Emva kokuchongwa kwe-AHP: Amagqabantshintshi e-Acute Hepatic Porphyria

Umxholo

• Uxilongo
• Iimpawu zokubeka iliso
• Unyango
• Uvavanyo lwezonyango
• Ukulawula uhlaselo
• Ukwenza utshintsho kwindlela yokuphila
• Uxinzelelo kunye nempilo yengqondo
• Uvavanyo lwemfuzo
• Yise kude

I-acute hepatic porphyria (AHP) ibandakanya ukuphulukana neeproteni ze-heme ezinceda ukwenza iiseli ezibomvu ezisempilweni. Ezinye iimeko ezininzi zabelana ngeempawu zokuphazamiseka kwegazi, ke ukuvavanywa kwe-AHP kungathatha ixesha.

Ugqirha wakho uya kukufumanisa ukuba une-AHP emva kwegazi, umchamo kunye novavanyo lofuzo. Emva kokuxilongwa, unyango kunye nenkqubo yolawulo ingaqala.

Ukuxilongwa kwe-AHP kunokuphakamisa imibuzo emininzi. Unokuzibuza malunga nokhetho lwakho kunye namanye amanyathelo onokuwathatha ukuthintela uhlaselo lwexesha elizayo.

Funda ngakumbi ngamanyathelo onokuwathatha kunye nogqirha emva kokuxilongwa kwe-AHP.

Uxilongo

Kuqhelekile ukuba i-AHP iqale ngenxa yokuvela kwayo okuphantsi kunye neempawu ezibanzi. Iqela lakho lokhathalelo lwempilo liza kusebenzisa iimvavanyo ezininzi ukukhangela iimpawu kunye nokuqwalaselwa kwesifo se-hepatic porphyria.

Uvavanyo lubandakanya:

• Uvavanyo lomchamo lwe-porphobilinogen (PBG)
• Iskena sekhompyuter ye-tomography (CT)
• X-reyi yesifuba
• i-echocardiogram (EKG)
• ubalo lwegazi olupheleleyo (CBC)
• uvavanyo lwemfuza

Uvavanyo lomchamo lwe-PBG luhlala luthathwa njengeyona nto ibaluleke kakhulu kuba umchamo we-PBG uhlala uphakanyisiwe ngexesha lokuhlaselwa ngamandla.

Ukuchongwa kuhlala kuqinisekiswa kuvavanyo lwemfuzo lomntu lowo kuvavanywa kunye namalungu osapho.

Iimpawu zokubeka iliso

Inxalenye yesicwangciso esihle solawulo lwe-AHP kukuqonda iimpawu zohlaselo. Oku kuya kukunceda wazi ukuba kufuneka wenze nini ngaphambi kokuba kukhokelele kubunzima obukhulu.

NgokweZiko leSizwe lezeMpilo, iintlungu eziswini zesisu lolona phawu luqhelekileyo lokuhlaselwa kwe-AHP. Intlungu inokudlulela kwamanye amalungu omzimba wakho, anje ngala:

• iingalo
• imilenze
• umva

Ukuhlaselwa kwe-AHP kunokubangela:

• ubunzima bokuphefumla, njengokuvutha okanye imvakalelo exineneyo emqaleni wakho
• ukuqhina
• umchamo omnyama-mnyama
• ubunzima bokuchama
• igazi elonyukayo
• ukonyuka kwentliziyo okanye ukubetha kwentliziyo okubonakalayo
• isicaphucaphu
• ukunxanwa okujika kube kukuphela kwamanzi
• ukuxhuzula okanye ukubona izinto ezingekhoyo kuzo
• ukugabha
• buthathaka izihlunu

Biza ugqirha wakho ukuba unazo naziphi na kwezi mpawu zingasentla. Ugqirha wakho angakuhambisa esibhedlele ukuze ufumane unyango.

Unyango

Amanyathelo othintelo aphambili ekuyekiseni uhlaselo lwe-AHP kunye nokuphucula umgangatho wobomi. Ugqirha wakho unokuyalela uhlobo oluthile lwehemme ekuthiwa yihemin, eya kuthi incede umzimba wakho wenze iiproteni zehemoglobin.

I-Heme iyafumaneka njengommiselo womlomo, kodwa inokunikwa kwakhona ngenaliti. I-Hemin IVs zisetyenziswa ezibhedlele ngexesha lokuhlaselwa kwe-AHP.

Kuxhomekeka kwimeko yakho, ugqirha wakho unokucebisa ngezi ndlela zilandelayo:

• Izixhobo zeswekile unokunikezwa ngomlomo njengeepilisi zeswekile okanye ngaphakathi ngaphakathi ukunceda umzimba wakho ube ne-glucose eyaneleyo yokwenza iiseli ezibomvu zegazi.
• I-Gonadotropin-ekhupha i-hormone agonist lunyango olusetyenziselwa abantu ababhinqileyo abaphulukana ne-heme ngexesha lokuya exesheni.
• Phlebotomy yinkqubo yokususa igazi esetyenziselwa ukuphelisa ubungakanani obugqithisileyo bentsimbi emzimbeni.
• Unyango lwe-Gene njenge-givosiran, ethi ngo-Novemba ngo-2019.

I-Givosiran yayizimisele ukuba yehlise inqanaba apho iimveliso zetyhefu ziveliswa esibindini, zikhokelela kuhlaselo oluncinci lwe-AHP.

Ukukhetha unyango olululo kufuna uvavanyo lwegazi rhoqo. Ugqirha wakho unokulinganisa i-heme, i-iron, kunye nezinye izinto ukuze abone ukuba unyango lwakho luyasebenza okanye ufuna uhlengahlengiso kwisicwangciso sakho se-AHP.

Uvavanyo lwezonyango

Abaphandi bazama ukuchonga kunye nokuphuhlisa unyango olutsha njenge-givosiran ukunceda ukulawula le meko. Unokucinga ngokubuza ugqirha wakho malunga naluphi na uvavanyo lwezonyango olunokuba lulungele wena.

Ezi zilingo zinokubonelela ngonyango lwasimahla, kunye nembuyekezo. Unokufunda ngakumbi ngeClinicalTrials.gov.

Ukulawula uhlaselo

Ukulawula i-AHP kuhlala kuxhomekeke kulawulo lokucutha. Kodwa xa uhlaselo lwenzeka, kubalulekile ukuba ufune unyango kunye nokunciphisa iintlungu.

Uhlaselo lwe-AHP ludla ngokufuna ukulaliswa esibhedlele. Apho unokunikwa ihemme ngaphakathi ngaphakathi ngelixa ubekwe iliso kwiimpawu zezintso okanye ukusilela kwesibindi.

Ayilulo lonke uhlaselo lwe-AHP olufuna utyelelo esibhedlele. Nangona kunjalo, iintlungu ezibi kakhulu okanye iimpawu ezibonakalayo ziya kuthi zifuna ukhathalelo olungxamisekileyo.

Ugqirha wakho unokukunika amayeza, anjenge-beta-blockers yoxinzelelo lwegazi oluphezulu, i-antiemetic yokugabha, okanye amayeza okuphelisa iintlungu, ukunyanga iimpawu zohlaselo

Ukwenza utshintsho kwindlela yokuphila

Ngelixa kungekho sicwangciso sithile sendlela yokuphila sinokwenza i-AHP ihambe, zikhona ezinye izinto ezibangela i-AHP ekufuneka uyazi.

Oku kubandakanya:

• ukutya iiprotein ezininzi
• ukuzila ukutya
• ukutya okuphezulu kwentsimbi
• amayeza okubuyisela i-hormone
• izidlo zekhalori ezisezantsi
• izidlo zecarb ezisezantsi
• izongezo zentsimbi (i-OTC okanye amayeza)
• ukutshaya

Uxinzelelo kunye nempilo yengqondo

Ukuba nesifo esinganyangekiyo njenge-AHP kunokuba noxinzelelo, ngakumbi kuba sisifo esinqabileyo. Kubalulekile ukulawula uxinzelelo lwakho kangangoko.

Ngelixa uxinzelelo ayingonobangela ngqo wokuhlaselwa kwe-AHP, inokunyusa umngcipheko wakho.

I-Porphyrias inokukhokelela kwezinye iimeko zempilo yengqondo, ezinje:

• ixhala
• ukudakumba
• ngomsindo
• ifumanas

Gcina ababoneleli bakho bezempilo behlaziywa nakweyiphi na impawu zempilo yengqondo onokuba uhlangabezana nazo, ezinje:

• uloyiko
• ukuphuthelwa
• ukucaphuka
• Ukuphelelwa ngumdla kwimisebenzi yakho yesiqhelo

Ezo mpawu zinokuqwalaselwa njengenxalenye yesicwangciso sakho sezempilo.

Awuwedwa ekujonganeni neempawu zakho ze-AHP, ke ukufikelela kwabanye kunokuba luncedo kakhulu.

Uvavanyo lwemfuzo

Ukuba ufunyaniswe une-AHP, ugqirha wakho unokucebisa ukuvavanywa kofuzo kubantwana bakho okanye amanye amalungu osapho.

Ugqirha wakho unokujonga ii-enzymes ezithile esibindini ukunceda ukumisela ukuba izihlobo zakho eziphilayo zisengozini ye-AHP.

Ukuvavanywa kwemfuza akunako ukuthintela ukuqala kwe-AHP, kodwa kunokunceda abantu obathandayo ukuba bajonge ukukhula kweempawu ezinxulumene noko.

Yise kude

Ukufumana uxilongo lwe-AHP kunokuba luxinzelelo ekuqaleni, kodwa ugqirha wakho ukhona ukuze aphendule yonke imibuzo yakho kwaye aqiniseke ukuba ufumana olona nyango lulungileyo.

Imbonakalo yabantu abane-AHP ilungile. Ukulawula iimpawu zakho ngonyango kunye notshintsho kwindlela yokuphila, kunokukunceda uqhubeke nemisebenzi yakho yemihla ngemihla ngemicimbi embalwa.