Ingaba umntwana wakho uhlala enefiva engaziwayo? Oku kusenokuba kubangelwa zizifo ezibizwa ngokuba yi-SAIDs (Systemic Autoinflammatory Diseases)!

Ngaba umntwana wakho omncinci ufumana umkhuhlane rhoqo? Ngamanye amaxesha ufumana umkhuhlane kube kanye okanye kabini ngenyanga, kodwa oogqirha bathi akukho ntsholongwane, oko kukuthi, akukho ntsholongwane okanye iibhaktheriya emzimbeni. Ngamaxesha anjalo, njengomama okanye utata, unengqiqo kakhulu ukuziva uloyiko olukhulu kunye noxhala. "Kutheni oku kwenzeka kumntwana wam?" Mhlawumbi ucinge izihlandlo eziliwaka. Mhlawumbi isizathu soku yinto ongazange uyive ngaphambili. Namhlanje, siza kuthetha nge -SAIDs (Systemic Autoinflammatory Diseases), ezinzima ukuyiqonda kwaye zinokubangela umkhuhlane rhoqo. Ngaphambili, ezi zazibizwa ngokuba yi-`(Periodic Fever Syndromes)` okanye `(Recurrent Fever Syndromes)`.

Zithini ezi SAIDs (Systemic Autoinflammatory Diseases)?

Ngamafutshane, ii-SAID liqela lezifo ezenzeka ngenxa yokungasebenzi kakuhle okanye ingxaki yokulawula inkqubo yomzimba yethu yokuzikhusela . Okwenzekayo kukuba, ngaphandle kwesizathu sangaphandle, oko kukuthi, intsholongwane okanye intsholongwane, umzimba udala ukudumba nje. Yiyo loo nto lo mkhuhlane uxhaphakileyo uvela.

Ngoku usenokuba ucinga, "Owu, ingaba le yenye yezo Zifo Zokuzikhusela Ngokwasemzimbeni?" Hayi, ezi zimbini zahlukile kancinci. KwiZifo Zokuzikhusela Ngokwasemzimbeni, umzekelo, kwizifo ezifana neRheumatoid Arthritis okanye iLupus, inkqubo yomzimba wethu yokuzikhusela eguquguqukayo ihlasela ngempazamo iiseli zayo eziphilileyo. Kodwa kwi-SAIDs, ingxaki ikunkqubo yomzimba yokuzikhusela engaphakathi.

Ii-SAID zinqabile kakhulu kunezifo ezizikhuselayo emzimbeni. Uninzi lwexesha, ezi zizinto ezidluliselwa kwilifa , oko kuthetha ukuba zibangelwa kukuguquka kwezakhi zofuzo okanye uhlobo oluthile.

Ezi SAIDs zidla ngokuqala (kodwa azisoloko ziqala) xa umntwana wakho eselusana okanye esemncinci . Umntwana uya kuba neziqendu okanye ukuhlaselwa sisifo ngamaxesha athile. Ngezi xendu, umkhuhlane kunye nezinye iimpawu zinokuvela. Nangona kunjalo, phakathi kwezi "hlaselo," umntwana usenokungabi nazimpawu. Akukho nyango lweSAIDs, kodwa kudla ngokubakho unyango olusebenzayo lokulawula iimpawu.

Ziziphi iintlobo eziphambili ze-SAIDs?

Abaphandi bafumanise malunga neentlobo ezingama-60 ze-SAID, kwaye ezinye ziyafunyanwa. Nazi ezinye zeentlobo eziqhelekileyo:

• Umkhuhlane weMediterane wosapho (FMF): Le yeyona ixhaphakileyo kwizifo ezibangelwa lufuzo, eziphindaphindayo. Inokubangela ukudumba okubuhlungu esiswini, esifubeni nasemalungeni omzimba womntwana.
• Umkhuhlane odla ngokuvela rhoqo, i-aphthous-stomatitis, i-pharyngitis, i-adenitis (PFAPA): Oku kudla ngokuqala kubantwana abancinci, ngokuqhelekileyo ngaphambi kweminyaka emi-4. Nangona kunjalo, kwabanye abantwana, le meko inokuphela emva kweminyaka eli-10.
• I-Tumor necrosis factor receptor-associated periodic fever syndrome (i-TRAPS): Oku kunokwenzeka ukususela ebuntwaneni ukuya ebusheni, kwaye ngamanye amaxesha ukuya ebudaleni.
• Ukungabikho kwe-Mevalonate kinase (MKD): Ngaphambili yayisaziwa ngokuba yi-Hyper-IgD syndrome, oku kudla ngokuqala ngaphambi kokuba umntwana abe nonyaka omnye ubudala.
• Izifo ezibangelwa kukudumba okubangelwa yi-NLRP3: Oku ngaphambili kwakubizwa ngokuba yi-Cryopyrin-associated periodic syndromes (CAPS). Kukho ezinye iintlobo ezintathu ngaphakathi koku.
• Isifo sika-Adult-onset Still's (AOSD): Njengoko igama lisitsho, esi sifo siqala xa umntu emdala. Luhlobo lomntu omdala lwesifo esibizwa ngokuba yi-systemic juvenile idiopathic arthritis (JIA).
• Isifo se-granulomatous esinxulumene ne-NOD-2 (i-Blau syndrome): Esi sidla ngokuqala ngaphambi kokuba sifikelele kwiminyaka emi-4 ubudala. Sichaphazela kakhulu ulusu lomntwana, amehlo kunye namalungu.

Zithini iimpawu ze-SAIDs?

Iimpawu ze-SAIDs zihlala ziqala kwasebuntwaneni. Uphawu oluphambili noluqhelekileyo yimfiva eyenzeka rhoqo okanye eyenzeka rhoqo . Njengoko bekutshiwo ngaphambili, umntwana usenokuba sempilweni ngexesha apho kungekho mfiva. Nangona kunjalo, kukho ezinye iimpawu ezikhethekileyo kuhlobo ngalunye lwe-SAID:

• Kwi-FMF: Isisu somntwana, isifuba, kunye namalungu anokuba buhlungu kakhulu kwaye adumbe. Ngamanye amaxesha, kunokubakho irhashalala elukhumbeni kwimilenze esezantsi okanye emaqatheni.
• Kwi-PFAPA: Umphimbo obuhlungu, izilonda emlonyeni, ii-lymph nodes ezivuvukileyo entanyeni. Ezi zezona mpawu ziphambili.
• Kwi-TRAPS: Umzimba unokubanda kwaye ube nomkhuhlane. Umntwana unokuba neentlungu zemisipha emzimbeni nasezingalweni. Kusenokuvela irhashalala ebomvu ebuhlungu, enokusasazeka ukusuka ezingalweni ukuya emilenzeni ize iye emzimbeni.
• Kwi-MKD: Usenokuba neempawu ezifana nomkhuhlane ezifana nokugodola, intloko ebuhlungu, isisu esibuhlungu, ukungabi namdla wokutya, kunye nezinye iimpawu.
• Kwizifo ze-NLRP3: ukurhawuzelela kwesikhumba, iintloko ezibuhlungu, ukungondleki kakuhle, iintlungu zamalungu, kunye nokudumba kwamehlo (conjunctivitis) kunokwenzeka.
• Kwi-AOSD: ukurhawuzelela kwesikhumba, iintlungu zamalungu, iintlungu zemisipha. Abanye abantu banokuba nomphimbo obuhlungu, iintlungu zesisu, ukudinwa, kunye nokuziva bebuthathaka.
• Kwi-Blau syndrome: Umntwana usenokuba nezilonda zesikhumba ezingalweni, emilenzeni, okanye phakathi kwesinqe. Basenokuba nentlungu yamalungu kunye nentlungu yamehlo.

Khawucinge nje, intombi kaNalini ibisoloko inomkhuhlane ophakamileyo iintsuku ezintathu ukuya kwezine nyanga zonke kwiinyanga ezimbalwa ezidlulileyo. Kunye noko, ikwavelisa izilonda ezincinci emlonyeni wayo kunye namaqhuma entanyeni yayo. Xa wayibonisa ugqirha, wathi isenokuba yiPFAPA.

Zithini izizathu ze-SAIDs?

Uninzi lwee-SAID zizifo zemfuza . Oku kuthetha ukuba nganye kwezi syndromes ibangelwa kukwahluka kwemfuza yethu. Nazi iintlobo eziqhelekileyo ze-SAIDs kunye nemfuza ezizichaphazelayo:

• I-FMF: I-gene ebizwa ngokuba yi-`(MEFV)`. Le gene iyalela ukuveliswa kweproteni `(pyrin)`.
• I-PFAPA: Eyona jini ichaphazela oku ayikafunyaniswa.
• IMIGXA: I-gene ebizwa ngokuba yi-`(TNFRSF1A)`. Kulapho imiyalelo yokwenza iproteni `(i-tumor necrosis factor receptor - TNFR)` ivela khona.
• I-MKD: I-gene `(MVK)`. Oku kufundisa ukuveliswa kweproteni ebizwa ngokuba yi-`(mevalonic kinase)`.
• Izifo ze-NLRP3: Ijini ebizwa ngokuba yi-`(NLRP3)`. Oku kunika imiyalelo yokwenza iproteni ebizwa ngokuba yi-`(cryopyrin)`.
• I-AOSD: Isizathu esichanekileyo soku asikaziwa.
• I-Blau syndrome: I-gene `(NOD2)`. Oku kufundisa ukuveliswa kweproteni `(NOD2)`.

Iinkcukacha zezi jini zingabonakala zinzima kuwe. Ngamafutshane, ezi jini zichaphazela ukuveliswa kweeproteni ezithile ezilawula ukudumba emizimbeni yethu. Yiyo loo nto umzimba udala iimeko zokudumba ngaphandle kwesizathu.

Yintoni enokwenzeka ukuba ii-SAID azinyangwa kakuhle?

Kubaluleke kakhulu ukufumana unyango olufanelekileyo lwezi SAID. Kuba ukuba ukudumba emzimbeni kuyaqhubeka, imeko ebizwa ngokuba yi `(amyloidosis)` inokwenzeka. Oku kuthetha ukuba uhlobo oluthile lweproteni lufakwa kwizintso zethu, olunokubangela umonakalo ongunaphakade kwizintso . Ke ngoko, ukuba kukho iimpawu, kubaluleke kakhulu ukufuna ingcebiso kagqirha ngaphandle kokuzityeshela.

Oogqirha bazixilonga njani ii-SAID?

Ukuchonga ii-SAID kunokuba nzima kuba iimpawu zinokufana nezezinye izifo ezinzulu, ezifana ne-lupus okanye i-lymphoma. Ngoko ke , kungcono ukubona ingcali yamathambo, ugqirha oqeqeshwe ngokukodwa kwizifo ezibangelwa kukudumba , ukuze axilonge ngokuchanekileyo aze alawule imeko yomntwana wakho.

Ingcali yezifo zamathambo yomntwana wakho iza kujonga izinto ezininzi ukuze ifumanise ii-SAID. Iza kukubuza ngeempawu zomntwana wakho kunye nokuba kukho umntu kusapho lwakho okhe waba nomkhuhlane rhoqo. Ugqirha unokurhanela le meko ukuba:

• Ukuba umntwana usoloko enomkhuhlane .
• Ukuba kukho umntu kusapho okhe wanembali yomkhuhlane rhoqo .
• Ukuba umntwana ungowohlanga oluthile, oko kuthetha ukuba unokufumana umkhuhlane onjalo rhoqo.

Zeziphi iimvavanyo ezisetyenziswayo?

Ukuqinisekisa ukuxilongwa, ingcali yezifo zamathambo yomntwana wakho inokucebisa iimvavanyo ezininzi. Ezinye zezi ziquka:

• Uvavanyo lwelabhoratri: Uvavanyo lwegazi olufana ne-`(C-reactive protein - CRP)` kunye ne-`(Complete blood count - CBC)` lunokujonga ukudumba emzimbeni. Olu vavanyo luba `(positive)` xa unomkhuhlane kwaye lubuyela kwimeko yesiqhelo xa umkhuhlane uphelile.
• Uvavanyo lomchamo: Umchamo ujongwa ukuba unamanqanaba aphezulu eeproteni. Kwimeko ye-MKD, umchamo ujongwa ukuba unamanqanaba aphezulu e-organic acid ebizwa ngokuba yi-``mevalonic acid''.
• Uvavanyo lwemfuza: Uvavanyo lwemfuza lunokujonga iintlobo zemfuza ezikhankanyiweyo apha ngasentla. Nangona kunjalo, abanye abantwana abane-SAIDs basenokungazifumani iintlobo zemfuza, ngoko ke iziphumo zovavanyo zisenokuba azichanekanga.

Ziziphi iindlela zonyango ze-SAIDs?

Unyango lwe-SAIDs luxhomekeke kuhlobo kunye nobunzima bemeko . Akukho nyango lwezi meko, kodwa amayeza anokunceda ukulawula iimpawu zomntwana wakho . Ukuba umntwana wakho uhlaselwa kambalwa ngonyaka, unokulawula iimpawu ngeepilisi zokudambisa iintlungu kunye neziyobisi ezichasene nokuvuvukala ezingezizo ii-steroidal (NSAIDs). Nangona kunjalo, ukuba imeko inzima ngakumbi, ugqirha wakho unokucebisa ezinye iindlela zonyango:

• I-FMF: Oku kunokunyangeka ngeyeza elibizwa ngokuba yi-`(Colchicine)` ukunciphisa ukudumba. Ukuba i-`(Colchicine)` ayinakunikwa umntwana, ungazama uhlobo lweyeza elibizwa ngokuba yi-`(biologic)`.
• I-PFAPA: Ii-steroids ezifana ne-prednisone zinokunciphisa ixesha "lokuhlaselwa" kwe-PFAPA. I-Cimetidine, iyeza elinikwa abanye abantwana lezilonda zesisu, linokunceda ekunciphiseni iimpawu.
• II-TRAPS: Eli yeza `(Canakinumab)` lisebenza kakhulu kwi-TRAPS. Kwakhona, iimpawu zingalawulwa ngamayeza okulwa nokuvuvukala afana ne-`(glucocorticoids)` achazwe ngugqirha.
• I-MKD: `(Canakinumab)` ikwalunyango olusebenzayo lwe-MKD. `(NSAIDs)` okanye `(steroids)` zinokunceda ngexesha "lohlaselo".
• Izifo ze-NLRP3: Ii-immunomodulators ezifana neCanakinumab, iRilonacept, kunye ne-Anakinra zisetyenziswe ngempumelelo ukunyanga izifo ezinxulumene ne-NLRP3.
• I-AOSD: Amayeza ahlukeneyo okulwa nokuvuvukala afana nee-steroids, amayeza okulwa nokuvuvukala kwamathambo (ii-DMARD), kunye ne-biologics zisetyenziselwa ukunyanga i-AOSD.
• I-Blau syndrome: Ngokuxhomekeke kwiimpawu zomntwana, amayeza okunciphisa amasosha omzimba, amayeza okuthintela isifo se-tumor necrosis factor (TNF), kunye/okanye amayeza asetyenziswa emehlo angazanywa.

Ukufunda ngala mayeza kunokukwenza uzive usoyika kancinci. Kodwa khumbula, onke la mayeza anikwa phantsi kweliso elibukhali likagqirha, ngendlela efanela umntwana. Ngoko ke, kubalulekile ukulandela unyango kanye njengoko ugqirha etshilo.

Ingaba imeko ye-SAIDs iyaphela?

Ezinye ii-SAID ziimeko zobomi bonke . Ezinye zinokuhlala iminyaka embalwa kwaye ziphele xa ukhula . Ezinye iimeko zihlala ubomi bonke, kodwa ngokuhamba kwexesha, ukuhlaselwa rhoqo kunokuncipha kwaye iimpawu zingabi nzima kakhulu. Ugqirha wakho uza kukuchazela iinkcukacha zesimo somntwana wakho.

Ukunyamekela umntwana one-SAID kunokuba nzima. Ukuba nawe ukhe wayenza i-SAID, ungasebenzisa amava akho ukunceda ekunyamekeleni umntwana wakho. Ukuqonda kwakho kubalulekile ekuncedeni umntwana wakho aphile nale meko yobomi bakhe bonke.

Eyona nto ibalulekileyo kukufuna unyango koogqirha abanamava abanolwazi nge-SAIDs. Banokunceda ekulawuleni iimpawu zomntwana wakho kwaye bamncede abe nobuntwana obungcono kangangoko.

Izinto ezibalulekileyo ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, makhe siphinde sichaze ezinye zezona ngongoma zibalulekileyo ekufuneka uzikhumbule kwinto esithethe ngayo:

• Ukuba umntwana wakho unefiva rhoqo, engachazwanga , oko kungaba luphawu lwe-SAID.
• Ii-SAID azibangelwa lusulelo , kodwa yingxaki kwinkqubo yomzimba yokuzikhusela.
• Ezi zizifo ezingaqhelekanga ezidla ngokubangelwa zizizathu zemfuza .
• Iimpawu zinokwahluka ngokuxhomekeke kuhlobo lwe-SAID, kodwa umkhuhlane oqhelekileyo luphawu oluphambili .
• Kubalulekile ukubona i-Rheumatologist ukuze ufumane uxilongo oluchanekileyo kunye nonyango.
• Nangona unyango lungenakusinyanga ngokupheleleyo esi sifo, iimpawu zinokulawulwa kakuhle .
• Musa ukuzityeshela iimpawu, kuba zinokukhokelela ekulimaleni kwezintso ukuba azinyangwa.

Ukuba unemibuzo engaphezulu malunga noku, ungathandabuzi ukuthetha nogqirha wakho wosapho okanye ingcali yezifo zamathambo. Baya kukwazi ukukunceda ngakumbi.

Ii -SAID, izifo zomkhuhlane ophindaphindayo, umkhuhlane ophindaphindayo, izifo ezibangelwa kukudumba komzimba, umkhuhlane, umkhuhlane ebantwaneni, izifo zemfuza