Kodi Porphyria ndi chiyani? Musachite mantha, tiyeni tikambirane mwachidule.

Kodi munayamba mwamvapo kutentha kwambiri kapena kutupa pakhungu lanu mukatuluka padzuwa? Kapena munayamba mwamvapo kupweteka kwambiri m'mimba ndi nseru ndi kusanza popanda chifukwa chomveka? Zizindikirozi ndi zosiyana ndi matenda wamba. Mwina kumbuyo kwa izi pali matenda osowa kwambiri omwe anthu ambiri sanamvepo. Umenewo ndi matenda ena otchedwa Porphyria . Musachite mantha mukamva dzinali. Lero, tikambirana za izi mosavuta komanso m'njira yomwe mungamvetse.

Mwachidule, kodi Porphyria ndi chiyani?

Kuti timvetse izi, choyamba tiyeni tiganizire za fakitale yaying'ono m'thupi lathu. Pali puloteni yofunika kwambiri yomwe imapatsa maselo ofiira athu mtundu wofiira ndipo imanyamula mpweya kuchokera m'mapapo kupita ku thupi lonse. Imatchedwa 'heme'. Heme iyi imapangidwa m'thupi lathu m'magawo angapo, monga mzere wolumikizira. Monga momwe zimakhalira posonkhanitsa galimoto. Pa sitepe iliyonse, mapuloteni apadera otchedwa ma enzyme amagwira ntchito.

Tangoganizirani, chimachitika ndi chiyani ngati gawo limodzi mu unyolo uwu wopanga lakhala lovuta, kapena ngati wantchito mmodzi (enzyme) sagwira ntchito bwino? Gawo lotsatira silingapangidwe, ndipo zinthu zopangira zikuwunjikana, sichoncho? Mofananamo, pamene cholakwika chikuchitika pa gawo lopanga heme, mankhwala opangidwa mu gawo lapitalo, otchedwa 'porphyrins' ndi 'porphyrin precursors', amayamba kuwunjikana m'thupi. Ndi kuwunjikana kwa mankhwala awa m'thupi komwe kumayambitsa zizindikiro za porphyria. Izi zimatha kuwononga dongosolo lamanjenje kapena khungu.

Kodi mitundu ikuluikulu ya porphyria ndi iti?

Porphyria ingagawidwe m'magulu awiri akuluakulu, kutengera momwe zizindikiro zimawonekera. Kumvetsetsa gululi kudzakuthandizani kumvetsetsa bwino matendawa.

Pali mitundu ina ingapo ya matenda amitundu iwiri ikuluikulu. Mwachitsanzo, mtundu wotchedwa `Acute Intermittent Porphyria (AIP)`, womwe umagwera pansi pa acute porphyria, ndi mtundu wotchedwa `Porphyria Cutanea Tarda (PCT)`, womwe umagwera pansi pa cutaneous porphyria, supezeka kawirikawiri.

Kodi ndi zinthu ziti zomwe zimayambitsa zizindikiro?

Ngakhale kuti porphyria ndi matenda a majini, zizindikiro sizimawonekera nthawi zonse. Pali zinthu zina zomwe zingayambitse zizindikiro, kapena "kuukira". Izi zimatchedwa 'zoyambitsa'. Kupewa izi ndiyo njira yabwino kwambiri yothanirana ndi matendawa.

• Mankhwala ena: Mankhwala ena ochepetsa ululu , mapiritsi oletsa kubereka, mankhwala oletsa ululu, ndi mankhwala ena oletsa khunyu angayambitse izi. Ngati muli ndi porphyria, muyenera kulankhula ndi dokotala wanu musanamwe mankhwala aliwonse.
• Mowa ndi kusuta : Kumwa mowa wambiri ndi kusuta fodya, makamaka nthawi imodzi, ndi zinthu zomwe zimayambitsa vutoli.
• Zakudya ndi kusala kudya: Zinthu monga zakudya zopatsa mphamvu zochepa , zakudya zochepetsa thupi kwambiri, komanso kusala kudya zingayambitse zizindikiro.
• Kupsinjika maganizo ndi thupi: Zinthu monga kugwira ntchito mopitirira muyeso, kupsinjika maganizo, ndi opaleshoni zingakhudze.
• Matenda: Zizindikiro zimatha kuchitika chifukwa cha kupsinjika maganizo komwe kumachitika chifukwa cha matenda ena kapena matenda ena.
• Kuwala kwa dzuwa: Kuwala kwa dzuwa ndiye chinthu chachikulu chomwe chimayambitsa matenda a Cutaneous Porphyria kwa anthu omwe ali ndi matenda a shuga.

Kodi muyenera kupita kwa dokotala liti? Kodi matendawa amapangidwa bwanji?

Popeza matendawa ndi osowa ndipo zizindikiro zake zimafanana ndi matenda ena ofala, nthawi zina zimatenga nthawi kuti munthu adziwe matendawa. Ndikofunikira kuonana ndi dokotala ngati muli ndi zizindikiro zotsatirazi, makamaka ngati zimachitika pafupipafupi.

Mu porphyria yoopsa

Ngati mukumva kupweteka kwambiri m'mimba pamodzi ndi zizindikiro monga nseru, kusanza, kudzimbidwa, kugunda kwa mtima, kuthamanga kwa magazi, kufooka kwa minofu, kusokonezeka, kapena kukomoka, ndikofunikira kwambiri kupita kuchipatala nthawi yomweyo, makamaka ku Emergency Treatment Unit (ETU), chifukwa vutoli nthawi zina likhoza kukhala pachiwopsezo.

Mu Cutaneous Porphyria

Ngati khungu lanu likuyamba kutupa, zilonda, kutupa, kufiira, kapena kukanda mosavuta mutakhala padzuwa, pitani kwa dokotala wa khungu.

Njira yodziwira matenda:

1. Kuyezetsa kuchipatala: Choyamba, dokotala adzakuyezetsani, akumvetsera mosamala zizindikiro zanu komanso mbiri ya matenda a m'banja mwanu.

2. Mayeso a mu labotale: Ngati mukukayikira kuti pali porphyria, mkodzo wanu, magazi, komanso mwina ndowe zanu zidzayesedwa kuti zione kuchuluka kwa porphyrins. Pa milandu yoopsa, mkodzo ukhoza kukhala wofiira kapena wofiirira.

3. Kuyesa kwa majini: Ngati matendawa atsimikiziridwa ndi mayeso a labotale, mayeso a majini angachitike kuti adziwe kusintha kwa majini komwe kunayambitsa matendawa.

Kodi njira zochiritsira ndi ziti?

Mankhwala a porphyria amasiyana malinga ndi mtundu wa matenda komanso kuopsa kwa zizindikiro. Ngakhale kuti palibe mankhwala, zizindikiro zimatha kuthetsedwa ndipo munthu akhoza kukhala ndi moyo wabwinobwino.

Chithandizo cha Acute Porphyria

Zizindikiro zikayamba kukulirakulira (kuukira koopsa), kugonekedwa kuchipatala ndi kulandira chithandizo ndikofunikira.

• Kupereka madzi amchere (IV fluids): Kusunga mchere ndi madzi m'thupi moyenera.
• Jakisoni wa Heme: Kuwongolera kusowa kwa heme m'thupi ndikuchepetsa kuchuluka kwa porphyrin komwe kumasonkhana.
• Kupereka shuga: Kupereka shuga monga dextrose kudzera m'mitsempha kapena pakamwa kumawonetsa chiwindi kuti chichepetse kupanga porphyrin.
• Mankhwala ena: Mankhwala ochepetsa ululu pa ululu waukulu, ndi mankhwala ochizira nseru ndi kusanza.
• Mankhwala atsopano monga Givosiran (Givlaari): Kwa anthu omwe ali ndi zizindikiro zobwerezabwereza, mankhwalawa amaperekedwa mwezi uliwonse kuti achepetse kuchuluka kwa matendawa komanso kuopsa kwake.

Chithandizo cha Cutaneous Porphyria

• Chitetezo ku dzuwa:Ichi ndiye chinthu chofunikira kwambiri. Kuvala manja aatali, zipewa, ndi kugwiritsa ntchito mafuta oteteza ku dzuwa okhala ndi chitetezo chapamwamba, monga SPF 50+, ndikofunikira.
• Kuchotsa magazi m'thupi: Izi zimaphatikizapo kuchotsa magazi ochepa (pafupifupi unit imodzi) m'thupi milungu ingapo iliyonse. Izi zimathandiza kuchepetsa kuchuluka kwa chitsulo chowonjezera m'thupi komanso zimathandiza kuchotsa ma porphyrins omwe asonkhana m'chiwindi.
• Mankhwala: Mankhwala ochepa monga chloroquine kapena hydroxychloroquine angathandize kuti ma porphyrins achotsedwe mwachangu m'chiwindi.