Ni ibisanzwe kumva ufite ubwoba kandi ugatungurwa iyo muganga akubwira ko hari umuntu wo mu muryango wawe, cyane cyane umwana muto, ufite indwara ya Pompe. Ni indwara idakunze kugaragara, iterwa n'uturemangingo, bityo ni ibisanzwe guhangayika. Ariko reka nkubwire, ntugahangayike. Nubwo nta muti uhari kuri ubu, hari uburyo bwinshi bwiza bwo kuvura bushobora gufasha abarwayi kubaho igihe kirekire kandi nta ngorane nyinshi bafite.
Uburyo bw'ingenzi bwo kuvura indwara ya Pompe ni ERT (Enzyme Replacement Therapy).
Muri make, indwara ya Pompe iterwa no kubura enzyme ikenewe cyane ku turemangingo tw’imitsi mu mibiri yacu. Iyo iyi enzyme ibuze, ubwoko bw’isukari yitwa glycogen irundanya mu turemangingo, ikangiza imitsi. Tekereza nk’aho hari abakozi bake bakora mu mibiri yacu, kandi tuyita enzymes. Muri Pompe, iyi enzyme ntikora neza.
Rero, ubuvuzi bwo gusimbuza enzyme (ERT) niho dutanga enzyme ibura, cyangwa umukozi, iturutse hanze y'umubiri. Kuri ibi, umuti urimo enzyme y'ubukorano yitwa `alglucosidase alfa` uhabwa umubiri binyuze mu mitsi (IV infusion). Ubu buryo ni bwo buryo bwonyine bwemewe ku ndwara ya Pompe.
Ubu buryo bwo kuvura bwa ERT bwongereye cyane igihe cyo kubaho cy’abarwayi ba Pompe, cyane cyane impinja. Mu bihe byashize, byari gake cyane ko umwana urwaye iyi ndwara aramba arengeje umwaka.
Iyo abana badafite ERT, barwara indwara ya Pompe bagenda babyimba imitsi y'umutima, bagaca intege indi mitsi mu mubiri wabo, hanyuma bagapfa mu mwaka wa mbere w'ubuzima bwabo. Banatakaza imitsi ikenewe kugira ngo bahumeke. Nyuma y'uko ubuvuzi bwa ERT butangiye, ibi byahindutse burundu. Abana babanje kuvurwa na ERT ubu bari mu kigero cy'imyaka 20.
Kuki ari ngombwa cyane gutangira kuvurwa hakiri kare?
Iki ni cyo kintu cy'ingenzi cyane. Ubuvuzi bwa ERT bushobora guhagarika indwara gukomeza kwiyongera, ariko ntibushobora gusubiza inyuma ibyangiritse byari byarakorewe ku mitsi. Ibi bivuze ko iyo ubuvuzi butinze, ushobora gutakaza ubushobozi bwo guhumeka cyangwa kugenda.
Abaganga bagerageza gutangira imiti ya ERT mu minsi mike ya mbere y'ubuzima bwabo , cyane cyane ku bana bato, nyuma yo gusuzuma iyo ndwara. Ndetse no gutinda gato bishobora gutuma umwana atabasha kugenda burundu.
Ku bantu barwaye indwara ya Pompe itangira mu gihe cya nyuma, muganga azasuzuma buri gihe ibimenyetso kugira ngo amenye igihe cyiza cyo gutangira ERT.
Uru rugendo si urwe wenyine - inkunga y'itsinda ry'impuguke
Nubwo ubuvuzi bwa ERT bushobora kurokora ubuzima, ntabwo buhagije. Kugira ngo umuntu urwaye indwara ya Pompe agire ubuzima bwiza, hakenewe itsinda ry'inzobere mu nzego zitandukanye. Inzobere mu by'imiterere y'umubiri (genetic scientific) akenshi ni we muntu w'ibanze ushinzwe gucunga ubuvuzi.
Reba imbonerahamwe iri hepfo ku bwoko bw'inzobere zishobora gukenerwa kuri ibi n'ubwoko bw'ubufasha bazatanga.
| Umuganga/umuvuzi w'inzobere | Ni ubuhe bufasha babona? |
|---|---|
| Inzobere mu by'umutima | Ingaruka ku mutima zirakurikiranwa kandi hagatangwa ubuvuzi bukenewe. |
| Inzobere mu by'imitsi | Bazakurikirana ibibazo byo guhumeka no gutanga imashini zifasha guhumeka nibiba ngombwa. |
| Inzobere mu ndwara zo mu gifu | Ivura ibibazo byo kumira no kugogora. |
| Inzobere mu by'imirire | Tanga intungamubiri zikenewe kugira ngo ukure neza, kandi nibiba ngombwa, indyo yihariye cyangwa kugaburira hakoreshejwe umuyoboro wo kugaburira ni byiza. |
| Umuganga w'umubiri | Imyitozo ngororamubiri n'ubuvuzi bitangwa kugira ngo bikomeze imitsi kandi bigabanye intege nke. |
| Umuvuzi w'akazi | Kubafasha gukora imirimo ya buri munsi (nk'iyambarwa, kurya, n'ibindi) ku giti cyabo. Iyo bibaye ngombwa, bigishwa gukoresha ibikoresho bibafasha nk'inkoni. |
| Umuvuzi w'indwara zo kuvuga | Bifasha mu guhangana n'ingorane zo kuvuga ziterwa n'intege nke z'imitsi yo mu maso n'ururimi. |
Ubundi buryo bwo kuvura usibye ERT
Imiti igira ingaruka ku budahangarwa bw'umubiri
Imibiri y'abana bamwe ibona ko ubu buryo bwo kuvura imisemburo yo hanze (ERT) ari 'ubw'abanyamahanga' maze igatangira gukora ubudahangarwa bwo kuyirwanya. Ibi byitwa ``CRIM-negative``. Ibi nibibaho, uburyo bwo kuvura ERT ntibuzakora neza.
Kugira ngo hirindwe ibi, abaganga batangira kuvura indwara zo kwihanganisha ubudahangarwa bw'umubiri (ITI) . Muri ibyo, hamwe n'ubuvuzi bwa ERT, hatangwa imiti myinshi igenzura ubudahangarwa bw'umubiri.
- Rituximab
- Methotrexate
- Indwara y'ubudahangarwa bw'umubiri iterwa mu mitsi (IVIG)
Icyo iyi miti ikora ni "gushuka" ubudahangarwa bw'umubiri bugatuma umuntu yumva ko ERT ari ikintu cy'umubiri. Hanyuma umubiri ntuzarwanya ERT.
Ubuvuzi bw'umubiri n'ubuvuzi bw'akazi
Kubera ko intege nke z'imitsi ari kimwe mu bintu by'ingenzi biranga indwara ya Pompe, ubuvuzi bw'umubiri ni ingenzi. Bushobora gufasha gukomeza imitsi no gukomeza imikorere y'umubiri. Ubuvuzi bw'akazi bushobora kandi gufasha guteza imbere ubumenyi bukenewe kugira ngo umuntu akore imirimo ya buri munsi ku giti cye.
Uburyo bwo kuvura imivugire n'imirire
Kubera ko imitsi ikenewe kugira ngo umuntu ahekenya kandi amire ibiryo ishobora kuba idakomeye, umuganga w’imivugire ashobora kumufasha mu myitozo yo kumira. Nanone, hari igihe umuntu ashobora gukenera umuyoboro wo kugaburira umwana kugira ngo agire intungamubiri zikenewe. Inzobere mu by’imirire ishobora gutanga ubuyobozi bukenewe kuri ibi.
Uruhare rwawe narwo ni ingenzi cyane.
Mu gihe ubana n'indwara ya Pompe, uruhare rwawe nk'umurwayi cyangwa umubyeyi ni ingenzi cyane.
Ryan Colburn, ufite iyi ndwara, agira ati: "Ikintu cy'ingenzi nakoze ni ukwiga ku ndwara ya Pompe. Yampaye icyizere n'ihumure mu kubaho na yo buri munsi."
Kubwibyo, ugomba gukorana na muganga wawe kugira ngo wumve ibyo ukeneye kandi ufate iya mbere mu kubona ubuvuzi bwiza. Ntukigere utinya kubaza muganga wawe ikibazo icyo ari cyo cyose ufite kuri iyi ndwara cyangwa uburyo bwo kuyivura.
Ubutumwa bwo kujyana mu rugo
- Nubwo indwara ya Pompe idashobora gukira burundu, uburyo bwo kuvura bwa none bushobora gufasha abarwayi kuramba no kubaho neza.
- Uburyo bw'ingenzi bwo kuvura ni uburyo bwo gusimbuza imisemburo (ERT). Ubu ni uburyo bwo kuvura burokora ubuzima.
- Ni ngombwa cyane gutangira kuvurwa vuba bishoboka, kuko kwangirika kw'imitsi bidashobora gusubizwa inyuma.
- Uretse ERT, inkunga ituruka ku nzobere zitandukanye nko kuvura umubiri, imirire, no kuvura indwara zo kuvuga ni ngombwa.
- Nk'umurwayi cyangwa umubyeyi, ushobora kugera ku musaruro mwiza umenye neza iby'iyi ndwara no gukorana bya hafi na muganga wawe.
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