Ngemuva kokuxilongwa kwe-AHP: Sibutsetelo se-Acute Hepatic Porphyria

-Delile

• Ukuxilongwa
• Izimpawu zokuqapha
• Ukwelashwa
• Ukuhlolwa kwemitholampilo
• Ukuphatha ukuhlaselwa
• Ukwenza izinguquko endleleni yokuphila
• Ukucindezeleka nempilo yengqondo
• Ukuhlolwa kofuzo
• Ukudla okudlela endlini

I-acute hepatic porphyria (AHP) ihilela ukulahleka kwamaprotheni we-heme asiza ekwenzeni amaseli abomvu aphilile enempilo. Ezinye izimo eziningi zabelana ngezimpawu zalesi sifo segazi, ngakho-ke ukuhlolwa kwe-AHP kungathatha isikhathi.

Udokotela wakho uzokuxilonga nge-AHP ngemuva kokuhlolwa kwegazi, umchamo, nokuhlolwa kofuzo. Ngemuva kokuxilongwa kwakho, inqubo yokwelashwa nokuphathwa ingaqala.

Ukuxilongwa kwe-AHP kungaphakamisa imibuzo eminingi. Ungase uzibuze ngezinketho zakho zokwelashwa nezinye izinyathelo ongazithatha ukuvimbela ukuhlaselwa okuzayo.

Funda kabanzi ngezinyathelo wena nodokotela wakho eningazithatha ngokulandela ukuxilongwa kwe-AHP.

Ukuxilongwa

Kuvamile ukuthi i-AHP ibe ekuqaleni ngenxa yokuvela kwayo okuphansi nezimpawu ezibanzi. Ithimba lakho lezokunakekelwa kwempilo lizosebenzisa izivivinyo eziningi ukuhlola izimpawu futhi licabangele ukuxilongwa okunamandla kwe-hepatic porphyria.

Ukuhlolwa kufaka:

• ukuhlolwa komchamo kwe-porphobilinogen (PBG)
• ukuskena kwe-computed tomography (CT)
• X-ray esifubeni
• i-echocardiogram (EKG)
• ukubalwa kwegazi okuphelele (CBC)
• ukuhlolwa kofuzo

Ukuhlolwa komchamo we-PBG kuvame ukubhekwa njengokubaluleke kakhulu ngoba umchamo we-PBG uvame ukuphakama ngesikhathi sokuhlaselwa okunamandla.

Ukuxilongwa kuvame ukuqinisekiswa ngokuhlolwa kofuzo komuntu ohlolwayo kanye namalungu omndeni wakhe.

Izimpawu zokuqapha

Ingxenye yohlelo oluhle lokuphathwa kwe-AHP ukuqonda izimpawu zokuhlaselwa. Lokhu kuzokusiza wazi ukuthi kufanele wenze nini ngaphambi kokuthi kuholele ezinkingeni ezinkulu.

Ngokusho kweNational Institutes of Health, ubuhlungu obukhulu besisu buyisifo esivame kakhulu sokuhlaselwa kwe-AHP okuseduze. Ubuhlungu bungadlulela kwezinye izingxenye zomzimba wakho, njengezi:

• izingalo
• imilenze
• emuva

Ukuhlaselwa kwe-AHP nakho kungadala:

• ubunzima bokuphefumula, njengokuphefumula noma ukuzwa okuqinile emphinjeni wakho
• ukuqunjelwa
• umchamo onombala omnyama
• ubunzima bokuchama
• umfutho wegazi ophakeme
• ukukhuphuka kwenhliziyo noma ukushaya kwenhliziyo okubonakalayo
• isicanucanu
• ukoma oluphenduka ukomisa amanzi emzimbeni
• ukuquleka noma ukusangana
• ukuhlanza
• imisipha ebuthakathaka

Shayela udokotela wakho uma uhlangabezana nalezi zimpawu ezingenhla. Udokotela wakho angakuqondisa esibhedlela ukuze uthole ukwelashwa.

Ukwelashwa

Izindlela zokuzivikela ziyisihluthulelo sokumisa ukuhlaselwa kwe-AHP nokwenza ngcono impilo yakho. Udokotela wakho cishe uzokunika uhlobo oluthile lwe-heme olubizwa ngokuthi i-hemin, oluzosiza umzimba wakho wenze amaprotheni e-hemoglobin.

I-Heme iyatholakala njengomuthi kadokotela womlomo, kepha inganikezwa futhi njengomjovo. AmaHemin IVs asetshenziswa ezibhedlela ngesikhathi sokuhlaselwa kwe-AHP.

Ngokuya ngesimo sakho, udokotela wakho angancoma okulandelayo:

• Izithako ze-glucose unganikezwa ngomlomo njengamaphilisi kashukela noma ngemithambo yegazi ukusiza umzimba wakho ube ne-glucose eyanele yokwenza amangqamuzana abomvu egazi.
• I-Gonadotropin-ikhulula i-hormone agonist ngumuthi kadokotela osetshenziselwa abesifazane abalahlekelwa i-heme ngesikhathi sokuya esikhathini.
• I-Phlebotomy inqubo yokususa igazi esetshenziselwa ukuqeda insimbi eyeqile emzimbeni.
• Ukwelashwa kwe-Gene njenge-givosiran, yona ngoNovemba 2019.

IGivosiran ibizimisele ukwehlisa izinga lemikhiqizo enobuthi ekhiqizwa esibindini, okuholele ekuhlaselweni okuncane kwe-AHP.

Ukukhetha imishanguzo efanele kudinga nokuhlolwa njalo kwegazi. Udokotela wakho angalinganisa i-heme, i-iron, nezinye izinto ukuze abone ukuthi ukwelashwa kwakho kuyasebenza yini noma uma udinga ukulungisa okuthile kuhlelo lwakho lwe-AHP.

Ukuhlolwa kwemitholampilo

Abaphenyi bazama ukukhomba nokwakha izindlela ezintsha zokwelapha ezifana ne-givosiran ukusiza ukuphatha lesi simo. Ungacabanga ukubuza udokotela wakho nganoma yiziphi izivivinyo zokwelashwa ezingakulungela kahle.

Lezi zivivinyo zinganikeza ukwelashwa kwamahhala, kanye nesinxephezelo. Ungafunda futhi kabanzi nge-ClinicalTrials.gov.

Ukuphatha ukuhlaselwa

Ukuphatha i-AHP kuvame ukuncika ekuphatheni izimbangela. Kodwa lapho ukuhlaselwa kwenzeka, kubalulekile ukufuna ukwelashwa nokuqeda izinhlungu.

Ukuhlaselwa kwe-AHP kuvame ukudinga ukulaliswa esibhedlela. Lapho unganikezwa i-heme ngemithambo yegazi ngenkathi ubhekwe izimpawu zezinso noma ukwehluleka kwesibindi.

Akukhona konke ukuhlaselwa kwe-AHP okudinga ukuvakashelwa esibhedlela. Kodwa-ke, ubuhlungu obukhulu noma izimpawu ezibalulekile kungenzeka zidinge ukunakekelwa okuphuthumayo.

Udokotela wakho angakunikeza imithi, efana ne-beta-blockers yomfutho wegazi ophakeme, i-antiemetic yokuhlanza, noma imishanguzo yokuqeda izinhlungu, ukwelapha izimpawu zokuhlaselwa

Ukwenza izinguquko endleleni yokuphila

Yize lungekho uhlelo oluthile lokuphila olungenza i-AHP ihambe, kunezinye izimbangela ze-AHP okufanele wazi.

Lokhu kufaka phakathi:

• ukudla amaprotheni amaningi
• ukuzila ukudla
• ukudla okuphezulu kwensimbi
• imishanguzo yokushintsha ama-hormone
• Ukudla okuphansi kwekhalori
• ukudla okuphansi kwe-carb
• izithako zensimbi (i-OTC noma incwadi kadokotela)
• ukubhema

Ukucindezeleka nempilo yengqondo

Ukuba nesifo esingelapheki njenge-AHP kungakucindezela, ikakhulukazi ngoba kuyisifo esiyivelakancane. Kubalulekile ukuphatha ukucindezeleka kwakho ngangokunokwenzeka.

Ngenkathi ukucindezeleka kungeyona imbangela eqondile yokuhlaselwa kwe-AHP, kungakhuphula ingozi yakho eyodwa.

I-Porphyrias nayo ingaholela kwezinye izimo zempilo yengqondo, njenge:

• ukukhathazeka
• ukudana
• impisi
• phobias

Gcina abahlinzeki bakho bezokunakekelwa kwempilo bevuselelwa kunoma yiziphi izimpawu zempilo yengqondo okungenzeka uhlangabezana nazo, njenge:

• uvalo
• ukuqwasha
• ukucasuka
• ukulahlekelwa intshisekelo emisebenzini yakho ejwayelekile

Izimpawu ezinjalo zingabhekiswa njengengxenye yohlelo lwakho lokunakekelwa kwezempilo.

Awuwedwa ekubhekaneni nezimpawu zakho ze-AHP, ngakho ukufinyelela kwabanye kungasiza kakhulu.

Ukuhlolwa kofuzo

Uma uthola ukuthi une-AHP, udokotela wakho angancoma ukuhlolwa kofuzo kwezingane zakho noma amanye amalungu omndeni.

Udokotela wakho angafuna ama-enzyme athile esibindi ukusiza ukuthola ukuthi izihlobo zakho eziphilayo zisengozini ye-AHP.

Ukuhlolwa kofuzo akukwazi ukuvimbela ukuqala kwe-AHP, kepha kungasiza abantu obathandayo ukuthi babheke ukuthuthukiswa kwezimpawu ezihlobene.

Ukudla okudlela endlini

Ukuthola ukuxilongwa kwe-AHP kungakucindezela ekuqaleni, kepha udokotela wakho ukhona ukuphendula yonke imibuzo yakho futhi aqiniseke ukuthi uthola ukwelashwa okuhle kakhulu.

Umbono wabantu abane-AHP muhle. Ukuphatha izimpawu zakho ngezindlela zokwelashwa nezinguquko kwendlela yokuphila, kungakusiza ukwenza imisebenzi yakho yansuku zonke ngezinkinga ezimbalwa.