Kodi mudamvapo za matenda a Gaucher? Mwina ayi. Chifukwa ndi matenda osowa, si matenda ofala m'dziko lathu. Koma popeza ndi matenda a majini omwe amapatsirana kuchokera ku mibadwomibadwo, ndikofunikira kwambiri kuwadziwa. Matendawa angayambitse zizindikiro zosiyanasiyana, kuyambira mafupa ofooka mpaka kufiira ngakhale ndi bala laling'ono.
Mwachidule, kodi matenda a Gaucher ndi chiyani?
Chabwino, tiyeni tinene motere. Pali enzyme yapadera m'thupi lathu yomwe imathandiza kuswa ndi kuchotsa mitundu ina ya mafuta. M'thupi la munthu amene ali ndi matenda a Gaucher, enzyme iyi sigwira ntchito bwino. Kenako chomwe chimachitika ndichakuti mitundu imeneyo ya mafuta imasungidwa m'malo osiyanasiyana a thupi, makamaka m'chiwindi, ndulu, ndi m'mafupa . Mafuta akamasonkhana motere, mavuto osiyanasiyana azaumoyo amayamba.
Pakadali pano palibe mankhwala a matendawa, koma kutengera mtundu wa matenda a Gaucher omwe muli nawo, pali mankhwala abwino kwambiri oletsa zizindikiro.
Pali mitundu itatu ikuluikulu ya matenda a Gaucher:
1. Mtundu 1: Uwu ndi mtundu wofala kwambiri. Siumakhudza mitsempha. Zizindikiro zake zimakhala zochepa kwambiri kwa anthu ena, koma zimatha kukhala zoopsa kwambiri kwa ena. Nthawi zina sizingakhale ndi zizindikiro konse. Pali mankhwala abwino a mtundu uwu.
2. Mtundu wachiwiri ndi mtundu wachitatu: Mitundu yonseyi ndi yoopsa kwambiri kuposa mtundu woyamba chifukwa imakhudza dongosolo la mitsempha, lomwe ndi ubongo ndi msana. Makanda omwe ali ndi mtundu wachiwiri nthawi zambiri sakhala ndi moyo wopitirira zaka ziwiri. Mtundu wachitatu umawononganso ubongo, koma zizindikiro zimawonekera pang'ono ali ana.
Kodi n’chiyani chimayambitsa matenda a Gaucher?
Iyi si matenda omwe angapatsiridwe kuchokera kwa munthu kupita kwa munthu ngati chimfine. Iyi ndi matenda a majini omwe amapatsiridwa kuchokera ku mibadwomibadwo. Amayamba chifukwa cha vuto la jini lotchedwa GBA.
Tangoganizirani, mwana angatenge matendawa pokhapokha ngati atengera jini lolakwika la GBA kuchokera kwa amayi ndi abambo ake. Ngakhale wina atakhala kuti alibe matendawa, akhoza kukhala ndi jini lolakwikali m'thupi mwake (kukhala wonyamulira) ndikulipereka kwa ana ake.
Matendawa amapezeka kwambiri pakati pa anthu ochokera ku fuko la Ayuda (Ashkenazi Jews) ku Eastern ndi Central Europe.
Kodi zizindikiro za matendawa ndi ziti?
Zizindikiro zimatha kusiyana kwambiri kuchokera kwa munthu ndi munthu. Zimasiyananso kutengera mtundu wa matenda. Tiyeni tiwone zizindikiro zokhudzana ndi mtundu uliwonse.
| Mtundu wa Matenda | Zizindikiro Zodziwika |
|---|---|
| Mtundu 1 |
|
| Mtundu Wachiwiri (Kwa makanda apakati pa miyezi 3-6) | |
| Mtundu 3 (Kuyambira ali mwana) | |
| Matenda a mtima (Mtundu wa 3c) (Mtundu wosowa) |
Kodi mungazindikire bwanji matendawa?
Mukapita kwa dokotala yemwe ali ndi zizindikiro izi, angakufunseni mafunso monga:
- Kodi munayamba liti kuzindikira zizindikiro zake?
- Kodi banja lanu lachokera kuti?
- Kodi pali aliyense m'mibadwo yakale ya banjali amene wakhala ndi mavuto a thanzi ngati awa?
- Kodi pali wachibale kapena banja lanu lomwe lakhala ndi ana osakwana zaka ziwiri omwe anamwalira?
Ngati dokotala wanu akukayikira kuti muli ndi matenda a Gaucher, akhoza kutsimikizira izi poyesa magazi kapena malovu . Adzafunikanso kuyesedwa pafupipafupi kuti awone momwe matendawa akuyendera.
Kuphatikiza apo, MRI scan ingachitike kuti ione ngati chiwindi kapena ndulu zatupa, ndipo kuyezetsa kuchuluka kwa mafupa kungachitike kuti ione ngati mafupawo ali ndi kuchulukana.
Kodi mankhwala a matenda a Gaucher ndi ati?
Njira zochiritsira zimadalira mtundu wa matenda ndi kuopsa kwa zizindikiro. Ngati zizindikirozo ndi zochepa kwambiri, palibe chithandizo chomwe chingafunike.
Njira zazikulu zochizira
- Chithandizo Chosintha Ma Enzyme (ERT): Ichi ndi chithandizo chachikulu cha anthu ena omwe ali ndi matenda a shuga amtundu woyamba ndi wachitatu. Izi zimaphatikizapo kupatsa thupi enzyme yomwe ilibe mphamvu. Mankhwalawa amaperekedwa kudzera m'mitsempha (IV) kamodzi pa milungu iwiri iliyonse. Amathandiza kuchepetsa kuchepa kwa magazi m'thupi, kulimbitsa mafupa, ndikuchiritsa ndulu ndi chiwindi chokulirapo. Komabe, chithandizochi sichigwira ntchito bwino pamavuto a mitsempha omwe amakhudza ubongo. `Imiglucerase (Cerezyme)` ndi `Velaglucerase alfa (VPRIV)` ndi mankhwala amtunduwu.
- Mankhwala Ochepetsa Matenda a Substrate (SRT): Awa ndi mapiritsi omwe amawongolera kupanga mafuta omwe amaunjikana m'thupi la matenda a Gaucher. Eliglustat (Cerdelga) ndi Miglustat (Zavesca) ndi mankhwala otere. Amaperekedwa kwa odwala akuluakulu a mtundu woyamba omwe sali oyenera kulandira chithandizo cha ERT.
Chofunika kwambiri n’chakuti, palibe mankhwala omwe angaletse kuwonongeka kwa ubongo komwe kumachitika chifukwa cha matenda a shuga amtundu wachitatu, koma ofufuza akupitilizabe kugwira ntchito pa izi.
Mankhwala ena othandizira
- Kuthira magazi chifukwa cha kuchepa kwa magazi m'thupi
- Mankhwala olimbitsa mafupa ndi kuchepetsa ululu
- Opaleshoni yosinthira mafupa kuti ithandize kuyenda bwino
- Opaleshoni yochotsa ndulu yokulira
- Mankhwala oletsa ululu
- Kumwa zakudya zina monga vitamini D ndi calcium ngati dokotala akulangizani
Kukhala ndi matendawa ndi zomwe mungayembekezere
Popeza matendawa ndi osiyana kwa munthu aliyense, muyenera kugwira ntchito limodzi ndi dokotala wanu kuti mupeze njira yochiritsira yoyenera kwa inu. Chithandizo chingakuthandizeni kukhala ndi moyo wabwino ndikuwonjezera moyo wanu.
Mwana amene ali ndi matenda a Gaucher angakule pang'onopang'ono kuposa ana ena. Akhozanso kuchedwa kutha msinkhu. Kutengera ndi zizindikiro zake, mungafunike kupewa kuchita zinthu monga masewera olimbitsa thupi. Anthu ena angafunike kuyesetsa kwambiri kuti akhalebe ndi thupi chifukwa cha ululu waukulu komanso kutopa.
Kukhala ndi vuto ngati ili ndi vuto lalikulu, choncho ndikofunikira kwambiri kufunafuna thandizo kuchokera kwa achibale ndi abwenzi, komanso upangiri wamaganizo ngati pakufunika kutero.
Uthenga Wopita Nawo Kunyumba
- Matenda a Gaucher ndi matenda a majini omwe amapatsirana m'mibadwo yambiri, osati matenda omwe amapatsirana kuchokera kwa munthu wina kupita kwa wina.
- Zizindikiro zimatha kusiyana kwambiri kutengera mtundu wa matenda komanso munthu payekha. Kwa anthu ena, zizindikiro zake zimakhala zochepa kwambiri.
- Ndikofunikira kwambiri kuzindikira matendawa ndikuyamba kulandira chithandizo msanga.
- Ngakhale kuti pali mankhwala opambana kwambiri (ERT ndi SRT) a mtundu woyamba ndi zina mwa zizindikiro za mtundu wachitatu, matendawa sangachiritsidwe kwathunthu.
- Ngati inu kapena mwana wanu muli ndi matendawa, ndikofunikira kutsatira ndondomeko yoyenera ya chithandizo mwa kugwira ntchito limodzi ndi dokotala wanu.
- Kupeza chithandizo kuchokera ku mabanja ndi magulu othandizira kumathandiza kwambiri kuti munthu akhale ndi mphamvu m'maganizo.
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