Ingabe ingane yakho ivele ibe nomkhuhlane njalo? Ingabe iba ngcono ngemva komkhuhlane izinsuku ezimbalwa, bese iphinda ibe nomkhuhlane ezinsukwini ezimbalwa kamuva? Ungase uzibuze ukuthi kungani lokhu kwenzeka ngaphandle kwegciwane noma ukutheleleka ngamagciwane. Lokhu kungaba ngenxa yesimo esingavamile esibizwa ngokuthi ama-SAID (Systemic Autoinflammatory Diseases) , abantu abaningi abangakaze bezwe ngaso. Ungakhathazeki, ake sixoxe ngakho ngamazwi alula futhi siqonde konke.
Ayini ama-SAID? Masiqonde kalula.
Kalula nje, ama-SAID ayiqembu lezifo ezibangela umkhuhlane ophindaphindayo ngaphandle kokutheleleka (njengamagciwane noma amabhaktheriya). Imbangela yalokhu ukungasebenzi kahle kwesimiso somzimba sokuzivikela somzimba sengane yethu. Lokhu kwakubizwa ngokuthi 'ama-Periodic Fever Syndromes' ngaphambilini.
Manje ungase uzibuze, ingabe lesi yisifo 'esihlasela umzimba'? Sivame ukuzwa ngezifo ezihlasela umzimba njenge-Rheumatoid Arthritis noma i-Lupus. Cha, lokhu kuhlukile.
Ake sithi sinebutho lokuzivikela emzimbeni wethu, isimiso sethu sokuzivikela. Leli butho linezingxenye ezimbili eziyinhloko:
1. Uhlelo Lokuzivikela Olungokwemvelo: Leli yibutho esizalwa nalo, futhi lingelokuqala ukuhlasela noma yiliphi igciwane.
2. Uhlelo Lokuzivikela Oluzivumelanisa Nezimo: Leli yibutho eliqeqeshwe ngokukhethekile ukulwa namagciwane ahlukahlukene njengoba sikhula.
Kuma-SAID, inkinga isesimisweni somzimba sokuzivikela esingokwemvelo. Leso simiso somzimba sokuzivikela siyaphazamiseka bese siqala ukulwa emzimbeni ngaphandle kwesitha. Umphumela walokhu kulwa ukuvuvukala emzimbeni kanye nomkhuhlane.
Kodwa ezifweni zokuzivikela komzimba esikhulume ngazo, inkinga ikwebutho elizivumelanisa nezimo eliqeqeshwa kamuva. Lelo butho alikwazi ukubona amangqamuzana amahle emzimbeni walo bese liqala ukuwahlasela.
Ama-SAID awavamile kakhulu kunezifo ezizimele. Avame ukubangelwa yizimbangela zofuzo , okusho ukuthi azuzwe njengefa. Lezi zimpawu zivame ukuqala lapho ingane isencane kakhulu , njengasebuntwaneni noma esemncane. Ingane ngokuzumayo iba nesigameko noma ukuhlaselwa ukugula okunomkhuhlane nezinye izimpawu, okuvame ukuxazululeka zingakapheli izinsuku ezimbalwa. Ingane ingase ibe nempilo enhle ngokuphelele uma ingaguli. Nakuba kungekho ukwelashwa, kunezindlela zokwelapha ezisebenzayo ezingalawula izimpawu.
Yiziphi izinhlobo eziyinhloko ze-SAIDs?
Abacwaningi sebethole izinhlobo ezingaba ngu-60 ze-SAID kuze kube manje, futhi izinhlobo ezintsha zitholakala ngaso sonke isikhathi. Nazi ezinye zezinhlobo ezivame kakhulu.
| Igama lesifo (Uhlobo lwe-SAID) | Isingeniso esifushane |
|---|---|
| Umkhuhlane WaseMedithera Womndeni (FMF) | Lolu uhlobo oluvame kakhulu lwe-SAID oluhlonzwe ngofuzo, olubangela ukuvuvukala okubuhlungu esiswini, esifubeni nasemalungeni. |
| Umkhuhlane Wesikhathi Esithile, I-Aphthous-Stomatitis, I-Pharyngitis, I-Adenitis (PFAPA) | Kuqala ezinganeni ezincane, ikakhulukazi ngaphambi kweminyaka engu-4. Izimpawu ezifana nomphimbo obuhlungu, izilonda zomlomo, kanye nezindlala ezivuvukele entanyeni ziza nomkhuhlane. Ngokuvamile kuyaphela ngemva kweminyaka engu-10. |
| Isifo Sezinkathi Esihambisana Ne-Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS) | Lokhu kungaqala ebuntwaneni noma ngisho nasebusheni. |
| Ukuntuleka kwe-Mevalonate Kinase (MKD) | Lokhu kuvame ukuqala ngaphambi kokuba ingane ibe nonyaka owodwa ubudala. |
| Izifo Ezihambisana Nokuvuvukala Okuzimele ze-NLRP3 (CAPS) | Empeleni lokhu kuyinhlanganisela yezifo ezintathu ezahlukene. |
| Isifo Sokuqala Kwabantu Abadala (AOSD) | Njengoba igama lisho, lesi yisifo esiqala lapho umuntu esemdala. |
| I-Blau Syndrome | Lokhu kuvame ukwenzeka ezinganeni ezingaphansi kweminyaka engu-4. Kuthinta kakhulu isikhumba, amehlo kanye namalunga. |
Yiziphi izimpawu ezivamile zalezi zifo?
Uphawu oluyinhloko noluvame kakhulu lwe-SAIDs umkhuhlane ophindaphindayo . Kodwa-ke, ngaphezu komkhuhlane, ezinye izimpawu zingase zivele kuye ngohlobo lwesifo.
Into ebalulekile ukuthi lezi zimpawu zenzeka kuphela ngesikhathi sokugula. Uma leso sikhathi sesiphelile, ingane ingaba nempilo enhle ngokuphelele ngaphandle kwezimpawu.
| Igama lesifo (Uhlobo lwe-SAID) | Izimpawu okufanele uziqaphele |
|---|---|
| I-FMF | Ubuhlungu obukhulu esiswini, esifubeni nasemalungeni. Izilonda zesikhumba emilenzeni engezansi noma emaqakaleni. |
| I-PFAPA | Umphimbo obuhlungu, izilonda emlonyeni, ama-lymph node avuvukele entanyeni. |
| IZINGIBE | Umzimba uzizwa ubanda futhi uqhaqhazela. Kunobuhlungu emisipheni yomzimba, ikakhulukazi esiqwini nasezingalweni. Amabala abomvu abuhlungu angavela kuwo wonke umzimba. |
| I-MKD | Umzimba uzizwa ubanda futhi uqhaqhazela, unekhanda elibuhlungu, ubuhlungu besisu, ukuphelelwa yisifiso sokudla, kanye nezimpawu ezifana nezomkhuhlane ovamile. |
| Izifo ze-NLRP3 | Ukuqubuka kwesikhumba, ikhanda elibuhlungu, ukukhathala, ubuhlungu bamalunga, kanye nokutheleleka kwamehlo (conjunctivitis). |
| I-Blau Syndrome | Izilonda zesikhumba ezingalweni, emilenzeni, noma esiqwini sengane. Ubuhlungu bamalunga kanye nobuhlungu bamehlo. |
Kungani lezi zifo zenzeka? Ziyini izimbangela?
Njengoba sishilo ngaphambili, ama-SAID amaningi ayizifo zofuzo . Lokhu kusho ukuthi lezi zifo zibangelwa ukuguqulwa (ukuguquguquka) kwezakhi zofuzo ezithile.
| Igama lesifo (Uhlobo lwe-SAID) | I-Associated Gene |
|---|---|
| I-FMF | I-MEFV gene |
| I-PFAPA | Isizathu esiqondile asikatholakali okwamanje. |
| IZINGIBE | I-gene ye-TNFRSF1A |
| I-MKD | I-MVK gene |
| Izifo ze-NLRP3 | I-NLRP3 gene |
| I-AOSD | Isizathu esiqondile asikatholakali okwamanje. |
| I-Blau Syndrome | I-gene ye-NOD2 |
Yini engenzeka uma ukwelashwa okufanele kungatholakali?
Lena inkinga ebaluleke kakhulu. Kubalulekile ukulawula lesi simo ngokuthatha ukwelashwa okufanele. Ngoba, uma kukhona ukuvuvukala okuqhubekayo emzimbeni kanje, kungaholela esimweni esibizwa ngokuthi i-Amyloidosis . Kalula nje, uhlobo oluthile lweprotheni lungafakwa ezinso zethu, okubangela umonakalo ohlala njalo ezinso. Ngakho-ke, kubaluleke kakhulu ukuthola lesi sifo bese uselapha ngokushesha.
Udokotela usithola kanjani lesi sifo?
Uma ngikhuluma iqiniso, ukuxilonga ama-SAID kungaba nzima kancane ngoba izimpawu zingafana nezezinye izifo ezinkulu, njenge-lupus.
Ngakho-ke, into ebaluleke kakhulu ukubona udokotela ogxile kulezi zinhlobo zezifo. Udokotela ogxile ezifweni ezihlobene namalunga kanye nesimiso somzimba sokuzivikela ubizwa ngokuthi i-Rheumatologist .
Udokotela wakho uzocabangela izici eziningana ukuze athole ukuthi unesifo. Cishe uzobuza ngezimpawu zengane yakho nokuthi ngabe kukhona yini umlando womndeni walesi simo. Udokotela wakho angase asole ama-SAID, ikakhulukazi uma:
- Uma ingane ivame ukuba nomkhuhlane.
- Uma kukhona emndenini oke waba nezifo ezifana nomkhuhlane eziphindaphindayo njengalesi.
- Njengoba lezi zifo zivamile phakathi kwamaqembu ezinhlanga ezithile, lokho kuyacatshangelwa.
Yiziphi izivivinyo ezenziwayo?
Udokotela angase ancome ukuhlolwa okuningana ukuqinisekisa lesi sifo.
- Ukuhlolwa kwegazi (Ukuhlolwa kwelebhu): Ukuhlolwa okufana ne -C-reactive protein (CRP) kanye ne -CBC (Full Blood Count) kungahlola ukuvuvukala emzimbeni. La mazinga ayanda ngesikhathi sokugula futhi abuyele emazingeni ajwayelekile lapho umzimba uphilile.
- Ukuhlolwa komchamo: Kuhlola amazinga aphezulu eprotheyini emchameni. Kwezinye izifo, njenge-MKD, ukuhlolwa komchamo kungathola ama-asidi athile.
- Ukuhlolwa kwezakhi zofuzo: Lokhu kuhlolwa kungathola ukuguquka kwezakhi zofuzo. Kodwa-ke, ngezinye izikhathi, ingane ingaba ne-SAID kodwa ukuhlolwa kwezakhi zofuzo kungase kungayitholi. Ngakho-ke, noma ngabe umphumela wokuhlolwa ungemuhle, akunakushiwo ngokuphelele ukuthi lesi sifo asikho.
Yiziphi izindlela zokwelapha lokhu?
Ukwelashwa kwe-SAID kuncike ohlotsheni kanye nobukhali besifo. Nakuba lezi zifo zingelapheki ngokuphelele, imithi ingazilawula kakhulu izimpawu.
- I-FMF: Kusetshenziswa umuthi obizwa ngokuthi i-Colchicine kulokhu. Unciphisa ukuvuvukala emzimbeni. Uma lowo muthi ungasebenzi enganeni, ingaphendukela emithini efana ne -Canakinumab .
- I-PFAPA: Ama-steroid afana ne -Prednisone anganikezwa isikhathi esifushane ukuze kuncishiswe ngokushesha isikhathi sokugula.
- IZINGIBE: I-CanakinumabLo muthi uyasebenza kakhulu. Futhi, kusetshenziswa imithi yokulwa nokuvuvukala efana ne -glucocorticoids .
- I-MKD: Umuthi i -Canakinumab nawo uyasebenza kulokhu. Ngesikhathi sokugula, ama-NSAID (ama-painkiller) noma ama-steroid angasiza.
- Izifo ze-NLRP3: Imithi yokuguqula amasosha omzimba njengeCanakinumab, iRilonacept , kanye ne - Anakinra isetshenziswa ngempumelelo kulokhu.
- I-Blau Syndrome: Kuye ngezimpawu, kungasetshenziswa imithi yokucindezela amasosha omzimba, imithi yokuvimbela i-TNF, kanye nemithi yamehlo.
Ingabe lesi simo sizophulukiswa ngokuphelele?
Amanye ama-SAID ahlala isikhathi eside. Kodwa-ke, amanye, njenge-PFAPA, angase aphele ngokwawo njengoba ingane ikhula. Ngisho noma kuyizifo zokuphila konke, ukuvama kokugula kanye nobunzima bezimpawu kungancipha ngokuhamba kwesikhathi. Udokotela wakho uzokunikeza imininingwane ethile ngesimo sengane yakho.
Ukunakekela ingane enalesi simo kungaba yinselele. Kodwa ngosizo lochwepheshe abafanele, ungakwazi ukulawula izimpawu zengane yakho futhi uyinike ubuntwana obuhle kakhulu.
Umlayezo Wokuya Nawe Ekhaya
- Uma ingane yakho ivame ukuba nomkhuhlane ngaphandle kwesizathu esicacile, ungayinaki nje bese ubona udokotela ngokushesha.
- Ama-SAID ayiqembu lezifo ezingavamile ezibangelwa inkinga engxenyeni engaphakathi yesimiso somzimba sokuzivikela.
- Lezi akuzona izifo ezithelelanayo; zivame ukubangelwa izici zofuzo.
- Kubaluleke kakhulu ukubona udokotela wamathambo (udokotela ochwepheshe bamalunga kanye nesimiso somzimba sokuzivikela) ukuze athole lesi sifo ngokunembile.
- Nakuba lokhu kungelapheki ngokuphelele, imithi ingasiza ekulawuleni izimpawu futhi ikuvumele ukuthi uphile impilo evamile.
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