Uma unesifo sesikhathi eside, noma esingamahlalakhona, njenge-ATTR amyloidosis esizuzwe njengefa, udinga ithimba elikhulu lodokotela kanye nochwepheshe bezempilo ukuze bakusize usilawule. Lokhu kuyiqiniso ikakhulukazi kubantu abane-hATTR amyloidosis. Lesi yisimo sofuzo. Okwenzekayo ukuthi iphrotheni engajwayelekile ebizwa ngokuthi i-amyloid inqwabelana ezithweni ezibalulekile njengenhliziyo yakho, izinso, isimiso sezinzwa, kanye namathumbu. Ngakho-ke, ngenxa yokuthi lesi simo sithinta izingxenye eziningi zomzimba wakho, ithimba lakho lezempilo le-hATTR lizofaka ochwepheshe abaningana.
Ubani oseqenjini lakho lezokwelapha le-hATTR amyloidosis?
Manje ake sibheke ochwepheshe bezokwelapha ababalulekile abazokusiza ngokunakekelwa kwempilo yakho. Ndawonye, bazokusiza ukuthi uphathe kangcono isimo sakho.
Udokotela wezinzwa
Ku-hATTR, isimo esibizwa ngokuthi i-peripheral neuropathy sivame kakhulu. Kalula nje, lokhu kuwukulimala kwemizwa ezandleni zakho, ezinyaweni, nasemilenzeni engezansi. Ungase ube nezimpawu ezinjengalezi:
- Ubuhlungu
- Ukuba ndikindiki
- Ukuvuvukala
- Ubuthakathaka emilenzeni
- Kuzwakala sengathi kuhlatshwa njengenduku.
Njengoba lesi sifo siqhubeka, imisipha yakho ingase iqine futhi ibe buthaka, okwenza kube nzima ukuhamba nokwenza noma yini edinga amakhono amahle okunyakaza.
Udokotela wezinzwa uzokwenza izivivinyo, njengokuhlolwa kokuqhuba izinzwa, ukuze aqaphe ngokuseduze ukusebenza kwezinzwa zakho eziseceleni. Lokhu kuzosiza ekunqumeni ukuthi unesifo sezinzwa nokuthi sibi kangakanani.
Izindlela zokwelapha eziningi ezivunyelwe i-hATTR zingasiza ekwelapheni isimo sakho sezinzwa. Udokotela wakho angase futhi akunikeze eminye imithi yokusiza ngobuhlungu bezinzwa.
| Isigaba sezidakamizwa | Izibonelo zezidakamizwa |
|---|---|
| Ukwelashwa okuvunyiwe kwe-hATTR | Eplontersen (Wainua), Inotersen (Tegsedi), Patsiran (Onpattro), Vutrisiran (Amvuttra) |
| Imithi yobuhlungu bemizwa | I-Amitriptyline, i-Duloxetine (i-Cymbalta), i-Gabapentin (i-Neurontin), i-Pregabalin (i-Lyrica) |
Udokotela wenhliziyo
Lo dokotela welapha izifo zenhliziyo nemithambo yegazi. Abantu abane-hATTR basengozini enkulu yesifo senhliziyo. Lokhu kungenxa yokuthi ama-amyloid deposits anganqwabelana enhliziyweni. Lokhu kungaholela ezimweni ezilandelayo:
Into ebaluleke kakhulu ukuqaphela lezi zimpawu nokufuna iseluleko sezokwelapha ngokushesha okukhulu uma kudingeka.
| Imiphumela engaba khona enhliziyweni | Incazelo elula |
|---|---|
| Ukwehluleka Kwenhliziyo Okubangelwa Ukuqina Kwenhliziyo | Inhliziyo iba buthaka futhi uketshezi luqoqana emaphashini nakwezinye izicubu. |
| I-Hypertrophic Cardiomyopathy | Ukuqina kwemisipha yenhliziyo. |
| Isifo Senhliziyo Esibangelwa Yinhliziyo | Ukuvaleka kwemithambo yegazi ehlinzeka inhliziyo. |
| I-Arrhythmia | Ukushaya kwenhliziyo okungajwayelekile. |
| I-Syncope | Ukuzizwa unesizungu noma ukuphelelwa yithemba. |
Uma kutholakale ukuthi une-hATTR, udokotela wakho wenhliziyo uzokwenza izivivinyo eziningana ukuze abone ukuthi inhliziyo yakho isebenza kahle kangakanani. Lokhu kungafaka phakathi i- echocardiogram , i- MRI scan, ukuhlolwa kokuhamba ngezinyawo kwemizuzu eyi-6, kanye nokuhlolwa kwegazi. Uma inhliziyo yakho ibuthakathaka, uzonikezwa imithi yokulawula lesi simo, njengemithi yokukhipha isisu, imikhawulo kasawoti kanye noketshezi, kanye ne -pacemaker .
Udokotela wamehlo
Uma une-hATTR, kubalulekile ukubona uchwepheshe wamehlo njalo. Cishe u-20% wezinguquko zofuzo ezibangela i-hATTR zithinta amehlo. Lokhu kungabangela izimpawu ezifana nalezi:
- Ama-floating amnyama (ukubona amachashazi amnyama entanta phambi kwamehlo)
- Amehlo omile
- I-Glaucoma noma ukucindezeleka kwamehlo okwenyuka
- Ukungacaci kahle kwe-vitreous (ukufiphala kwejeli ecacile egcwalisa inhlamvu yeso)
- Ezinye izinguquko zombono
Uma kudingeka, udokotela wakho wamehlo angenza ukuhlinzwa okubizwa ngokuthi i-trabeculectomy ukwelapha i-glaucoma. Angenza nokuhlinzwa okubizwa ngokuthi i-vitrectomy ukususa ijeli esweni.
Umeluleki Wezakhi Zofuzo
Uma udokotela wakho esola ukuthi une-hATTR, uzokudlulisela kumeluleki wezakhi zofuzo . Bangakuhlola ukuguquka kwezakhi zofuzo okungase kubangele lesi sifo. Futhi, uma othile emndenini wakho oseduze (umzali, umfowenu noma ingane) etholakale ene-hATTR, kufanele nakanjani uhlolwe. Lokhu kungenxa yokuthi amalungu omndeni aseduze anethuba elingu-50% lokuthola lolu guquko lwezakhi zofuzo. Umeluleki wezakhi zofuzo uzochaza lezi zivivinyo kuwe futhi axoxe nawe uma imiphumela isifikile.
Amanye amalungu abalulekile eqembu lakho lezokwelapha
Ngaphezu kochwepheshe okukhulunywe ngabo ngenhla, abanye abaningana badlala indima ebalulekile enhlalakahleni yakho.
| Ochwepheshe | Bangakusiza kanjani? |
|---|---|
| Udokotela Wezinzwa | Cishe ingxenye yesithathu yabantu abane-hATTR bangase babe nokufakwa kwe-amyloid ezinso zabo. Ungase ungadingi ukubona lochwepheshe kuze kube yilapho unezimpawu zesifo sezinso. Udokotela wakho oyinhloko uzohlola ukusebenza kwezinso zakho ngokuhlolwa kwegazi kwaminyaka yonke (njenge -serum creatinine ). Uma lokhu kuhlolwa kungavamile, uzothunyelwa kudokotela wezinso. |
| Uchwepheshe Wezamathambo | Iziguli ezine-hATTR zisengozini enkulu yokuthola i-carpal tunnel syndrome . Lokhu kungabangelwa ukubekwa kwe-amyloid ezihlakaleni. Kunganconywa ukufakelwa izihlakala zesandla kanye nokuzivocavoca umzimba ngenkathi ulele. Ezimweni ezimbi kakhulu, kungadingeka ukuhlinzwa. |
| Udokotela Wezokwelapha Ngomzimba/Emsebenzini | I-hATTR ingenza kube nzima ukuhamba nokwenza imisebenzi yansuku zonke. Abelaphi bomzimba kanye nabelaphi bomsebenzi bangakusiza ukuthi wenze le misebenzi ibe lula. Ucwaningo lukhombisile ukuthi ukuhlanganisa lezi zindlela zokwelapha nezinye izindlela zokwelapha, njengemithi, kungaveza imiphumela emihle. |
| Umhlinzeki Wokunakekelwa Okuyinhloko | Ngisho noma ubona ochwepheshe abaningi, udokotela womndeni wakho noma udokotela wokunakekelwa okuyinhloko uyingxenye ebalulekile yethimba lakho. Angakwenza ukuhlolwa kwakho kwempilo kwaminyaka yonke, akwelaphe uma ugula, futhi asebenze "njengomholi" wethimba lakho lezokunakekelwa kwempilo, exhumana nabo bonke abanye odokotela. |
Ngokuvamile, uzokwazi ukuthola zonke izinsizakalo ozidingayo esikhungweni sezokwelapha noma esibhedlela esikhulu esikhethekile ekwelapheni i-hATTR amyloidosis. Lokhu kwenza kube lula ngabo bonke odokotela bakho ukuthi basebenzisane njengeqembu. Khuluma nodokotela wakho wokunakekelwa okuyinhloko mayelana nokuxhumanisa ukwelashwa kwakho.
Umlayezo Wokuya Nawe Ekhaya
- I-hATTR amyloidosis yisifo esiyinkimbinkimbi esithinta izitho eziningi zomzimba, ngakho-ke sidinga ithimba lodokotela abanolwazi oluhlukile ukuze basiphathe.
- Ithimba lakho lezokwelapha lingafaka ochwepheshe ezindaweni ezifana ne-neurology, i-cardiology, i-ophthalmology, kanye ne-nephrology.
- Udokotela wakho wokunakekelwa okuyinhloko (udokotela womndeni) uyisixhumanisi esiyinhloko esixhumanisa lonke leli qembu.
- Khuluma ngokukhululekile nethimba lakho lezokwelapha ngesimo sakho, ukwelashwa, kanye nanoma yiziphi izinkinga ongase ube nazo. Ungesabi ukubuza imibuzo. Uyilungu elisebenzayo lethimba lakho lezempilo.
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