Iyini i-Porphyria? Izimpawu, Izimbangela, & Ukwelashwa

I-Porphyria yiqembu lemithi yobudlova eyingavamile ethonya indlela umzimba wakho okwenza ngayo i-heme, ingxenye ebalulekile yamaseli ebomvu egazi. Uma le nqubo iphosakele, izinto ebizwa ngokuthi yi-porphyrins ziyakha emzimbeni wakho futhi zingabangela uhlu olukhulu lwezimpawu.

Cabanga ngayo njengomugqa wokubutha wefektri lapho enye yemishini ingasebenzi kahle. Umzimba wakho unezinyathelo ezisishiyagalombili zokwenza i-heme, futhi i-porphyria kwenzeka lapho enye yalezi zinyathelo iphazamiseka. Lokhu kuphazamiseka kungathinta isikhumba sakho, i-nervous system, noma kokubili, kuye ngohlobo olukhona.

Ziyini izimpawu ze-porphyria?

Izimpawu ze-porphyria zihluka kakhulu kuye ngohlobo olukhona, kodwa ngokuvamile ziwela ezinhlotsheni ezimbili eziyinhloko: izinkinga zesikhumba nezehlakalo ezithinta umzimba wakho wonke. Abantu abaningi abazi ukuthi bane-porphyria ngoba izimpawu zingaba zingenamandla noma zikhohliswe kalula ngezimo ezinye.

Ake sibheke izindlela ezahlukene i-porphyria engabonakala ngazo ekuphileni kwakho, sikhumbula ukuthi akuwona wonke umuntu ohlangabezana nazo zonke lezi zimpawu:

Izimpawu ezihlobene Nesikhumba

Uma unohlobo oluthinta isikhumba sakho, ungase uqaphele lezi zinguquko uma ukhombiswe ukukhanya kwelanga:

• Ukubhebhetheka nokwenzakala ezindaweni ezivezwe elangeni njengezandla zakho, ubuso, nezingalo
• Ukukhula okwandayo kwezinwele ebusweni nasezandleni
• Isikumba esiba buthakathaka kakhulu futhi siqhekeka kalula
• Amabala amnyama noma akhanyayo ombala wesikhumba
• Ukukhuluphala kwesikhumba, ikakhulukazi ezinqanqemeni zakho

Lezi zinguquko zesikhumba zenzeka ngoba i-porphyrins yenza isikhumba sakho sibe nomuzwa omkhulu wokukhanya. Ngisho nokukhombiswa okufushane kwelanga kungabangela ukubhebhetheka okuhlungu okuthatha amasonto ukuphulukisa.

Izimpawu zehlakalo elikhulu

Amanye ama-types e-porphyria abangela ukuhlaselwa okungazelelwe, okukhulu okungathatha izinsuku noma amasonto. Lezi zikhathi zivame ukuqala kancane bese ziqina:

• Ubuhlungu obukhulu besisu obujulile futhi obucindezelayo
• Ukupukeka nokuhlanza okungapheli
• Ukuvimbela noma isifo sohudo
• Ubuthakathaka bemisipha, ikakhulukazi ezandleni nasezinzwaneni
• Ukukhahlela noma ukungabi namqondo ezandleni nasezinzwaneni
• Ukudideka, ukukhathazeka, noma ukushintsha kwemizwelo
• Umbhilo obomvu omnyama noma obusundu
• Izinga lokugijima lenhliziyo kanye nokuphakama kwegazi

Lezi zihlazo zingathintwa ukucindezeleka, imithi ethile, utshwala, noma ukushintsha kwamahomoni. Ubuhlungu besisu bukhulu kangangokuthi abantu abaningi bayaphela emagumbini okuphuthumayo becabanga ukuthi bane-appendicitis noma enye isimo esinzima.

Izimpawu ezingavamile nezinzima

Kwezinye izimo, i-porphyria ingabangela izinkinga ezinzima ezidinga ukunakekelwa kwezokwelapha ngokushesha:

• Ukuhlukunyezwa noma ukulahlekelwa yiwoyela
• Ubuthakathaka bemisipha obukhulu obuthinta ukuphefumula
• Ukudideka kwengqondo noma amaphupho
• Ukuqina kwezandla noma izinzwani
• Izinkinga zokuphefumula ezidinga ukusekelwa kokuphefumula

Lezi zimpawu ezinzima zivame kakhulu ngesikhathi sezihlazo ezinzima futhi zibonisa ukuthi ukuxilongwa okulungile nokuphathwa kubaluleke kangakanani.

Imiphi imikhakha ye-porphyria?

Kunezinhlobo eziyisishiyagalombili ze-porphyria, ngayinye ibangelwa yinkinga ngesinyathelo esihlukile ekwenzeni i-heme. Odokotela bayazihlukanisa zibe izigaba ezimbili eziyinhloko ngokusekelwe lapho izinkinga zivele khona kakhulu: izinhlobo ezinzima ezibangela ubuhlungu, nezinhlobo ze-cutaneous ezithinta ikakhulukazi isikhumba sakho.

I-porphyrias ebukhali

Lezi zinhlobo zingabangela izihlazo eziqubukezelayo, ezinzima ezithinta umzimba wakho wonke:

• I-porphyria ebuhlungu ehlala isikhathi esifushane (AIP) - Uhlobo oluvamile kakhulu olukhulu, lubangela ubuhlungu obukhulu besisu kanye nezimpawu zezinzwa
• I-coproporphyria elidluliselwa ezizukulwaneni (HCP) - Ingabangela kokubili ukuhlaselwa okukhulu kanye nokuzwela kwelanga
• I-porphyria ehlukahlukene (VP) - Ihlanganisa ukuhlaselwa okukhulu nezingxaki zesikhumba, kuvamile eNingizimu Afrika
• Ukuntuleka kwe-ALA dehydratase porphyria - Kunqabile kakhulu, ngokuvamile kuvela ebuntwaneni

I-porphyrias yesikhumba

Lezi zinhlobo zithinta ikakhulukazi isikhumba sakho uma sisuke silotshwe ilanga:

• I-porphyria cutanea tarda (PCT) - Uhlobo oluvamile kakhulu ngokujwayelekile, lubangela ukubola kwesikhumba nokwenzakala
• I-Erythropoietic protoporphyria (EPP) - Ibanga ubuhlungu obukhulu nokukhula uma kulotshwe ilanga
• I-Congenital erythropoietic porphyria (CEP) - Kunqabile kakhulu, izinkinga ezinkulu zesikhumba kusukela ekuzalweni
• I-Hepatoerythropoietic porphyria (HEP) - Kunqabile kakhulu, ihlanganisa izinkinga zesibindi nesikhumba

Uhlobo ngalunye lunomklamo walo wezimpawu nezici eziyibangela, kungakho ukufumana ukuxilongwa okulungile kubalulekile ekwelapheni okulungile.

Yini ebangela i-porphyria?

Izinhlobo eziningi ze-porphyria zibangelwa yizinguquko zezinto ezihlala zifuywe ovela kubazali bakho, kepha ezinye zingathuthuka kamuva ekuphileni ngenxa yezinye izici. Iinguquko zezinto ezihlala zifuywe zithinta ama-enzyme asiza ukwenza i-heme emzimbeni wakho.

Nawa amaqhinga aphambili okukhula kwe-porphyria:

Izimbangela zezinto ezihlala zifuywe ezidluliselwa ezizukulwaneni

• Ukuguqulwa kwe-gene okudluliselwa kubazali oyedwa noma bobabili
• Ukudluliselwa kwe-Autosomal dominant (kudingeka ikhophi eyodwa kuphela ye-gene ovela kumzali oyedwa)
• Ukudluliselwa kwe-Autosomal recessive (kudingeka amakhophi omabili ovela kubazali bobabili)
• Ukudluliselwa kwe-X-linked (kunqabile, kudluliselwa nge-chromosome ye-X)

Ngisho noma uzuze ushintsho oluthile lwe-genetic, ungase ungalutholi lutho olukhulu. Abantu abaningi baphila impilo evamile, enempilo ngaphandle kokwazi ukuthi banalo.

Izimbangela eziqalekayo

Ezinye izinhlobo, ikakhulukazi i-porphyria cutanea tarda, zingathuthuka ngaphandle kokudluliselwa kwe-genetic:

• Isifo sesibindi noma ukulimala kwesibindi
• Ukugcwala kwesinyithi emzimbeni
• Ukutheleleka yi-Hepatitis C
• Ukutheleleka yi-HIV
• Ukusebenzisa utshwala ngokweqile
• Ukubhema
• Imithi ethile noma amakhemikhali

Lezi zinto zingabangela i-porphyria kubantu abangase babe nokuthambekela kwe-genetic kodwa abangayithuthuki leyo simo.

Nini kufanele ubona udokotela nge-porphyria?

Kufanele ufunesekhe imithi uma uhlushwa yizimpawu ezingachazeki ezingase zibonise i-porphyria, ikakhulukazi uma ziqhubeka noma zikhona emndenini wakho. Ukuxilongwa kwasekuqaleni kungavimbela izinkinga ezinzima futhi kukusize uphathe le simo kangcono.

Xhumana nodokotela wakho ngokushesha uma uqaphela:

• Ubuhlungu obukhulu besisu obuphindaphindayo obungena sizathu esicacile
• Isikhumba esiqhuma noma esinamaqhubu kalula elangeni
• Urin obomvu omnyama noma obusundu, ikakhulukazi ngesikhathi sesifo
• Ubuthakathaka bemisipha obuza futhi buyaphela
• Umlando womndeni we-porphyria noma ukuhlaselwa okungachazeki

Nini kufanele ufune ukunakekelwa okusheshayo

Shaya u-911 noma uhambe egumbini lemiphumela ngokushesha uma uhlushwa yi:

• Ubuhlungu obukhulu besisu nokuhlanza okungapheli
• Ubuthakathaka bemisipha obuthinta ukuphefumula kwakho
• Ukuhlinzwa noma ukulahlekelwa ukwazi
• Ukudideka okukhulu noma ukuqamba amanga
• Ukuqina noma ukungakwazi ukuhambisa izandla noma imilenze

Lezi zimpawu zingase zibonise ukuhlaselwa okukhulu kwe-porphyria okudinga ukwelashwa ngokushesha ukuvimbela ukulimala okungapheli.

Yiziphi izinto ezingozi ze-porphyria?

Ukuqonda izimbangela ezingase zikubangele ukuba ube ne-porphyria kungakusiza wena nodokotela wakho ukuthi nibone ukuthi kufanele uhlolwe yini i-porphyria noma uthathe izinyathelo zokuvimbela ukuhlaselwa. Ezinye izimbangela awukwazi ukuzishintsha, kanti ezinye ungazilawula.

Izimbangela ezivela egazini nakwabasemndenini

• Ukuba nomzali, umfowethu, noma ingane enesifo se-porphyria
• Ukuba ngowesizwe esithile (ezinye izinhlobo ziyavame kakhulu kubantu abathile)
• Ukuba nezinye izimo zezofu ezihlasela isibindi

Izimbangela zendlela yokuphila nempilo

Izinto eziningi zingandisa amathuba okuthola i-porphyria noma ukuqalisa ukuhlaselwa:

• Ukusebenzisa utshwala kakhulu
• Ukubhema utshaba
• Isifo sesibindi nganoma yisiphi isizathu
• Ukukhuluphala kwe-Iron
• Ukutheleleka okuthile okuvela evirusi njenge-hepatitis C noma i-HIV
• Ukuba owesifazane (ushintsho lwe-hormone lungaqalisa ukuhlaselwa)
• Ukudla okunciphile noma ukudla okuncane
• Amazinga aphezulu engcindezi

Imithi nezinto eziqalisa ukuhlaselwa

Izinto ezithile zingaqalisa ukuhlaselwa kwe-porphyria kubantu abanesisindo se-genetic:

• Imithi ethile (i-barbiturates, imithi ye-sulfa, amanye ama-antibiotic)
• Imithi ye-hormone noma ushintsho
• Ukuvezwa ezintweni ezithile zekemikhali noma eziyingozi
• Ukuthola i-lead

Uma une-porphyria, udokotela wakho uzokunika uhlu lwemithi nezinto okufanele uzigweme ukuze uvikele ukuqalisa ukuhlaselwa.

Ziyini izinkinga ezingase zenzeke ngenxa ye-porphyria?

Nakuba abantu abaningi abane-porphyria baphila impilo evamile ngokulawulwa okuhle, ezinye izinkinga zingase zenzeke uma isimo singalawulwa kahle noma uma kwenzeka ukuhlaselwa okukhulu. Ukuqonda lokhu kukusiza ukwazi ukuthi kungani ukulandela uhlelo lwakho lokwelashwa kubaluleke kangaka.

Izinkinga zokuhlaselwa okukhulu

Phakathi nokuhlaselwa okukhulu, izinkinga eziningi ezinkulu zingase zenzeke okungase kudinge ukunakekelwa okukhulu kwezokwelapha:

• Ukwehluleka kokuphefumula okudinga ukusekelwa kokuphefumula
• Ukuphelelwa amanzi okukhulu kanye nokungalingani kwamathumba
• Izinga eliphezulu legesi elimaza izitho
• Ukuhlupheka okuthinta ingqondo
• Ubuthakathaka bemisipha okwesikhashana noma okungapheli
• Izimpawu zezengqondo ezihlanganisa ukucindezeleka nokukhathazeka

Lezi zinkinga ziningi uma izifo zingaphathwa ngokushesha noma uma uneziqephu eziningi ezinzima ngokuhamba kwesikhathi.

Izinkinga zesikhathi eside

Abanye abantu bangaba nezinkinga eziqhubekayo, ikakhulukazi uma i-porphyria yabo ingalawulwa kahle:

• Ubuhlungu obuhlala njalo obuthinta imisebenzi yansuku zonke
• Ukonakala kwesibindi ngenxa yeziqephu eziningi
• Izinkinga zesibindi noma ukwanda komngcipheko womdlavuza
• Ukonakala kwama-nerve okungapheli okubangela ubuthakathaka noma ukungabi namandla
• Amabala amnyama kakhulu nokubola kokubukeka ngenxa yokuthinteka kwesikhumba
• Ukucindezeleka nokukhathazeka ngenxa yokuphila nobuhlungu obuhlala njalo

Izinkinga ezingavamile kodwa ezinzima

Ezimweni ezimbi kakhulu, ikakhulukazi ngezinhlobo ezithile ezingavamile ze-porphyria:

• Ukuntuleka kwegazi okukhulu okudinga ukuthola igazi njalo
• Izinkinga ze-bone marrow
• Ukwanda komngcipheko womdlavuza wesibindi
• Amagatsha ngenxa yokushintsha kokwakheka kwe-bile
• Izinkinga zokushintsha komjikelezo wenhliziyo ngesikhathi seziqephu ezinzima

Izindaba ezinhle ukuthi ngokuxilongwa kahle nokuphathwa, iningi lalezi zinkinga lingathintelwa noma liphathwe kahle.

Ungayivimba kanjani i-porphyria?

Nakuba ungakwazi ukuvimbela i-porphyria ezuzwe, ungathatha izinyathelo zokunciphisa ingozi yakho yokuthuthukisa izimpawu noma ukubangela iziqephu. Ukuvimbela kugxile ekugwemeni izinto ezibangela izinkinga nokugcina impilo enhle.

Ukuvimbela i-porphyria ezuzwe

Ngezinhlobo ezingelona iziwundlu, unganciphisa ingozi yakho ngokuthi:

• Ukunciphisa ukusetshenziswa kotshwala noma ukukuyeka ngokuphelele
• Ukungabhemi utshaba
• Ukuthola i-vaccine ye-hepatitis B
• Ukwenza izinto ngokucophelela ukuze ugweme i-hepatitis C ne-HIV
• Ukuphatha noma yiziphi izimo zesibindi ngokufanele
• Ukugwema izithasiselo zesinyithi ezingadingekile

Ukuphepha ukuhlaselwa uma une-porphyria

Uma ukwaziswe ukuthi une-porphyria, ungavimba ukuhlaselwa okuningi ngokuthi:

• Ugweme imithi eyaziwayo ngokubangela ukuhlaselwa
• Uphathe ukucindezeleka ngezindlela zokuphumula
• Udla ukudla okuvamile futhi ugweme ukudla okungapheli
• Uthole ubuthongo obanele
• Ukunciphisa ukusetshenziswa kotshwala
• Ukusebenzisana nodokotela wakho phakathi nezinguquko ze-hormonal
• Ukuvikela isikhumba sakho ekukhanyeni kwelanga uma une-cutaneous type

Izincazelo zokuhlela umndeni

Uma une-porphyria futhi uhlela umndeni:

• Cabanga ngokwelulekwa ngokuzalwa ukuze uqonde ingozi yokudluliselwa
• Xoxa ngokuhlela umndeni nochwepheshe oqondileyo i-porphyria
• Hlela ukuqapha ngesikhathi sokukhulelwa uma ungowesifazane
• Cabanga ukuhlola amalungu omndeni angase abe sengozini

Khumbula ukuthi ukuba ne-gene akusho ukuthi wena noma abantwana bakho nizothola izimpawu ngokuqinisekile. Abantu abaningi baphila impilo yabo yonke bengakwazi ukuthi bathethe izinguquko ezivela ku-genetic.

I-porphyria ihlonishwa kanjani?

Ukuhlola i-porphyria kungaba inselele ngoba izimpawu zivame ukulingisa ezinye izimo, futhi odokotela abaningi abayiboni njalo. Ikhona ukulinganisa i-porphyrins nezinto ezihlobene emathunjini, egazini, noma emathunjini ngesikhathi sezimo.

Udokotela wakho cishe uzoqala ngomlando wakho wezokwelapha kanye nezimpawu, bese u-oda ukuhlolwa okuqondile ukuze aqinisekise ukuxilongwa.

Ukubheka okuqala

Udokotela wakho uzobuza imibuzo eningiliziwe mayelana:

• Izimpawu zakho nokuthi zenzeka nini
• Umbukiso womlando wemindeni we-porphyria noma izimpawu ezifanayo
• Imithi oyithathayo
• Ukusebenzisa utshwala nezici zesitayela sokuphila
• Izinto eziqala ukuletha izimpawu

Izivivinyo zelebhu

Izivivinyo eziningi zingasiza ekuxilongeni i-porphyria, futhi ukuthi yiziphi ozidingayo kuncike ezimpawu zakho:

• Ukuqoqwa kwamanyikwe we-24-hour - Ukulinganisa i-porphyrins nezikhombisi ezihlobene
• Izivivinyo zegazi - Hlola i-porphyrins ethile e-plasma
• Izibonelo zesitulo - Bheka izinhlobo ezithile ze-porphyrins
• Ivivinyo le-genetic - Khomba ukuguqulwa okuqondile kwe-gene
• Izivivinyo zenkambiso ye-enzyme - Linganisa ukuthi izinhlobo ezithile ze-enzymes zisebenza kangcono kangakanani

Isikhathi salezi zivivinyo kubalulekile. Ngalezo ezinkulu, izibonelo eziqoqwe ngesikhathi sokuhlaselwa zinezela kakhulu ukukhombisa imiphumela engavamile kunesibonelo esithathwe lapho uzizwa kahle.

Ivivinyo elikhethekile

Ngezinye izikhathi kudingeka izivivinyo ezengeziwe ukuze kutholakale ngokuqondile ukuthi yiluphi uhlobo lwe-porphyria olukhona:

• Ukuhleleka kwe-DNA ukuze kutholakale ukuguqulwa okuqondile kwe-genetic
• Ivivinyo lolwemi ukuqonda izindlela zokudluliselwa
• Ukuphinda ukuhlolwa ngezigaba ezahlukene zezimpawu
• Izivivinyo zokulahla ezinye izimo ezibangela izimpawu ezifanayo

Ukuthola ukuxilongwa okulungile kuvame ukudinga ukusebenzisana nochwepheshe oqonda i-porphyria, njengoba ukuhlolwa kungaba nzima futhi imiphumela idinga ukuhumusha ngokucophelela.

Iyini impilo ye-porphyria?

Ukuphathwa kwe-porphyria kugxile ekuphathweni kwezimpawu, ukuvimbela ukuhlaselwa, nokubhekana nezinkinga lapho zenzeka. Indlela ethile kuncike ohlotsheni olukhona nokuthi izimpawu zakho zinzima kangakanani.

Ukuphathwa kokuhlasela okukhulu

Uma une-porphyria attack enkulu, ukwelashwa kugxile ekuyekiseni ukuhlaselwa nokuphatha ubuhlungu:

• Ukugonywa nge-hemin - Ukunika umzimba wakho i-heme elidingekayo ukuze uqede ukuhlaselwa
• Ukwelashwa nge-glucose - I-glucose enkulu ingasiza ekuhlaselweni okukhulu
• Ukulawulwa kobuhlungu - Imithi ebuhlungu enkulu ukulawula ubuhlungu obukhulu besisu
• Ukulawulwa kokusabela - Imithi yokuyeka ukugabha nokuphazamiseka
• Ukuqiniswa kwamanzi - Amanzi e-IV ukuvimbela ukuphelelwa amanzi
• Ukulawulwa kokucindezeleka kwegazi - Imithi yolawulo lokucindezeleka kwegazi eliphezulu

Ukubulawa okuningi okukhulu kudinga ukungena esibhedlela ukuze odokotela bakwazi ukukugcina uqaphile futhi banikeze ukwelashwa okukhulu.

Ukwelashwa kwe-porphyria yesikhumba

Uma i-porphyria yakho ikhuthaza ikakhulukazi isikhumba sakho, ukwelashwa kugxila ekuvikeleni isikhumba sakho nokunciphisa amazinga e-porphyrin:

• I-Phlebotomy - Ukususwa okuvamile kwegazi ukuze kunciphise i-iron ne-porphyrins
• Imithi yokulwa ne-malaria - I-hydroxychloroquine ephansi ukuze kusulwe i-porphyrins
• Ukuvikela ilanga - Ukugwema okukhulu kokukhanya kwelanga nokukhanya kwe-UV
• Ukukhathalela izilonda - Ukwelashwa okuhle kwezibilini zesikhumba nezihlazo
• Ukuncipha kwe-iron - Ukugwema izithasiselo ze-iron nokulawula ukwanda kwe-iron

Ukwelashwa okuvimbelayo

Kubantu abanezehlakalo ezivamile, odokotela bangase bancoma imithi yokuvikela:

• Ukufakwa kwe-hemin okuvamile ukuvimbela ukuhlaselwa
• Ukwelashwa kokucindezela ama-hormone kwabesifazane abahlasela ngezikhathi zabo zenyanga
• I-Givosiran (Givlaari) - Imithi entsha eyancipha ubukhulu bokuhlaselwa kwe-acute intermittent porphyria
• Ukushintsha indlela yokuphila ukuze ugweme izinto ezibangela ukuhlaselwa

Ukwelashwa okusha nokuphakamayo

Ucwaningo lwezokwelapha luyaqhubeka nokuthuthukisa ukwelashwa okusha kwe-porphyria:

• Izindlela zokwelapha i-gene ezifundwa ezivivinyweni zeklinikhi
• Ukuhlinzwa kwesibindi samacala abukhali angaphenduli ezinye izindlela zokwelapha
• Imithi emisha ehlose izinyathelo ezithile ekukhiqizweni kwe-heme
• Ukuhlinzwa komgogodla wethambo ngezinhlobo ezithile ezingavamile

Uhlelo lwakho lokwelapha luzokwenziwa ngokwezinhlobo zakho ze-porphyria nokuthi kukuthinta kanjani ngokukhethekile.

Ungayiphatha kanjani i-porphyria ekhaya?

Ukuphatha i-porphyria ekhaya kuhilela ukugwema izinto ezikhuthazayo, ukuqaphela izimpawu zokuqala, nokugcina impilo enhle iyonke. Ngokusebenzisa indlela efanele, abantu abaningi abane-porphyria baphila impilo egcwele, esebenzayo.

Izindlela zokuphatha imihla ngemihla

Lezi zinyathelo zingakusiza uhlale uphilile futhi unciphise ingozi yokuhlaselwa:

• Gcina idayari yezimpawu ukuze ubone izinto ezikhuthazayo zakho
• Gcina amashejuli okulala njalo futhi ulawule ukucindezeleka
• Dla ukudla okujwayelekile, okujwayelekile futhi ugweme ukudla okungapheli
• Hlala uthambile, ikakhulukazi ngesikhathi sezulu esishisayo
• Thatha imithi ngokuqondile njengoba kuqokiwe
• Gqoka ubucwebe be-alamu yezokwelapha obuveza isimo sakho

Ukuvikelwa kwelanga ngezinhlobo zesikhumba

Uma i-porphyria yakho ithonya isikhumba sakho, ukuvikelwa kwelanga kuba kubalulekile:

• Sebenzisa i-sunscreen ebanzi-spectrum ene-SPF 30 noma ngaphezulu
• Gqoka izingubo ezinde, ibhulukwe, neziqalo ezibanzi ngaphandle
• Funani isithunzi ngesikhathi sehora lelanga elikhulu (10 AM kuya ku-4 PM)
• Cabanga ngokugqoka okuvikelayo kwe-UV namafilimu ewindi
• Qaphela ukuthi ukukhanya okungaphakathi kungakhuthaza nezimpawu

Ukuphatha izimpawu zokuhlukunyezwa okukhulu

Uma uzizwa ukuhlaselwa kuqala, thatha isinyathelo ngokushesha:

• Xhumana nodokotela wakho ngokushesha
• Misa noma iyiphi imithi engase ikhuthaze ukuhlaselwa
• Yandisa ukudla kwe-carbohydrate uma kunconywe udokotela wakho
• Hlala uthambile futhi ukhululeke
• Ube nomuntu okhona ukuze akusize uthole ukunakekelwa kwezokwelapha
• Gcina ulwazi loxhumana nabo oluphuthumayo olutholakala kalula

Ukubumba inethwekhi yakho yokusekela

Ukuphila ne-porphyria kulula uma unekusekela:

• Fundisa amalungu omndeni wakho ngesimo sakho nezinto ezibangela
• Xhumana namaqembu okusekela i-porphyria ku-inthanethi noma endaweni
• Sebenza neqembu lokukhathalela impilo eliyazi i-porphyria
• Cabanga ngokwelulekwa ukuze usize ukubhekana nesifo esingapheli
• Hlela kusengaphambili uhambo nezimo eziphuthumayo

Khumbula ukuthi ukuphatha i-porphyria kuyilwimi phakathi kwakho neqembu lakho lokukhathalela impilo. Unganqikazi ukufinyelela uma udinga usizo noma unemibuzo.

Kufanele ulungiselele kanjani ukubhekana nodokotela wakho?

Ukulungiselela kahle ukubhekana nodokotela wakho kungasiza ukuqinisekisa ukuthi uthola ukuxilongwa okuchane kakhulu nelenani lokwelapha eliphumelelayo. Njengoba i-porphyria ingaba nzima ukuyixilonga, ulwazi olukuphelayo lubalulekile.

Ngaphambi kokubhekana kwakho

Butha lolu lwazi olubalulekile ukuze uhlanganyele nodokotela wakho:

• Idayari enemininingwane yesimptomu efaka izinsuku, izinto ezibangela, nobunzima
• Uhlu oluphelele lwazo zonke izidakamizwa, izithasiselo, namavithamini
• Umbhalo womndeni wezokwelapha, ikakhulukazi noma yiziphi izifo ezingachazwanga
• Izithombe zezinkinga zesikhumba uma unezimpawu zesikhumba
• Imiphumela yokuhlolwa yangaphambili namarekhodi ezempilo
• Uhlu lwemibuzo ofuna ukuyibuza

Imibuzo okufanele uyibuze udokotela wakho

Cabanga ukuthi ubuze le mibuzo ebalulekile ngesikhathi sokuvakasha kwakho:

• Yiluphi uhlobo lwe-porphyria enayo, futhi lokho kusho ukuthini?
• Ziyini izinto zami ezibangela, futhi ngingazigwema kanjani?
• Yiziphi izidakamizwa engingazigwema?
• Ngingazitshela kanjani uma nginamahloni?
• Kufanele ngifune ukunakekelwa okuphaphandayo nini?
• Amalungu omndeni wami kufanele ahlolwe?
• Lokhu kuyokuthinta kanjani impilo yami yansuku zonke nezincomo zami zesikhathi esizayo?

Okufanele ukulindele ngesikhathi sokubhekana

Udokotela wakho cishe uzo:

• Thatha umlando wezokwelapha ophelele
• Yenza ukuhlolwa komzimba
• Yala ukuhlolwa okuthile kwegazi, umchamo, noma izinsalela
• Xoxa ngezinketho zokwelapha ngokuya ngezimpawu zakho
• ukunika uhlu lokugwema izinto ezikhuthazayo
• Hlela ukuvakasha okulandelayo kokulandelela

Ungesabi ukucela incazelo uma ungaqondi lutho. I-Porphyria ingaba yinkimbinkimbi, futhi kubalulekile ukuthi uzizwe ukhululekile ngecebo lakho lokwelapha.

Iyini into ebaluleke kakhulu mayelana ne-porphyria?

I-Porphyria yiqembu leemeko ezingavamile kodwa eziphatheka kalula ezihlasela indlela umzimba wakho okwenza ngayo i-heme, ingxenye ebalulekile yamaseli ebomvu egazi. Nakuba ingabangela izimpawu ezinkulu, abantu abaningi abane-porphyria bangaphila impilo evamile, enempilo ngokuxilongwa okulungile nokwelashwa.

Izinto ezibaluleke kakhulu okumele uzikhumbule ukuthi ukuxilongwa kwasekuqaleni kwenza umehluko omkhulu, izimpawu zivame ukuvinjelwa ngokugwema izinto ezikhuthazayo, kanti ukwelashwa okuphumelelayo kukhona kokubili ukuhlaselwa okukhulu nokuphathwa okuqhubekayo.

Uma ucindezela ukuthi ungase ube ne-porphyria, ikakhulukazi uma unezimpawu eziphindaphindayo ezingachazwanga noma umlando womndeni wale meko, ungangabazi ukukhuluma nodokotela wakho. Ngeqembu lezokwelapha elifanele nendlela yokuphatha, ungalawola isimo sakho futhi ugcine ikhwalithi enhle yokuphila.

Khumbula ukuthi ukuba ne-porphyria akuchazi wena. Abantu abaningi abane le meko basebenza, bahamba, babe nemindeni, futhi balandele amaphupho abo njengabanye abantu. Okubalulekile ukuqonda isimo sakho, ukusebenza nabaphakeli bezempilo abanolwazi, nokudlala indima ephambili ekuphatheni impilo yakho.

Imibuzo evame ukubuzwa mayelana ne-porphyria

Q1: Ingabe i-porphyria iyadluliseleka?

Cha, i-porphyria ayikhuli. Awukwazi ukuyithola kwabanye noma ukuyandulela kwabanye. Izinhlobo eziningi zizimo ezivela ezifuyweni oziZuza kubazali bakho, kanti ezinye zithuthukiswa ngenxa yezinkinga zesibindi noma ezinye izinkinga zempilo. Lesi simo sithinta indlela umzimba wakho okwenza ngayo i-heme, okuyinqubo yangaphakathi engabandakanyi izinto eziphethe ukutheleleka.

UMbuzo 2: Ingaphiliswa yini i-porphyria?

Okwamanje, ayikho impilo ye-porphyria, kodwa lesi simo singaphathwa kahle kakhulu ngokwelashwa okuhle. Abantu abaningi abane-porphyria baphila impilo evamile ngokuphelele ngokugwema izinto ezikhuthazayo nokulandela izinhlelo zabo zokwelashwa. Abacwaningi basebenza ezindleleni zokwelapha ezintsha, kufaka phakathi i-gene therapy, engase inikeze izinketho ezinhle nakakhulu esikhathini esizayo. Ukugxila kukuvimbela ukuhlaselwa nokuphatha izimpawu lapho kwenzeka.

UMbuzo 3: Ivamile kangakanani i-porphyria?

I-Porphyria iyindlala, ithinta cishe umuntu oyi-1 ku-25 000 ngokujwayelekile. Noma kunjalo, ubukhulu buyahluka kakhulu ngohlobo nendawo yomhlaba. I-Porphyria cutanea tarda yilona hlobo oluvamile, kanti ezinye izinhlobo ezifana ne-congenital erythropoietic porphyria ziyindlala kakhulu. Izinhlobo ezithile zivame kakhulu kubantu abathile ngenxa yezinto ezivela ezifuyweni.

UMbuzo 4: Ingabe abesifazane abane-porphyria bangaba nabantwana ngokuphephile?

Abesifazane abaningi abane-porphyria bangaba nokukhulelwa okuhle, kodwa kudinga ukuhlelwa ngokucophelela nokubhekwa ngabahlinzeki bezempilo abanolwazi ekuphatheni i-porphyria phakathi kokukhulelwa. Ukushintsha kwamahomoni phakathi kokukhulelwa kungabangela ukuhlaselwa kwabesifazane abathile, ngakho ukubhekwa okuqondile kwezokwelapha kubalulekile. Ukuqondisa ngezinto ezivela ezifuyweni kungakusiza ukuqonda ingozi yokudlulisa lesi simo kubantwana bakho nokwenza izinqumo ezinolwazi zokuhlela umndeni.

UMbuzo 5: Kufanele ngenzeni uma ngicabanga ukuthi nginamahloni e-porphyria?

Uma uthi ucabanga ukuthi uhlushwa ukuhlaselwa yi-porphyria, xhumana nodokotela wakho ngokushesha noma uhambe e-emergency room uma izimpawu zinzima. Ungalindi ukubona ukuthi izimpawu ziyathuthuka ngokwazo. Yeka ukuthatha noma yimiphi imithi engase iqhube izimpawu, hlala uthambile, futhi ube nomuntu ozosiza ukuthi uthole ukunakekelwa kwezokwelapha. Ukwelashwa kwasekuqaleni kokuhlasela kuholela eziphumeleni ezingcono futhi kungavimbela izinkinga ezinzima.