Ngaba uyayazi le sifo singaqhelekanga sezintso? (I-Atypical Hemolytic Uremic Syndrome - aHUS)

Khawucinge ngamahlwili egazi akhiwa ngaphakathi kwemithambo yegazi emincinci emzimbeni wakho. Ingakumbi kwimithambo yegazi kwizintso zakho. Xa la mahlwili egazi ethintela ukuhamba kwegazi ukuya kwizintso zakho, iingxaki ezinkulu ezifana nokungasebenzi kakuhle kwezintso zinokwenzeka. Yiloo nto eyenzekayo kwi-Atypical Hemolytic Uremic Syndrome, okanye i-aHUS. Nangona kungekho nyango lwale meko, inokulawulwa kakuhle ngonyango olufanelekileyo .

Yintoni umahluko phakathi kwe-HUS "eqhelekileyo" kunye ne-HUS "engaqhelekanga"?

Le meko, ebizwa ngokuba yi-aHUS, yahlukile kancinci kwi-HUS "eQhelekileyo" esiyibona rhoqo. Isizathu esiphambili se-HUS eqhelekileyo ziintlobo ezithile zeebhaktheriya ezibangela urhudo, umzekelo, usulelo lweebhaktheriya ezifana ne-`(E. coli)` kunye ne-`(Shigella)`. Nangona kunjalo, isizathu se-aHUS sinzima ngakumbi.

Esi sifo, esibizwa ngokuba yi-aHUS, sinokuvela nakweyiphi na iminyaka. Kubantwana abancinci, sichaphazela abafana namantombazana ngokulinganayo. Nangona kunjalo, njengoko bekhula, abafazi banamathuba amaninzi okufumana esi sifo. Oku kungenxa yokuba ukukhulelwa kunokuba yimbangela yokuba esi sifo sivele. Sisifo esingaqhelekanga kakhulu. Nangona kungaziwa ukuba bangaphi abantu abanale sifo, ezinye izifundo zibonisa ukuba ngabantu ababini kuphela kwisigidi kumazwe afana ne-United States abanale sifo.

Yintoni ebangela i-aHUS?

Uninzi lweemeko ze-aHUS zibangelwa lutshintsho kwiijini, olubizwa ngokuba yi-gene mutation . Nangona kunjalo, ayinguye wonke umntu one-gene mutation oza kuba nesi sifo. Ukuze kuvele iimpawu, kufuneka kubekho into ebangela eso sifo.

Ngamafutshane, umntu onesifo esidluliselwa kwimfuza unokuba nesifo ukuba kwenzeka enye yezi zinto zilandelayo:

• Ukukhulelwa
• Iimeko ezithile ezisulelayo
• Umhlaza
• Amanye amayeza
• Ukufakelwa amalungu omzimba
• Ezinye izifo ezingapheliyo, ezifana ne-"systemic sclerosis" okanye "uxinzelelo olubi lwegazi"

Kodwa ngamanye amaxesha, malunga nesiqingatha sezigulane ze-aHUS, oogqirha abanayo inguqu yemfuza abanokuyifumana. Ezi meko sizibiza ngokuba yi-"idiopathic." Oko kuthetha ukuba akukho sizathu sinokufunyanwa. Abaphandi bakholelwa ukuba le meko ibangelwa yinguqu yemfuza engekaziwa.

Zithini iimpawu ze-aHUS?

Iimpawu ze-aHUS zihlala zibonakala ngequbuliso emva kokuba kuvele into ebangela ukuba izintso ziqhume. Ezi zifo zinokuba buthathaka okanye zibe nzima. Kwiimeko ezincinci, izintso zisenokungachaphazeleki kakhulu. Kwiimeko ezinzima, zinokubangela ukuba izintso zingaphumeleli.

Kwizigaba zokuqala zesifo, unokubona iimpawu ezinjalo:

• Ubuthathaka obukhulu kunye nokungabi nabomi
• Ukuziva ungaphili kakuhle

Esi sisifo esiqhubekayo. Oku kuthetha ukuba iimpawu ziya zisiba mandundu ngokuhamba kwexesha, ingakumbi ukuba isifo asibonwanga kwangethuba. Nangona izintso zezona zijoliswe kakhulu kwi-aHUS, ezinye izitho ezifana nengqondo, isibindi, imiphunga kunye nentliziyo nazo zinokuchaphazeleka kukuvaleka kwemithambo yegazi.

Ifunyaniswa njani i-aHUS?

Ukuba akukho mntu kusapho lwakho owakha waba nesi sifo ngaphambili, kunokuba nzima kancinci ukufumanisa i-HUS. Ugqirha ogxile kwizifo zezintso (i-nephrologist) okanye ugqirha ogxile kwizifo zegazi (i-hematologist) ufanelekile kakhulu ukusifumanisa. Baza kukhangela ezi mpawu zintathu:

1. I-Hemolytic anemia

2. Ukwehla kwenani leeplatelet

3. Iingxaki zokusebenza kwezintso

Ugqirha wakho unokuyalela iimvavanyo ezahlukeneyo ukuqinisekisa le meko.

Ziziphi iindlela zonyango?

Kukho iintlobo ezimbini zamayeza avunyiweyo yi-FDA ukunyanga i-aHUS.

• `I-Eculizumab (iSoliris)`
• `Ravulizumab-cwcz (Ultomiris)`

Zombini ezi ziyobisi ziphantsi kweqela elibizwa ngokuba yi-"monoclonal antibodies." Ngamafutshane, ezi ziiproteni ezikhethekileyo ezenziwe kwilebhu ezisebenza njengee-antibodies eziveliswa ngokwendalo emizimbeni yethu. Ezi ziyobisi zinokunyusa inani lee-platelet kunye neeseli ezibomvu zegazi egazini. Ukuba olu nyango luqalwe kwangethuba kwesi sifo, lunokubuyisela umonakalo kwizintso.

La mayeza anikwa njengeenaliti. Nangona kunjalo, anokubangela iziphumo ebezingalindelekanga ezifana nentloko ebuhlungu, umkhuhlane, isicaphucaphu, kunye neentlungu zomzimba. Ugqirha wakho uza kukwazisa ngezi zinto.

Ukongeza, unyango lwe-plasma lukwasetyenziselwa ukunyanga iimpawu. Kule meko, isigulana sinikwa i-plasma ethathwe kumntu ophilileyo okanye i-plasma ikhutshwe egazini lesigulana ize ithatyathelwe indawo yi-plasma ephilileyo.

Ukuba izintso aziphenduli kwezi ndlela zonyango, i-kidney dialysis okanye i-kidney transplant inokuba yimfuneko.

Iimeko apho kufuneka uye esibhedlele ngoko nangoko

Ukuba kuvela ezi mpawu zilandelayo ziqatha, yiya kwiSebe leNgxamiseko (i-ETU) ngokukhawuleza kwisibhedlele esikufutshane.

• Ukuphuma komchamo kuphantsi kakhulu.
• Ukudideka okanye ukulahlekelwa kukucaca kwengqondo.
• Ukudumba okukhulu komzimba.
• Ubunzima bokuphefumla.

Ungayigcina njani impilo yengqondo ngelixa uphila nesi sifo?

Kuqhelekile ukuba uzive uxinezelekile kwaye uxhalabile xa uphila nesifo esinganyangekiyo njenge-aHUS. Okukhona isifo sinzima kwaye iimpawu zakho zisanda, kokukhona impembelelo enkulu kwimpilo yakho yengqondo iba nkulu. Kodwa akufuneki ujongane noku wedwa.

• Thetha nosapho lwakho nabahlobo bakho: Ukwabelana ngeengcinga zakho kunye neemvakalelo zakho nomntu omthembayo kunokuba luncedo kakhulu.
• Thetha noogqirha kunye nabacebisi: Thetha nogqirha wakho ngale nto. Banokukuthumela kumcebisi wezempilo yengqondo ukuba kuyimfuneko.
• Cinga ngempilo yakho iyonke: Lala ngokwaneleyo. Fumana iindlela zokunciphisa uxinzelelo. Yitya ukutya okunempilo kwizintso zakho. Yenza umthambo ukuba unako. Ukuba sempilweni emzimbeni kukwakunceda uhlale uphilile engqondweni.

Nangona le yimeko yobomi bonke, xa uxilongwa kwangethuba, unyango olufanelekileyo , kunye nokunamathela kwiingcebiso zonyango, ungayilawula imiphumo yesi sifo kwaye uphile ubomi obuqhelekileyo. Ngoko ke, hlala unxibelelana nogqirha wakho kwaye ulandele isicwangciso sakho sonyango.