Ingaba wena okanye umntwana wakho une-MPS I? Masifunde ngesitofu sokugonya iLaronidase!

Ngamanye amaxesha sinezifo esingenakuzicinga, akunjalo? Esinye sezimo ezinjalo yiMucopolysaccharidosis I, esiyibiza ngokuba yiMPS I ngamafutshane. Esi sisifo esingaqhelekanga. Ukuba wena okanye umntwana wakho unale meko, ndiyaqonda ukuba inokuba nzima kwaye ixinezeleke kangakanani. Kodwa ungakhathazeki, kukho unyango olunokunceda ukulawula ezi mpawu. Namhlanje siza kuthetha ngelinye iyeza elibaluleke kangaka, eliyigonyo elibizwa ngokuba yiLaronidase. Makhe sibone ukuba lenza ntoni kwaye lisetyenziswa njani.

Luhlobo luni lweyeza iLaronidase?

Ngamafutshane, iLaronidase liyeza elibuyisela i -enzyme ekhethekileyo engekhoyo okanye engaphelelanga emzimbeni wethu. Khawuthelekelele, ezi enzymes zinceda yonke into emzimbeni wethu ukuba isebenze kakuhle. Njengabasebenzi abancinci. Ngoku, emzimbeni wabantu abane-MPS I, i-enzyme ethile ayikho okanye ayisebenzi kakuhle. Eli yeza linikwa ukuzalisa loo ntsilelo. Oku kunceda ukulawula ezinye zeempawu zesifo. Kodwa into enye ekufuneka uyikhumbule kukuba eli asiloyeza eliphilisa ngokupheleleyo isifo. Kodwa linceda kakhulu ngokunciphisa iimpawu nokwenza ubomi bube lula kancinci.

Ngamanye amaxesha ungalibona eli yeza phantsi kwegama lophawu i-Aldurazyme. Eli yeza lingasetyenziselwa nezinye izinto. Ukuba unemibuzo ngalo, buza ugqirha wakho okanye ikhemisti .

Zeziphi izinto ekufuneka uzixelele iqela lakho lezonyango ngaphambi kokuba uqalise eli yeza?

Ngaphambi kokuba uqale olu nyango lweLaronidase, ngokuqinisekileyo kufuneka uxelele iqela lakho lezonyango, oko kukuthi, ugqirha kunye nomongikazi, ngezi zinto. Kuba kubaluleke kakhulu ukuba bazazi ezi zinto ukuze bakuncede ngokufanelekileyo.

• Sixelele ukuba wakha waba ne -allergy kule yeza, naluphi na olunye unyango, ukutya, iidayi, okanye izithinteli.
• Ukuba ukhulelwe okanye unethemba lokukhulelwa, baxelele.
• Ukuba ungumama oncancisayo, kufuneka ukhankanye loo nto.

Ukuba ubaxelela ezi zinto ngaphandle kokuzifihla, baya kukwazi ukukuphatha ngendlela ekufaneleyo. Akunjalo?

Ulifumana njani eli yeza?

I-Laronidase inikwa njenge- infusion emthanjeni. Eli asiloyeza onokulithatha ekhaya wedwa.Inikwa yingcali yezonyango eqeqeshiweyo esibhedlele okanye ekliniki. Oko kuthetha ukuba ugqirha okanye umongikazi uza kukunika yona. Ngoko ke akukho nto yokoyika, bayakwazi ukuyenza kakuhle.

Ngaba eli yeza linokunikwa abantwana abancinci?

Ewe, eli yeza linokunikwa iintsana ezineminyaka eyi-6 ubudala kwezinye iimeko ezikhethiweyo. Nangona kunjalo, kukho izinto ekufuneka uzilumkele xa ulinika abantwana abancinci. Ke ngoko, ukuba unika umntwana wakho eli yeza, kungcono ukulixoxa kakuhle nogqirha wezingane zakho uze ufumane zonke iinkcukacha.

Kuza kwenzeka ntoni ukuba uthatha amayeza amaninzi kakhulu?

Ekubeni eli yeza linikwa phantsi kweliso likagqirha, amathuba okuba lisetyenziswe ngokugqithisileyo aphantsi kakhulu. Nangona kunjalo, kwiimeko ezingaqhelekanga apho ucinga ukuba ulisele kakhulu iyeza, okanye ukuba uziva into engaqhelekanga, kufuneka utsalele umnxeba kwiziko lolawulo lwetyhefu okanye kwigumbi longxamiseko ngokukhawuleza. Nangona kunjalo, ndiyaphinda, amathuba okuba oku kwenzeke aphantsi, kuba oku kunikwa phantsi kweliso likagqirha.

Kubalulekile: Eli yeza lelakho wedwa. Ungaze wabelane ngalo nabanye.

Yintoni omawuyenze ukuba uphoswe yidosi yeyeza?

Kubaluleke kakhulu ukulithatha eli yeza ngexesha. Ukuba awukwazi ukuya esibhedlele okanye ekliniki ngosuku okanye ngexesha elicwangcisiweyo, okanye ukuba uphoswe yidosi, fowunela ugqirha wakho okanye ingcali yezempilo ngokukhawuleza. Baza kukuxelela into omawuyenze ngokulandelayo.

Ngaba eli yeza lingadibana namanye amayeza? (Iindlela elisebenzisana ngazo)

Ngokubanzi, eli yeza, iLaronidase, akulindelekanga ukuba libangele ukudibana okukhulu namanye amayeza. Oko kukuthi, alisabela kangako kwamanye amayeza.

Nangona kunjalo, oku akuthethi ukuba akuyi kubakho ngxaki. Ngoko ke, kungcono ukuxelela ugqirha wakho ngawo onke amayeza owathathayo, kungekuphela nje lawo anikwe ngugqirha wakho, kodwa nalawo uwathengayo kwikhemesti (amayeza angengomyalelo kagqirha), amayeza esintu (imifuno), iivithamini ( izongezo zokutya). Kwakhona, xelela ugqirha wakho ukuba uyatshaya, usela utywala, okanye usebenzisa ezinye iziyobisi. Ezinye izinto zinokusebenzisana namayeza akho ngandlela thile. Olu luhlu aluchazi yonke intsebenziswano enokwenzeka.

Zeziphi izinto ekufuneka uzilumkele ngakumbi xa usebenzisa eli yeza?

Xa unikwa eli yeza, iLaronidase, imeko yakho iya kuhlolwa ngononophelo ngoogqirha bakho.Oko kuthetha ukuba bahlala bejonge kuwe.

Kubalulekile nokubona ugqirha wakho rhoqo ukuze ubone ukuba iimpawu zakho ziya zisiba ngcono okanye zimbi. Ukuba iimpawu zakho aziphucuki okanye zibonakala zisiba mandundu, qiniseka ukuba uxelele ugqirha wakho okanye ingcali yezempilo.

"Enye into, ukuze uyiqonde ngcono i-MPS I kunye nonyango lwayo, kukho irejista ekhethekileyo eqokelela ulwazi lwesigulana. Thetha nogqirha wakho malunga nokufaka igama lakho koku. Kuya kuba luncedo olukhulu kuphando lwexesha elizayo ngesi sifo."

Zithini iziphumo ebezingalindelekanga ezinokubakho zale yeza?

Njengayo nayiphi na iyeza, olu gonyo lweLaronidase lunokubangela iziphumo ebezingalindelekanga kwabanye abantu. Kodwa ayinguye wonke umntu ozifumanayo. Kukho ezinye iziphumo ebezingalindelekanga ekufuneka uxelele ugqirha wakho ngoko nangoko ukuba uyazibona. Kukho ezinye ezihlala zingeyongozi kangako. Masibone ukuba zeziphi.

Iziphumo ebezingalindelekanga ekufuneka uzixele kugqirha wakho ngoko nangoko:

"Ukuba ufumana naziphi na kwezi mpawu, kufuneka uxelele ugqirha wakho okanye ingcali yezempilo ngokukhawuleza kangangoko kunokwenzeka:"

• Iimpendulo ze-allergy : Ezi ziquka ukurhawuzelela kwesikhumba, ukurhawuzelela okanye ukurhawuzelela, kunye nokudumba kobuso, imilebe, okanye ulwimi.
• Iingxaki zokuphefumla .
• Utshintsho kwimbono .
• Iintlungu zesifuba .
• Ukukhwehlela .
• Ifiva .
• Ukudumba kweenyawo okanye izandla .
• Ubuso obubomvu okanye ukurhawuzelela .
• Uxinzelelo lwegazi oluphantsi .
• Intlungu, ukurhawuzelela, ukuba ndindisholo ezandleni okanye ezinyaweni .
• Ububomvu, iintlungu, okanye ukudumba kwindawo apho kufakwe khona inaliti .
• Ukutyheli kwamehlo okanye ulusu - Oku kungaba yingxaki yesibindi.

Iziphumo ebezingalindelekanga ezingadingi ngcebiso kagqirha, kodwa kufuneka zixelwe kugqirha ukuba ziyaqhubeka:

"Ezi zinto ngamanye amaxesha zinokwenzeka emva kokufumana isitofu. Uninzi lwexesha, azibalulekanga kangako, kodwa ukuba ziyaqhubeka, okanye ukuba ziyakukhathaza, licebo elihle ukuxelela ugqirha wakho ."

• Ukutsha okuncinci okanye ubuhlungu kwindawo apho kufakwe khona inaliti .

Olu luhlu aluzizo zonke iziphumo ebezingalindelekanga ezinokubakho. Ukuba ubona nantoni na engabonakaliyo, thetha nogqirha wakho. Ungaxela ezi ziphumo ebezingalindelekanga kwi-FDA (Food and Drug Administration) ku-1-800-FDA-1088.

Uligcina phi eli yeza?

Lo asingombandela ubaluleke kakhulu, kuba eli yeza aliyonto onokuya nayo ekhaya. Linikwa esibhedlele okanye eklinikhi kuphela. Ngoko ke akufuneki ukhathazeke ngokugcina eli yeza likhuselekile, abasebenzi bezonyango baza kuyenza loo nto.

Okokugqibela, eyona nto ibalulekileyo

Kulungile, ngoko ke ndiyathemba ukuba ngoku uyiqonda ngcono i-Laronidase vaccine esithethe ngayo. Khumbula, eli liyeza elinikwa phantsi kweliso logqirha okhethekileyo ukunceda ukulawula iimpawu zesifo esingaqhelekanga esibizwa ngokuba yi-MPS I.

"Ukuba wena okanye umntwana wakho ufumana olu nyango, landela imiyalelo kagqirha kanye njengoko ekuxelela. Ukuba unemibuzo okanye amathandabuzo, musa ukuwagcina kuwe. Akho ukuze akuncede. Nangona eli yeza lingayi kunyanga isifo ngokupheleleyo, liya kwenza ubomi bube lula kancinci kwaye likunike amandla okulwa neempawu. Ungaze ulahle ithemba! "

Le ncwadana sisishwankathelo kuphela. Isenokungabi nalo lonke ulwazi. Ukuba unemibuzo engaphezulu malunga nale yeza, nceda uthethe nogqirha wakho, usokhemesti, okanye umboneleli wezempilo.

I- Laronidase, i-MPS I, i-Mucopolysaccharidosis I, unyango lwe-enzyme, i-Aldurazyme, izifo ezingaqhelekanga