Masifunde ngesitofu sokugonya i-Idursulfase esinikwa abo baneHunter Syndrome.

Ngaba wena okanye umntu omaziyo unesifo esingaqhelekanga esibizwa ngokuba yiHunter Syndrome? Ukuba kunjalo, mhlawumbi ukhe weva ngesitofu esibizwa ngokuba yi-Idursulfase. Ligama elintsonkothileyo, kodwa namhlanje siza kuthetha ngeli yeza ngendlela elula kakhulu, ngendlela onokuyiqonda. Kuba kubaluleke kakhulu ukuba nolwazi olupheleleyo ngezinto ezinje.

Lithini eli yeza libizwa ngokuba yi-Idursulfase?

Ngamafutshane, iHunter Syndrome yimeko apho i-"enzyme" ekhethekileyo, iproteni enceda kwiinkqubo zeekhemikhali emzimbeni wethu, ingekho okanye ingasebenzi kakuhle. Le "enzyme" inceda ukuqhekeza ezinye iimolekyuli ezintsonkothileyo emzimbeni wethu kwaye zibe lula, oko kuthetha ukuba zinokususwa emzimbeni xa umsebenzi wazo ugqityiwe. Ngoko ke xa le "enzyme" ingekho, ezo molekyuli zintsonkothileyo ziqokelelana kwiindawo ezahlukeneyo zomzimba, zibangela iingxaki kunye neempawu ezahlukeneyo.

I-IDursulfase liyeza elenziwa elabhoratri ukuze lithathe indawo ye-enzyme engekhoyo, kwaye liyasebenza. Oko kukuthi, lithatha indawo ye-enzyme engekhoyo, okanye endaweni yoko, lunyango lokutshintsha i-enzyme. Kodwa into enye ekufuneka uyikhumbule kakuhle kukuba olu asilonyango olupheleleyo . Kodwa lunokunceda ukulawula iimpawu, lulawule ukuba imeko imbi, kwaye lwenze ubomi bube lula kancinci.

Eli yeza ngamanye amaxesha lingasetyenziselwa ezinye izinto, ngoko ke ukuba unemibuzo, buza ugqirha wakho okanye ikhemisti.

Igama eliqhelekileyo lemveliso yale yeza yi-`(Elaprase)`.

Yintoni omele uyixelele ugqirha wakho ngaphambi kokuba usebenzise eli yeza?

Ngoku, ngaphambi kokuba uqale ukusebenzisa la mayeza, kukho izinto ezimbalwa ekufuneka uzixelele ugqirha wakho okanye iqela lakho lokhathalelo. Oku kwenzelwa ukuba bakunike unyango olufanelekileyo ngokukhuselekileyo. Cinga ngako njengokwazi kwangaphambili ukuba zeziphi iingxaki oza kudibana nazo endleleni.

Nazi izinto ekufuneka uzaziswe:

• Ngaba okwangoku unesifo kunye nomkhuhlane?
• Ngaba unesifo sentliziyo? Oko kuthetha uhlobo oluthile lwengxaki yentliziyo.
• Ngaba unesifo semiphunga? Njengeengxaki zokuphefumla.
• Ngaba wakha waba ne-allergy kwi-Idursulfase, elinye iyeza, ukutya, iidayi zokutya, okanye izithinteli? Oku kungabandakanya amaqhuqhuva, ukurhawuzelela, okanye ubunzima bokuphefumla.
• Ngaba wakha wadumba ulwimi, ubuso, okanye imilebe, wanengxaki yokuphefumla, wanengxaki yokuginya, waphelelwa lilizwi, okanye waqina umqala?Ngaba oko kwakha kwenzeka? Oku kubaluleke kakhulu, kuba izinto ezinjalo zinokwenzeka nokuba usela eli yeza.
• Ukhulelwe okanye unethemba lokukhulelwa?
• Ngaba uyancancisa?

Kubaluleke kakhulu kukhuseleko lwakho ukuthetha ngezi zinto ngaphandle kokuzifihla.

Ulisebenzisa njani eli yeza?

Eli yeza linikwa njengenaliti emthanjeni . Ngokuchanekileyo, "liyi-infusion" ye-saline, enikwa emzimbeni kangangeeyure ezininzi. Le ayisiyonto unokuyenza ekhaya . Kufuneka uyinikwe yingcali yezempilo eqeqeshiweyo, njengogqirha okanye umongikazi, esibhedlele okanye eklinikhi.

Ukuba unika abantwana eli yeza:

Eli yeza ngamanye amaxesha linokunikwa iintsana ezineminyaka eli-16 ubudala, kunye nabantwana abaneminyaka eli-1 neenyanga ezi-4 ubudala, kwiimeko ezithile. Nangona kunjalo, kukho amanyathelo akhethekileyo okuzikhusela. Ke ngoko, ukuba unika umntwana wakho oku, kubalulekile ukuthetha nogqirha wakho wabantwana ngononophelo kwaye ufumane iinkcukacha.

Ukugqithisa:

Ukuba ucinga ukuba usele kakhulu eli yeza, okanye ukuba ukrokrela ukuba ulisele, kufuneka ufowunele iziko lokulawula ityhefu ngokukhawuleza okanye igumbi likaxakeka lesibhedlele elikufutshane. Kubaluleke kakhulu ukwenza ngokukhawuleza.

Inqaku elibalulekileyo: Eli yeza limiselwe wena wedwa. Musa ukunika omnye umntu eli yeza nangasiphi na isizathu , nokuba baneempawu ezifanayo nezakho.

Yintoni omawuyenze ukuba uphoswe yidosi yeyeza?

Akuyongcinga ilungileyo ukutsiba idosi yale yeza. Eli liyeza ekufuneka lithathwe rhoqo nangamaxesha athile. Ukuba uliphosa, iziphumo zonyango zinokuncipha. Ukuba awukwazi ukuza uthabathe iyeza ngosuku olucwangcisiweyo, qiniseka ukuba uthetha nogqirha wakho okanye ingcali yezempilo . Baza kukuxelela into omawuyenze ngokulandelayo.

Ngaba ezinye izinto zingasebenzisana nale yeza?

Eli yeza alisoloko libonakala libangela iingxaki ezinkulu kwamanye amayeza (akulindelekanga ukuba kubekho ukusebenzisana). Oko kukuthi, alisoloko lisabela kwamanye amayeza.

Nangona kunjalo, kungcono ukuxelela ugqirha wakho ngayo yonke imithi oyithathayo , kuquka amayeza kagqirha, amayeza angagunyaziswanga ngugqirha, imifuno, kunye nezongezo zokutya. Kwakhona, xelela ugqirha wakho ukuba uyatshaya, usela utywala, okanye usebenzisa ezinye iziyobisi ezingekho mthethweni.Ukuba uyavuma, baxelele ngayo loo nto, kuba ezinye izinto zinokusebenzisana namayeza akho ngandlela thile okanye zonyuse iziphumo ebezingalindelekanga.

Yintoni endifanele ndiyilumkele xa ndisebenzisa eli yeza?

Ngelixa usela eli yeza, kubaluleke kakhulu ukubona ugqirha wakho rhoqo ukuze ahlolwe . Ukuba iimpawu zakho aziphucuki okanye zibonakala zisiba mandundu, xelela ugqirha wakho. Musa ukungayinaki nantoni na.

Kukwakho nerejista yezigulana ezineHunter Syndrome. Oku kubavumela ukuba baqokelele ulwazi kuwe ixesha elide, oko kuthetha ukuba banokufunda ngakumbi ngesi sifo. Ungabuza ingcali yakho yezempilo ngolwazi oluthe kratya malunga noku. Ungajoyina ukuba unomdla.

Zithini iziphumo ebezingalindelekanga ezinokubakho zale yeza?

Njengayo nayiphi na iyeza, isitofu sokugonya i-Idursulfase sinokubangela ezinye iziphumo ebezingalindelekanga. Ezinye zazo zinzima kwaye kufuneka zixelwe kugqirha wakho ngoko nangoko. Ezinye zazo ziqhelekile kwaye ziya kuphela kwiintsuku ezimbalwa.

Iziphumo ebezingalindelekanga ekufuneka uzixelele ugqirha wakho ngoko nangoko:

Ukuba ufumana naziphi na kwezi mpawu , kufuneka uxelele ugqirha wakho okanye ingcali yezempilo ngokukhawuleza. Ezi zisenokuba ziimpawu zesifo esinzulu.

• Iimpendulo ze-allergy, eziquka ukurhawuzelela kwesikhumba, ukurhawuzelela okanye amaqhuqhuva, ukudumba kobuso, imilebe, okanye ulwimi. Oku kunokwenzeka ngexesha okanye emva nje kokuba kunikwe isitofu.
• Iingxaki zokuphefumla. Uzive ngathi uyafuthaniseka, kufuneka uphefumle ngokukhawuleza.
• Intlungu yesifuba okanye ukuxinana.
• Ukudinwa; ukuziva utyhafile okanye unentloko ebuhlungu; ukuwa ngenxa yokulahlekelwa yibhalansi; ukuziva ubuthathaka okanye udiniwe ngendlela engaqhelekanga.
• Ukubetha kwentliziyo okukhawulezayo nokungaqhelekanga.
• Umkhuhlane okanye ukubanda. Ezi zinokuba luphawu losulelo.

Iziphumo ebezingalindelekanga ezingezizo ezinzulu, kodwa ekufuneka zixelwe kugqirha wakho ukuba ziyaqhubeka:

Ezi zinto zihlala ziphela emva kweentsuku ezimbalwa. Kodwa ukuba ziyaqhubeka, okanye ukuba ziyakukhathaza, xelela ugqirha wakho.

• Ukukhwehlela.
• Urhudo.
• Ukuhlanjwa kobuso. Oku kuthetha ukuba ubuso buba bomvu kwaye bube shushu.
• Intloko ebuhlungu.
• Intlungu yezihlunu okanye yamalungu.
• Isicaphucaphu, ukuhlanza.
• Ukudinwa.

Olu luhlu lusenokungabandakanyi zonke iziphumo ebezingalindelekanga. Ukuba ubona nantoni na engaqhelekanga, fowunela ugqirha wakho ukuze akunike ingcebiso yezonyango. Ungazixela ezi ziphumo ebezingalindelekanga kwi-FDA (Food and Drug Administration) e-United States ku-1-800-FDA-1088. ESri Lanka, ungazixela kwiziphathamandla zempilo ezifanelekileyo ngogqirha wakho.

Uligcina phi eli yeza?

Lo ngumbuzo obuzwa ngabantu abaninzi. Akuvumelekanga ukugcina eli yeza le-Idursulfase ekhaya. Lisetyenziswa kwaye ligcinwe esibhedlele okanye eklinikhi kuphela. Isizathu soku yindlela elinikwa ngayo kunye neemeko zobushushu ezikhethekileyo ekufuneka ligcinwe kuzo.

Okokugqibela, inqaku elibalulekileyo

Ingaba esi sisishwankathelo nje sento esithethe ngayo? Ayigubungeli zonke iinkcukacha. Imeko yomntu wonke yahlukile. Ngoko ke, ukuba unemibuzo okanye iinkxalabo malunga nale yeza, qiniseka ukuba ubuza ugqirha wakho, ikhemisti, okanye omnye umboneleli wezempilo. Banokukunika ulwazi oluchanekileyo ngemeko yakho.

Izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, nazi izinto ekufuneka uzikhumbule koko sithethe ngako:

• I-Idursulfase liyeza elisindisa ubomi elithatha indawo ye-enzyme engekhoyo kubantu abaneHunter Syndrome.
• Olu asilonyango olupheleleyo , kodwa lunceda ukulawula iimpawu kunye nokuphucula umgangatho wobomi.
• Ngaphambi kokuba uqalise amayeza , kubalulekile ukuxelela ugqirha wakho ngazo zonke iimeko zakho zempilo , ii-allergies, kunye namanye amayeza owasebenzisayo.
• Oku kunikwa njengokufakwa emthanjeni esibhedlele ngumntu oqeqeshiweyo.
• Musa ukulibazisa idosi ethile yeyeza. Ukuba awukwazi, xelela ugqirha wakho ngoko nangoko.
• Kunokubakho iziphumo ebezingalindelekanga ezinzulu kunye neziphumo ebezingalindelekanga eziqhelekileyo. Xelela ugqirha wakho ngayo nayiphi na into engaqhelekanga, nokuba incinci kangakanani na.
• Eli yeza lelakho wedwa. Musa ukulinika omnye umntu.
• Musa ukoyika ukubuza ugqirha wakho nayiphi na imibuzo okanye iinkxalabo onokuba nazo. Unelungelo lokwazi ngempilo yakho.

I- Idursulfase, iHunter Syndrome, unyango lokutshintshwa kwe-enzyme, i-Elaprase, Iziphumo ebezingalindelekanga, Amayeza, Izifo ezingaqhelekanga