Ingabe ingane yakho ivame ukuba nomkhuhlane? Kungenzeka yini ukuthi kungenxa ye-SAIDs (Systemic Autoinflammatory Diseases)?

O, ingabe ingane yakho ivame ukuba nomkhuhlane kulezi zinsuku? Lapho nje ucabanga ukuthi usungcono, ingabe uyavuka futhi? Ngezinye izikhathi imbangela yalokhu ingase ingabi yigciwane noma amagciwane avamile. Namuhla sizokhuluma ngesimo esiyinkimbinkimbi kancane, kodwa kubaluleke kakhulu ukwazi ngaso. Lokhu kubizwa ngokuthi ama-SAID, okusho ukuthi i-Systemic Autoinflammatory Diseases . Esikhathini esidlule, lokhu kwakubizwa ngokuthi `(Periodic Fever Syndromes)` noma `(Recurrent Fever Syndromes)`.

Ayini ama-SAID? Masiqonde kalula.

Kalula nje, ama-SAID ayiqembu lezifo ezibangelwa ukungasebenzi kahle noma inkinga yokulawula ohlelweni lokuzivikela lomzimba esizalwa nalo . Yingakho ingane ingathola umkhuhlane njalo ngisho noma ingenayo igciwane (njengegciwane noma amagciwane).

Into ebaluleke kakhulu ukuthi lezi zimo ezibizwa ngokuthi ama-SAID akufanele zididaniswe `(Izifo Ezizimele)` (isibonelo `(Isifo Samathambo Esigugayo)` noma `(I-Lupus)` okungenzeka ukuthi uke wezwa ngazo. Ezifweni `(Izifo Ezizimele)`, ngenxa yephutha elithile `(isistim somzimba esizivumelanisa nezimo)` semizimba yethu, leso simiso sihlasela amangqamuzana aso aphilile. Kodwa kuma-SAID, kwenzeka okuhlukile.

Ama-SAID awavamile kakhulu kunezifo ezizimele. Avame ufuzo futhi abangelwa ukuguquka kwezakhi zofuzo .

Ngokuvamile, kodwa hhayi njalo, izimpawu ze-SAID ziqala lapho ingane isencane kakhulu, kungaba usana noma ingane encane . Ingane yakho ingase ibe "nezikhathi" zomkhuhlane nezinye izimpawu ezihambisana nalesi simo. Phakathi kwalezi zikhathi, ingane yakho ingase ingabi nazimpawu nhlobo. Nakuba kungekho ukwelashwa kwe-SAID, kuvame ukuba khona ukwelashwa okusiza ukulawula izimpawu zengane yakho.

Yiziphi izinhlobo eziyinhloko ze-SAIDs?

Abacwaningi bathole izinhlobo ezingaba ngu-60 ze-SAID, futhi ezintsha zisatholakala. Nazi ezinye zezinhlobo ezivame kakhulu ze-SAID:

• Umkhuhlane waseMedithera womndeni (i-FMF): Lona umkhuhlane ovame kakhulu ozuzwe ngofuzo, ophindaphindayo. Ungabangela ukuvuvukala okubuhlungu kwesisu, isifuba, kanye namalunga ezinganeni.
• Umkhuhlane ovamile, i-aphthous-stomatitis, i-pharyngitis, i-adenitis (PFAPA): Lokhu kuvame ukuqala ezinganeni ezincane ngaphambi kweminyaka engu-4. Ngezinye izikhathi, lesi simo singaba ngcono ngokwaso ngemva kweminyaka engu-10.
• I-Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Lokhu kungenzeka ebuntwaneni, ebusheni, noma ngisho nasebudaleni.
• Ukuntuleka kwe-Mevalonate kinase (MKD): Ngaphambilini kwakuthiwa ``i-Hyper-IgD syndrome'', lokhu kuvame ukuqala ngaphambi kokuba ingane ibe nonyaka owodwa ubudala.
• Izifo ezibangelwa ukuvuvukala okuzenzakalelayo ezihlobene ne-NLRP3:Lokhu kwakubizwa ngaphambilini ngokuthi `(Cryopyrin-associated periodic syndromes - CAPS)`. Kunezinhlobo ezintathu zalesi simo.
• Isifo sika-Adult-onset Still (AOSD): Njengoba igama lisikisela, lesi yisimo sokuqala sobuntwana esibizwa ngokuthi "Systemic Juvenile Idiopathic Arthritis (JIA)" esivela kubantu abadala.
• Isifo se-granulomatous esihlobene ne-NOD-2 (i-Blau syndrome): Lokhu kuvame ukuqala ngaphambi kweminyaka engu-4. Kuthinta kakhulu isikhumba, amehlo, kanye namalunga engane.

Ziyini izimpawu ze-SAID?

Izimpawu ze-SAID zivame ukuqala ebuntwaneni. Uphawu oluyinhloko noluvame kakhulu umkhuhlane ovamile . Ngesikhathi somkhuhlane, abantu bavame ukuba nempilo enhle futhi bangabi nazimpawu. Kodwa-ke, kungase kube nezinye izimpawu eziqondene nohlobo ngalunye lwe-SAID. Ake sibone ukuthi ziyini:

• I-FMF: Lokhu kungabangela ukuvuvukala nobuhlungu obukhulu esiswini sengane, esifubeni nasemalungeni . Kungase kube nokuqubuka emilenzeni engezansi noma emaqakaleni.
• I-PFAPA: Lokhu kungabangela umphimbo obuhlungu, izilonda emlonyeni, kanye nokuvuvukala kwama-lymph node entanyeni . Isibonelo, uma ingane yakho ikhombisa izimpawu ezifana ne-tonsillitis evame ukwenzeka kanye nezilonda emlonyeni, kungaba yi-PFAPA.
• IZINGCINGO: Lokhu kungabangela ukubanda kanye nobuhlungu bemisipha esiqwini/esithweni somzimba nasezingalweni . Kungabangela nokuqubuka okubomvu okubuhlungu okuqala ezingalweni nasemilenzeni bese kusakazeka emzimbeni.
• I-MKD: Lokhu kungabangela izimpawu ezifana nokubanda, ikhanda elibuhlungu, ubuhlungu besisu, ukulahlekelwa yisifiso sokudla, kanye nezimpawu ezifana nomkhuhlane .
• Izifo ze-NLRP3: Lesi simo singabangela ukuqubuka kwesikhumba, ikhanda elibuhlungu, ubuthakathaka, ubuhlungu bamalunga, kanye ne-pink eye/conjunctivitis .
• I-AOSD: Lesi yisimo esibonakala ngokuqubuka kwesikhumba, ubuhlungu bamalunga, kanye nobuhlungu bemisipha . Abanye abantu bangase babe nomphimbo obuhlungu, ubuhlungu besisu, ukukhathala, kanye nokungaphatheki kahle.
• I-Blau syndrome: Lokhu kungabangela izilonda zesikhumba ezingalweni, emilenzeni, noma phakathi kwesitho somntwana wakho . Kungabangela nobuhlungu bamalunga kanye nobuhlungu bamehlo .

Yiziphi izimbangela ze-SAID?

Ama-SAID amaningi ayizifo zofuzo . Okusho ukuthi, ukuguquka okuthile (okuhlukile) ku-gene ethile kubangela isifo ngasinye. Ake sithi imizimba yethu inesethi yemiyalelo (izakhi zofuzo) esitshela ukuthi siwenze kanjani umsebenzi ngamunye, ngakho-ke uma uhlamvu olulodwa kuleyo ncwadi lungalungile, lungabangela ukuthi wonke umsebenzi uhambe kabi.

Nazi izinhlobo eziyinhloko zama-SAID kanye nezakhi zofuzo ezihlobene nawo:

• I-FMF:I-gene `MEFV` yiyo enikeza imiyalelo yokuthi iprotheni `(pyrin)` ingenziwa kanjani.
• I-PFAPA: Imbangela eqondile yalokhu ayikakaqinisekiswa .
• IZINGCINGO: I-gene `TNFRSF1A`. Lokhu kufundisa ukukhiqizwa kwephrotheni ebizwa ngokuthi `(i-tumor necrosis factor receptor - TNFR)`.
• I-MDK: I-gene ebizwa ngokuthi i-`MVK`. Lokhu kufundisa ukukhiqizwa kweprotheni ebizwa ngokuthi i-`(mevalonic kinase)`.
• Izifo ze-NLRP3: I-gene `NLRP3`. Lokhu kufundisa ukukhiqizwa kwephrotheni ebizwa ngokuthi `(cryopyrin)`.
• I-AOSD: Imbangela eqondile yalokhu ayikatholakali .
• I-Blau syndrome: I-gene `NOD2`. Lokhu kufundisa ukukhiqizwa kwephrotheni ebizwa ngokuthi `(NOD2)`.

Kubalulekile ukuqonda ukuthi uma lokhu kubangelwa izici zofuzo, akulona iphutha labazali. Lezi izinqubo eziyinkimbinkimbi kakhulu zebhayoloji.

Izinkinga ezibangelwa yi-SAIDs

Kubaluleke kakhulu ukuthola ukwelashwa okufanele kwalezi zimo. Ngoba, uma ukuvuvukala kuqhubeka emzimbeni, kungaholela esimweni esibizwa ngokuthi `(Amyloidosis). `(Amyloidosis)` kuyi -protein deposit ezinso . Lokhu kungabangela umonakalo ohlala njalo ezinso . Ngakho-ke, uma kunezimpawu, kubalulekile ukufuna iseluleko sezokwelapha ngokushesha.

Ama-SAID atholakala kanjani?

Uma ngikhuluma iqiniso, ukuxilonga ama-SAID kungaba nzima kancane ngoba izimpawu zingafana nezezinye izimo ezingathi sína, njenge-lupus noma i-lymphoma. Ngakho-ke, kubalulekile ukubona udokotela oqeqeshwe ngokukhethekile ezifweni zokuvuvukala (udokotela wamathambo) ukuze axilonge futhi aphathe kahle isimo sengane yakho.

Udokotela wezifo zamathambo wengane yakho uzosebenzisa izinto eziningana ukuxilonga ama-SAID. Uzobuza ngezimpawu zengane yakho nokuthi ngabe kukhona yini emndenini wakho oke waba nalezi zimo (umlando womndeni wemvelo). Udokotela angase asole ukuthi ingane yakho ine-Periodic Fever Syndrome uma:

• Uma unemfiva njalo
• Uma kukhona emndenini onomlando wemfiva ephindaphindayo kanjena
• Uma ungoweqembu labantu abavame ukuba nomkhuhlane onjalo

Yiziphi izivivinyo ezisetshenziswayo?

Udokotela wakho angase ancome ukuhlolwa okuningana ukuqinisekisa ukuxilongwa. Lokhu kungafaka:

• Ukuhlolwa kwelebhu: Ukuhlolwa okufana ne-`(C-reactive protein - CRP)` kanye ne-`(Complete Blood Count - CBC)` kungahlola ukuvuvukala emzimbeni. Lokhu kuhlolwa kuba `(positive)` uma kunomkhuhlane, futhi kubuyela emazingeni ajwayelekile lapho umkhuhlane uphela.
• Ukuhlolwa komchamo:Ukuhlolwa kwephrotheni yomchamo kungenziwa ukuhlola amazinga aphezulu ephrotheni emchameni. Ku-MKD, ukuhlolwa komchamo kwama-organic acid kuzobonisa amazinga aphezulu e-mevalonic acid.
• Ukuhlolwa kofuzo: Lokhu kungahlola ukuhlukahluka kofuzo. Kodwa-ke, ezinye izingane ezine-SAIDs kungenzeka zingatholakali ukuhlukahluka kofuzo. Ngakho-ke, noma ngabe imiphumela yokuhlolwa "ingemihle", kungenzeka ukuthi i-SAID ayinakugwenywa.

Iphathwa kanjani i-SAIDs?

Ukwelashwa kwe-SAID kuyahlukahluka kuye ngohlobo kanye nobunzima besifo . Nakuba lezi zifo zingelapheki ngokuphelele, izimpawu zengane zingalawulwa kahle ngokusebenzisa imithi . Isibonelo, uma ingane yakho ithola la ma-cold izikhathi ezimbalwa ngonyaka, imithi yobuhlungu kanye nemithi yokulwa nokuvuvukala engeyona i-steroidal (NSAIDs) ivame ukulawula izimpawu. Kodwa-ke, uma lesi sifo sibi kakhulu, udokotela angase asikisele ezinye izindlela zokwelapha.

Nazi ezinye izindlela zokwelapha ezinjalo:

• I-FMF: Lokhu kungelashwa ngomuthi obizwa ngokuthi `(Colchicine)` ukuze kuncishiswe ukuvuvukala. Uma ungakwazi ukuthatha `(Colchicine)`, ungazama uhlobo `(Biologic)` lomuthi obizwa ngokuthi `(Canakinumab)`.
• I-PFAPA: Ukuqubuka kwe-PFAPA kungafinyezwa ngokunikeza ama-steroid (ngokuvamile i-prednisone). Ezinye izingane ziye zakwazi ukulawula izimpawu zazo ngokusebenzisa i-cimetidine, umuthi osetshenziselwa ukwelapha izilonda zesisu.
• IZINGCINGO: Umuthi `(Canakinumab)` usebenza kahle kakhulu kwi-TRAPS. Futhi, imithi elwa nokuvuvukala kadokotela efana ne-`(Glucocorticoids)` ingasiza ekunciphiseni izimpawu.
• I-MKD: `(Canakinumab)` iyindlela enhle yokwelashwa kwe-MKD. Ukusebenzisa `(NSAIDs)` noma `(Steroids)` ngesikhathi somkhuhlane nakho kungaba usizo.
• Izifo ze-NLRP3: Ezinye izidakamizwa zokulawula amasosha omzimba ezifana neCanakinumab kanye neRilonacept, Anakinra, ziyasebenza ekwelapheni izifo ze-NLRP3.
• I-AOSD: Izinhlobo ezahlukene zemithi yokulwa nokuvuvukala zisetshenziselwa i-AOSD. Lokhu kufaka phakathi `(ama-Steroids)`, `(imithi yokulwa nokuvuvukala eguqula izifo - ama-DMARD)`, kanye `(ama-Biologics)`.
• I-Blau syndrome: Kuye ngezimpawu zengane, imithi yokucindezela amasosha omzimba, imithi yokuvimbela i-tumor necrosis factor (TNF), kanye/noma imithi yamehlo esetshenziswa endaweni yokwelashwa ingazanywa.

Ingabe isimo se-SAID siba ngcono ngokwaso?

Amanye ama-SAID angahlala impilo yonke . Amanye angase ahlale iminyaka embalwa futhi abe ngcono njengoba ekhula. Nakuba ezinye izimo ziphila impilo yonke, ukuvama komkhuhlane kungancipha ngokuhamba kwesikhathi futhi izimpawu zingase zingabi zimbi kakhulu . Udokotela wakho angakunikeza imininingwane ethile ngesimo esithile sengane yakho.

Ukunakekela ingane ene-SAID kungaba yinselele. Mhlawumbe nawe uke wakhuluma nge-SAID, kodwa ungasebenzisa okuhlangenwe nakho kwakho ukusiza ukunakekelwa kwengane yakho futhi uyisize ibhekane nalesi simo sokuphila kwayo yonke.

Into ebaluleke kakhulu ukufuna ukwelashwa kochwepheshe abanolwazi oluphelele ngezinkinga ze-SAID. Bangasiza ekuphatheni izimpawu zengane yakho futhi bayisize ibe nobuntwana obuhle kakhulu.

Izinto ezibaluleke kakhulu okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, ake sifingqe ezinye zezinto okudingeka uzikhumbule kulokho esikhulume ngakho.

• Uma ingane yakho inemfiva evame ukungachazeki , khuluma nodokotela ngayo. Kungaba uphawu lwe-SAID.
• Ama-SAID ayinkinga yesimiso somzimba sokuzivikela esingokwemvelo , hhayi isifo esivamile esithathelwanayo.
• Lezi zihlukile ezifweni "ezizivikela ngokuzenzakalelayo" .
• Esikhathini esiningi, lokhu kubangelwa izici zofuzo .
• Kubalulekile ukubonana nodokotela wezifo zamalunga ukuze uthole ukuxilongwa okunembile.
• Ukwelashwa kungalawula izimpawu kahle .
• Ukwelashwa kusenesikhathi kubalulekile ukuvimbela ukulimala kwezinso.

Uma uneminye imibuzo mayelana nalokhu, ungesabi ukubuza udokotela womndeni wakho noma udokotela wezingane. Bazokwazi ukukusiza kabanzi.

Ama -SAID, umkhuhlane ozuzwe njengefa, umkhuhlane ovamile, umkhuhlane ezinganeni, amasosha omzimba, izifo zofuzo, isazi samathambo