Kodi nthawi zina mumamva kupweteka kosapiririka ndi kutupa m'manja ndi m'mapazi anu, pamodzi ndi madontho ofiira ang'onoang'ono pathupi lanu? Mukakumana ndi zizindikiro zomwe sizikugwirizana, zingakhale vuto lalikulu. Lero tikulankhula za vuto lomwe lingayambitse zizindikiro zofanana, koma anthu ambiri m'dziko lathu sanamvepo za matendawo. Umenewo ndi matenda a Fabry.
Mwachidule, kodi matenda a Fabry ndi chiyani?
Matenda a Fabry ndi matenda a majini omwe amabadwa nawo kuchokera ku majini athu. Ndi osowa kwambiri. Munthu amene ali ndi matendawa amakhala ndi mafuta ambiri m'maselo a thupi lake. Tangoganizani zomwe zingachitike ngati wosonkhanitsa zinyalala sanabwere kunyumba kwathu kwa masiku angapo? Zinyalala zimasonkhana m'nyumba mwathu, sichoncho? Umu ndi momwe zimakhalira. Mu matendawa, enzyme yomwe imaphwanya ndikuchotsa mafuta awa m'maselo athu siipangidwa m'thupi, kapena siigwira ntchito bwino.
Mafuta akamasonkhana motere, mitsempha yathu yamagazi imayamba kuchepa. Izi zitha kuwononga khungu, impso, mtima, ubongo, ndi dongosolo lamanjenje . Madokotala amatchanso izi kuti "vuto losungira zinthu." Zizindikirozi nthawi zambiri zimayamba ali ana. Ndipo zimapezeka kwambiri mwa amuna kuposa akazi.
Musadandaule. Ngakhale kuti palibe mankhwala a matendawa, pali mankhwala abwino omwe alipo masiku ano omwe angakuthandizeni kuthana ndi zizindikiro zanu ndikukhala moyo wabwinobwino.
Kodi n’chiyani chimayambitsa matenda a Fabry?
Uwu ndi matenda obadwa nawo. Izi zikutanthauza kuti ndi chinthu chomwe mumalandira kuchokera kwa amayi kapena abambo anu. Tikufuna enzyme yotchedwa alpha-galactosidase A kuti iwononge zinthu monga mafuta, sera, ndi mafuta acids m'thupi lathu. Munthu amene ali ndi matenda a Fabry alibe enzyme iyi, kapena ngati ali nayo, sigwira ntchito bwino. Chifukwa chake, monga ndanenera kale, mafuta amtundu uwu amayamba kusonkhana mkati mwa maselo.
Kodi zizindikiro za matendawa ndi ziti?
Zizindikiro za matenda a Fabry zimatha kusiyana malinga ndi munthu. Anthu ena amakhudzidwa kwambiri ndi zizindikirozi, pomwe ena sakhudzidwa kwambiri. Tiyeni tiwone zizindikiro zazikulu zomwe zingawonekere.
| Chizindikiro | Kufotokozera |
|---|---|
| Ululu ndi kutupa m'miyendo | Ululuwu umawonjezeka panthawi yochita masewera olimbitsa thupi, mukakhala ndi malungo, mukakhala ndi kutentha kwambiri, kapena mukakhala wotopa. |
| Madontho a pakhungu | Madontho ang'onoang'ono, ofiira akuda kapena ofiirira omwe amapezeka kwambiri pakati pa matako ndi mawondo. |
| Zofooka za maso | Matenda monga kusawona bwino kapena diso lofooka. |
| Kuvutika kumva | Kutaya kumva kapena "kulira" kosalekeza m'makutu. |
| Kuchepa kwa thukuta | Thukuta lochepa kuposa la munthu wamba limatuluka thukuta. |
| Mavuto a m'mimba | Kupweteka m'mimba komanso kufunika kochita chimbudzi nthawi yomweyo mutadya. |
Mavuto aakulu
Matenda a Fabry angayambitse mavuto aakulu pakapita nthawi. Chiwopsezochi chimakhala chachikulu makamaka mwa amuna. Komabe, akazi omwe ali ndi jini ya matendawa (onyamula) nawonso amatha kukhala ndi zizindikiro. Chifukwa chake, ndikofunikira kwambiri kuti akazi adziwe za matendawa ndikupempha upangiri wa dokotala nthawi zonse.
- Kuwonjezeka kwa chiopsezo cha matenda a mtima kapena sitiroko .
- Kuwonongeka kwakukulu kwa impso ndi kulephera kwa impso.
- Kuthamanga kwa magazi kwambiri.
- Kulephera kwa mtima ndi kukula kwa mtima.
- Kuchepa kwa mafupa (Osteoporosis).
Kodi mungadziwe bwanji matendawa molondola?
Kunena zoona, nthawi zina zimatenga nthawi yayitali kuti munthu adziwe matenda a Fabry. Chifukwa chake n'chakuti zizindikiro zake zimafanana ndi za matenda ena ambiri. Chifukwa chake, anthu ena sadziwa kuti ali ndi matenda a Fabry mpaka patapita zaka zambiri zizindikirozo zitayamba. Panthawi imeneyo, mwina adakumana ndi madokotala osiyanasiyana chifukwa cha zizindikiro zosiyanasiyana, ndipo nthawi zina adalandira matenda olakwika.
Ngati wina m'banja mwanu wakhala ndi zizindikiro izi ndipo mukuganiza kuti muli pachiwopsezo, ndi bwino kulankhula ndi dokotala wanu za kuyezetsa majini .
Mukapita kukaonana ndi dokotala, adzakuyesani ndikufunsani mafunso monga awa:
- Mukupeza bwanji?
- Kodi zizindikiro zanu ndi ziti?
- Kodi izi zinayamba liti?
- Kodi pali aliyense m'banja mwanu amene ali ndi vuto lililonse mwa izi?
Kutengera ndi izi, ngati dokotala akukayikira kuti izi zitha kukhala matenda a Fabry, adzakufunsani kuti muyesedwe magazi (kuti muwone kuchuluka kwa enzyme ya alpha-galactosidase A yomwe ndatchulayi) kapena muyesedwe DNA .
Kodi chimachiritsidwa bwanji?
Pali njira ziwiri zazikulu zochizira matenda a Fabry.
1. Chithandizo Chosintha Ma Enzyme (ERT): Iyi ndi njira yomwe imagwiritsidwa ntchito kwambiri. Izi zimaphatikizapo kupatsa thupi enzyme yomwe ikusowa kapena yomwe sikugwira ntchito bwino kudzera mu saline solution. Izi nthawi zambiri zimachitika kuchipatala kapena kuchipatala pafupifupi kamodzi pa milungu iwiri iliyonse. Chithandizochi chimaletsa kusonkhanitsa mafuta m'thupi ndikuletsa ululu ndi zizindikiro zina.
2. Migalastat (Galafold): Iyi ndi mankhwala atsopano omwe angatengedwe ngati piritsi. Amagwira ntchito pokhazikitsa enzyme yomwe ikugwira ntchito bwino komanso kukonza ntchito yake.
Kuwonjezera pa mankhwala akuluakulu awa, dokotala wanu angakulangizeni mankhwala ena kutengera zizindikiro zanu.
- Mankhwala ochepetsa ululu chifukwa cha kuwonongeka kwa mitsempha.
- Mankhwala a matenda am'mimba.
- Mankhwala ochepetsa magazi kuti magazi asaundane.
- Mankhwala oteteza kuthamanga kwa magazi ndi impso.
Ngati matendawa awononga impso kwambiri, dialysis kapena ngakhale kuyika impso zina pa malo enaake kungakhale kofunikira.
Mayeso omwe amawunika matenda anu
Pa nthawi ya chithandizo, dokotala wanu nthawi zonse amachita mayeso osiyanasiyana kuti ayang'anire vuto lanu.
| Mayeso | Kufotokozera kosavuta |
|---|---|
| EKG (Electrocardiogram) | Kuyeza ntchito yamagetsi ya mtima, kuwona kugunda kwa mtima ndi kayimbidwe kake. |
| Echocardiogram | Kujambula mtima pogwiritsa ntchito ultrasound. Izi zimayesa thanzi ndi ntchito ya ziwalo za mtima. |
| MRI ya ubongo | Kupeza zithunzi zatsatanetsatane za ubongo ndi ziwalo zina. |
| Kujambula kwa CT | Kujambula zithunzi zamkati mwa thupi pogwiritsa ntchito X-ray. |
| Mayeso ena | Mayeso a magazi, mkodzo, chithokomiro, mayeso a kumva ndi kuona, mayeso a ntchito ya mapapo. |
Uthenga Wopita Nawo Kunyumba
- Matenda a Fabry ndi matenda obadwa nawo komanso obadwa nawo. Si opatsirana.
- Zizindikiro zambiri zosagwirizana zimatha kuchitika limodzi, monga kupweteka kwambiri m'miyendo, mawanga ofiira pakhungu, komanso kuchepa kwa thukuta.
- Zingatenge nthawi kuti matenda adziwike, koma mayeso a magazi ndi DNA angatsimikizire matendawa.
- Ngakhale kuti matendawa sangachiritsidwe kwathunthu, tsopano pali mankhwala othandiza (monga ERT) omwe angathe kuletsa zizindikiro, kupewa mavuto aakulu, komanso kuthandiza anthu kukhala ndi moyo wabwinobwino.
- Musamalumphe mankhwala ndi mayeso omwe dokotala wanu amakupatsani. Kutsatira malangizo a dokotala nthawi zonse ndikofunikira kwambiri pa thanzi lanu lamtsogolo.
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