Isifo sikaWilson

Umxholo

• Iimpawu kunye neempawu zesifo sikaWilson
• Inxulumene nesibindi
• Neurological
• Amakhonkco e-Kayser-Fleischer kunye ne-sunflower cataract
• Ezinye iimpawu
• Yintoni unobangela kwaye ngubani osemngciphekweni wesifo sikaWilson?
• Sichongwa njani isifo sikaWilson?
• Uvavanyo lomzimba
• Uvavanyo lweLebhu
• Sinyangwa njani isifo sikaWilson?
• Inqanaba lokuqala
• Inqanaba lesibini
• Inqanaba lesithathu
• Yintoni imbonakalo yesifo sikaWilson?
• Ngaba ungasithintela isifo sikaWilson?
• Amanyathelo alandelayo

Siyintoni isifo sikaWilson?

Isifo sikaWilson, esikwabizwa ngokuba kukuwohloka kwe-hepatolenticular kunye ne-lenticular degeneration, sisifo esinqabileyo semfuza esibangela ityhefu yobhedu emzimbeni. Ichaphazela malunga ne-1 kubantu abangama-30,000 kwihlabathi liphela.

Emzimbeni ophilileyo, isibindi sihluza ubhedu olugqithisileyo kwaye siyikhuphe ngomchamo. Ngesifo sikaWilson, isibindi asikwazi ukususa ubhedu olongezelelweyo ngokufanelekileyo. Ubhedu olongezelelweyo emva koko luyakha kumalungu anje ngengqondo, isibindi namehlo.

Ukuxilongwa kwangoko kubalulekile ekunqandeni ukuqhubela phambili kwesifo sikaWilson. Unyango lunokubandakanya ukuthatha amayeza okanye ukufakelwa isibindi. Ukulibaziseka okanye ukungalufumani unyango kunokubangela ukusilela kwesibindi, ukonakala kwengqondo, okanye ezinye iimeko ezisongela ubomi.

Thetha nogqirha wakho ukuba usapho lwakho lunembali yesifo sikaWilson. Abantu abaninzi abanale meko baphila ubomi obuqhelekileyo, obunempilo.

Iimpawu kunye neempawu zesifo sikaWilson

Iimpawu kunye neempawu zesifo sikaWilson ziyahluka ngokubanzi, kuxhomekeke kwiliphi ilungu elichaphazelekayo. Banokuphazama ngenxa yezinye izifo okanye iimeko. Isifo sikaWilson sinokufunyanwa kuphela ngugqirha kwaye kuvavanyo lokuqonda isifo.

Inxulumene nesibindi

Iimpawu ezilandelayo zingabonisa ukuqokelelwa kobhedu esibindini:

• ubuthathaka
• ndiziva ndidiniwe
• ukuhla ukusinda
• isicaphucaphu
• ukugabha
• Ukuphelelwa ngumdla wokutya
• ukurhawuzelela
• i-jaundice, okanye umbala otyheli kolusu
• ukudumba, okanye ukudumba kwemilenze nesisu
• intlungu okanye ukudumba esiswini
• spider angiomas, okanye isebe lemithambo yegazi ebonakalayo eluswini
• izihlunu zemithambo

Uninzi lwezi mpawu, njenge-jaundice kunye ne-edema, ziyafana kwezinye iimeko ezinje ngokusilela kwesibindi kunye nezintso. Ugqirha wakho uya kuqhuba iimvavanyo ezininzi ngaphambi kokuqinisekisa ukuxilongwa kwesifo sikaWilson.

Neurological

Ukuqokelelwa kobhedu kwingqondo kunokubangela iimpawu ezinje:

• inkumbulo, intetho, okanye ukungaboni kakuhle
• ukuhamba ngendlela engaqhelekanga
• migraines
• ukuvuza amathe
• ukuphuthelwa
• ukungazinzi ngezandla
• utshintsho kubuntu
• utshintsho kwiimvakalelo
• ukudakumba
• iingxaki esikolweni

Kwinqanaba eliphambili, ezi zimpawu zinokubandakanya ukuphazamiseka kwemisipha, ukuxhuzula, kunye nentlungu yomsipha ngexesha lokuhamba.

Amakhonkco e-Kayser-Fleischer kunye ne-sunflower cataract

Ugqirha wakho uya kujonga iiringi zeKayser-Fleischer (KF) kunye ne-sunflower cataract emehlweni. Amakhonkco e-KF angaqhelekanga ngombala wegolide -imdaka emehlweni abangelwa ziidipozithi zobhedu olugqithisileyo. Amakhonkco e-KF abonakala malunga neepesenti ezingama-97 zabantu abanesifo sikaWilson.

I-Sunflower cataract ibonakala kubantu aba-1 kwaba-5 abanesifo sikaWilson. Eli liziko elinemibala eyahlukeneyo enezithethi eziphuma ngaphandle.

Ezinye iimpawu

Ukwakhiwa kobhedu kwamanye amalungu kunokubangela:

• ukutshintsha umbala oluhlaza kwizikhonkwane
• amatye ezintso
• ukukhubazeka kwamathambo ngaphambi kwexesha, okanye ukungabikho kwethambo
• isifo samathambo
• izitenxo zokuya exesheni
• uxinzelelo lwegazi oluphantsi

Yintoni unobangela kwaye ngubani osemngciphekweni wesifo sikaWilson?

Utshintsho kwi I-ATP7B yemfuza, ethi ikhowudi yokuhambisa ubhedu, ibangela isifo sikaWilson. Kuya kufuneka uzuze ilifa kubazali bobabini ukuze ube nesifo sikaWilson. Oku kunokuthetha ukuba omnye wabazali bakho unale meko okanye uphethe ufuzo.

Imfuza inokweqa isizukulwana, ke unokufuna ukujonga ngakumbi kunabazali bakho okanye uthathe uvavanyo lwemfuzo.

Sichongwa njani isifo sikaWilson?

Isifo sikaWilson sinokuba nzima koogqirha ekuchongeni okokuqala. Iimpawu ziyafana neminye imiba yezempilo enje ngetyhefu enzima yentsimbi, i-hepatitis C, kunye ne-cerebral palsy.

Ngamanye amaxesha ugqirha wakho uya kuba nakho ukulawula isifo sikaWilson emva kokuba iimpawu ze-neurological zivela kwaye akukho ring ye-KF ibonakala.Kodwa oku akusoloko kunjalo kubantu abaneempawu ezithile zesibindi okanye abanazo ezinye iimpawu.

Ugqirha uya kubuza malunga neempawu zakho kwaye abuze nembali yosapho lwakho. Baza kuphinda basebenzise iimvavanyo ezahlukeneyo ukukhangela umonakalo obangelwe kukuqokelela kobhedu.

Uvavanyo lomzimba

Ngexesha lomzimba wakho, ugqirha wakho uya:

• vavanya umzimba wakho
• mamela izandi esiswini
• jonga amehlo akho phantsi kokukhanya okukhanyayo kumakhonkco e-KF okanye kwi-cataract sunflower
• kuvavanya iimoto zakho kunye nezakhono zememori

Uvavanyo lweLebhu

Uvavanyo lwegazi, ugqirha wakho uza kuzobeka iisampulu kwaye zibahlolisise elebhu ukujonga:

• Ukungaqhelekanga kwi-enzymes yakho yesibindi
• amanqanaba obhedu egazini
• amanqanaba asezantsi e-ceruloplasmin, iprotein ethwala ubhedu egazini
• ijini eguqulweyo, ekwabizwa ngokuba kuvavanyo lwemfuzo
• iswekile esezantsi yegazi

Ugqirha wakho unokukucela ukuba uqokelele umchamo wakho iiyure ezingama-24 ukukhangela ingqokelela yobhedu.

Sinyangwa njani isifo sikaWilson?

Unyango oluyimpumelelo lwesifo sikaWilson luxhomekeke kwixesha elingaphezulu kweyeza. Unyango kaninzi lwenzeka ngokwamanqanaba amathathu kwaye kufuneka luhlale ubomi bonke. Ukuba umntu uyeka ukuthatha amayeza, ubhedu bunokuphinda lubuye.

Inqanaba lokuqala

Unyango lokuqala kukususa ubhedu oluninzi emzimbeni wakho ngokukhohlisa unyango. Iiarhente ze-Chelating zibandakanya iziyobisi ezinje nge-d-penicillamine kunye ne-trientine, okanye iSyprine. La machiza aya kususa ubhedu olongezelelekileyo kumalungu akho aze awukhuphele kwigazi. Izintso zakho ziya kucoca ubhedu kumchamo.

I-Trientine ineempembelelo ezimbalwa ezichaziweyo kune-d-penicillamine. Iziphumo ebezingalindelekanga ezinokubakho d-penicillamine zibandakanya:

• ifiva
• irhashalala
• Imiba yezintso
• Imiba yomongo wethambo

Ugqirha wakho uya kubonelela ngeedosi ezisezantsi zokukhohlisa iziyobisi ukuba ukhulelwe, njengoko zinokubangela iziphene zokuzalwa.

Inqanaba lesibini

Injongo yesigaba sesibini kukugcina amanqanaba esiqhelo obhedu emva kokususwa. Ugqirha wakho uya kukumisela i-zinc okanye i-tetrathiomolybdate ukuba ulugqibile unyango lokuqala okanye ungabonisi zimpawu kodwa unesifo sikaWilson.

I-Zinc ethathwe ngomlomo njengeetyuwa okanye i-acetate (iGalzin) igcina umzimba ungafumani ubhedu ekutyeni. Unokuba nesisu esincinci esibuhlungu ekuthatheni i-zinc. Abantwana abanesifo sikaWilson kodwa akukho zimpawu zinokufuna ukuthatha i-zinc ukuthintela imeko ukuba ibe mandundu okanye icothe inkqubela phambili.

Inqanaba lesithathu

Emva kokuba iimpawu ziphuculwe kwaye amanqanaba akho obhedu aqhelekileyo, uya kufuna ukugxila kunyango lokugcina ixesha elide. Oku kubandakanya ukuqhubeka kwe-zinc okanye ukunyanga unyango kwaye ujonge rhoqo amanqanaba obhedu.

Unokulawula amanqanaba akho obhedu ngokuphepha ukutya okunamanqanaba aphezulu, anje ngala:

• iziqhamo ezomisiweyo
• isibindi
• amakhowa
• amandongomane
• iqokobhe
• itshokholethi
• iivithamini

Unokufuna ukujonga amanqanaba akho asekhaya ekhaya, nawo. Kunokubakho ubhedu olongezelelekileyo emanzini akho ukuba ikhaya lakho linemibhobho yobhedu.

Amayeza angathatha naphina ukusuka kwiinyanga ezine ukuya kwezintandathu ukusebenza kumntu ohlangabezana neempawu. Ukuba umntu akaphenduli kolu nyango, banokufuna ukufakelwa isibindi. Ukufakelwa kwesibindi ngempumelelo kunokuphilisa isifo sikaWilson. Inqanaba lokuphumelela kokufakwa kwesibindi ngama-85 epesenti emva konyaka omnye.

Yintoni imbonakalo yesifo sikaWilson?

Kwangoko ufumanisa ukuba unayo imfuza yesifo sikaWilson, ngcono isifo sakho sisengqondweni. Isifo sikaWilson sinokukhula sibe sisibindi kunye nomonakalo wobuchopho ukuba asinyangwa.

Unyango lwakwangoko lunokunceda ukuguqula imiba ye-neurological kunye nokonakala kwesibindi. Unyango kwinqanaba elizayo linokuthintela inkqubela phambili yesi sifo, kodwa ayisayi kuhlala ibuyisela umonakalo. Abantu abakumanqanaba aphambili banokufunda indlela yokulawula iimpawu zabo ebomini babo.

Ngaba ungasithintela isifo sikaWilson?

Isifo sikaWilson sisifo esivela kubazali siye kubantwana babo. Ukuba abazali banomntwana onesifo sikaWilson, banokuba nabanye abantwana abanesi sifo.

Nangona ungeke ukwazi ukukhusela isifo sikaWilson, unokulibazisa okanye unciphise ukuqala kwimeko. Ukuba ufumanisa ukuba unesifo sikaWilson kwangethuba, unokukwazi ukuthintela iimpawu ekuboniseni ngokuthatha amayeza afana ne-zinc. Ingcali yemfuza inokunceda abazali ukuba babeke emngciphekweni wokudlulisela isifo sikaWilson ebantwaneni babo.

Amanyathelo alandelayo

Yenza idinga nogqirha wakho ukuba wena okanye umntu omaziyo unokuba nesifo sikaWilson okanye ubonakalisa iimpawu zokungaphumeleli kwesibindi. Esona salathisi sikhulu kule meko yimbali yosapho, kodwa uhlobo oluguqukileyo lunokweqa isizukulwana. Unokufuna ukubuza uvavanyo lwemfuza kunye nolunye uvavanyo ugqirha wakho aya kulucwangcisa.

Uya kufuna ukuqala unyango lwakho ngokukhawuleza ukuba ufumana isifo kwi-Wilson's disease. Unyango lwakwangoko lunokunceda ukuthintela okanye ukulibazisa imeko, ngakumbi ukuba awukabonisi zimpawu okwangoku. Amayeza afaka iiarhente zokukopa kunye ne-zinc kwaye kungathatha ukuya kwiinyanga ezintandathu ukusebenza. Nangona amanqanaba akho obhedu ebuyela esiqhelweni, kuya kufuneka uqhubeke nokusela amayeza, njengoko isifo sikaWilson sisimo sobomi bonke.