Ake sifunde ngamaprotheni angajwayelekile afakwa enhliziyweni? (I-Cardiac Amyloidosis)

Ingabe ngezinye izikhathi uzizwa ukhathele noma unenkinga yokuphefumula? Ungase ungazinaki kakhulu lezi zinto. Kodwa ngezinye izikhathi lezi zingaba uphawu lwenkinga yenhliziyo. Namuhla sizokhuluma ngesimo esithinta inhliziyo, okungenzeka ukuthi awukaze uzwe ngaso , kodwa esibaluleke kakhulu ukwazi ngaso. Ungakhathazeki, sizokhuluma ngalokhu kalula nangendlela ongayiqonda.

Kuyini lokhu (i-Cardiac Amyloidosis)?

Kalula nje, `(I-Cardiac Amyloidosis)` yisifo esibangelwa amaprotheni angasebenzi kahle emzimbeni wethu, ahlala enhliziyweni nasezindaweni ezizungezile. Cabanga ngakho njengokuvaleka kwepayipi lamanzi. Lokhu kwakheka kwamaprotheni kunciphisa ikhono lenhliziyo yakho lokupompa igazi. Inhliziyo kufanele isebenze kanzima. Ekugcineni, lo mzamo owengeziwe ungenza buthaka futhi ulimaze inhliziyo, okuholela `(Ukwehluleka Kwenhliziyo)`.

Kungaba nezizathu eziningi zalokhu. Abanye abantu bakuthola njengefa kubazali babo, okusho ukuthi kuyizakhi zofuzo . Abanye bangase babe nalesi simo ngaphandle kokuxhumana kwezakhi zofuzo. Singabangelwa nezinye izifo, njengomdlavuza. Nakuba lesi simo, esibizwa ngokuthi "i-Amyloidosis", singelapheki ngokuphelele, kunezindlela zokwelapha ezinhle zezinhlobo ezithile.

Yiziphi izinhlobo ze-cardiac Amyloidosis?

Amaprotheni emizimbeni yethu ayimikhondomu emide yama-molecule. Lawa maprotheni asisiza senze izinto eziningi, okuhlanganisa ukuhlinzeka ngamandla, ukwenza ukusabela kwamakhemikhali emizimbeni yethu, nokudlulisa imiyalezo phakathi kwezinhlelo ezahlukene. Kodwa kunenkinga. Amaseli ethu angasebenzisa kuphela lawa makhondomu ephrotheni uma esesimweni esifanele. Izinkinga zokugoqeka kwamaprotheni, njenge-amyloidosis, zibangela ukuthi lawa maprotheni aphenduke, agoqeke kabi, futhi angakwazi ukusebenza kahle.

Uma amaprotheni engahlelekile kahle, ayahlangana futhi umzimba awukwazi ukuwasebenzisa. Uma engenandawo yokuya, lawa maprotheni ahlangene ayahlangana, anamathele ndawonye, ​​​​futhi anamathele ezingxenyeni ezahlukene zomzimba.

I-Amyloidosis ingathinta izinhlelo eziningi emzimbeni, ikakhulukazi inhliziyo. Kunezinhlobo ezintathu eziyinhloko ze-amyloidosis ezithinta inhliziyo (ezinye izinhlobo nazo zingathinta inhliziyo, kodwa azivamile kakhulu).

I-AL (Uchungechunge Olukhanyayo) i-Amyloidosis

Umzimba wethu usebenzisa izinto ezibizwa ngokuthi "amaketanga okukhanya" njengengxenye yesimiso sawo sokuzivikela komzimba. Lokhu kusivikela kumagciwane afana namagciwane namagciwane. "I-AL Amyloidosis" yenzeka lapho amangqamuzana asemongweni wethu engasebenzi kahle futhi ekhiqiza "amaketanga okukhanya" amaningi kakhulu. Lokhu kungenzeka ngokwako, noma kungabangelwa izinhlobo ezithile zomdlavuza wegazi noma umdlavuza wesimiso sokuzivikela komzimba. Lolu hlobo luvame ukutholakala ngemva kweminyaka engu-50.

i-ATTR amyloidosis

I-Transthyretin (TTR) iyiprotheni esiza ukuhambisa amakhemikhali athile emizimbeni yethu. Iqanjwe ngokuthi ithutha i-hormone ye-thyroid kanye ne-vitamin A (retinol). Ngaphambilini yayibizwa ngokuthi i-Thyroxine-binding prealbumin (TBPA), kodwa lelo gama alisasetshenziswa.

Izimpawu ze -'ATTR Amyloidosis' ziyahlukahluka kakhulu. Zingabonakala zisencane njengeminyaka engama-20 noma zize zifike eminyakeni engama-70. Kunezinhlobo ezimbili eziyinhloko:

• I-ATTR Eyifa: Lesi yisimo sofuzo. Lokhu kusho ukuthi usizuza njengefa kumama wakho, kuyise, noma kokubili.
• I-ATTR yohlobo lwasendle: Lokhu kwenzeka lapho iphrotheni ye-TTR iqala ukungasebenzi kahle, ngaphandle kokuguqulwa kwezakhi zofuzo. Lokhu kuvame ukwenzeka ngemva kweminyaka engu-60. Ngaphambilini yayibizwa ngokuthi i-Senile Systemic Amyloidosis noma i-Senile Cardiac Amyloidosis, kodwa la magama awasasetshenziswa kakhulu.

I-amyloidosis ehlobene ne-dialysis

Abantu asebeneminyaka besebenzisa i-dialysis ngenxa yesifo sezinso basengozini enkulu yokuthola i-amyloidosis. Lokhu kungenxa yokuthi iphrotheni ebizwa ngokuthi i-beta-2 microglobulin (i-beta-2 microglobulin) ayivamisile ukuhluzwa ngesikhathi senqubo ye-dialysis. Le phrotheni, eyaziwa ngokuthi i-beta-2Ms, iqoqana enhliziyweni nakwezinye izingxenye zomzimba.

Izinhlobo ezingavamile (i-cardiac Amyloidosis)

Ngaphezu kwalokhu, kunezinhlobo eziningana ze-"Cardiac Amyloidosis", kodwa azivamile kakhulu.

Ubani onamathuba amaningi okuthuthukisa lokhu?

Kunezici eziningana eziyinhloko ezithinta ukuthi ubani othola lesi sifo:

• Ubudala: Nakuba abanye abantu bengaba ne-amyloidosis lapho beneminyaka engama-20, ngokuvamile ayivamile kubantu abangaphansi kweminyaka engama-40. Lesi sifo sivame ukutholakala ngemva kweminyaka engama-50, ikakhulukazi ngesimo se-ATTR amyloidosis yasendle.
• Ubulili: Amadoda anamathuba amaningi okuthola i-cardiac amyloidosis kunabesifazane. Ikakhulukazi nge-wild-type ATTR amyloidosis, kunamadoda angu-25 kuya kwangu-50 athintekile kuwo wonke owesifazane.
• Izwe lokuzalwa: Ukuguquka kwezakhi zofuzo okubangela i-ATTR amyloidosis yomndeni kuvame kakhulu kwamanye amazwe. Isibonelo, amazwe afana nePortugal, iJapan, iSweden, iFinland, kanye namanye amazwe amaningana aseYurophu.
• Uhlanga/ubuzwe: Olunye uguquko lwezakhi zofuzo olubangela i-ATTR amyloidosis yomndeni lutholakala cishe ku-4% wabantu abamnyama abadabuka eNtshonalanga Afrika. Lokhu kufaka phakathi ama-Afrika aseMelika kanye nabantu abamnyama abavela eLatin America naseCaribbean.
• I-dialysis eqhubekayo iminyaka: Uma uqhubeka no-dialysis, kulapho amathuba okuba nalezi zimpawu abe maningi khona.

Sivame kangakanani lesi sifo?

Ngokuvamile, i-`(Amyloidosis)` iyisifo esingavamile. Kodwa-ke, ezinye izinhlobo ze-`(Amyloidosis)` zivamile kakhulu kunezinye.

• I-AL Amyloidosis: E-United States kuphela, kubikwa amacala amasha angaba ngu-4,000 e-AL Amyloidosis minyaka yonke. Lokho cishe kungumuntu oyedwa kubantu abadala abangu-64,500.
• I-'ATTR Amyloidosis' Enefa: Lesi simo singabonakala kubantu abamnyama abangafika ku-4% abavela eNtshonalanga Afrika. Kubantu abavela eYurophu, sibonakala cishe koyedwa kwabayizinkulungwane eziyikhulu. Kodwa-ke, emazweni okukhulunywe ngawo ngenhla, leli nani liphezulu kakhulu. Isibonelo, ePortugal, cishe oyedwa kwabangu-538 unalesi simo.
• I-Wild-type ATTR Amyloidosis: Ochwepheshe bakholelwa ukuthi lesi yisimo esingahlolwanga kahle, kanti izilinganiso zisikisela ukuthi singathinta amadoda afinyelela ku-20% angaphezu kweminyaka engu-80.
• I-amyloidosis ehlobene ne-dialysis: Lokhu kwenzeka cishe kubantu abangu-20% abasebenzisa i-dialysis iminyaka emibili kuya kwemine. Kwenzeka cishe kubo bonke abasebenzisa i-dialysis iminyaka engu-13 noma ngaphezulu.

Lesi sifo sithinta kanjani umzimba?

I-Amyloidosis yenhliziyo yisifo esishintsha isakhiwo senhliziyo yakho futhi siphazamise ikhono layo lokupompa. Kwenzeka ngezindlela ezilandelayo:

• Ukuqina kwezindonga zenhliziyo: Lapho amaprotheni e-`(Amyloid)` efakwa emisipheni yenhliziyo, izindonga zenhliziyo ziyaqina futhi inhliziyo iyakhula. Uma lokhu kwenzeka, inhliziyo kufanele isebenze kanzima ukupompa igazi emzimbeni wonke. Lo mzamo owengeziwe ekugcineni uholela ku-`(Heart Failure)` .
• I-amyloidosis yenhliziyo ingabangela ukungalingani kwe-rhythmias yenhliziyo, noma izimpawu zikagesi ezibuthakathaka enhliziyweni. I-atrial fibrillation uhlobo oluvamile lwe-arrhythmia oluhlotshaniswa ne-amyloidosis.
• Ama-Amyloid deposits: Amaprotheni e-Amyloid angamaphrotheni afana ne-wax, anamathelayo ahlangana kalula futhi akhe amaqoqo amakhulu. Uma la maqoqo ebhajwa ku-valve yenhliziyo, i-valve ingavinjelwa kancane, ivimbele ukugeleza kwegazi.

Ziyini izimpawu?

Akuwona wonke umuntu one-genetic mutation engabangela i-amyloidosis ozoba nalesi sifo. Abanye abantu abakaze babe naso. Abanye bayasithola, kodwa asibi kakhulu kangako.

Izimpawu ze-cardiac Amyloidosis zivame ukubandakanya inhliziyo, kodwa zingabandakanya nezinye izitho ezibalulekile njengesibindi nezinso. Ezigabeni zokugcina ze-cardiac Amyloidosis, izimpawu zokwehluleka okukhulu kwenhliziyo (ezibalwe ngezansi) zingavela.

Izimpawu ezingaba khona zingafaka:

• Ukuphelelwa umoya: Lokhu kungenzeka uma usebenza noma ulele phansi.
• Ukuvuvukala okubangelwa ukunqwabelana koketshezi: Lokhu kuvame ukubonakala emilenzeni, emaqakaleni, embotsheni nasesiswini.
• Ukukhathala:Ukuzizwa ukhathele kakhulu izinsuku eziningana noma ngaphezulu.
• Ukushaya Kwenhliziyo Okubuhlungu: Umuzwa ongathandeki owuzwa sengathi inhliziyo yakho iyashaya ngaphandle kokuthi wenze noma yimuphi umzamo.
• Imithambo ye-Varicose entanyeni: Lokhu kwenzeka lapho inhliziyo ihluleka ukusebenza, ngoba inenkinga yokupompa kahle. Lokhu kucindezela okwandayo kubeka ingcindezi emithanjeni entanyeni, okubangela ukuba ikhule.
• I-Hepatomegaly: Lokhu futhi kuyinkinga evamile yokwehluleka kwenhliziyo, futhi kuyisizathu esifanayo esenza imithambo entanyeni ikhukhumale. Ukucindezela okukhulu okuvela enhliziyweni kubeka ingcindezi emithanjeni yegazi esibindini, okubangela ukuba isitho sivuvuke.
• Izinkinga zezinso: Izinto ezifana nokuvuvukala kwezinso noma izinguquko ekusebenzeni kwezinso.

Ezinye izinto ezingaba izimpawu ze-"Cardiac Amyloidosis" ezingahlobene nenhliziyo, isibindi, noma izinso:

• Ukuphuka okungavamile.
• Ulimi oluvuvukile.
• I-Carpal Tunnel Syndrome (Ukucindezeleka Kwezinzwa Zamaphaphu - Lokhu kungaba uphawu lwesixwayiso sokuqala, ngokuvamile iminyaka ngaphambi kokuba lesi sifo sivele).
• I-Lumbar Spinal Stenosis (ukuncipha kwesikhala esizungeze intambo yomgogodla emhlane ongezansi).
• Izinkinga zokubona.
• Izinkinga zokuzwa kanye nokungezwa.
• Ukuba ndikindiki noma ukuqaqamba ezandleni noma ezinyaweni.

Yiziphi izimbangela ze-cardiac Amyloidosis?

Kunezimbangela eziningi ezahlukene zezinhlobo ezahlukene ze-amyloidosis, kodwa eziningi zazo zihlobene ne -DNA yethu.

Cabanga ngakho njengencwadi yokupheka yomndeni. Iqukethe imiyalelo yokuthi amangqamuzana ethu kufanele asebenze kanjani, indlela yokwenza amaprotheni athile. Ukudlulisela i-DNA kubazali kubantwana kufana nokubhalela abazali bakho leyo ncwadi yokupheka ngesandla.

Ukuguqulwa kwezakhi zofuzo kufana nokubhala phansi encwadini yakho yokupheka ye-DNA. Uhlamvu olukhulu lungaphazamisa iresiphi. Kufana ncamashi nezifo ezifana ne-amyloidosis. Amaseli akho asebenza kanzima ngangokunokwenzeka, kodwa azi kuphela ukuthi alandele kanjani iresiphi. Lokhu kubhala phansi kungakwenza ngezindlela ezimbili.

Ifa

I-Amyloidosis yenhliziyo ivame ukuba yisimo somndeni. Lokhu kusho ukuthi uyizuza njengefa. Lokhu kwenzeka lapho oyedwa noma bobabili abazali bakho bene-mutation ku-DNA yabo, futhi iphutha kuleyo "ncwadi yeresiphi" likopishwa kweyakho.

Kutholwe

Izifo ezitholakalayo yizifo ongazitholi njengefa, kodwa ezikhula ngesikhathi esithile empilweni yakho. I-Wild-type ATTR amyloidosis kanye ne-dialysis-related amyloidosis zombili ziyizibonelo zalezi zifo ezitholakalayo. Kodwa akukho neyodwa yalezi ezihlobene nokuguqulwa kwezakhi zofuzo ku-DNA yakho.

• I-Wild-type ATTR amyloidosis yenzeka ngoba umzimba wakho wenza amaprotheni ngendlela efanele, kodwa ngokuhamba kwesikhathi lawo maprotheni aya ngokuya engazinzile (yingakho lesi sifo sihlobene nokuguga). Ekugcineni, iphrotheni imane igoqeka ngendlela engafanele.
• I-amyloidosis ehlobene ne-dialysis yenzeka lapho iphrotheni evamile engenakuhlungwa yi-dialysis, njengezinso zakho, inqwabelana emzimbeni wakho. Ngokuhamba kwesikhathi, leyo phrotheni inqwabelana ize ifike ezingeni lapho ibangela izinkinga.

Ingabe lokhu kuyathelelana?

Cha, `(Amyloidosis)` akusona isifo esithathelwanayo nganoma iyiphi indlela.

Udokotela ukuxilonga kanjani lokhu? (Ukuxilongwa)

Udokotela uzoqala ngokusola ukuthi une-(Cardiac Amyloidosis)` ngokusekelwe ezimpawini zakho, ukuhlolwa ngokomzimba (udokotela ubheka, uzwa, futhi ulalele umzimba wakho ukuze athole izimpawu zesifo), kanye nomlando wezokwelapha womndeni wakho. Bazobe sebekwenza ukuhlolwa kokuxilonga, ukuhlolwa kwezithombe, kanye nokuhlolwa kwelebhu ukuqinisekisa ukuthi une-(Cardiac Amyloidosis)` noma cha.

Uma othile emndenini wakho enesimo somndeni se-amyloidosis, ungase ungadingi ukuhlolwa okuthile noma ungase udinge ukuhlolwa okunye. Ukuhlolwa kofuzo kungenziwa futhi ukuze kubonakale ukuthi unezinguquko zofuzo ezingabangela i-cardiac amyloidosis.

Yiziphi izivivinyo ezenziwayo ukuze kutholakale lesi sifo?

Uma udokotela wakho esola ukuthi une-"Cardiac Amyloidosis", angase akucele ukuhlolwa okukodwa noma ngaphezulu okungezansi.

Ukuhlolwa kwelebhu

Lezi zivivinyo zithatha amasampula ezicubu, igazi, noma olunye uketshezi lomzimba. Kusetshenziswa idayi ekhethekile ebizwa ngokuthi iCongo red kulezi zivivinyo eziningi. Le dayi ibangela ukuthi izindawo lapho i-amyloid ibekwe khona zikhanye ngokuluhlaza ngaphansi kwezimo ezithile zokukhanya.

• I-biopsy yezicubu: Kulokhu, odokotela bathatha isampula encane yezicubu endaweni, njengemisipha yenhliziyo, bese beyihlola elebhu. Amasampula ezicubu angathathwa nakwezinye izindawo, kodwa uma isampula evela endaweni engeyona eyenhliziyo iveza umphumela omuhle, idinga ukuqinisekiswa ngokuhlolwa kwezithombe.
• Ukuhlolwa kwegazi: Kwezinye izinhlobo ze-cardiac amyloidosis, amaprotheni e-amyloid ajikeleza egazini. Ukuhlolwa kwelebhu kungawathola la maprotheni.
• Ukuhlolwa komchamo: Kwezinye izimo, amaprotheni e-amyloid angase abe khona emchameni. Ukuhlolwa kokuba khona kwala maprotheni emchameni nakho kungasiza ekuxilongweni.

Ukuhlolwa kwezithombe

Ngenxa yokuthi i-cardiac amyloidosis ingabangela ukuba inhliziyo yakho ikhule futhi ishintshe isimo, ukuhlolwa kwezithombe kungasiza ekuxilongeni lesi simo. Ukuhlolwa okungenziwa kufaka phakathi:

• I-Echocardiogram:Lokhu kuhlolwa kufana nelulwane elisebenzisa umsindo ukuthola indlela yalo. Lisebenzisa amaza omsindo avame kakhulu. Idivayisi ekhipha umsindo ibekwa esikhumbeni sesifuba, futhi odokotela bangabona inhliziyo ngokubheka ukuthi amaza omsindo agxuma kanjani ezakhiweni ezahlukene ngaphakathi komzimba. Lokhu kuwusizo kakhulu ekuboneni ukuthi izingxenye zenhliziyo zijiyile ngendlela engavamile.
• I-Scintigraphy: Kulolu vivinyo, odokotela bafaka i-tracer, into enomsebe omncane (kodwa engenangozi), egazini lakho. I-tracer iqoqana ezindaweni lapho iphrotheni ye-amyloid iqongelele khona. Lezi zindawo zingabonakala kalula ngokuhlolwa kwesithombe okubizwa ngokuthi i-SPECT scan (Single-Photon Emission Computed Tomography) .
• I-Magnetic Resonance Imaging (MRI): Lolu vivinyo lusebenzisa ubuchwepheshe obunamandla kakhulu be-magnet kanye nekhompyutha ukudala isithombe senhliziyo. I-MRI inemininingwane eminingi, ngakho odokotela bangabona ukuqina noma ezinye izinguquko enhliziyweni.

I-Electrocardiogram (ECG noma EKG)

Ku-ECG, izinzwa eziningana (ngokuvamile ezingu-12) zinamathele esikhumbeni sesifuba sakho. Izinzwa zibona ugesi ogeleza enhliziyweni yakho futhi zibonise amandla alowo mqondiso kagesi njengegagasi ephepheni noma esikrinini. Ochwepheshe abaqeqeshwe, njengodokotela, bangahlaziya igagasi ukuze babone ukuthi kukhona yini izinguquko ezingavamile endleleni izingxenye ezithile zenhliziyo yakho eziqhuba ngayo ugesi.

Ukuhlolwa kofuzo

Kuye ngemiphumela yokuhlolwa okunye, udokotela wakho angase ancome ukuhlolwa kwezakhi zofuzo. Lokhu kuhlolwa kungakhomba izinguquko ku-DNA yakho ezingase zibangele i-amyloidosis yakho.

Ingabe ikhona indlela yokwelapha i-Amyloidosis?

Izinhlobo eziningi ze-`(Cardiac Amyloidosis)` zingelashwa, kodwa lesi sifo asikwazi ukwelashwa ngokuphelele ngaso sonke isikhathi . Ukwelashwa kuyahlukahluka kuye ngohlobo lwe-`(Amyloidosis). Ngokuvamile, ukuxilongwa kwasekuqaleni kubaluleke kakhulu . Uma `(Amyloidosis)` itholakala kusenesikhathi, umonakalo wesikhathi eside enhliziyweni ungancishiswa. Uma ingatholakali futhi ingelashwa kusenesikhathi, umonakalo ohlala njalo ungaba khona. Okuwukuphela kwendlela yokulungisa umonakalo wenhliziyo ohlala njalo `(Ukufakelwa Kwenhliziyo)`.

I-AL amyloidosis

Ukwelashwa kwe-AL Amyloidosis kufana kakhulu nezindlela ezivamile zokwelapha umdlavuza. Lezi yizi:

• Ukwelashwa ngamakhemikhali: Njengomdlavuza, ukwelashwa ngamakhemikhali kungabhubhisa amaseli e-plasma angasebenzi kahle.
• Ukufakelwa kwamaseli e-stem: Lokhu kwelashwa kuvame ukwenziwa kanye ne-chemotherapy, futhi kushintsha amaseli e-plasma angasebenzi kahle ngamaseli amasha. Lawa maseli e-stem avame ukuthathwa kuwe (autologous). Ngenxa yokuthi angawakho, umzimba wakho uyawaqaphela futhi awuwenqabi noma awusabeli kabi kuwo.
• Ukwelashwa nge-immunotherapy:Isimiso sakho somzimba sokuzivikela singavame ukubona nokubhubhisa amaseli angasebenzi kahle. Ezifweni ezifana nomdlavuza noma i-AL Amyloidosis, isimiso sakho somzimba sokuzivikela asiboni lawo maseli njenganephutha. I-immunotherapy ifundisa isimiso sakho somzimba sokuzivikela ukuthi siqaphele futhi sibhubhise amaseli anephutha.

i-ATTR amyloidosis

Lolu hlobo lwe-"(Amyloidosis)" lwenzeka lapho isibindi sakho senza amaprotheni aphuka kalula. Le nkinga idinga ukuyeka konke ukwelashwa. Uhlu olungezansi luhlanganisa kokubili ukwelashwa okuvunyelwe nguhulumeni kanye nalezo ezisesesigabeni sokucwaninga.

• Ukufakelwa kwesibindi: Esikhathini esidlule, ukufakelwa kwesibindi kwakuyindlela kuphela yokuvimba isibindi sakho ekwenzeni amaprotheni angalungile. Nakuba lokhu kungaseyona yodwa inketho, kuseyindlela ewusizo nephumelelayo.
• Izithulisi zofuzo: I-ATTR Amyloidosis ibangelwa ukuguquka kofuzo ku-DNA yakho, njengokubhala iphutha encwadini yeresiphi. Izithulisi zofuzo ziyimithi esebenza njengekhadi leresiphi yesikhashana. Esikhundleni sokuthi amangqamuzana akho alandele iresiphi engalungile, awanikeza imiyalelo emisha ukuze akwazi ukwenza iphrotheni ngendlela efanele.
• Iziqinisi: Le mithi ivimba ama-molecule e-TTR ukuthi angagoqeki noma aqhekeke.
• Izithibi ze-Fibril: Lapho amaprotheni e-amyloid eqala ukuhlangana ndawonye, ​​akha izakhiwo ezifana ne-fiber ezibizwa ngokuthi ama-fibrils. Le mithi iyayekisa ukwakheka kwama-fibrils.

I-amyloidosis ehlobene ne-dialysis

Lolu hlobo lwe-`(Amyloidosis)` luvela kubantu abadinga i-`(dialysis)` ngoba iphrotheni ye-`(beta-2M)` iyaqoqana. Ngokuvamile, izinso zakho ziyahlunga bese zisusa leyo phrotheni, kodwa i-`(dialysis)` evamile ayikwazi ukwenza lokho. Yingakho kuthatha iminyaka ukuthi abantu abasebenzisa i-`(dialysis)` bathole lesi sifo. Njengamanje, kunezindlela ezimbili zokuselapha:

• Ukufakelwa kwezinso: Izinso eziphilile zisho ukuthi awusadingi i-dialysis, futhi izinso ezifakwe kwenye indawo zingahlunga iphrotheni ye-beta-2M eningi. Ukufakelwa kwezinso kungase kumise i-amyloidosis ehlobene ne-dialysis ukuthi ingabi yimbi kakhulu, kodwa abacwaningi abaqiniseki ukuthi izosusa yini iphrotheni ye-amyloid eqongeleleke emzimbeni.
• Izihlungi ezikhethekile: Nakuba izihlungi ze-dialysis ezivamile zingakwazi ukususa amaprotheni e-beta-2M, kunezihlungi ezikhethekile ezingazisusa okungenani ingxenye. Ngeshwa, lezi zihlungi azihlungi yonke iphrotheni, futhi azihlungi yonke. Lokhu kusho ukuthi ngisho nangemva kweminyaka eminingi ye-dialysis, i-amyloidosis ehlobene ne-dialysis ingagcina ivele.

Ukwelashwa okungaqondile

Kunezinhlobo ezahlukene zokwelapha kanye nemithi engasiza abantu abane-Cardiac Amyloidosis. Lokhu kuvame ukwelapha izimpawu zesifo futhi kungafaka:

• Ukufakelwa inhliziyo: Abantu abanomonakalo omkhulu wenhliziyo ngenxa ye-amyloidosis yenhliziyo ngezinye izikhathi badinga ukufakelwa inhliziyo. Lokhu kuhlinzwa kuvame ukwenziwa ngemva kokuba imbangela ye-amyloidosis isixazululiwe noma seyelashwe.
• Imithi yokulwa nokushaya kwenhliziyo: Le mithi isiza ekuvimbeleni ukushaya kwenhliziyo okungajwayelekile, okunye kwakho okungaba yingozi.
• Amadivayisi afakelwa: Lawa madivayisi afaka phakathi ama-pacemaker kanye nama-defibrillator e-cardioverter afakelwa (i-ICD) . Aphatha izinga lokushaya kwenhliziyo yakho ngokuletha ukushaqeka kukagesi nokuvimbela ukungasebenzi kahle kwenhliziyo okuyingozi.
• Imithi yokukhipha isisu: Le mithi isiza ngokukhethekile ekugcinweni koketshezi (uphawu oluvamile lokwehluleka kwenhliziyo) ngokuthuthukisa ukusebenza kwezinso zakho. Kodwa-ke, akufanele uthathe le mithi uma unesifo esinjengesifo sezinso esibi.
• I-Doxycycline : Amaketanga okukhanya ku-AL amyloidosis nawo anobuthi emaseli enhliziyo yakho. Ngenxa yezizathu ochwepheshe abangakaziqondi ngokugcwele, i-antibiotic i-doxycycline ingamelana naleyo miphumela enobuthi.

Ingabe ikhona imiphumela emibi yokwelashwa?

Izinkinga nemiphumela emibi yokwelashwa kwalesi sifo iyahlukahluka kakhulu. Ngezansi kunezinye izinto ezivamile ezingaba khona. Kodwa-ke, udokotela wakho angakuchazela kangcono izinkinga, imiphumela emibi, kanye nezinye izingozi. Lokhu kungenxa yokuthi bayazi isimo sakho mathupha, ngakho-ke bangakwazi ukulungisa ulwazi lwabo kanye nezincazelo zabo ukuze zivumelane nesimo sakho esithile.

Ngizozizwa ngingcono ngokushesha kangakanani ngemva kokwelashwa?

Ukwelashwa kwe-Amyloidosis yenhliziyo kuvame ukukhawulelwa ekuphatheni izimpawu zakho. Ezinye izindlela zokwelapha zingase zinikeze impumuzo ethile, kodwa imiphumela ingeyesikhashana. Kwezinye izimo, izindlela zokwelapha zingabangela imiphumela emibi engakwenza uzizwe kabi isikhashana, kodwa udokotela wakho angakunikeza imithi kanye nesiqondiso ukusiza ekunciphiseni ubukhali baleyo miphumela emibi.

Ingabe lokhu kungavinjelwa? Noma ingabe ingozi ingancishiswa?

Ngeshwa, i-amyloidosis ayinakuvinjelwa (ngaphandle kokukodwa, bheka ngezansi). Njengoba imbangela ye-amyloidosis engazuzwa njengefa ingaziwa, ayikho indlela yokuyivimbela. I-amyloidosis ezuzwa njengefa nayo ayinakuvinjelwa, ngoba ikwi-DNA oyizuza kubazali bakho.

Okuwukuphela kwento ehlukile yi-cardiac amyloidosis, ehlotshaniswa ne-dialysis. Lesi simo singavinjelwa uma ungenayo i-dialysis isikhathi eside, noma uma usebenzisa izihlungi ezikhethekile ukuvimba ukuqongelela kwamaprotheni e-beta-2M. Kodwa-ke, lezi zihlungi ngokuvamile azikuvimbeli ngokuphelele ukuqongelela, okusho ukuthi zivame ukuba yindlela nje yokulibazisa ukuqala kwesifo.

Iyini inhloso yalesi sifo?

I-Amyloidosis yenhliziyo iyisifo esiqhubekayo, esinezimpawu zokwehluleka kwenhliziyo eziya ziba zimbi kakhulu. Ekugcineni, lesi sifo singabuthakatha inhliziyo yakho kuze kube yilapho ingasakwazi ukusebenza. Uma ingelashwa, isimo siba sibi kakhulu.

Ngaphandle kokwelashwa, isikhathi sokusinda esimaphakathi kubantu abane-AL Amyloidosis singaphansi kwezinyanga eziyisishiyagalombili. Kulabo abanezimo ezinzima kakhulu, kungaba izinyanga ezintathu kuya kweziyisithupha. Kulabo abane-ATTR Amyloidosis, isikhathi sokusinda esimaphakathi siyiminyaka emibili kuya kwemihlanu. Kulabo abanemindeni engalashwanga ene-ATTR Amyloidosis, isimo sibi kakhulu, isikhathi sokusinda esimaphakathi siyiminyaka emibili kuya kwemithathu.

Ngingazinakekela kanjani futhi ngiphathe izimpawu zami?

Ngeshwa, azikho izinto eziningi ongazenza ukuze uzisize ngokushesha ngemva kokuba usuthole i-amyloidosis. Uma othile emndenini wakho enalesi sifo, kufanele ukhulume nodokotela wakho mayelana nokuhlolwa kwezakhi zofuzo. Nakuba kungebona bonke abantu abanokuguqulwa kwezakhi zofuzo okuhlobene ne-amyloidosis yenhliziyo abazoba nalesi sifo, kubalulekile ukwazi ukuthi ungasithola yini. Ngaleyo ndlela, ungaqaphela izimpawu bese uqala ukwelashwa kusenesikhathi.

Uma une-`(Cardiac Amyloidosis)`, kubaluleke kakhulu ukulandela imiyalelo kadokotela wakho mayelana nemithi nokwelashwa. Eminingi yale mithi isebenza ngezindlela ezithile kakhulu, futhi ukuze isebenze kahle, kubalulekile ukuyithatha njengoba nje udokotela wakho ekutshelile.

Kufanele ngibone nini udokotela noma ngifune usizo oluphuthumayo?

Ngemva kokuthola ukuthi une-Cardiac Amyloidosis, udokotela wakho uzohlela ukuvakashelwa njalo. Kuye ngokuthi isimo sakho sibucayi kangakanani, kungase kudingeke ukuthi ubonane nodokotela wakho kaningi.

Udokotela wakho uzokutshela nokuthi yiziphi izimpawu okufanele zibangele ukulashwa kwezokwelapha. Kodwa-ke, ngokuvamile, shayela udokotela wakho uma unezinye zezimpawu ezilandelayo:

• Ukuzizwa unesizungu noma ubuthakathaka lapho uhleli noma umile: Lokhu kubizwa ngokuthi "i-Orthostatic Hypotension." Lokhu kwenzeka ngoba umfutho wegazi ophansi unciphisa inani legazi eliya ebuchosheni uma usukuma.
• Ukuvuvukala kwezitho noma isisu: Lokhu kuvame ukubangelwa ukugcinwa koketshezi.
• Ukwehla kwesisindo okuzumayo nokuphawulekayo: Lokhu kuyimpawu ebalulekile, ikakhulukazi uma ungazami ukwehlisa isisindo.

Kufanele ngiye nini e-ER (Igumbi Eliphuthumayo)?

Ngenxa yokuthi i-cardiac amyloidosis ingaphazamisa kakhulu ukusebenza kwenhliziyo yakho, ezinye izimpawu zibonisa ukuthi udinga usizo lwezokwelapha ngokushesha.Kuyadingeka. Ezinye zazo yilezi:

• Ukuzwa ukushaya kwenhliziyo.
• Ukushaya kwenhliziyo okusheshayo, okuhamba kancane, noma okungajwayelekile ngendlela engavamile.
• Ubunzima bokuphefumula nganoma yisiphi isizathu.

Okokugcina, amaphuzu ambalwa abalulekile (Umyalezo Wokuya Ekhaya)

I-Amyloidosis yenhliziyo empeleni iyisimo esiyinkimbinkimbi, kodwa kubaluleke kakhulu ukuqaphela.

Khumbula, nakuba lesi sifo singaqhubeka kancane kancane, ukutholakala kusenesikhathi kanye nokwelashwa okufanele kungasiza kakhulu ekwandiseni isikhathi sakho sokuphila kanye nokuthuthukisa ikhwalithi yokuphila kwakho.

Ngezinye izikhathi ukufakelwa izitho zomzimba kungasiqeda lesi simo. Ngenxa yemithi emisha kanye nokwelashwa, abantu abaningi abanalesi sifo bangakwazi ukulawula izimpawu zabo futhi baphile iminyaka eminingi ngemva kokuba lesi sifo siqalile.

Uma unokungabaza noma imibuzo ngalokhu, khuluma nodokotela wakho . Bangakunikeza iseluleko esifanele. Ungesabi, thola ulwazi, buza imibuzo. Awuwedwa.

I- Amyloidosis Yenhliziyo, I-Amyloidosis Yenhliziyo, Ukufakwa Kwamaprotheni, Isifo Senhliziyo, Izimpawu Zesifo Senhliziyo, Ukwelashwa kwe-Amyloidosis, i-AL Amyloidosis, i-ATTR Amyloidosis, Isifo Senhliziyo