Izinto ezi-6 Ozozibuza Udokotela Wakho Uma Ukwelashwa Kwakho Kwe-AHP Kungasebenzi

-Delile

• Ngazi kanjani ukuthi ngiphinde ngahlaselwa futhi?
• Ngabe kuzofanele ngiye esibhedlela?
• Yikuphi ukwelashwa okutholakalayo ehhovisi lakho?
• Ngidinga i-phlebotomy?
• Imiphi imishanguzo kadokotela esiza nge-AHP?
• Ngabe kukhona ushintsho lwendlela yokuphila oluzosiza?
• Ukudla okudlela endlini

Ukwelashwa kwe-acute hepatic porphyria (AHP) kuyahluka ngokuya ngezimpawu zakho nempilo yakho yonke. Ukuphatha isimo sakho kuyisihluthulelo sokuvimbela izinkinga.

Kodwa-ke, kubalulekile ukukhuluma nodokotela wakho uma izimpawu zakho ziba zimbi noma uhlaselwa kakhulu kunokujwayelekile.

Cabanga ngale mibuzo elandelayo njengesiqalo lapho uxoxa nodokotela wakho mayelana nokwelashwa kwe-AHP.

Ngazi kanjani ukuthi ngiphinde ngahlaselwa futhi?

Ngaphandle kohlelo oluphelele lokuphatha, ukuhlaselwa kwe-AHP kusenokwenzeka.

Izimpawu zingavela noma nini lapho umzimba wakho ungenayo i-heme eyanele yokwenza amaprotheni e-hemoglobin kumaseli akho abomvu egazi. Amaprotheni afanayo atholakala emisipheni nasenhliziyweni yakho.

Buza udokotela wakho ukuthi ngabe kukhona yini izimpawu okufanele uzibheke ezingakhombisa ukuhlaselwa kwe-AHP. Lokhu kungafaka:

• ubuhlungu obukhulayo
• ubuhlungu besisu
• isicanucanu
• ukuhlanza
• ukuphefumula kanzima
• ukwanda komfutho wegazi nokushaya kwenhliziyo
• ukoma komzimba
• isithuthwane

Ngabe kuzofanele ngiye esibhedlela?

Udokotela wakho angancoma ukuvakashelwa esibhedlela uma uhlaselwa yi-AHP. Izimpawu ezimnene kungenzeka zingakuvumeli ukulala esibhedlela njengokuhlaselwa okunzima.

Kufanele uye esibhedlela uma unezinguquko ezinkulu ekucindezelekeni kwegazi noma ekushayeni kwenhliziyo, ekuqulekeni, noma ulahlekelwa ukwazi. Ubuhlungu obukhulu bungabhekiswa esibhedlela, futhi.

Uma usesibhedlela, unganikezwa imishanguzo ngemithambo ukuze unqande ngokushesha ukuhlaselwa. Udokotela wakho futhi angakuqapha ngezinkinga ezinzima ngezinso zakho noma isibindi.

Uma ungaqiniseki ukuthi udinga ukuya esibhedlela yini, shayela udokotela wakho noma umcele ukuthi akunikeze inombolo yocingo yangemuva kwamahora ongayishayela ukuthola izeluleko.

Yikuphi ukwelashwa okutholakalayo ehhovisi lakho?

Izindlela eziningi zokwelashwa eziphuthumayo ezitholakalayo ze-AHP esibhedlela ziyatholakala nasehhovisi likadokotela wakho.

Lokhu kuvame ukunikezwa ngemithamo ephansi njengengxenye yohlelo lwesondlo, kunokuba kube ukwelashwa okuphuthumayo.

Ukwelashwa okunjalo kufaka:

• I-glucose efakwa emithanjeni: isiza ukulawula amazinga e-glucose uma ungatholi ngokwanele ukudala amangqamuzana egazi abomvu
• i-hemin emthanjeni: uhlobo lokwenziwa lwe-heme olunikezwa izikhathi ezimbalwa ngenyanga ukuvimbela ukuhlaselwa yi-AHP
• hemin imijovo: uhlobo lokuphathwa kwe-heme lunconywa uma umzimba wakho wenza ama-porphyrin amaningi kakhulu hhayi i-heme eyanele
• phlebotomy: inqubo yokususa igazi ehlose ukususa i-iron eyeqile emzimbeni
• i-gonadotropin-ikhulula i-hormone agonistumuthi kadokotela osetshenziselwa abesifazane ukulahlekelwa i-heme ngesikhathi sabo sokuya esikhathini
• izindlela zokwelapha zofuzo: lokhu kufaka phakathi i-givosiran, eyehlisa izinga lapho imikhiqizo enobuthi ikhiqizwa khona esibindi

Ngidinga i-phlebotomy?

I-phlebotomy isetshenziswa kuphela ku-AHP uma unensimbi eningi egazini lakho. I-Iron ibalulekile ekwakheni nasekulondolozeni amangqamuzana egazi abomvu, kepha amazinga aphezulu angadala ukuhlaselwa kwe-AHP.

I-Phlebotomykunciphisa izitolo zensimbi, okuthuthukisa i-heme synthesis ephazanyiswe ukuvinjelwa kwe-ferro-mediated kwe-uroporphyrinogen decarboxylase. Ukuhlolwa kwegazi njalo kungasiza ukuqinisekisa ukuthi i-ayoni yakho isezingeni elifanele.

Uma udinga i-phlebotomy, ingenziwa ngokuya ngaphandle. Ngesikhathi senqubo, udokotela wakho uzosusa elinye igazi lakho ukuze kususwe i-iron eyeqile.

Imiphi imishanguzo kadokotela esiza nge-AHP?

Uma unamazinga aphansi kashukela kodwa awudingi i-glucose IV, udokotela wakho angancoma amaphilisi kashukela.

Amanye ama-hormone agonists nawo angasiza abesifazane abasesikhathini. Ngesikhathi sokuya esikhathini, ungaba sengozini yokulahlekelwa i-heme eningi.

Udokotela wakho angakunika i-leuprolide acetate, uhlobo lwe-gonadotropin-ikhipha i-hormone agonist. Lokhu kuzosiza ukuvimbela ukulahleka okuqhubekayo kwe-heme phakathi nemijikelezo yakho yokuya esikhathini, okungavimbela ukuhlaselwa kwe-AHP.

Imithi yokwelapha efana ne-givosiran (Givlaari) nayo ingahle inqunyelwe ukunciphisa umkhiqizo onobuthi wesibindi. Igivosiran evunyelwe ngoNovemba 2019.

Ngabe kukhona ushintsho lwendlela yokuphila oluzosiza?

Ukudla, imithi, kanye nokukhethwa kwendlela yokuphila kwesinye isikhathi kungadala i-AHP. Ukunciphisa lezi zimbangela - noma ukuzigwema - kungasiza ukusekela uhlelo lwakho lokwelashwa futhi kunciphise ubungozi bokuhlaselwa.

Tshela udokotela wakho ngayo yonke imishanguzo, izithasiselo, nemikhiqizo oyisebenzisayo.

Ngisho nesengezo sokuthengwa kwamanani singaphazamisa isimo sakho. Ezinye zezinto ezivame kakhulu ukubekwa esikhundleni kwama-hormone nezengezo zensimbi.

Ukubhema nokuphuza kungenza i-AHP yakho ibe yimbi kakhulu. Ayikho inani lokubhema eliphilile. Kepha abanye abantu abadala abane-AHP bangakwazi ukuphuza ngokulinganisela. Buza udokotela wakho ukuthi ngabe kunjalo kuwe.

Zama ukunamathela kuhlelo lokudla olunempilo nokuzivocavoca. Uma une-AHP, ukuzincisha ukudla kungayiqeda i-heme futhi kwenze izimpawu zakho zibe zimbi kakhulu.

Uma udinga ukwehlisa isisindo, cela udokotela wakho akusize udale uhlelo lokunciphisa isisindo olungazimbi izimpawu zakho.

Ekugcineni, dala uhlelo lokusiza ukucindezeleka bese ulusebenzisa. Ayikho impilo yomuntu engenakho ukucindezeleka futhi ukuba nesimo esiyinkimbinkimbi njenge-AHP kungadala ukucindezeleka okuqhubekayo. Uma ucindezeleke ngokwengeziwe, iba sengozini enkulu yokuhlaselwa.

Ukudla okudlela endlini

I-AHP yisifo esingajwayelekile futhi esiyinkimbinkimbi. Kuningi okusazofundwa ngakho. Kubalulekile ukuhlala uthintana nodokotela wakho ubatshele uma ungacabangi ukuthi uhlelo lwakho lokwelashwa luyasebenza.

Ukukhuluma nodokotela wakho kungabasiza ukuthi baqonde isimo sakho futhi bancome ukwelashwa okusebenzayo.