Ndizachibadwa kuchita mantha komanso kudabwa dokotala akakuuzani kuti winawake m'banja mwanu, makamaka mwana wamng'ono, ali ndi matenda a Pompe. Ndi matenda osowa, omwe amabadwa mwachibadwa, kotero ndi zachibadwa kuda nkhawa. Koma ndikuuzeni, musadandaule. Ngakhale pakadali pano palibe mankhwala a matendawa, pali mankhwala ambiri othandiza omwe angathandize odwala kukhala ndi moyo wautali komanso wopanda mavuto ambiri.
Chithandizo chachikulu cha matenda a Pompe ndi ERT (Enzyme Replacement Therapy).
Mwachidule, matenda a Pompe amayamba chifukwa cha kusowa kwa enzyme yomwe ndi yofunika kwambiri m'maselo a minofu m'thupi lathu. Pamene enzyme iyi ikusowa, mtundu wa shuga wotchedwa glycogen umasonkhana mkati mwa maselo, kuwononga minofu. Taganizirani ngati pali antchito ang'onoang'ono omwe amagwira ntchito m'thupi lathu, ndipo timawatcha ma enzyme. Mu matenda a Pompe, wantchito uyu sagwira ntchito bwino.
Chifukwa chake, Enzyme Replacement Therapy (ERT) ndi komwe timapereka enzyme yosowa, kapena wogwira ntchito, kuchokera kunja kwa thupi. Pachifukwa ichi, mankhwala okhala ndi enzyme yopangidwa yotchedwa `alglucosidase alfa` amaperekedwa ku thupi kudzera mu mtsempha (IV infusion). Chithandizo ichi ndi chokhacho chomwe chavomerezedwa pakadali pano pa matenda a Pompe.
Chithandizo cha ERT ichi chawonjezera kwambiri nthawi ya moyo wa odwala a Pompe, makamaka makanda. Kale, sizinali zachilendo kuti mwana amene ali ndi matendawa apulumuke kupitirira chaka chimodzi.
Popanda chithandizo cha ERT, makanda omwe ali ndi matenda a Pompe pang'onopang'ono amalimbitsa minofu ya mtima wawo, kufooketsa minofu ina m'thupi lawo, ndikumwalira mkati mwa chaka choyamba cha moyo wawo. Amatayanso minofu yomwe imafunika kuti apume. Chithandizo cha ERT chitayamba, vutoli linasintha kwathunthu. Makanda omwe adalandira chithandizo cha ERT koyamba tsopano ali ndi zaka 20.
N’chifukwa chiyani n’kofunika kwambiri kuyamba kulandira chithandizo msanga?
Ichi ndiye chinthu chofunikira kwambiri. Chithandizo cha ERT chingaletse matendawa kuti asakule kwambiri, koma sichingabwezeretse kuwonongeka komwe kwachitika kale ku minofu. Izi zikutanthauza kuti ngati chithandizo chachedwa, mutha kutaya mphamvu yopuma kapena kuyenda.
Madokotala amayesa kuyamba chithandizo cha ERT m'masiku oyamba a moyo , makamaka kwa makanda, atazindikira vutoli. Ngakhale kuchedwa pang'ono kungalepheretse mwana kuyenda kwamuyaya.
Kwa anthu omwe ali ndi matenda a Pompe omwe amayamba mochedwa, dokotala nthawi zonse amafufuza ndikuwunika zizindikiro kuti adziwe nthawi yabwino yoyambira ERT.
Ulendo uwu si ulendo wongoyenda wekha - thandizo lochokera ku gulu la akatswiri
Ngakhale chithandizo cha ERT chingapulumutse moyo, sichikwanira. Kuti munthu amene ali ndi matenda a Pompe akhale ndi moyo wabwino kwambiri, pakufunika gulu la akatswiri osiyanasiyana. Katswiri wa majini nthawi zambiri ndiye amene amayang'anira chithandizo.
Onani tebulo ili m'munsimu kuti mudziwe mitundu ya akatswiri omwe angafunikire pa izi komanso mtundu wa chithandizo chomwe angapereke.
| Dokotala/katswiri wothandiza | Kodi amalandira thandizo lotani? |
|---|---|
| Katswiri wa mtima | Zotsatira zake pamtima zimayang'aniridwa ndipo chithandizo chofunikira chimaperekedwa. |
| Katswiri wa matenda a m'mapapo | Adzayang'anira mavuto opuma ndikupereka ma ventilator ngati pakufunika kutero. |
| Katswiri wa matenda a m'mimba | Amachiza mavuto omeza ndi kugaya chakudya. |
| Katswiri wa zakudya | Perekani zakudya zofunikira kuti zikule bwino, ndipo ngati pakufunika, zakudya zapadera kapena kudyetsa kudzera mu chubu chodyetsera zimalimbikitsidwa. |
| Katswiri Wothandiza Anthu Odwala Matenda a Thupi | Maseŵero olimbitsa thupi ndi mankhwala amaperekedwa kuti alimbikitse minofu ndikuchepetsa kufooka. |
| Katswiri wa Zachipatala | Kuwathandiza kuchita ntchito za tsiku ndi tsiku (monga kuvala, kudya, ndi zina zotero) paokha. Ngati pakufunika kutero, amaphunzitsidwa kugwiritsa ntchito zipangizo zothandizira monga ndodo. |
| Katswiri Wolankhula | Zimathandiza kuthetsa mavuto olankhula omwe amayamba chifukwa cha kufooka kwa minofu ya nkhope ndi lilime. |
Mankhwala ena kupatula ERT
Mankhwala omwe amakhudza chitetezo cha mthupi
Matupi ena a makanda amazindikira kuti chithandizo cha enzyme choperekedwa kunja (ERT) ichi ndi 'chachilendo' ndipo amayamba kupanga ma antibodies motsutsana nacho. Izi zimatchedwa vuto la `CRIM-negative`. Ngati izi zitachitika, chithandizo cha ERT sichigwira ntchito bwino.
Pofuna kupewa izi, madokotala amayamba chithandizo cha ITI (immuno tolerance induction induction) . Pachifukwa ichi, pamodzi ndi chithandizo cha ERT, mankhwala angapo amaperekedwa omwe amalamulira chitetezo cha mthupi.
- Rituximab
- Methotrexate
- Immunoglobulin ya m'mitsempha (IVIG)
Zimene mankhwala amenewa amachita ndi "kunyenga" chitetezo cha mthupi kuti chiganize kuti ERT ndi chinthu cha thupi. Kenako thupi silidzalimbana ndi ERT.
Kuchiza thupi ndi chithandizo cha ntchito
Popeza kufooka kwa minofu ndi chizindikiro chachikulu cha matenda a Pompe, chithandizo chamankhwala ndi chofunikira. Chingathandize kulimbitsa minofu ndikusunga magwiridwe antchito a thupi. Chithandizo chamankhwala chantchito chingathandizenso kukulitsa luso lofunikira kuti munthu agwire ntchito za tsiku ndi tsiku payekha.
Kulankhula ndi kuchiza zakudya
Popeza minofu yofunika kutafuna ndi kumeza chakudya ingakhale yofooka, katswiri wolankhula angathandize pochita masewera olimbitsa thupi pomeza. Komanso, nthawi zina pangafunike chubu chodyetsera mwana kuti apeze zakudya zofunikira. Katswiri wa zakudya angapereke malangizo ofunikira pa izi.
Udindo wanu nawonso ndi wofunika kwambiri.
Mukakhala ndi matenda a Pompe, chothandizira chanu monga wodwala kapena kholo n'chofunika kwambiri.
"Chinthu chofunika kwambiri chomwe ndinachita chinali kuphunzira za matenda a Pompe. Anandipatsa chidaliro chachikulu komanso chitonthozo pokhala nawo tsiku lililonse," akutero Ryan Colburn, yemwe ali ndi matendawa.
Choncho, muyenera kugwira ntchito ndi dokotala wanu kuti mumvetse zosowa zanu ndikutsogolera pakupeza chithandizo chabwino kwambiri. Musamaope kufunsa dokotala wanu mafunso aliwonse omwe muli nawo okhudza matendawa kapena chithandizochi.
Uthenga Wopita Nawo Kunyumba
- Ngakhale kuti matenda a Pompe sangachiritsidwe kwathunthu, mankhwala a masiku ano angathandize odwala kukhala ndi moyo wautali komanso wabwino.
- Chithandizo chachikulu ndi enzyme replacement therapy (ERT). Ichi ndi chithandizo chopulumutsa moyo.
- Ndikofunikira kwambiri kuyamba chithandizo mwamsanga, chifukwa kuwonongeka kwa minofu sikungatheke.
- Kuwonjezera pa ERT, thandizo la akatswiri osiyanasiyana, monga chithandizo cha thupi, zakudya, ndi chithandizo cha kulankhula, n'kofunika kwambiri.
- Monga wodwala kapena kholo, mutha kupeza zotsatira zabwino kwambiri mwa kudziwa bwino za matendawa komanso kugwira ntchito limodzi ndi dokotala wanu.
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