Uyazazi yini lezi zifo zezinso ezingavamile? (I-Atypical Hemolytic Uremic Syndrome - aHUS)

Cabanga ngamahlule egazi akhiwa ngaphakathi kwemithambo yegazi emincane emzimbeni wakho. Ikakhulukazi emithanjeni yegazi ezinso zakho. Uma la mahlule egazi evimba ukugeleza kwegazi ezinso zakho, izinkinga ezinkulu njengokwehluleka kwezinso zingavela. Yilokho okwenzekayo ku-Atypical Hemolytic Uremic Syndrome, noma i-aHUS. Nakuba kungekho ukwelashwa kwalesi simo, singaphathwa kahle ngokwelashwa okufanele .

Uyini umehluko phakathi kwe-HUS "ejwayelekile" ne-"atypical"?

Lesi simo, esibizwa ngokuthi i-aHUS, sihluke kancane ku-HUS "Ejwayelekile" esivame ukuyibona. Imbangela eyinhloko ye-HUS ejwayelekile izinhlobo ezithile zamagciwane abangela isifo sohudo, isibonelo, izifo ezibangelwa amagciwane njenge-`(E. coli)` kanye ne-`(Shigella)`. Kodwa-ke, imbangela ye-aHUS iyinkimbinkimbi kakhulu.

Lesi sifo, esibizwa ngokuthi i-aHUS, singavela kunoma yimuphi ubudala. Ezinganeni ezincane, sithinta abafana namantombazane ngokulinganayo. Kodwa-ke, njengoba bekhula, abesifazane banamathuba amaningi okuthola lesi sifo. Lokhu kungenxa yokuthi ukukhulelwa kungaba yimbangela yokuvela kwalesi sifo. Kuyisifo esingavamile kakhulu. Nakuba kungaziwa kahle ukuthi bangaki abantu abanalesi sifo, ezinye izifundo ziphakamisa ukuthi abantu ababili kuphela kwabayisigidi emazweni afana ne-United States abanalesi sifo.

Yini ebangela i-aHUS?

Iningi lamacala e-aHUS abangelwa ushintsho kuma-gene, olubizwa ngokuthi i-gene mutation . Kodwa-ke, akuwona wonke umuntu one-gene mutation ozoqala lesi sifo. Ukuze izimpawu zivele, kudingeka imbangela.

Kalula nje, umuntu onesifo esithathelwana ngofuzo angasithola lesi sifo uma kwenzeka enye yalezi zinto ezilandelayo:

• Ukukhulelwa
• Izimo ezithile ezithelelanayo
• Umdlavuza
• Eminye imithi
• Ukufakelwa isitho somzimba
• Ezinye izifo ezingamahlalakhona, isibonelo "i-systemic sclerosis" noma "i-hypertension embi"

Kodwa ngezinye izikhathi, cishe engxenyeni yeziguli ze-aHUS, odokotela abanalo ushintsho lwezakhi zofuzo abangaluthola. Sibiza lezi zimo ngokuthi "i-idiopathic." Lokho kusho ukuthi akukho sizathu esingatholakala. Abacwaningi bakholelwa ukuthi lesi simo sibangelwa ushintsho lwezakhi zofuzo olungakatholakali.

Ziyini izimpawu ze-aHUS?

Izimpawu ze-aHUS zivame ukuvela ngokuzumayo ngemva kwesifo. Lokhu kuqubuka kungaba kuncane noma kube kubi kakhulu. Ezimweni ezincane, izinso zingase zingathinteki kakhulu. Ezimweni ezimbi kakhulu, zingaholela ekuhlulekeni kwezinso.

Ezigabeni zokuqala, ungabona izimpawu ezifana nalezi:

• Ubuthakathaka obukhulu kanye nokungabi nampilo
• Ukuzizwa ungaphilile kahle

Lesi yisifo esiqhubekayo. Lokhu kusho ukuthi izimpawu ziya ziba zimbi ngokuhamba kwesikhathi, ikakhulukazi uma lesi sifo singatholakali kusenesikhathi. Nakuba izinso ziyizisulu eziyinhloko ze-aHUS, ezinye izitho ezifana nobuchopho, isibindi, amaphaphu, nenhliziyo nazo zingathinteka ukuvaleka kwemithambo yegazi.

I-aHUS itholakala kanjani?

Uma kungekho muntu emndenini wakho oke waba nalesi sifo ngaphambili, kungaba nzima kancane ukuthola i-aHUS. Udokotela ogxile ezifweni zezinso (udokotela wezinzwa) noma udokotela ogxile ezifweni zegazi (udokotela wezinzwa) ungcono kakhulu ukusithola. Bazobheka kakhulu lezi zimpawu ezintathu:

1. I-Hemolytic anemia

2. Ukwehla kwenani lama-platelet

3. Izinkinga zokusebenza kwezinso

Udokotela wakho angase acele ukuhlolwa okuhlukahlukene ukuqinisekisa lesi simo.

Yiziphi izindlela zokwelapha?

Kunezinhlobo ezimbili zemithi evunyelwe yi-FDA yokwelapha i-aHUS.

• `I-Eculizumab (i-Soliris)`
• `Ravulizumab-cwcz (Ultomiris)`

Zombili lezi zidakamizwa zingaphansi kweqembu elibizwa ngokuthi "ama-antibodies e-monoclonal." Kalula nje, lezi zingamaprotheni akhethekile enziwa elabhorethri asebenza njengama-antibodies akhiqizwa ngokwemvelo emizimbeni yethu. Le mithi ingandisa inani lama-platelet namaseli abomvu egazi egazini. Uma lokhu kwelashwa kuqalwa ekuqaleni kwalesi sifo, kungabuyisela emuva ukulimala kwezinso.

Le mithi inikezwa njengemijovo. Kodwa-ke, ingabangela imiphumela emibi efana nekhanda elibuhlungu, umkhuhlane, isicanucanu, kanye nobuhlungu bomzimba. Udokotela wakho uzokwazisa ngalokhu.

Ngaphezu kwalokho, ukwelashwa nge-plasma nakho kuyasetshenziswa ukwelapha izimpawu. Kulokhu, isiguli sinikezwa i-plasma ethathwe kumuntu ophilile noma i-plasma ikhishwe egazini lesiguli bese ithathelwa indawo yi-plasma enempilo.

Uma izinso zingasabeli kulezi zindlela zokwelapha, kungadingeka ukuthi kukhishwe i-dialysis yezinso noma kufakwe ukufakelwa kwezinso .

Izimo lapho udinga ukuya esibhedlela ngokushesha

Uma kuvela izimpawu ezilandelayo ezinzima, hamba ngokushesha uye eMnyangweni Wezimo Eziphuthumayo (i-ETU) wesibhedlela esiseduze.

• Ukukhishwa komchamo okuphansi kakhulu.
• Ukudideka noma ukulahlekelwa ukucaca kwengqondo.
• Ukuvuvukala okukhulu komzimba.
• Ubunzima bokuphefumula.

Ungalondoloza kanjani impilo yengqondo ngenkathi uphila nalesi sifo?

Kuvamile ukuzizwa ucindezelekile futhi ukhathazekile lapho uphila nesifo esingamahlalakhona njenge-aHUS. Uma ukugula kukubi kakhulu futhi unezimpawu eziningi, kulapho umthelela omkhulu empilweni yakho yengqondo. Kodwa akudingeki ubhekane nalokhu wedwa.

• Khuluma nomndeni wakho nabangane: Ukwabelana ngemicabango nemizwa yakho nomuntu omethembayo kungaba impumuzo enkulu.
• Khuluma nodokotela nabeluleki: Khuluma nodokotela wakho ngalokhu. Bangakudlulisela kumeluleki wezempilo yengqondo uma kudingeka.
• Cabanga ngempilo yakho iyonke: Lala ngokwanele. Thola izindlela zokunciphisa ukucindezeleka. Yidla ukudla okuhle ezinso zakho. Vivinya umzimba uma ukwazi. Ukuba nempilo enhle ngokomzimba nakho kuyakusiza ukuthi uhlale uphilile ngokwengqondo.

Nakuba lesi kuyisimo esihlala isikhathi eside, ngokuxilongwa kusenesikhathi, ukwelashwa okufanele , kanye nokunamathela ezeluleko zezokwelapha, ungalawula imiphumela yalesi sifo futhi uphile impilo evamile. Ngakho-ke, hlala uxhumane nodokotela wakho futhi ulandele uhlelo lwakho lokwelashwa.