Ese umwana wawe aba ari munini gato kurusha abandi bana iyo avutse? Iyo ibyo bibaye, ushobora kuba wishimye, ariko ushobora no kugira amatsiko, wenda ndetse ugahangayika, ukibaza uti "Kuki umwana wanjye ari munini cyane?" Akenshi, ibi biterwa gusa n'uko wavutse ufite umwana muremure kandi ufite ubuzima bwiza. Ariko, ni gake cyane, ibi bishobora guterwa n'indwara idakunze kugaragara mu maraso. Uyu munsi, turimo kuvuga kuri imwe muri izo ndwara, Beckwith-Wiedemann Syndrome (BWS) . Ntutinye kumva iri zina. Niba ubizi, wowe na muganga wawe mushobora gufatanya kwita ku mwana wawe neza.
Mu magambo make, Beckwith-Wiedemann Syndrome (BWS) ni iki?
Iyi ni indwara idakunze kugaragara mu buryo bw’uturemangingo. Icyaba gihari ni uko zimwe mu ngirabuzimafatizo zigira uruhare mu mikurire y’umubiri w’umwana zikora cyane. Ingaruka zabyo ni uko ibice bimwe by’umubiri w’umwana cyangwa umubiri wose bikura vuba kurusha uko bisanzwe.
Ibi kandi byitwa "Beckwith-Wiedemann spectrum." "Spectrum" bivuze nk'umubare w'indwara. Ibi bivuze ko iyo ndwara idafata buri mwana mu buryo bumwe. Hari abana bashobora kugira ikimenyetso kimwe cyangwa bibiri gusa kijyanye na byo. Abandi bana bashobora kugira ibimenyetso byinshi. Kubwibyo, nta bana babiri bafite iyi ndwara babaho kimwe.
Igikuru ni uko nubwo nta muti urambye w'iki kibazo, hari uburyo bwiza bwo kuvura ibimenyetso no gukumira ingorane. Iyo ubuvuzi buvuwe neza, abenshi muri abo bana babaho ubuzima busanzwe kandi bufite ubuzima bwiza.
Ni ibihe bintu by'ingenzi biranga iyi ndwara?
Nkuko twabivuze mbere, si buri mwana wese uzagira ibi bimenyetso byose. Ariko hari bike bikunze kugaragara. Muganga wawe azakeka ko BWS iramutse ibonye kimwe cyangwa byinshi muri ibi bimenyetso.
| Ibiranga | Ibisobanuro byoroshye |
|---|---|
| Kinini kuruta ibisanzwe (Macrosomie) | Mu gihe cyo kuvuka, ibiro byabo n'uburebure bwabo biba biri hejuru cyane ugereranyije n'iby'umwana usanzwe. Baba barebire ugereranyije n'abandi bana bari mu kigero kimwe. Ariko, uku gukura vuba akenshi kuragabanuka iyo bageze ku myaka 8, kandi bakongera uburebure busanzwe iyo bamaze gukura. |
| Ururimi runini (Macroglossia) | Ururimi ni runini kuruta ibisanzwe. Ibi bishobora kugora umwana konka no kuvuga nyuma. Hari igihe no guhumeka bishobora kubaho. |
| Ibibazo byo ku rukuta rw'inda (Omphalocele / Umbilical Hernia) | Omphalocele: Indwara aho amara y'umwana, kimwe n'amara, anyura mu muhogo agapfukwa n'agahu gato aho kuba mu nda. Hernia y'umuhogo: Inzira y'imitsi yo mu nda inyura mu muhogo. Ibi bishobora gukosorwa no kubagwa. |
| Kwaguka k'uruhande rumwe rw'umubiri (Hemihyperplasia) | Uruhande rumwe rw'umubiri (urugero, uruhande rw'iburyo) rukura kuruta urundi ruhande (uruhande rw'ibumoso). Ibi bishobora kugarukira ku ruhande rwose cyangwa ku kuboko kumwe cyangwa ukuguru kumwe gusa. |
| Isukari nke mu maraso (Hypoglycemia) ku mwana ukiri mu ruhinja | Mu minsi ya mbere cyangwa ibyumweru bya mbere nyuma yo kuvuka, igipimo cy'isukari mu maraso y'umwana gishobora kugabanuka cyane. Kugikoresha neza ni ingenzi mu mikurire y'ubwonko. |
| Ibindi bintu | Umwijima wagutse (hepatomegaly), indwara z’impyiko, n’uduheri duto cyangwa iminkanyari mu duti two mu matwi bishobora kugaragara. |
Ese koko twagombye gutinya ibyago byo kurwara kanseri?
Iyi ni yo ngingo itera ubwoba ababyeyi cyane iyo bigeze kuri BWS. Yego, abana barwaye BWS bafite ibyago byinshi byo kurwara ubwoko bumwe na bumwe bwa kanseri mu bana (cyane cyane kanseri ya Wilms, kanseri y'impyiko, na hepatoblastoma, kanseri y'umwijima) kurusha abandi bana.
Ariko, tugomba gusobanukirwa ibintu bike neza hano.
1. Nubwo ibyago biri hejuru, muri rusange biracyari bike. Ibi byago bigira ingaruka ku bana bagera kuri 7-8 ku 100 bafite BWS.
2. Iyi ngaruka iba nyinshi cyane mu myaka 8 ya mbere y'ubuzima. Nyuma y'ibyo, ibyago biragabanuka cyane.
3. Ikintu cy'ingenzi cyane - kwisuzumisha kwa muganga buri gihe!Kubera ko abaganga bazi neza ibi byago, abana bose bafite BWS bapimwa ku gihe gisanzwe. Ubusanzwe, gupima mu nda hifashishijwe ultrasound no gupima amaraso (AFP test) bikorwa buri mezi atatu.
Akamaro gakomeye ko kwisuzumisha buri gihe ni uko, iyo kanseri igaragaye, ishobora kugaragara hakiri kare. Hanyuma , amahirwe yo gukira burundu hamwe no kuvurwa aba menshi cyane. Kubwibyo, icy'ingenzi ni ukudatinya ibi bitari ngombwa, ahubwo ni ugukora ibizamini ku gihe hakurikijwe amabwiriza ya muganga.
Kuki ibi bibaho? Impamvu ni izihe?
Impamvu y'ibi iragoye gato. Buri selile mu mubiri wacu ifite ikintu cyitwa chromosomes. Ibi ni nk'ibitabo by'amabwiriza yo kubaka imibiri yacu. Muri BWS, hari impinduka mu mikorere ya za gene nyinshi kuri chromosome 11 zigenga imikurire.
Mu magambo make, jeni "zigenzura" gukura zirarushaho gutuza, naho jeni "zitera" gukura zigakora cyane. Ibi byitwa "genomic imprinting ".
- Ibi akenshi biba ari ibintu bihurirana: hafi 85% by'abana barwaye BWS nta muryango baba bafite iyi ndwara. Ibi bivuze ko iyi mpinduka mu ngirabuzimafatizo ibaho ku bw'impanuka, ku buryo butunguranye.
- Ntibikunze kubyara umwana: Ijanisha rito, hagati ya 10-15%, rishobora kubyara impinduka mu ngirabuzimafatizo zijyanye n'iyi ndwara bivuye ku babyeyi babo.
- Isano ijyanye na ART: Ubushakashatsi bwagaragaje ko abana batwite binyuze mu ikoranabuhanga rifasha mu kubyara (ART), nka IVF (In Vitro Fertilization) , bafite ibyago byinshi byo kurwara indwara nka BWS. Ariko, ubushakashatsi kuri iyi sano buracyakomeje.
Abaganga bapima iki kibazo gute? (Isuzuma)
BWS ishobora gusuzumwa mbere cyangwa nyuma yuko umwana avutse.
Gusuzuma igihe cy'ivuka
Mu gihe cyo gupima ultrasound mu gihe utwite, muganga ashobora gukeka ko BWS ihari niba abonye ibi bikurikira:
- Umwana ni munini kuruta uko bisanzwe.
- Ubwinshi bw'amazi yo mu nda (amniotic fluid) hafi y'umwana (polyhydramnios)
- Kwaguka kwa placenta
- Kwaguka kw'impyiko (nephromagaly)
- Ikibazo cy'urukuta rw'inda (omphalocele)
Iyo ubonye ibi bimenyetso, muganga wawe ashobora kwemeza iyo ndwara akoresheje ikizamini cyihariye cya genetiki, nka amniocentesis (ikizamini cy'amazi ya amniotic).
Nyuma yo kuvuka k'umwana (Isuzuma rya nyuma yo kubyara)
Nyuma yo kuvuka k'umwana, muganga azasuzuma umwana arebe ibimenyetso by'umubiri twavuze mbere (ururimi runini, kwaguka k'uruhande rumwe rw'umubiri, nibindi). Niba hari gushidikanya, amaraso y'umwana ashobora gufatwa hanyuma hagakorwa ikizamini cyihariye cya genetiki kugira ngo hemezwe niba umwana afite BWS n'icyayiteye.
Ni ubuhe buryo bwo kuvura? (Ubuvuzi)
Kubera ko BWS ishobora kugira ingaruka ku bice byinshi by'umubiri, umwana ntabwo avurwa n'umuganga umwe gusa. Itsinda ry'inzobere, barimo umuganga w'abana, abaganga b'inzobere mu kubaga, n'abaganga b'imivugire, ni bo bita ku mwana.
Uburyo bwo kuvura bugenwa hashingiwe ku bimenyetso by'umwana.
| Ikibazo | Kuvura no gucunga |
|---|---|
| Isukari nke mu maraso (Hypoglycemia) | Gutanga isukari (glucose) binyuze mu mitsi, gutanga amata kenshi, rimwe na rimwe no gutanga imiti. Ibi bivurwa neza cyane mu bitaro. |
| Ururimi runini (Macroglossia) | Gukoresha ibere ryabugenewe mu konsa, kuvura indwara zo kuvuga, gukoresha imashini ya CPAP mu gihe umuntu afite ibibazo byo guhumeka mu gihe asinziriye. Hari abana bashobora kubagwa ururimi rwabo. |
| Ibibazo by'inkuta zo mu nda (Omphalocele) | Ako kanya umwana akimara kuvuka cyangwa nyuma gato y’aho, ingingo zasohotse zibagwa zigasubizwa mu nda hanyuma urukuta rw’inda rugafungwa. |
| Kwaguka k'uruhande rumwe rw'umubiri (Hemihyperplasia) | Iyo hari itandukaniro mu burebure bw'amaguru, rishobora gukosorwa hakoreshejwe inkweto zidasanzwe (orthotics). Muganga ahora akurikirana uko kwiyongera kw'amaguru. |
| Ingaruka za kanseri | Kugeza ku myaka umunani, gupima mu nda no gupima amaraso (AFP) bikorwa buri mezi 3. |
Ni ubuhe buryo bw'ejo hazaza umwana azaba afite? (Ibisobanuro ku gihe azahura nacyo)
Iki gishobora kuba ikibazo gikomeye mu mutwe wawe. Nubwo BWS ari indwara igoye, abana benshi bafite iyi ndwara babaho neza cyane, bishimye kandi babayeho neza.
Hari ibintu byinshi bigena ahazaza h'umwana:
- Ni ibihe bimenyetso umwana afite kandi ni ibihe biremereye?
- Uburyo indwara yasuzumwe vuba.
- Niba ubuvuzi n'isuzuma rya kanseri bikorwa ku gihe.
Iyo indwara imenyekanye hakiri kare, igahabwa ubuvuzi bukwiye, kandi igakomeza gukurikiranwa na muganga buri gihe, umwana ashobora kwitega ibisubizo byiza cyane.
Iyo umenye ko umwana wawe afite BWS, ushobora kumva utangaye, ubabaye, kandi ufite ubwoba. Ni ibisanzwe. Ushobora kuba ufite ibibazo byinshi. Vugana na muganga wawe kuri ibi byose. Azagufasha gusobanukirwa neza uko umwana wawe amerewe. Wibuke ko utari wenyine. Hari itsinda rikomeye ry'abaganga bashobora kugufasha. Muri kumwe, mushobora gushyiraho ahantu heza ho gukura umwana wanyu yishimye kandi afite ubuzima bwiza.
Ubutumwa bwo kujyana mu rugo
- Indwara ya Beckwith-Wiedemann Syndrome (BWS) ni indwara idakunze kugaragara mu ngirabuzimafatizo igira ingaruka ku mikurire y'umwana. Ntigira ingaruka ku bana bose kimwe.
- Ibintu by'ingenzi birimo kuba binini kuruta ibisanzwe, ururimi runini, ibibazo by'urukuta rw'inda, no kwaguka k'uruhande rumwe rw'umubiri.
- Abana bafite BWS bafite ibyago byinshi byo kurwara kanseri imwe n'imwe mu bwana, ariko gusuzumwa buri gihe bishobora kubagaragaza hakiri kare kandi bikabakiza burundu.
- Nubwo nta muti urambye w'iyi ndwara, hari uburyo bwiza bwo kuvura ibibazo bivuka.
- Iyo ubuvuzi buvuwe neza kandi bugakurikiranwa neza, abana benshi bafite BWS babaho ubuzima busanzwe, bufite ubuzima bwiza kandi bwuzuye. Ni ngombwa gukorana bya hafi na muganga wawe.
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