Ngaba umntwana wakho usoloko enesifo somkhuhlane? Ngaba kungenxa ye-SAIDs (Systemic Autoinflammatory Diseases)?

Owu, ngaba umntwana wakho usoloko efumana umkhuhlane kule mihla? Xa ucinga ukuba uphilile, ingaba uyaphinda uvuke? Ngamanye amaxesha unobangela woku usenokuba awuyontsholongwane okanye iintsholongwane eziqhelekileyo. Namhlanje siza kuthetha ngemeko enzima kancinci, kodwa kubaluleke kakhulu ukuyazi. Oku kubizwa ngokuba yi-SAIDs, emele iSystemic Autoinflammatory Diseases . Ngaphambili, oku kwakubizwa ngokuba yi-`(Periodic Fever Syndromes)` okanye `(Recurrent Fever Syndromes)`.

Zithini ii-SAID? Masiziqonde ngokulula.

Ngamafutshane, ii-SAID liqela lezifo ezibangelwa kukungasebenzi kakuhle okanye ingxaki yokulawula inkqubo yomzimba yethu yokuzikhusela . Yiyo loo nto umntwana enokufumana umkhuhlane rhoqo nangona engenasosulelo (njengentsholongwane okanye intsholongwane).

Eyona nto ibalulekileyo kukuba ezi meko zibizwa ngokuba zii-SAIDs akufuneki zidideke ne-(Autoimmune Diseases)` (umzekelo `(Rheumatoid Arthritis)` okanye `(Lupus)` osenokuba ukhe weva ngazo. Kwizifo ze-`(Autoimmune)`, ngenxa yempazamo ethile kwi-`(adaptive immune system)` yomzimba wethu, loo nkqubo ihlasela iiseli zayo eziphilileyo. Kodwa kwi-SAIDs, kwenzeka into eyahlukileyo.

Ii-SAID zinqabile kakhulu kunezifo ezizikhuselayo emzimbeni. Zihlala zizuzwa njengelifa kwaye zibangelwa kukuguquka kwezakhi zofuzo .

Ngokwesiqhelo, kodwa hayi rhoqo, iimpawu ze-SAID ziqala xa umntwana esemncinci kakhulu, nokuba uselusana okanye usemncinci . Umntwana wakho usenokuba "nexesha" lomkhuhlane kunye nezinye iimpawu ezihambisana nale meko. Phakathi kwezi xesha, umntwana wakho usenokungabi nazo kwaphela iimpawu. Nangona kungekho nyango lwe-SAIDs, kukho unyango olunceda ukulawula iimpawu zomntwana wakho.

Ziziphi iintlobo eziphambili ze-SAIDs?

Abaphandi bafumanise malunga neentlobo ezingama-60 ze-SAID, kwaye ezintsha zisafunyanwa. Nazi ezinye zezona ntlobo zixhaphakileyo ze-SAID:

• Umkhuhlane weMediterane wosapho (FMF): Le yeyona ixhaphakileyo kwizifo zomkhuhlane ezifunyanwa ngokwemfuza, eziphindaphindayo. Inokubangela ukudumba okubuhlungu kwesisu, isifuba kunye namalungu eeseli ebantwaneni.
• Umkhuhlane odla ngokuvela rhoqo, i-aphthous-stomatitis, i-pharyngitis, i-adenitis (PFAPA): Oku kudla ngokuqala kubantwana abancinci ngaphambi kweminyaka emi-4. Ngamanye amaxesha, imeko inokuba ngcono ngokwayo emva kweminyaka eli-10.
• I-Tumor necrosis factor receptor-associated periodic syndrome (i-TRAPS): Oku kunokwenzeka ebuntwaneni, ebusheni, okanye nasebudaleni.
• Ukungabikho kwe-Mevalonate kinase (MKD): Ngaphambili yayibizwa ngokuba yi-"Hyper-IgD syndrome", oku kudla ngokuqala ngaphambi kokuba umntwana abe nonyaka omnye ubudala.
• Izifo ezibangelwa kukuvuvukala komzimba ezinxulumene ne-NLRP3:Oku bekubizwa ngaphambili ngokuba yi-`(Cryopyrin-associated periodic syndromes - CAPS)`. Kukho ezinye iintlobo ezintathu ngaphakathi koku.
• Isifo sika-Adult-onset Still's (AOSD): Njengoko igama lisitsho, esi sisifo sobuntwana esiqala kubantu abadala esibizwa ngokuba yi-"Systemic Juvenile Idiopathic Arthritis (JIA)" (Systemic Juvenile Idiopathic Arthritis (JIA)").
• Isifo se-granulomatous esinxulumene ne-NOD-2 (i-Blau syndrome): Oku kudla ngokuqala ngaphambi kokuba kudlule iminyaka emi-4. Sichaphazela kakhulu ulusu lomntwana, amehlo kunye namalungu.

Zithini iimpawu ze-SAIDs?

Iimpawu ze-SAIDs zihlala ziqala ebuntwaneni. Uphawu oluphambili noluqhelekileyo yimfiva eyenzeka rhoqo . Ngexesha lomkhuhlane, abantu banokuba sempilweni kwaye bangabi nazimpawu. Nangona kunjalo, kunokubakho ezinye iimpawu ezikhethekileyo kuhlobo ngalunye lwe-SAID. Makhe sibone ukuba zeziphi:

• I-FMF: Oku kunokubangela ukudumba kunye nentlungu eqatha esiswini somntwana, esifubeni, nasemalungeni . Kunokubakho nokurhawuzelela emilenzeni engezantsi okanye emaqatheni.
• I-PFAPA: Oku kunokubangela umphimbo obuhlungu, izilonda emlonyeni, kunye nokudumba kwama-lymph nodes entanyeni . Umzekelo, ukuba umntwana wakho ubonisa iimpawu ezifana ne-tonsillitis exhaphakileyo kunye nezilonda emlonyeni, isenokuba yi-PFAPA.
• IMIGXA: Oku kunokubangela ukubanda kunye neentlungu zemisipha esiqwini/esinqeni nasezingalweni . Kunokubangela nokurhawuzelelwa okubomvu okubuhlungu okuqala ezingalweni nasemilenzeni kudlulele emzimbeni.
• I-MKD: Oku kunokubangela iimpawu ezifana nokugodola, intloko ebuhlungu, isisu esibuhlungu, ukungabi namdla wokutya, kunye neempawu ezifana nomkhuhlane .
• Izifo ze-NLRP3: Le meko inokubangela ukurhawuzelela kwesikhumba, iintloko ezibuhlungu, ukungondleki kakuhle, iintlungu zamalungu, kunye neliso elipinki/i-conjunctivitis .
• I-AOSD: Le yimeko ebonakaliswa kukurhawuzelelwa lusu, iintlungu zamalungu, kunye neentlungu zemisipha . Abanye abantu banokuba nomphimbo obuhlungu, iintlungu zesisu, ukudinwa, kunye nokungakhululeki.
• I-Blau syndrome: Oku kunokubangela izilonda zesikhumba ezingalweni, emilenzeni, okanye phakathi kotyando lomntwana wakho . Kunokubangela neentlungu zamalungu kunye nentlungu yamehlo .

Zithini izizathu ze-SAIDs?

Ii-SAID ezininzi zizifo zemfuza . Oko kukuthi, utshintsho oluthile (utshintsho) kwimfuza ethile lubangela isifo ngasinye. Khawuthelekelele ukuba imizimba yethu inemiyalelo (iimfuza) esixelela indlela yokwenza umsebenzi ngamnye, ngoko ke ukuba unobumba omnye kuloo ncwadi akachanekanga, unokubangela ukuba umsebenzi oseleyo uhambe gwenxa.

Nazi iintlobo eziphambili ze-SAIDs kunye ne-genes ezinxulumene nazo:

• I-FMF:I-gene ethi `MEFV` yiyo enika imiyalelo yokwenza iproteni `(pyrin)`.
• I-PFAPA: Isizathu esichanekileyo soku asikafunyanwa .
• IMIGXA: Ijini `TNFRSF1A`. Oku kufundisa ukuveliswa kweproteni ebizwa ngokuba yi`(tumor necrosis factor receptor - TNFR)`.
• MDK: Ijini ebizwa ngokuba yi-`MVK`. Oku kufundisa ukuveliswa kweproteni ebizwa ngokuba yi-`(mevalonic kinase)`.
• Izifo ze-NLRP3: I-gene `NLRP3`. Oku kufundisa ukuveliswa kweproteni ebizwa ngokuba yi`(cryopyrin)`.
• I-AOSD: Isizathu esichanekileyo soku asikafunyanwa .
• I-Blau syndrome: I-gene `NOD2`. Oku kufundisa ukuveliswa kweproteni ebizwa ngokuba yi`(NOD2)`.

Kubalulekile ukuqonda ukuba xa ezi zinto zibangelwa zizinto ezibangelwa yimfuza, asilotyala labazali. Ezi ziinkqubo zebhayoloji ezintsonkothileyo kakhulu.

Iingxaki ezibangelwa yi-SAIDs

Kubaluleke kakhulu ukufumana unyango olufanelekileyo kwezi meko. Kuba, ukuba ukudumba kuyaqhubeka emzimbeni, kunokukhokelela kwimeko ebizwa ngokuba yi-'(Amyloidosis). '(Amyloidosis)' yiproteni egciniweyo kwizintso . Oku kunokubangela umonakalo ongunaphakade kwizintso . Ke ngoko, ukuba kukho iimpawu, kubalulekile ukufuna ingcebiso kagqirha ngokukhawuleza.

Zichongwa njani ii-SAID?

Inyaniso kukuba, ukufumanisa i-SAIDs kunokuba nzima kancinci kuba iimpawu zinokufana nezezinye izifo ezinzulu, ezifana ne-lupus okanye i-lymphoma. Ngoko ke, kubalulekile ukubona ugqirha oqeqeshwe ngokukodwa kwizifo ezibangelwa kukudumba (ingcali yamathambo) ukuze axilonge ngokuchanekileyo aze alawule imeko yomntwana wakho.

Ingcali yezifo zamathambo yomntwana wakho iza kusebenzisa izinto ezininzi ukuxilonga ii-SAID. Uza kubuza ngeempawu zomntwana wakho kunye nokuba kukho nabani na kusapho lwakho okhe waba nezi meko (imbali yosapho lwebhayoloji). Ugqirha unokurhanela ukuba umntwana wakho unePeriodic Fever Syndrome ukuba:

• Ukuba unesifo somkhuhlane rhoqo
• Ukuba kukho umntu kusapho lwakhe okhe waba nefiva efana nale
• Ukuba ungomnye weqela labantu abanengxaki yomkhuhlane onjalo

Zeziphi iimvavanyo ezisetyenziswayo?

Ugqirha angacebisa iimvavanyo ezininzi ukuqinisekisa ukuxilongwa. Ezi zingabandakanya:

• Uvavanyo lwelabhoratri: Uvavanyo olufana ne-`(C-reactive protein - CRP)` kunye ne-`(Complete Blood Count - CBC)` lunokujonga ukudumba emzimbeni. Olu vavanyo luba `(positive)` xa kukho umkhuhlane, kwaye lubuyela kumaxabiso aqhelekileyo xa umkhuhlane uphela.
• Uvavanyo lomchamo:Uvavanyo lweprotheyini yomchamo lungenziwa ukujonga amanqanaba aphezulu eprotheyini kumchamo. Kwi-MKD, uvavanyo lomchamo lwee-organic acids luya kubonisa amanqanaba aphezulu e-mevalonic acid.
• Uvavanyo lwemfuza: Oku kunokujonga iinguqulelo zemfuza. Nangona kunjalo, abanye abantwana abane-SAIDs basenokungafumani uhlobo lwemfuza. Ke ngoko, nokuba iziphumo zovavanyo ``zingalunganga``, amathuba okuba ne-SAID awanakukhutshwa.

Ziphathwa njani ii-SAID?

Unyango lwe-SAIDs luyahluka ngokuxhomekeke kuhlobo kunye nobunzima besifo . Nangona ezi zifo zingenakunyangwa ngokupheleleyo, iimpawu zomntwana zinokulawulwa kakuhle ngokusebenzisa amayeza . Umzekelo, ukuba umntwana wakho ufumana la maqhuqhuva amaxesha ambalwa ngonyaka, amayeza okudambisa iintlungu kunye namayeza okulwa nokuvuvukala angengo-steroidal (NSAIDs) adla ngokulawula iimpawu. Nangona kunjalo, ukuba isifo sibi kakhulu, ugqirha unokucebisa ezinye iindlela zonyango.

Nazi ezinye zonyango ezinjalo:

• I-FMF: Oku kunokunyangeka ngeyeza elibizwa ngokuba yi-`(Colchicine)` ukunciphisa ukudumba. Ukuba awukwazi ukuthatha i-`(Colchicine)`, ungazama uhlobo lweyeza elibizwa ngokuba yi-`(Biologic)`.
• I-PFAPA: Ukudumba kwe-PFAPA kunokuncitshiswa ngokunika ii-steroids (ngokuqhelekileyo i-prednisone). Abanye abantwana baye bakwazi ukulawula iimpawu zabo ngokusebenzisa i-cimetidine, iyeza elisetyenziselwa ukunyanga izilonda zesisu.
• IINGXAKI: Iyeza elithi `(Canakinumab)` lisebenza kakhulu kwi-TRAPS. Kwakhona, amayeza okulwa nokuvuvukala abhalwe ngugqirha afana ne-`(Glucocorticoids)` anokunceda ekunciphiseni iimpawu.
• I-MKD: `(Canakinumab)` lukhetho olufanelekileyo kunyango lwe-MKD. Ukusebenzisa `(NSAIDs)` okanye `(Steroids)` ngexesha lomkhuhlane nako kunokuba luncedo.
• Izifo ze-NLRP3: Ezinye izixhobo zokulawula amasosha omzimba ezifana neCanakinumab kunye neRilonacept, Anakinra, ziyasebenza ekunyangeni izifo ze-NLRP3.
• I-AOSD: Iindidi ezahlukeneyo zamayeza okulwa nokuvuvukala zisetyenziselwa i-AOSD. Ezi ziquka `(Steroids)`, `(Amayeza okulwa nokuvuvukala aguqula izifo - ii-DMARD)`, kunye `(Biologics)`.
• I-Blau syndrome: Ngokuxhomekeke kwiimpawu zomntwana, amayeza okunciphisa amasosha omzimba, amayeza okuthintela isifo se-tumor necrosis factor (TNF), kunye/okanye amayeza asetyenziswa emehlo angazanywa.

Ingaba imeko ye-SAIDs iba ngcono ngokwayo?

Ezinye ii-SAID zinokuhlala ubomi bonke . Ezinye zinokuhlala iminyaka embalwa kwaye zibe ngcono xa ukhula. Nangona ezinye iimeko zihlala ubomi bonke, ukuphindaphinda komkhuhlane kunokuncipha ngokuhamba kwexesha kwaye iimpawu zisenokungabi nzima kangako . Ugqirha wakho angakunika iinkcukacha ezithile malunga nemeko ethile yomntwana wakho.

Ukunyamekela umntwana one-SAID kunokuba nzima. Mhlawumbi nawe ukhe wathetha, kodwa ungasebenzisa amava akho ukunceda umntwana wakho ukuba akhathalele kwaye umncede ajongane nale meko yobomi bakhe bonke.

Eyona nto ibalulekileyo kukufuna unyango kwiingcali ezinolwazi olunzulu ngeengxaki ze-SAIDs. Zinokunceda ekulawuleni iimpawu zomntwana wakho kwaye zimncede abe nobuntwana obungcono kangangoko.

Izinto ezibalulekileyo ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, masishwankathele ezinye zezinto ekufuneka uzikhumbule koko sithethe ngako.

• Ukuba umntwana wakho unefiva rhoqo, engachazwanga , thetha nogqirha ngayo loo nto. Isenokuba luphawu lwe-SAID.
• Ii-SAIDs ziyingxaki yenkqubo yomzimba yokuzikhusela , ayisosifo esiqhelekileyo esosulelayo.
• Ezi zahlukile kwizifo ezibizwa ngokuba yi-"Autoimmune" .
• Ixesha elininzi, ezi zinto zibangelwa zizinto ezibangela ufuzo .
• Kubalulekile ukubona iRheumatologist ukuze ufumane ukuxilongwa ngokuchanekileyo.
• Unyango lunokulawula iimpawu kakuhle .
• Unyango lwangethuba lubalulekile ukuthintela ukonakala kwezintso.

Ukuba uneminye imibuzo malunga noku, ungoyiki ukubuza ugqirha wosapho lwakho okanye ugqirha wabantwana. Baya kukwazi ukukunceda ngakumbi.

Ii -SAID, umkhuhlane ozuzwe njengelifa, umkhuhlane oqhelekileyo, umkhuhlane ebantwaneni, inkqubo yomzimba yokuzikhusela, izifo zemfuza, ingcali yezifo zamathambo