Asizinaki kangako ezinye zeempawu ezenzeka emizimbeni yethu, akunjalo? Sicinga ukuba yintlungu encinci, irhashalala elincinci lolusu, okanye ukudinwa nje. Kodwa ngamanye amaxesha, emva kwezi mpawu zibonakala ziqhelekile, kunokubakho imeko yezonyango engaqhelekanga nembi kakhulu. Namhlanje siza kuthetha ngesifo esinye esifana neso semfuza, iFabry Disease. Nangona esi sisifo esingaqhelekanga, kunokuba kubaluleke kakhulu kuwe nakusapho lwakho ukuba nisazi ngaso.
Yintoni isifo sikaFabry?
Ngamafutshane, isifo sikaFabry sisifo semfuza apho uhlobo oluthile lwamafutha kwiiseli zomzimba wethu lungenakwahlulwa ngokufanelekileyo, nto leyo ebangela ukuba aqokeleleke kwizitho zethu zomzimba. Xa la mafutha eqokelelwa, anokonakalisa izitho ezibalulekileyo emzimbeni wethu, njengezintso, intliziyo, kunye nenkqubo yemithambo-luvo.
Into engaqhelekanga ngesi sifo kukuba iimpawu ziyahluka kumntu nomntu . Nangona abanye abantu beneempawu ezingabonakaliyo nezingabonakaliyo, kwabanye zinokuba nzima ngokwaneleyo ukuba zichaphazele ubomi babo. Kwakhona, kuba ezi mpawu zifana nezezinye izifo eziqhelekileyo, ngamanye amaxesha kunokwenzeka ukuba isifo sibonwe emva kwexesha.
Ziziphi iimpawu eziqhelekileyo?
Umntu onesifo sikaFabry unokuba neempawu ezininzi ngaxeshanye. Makhe sijonge ezona ziphambili nezixhaphakileyo kwezi.
| Uphawu | Ivakalelwa njani le nto? |
|---|---|
| Intlungu ezandleni nasezinyaweni | Ukutsha, ukungaziva, ukurhawuzelelwa ziinaliti , okanye iintlungu ezibukhali ezintendeni nasezinyaweni zezandla. Ngamanye amaxesha le ntlungu ayinakunyamezeleka. |
| Amabala esikhumba | Ihlala ibonakala njengeqela lamabala amancinci, abomvu okanye amfusa kwindawo ephakathi kweempundu namadolo. La mabala adla ngokungabi nantlungu. |
| Ukuncipha kokubila | Ukubila kancinci kunesiqhelo, okanye kwabanye abantu ababi naso kwaphela isithukuthezi, nto leyo eyenza kube nzima ukunyamezela imozulu eshushu okanye ukuzilolonga. |
| Umkhuhlane oqhelekileyo | Ukuva rhoqo umkhuhlane ngaphandle kwesizathu esithile. |
| Ukudumba kwemilenze | Ukudumba, ingakumbi emilenzeni esezantsi, emaqatheni nasezinyaweni. Oku kungaba luphawu lokuba izintso ziyachaphazeleka. |
| Ukudinwa kakhulu | Ukuziva udiniwe kakhulu kwaye ubuthathaka imini yonke obungapheli nokuba ulele okanye uphumle kamnandi ebusuku. |
| Iingxaki zenkqubo yokugaya ukutya | Iimpawu ziquka iintlungu zesisu emva nje kokutya, ukuziva uzele, ukuhamba rhoqo kwesisu, isicaphucaphu, kunye nokuhlanza. |
Ingaba kukho umahluko kwindlela echaphazela ngayo abafazi kunye namadoda?
Ewe, ngokuqinisekileyo. Ngenxa yemfuza yesi sifo, amadoda anamathuba amaninzi okusifumana . Kwakhona, iimpawu zibonakala kakhulu kwaye zinzima kumadoda.
Nokuba abafazi bafumana isifo sikaFabry, uninzi lwabo lusenokungabi nazo naziphi na iimpawu okanye lube neempawu ezincinci kakhulu. Nangona kunjalo, oku akuthethi ukuba abafazi abachaphazeleki sesi sifo. Abanye abafazi banokuba neempawu ezinzima, ingakumbi iintlungu zemithambo-luvo. Ngoko ke, ayingombono ulungileyo ukungawahoyi la mpawu kuba zincinci.
Oku kuzichaphazela njani izitho ezahlukeneyo zomzimba?
Ukuqokelelwa kwamafutha kwisifo sikaFabry konakalisa iindawo ezahlukeneyo zomzimba. Makhe sibone ukuba njani.
Izintso
Enye yezona ngxaki zinzima neziyingozi zesi sifo kukungasebenzi kakuhle kwezintso. Amafutha agcinwe kwimithambo yegazi anciphisa imithambo yegazi ehambisa izintso, nto leyo ebangela umonakalo kwizintso. Iimpawu zingabandakanya iproteni okanye igazi kumchamo, kunye nokudumba kwemilenze. Kwiimeko ezinzima, i-dialysis yobomi bonke okanye ukufakelwa kwezintso kunokufuneka.
Inkqubo yemithambo-luvo
Abantu abaninzi baqala ukufumana isifo sikaFabry esineentlungu zemithambo-luvo. Iimpawu eziphambili kukutsha, ukungaziva, kunye nentlungu enganyamezelekiyo, ingakumbi ezandleni nasezinyaweni. Ezi meko zibuhlungu zinokuhlala iiyure okanye iintsuku. Le ntlungu inokukhatshwa ziintlungu zomzimba, umkhuhlane, kunye nokudinwa kakhulu.
Intliziyo
Isifo sentliziyo sixhaphakile. Sinokubangela ukubetha kwentliziyo okungahambelaniyo, ukutyeba kwemisipha yentliziyo, okanye iingxaki ngeevalvu zentliziyo (ezifana nokungasebenzi kakuhle kwe-mitral). Ezi meko zinokunyusa umngcipheko weengxaki ezinkulu ezifana nestroke.
Ulusu
Uphawu oluphambili ngamabala abomvu amnyama (ii-angiokeratomas) esixoxe ngawo ngaphambili. Kumakhwenkwe, la mabala abonakala kakhulu ezandleni, emadolweni, ezingqinibeni, nakwindawo ephakathi kwezinqe namadolo. Abafazi abanakukwazi ukufumana la mabala.
Amehlo
Olu luphawu oluthile. Imigca emhlophe efana nomhlwa okanye iipateni ezifana nezangqa (ii-corneal whorls) zinokubonakala kumphezulu weliso kufutshane ne-cornea. Nangona ezi zingaphazamisi umbono, zizinto ezibalulekileyo kakhulu kugqirha ukuze afumanise isifo sikaFabry.
Olu hlobo lotshintsho lwamehlo alufane lubonwe kwezinye izifo, ngoko ke ukuba nalo phawu luncedo kakhulu ekuxilongeni isifo sikaFabry.
Iindlebe
Abanye abantu banokufumana ingxolo ezindlebeni, okanye "i-Tinnitus". Ukulahlekelwa kukuva nako kunokubakho kancinci kancinci ngokuhamba kwexesha okanye ngequbuliso.
Imiphunga kunye nenkqubo yokugaya ukutya
Iimpawu zokuphefumla ezifana nesandi 'sokukrwitsha' xa uphefumla, ukuphefumla okufutshane, kunye nokukhohlela rhoqo zinokubakho. Kwakhona, malunga nesiqingatha sabasetyhini bafumana iimpawu ezifana ne-Irritable Bowel Syndrome (IBS) (iintlungu zesisu, ukudumba).
Ngaba kukho iintlobo ezimbini zesifo? (Uhlobo 1 kunye nohlobo 2)
Ewe. Isifo sikaFabry sahlulwe kakhulu saba ziintlobo ezimbini.
1. Uhlobo oluqhelekileyo (Uhlobo 1): Oku kudla ngokuqala ebuntwaneni. Ukudumba kunye nentlungu emalungeni zisenokuba yenye yeempawu zokuqala ebuntwaneni.
2. Uhlobo lokuqalisa emva kwexesha (Uhlobo 2): Kolu hlobo, ubuntwana kunye nokufikisa ziqhelekile. Iimpawu ziqala ukubonakala emva kweminyaka engama-20 okanye ngaphezulu.
Nokuba loluphi na uhlobo, esi sifo sidla ngokuba mandundu xa sikhula. Isizathu esiphambili soku kukwanda kwamafutha emzimbeni ngokuhamba kwexesha.
Usenokucinga ukuba esi sisifo esingaqhelekanga kwaye akunakwenzeka ukuba senzeke kuwe. Kodwa ukuba wena okanye umntu kusapho lwakho ubeneempawu ezininzi ezikhankanyiweyo kweli nqaku ixesha elide, nceda ungasityesheli. Eyona nto ingcono onokuyenza kukubona ugqirha ngokukhawuleza kwaye uthethe ngaso.Uza kuqhuba iimvavanyo ezifunekayo aze afumanise ukuba yintoni kanye kanye ebangela ezi mpawu.
Umyalezo Wokuya Ekhaya
- Isifo sikaFabry sisifo esingaqhelekanga esibangelwa lufuzo esibangela ukuba amafutha aqokeleleke kwizitho zomzimba.
- Iimpawu eziphambili kukudumba kwezandla neenyawo, ukuba ndindisholo, amabala abomvu amnyama eluswini, ukuncipha kokubila, kunye nokudinwa kakhulu.
- Esi sifo singonakalisa kakhulu amalungu omzimba abalulekileyo afana nezintso, intliziyo, kunye nenkqubo yemithambo-luvo.
- Iimpawu ziqhelekile kwaye zinzima kakhulu kumadoda, ngelixa abafazi besenokuba neempawu ezincinci okanye bangabi nazo kwaphela.
- Ukuba wena okanye umntu kusapho lwakho unempawu enye okanye ezingaphezulu kwezi zikhankanyiweyo kweli nqaku kwaye zihlala ixesha elide, nceda udibane nogqirha wakho ngokukhawuleza ukuze akunike ingcebiso.
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