Asinaki kakhulu ezinye zezimpawu ezenzeka emizimbeni yethu, akunjalo? Sikubheka njengobuhlungu obuncane, ukuqubuka kwesikhumba okuncane, noma ukukhathala nje. Kodwa ngezinye izikhathi, ngemuva kwalezi zimpawu ezibonakala zivamile, kungase kube nesimo sezokwelapha esingavamile nesingathi sína. Namuhla sizokhuluma ngesifo esisodwa esinjalo sofuzo, i-Fabry Disease. Nakuba lesi kuyisifo esingavamile, kungaba kubaluleke kakhulu kuwe nomndeni wakho ukuba niqaphele.
Iyini Isifo sikaFabry?
Kalula nje, isifo sikaFabry yisifo sofuzo lapho uhlobo oluthile lwamafutha emaseli emizimba yethu lungenakuhlukaniswa kahle, okubangela ukuba akheke ezithweni zethu zomzimba. Uma la mafutha eqoqana, angalimaza izitho ezibaluleke kakhulu emzimbeni wethu, njengezinso, inhliziyo, kanye nesistimu yezinzwa.
Isici esingavamile salesi sifo ukuthi izimpawu ziyahlukahluka kumuntu nomuntu . Nakuba abanye abantu benezimpawu ezicashile kakhulu, ezingacacile kahle, kwabanye zingaba zimbi ngokwanele ukuthi zithinte izimpilo zabo. Futhi, ngoba lezi zimpawu zifana nezezinye izifo ezivamile, ngezinye izikhathi kungenzeka ukuthola lesi sifo sekwephuzile.
Yiziphi izimpawu ezivamile?
Umuntu onesifo sikaFabry angaba nezimpawu eziningana ngesikhathi esisodwa. Ake sibheke eziyinhloko nezivame kakhulu kuzo.
| Isibonakaliso | Kuzwakala kanjani lokhu? |
|---|---|
| Ubuhlungu ezandleni nasezinyaweni | Ukusha, ukungazweli, izinzipho nezinaliti , noma ubuhlungu obukhulu ezintendeni nasematheni ezandla. Ngezinye izikhathi lobu buhlungu abubekezeleleki. |
| Amabala esikhumba | Ngokuvamile kuvela njengeqoqo lamabala amancane, abomvu noma ansomi endaweni ephakathi kwezinqe namadolo. Lawa ngokuvamile awabuhlungu. |
| Ukujuluka okunciphile | Ukujuluka kancane kunokujwayelekile, noma kwabanye abantu abajuluki nhlobo, okwenza kube nzima ukubekezelela isimo sezulu esishisayo noma ukuzivocavoca umzimba. |
| Umkhuhlane ovamile | Ukuzwa njalo umkhuhlane ngaphandle kwesizathu esithile. |
| Ukuvuvukala kwemilenze | Ukuvuvukala, ikakhulukazi emilenzeni engezansi, emaqakaleni nasezinyaweni. Lokhu kungaba uphawu lokuthi izinso ziyathinteka. |
| Ukukhathala okukhulu | Ukuzizwa ukhathele kakhulu futhi ubuthakathaka usuku lonke obungapheli ngisho nangemva kokulala kahle noma ukuphumula. |
| Izinkinga zesistimu yokugaya ukudla | Izimpawu zifaka phakathi ubuhlungu besisu ngokushesha ngemva kokudla, ukuzizwa usuthi, ukunyakaza kwamathumbu njalo, isicanucanu kanye nokuhlanza. |
Ingabe kukhona umehluko endleleni ethinta ngayo abesifazane nabesilisa?
Yebo, impela. Ngenxa yofuzo lwalesi sifo, amadoda anamathuba amaningi okusithola . Futhi, izimpawu zibonakala kakhulu futhi zinzima kwabesilisa.
Ngisho noma abesifazane bethola isifo sikaFabry, abaningi bangase bangabi nazo izimpawu noma babe nezimpawu ezincane kakhulu. Kodwa-ke, lokhu akusho ukuthi abesifazane abathinteki yilesi sifo. Abanye besifazane bangase babe nezimpawu ezinzima, ikakhulukazi ubuhlungu bemizwa. Ngakho-ke, akuwona umqondo omuhle ukungazinaki izimpawu ngoba zincane.
Lokhu kuzithinta kanjani izitho ezahlukene zomzimba?
Ukuqongelela kwamafutha esifweni sikaFabry konakalisa izingxenye ezahlukene zomzimba. Ake sibone ukuthi kanjani.
Izinso
Enye yezinkinga ezinkulu neziyingozi zalesi sifo ukwehluleka kwezinso. Ukugcwala kwamafutha emithanjeni yegazi kunciphisa imithambo yegazi ehlinzeka ngezinso, okubangela umonakalo ezinso. Izimpawu zingase zihlanganise iphrotheni noma igazi emchameni, kanye nokuvuvukala kwemilenze. Ezimweni ezimbi kakhulu, kungadingeka i-dialysis yempilo yonke noma ukufakelwa kwezinso.
Uhlelo lwezinzwa
Abantu abaningi baqala ukuzwa isifo sikaFabry ngobuhlungu bezinzwa. Izimpawu eziyinhloko ukushisa, ukungazweli, kanye nobuhlungu obungabekezeleleki, ikakhulukazi ezandleni nasezinyaweni. Lezi zimo ezibuhlungu zingahlala amahora noma ngisho nezinsuku. Lobuhlungu bungahambisana nobuhlungu bomzimba, umkhuhlane, kanye nokukhathala okukhulu.
Inhliziyo
Isifo senhliziyo sivamile futhi. Singabangela ukushaya kwenhliziyo okungajwayelekile, ukuqina kwemisipha yenhliziyo, noma izinkinga ngama-valve enhliziyo (njengokungakwazi ukusebenza kahle kwe-mitral). Lezi zimo zingandisa nengozi yezinkinga ezinkulu ezifana nesifo sohlangothi.
Isikhumba
Uphawu oluyinhloko amabala abomvu amnyama (ama-angiokeratomas) esixoxe ngawo ngaphambili. Kwabafana, lawa avame ukubonakala ezandleni, emadolweni, ezindololwaneni, kanye nendawo ephakathi kwezinqe namadolo. Abesifazane banamathuba amancane okuthola la mabala.
Amehlo
Lolu uphawu oluqondile kakhulu. Imicu emhlophe efana noboya noma amaphethini afana nendandatho (ama-corneal whorls) angavela ebusweni beso eduze kwe-cornea. Nakuba lokhu kungaphazamisi umbono, kuyizinkomba ezibaluleke kakhulu kudokotela ukuze axilonge isifo sikaFabry.
Lezi zinhlobo zokushintsha kwamehlo azivamile ukubonakala kwezinye izifo, ngakho-ke ukuba nalesi sici kuwusizo olukhulu ekuxilongeni isifo sikaFabry.
Izindlebe
Abanye abantu bangase babe nokukhala ezindlebeni, noma "i-Tinnitus". Ukulahlekelwa ukuzwa nakho kungenzeka kancane kancane ngokuhamba kwesikhathi noma ngokuzumayo.
Amaphaphu kanye nesistimu yokugaya ukudla
Izimpawu zokuphefumula ezifana nomsindo 'wokukloloda' lapho uphefumula, ukuphelelwa umoya, kanye nokukhwehlela njalo kungase kube khona. Futhi, cishe ingxenye yabesifazane ihlangabezana nezimpawu ezifana ne-Irritable Bowel Syndrome (IBS) (ubuhlungu besisu, ukuqunjelwa).
Ingabe kunezinhlobo ezimbili zalesi sifo? (Uhlobo 1 nohlobo 2)
Yebo. Isifo sikaFabry sihlukaniswe kakhulu ngezinhlobo ezimbili.
1. Uhlobo olujwayelekile (Uhlobo 1): Lokhu kuvame ukuqala ebuntwaneni. Ukuvuvukala nobuhlungu ezithweni kungaba olunye lwezimpawu zokuqala ebuntwaneni.
2. Uhlobo lokuqalisa sekwephuzile (Uhlobo 2): Kulolu hlobo, ubuntwana kanye nokuthomba kuyinto evamile. Izimpawu ziqala ukuvela ngemva kweminyaka engu-20 noma ngaphezulu.
Kungakhathaliseki ukuthi hlobo luni, lesi sifo sivame ukuba sibi kakhulu njengoba sikhula. Isizathu esiyinhloko salokhu ukwanda kwamafutha emzimbeni ngokuhamba kwesikhathi.
Ungase ucabange ukuthi lesi yisifo esingavamile futhi akunakwenzeka ukuthi sikwenzeke kuwe. Kodwa uma wena noma othile emndenini wakho sekuyisikhathi eside nibhekene nezimpawu eziningana ezishiwo kulesi sihloko, sicela ungasinaki. Into engcono kakhulu ongayenza ukubonana nodokotela ngokushesha okukhulu bese ukhuluma ngaso.Uzokwenza izivivinyo ezidingekayo futhi athole ukuthi yini ngempela ebangela lezi zimpawu.
Umlayezo Wokuya Nawe Ekhaya
- Isifo sikaFabry yisifo esingavamile esibangelwa izakhi zofuzo esibangela ukuba amafutha aqongelelane ezithweni zomzimba.
- Izimpawu eziyinhloko ukuvuvukala kwezandla nezinyawo, ukungazweli, amabala abomvu amnyama esikhumbeni, ukuncipha kokujuluka, kanye nokukhathala okukhulu.
- Lesi sifo singalimaza kakhulu izitho ezibalulekile njengezinso, inhliziyo kanye nohlelo lwezinzwa.
- Izimpawu zivame kakhulu futhi zinzima kwabesilisa, kuyilapho abesifazane bengase babe nezimpawu ezincane noma bangabi nazo nhlobo.
- Uma wena noma othile emndenini wakho enesinye noma ngaphezulu sezimpawu ezishiwo kulesi sihloko futhi ziqhubeka isikhathi eside, sicela uxhumane nodokotela wakho ngokushesha ukuze uthole iseluleko.
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