole i-FMF, udinga ukuthola amakhophi amabili e-gene variant kumama wakho nakubaba wakho. Uma unekhophi eyodwa kuphela, ngeke ube nezimpawu ze-FMF. Kodwa ungadlulisela leyo gene variant kubantwana bakho. Uma umlingani wakho naye enayo leyo gene variant, izingane zakho zingase zibe ne-FMF. Ukuhlolwa kwe-genetic kungakusiza ukuthi uthole ukuthi ungumthwali we-gene variant.

Kube nemibiko yemikhuhlane enjalo evame ukwenzeka kusukela ezikhathini zasendulo. Mhlawumbe lesi sifo esibizwa ngokuthi 'i-FMF' sisesidala. Abanye abacwaningi bacabanga ukuthi lesi simo kungenzeka ukuthi savela njengendlela yokusiza ekulweni nezifo ezinkulu ezifana 'nesifo sofuba' ezazisakazeke ngaleso sikhathi. Eqinisweni, kunezinzuzo ezithile zokuba nokuvuvukala emzimbeni, kodwa kuphela uma kuba kakhulu lapho kuba yinkinga. Ngenhlanhla, manje sekukhona imithi emihle yokulawula lesi simo. Ngakho-ke noma ngabe une-'FMF', ungaphila impilo evamile.

Into ebaluleke kakhulu okufanele uyikhumbule! (Umyalezo Wokuya Ekhaya)

Kulungile, manje usunokuqonda okungcono ngalokho esikhulume ngakho namuhla, `i-FMF`. Into ebaluleke kakhulu ukuthi uma wena noma othile emndenini wakho enomkhuhlane ngaphandle kwesizathu, ubuhlungu besisu, ubuhlungu bamalunga, njll., ungawuthathi njengomkhuhlane nje, kodwa bona udokotela ukuze uthole ukuthi kwenzekani ngempela. Uma ubona lesi sifo futhi uqala ukwelashwa kusenesikhathi, ungavimbela izinkinga eziningi futhi uphile impilo evamile. Khumbula, kubaluleke kakhulu futhi ukusebenzisa umuthi udokotela wakho awunikezayo njengoba kuqondiswe. Ungakhohlwa ukukhuluma nodokotela wakho nganoma yimiphi imibuzo noma ukukhathazeka ongase ube nakho.

I- FMF, i-Familial Fever Syndrome, imfiva evame ukwenzeka, izinhlungu zesisu, ubuhlungu bamalunga, izifo zofuzo, i-colchicine, i-amyloidosis