Khawucinge nje, ngaphandle kwesizathu esicacileyo, uhlala ufumana umkhuhlane, amalungu abuhlungu, kunye namaqhuqhuva amancinci emzimbeni wakho wonke. Ezi ziyanyamalala kwiintsuku ezimbini okanye ezintathu, emva koko ziphinde zivele. Ukuba into efana nale iyenzeka kuwe okanye kumntwana wakho, unobangela usenokuba sisifo esingaqhelekanga abantu abaninzi abangazange beve ngaso. Namhlanje sithetha ngesifo esinye esinjalo, iCAPS.
Ithetha ukuthini i-CAPS?
Ngamafutshane, iiCryopyrin-Associated Periodic Syndromes, okanye iiCAPS, liqela lezifo ezingaqhelekanga kakhulu ezichaphazela inkqubo yethu yokuzikhusela komzimba. Ngokwesiqhelo, inkqubo yethu yokuzikhusela komzimba iyasikhusela kwiintsholongwane nakwezinye izifo. Nangona kunjalo, kule meko yeCAPS, ngenxa yesiphene kwinkqubo yokuzikhusela komzimba ngokwayo, iqala ukuhlasela imizimba yethu. Oku kubizwa ngokuba 'sisifo sokuvuvukala komzimba' kwisayensi yezonyango. Oku kubangela ukudumba okungeyomfuneko emzimbeni, okubangela iimpawu ezifana nokurhawuzelela kwesikhumba, umkhuhlane, iintlungu zamalungu kunye nokudumba, kunye namehlo abomvu.
Kukho iintlobo ezintathu eziphambili zeCAPS. Masibone ukuba zeziphi.
| Igama leSifo | Inkcazo kunye nobunzima |
|---|---|
| Isifo Sokudumba Komkhuhlane Weentsapho (i-FCAS) | Olu lolona hlobo lubi kakhulu kwiintlobo ezintathu zesifo. Njengoko igama lisitsho, iimpawu zibangelwa kukuchatshazelwa ngumkhuhlane. Iimpawu eziphambili ngumkhuhlane, iintlungu zamalungu, kunye nezilonda zolusu. Ngokwesiqhelo akubangeli monakalo emzimbeni ixesha elide. |
| Isifo sikaMuckle-Wells (MWS) | Oku kunzulu ngakumbi kune-FCAS. Iimpawu zinokubangelwa kungekuphela nje ngumkhuhlane, kodwa nakuxinzelelo kunye nokuzilolonga . Iimpawu zinokuba nzima, kwaye abanye abantu banokuva . |
| Isifo Sokudumba Kweenkqubo Ezininzi Zokuzalwa Kwabantwana Abasandul’ Ukuzalwa (NOMID) | Esi sesona sifo sibi kakhulu nesingaqhelekanga kweli qela lezifo.Uhlobo. Oku kuqala kwangoko nje ukuba umntwana azalwe. Kubangela ukudumba kwizitho ezininzi zomzimba. Kukwabizwa ngokuba yiCINCA. Ukuba akunyangelwanga kakuhle, kunokubangela umonakalo omkhulu engqondweni, emehlweni nakwamanye amalungu omzimba . |
Yintoni ebangela i-CAPS?
Eyona nto ibangela oku kukutshintsha kwejini emzimbeni wethu. Ngokuchanekileyo, esi sifo sibangelwa kukuguquka kwejini okubizwa ngokuba yi-`NLRP3` .
Cinga ngale gene ethi `NLRP3` njengesicwangciso esiyalela imizimba yethu ukuba yenze iproteni ebizwa ngokuba yi `cryopyrin`. Ngokwesiqhelo, le protein ethi `cryopyrin` inceda imizimba yethu ivelise into ebizwa ngokuba yi `interleukin-1 (IL-1)`, enceda imizimba yethu ukulwa nosulelo.
Kodwa ngenxa yokuba i-'NLRP3' gene kumntu one-CAPS itshintshile, iproteni ye-'cryopyrin' nayo ayisebenzi kakuhle. Ihlala inika umzimba umqondiso wokuba wenze i-'IL-1' eninzi kakhulu. Oku kuveliswa ngokugqithisileyo kwe-'IL-1' yikona okubangela ukudumba okungeyomfuneko emzimbeni kwaye kubangele iimpawu ze-CAPS.
Esi sifo singenzeka ukuba umntwana ufumana i-gene eguquliweyo kumzali omnye kuphela, nokuba ngumama okanye ngutata. Ngamanye amaxesha, i-gene inokutshintsha ngokuzenzekelayo esibelekweni, ngaphandle kwabazali.
Zithini iimpawu?
Uphawu oluphambili noluqhelekileyo lwe-CAPS kukuqhambuka okubonakala njengamaqhuqhuva amancinci, angarhawuzeliyo emzimbeni wonke . Oku kubonakala rhoqo ekuzalweni. Abanye abantu baba nezi zilonda rhoqo, kodwa ziba nzima ngakumbi xa kuvela ukuqhambuka. Ezinye iimpawu ziyahluka ngokuxhomekeke kuhlobo lwe-CAPS onayo.
Iimpawu ezinokubonwa kwi-FCAS
Ezi mpawu zihlala iintsuku ezi-1-2.
- Ifiva
- Amehlo avuvukileyo
- Ukugodola
- Intlungu yamalungu
- Isicaphucaphu
- Intloko ebuhlungu
Iimpawu ezibonwa kwi-MWS
Ezi mpawu ziyafika ziphele. Zinokuhlala iintsuku ezi-1-3 ngexesha.
- Ukuqhambuka kwesikhumba
- Umkhuhlane kunye nokubanda
- Ubomvu bamehlo
- Intlungu yamalungu
- Intloko ebuhlungu kakhulu
- Ukulahlekelwa kukuva (abantwana abane-MWS banokulahlekelwa kuko konke okanye inxalenye yokuva kwabo xa befikelela ebudaleni)
- Isisu esibuhlungu
Iimpawu ezibonwa kwi-NOMID
Ezi zezona mpawu zinzima kakhulu.
- Intloko ebuhlungu kakhulu
- Amehlo avuvukileyo
- Intlungu kunye nokudumba kwamalungu
- Ukuhlanza
- Ukulahlekelwa ngumbono
- Ukulahlekelwa kukuva
Kubalulekile: Ukuba i-NOMID ayinyangwa kakuhle, iproteni ebizwa ngokuba yi-amyloid inokufakwa kwizitho ezibalulekileyo ezifana nezintso kunye namalungu, nto leyo ebangela umonakalo omkhulu ekuhambeni kwexesha.
Indlela yokuxilonga esi sifo?
Xa ubona ugqirha wakho enezi mpawu, uza kuqala akubuze ngembali yakho yezonyango kunye neyentsapho yakho. Emva koko, baza kukuhlola kwaye, ukuba kuyimfuneko, banokukuthumela kwiimvavanyo ezithile, ezifana nezi:
- Uvavanyo lwegazi nomchamo: Olu vavanyo lujonga amanqanaba aphezulu eeseli ezimhlophe zegazi kunye neproteni esabelayo kwi-C, eziziimpawu zokudumba, kunye neeproteni ezigqithisileyo kumchamo.
- I-biopsy yolusu: Isampuli encinci kakhulu yolusu lwakho iyathathwa ize ihlolwe phantsi kwe-microscope.
- Uvavanyo lwemfuza: Olu vavanyo lunokuqinisekisa ukuba unayo na inguqu kwimfuza yakho ye-NLRP3. Nangona kunjalo, ngamanye amaxesha abantu abane-CAPS banokuba nayo le mfuza ngokuqhelekileyo.
- Uvavanyo lwamehlo nendlebe: Olu vavanyo lwenziwa ukuze kubonwe ukuba umbono okanye ukuva kwakho kuyachaphazeleka na.
- Ukubhobozwa kweLumbar: Kuthathwa ulwelo oluncinci lomqolo kwi-spinal cord kuze kuhlolwe iimpawu zokudumba.
- I-MRI scan: Oku kungathatha imifanekiso eneenkcukacha zezakhiwo ezingaphakathi kwengqondo neendlebe ukuze kubonwe ukuba kukho naluphi na utshintsho.
Imibuzo yokubuza ugqirha
Kungumbono omhle ukubhala phantsi imibuzo onayo ephepheni xa usiya kugqirha. Ngale ndlela, awuyi kulibala nto. Ungabuza imibuzo efana nale:
- Luhlobo luni lweCAPS endinalo (okanye umntwana wam)?
- Ziziphi iimpawu ezinokubangela uhlobo lwesifo endinaso?
- Loluphi unyango olucebisayo? Luza kundinceda njani?
- Ngaba kukho naziphi na iziphumo ebezingalindelekanga zonyango?
- Ndingenza ntoni ekhaya ukunciphisa iimpawu?
- Zeziphi ezinye iimvavanyo ekufanele ndizenze? Ndingazenza nini?
- Ngaba kufuneka ndidibane nomcebisi wezemfuza?
Zithini iindlela zonyango?
Kubaluleke kakhulu ukuqala unyango lwesi sifo ngokukhawuleza, kuba xa amehlo, iindlebe, amathambo, okanye ingqondo yonakele, kunzima kakhulu ukuzibuyisela kwimeko yesiqhelo.
Unyango oluphambili ngamayeza athintela ukuveliswa ngokugqithisileyo kwento ebizwa ngokuba yi-IL-1 emzimbeni. La mayeza alawula ukudumba.
- I-Anakinra (iKineret)
- I-Canakinumab (i-Ilaris)
- I-Rilonacept (i-Arcalyst)
Ukongeza kwezi, ugqirha wakho unokucebisa nezinye iindlela zonyango, ezifana nezi:
- Ukunxiba ii-splints zokunceda iintlungu kunye nokudumba kwamalungu
- Ulungiso lwenyama
- Amayeza anjengee-NSAID, ii-steroids, okanye i-methotrexate ukunciphisa umkhuhlane kunye nokudumba
- Izixhobo zokuncedisa ukuva kubantu abanengxaki yokuva
- Utyando ukuba amalungu onakele
Ungazinyamekela njani?
Kunye nonyango, ukwenza utshintsho oluncinci kwindlela ophila ngayo kunokunceda kakhulu ekulawuleni iimpawu.
- Kuphephe ukubanda: Ukuba unesifo esifana ne-FCAS, ukubanda kunokwenza iimpawu zakho zibe mandundu. Ngoko zama ukuzikhwebula kwizinto ezifana nokuhlala kumagumbi e-AC nokuhlala kwiindawo ezibandayo.
- Nciphisa uxinzelelo: Izinto ezinje ngokucamngca, ukuzilolonga ngokuphefumla nzulu, kunye neyoga zinokunceda kakhulu koku.
- Ubomi obusempilweni:
- Lala kamnandi.
- Yitya ukutya okunesondlo.
- Kuphephe ukutshaya, utywala, nokutya okucutshungulweyo.
Umyalezo Wokuya Ekhaya
- I-CAPS yimeko engaqhelekanga, yemfuza ebangelwa sisiphene kwinkqubo yomzimba yokuzikhusela.
- Iimpawu zayo eziphambili ngumkhuhlane ophindaphindayo, izilonda zesikhumba ezingarhawuzeliyo, kunye nentlungu yamalungu.
- Oku kubangelwa kukuguquka kwejini ekuthiwa yi
NLRP3. - Kubalulekile ukufumanisa isifo nokuqala unyango ngokukhawuleza ukuze kuthintelwe umonakalo ongunaphakade emehlweni, ezindlebeni nakwamanye amalungu omzimba.
- Ukuba wena okanye umntwana wakho unezinye zezi mpawu, ungaze uzityeshele kwaye qhagamshelana nogqirha wakho ngokukhawuleza ukuze akunike icebiso.
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