Masifunde ngeeproteni ezingaqhelekanga ezifakwa entliziyweni? (I-Cardiac Amyloidosis)

Ngaba ngamanye amaxesha uziva udiniwe okanye unengxaki yokuphefumla? Usenokungazinaki kakhulu ezi zinto. Kodwa ngamanye amaxesha ezi zinokuba luphawu lwengxaki yentliziyo. Namhlanje siza kuthetha ngemeko echaphazela intliziyo, osenokuba awuzange uve ngayo , kodwa ibaluleke kakhulu ukwazi ngayo. Ungakhathazeki, siza kuthetha ngayo ngokulula nangendlela onokuyiqonda.

Yintoni le (iCardiac Amyloidosis)?

Ngamafutshane, `(Cardiac Amyloidosis)` sisifo esibangelwa ziiproteni ezimile kakubi emzimbeni wethu, ezihlala entliziyweni nangaphandle kwayo. Cinga ngayo njengokuvaleka kombhobho wamanzi. Oku kuqokelelana kweeproteni kunciphisa amandla entliziyo yakho okupompa igazi. Intliziyo ke kufuneka isebenze nzima. Ekugqibeleni, lo mzamo wongezelelweyo unokwenza buthathaka kwaye wonakalise intliziyo, nto leyo ekhokelela kwi`(Heart Failure)`.

Zininzi izizathu zoku. Abanye abantu bayizuza njengelifa kubazali babo, oko kuthetha ukuba yimfuza . Abanye banokuba nale meko ngaphandle konxibelelwano lwemfuza. Ingabangelwa nazezinye izifo, ezifana nomhlaza. Nangona le meko, ebizwa ngokuba yi-"Amyloidosis"`, ingenakunyangwa ngokupheleleyo, kukho unyango olufanelekileyo kwezinye iintlobo.

Ziziphi iintlobo ze-Amyloidosis yentliziyo?

Iiproteni emizimbeni yethu ziintambo ezinde zeemolekyuli. Ezi proteni zisinceda senze izinto ezininzi, kuquka ukubonelela ngamandla, ukwenza ii-chemical reactions emizimbeni yethu, kunye nokudlulisela imiyalezo phakathi kweenkqubo ezahlukeneyo. Kodwa kukho ingxaki. Iiseli zethu zinokusebenzisa ezi ntambo zeproteni kuphela ukuba zikwimo efanelekileyo. Iingxaki zokungasongi kakuhle kweeproteni, ezifana ne-amyloidosis, zibangela ukuba ezi proteni zijijeke, zisongeke kakuhle, kwaye zingakwazi ukusebenza kakuhle.

Xa iiproteni zingalungelelananga kakhulu, ziyadibana kwaye umzimba awukwazi ukuzisebenzisa. Xa zingenandawo yokuya, ezi proteni zidibeneyo ziyahlangana, zinamathele, kwaye zibambeke kwiindawo ezahlukeneyo zomzimba.

I-Amyloidosis inokuchaphazela iinkqubo ezininzi emzimbeni, ingakumbi intliziyo. Kukho iintlobo ezintathu eziphambili ze-amyloidosis ezichaphazela intliziyo (ezinye iintlobo nazo zinokuchaphazela intliziyo, kodwa azixhaphakanga kakhulu).

AL (Uludwe Olukhanyayo) I-Amyloidosis

Umzimba wethu usebenzisa izinto ezibizwa ngokuba yi-"light chain" njengenxalenye yenkqubo yawo yokuzikhusela. Ezi zisikhusela kwiintsholongwane ezifana neentsholongwane kunye neebhaktheriya. I-"AL Amyloidosis" yenzeka xa iiseli ezikumongo wethu wethambo zingasebenzi kakuhle kwaye zivelisa uninzi lwezi "light chain". Oku kungenzeka ngokwako, okanye kunokubangelwa ziintlobo ezithile zomhlaza wegazi okanye umhlaza wenkqubo yokuzikhusela. Olu hlobo ludla ngokuchongwa emva kweminyaka engama-50.

i-ATTR amyloidosis

I-Transthyretin (TTR) yiproteni enceda ukuhambisa iikhemikhali ezithile emizimbeni yethu. Igama layo livela kuba ithutha i-thyroid hormone kunye ne-vitamin A (retinol). Yayibizwa ngokuba yi-Thyroxine-binding prealbumin (TBPA), kodwa elo gama alisasetyenziswa.

Iimpawu ze -'ATTR Amyloidosis' ziyahluka kakhulu. Ingabonakala iselula njengeminyaka engama-20 okanye ifikelele kwiminyaka engama-70 ubudala. Kukho iintlobo ezimbini eziphambili:

• I-ATTR yelifa: Le yimeko yemfuza. Oku kuthetha ukuba uyizuza njengelifa kumama wakho, kutata wakho, okanye kubo bobabini.
• I-ATTR yohlobo lwasendle: Oku kwenzeka xa iproteni ye-TTR iqala ukungasebenzi kakuhle, ngaphandle kotshintsho lwemfuza. Oku kwenzeka emva kweminyaka engama-60. Ngaphambili yayibizwa ngokuba yi-Senile Systemic Amyloidosis okanye i-Senile Cardiac Amyloidosis, kodwa la magama awasasetyenziswa rhoqo.

I-amyloidosis enxulumene ne-dialysis

Abantu abaneminyaka besebenzisa i-dialysis ngenxa yesifo sezintso basengozini enkulu yokufumana i-amyloidosis. Oku kungenxa yokuba iproteni ebizwa ngokuba yi-beta-2 microglobulin (beta-2 microglobulin) ayihlali ihluzwa ngexesha lenkqubo ye-dialysis. Le proteni, eyaziwa ngokuba yi-beta-2Ms, iqokelelana entliziyweni nakwezinye iindawo zomzimba.

Iintlobo ezingaqhelekanga (iiCardiac Amyloidosis)

Ukongeza koku, kukho ezinye iintlobo ezahlukeneyo ze-"Cardiac Amyloidosis", kodwa azixhaphakanga kakhulu.

Ngubani onokuthi aphuhlise oku ngakumbi?

Kukho izinto ezininzi eziphambili ezichaphazela ukuba ngubani ofumana esi sifo:

• Ubudala: Nangona abanye abantu banokuhlakulela i-amyloidosis xa bekwiminyaka yabo yama-20, ngokuqhelekileyo ayiqhelekanga kubantu abangaphantsi kweminyaka engama-40. Esi sifo sidla ngokufunyaniswa emva kweminyaka engama-50, ingakumbi ngohlobo lwe-ATTR amyloidosis yasendle.
• Isini: Amadoda anamathuba amaninzi okufumana i-cardiac amyloidosis kunabasetyhini. Ingakumbi kwi-wild-type ATTR amyloidosis, kukho amadoda angama-25 ukuya kuma-50 achaphazeleka kumfazi ngamnye.
• Ilizwe lokuzalwa: Utshintsho lwemfuza olubangela i-ATTR amyloidosis yosapho luxhaphake kakhulu kwamanye amazwe. Umzekelo, amazwe afana nePortugal, iJapan, iSweden, iFinland, kunye namanye amazwe aliqela aseYurophu.
• Uhlanga/uhlanga: Olunye utshintsho lwemfuza olubangela i-ATTR amyloidosis yosapho lufumaneka kwi-4% yabantu abamnyama abazalwa eNtshona Afrika. Oku kuquka abantu abamnyama baseMelika kunye nabantu abamnyama abavela eLatin America naseCaribbean.
• I-dialysis eqhubekayo iminyaka: Okukhona uchitha ixesha elide usebenzisa i-dialysis, kokukhona amathuba okuba nezi mpawu ayanda.

Ixhaphake kangakanani le sifo?

Ngokubanzi, `(Amyloidosis)` sisifo esingaqhelekanga. Nangona kunjalo, ezinye iintlobo ze`(Amyloidosis)` zixhaphake kakhulu kunezinye.

• I-AL Amyloidosis: E-United States kuphela, malunga namatyala amatsha angama-4,000 e-AL Amyloidosis axelwa minyaka le. Loo nto ithetha ukuba umntu omnye kubantu abadala abangama-64,500 unesifo.
• I-'ATTR Amyloidosis' yelifa: Le meko inokubonwa kubantu abamnyama abafikelela kwi-4% abavela eNtshona Afrika. Kubantu abazalwa eYurophu, ibonwa kubantu abamalunga nekhulu lamawaka. Nangona kunjalo, kumazwe akhankanyiweyo apha ngasentla, eli nani liphezulu kakhulu. Umzekelo, ePortugal, umntu omnye kwabangama-538 unale meko.
• I-Wild-type ATTR Amyloidosis: Iingcali zikholelwa ukuba esi sisifo esingachazwanga kakuhle, kwaye uqikelelo lubonisa ukuba sinokuchaphazela ukuya kuthi ga kwi-20% yamadoda angaphezu kweminyaka engama-80 ubudala.
• I-amyloidosis enxulumene ne-dialysis: Oku kwenzeka kubantu abamalunga nama-20% abasebenzisa i-dialysis iminyaka emibini ukuya kwemine. Kuyenzeka phantse kubo bonke abantu abasebenzisa i-dialysis kangangeminyaka eli-13 nangaphezulu.

Esi sifo sichaphazela njani umzimba?

I-Amyloidosis yentliziyo sisifo esitshintsha ulwakhiwo lwentliziyo yakho kwaye siphazamise amandla ayo okupompa. Kwenzeka ngeendlela ezilandelayo:

• Ukuqina kweendonga zentliziyo: Xa iiproteni ze-`(Amyloid)` zibekwe kwimisipha yentliziyo, iindonga zentliziyo ziyaqina kwaye intliziyo iyakhula. Xa oku kusenzeka, intliziyo kufuneka isebenze nzima ukupompa igazi emzimbeni wonke. Lo mzamo wongezelelweyo ekugqibeleni ukhokelela kwi-`(Heart Failure)` .
• I-amyloidosis yentliziyo inokubangela ukungqubana kwentliziyo (i-arrhythmias), okanye iimpawu zombane ezibuthathaka entliziyweni. I-Atrial fibrillation luhlobo oluqhelekileyo lwe-arrhythmia olunxulunyaniswa ne-amyloidosis.
• Ii-Amyloid deposits: Iiproteni ze-Amyloid ziiproteni ezifana newax, ezinamathelayo ezihlangana ngokulula kwaye zenze ii-clumps ezinkulu. Ukuba ezi clumps zinamathela kwi-valve yentliziyo, i-valve inokuvaleka kancinci, ithintele ukuhamba kwegazi.

Zithini iimpawu?

Ayingabo bonke abantu abane-genetic change enokubangela i-amyloidosis abaya kuba nesi sifo. Abanye abantu abakaze babe naso. Abanye bayasifumana, kodwa asibi kakhulu.

Iimpawu ze-cardiac Amyloidosis zihlala zibandakanya intliziyo, kodwa zinokubandakanya nezinye izitho ezibalulekileyo ezifana nesibindi kunye nezintso. Kwinqanaba lokugqibela le-Cardiac Amyloidosis, iimpawu zokusilela okukhulu kwentliziyo (ezidweliswe ngezantsi) zinokwenzeka.

Iimpawu ezinokubakho zingabandakanya:

• Ukuphefumla kancinci: Oku kungenzeka xa usebenza okanye xa ulele phantsi.
• Ukudumba okubangelwa kukuqokelelwa kolwelo: Oku kudla ngokubonakala emilenzeni, emaqatheni, emathunjini, nasesiswini.
• Ukudinwa:Ukudinwa kakhulu kangangeentsuku ezininzi nangaphezulu.
• Ukubetha Kwentliziyo: Imvakalelo engathandekiyo ovakalelwa ngathi intliziyo yakho iyabetha ngaphandle kokuba wenze nantoni na.
• Imithambo ye-Varicose entanyeni: Oku kwenzeka xa intliziyo ingasebenzi kakuhle, kuba kunzima ukuyipompa kakuhle. Olu xinzelelo luphezulu lukwabeka uxinzelelo kwimithambo yentamo, nto leyo ebangela ukuba ikhule.
• I-Hepatomegaly: Le yingxaki eqhelekileyo yokusilela kwentliziyo, kwaye yimbangela efanayo yokuba imithambo entanyeni ivuvuke. Uxinzelelo oluphezulu oluvela entliziyweni lukwabeka uxinzelelo kwimithambo yegazi esibindini, nto leyo ebangela ukuba ilungu lidumbe.
• Iingxaki zezintso: Izinto ezifana nokudumba kwezintso okanye utshintsho ekusebenzeni kwezintso.

Ezinye izinto ezinokuba ziimpawu ze-"Cardiac Amyloidosis" ezingahambelaniyo nentliziyo, isibindi, okanye izintso:

• Ukudumba okungaqhelekanga.
• Ulwimi oludumbileyo.
• I-Carpal Tunnel Syndrome (Uxinzelelo lweMithambo yePulmonary - Oku kungaba luphawu lwesilumkiso kwangethuba, rhoqo kwiminyaka ngaphambi kokuba isifo sibonakale).
• I-Lumbar Spinal Stenosis (ukuncitshiswa kwendawo ejikeleze umthambo womqolo kumqolo ongezantsi).
• Iingxaki zokubona.
• Iingxaki zokuva kunye nokungeva.
• Ukuba ndindisholo okanye ukurhawuzelela ezandleni okanye ezinyaweni.

Zithini izizathu zeCardiac Amyloidosis?

Zininzi izizathu ezahlukeneyo zeentlobo ezahlukeneyo ze-amyloidosis, kodwa uninzi lwazo lunxulumene ne -DNA yethu.

Cinga ngayo njengencwadi yokupheka yosapho. Iqulethe imiyalelo yendlela iiseli zethu ezimele zisebenze ngayo, indlela yokwenza iiproteni ezithile. Ukudlulisela i-DNA kubazali kubantwana kufana nokubhalela abazali bakho loo ncwadi yeresiphi ngesandla.

Utshintsho lwemfuza lufana nokubhala phantsi kwe-DNA yakho. Unobumba omkhulu unokuphazamisa iresiphi. Kuyafana nqwa nezifo ezifana ne-amyloidosis. Iiseli zakho zisebenza nzima kangangoko zinako, kodwa zazi kuphela indlela yokulandela iresiphi. Olu kubhala phantsi lunokwenzeka kuwe ngeendlela ezimbini.

Ilifa

I-Amyloidosis yentliziyo idla ngokuba yingxaki yosapho. Oku kuthetha ukuba uyifumene njengelifa. Oku kwenzeka xa omnye okanye bobabini abazali bakho benotshintsho kwi-DNA yabo, kwaye impazamo ekuloo "ncwadi yeresiphi" ikopishwa kweyakho.

Ifunyenwe

Izifo ezifunyenweyo zizifo ongazifumani njengelifa, kodwa ezikhula ngaxa lithile ebomini bakho. I-Wild-type ATTR amyloidosis kunye ne-dialysis-related amyloidosis zombini ziyimizekelo yezi zifo ezifunyenweyo. Kodwa akukho nanye kwezi inxulumene notshintsho kwi-DNA yakho.

• I-Wild-type ATTR amyloidosis yenzeka kuba umzimba wakho wenza iiproteni ngokuchanekileyo, kodwa ngokuhamba kwexesha ezo proteni azizinzanga (yiyo loo nto esi sifo sinxulumene nobudala). Ekugqibeleni, iproteni imane igobeka ngendlela engafanelekanga.
• I-amyloidosis enxulumene ne-dialysis yenzeka xa iproteni eqhelekileyo engenakuhluzwa yi-dialysis, njengezintso zakho, iqokelelana emzimbeni wakho. Ekuhambeni kwexesha, loo proteni iqokelelana ide ibangele iingxaki.

Ingaba oku kuyasulela?

Hayi, `(Amyloidosis)` ayisosifo esosulelayo nangayiphi na indlela.

Ugqirha uyixilonga njani le nto? (Uxilongo)

Ugqirha uza kuqala acinge ukuba une-`(Cardiac Amyloidosis)` ngokusekelwe kwiimpawu zakho, uvavanyo lomzimba (ugqirha ujonga, uziva, kwaye umamele umzimba wakho ukuze abone iimpawu zesifo), kunye nembali yezonyango yosapho lwakho. Emva koko baza kwenza uvavanyo lokuxilonga, uvavanyo lwemifanekiso, kunye novavanyo lwelabhoratri ukuqinisekisa ukuba unayo na i-`(Cardiac Amyloidosis)` okanye akunjalo.

Ukuba kukho umntu kusapho lwakho one-amyloidosis yosapho, usenokungadingi vavanyo okanye ungadinga olunye uvavanyo. Uvavanyo lwe-genetic lunokwenziwa ukuze kubonwe ukuba ngaba une-genetic mutations enokubangela i-cardiac amyloidosis.

Zeziphi iimvavanyo ezenziwayo ukuze kufunyaniswe esi sifo?

Ukuba ugqirha wakho ukrokrela ukuba une-"Cardiac Amyloidosis", banokuyalela olunye okanye ngaphezulu kwezi vavanyo zilandelayo.

Uvavanyo lwelabhoratri

Olu vavanyo luthatha iisampulu zezicubu, igazi, okanye olunye ulwelo lomzimba. Idayi ekhethekileyo ebizwa ngokuba yiCongo red isetyenziswa kwezi vavanyo ezininzi. Le dayi ibangela ukuba iindawo apho i-amyloid ibekwe khona zikhanye luhlaza phantsi kweemeko ezithile zokukhanya.

• I-biopsy yezicubu: Kule nto, oogqirha bathabatha isampuli encinci yezicubu kwindawo ethile, njengemisipha yentliziyo, baze bayivavanye kwilebhu. Iisampuli zezicubu zinokuthathwa nakwezinye iindawo, kodwa ukuba isampuli evela kwindawo engeyiyo eyentliziyo inika iziphumo ezilungileyo, kufuneka iqinisekiswe ngovavanyo lwemifanekiso.
• Uvavanyo lwegazi: Kwezinye iintlobo ze-cardiac amyloidosis, iiproteni ze-amyloid zijikeleza egazini. Uvavanyo lwelabhoratri lunokufumanisa ezi proteni.
• Uvavanyo lomchamo: Kwezinye iimeko, iiproteni ze-amyloid zinokubakho kumchamo. Uvavanyo lobukho bezi proteni kumchamo lunokunceda ekuxilongweni.

Uvavanyo lokufota

Ngenxa yokuba i-cardiac amyloidosis inokubangela ukuba intliziyo yakho ikhule kwaye itshintshe imilo, iimvavanyo zomfanekiso zinokunceda ekuxilongeni imeko. Uvavanyo olunokwenziwa luquka:

• I-Echocardiogram:Olu vavanyo lufana nelulwane elisebenzisa isandi ukufumana indlela yalo. Lisebenzisa amaza esandi asebenza rhoqo. Isixhobo esikhupha isandi sibekwa eluswini lwesifuba, kwaye oogqirha banokubona intliziyo ngokujonga indlela amaza esandi aphuma ngayo kwizakhiwo ezahlukeneyo emzimbeni. Oku kuluncedo kakhulu ekuboneni ukuba iindawo ezithile zentliziyo zityebile ngokungaqhelekanga.
• I-Scintography: Kolu vavanyo, oogqirha bafaka i-tracer, into ene-radioactive kancinci (kodwa engenabungozi), egazini lakho. I-tracer iqokelela kwiindawo apho iproteni ye-amyloid iqokelele khona. Ezi ndawo zinokubonakala ngokulula ngovavanyo lwemifanekiso olubizwa ngokuba yi-SPECT scan (Single-Photon Emission Computed Tomography) .
• I-Magnetic Resonance Imaging (MRI): Olu vavanyo lusebenzisa i-magnet enamandla kakhulu kunye netekhnoloji yekhompyutha ukwenza umfanekiso wentliziyo. I-MRI ineenkcukacha ezininzi, ngoko oogqirha banokubona ukutyeba okanye ezinye iinguqu entliziyweni.

I-Electrocardiogram (ECG okanye i-EKG)

Kwi-ECG, ii-sensors ezininzi (ezidla ngokuba yi-12) zinamathele kulusu lwesifuba sakho. Ezi sensors zibona umbane ohamba entliziyweni yakho kwaye zibonisa amandla aloo mqondiso wombane njengegagasi ephepheni okanye kwisikrini. Iingcali eziqeqeshiweyo, ezifana noogqirha, zinokuhlalutya igagasi ukuze zibone ukuba kukho naluphi na utshintsho olungaqhelekanga kwindlela iindawo ezithile zentliziyo yakho eziqhuba ngayo umbane.

Uvavanyo lwe-genetic

Ngokuxhomekeke kwiziphumo zovavanyo oluthile, ugqirha wakho unokucebisa uvavanyo lwemfuza. Olu vavanyo lunokuchonga utshintsho kwi-DNA yakho olunokubangela i-amyloidosis yakho.

Ngaba kukho unyango lwe-Amyloidosis?

Iintlobo ezininzi ze-`(Cardiac Amyloidosis)` zinokunyangwa, kodwa esi sifo asinakunyangwa ngokupheleleyo rhoqo . Unyango luyahluka ngokuxhomekeke kuhlobo lwe-`(Amyloidosis). Ngokubanzi, ukuxilongwa kwangoko kubaluleke kakhulu . Ukuba `(Amyloidosis)` ifunyenwe kwangethuba, umonakalo wexesha elide entliziyweni unokuthintelwa. Ukuba awufunyaniswa kwaye awunyangwa kwangethuba, umonakalo ongunaphakade unokwenzeka. Indlela ekuphela kwayo yokulungisa umonakalo osisigxina wentliziyo `` (Ukufakelwa kwentliziyo)`.

I-AL amyloidosis

Unyango lwe-AL Amyloidosis lufana kakhulu neendlela eziqhelekileyo zokunyanga umhlaza. Nazi:

• Unyango lweKhemotherapy: Njengakwisifo somhlaza, unyango lwekhemotherapy lunokutshabalalisa iiseli zeplasma ezingasebenzi kakuhle.
• Ukufakelwa kweeseli ze-stem: Olu nyango ludla ngokwenziwa kunye ne-chemotherapy, kwaye luthatha indawo yeeseli ze-plasma ezingasebenzi kakuhle ngeeseli ezintsha. Ezi seli ze-stem zihlala zithathwa kuwe (autologous). Ngenxa yokuba zezakho, umzimba wakho uyaziqonda kwaye awuzilahli okanye awuziphenduli kakubi.
• Unyango lwe-immunotherapy:Inkqubo yakho yokuzikhusela komzimba iyakwazi ukubona nokutshabalalisa iiseli ezingasebenzi kakuhle. Kwizifo ezifana nomhlaza okanye i-AL Amyloidosis, inkqubo yakho yokuzikhusela yomzimba ayiziboni ezo seli njengezineengxaki. I-immunotherapy ifundisa inkqubo yakho yokuzikhusela yomzimba ukuba ibone kwaye itshabalalise iiseli ezineengxaki.

i-ATTR amyloidosis

Olu hlobo lwe-"(Amyloidosis)" lwenzeka xa isibindi sakho senza iiproteni eziqhekeka lula. Le ngxaki ifuna ukuba uyeke lonke unyango. Uluhlu olungezantsi luquka unyango oluvunyiweyo ngurhulumente kunye nolo lusesesigabeni sophando.

• Ukufakelwa kwesibindi: Ngaphambili, ukufakelwa kwesibindi yayiyeyona ndlela yokuthintela isibindi sakho ekwenzeni iiproteni ezingalunganga. Nangona le ingaseyona ndlela yodwa, iseyindlela eluncedo nesebenzayo.
• Izithulisi zemfuza: I-ATTR Amyloidosis ibangelwa kukuguquka kwemfuza kwi-DNA yakho, njengotshintsho olukwincwadi yeresiphi. Izithulisi zemfuza ngamayeza asebenza njengekhadi leresiphi yexeshana. Endaweni yokuba iiseli zakho zilandele iresiphi engalunganga, zinika imiyalelo emitsha ukuze zikwazi ukwenza iproteni ngokuchanekileyo.
• Izinzisi: La mayeza athintela iimolekyuli ze-TTR ukuba zingasongi okanye ziqhekeke.
• Izithinteli zeFibril: Xa iiproteni ze-amyloid ziqala ukuhlangana, zenza izakhiwo ezifana nefayibha ezibizwa ngokuba yi-fibrils. La mayeza ayayimisa ukwakheka kwe-fibrils.

I-amyloidosis enxulumene ne-dialysis

Olu hlobo lwe-`(Amyloidosis)` lukhula kubantu abafuna i-`(dialysis)` kuba iproteni ye-`(beta-2M)` iyaqokelelana. Ngokwesiqhelo, izintso zakho ziyahluza zize zisuse loo proteni, kodwa i-`(dialysis)` eqhelekileyo ayinakukwenza oko. Yingakho kuthatha iminyaka ukuba abantu abakwi-`(dialysis)` bavelise esi sifo. Okwangoku, kukho iindlela ezimbini zokusinyanga:

• Ukufakelwa kwezintso: Izintso eziphilileyo zithetha ukuba awusayidingi i-dialysis, kwaye izintso ezifakelweyo zinokuhluza iproteni ye-beta-2M engaphezulu. Ukufakelwa kwezintso kunokuthintela i-amyloidosis enxulumene ne-dialysis ukuba ingabi mandundu, kodwa abaphandi abaqinisekanga ukuba kuya kuyisusa na iproteni ye-amyloid eqokelelene emzimbeni.
• Izihluzo ezikhethekileyo: Nangona izihluzo eziqhelekileyo ze-dialysis zingenakuzisusa iiproteni ze-beta-2M, kukho izihluzo ezikhethekileyo ezinokuzisusa ubuncinane kancinci. Ngelishwa, ezi zihluzo azizihluzi zonke iiproteni, kwaye azizihluzi zonke. Oku kuthetha ukuba nangona emva kweminyaka emininzi ye-dialysis, i-amyloidosis enxulumene ne-dialysis inokuvela ekugqibeleni.

Unyango olungachazwanga ngqo

Kukho iintlobo ngeentlobo zonyango kunye namayeza anokunceda abantu abaneCardiac Amyloidosis. Ezi zihlala zinyanga iimpawu zesifo kwaye zingabandakanya:

• Ukufakelwa kwentliziyo: Abantu abonzakele kakhulu kwintliziyo ngenxa ye-cardiac amyloidosis ngamanye amaxesha badinga ukufakelwa intliziyo. Olu tyando ludla ngokwenziwa emva kokuba unobangela we-amyloidosis usonjululwe okanye unyangiwe.
• Amayeza okulwa nokubetha kwentliziyo: La mayeza anceda ekuthinteleni ukubetha kwentliziyo okungaqhelekanga, amanye awo anokuba yingozi.
• Izixhobo ezifakelwayo: Ezi zixhobo ziquka ii-pacemakers kunye nee-implantable cardioverter defibrillators (ICDs) . Zilawula izinga lentliziyo yakho ngokukhupha umbane kunye nokuthintela i-arrhythmias eyingozi.
• Amayeza okuhambisa umchamo: La mayeza anceda ngokukodwa ekugcinweni kolwelo (uphawu oluqhelekileyo lokusilela kwentliziyo) ngokuphucula ukusebenza kwezintso zakho. Nangona kunjalo, akufanele uwasebenzise la mayeza ukuba unesifo esifana nesifo sezintso esinzima.
• I-Doxycycline : Iintambo zokukhanya kwi-AL amyloidosis nazo ziyingozi kwiiseli zentliziyo yakho. Ngenxa yezizathu ezingakaziqondi ngokupheleleyo iingcali, i-antibiotic i-doxycycline inokuyilwa loo miphumo iyingozi.

Ngaba kukho naziphi na iziphumo ebezingalindelekanga zonyango?

Iingxaki kunye neziphumo ebezingalindelekanga zonyango lwesi sifo zahlukile kakhulu. Apha ngezantsi kukho ezinye izinto ezinokwenzeka eziqhelekileyo. Nangona kunjalo, ugqirha wakho unokukuchazela ngcono iingxaki, iziphumo ebezingalindelekanga, kunye neminye imingcipheko. Oku kungenxa yokuba bayayazi imeko yakho ngokwam, ngoko ke banokulungisa ulwazi lwabo kunye neenkcazo zabo ukuze zihambelane nemeko yakho ethile.

Ndiza kuziva bhetele nini emva konyango?

Unyango lwe-Amyloidosis yentliziyo ludla ngokukhawulelwa ekulawuleni iimpawu zakho. Ezinye iindlela zonyango zinokunika isiqabu, kodwa iziphumo zezokwexeshana. Kwezinye iimeko, unyango lunokubangela iziphumo ebezingalindelekanga ezinokukwenza uzive ungonwabanga okwethutyana, kodwa ugqirha wakho unokukunika amayeza kunye nesikhokelo sokunceda ukunciphisa ubukhali bezo ziphumo bezingalindelekanga.

Ngaba oku kungathintelwa? Okanye ngaba ingozi ingancitshiswa?

Ngelishwa, i-amyloidosis ayinakuthintelwa (ngaphandle kwesinye, jonga ngezantsi). Ekubeni unobangela we-amyloidosis engeyomfuzo ungaziwa, akukho ndlela yokuyithintela. I-amyloidosis efuzekileyo ayinakuthintelwa, kuba ikwi-DNA oyizuze kubazali bakho.

Okukuphela kwento eyahlukileyo yi-cardiac amyloidosis, enxulunyaniswa ne-dialysis. Le meko inokuthintelwa ukuba awuzange wenze i-dialysis ixesha elide, okanye ukuba usebenzisa izihluzo ezikhethekileyo ukunqanda ukwakheka kweeproteni ze-beta-2M. Nangona kunjalo, ezi zihluzo azihlali zikunqanda ngokupheleleyo ukwakheka, oko kuthetha ukuba zihlala ziyindlela nje yokulibazisa ukuqala kwesi sifo.

Iyintoni imbonakalo yesi sifo?

I-Amyloidosis yentliziyo sisifo esiqhubekayo, esineempawu zokusilela kwentliziyo eziya zisiba mandundu. Ekugqibeleni, esi sifo singenza intliziyo yakho ibe buthathaka ide ingakwazi ukusebenza. Ukuba ayinyangwa, imeko iba mbi kakhulu.

Ngaphandle konyango, ixesha eliqhelekileyo lokusinda kubantu abane-AL Amyloidosis lingaphantsi kweenyanga ezisibhozo. Kwabo baneemeko ezinzima kakhulu, linokuba ziinyanga ezintathu ukuya kwezintandathu. Kwabo bane-ATTR Amyloidosis, ixesha eliqhelekileyo lokusinda liyiminyaka emibini ukuya kwemihlanu. Kwabo baneentsapho ezinganyangwanga ezine-ATTR Amyloidosis, imeko imbi nangakumbi, ixesha eliqhelekileyo lokusinda liyiminyaka emibini ukuya kwemithathu.

Ndingazinyamekela njani kwaye ndilawule iimpawu zam?

Ngelishwa, azikho izinto ezininzi onokuzenza ukuze uzincede kwangoko emva kokuba ufumene i-amyloidosis. Ukuba kukho umntu kusapho lwakho onesifo, kufuneka uthethe nogqirha wakho malunga novavanyo lwemfuza. Nangona kungengabo bonke abantu abanotshintsho olunxulumene ne-cardiac amyloidosis abaya kuba nesifo, kubalulekile ukwazi ukuba ungasifumana na. Ngale ndlela, unokwazi ukubona iimpawu kwaye uqale unyango kwangethuba.

Ukuba une-`(Cardiac Amyloidosis)`, kubaluleke kakhulu ukulandela imiyalelo kagqirha wakho malunga namayeza kunye nonyango. Uninzi lwala mayeza lusebenza ngeendlela ezithile, kwaye ukuze asebenze kakuhle, kubalulekile ukuwathatha kanye njengoko ugqirha wakho ekuxelela.

Ndifanele ndimbone nini ugqirha okanye ndifune unyango olukhawulezileyo?

Emva kokuba ufunyaniswe une-Cardiac Amyloidosis, ugqirha wakho uya kucwangcisa utyelelo lokulandelela rhoqo. Ngokuxhomekeke ekubeni imeko yakho imbi kangakanani, unokufuna ukubona ugqirha wakho rhoqo.

Ugqirha wakho uza kukuxelela ukuba zeziphi iimpawu ekufuneka zibangele ukuba ufumane unyango. Nangona kunjalo, ngokubanzi, fowunela ugqirha wakho ukuba unezinye zezi mpawu zilandelayo:

• Ukuziva unesizungu okanye utyhafile xa uhleli okanye umile: Oku kubizwa ngokuba yi-"Orthostatic Hypotension." Oku kwenzeka kuba uxinzelelo lwegazi oluphantsi lunciphisa inani legazi eliya engqondweni xa umi.
• Ukudumba kwamalungu okanye isisu: Oku kudla ngokubangelwa kukugcinwa kolwelo.
• Ukwehla kobunzima ngequbuliso: Olu luphawu olubalulekileyo, ingakumbi xa ungazami ukunciphisa umzimba.

Ndingaya nini kwi-ER (iGumbi loNgxamiseko)?

Ngenxa yokuba i-cardiac amyloidosis inokuphazamisa kakhulu ukusebenza kwentliziyo yakho, ezinye iimpawu zibonisa ukuba udinga uncedo lwezonyango ngokukhawuleza.Kuyimfuneko. Ezinye zazo zezi:

• Ukuziva ukubetha kwentliziyo.
• Ukubetha kwentliziyo okukhawulezayo, okucothayo, okanye okungahambelaniyo ngendlela engaqhelekanga.
• Ubunzima bokuphefumla nangasiphi na isizathu.

Okokugqibela, amanqaku ambalwa abalulekileyo (Umyalezo Wokuya Ekhaya)

I-Amyloidosis yentliziyo yimeko enzima kancinci, kodwa kubaluleke kakhulu ukuyazi.

Khumbula, nangona esi sifo sinokuqhubeka kancinci, ukubonwa kwangethuba kunye nonyango olufanelekileyo kunokunceda kakhulu ekwandiseni ubomi bakho kwaye kuphucule umgangatho wobomi bakho.

Ngamanye amaxesha ukufakelwa amalungu omzimba kunokunyanga le meko. Ngenxa yamayeza amatsha kunye nonyango, abantu abaninzi abanale sifo banokulawula iimpawu zabo baze baphile iminyaka emininzi emva kokuba isifo siphuhlile.

Ukuba unemibuzo okanye amathandabuzo malunga noku, ngokuqinisekileyo thetha nogqirha wakho . Banokukunika icebiso elifanelekileyo. Ungoyiki, fumana ulwazi, buza imibuzo. Awuwedwa.

I- Amyloidosis yentliziyo, i-Amyloidosis yentliziyo, ukubekwa kweeproteni, isifo sentliziyo, iimpawu zesifo sentliziyo, unyango lwe-Amyloidosis, i-AL Amyloidosis, i-ATTR Amyloidosis, isifo sentliziyo