Ngaba kukho umntu kusapho lwakho, mhlawumbi umntwana wakho, onesifo esibizwa ngokuba yiNeurofibromatosis Type 1 (NF1)? Usenokuba ukhathazekile nge-neurofibromas (PN) ebuhlungu, enzima ukuyisusa, ehambelana nale meko. Kodwa namhlanje sithetha ngeyeza elitsha, elithembisayo elivunyiweyo ngoku ukuba lisetyenziswe kubantu abadala nabantwana ukunyanga esi sifo. Libizwa ngokuba yiGomekli (mirdametinib).
Yintoni iGomekli? Isebenza njani?
Cinga nje, imizimba yethu ineendlela ezahlukeneyo zokubonisa iimpawu ukuze iiseli zikhule. Kwi-NF1, enye yezi ndlela zokubonisa iimpawu isebenza kakhulu, nto leyo ekhokelela ekwakhekeni kweethumba ezijikeleze imithambo-luvo. Ezi thumba ngamanye amaxesha zikhula zijikeleze iindawo ezibalulekileyo ezifana nemithambo-luvo, imithambo yegazi, kunye namalungu angaphakathi, nto leyo eyenza kube nzima kakhulu ukuzisusa ngotyando, kwaye ngamanye amaxesha akunakwenzeka.
Yile nto eyenziwa yiGomekli. Ithintela ii-enzymes ezimbini emzimbeni wethu ezibizwa ngokuba yi-MEK1 kunye ne-MEK2. Ezi enzymes zezona zithumela imiqondiso yokuba kufuneka ii-tumor zikhule. Ngoko ke xa iGomekli imisa loo ndlela yesignali, ii-tumor ziyancipha, kwaye ngamanye amaxesha ii-tumor ziyancipha kwaye ziqale ukuncipha . Ngamafutshane, olu lolunye unyango olufanelekileyo kubantu abangakwaziyo utyando.
Ulisebenzisa njani eli yeza?
Ugqirha wakho uza kumisela idosi efanelekileyo yeGomekli kuwe okanye kumntwana wakho, ngokusekelwe kubude nobunzima bakho .
Olu nyango ludla ngokwenziwa kumjikelo weentsuku ezingama-28.
- Ufanele uthabathe eli yeza kabini ngemini, rhoqo emva kweeyure ezili-12 .
- Kufuneka usele amayeza ngale ndlela iintsuku ezingama-21 zilandelelana .
- Emva koko kufuneka uthabathe iyeza kangangeentsuku ezisi-7 .
- Xa iintsuku ezingama-28 zigqityiwe, umjikelo omtsha uqala kwakhona.
IGomekli iza ngeekhaphu. Nangona kunjalo, kwabo banengxaki yokuginya iikhaphu, kukwakho nepilisi enencasa yeediliya enokunyibilikiswa emanzini ize ithathwe. Nantsi indlela yokwenza:
1. Faka inani leepilisi ezichazwe ngugqirha kwikomityi yokulinganisa amayeza.
2. Yongeza amanzi angama-5 okanye ali-10 milliliters kuyo.
3. Jikelezisa indebe kangangemizuzu emibini ukuya kwemine de iipilisi zinyibilike ngokupheleleyo. Nje ukuba zinyibilike, ulwelo luya kuba mhlophe luze lubonakale ngathi lubisi kancinci.
4. Sela ngoko nangoko kwikomityi. Okanye ungayifunxa ngesirinji yomlomo .
5. Emva kokuba ugqibile ukusela, yongeza enye i-5-10 ml yamanzi kwikomityi enye, uxube, uze usele nayo. Oku kuya kunceda iyeza eliseleyo ukuba lingene emzimbeni.
6. Ukuba awuyiseli kwimizuzu engama-30 emva kokuyenza, lahla ulwelo oluseleyo.
Zithini izifundo malunga nempumelelo yeziyobisi zeGomekli?
Ukusebenza kakuhle nokhuseleko lwale chiza kuqinisekisiwe kwisifundo sezonyango esibizwa ngokuba yiReNeu study. Sasibandakanya abantwana nabantu abadala abali-111 abaneminyaka emi-2 nangaphezulu abaneethumba ze-PN ezingasebenziyo.
Iziphumo ziyakhuthaza ngokwenene.
| Isiphumo | Abantu abadala | Abantwana |
|---|---|---|
| Ukuncipha kobukhulu bethumba ubuncinane nge-20%. | 41% | 52% |
| Iziphumo zithathe iinyanga ezili-12 okanye ngaphezulu kubantu abangaphezu kwama-88% abaphendulileyo. | 88% | 90% |
Eyona nto ibalulekileyo kukuba ezi ziphumo ziqale ukubonakala kwiinyanga ezi-4 emva kokuqala unyango. Ngokuqhelekileyo kuthatha malunga neenyanga ezi-7-8 ukubona iziphumo zokuqala.
Kodwa khumbula, iziphumo zezi zifundo azifani kuye wonke umntu . Iziphumo zinokwahluka ngokuxhomekeke kwimeko yakho.
Uyazi njani ukuba iyeza liyasebenza?
Eyona ndlela iphambili yokwazi oku kukusebenzisa ii-MRI scans eziya ku-odolwa ngugqirha wakho. Ezi zinokujonga ukuba iithumba ziyancipha na ngobukhulu.
Ngaphezu koko, uza kuqala ukuqaphela umahluko ngokwakho.
- Ukunciphisa iintlungu: Iintlungu ezibangelwa sisisu ziyancipha kakhulu.
- Ukukwazi ukuhambahamba ngcono: Ukuba ubunobunzima bokuhambahamba ngenxa yeethumba, ezi thumba ziya kuncipha kwaye kuya kuba lula ukwenza imisebenzi yemihla ngemihla.
- Ukuphucula umgangatho wobomi:Lilonke, ndiqala ukuziva ndingcono kunangaphambili.
Kolu phando, iziphumo ezilungileyo, ezinje ngokunciphisa iintlungu, zibonwe nasemva kweenyanga ezi-3 zonyango.
Zeziphi iziphumo ebezingalindelekanga eziqhelekileyo?
Njengayo nayiphi na iyeza, iGomekli inokubangela ezinye iziphumo ebezingalindelekanga. Nangona kunjalo, uninzi lwazo lunokulawulwa.
| Iziphumo ebezingalindelekanga eziqhelekileyo | Izinto ezilula zokulawula |
|---|---|
| Ukurhawuzelela kwesikhumba, isicaphucaphu/ukuhlanza, urhudo, iintlungu zemisipha kunye namalungu, ukudinwa. |
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| Kubantwana, iintlungu zesisu, ukubomvu/ukudumba kolusu olujikeleze iinzipho, kunye neengxaki zentliziyo nazo zinokubakho. | Xela ugqirha wakho naziphi na ezi mpawu ngokukhawuleza , ingakumbi ukuba uneengxaki zentliziyo. |
Eyona nto ibalulekileyo: Ukuba ufumana naziphi na iziphumo ebezingalindelekanga, nokuba zincinci, qiniseka ukuba uxelele ugqirha wakho . Anganciphisa umthamo, ayeke ukuthatha amayeza okwethutyana, okanye akunike elinye iyeza lokulawula iziphumo ebezingalindelekanga.
Ezinye izinto ezikhethekileyo ekufuneka uzazi malunga nale yeza
Kukho izinto ezininzi ekufuneka uziqwalasele ngaphambi kokuba uqale iGomekli nangexesha lonyango.
1. Amehlo: Eli yeza linokuthi lichaphazele amehlo ngamanye amaxesha. Ke ngoko, ngaphambi kokuba uqale unyango,Kufuneka uhlolwe amehlo ngokupheleleyo. Kufuneka uhlolwe namehlo akho njengoko ugqirha wakho ekuyalele ngexesha lonyango. Ukuba ufumana naziphi na iimpawu ezifana nokungaboni kakuhle, zixele ngoko nangoko.
2. Ukusebenza kwentliziyo: Kwenziwa i -echocardiogram ngaphambi kokuba kuqaliswe iyeza, elilinganisa ukusebenza kwentliziyo. Oku kwenziwa rhoqo emva kweenyanga ezi-3 kunyaka wokuqala wonyango.
3. Ukukhulelwa: Eli yeza linokuba yingozi kumntwana ongekazalwa .
- Ukuba ungumfazi onokukwazi ukuzala, uza kunikwa uvavanyo lokukhulelwa ngaphambi kokuba uqale unyango. Kufuneka usebenzise uhlobo lokulawula ukukhulelwa olusebenza kakuhle ngexesha lonyango kwaye kangangeeveki ezi-6 emva kokuyeka amayeza.
- Ukuba uyindoda, kufuneka usebenzise ucwangciso-nzala olusebenzayo ngexesha lonyango kwaye kangangeenyanga ezi-3 emva kokuyeka amayeza. Thetha nogqirha wakho ngale nto ngokukhululekileyo.
Umyalezo Wokuya Ekhaya
- IGomekli (mirdametinib) lunyango olutsha oluvunyiweyo ukuba lusetyenziswe kubantu abadala nakubantwana abaneethumba ezingasebenziyo (PN) ezinxulumene nesifo iNeurofibromatosis Type 1 (NF1).
- Oku kuthintela iimpawu ezifunekayo ekukhuleni kwethumba, kunceda ithumba linciphe kwaye linciphise iintlungu.
- Kubaluleke kakhulu ukuba uhlolwe rhoqo amehlo, intliziyo, kunye ne-MRI phantsi kweliso likagqirha wakho ngexesha lonyango.
- Iziphumo ebezingalindelekanga zinokubakho, kodwa uninzi lwazo ziyalawuleka. Xelela ugqirha wakho ngoko nangoko malunga nokungonwabi.
- Akufuneki uqale, uyeke, okanye utshintshe umthamo wamayeza ngaphandle kwengcebiso kagqirha.
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