Ese umwana wawe afite utudomo duto dutukura ku mubiri we? Reka twige kuri PHACE Syndrome.

Ba mama na ba papa, mushobora kugira impungenge no gutinya iyo mubonye utudomo duto dutukura cyangwa utubyimba duto ku mubiri w'umwana wanyu, rimwe na rimwe ku maso no mu ijosi. Hari igihe ibi biba ari ibintu bizakira gusa, ariko gake cyane, bishobora kuba ikimenyetso cy'indwara idasanzwe kandi igoye nka PHACE Syndrome . Rero, nta kundi, reka tuganire birambuye ku ndwara ya PHACE Syndrome, icyo itera, n'icyo twakora niba ari ikintu nk'icyo.

Indwara ya PHACE ni iki?

Noneho, reka tubanze turebe icyo PHACE syndrome ari cyo. Mu magambo make, ni indwara idakunze kugaragara. Ibyo bivuze ko idakunze kubaho kuri buri wese. Ni indwara ivukana umwana akiri mu nda. Iyi ndwara ishobora kugira ingaruka ku bice bitandukanye by'umubiri w'umwana. Igira ingaruka cyane cyane ku ruhu, ubwonko, umutima, imitsi minini y'amaraso n'amaso .

Akenshi, hari inenge nke muri buri gice muri ibi bice. Hari abana bashobora kugaragaza ibimenyetso bike gusa byavuzwe hano hepfo. Indwara zikunze kugaragara ku bana barwaye PHACE ni hemangiomas , indwara z'imitsi yo mu bwonko , n'indwara z'umutima . Ibi bishobora kugaragara mu gihe cyo gutwita, mu gihe cyo kuvuka, cyangwa mu bwana.

Mu by’ukuri PHACE ni amagambo ahinnye y’indwara zitandukanye. Isobanura indwara zikomeye zijyanye n’iyi ndwara. Rimwe na rimwe yitwa PHACES syndrome. 'S' y’inyongera isobanura gucika kw’igufwa ry’igufwa ry’igufwa . Ibi bivuze ko igufwa riri hagati mu gituza, sternum, ritameze neza, cyangwa hari inenge ririmo.

Ni ibihe bimenyetso by'indwara ya PHACE?

None se, ni ibihe bimenyetso by'umwana urwaye PHACE syndrome? Ibi bishobora gutandukana bitewe n'umwana. Hari abana bagira ibimenyetso bike, abandi bakagira byinshi. Kandi uburemere bw'ibimenyetso nabwo buratandukanye bitewe n'umwana. Reka turebe ibimenyetso by'ingenzi:

• Hemangiomas: Izi ni utubyimba tugaragara kandi dufite ibara ry’uruhu. Mu by’ukuri tuba tugizwe no gukura kw’imitsi y’amaraso yiyongereye . Akenshi tugaragara ku mutwe, mu maso, mu ijosi, ku mubiri no ku maboko. Akenshi tuba tunini cyane kandi dushobora gukwirakwira. Ariko, rimwe na rimwe izi hemangiomas zishobora no kugira ingaruka ku ngingo z’imbere z’umwana. Ibi bibaye, ibibazo byo kutabona no kumva bishobora kubaho.

• Ibitagenda neza mu bwonko:Fossa y'inyuma ni agace k'ubwonko gagenzura imikorere y'ingenzi nko kuringaniza, guhuza imikorere, no guhumeka. Iyo hari ikintu kidasanzwe muri aka gace, umwana ashobora kugira ingendo zidakorwa ku bushake ndetse n'ubumuga bwo mu mutwe . Hashobora kandi kubaho impinduka mu miterere y'ubwonko ubwabwo.

• Umutima utari mwiza: Hari ubwo umutima udafite ubusembwa bushobora gutera ibimenyetso nko guhumeka nabi , gucika intege kw'imitsi , umuvuduko ukabije w'amaraso , guhangayika kenshi, no kubira ibyuya. Hari ubwo umutima w'umwana uba utarakura neza, kandi hashobora kubaho umwobo hagati y'ibyumba by'umutima (ventricular septal defect) . Cyangwa, hashobora kubaho kugabanuka k'umuyoboro w'amaraso (coarctation of the aorta).

• Ibitagenda neza mu mitsi: Iyo imitsi ijyana amaraso mu bwonko no mu ijosi igabanutse cyangwa ikagira ibindi bibazo, ubwonko bushobora kutagira amaraso ahagije. Ibi bishobora gutera indwara zikomeye nko kurwara imitsi itera amaraso mu bwonko , gucika intege , gucika imitsi mu bwonko , cyangwa kubura ibyiyumvo ku ruhande rumwe rw'umubiri.

• Ubusembwa bw'amaso: Amaso y'umwana, imitsi y'amaso, cyangwa imitsi y'amaraso mu maso bishobora kuba bitarakura neza. Ibi bishobora gutera kutabona neza, guta ubwenge , amaso mato cyane , imyitwarire idahwitse y'amaso, cyangwa amaso arembye .

• Ibitagenda neza mu magufwa y'ibere: Nkuko byavuzwe mbere, igufwa ry'ibere (sternum) riri hagati mu gituza cy'umwana rishobora kuba ritameze neza cyangwa ridakura neza. Ibi bishobora gutuma imicanga, utubyimba n'imyobo bigaragara muri ako gace.

Ingorane zishobora guterwa na PHACE syndrome

Abana barwaye PHACE syndrome bashobora kugira ibibazo bimwe na bimwe bitewe n'ibi bintu bidasanzwe mu miterere y'umubiri wabo no mu mitsi y'amaraso. Dore ibyo ari byo:

• Ibibazo by'uburinganire
• Ibibazo by'amenyo, urugero , hypoplasia ya enamel n'ibibazo by'imizi y'amenyo.
• Kugorwa no kumira
• Ubusembwa bw'imikorere y'imisemburo . Urugero, kugabanuka k'imikorere ya tiroyide no kugabanuka k'umusaruro w'imisemburo.
• Kuribwa umutwe kenshi, rimwe na rimwe migraine .
• Iterambere ribi
• Gutinda kuvuga no kuvuga
• Ingaruka zo kurwara indwara yo mu bwonko ziyongera.
• Ingingo z'imyororokere zidakura neza

Ni iki gitera indwara ya PHACE?

Abashakashatsi ntibaramenya neza impamvu bamwe mu bana barwara indwara ya PHACE. Ubushakashatsi bwerekana ko ishobora guterwa n’ibintu byombi bigize imiterere y’umubiri ndetse n’ibidukikije. Ariko, itandukaniro nyaryo ry’imiterere y’umubiri ribitera ntiriramenyekana neza. Impuguke zimwe na zimwe zemeza ko biterwa n’impinduka mu buryo butunguranye (de novo) zibaho mu gihe cyo gusama. Ibi bivuze ko akenshi idakomoka ku babyeyi.

Ni gute indwara ya PHACE ipimwa?

None se, abaganga bapima bate indwara ya PHACE? Ubusanzwe, umuganga azakora isuzuma ryimbitse ry’umubiri akanakora ibizamini . Bafite ibipimo byihariye byo gusuzuma , ari byo bishingirwaho. Ni bwo buryo bagena niba umwana afite indwara ya PHACE.

Hari igihe abaganga bashobora kubona icyo kibazo bahereye ku bipimo by’indwara ufata utwite. Ariko hari igihe bishobora kutagaragara kugeza igihe umwana avutse, cyangwa nyuma y’igihe gito.

Akenshi, muganga abanza gushaka hemangioma nini ku mubiri w'umwana, cyane cyane ku maso, mu ijosi, cyangwa ku mutwe. Kuba hariho hemangioma imwe ndetse n'ikindi kintu cy'ingenzi kijyanye na PHACE bishobora kuba bihagije kugira ngo hamenyekane indwara.

Byongeye kandi, ibizamini byinshi bishobora gukorwa kugira ngo harebwe neza ubwonko bw'umwana, umutima we, imitsi ye n'amaso ye. Ibi ni byo bizamini bikunze gukorwa:

• gupima CT (CT scan)
• Echocardiograms - Ibi bireba imiterere n'imikorere y'umutima.
• Isuzuma ry'amaso - rikorwa n'umuganga w'amaso.
• Ibizamini byo kumva
• Gupima MRI - Fata amashusho arambuye, cyane cyane ay'ubwonko n'imitsi y'amaraso.
• Gupima hakoreshejwe ikoranabuhanga rya Ultrasound

Indwara ya PHACE ivurwa ite?

Mu kuvura indwara ya PHACE, intego nyamukuru ni ukwirinda ingorane zishobora kubaho no gufasha umwana kubaho neza n'ibimenyetso bye. Uburyo bwo kuvura buratandukanye bitewe n'ingingo cyangwa ibice by'umubiri iyo ndwara igiraho ingaruka.Ibyo bivuze ko atari abana bose bahabwa ubuvuzi bumwe.

Ubu bwoko bw'ubuvuzi bukunze gukorwa:

• Uburyo bwo kuvura hemangiomas ku ruhu hakoreshejwe laser , cyangwa kubaha imiti (urugero: beta-blockers nka propranolol) kugira ngo ibagabanyirize.
• Gutanga uburezi bwihariye , ubuvuzi bwo kuvuga, na physiotherapy kugira ngo bifashe mu myigire ku ishuri.
• Hari igihe kubagwa biba ngombwa kugira ngo hakosorwe inenge mu mutima, mu mitsi y'amaraso, cyangwa mu bwonko.
• Gutanga imiti itandukanye yo kurwanya igicuri, kuribwa umutwe, no kudahuza kw'imisemburo.
• Gutanga ibikoresho bifasha mu kuvura ububabare bwo kutumva n'indorerwamo zo mu bwonko.

Imiryango myinshi iganira n'umujyanama mu by'imiterere y'umubiri kugira ngo bamenye byinshi ku bijyanye n'uburyo bwo kuvura abana babo, ndetse n'uburyo bwo kubafasha mu gihe bakura. Ibi bishobora kuba ingirakamaro cyane.

Ese ibi bishobora kwirindwa?

Ikibabaje ni uko nta buryo bwo kwirinda indwara ya PHACE. Kubera ko impamvu nyayo itazwi, nta kintu na kimwe wakora kugira ngo ugabanye ibyago byo kubyara umwana ufite iyi ndwara.

Ariko niba ushaka kumenya byinshi ku bijyanye n'amahirwe yawe yo kubyara umwana ufite ikibazo cy'uturemangingo, ushobora kuvugana na muganga wawe ku bijyanye no gupimwa uturemangingo .

Bigenda bite umwana wanjye aramutse afite PHACE syndrome? Ni iki cyamubaho?

Niba umwana wawe afite iyi ndwara, azakenera kwisuzumisha buri gihe kwa muganga we w’ibanze n’inzobere mu nzego zitandukanye (urugero: abaganga b’indwara z’umutima, inzobere mu ndwara z’imitsi, inzobere mu by’amaso) kugira ngo barebe neza ko ibimenyetso bye bitagize ingaruka ku mibereho ye. Iyi ni indwara isaba ko icungwa igihe kirekire.

Iyi ndwara ishobora kugira ingaruka ku mwana gusa, ndetse no kuri wowe n'abandi bagize umuryango. Kubwibyo, ni ngombwa cyane gutekereza ku buzima bwo mu mutwe bwawe. Hari abantu babona ihumure rikomeye n'imbaraga mu kuganira n'inzobere mu by'ubuzima bwo mu mutwe .

None se umuntu ufite iyi ndwara azamara igihe kingana iki? Biterwa n'uburemere bw'ibimenyetso by'umwana ndetse n'ingingo z'umubiri indwara yafashe.

Abana bafite ikibazo gikomeye cyo kwangirika k'ubwonko n'umutima baba bafite amahirwe menshi yo kubaho igihe gito. Ariko, iyo umwana afite ibimenyetso byoroheje kandi akaba yafashwe neza, ashobora kubaho ubuzima busanzwe.

Ni ryari ukwiye kujya kwa muganga?

Niba umwana wawe afite kimwe muri ibi bikurikira, menya neza ko wagiye kwa muganga:

• Niba ufite ikibazo cyo kurya cyangwa kunywa
• Iyo umwana adashoboye kugera ku ntego z'iterambere zijyanye n'imyaka ye, ni ukuvuga niba akura atinze (urugero: kudafata ijosi rye ku gihe, kutagenda, kutavuga).
• Niba udasubiza ibintu mu buryo bugaragara, ni ukuvuga niba ubona ikintu kidasanzwe mu maso yawe.
• Niba ubonye akadomo kanini, gatukura, ku mubiri wawe (nk'agakoko ka hemangioma).

Ni ryari ugomba kujyana umwana wawe mu bitaro vuba?

Ubonye ikintu nk'iki, hita ujyana umwana wawe mu cyumba cy’ubutabazi cya hafi, cyangwa uhamagare 1990:

• Niba ugaragaza ibimenyetso byo gucika intege mu mitsi (urugero: kunama k'uruhande rumwe rw'isura, kubura ubushobozi bwo gukora kw'ukuboko, kudashobora kuvuga).
• Niba ufite isereri.
• Niba ufite ikibazo cyo guhumeka, niba wumva urimo kubura umwuka.
• Iyo umutima utera nabi, cyangwa niba umwana ahinduka ubururu.

Ibibazo byo kubaza muganga/Madamu.

Ushobora kubaza muganga w'umwana wawe cyangwa umuforomo ibibazo nk'ibi. Ibi bizagufasha gusobanukirwa uko ibintu bimeze:

• Ese koko umwana wanjye afite PHACE syndrome? Ese ngomba gukora ibindi bipimo kugira ngo nemeze ko ari yo?
• Ese umwana wanjye akeneye kubagwa ? Niba ari byo, kuki kandi ryari?
• Ese hari uburyo bwo kuvura hemangioma y'umwana wanjye? Ese izakira burundu?
• Ese hari ingaruka mbi kuri ubu buryo bwo kuvura? Ni izihe?
• Ni ibihe bintu tugomba kwitondera cyane ku mwana?
• Imyaka y'ubuzima bw'umwana wanjye izaba imeze ite?
• Ese umwana azashobora kubaho ubuzima busanzwe afite iyi ndwara?

Amaherezo, hari icyo ugomba kuzirikana

Iyo umenye ko umwana wawe afite indwara idakunze kugaragara kandi utabizi neza, ushobora kumva utangaye cyane, ubabaye kandi ufite ubwoba. Biragoye kubyemera. Ibi bishobora kugira ingaruka zikomeye ku marangamutima yawe no ku muryango wawe.

Hari abana babaho neza kugeza bakuze bafite iyi ndwara. Ariko, ku bandi, igihe cyo kubaho gishobora kugabanuka bitewe n'ibimenyetso bikomeye. Kwinjira mu itsinda ry'abaterankunga, cyangwa kuvugana n'umujyanama mu by'imiterere y'umubiri cyangwa inzobere mu by'ubuzima bwo mu mutwe , bishobora kuba isoko ikomeye y'ihumure n'imbaraga kuri wowe n'umuryango wawe muri iki gihe. Bityo rero ntutindiganye kubona ubwo bufasha.

Wibuke ko utari wenyine. Hari abaganga, abaforomo, n'abandi benshi bashobora kugufasha guhangana n'ibi bibazo, gutega amatwi ibibazo byawe, no kuguha amakuru ukeneye. Rero, komeza ukomere kandi ugerageze guha umwana wawe ibyiza akeneye.

` Phase syndrome, hemangioma, indwara zo mu muryango, ubusembwa bw'ubwonko, ubumuga bw'umutima, ubumuga bw'imitsi, ubusembwa bw'amaso, indwara z'uturemangingo, indwara zidasanzwe, ubuzima bw'umwana