Iyo Sisteme y'Ubwugarizi Idashoboye (Primary Immunodeficiency) Ni Iki? Ibimenyetso, Intandaro, n'Ubuvuzi

Iyo sisteme y'ubwugarizi idakora neza kuva umuntu avuka kubera impinduka mu mbaraga z'umubiri. Tekereza kuri sisteme y'ubwugarizi yawe nk'itsinda ry'abarinzi b'umubiri wawe - iyo ifite ubu burwayi, bamwe mu bagize itsinda baba babuze cyangwa badashobora gukora akazi kabo neza. Ibi bituma ushobora kurwara indwara zandura zishobora kuba nta kibazo ku bandi ariko zikaba zikomeye kuri wewe.

Aya mararyi agera ku muntu 1 kuri 1,200 ku isi hose, nubwo abenshi batamenya ko barwaye imyaka myinshi. Inkuru nziza ni uko, hamwe n'ubuvuzi bukwiye, abantu benshi bafite ubu burwayi bashobora kubaho ubuzima buuzuye kandi buhamye.

Ibimenyetso by'Iyo Sisteme y'Ubwugarizi Idashoboye Ni Ibihe?

Ikimenyetso gikomeye cyane cy'iyi ndwara ni ukurwara indwara zandura kenshi, cyane, cyangwa ahantu hatasanzwe ugereranije n'abandi bantu. Izi si izirwara zisanzwe buri wese arwara - ahubwo ni indwara zandura zisa nkaho zirambanye, zigaruka kenshi, cyangwa zigira ingaruka ku bice by'umubiri bisanzwe bitarwara.

Dore ibimenyetso bisanzwe ushobora kubona:

• Indwara zikunze kwibasira imyanya y'ubuhumekero nka pneumonia, bronchitis, cyangwa indwara z'ibinywa
• Indwara z'uruhu zikira buhoro cyangwa zigaruka kenshi
• Ibibazo by'igogorwa harimo impiswi zidakira cyangwa indwara z'igifu
• Indwara z'amatwi zigaruka kenshi
• Gukira buhoro ibikomere, inenge, cyangwa ibikomere byavanyweho n'abaganga
• Kumva unaniwe cyangwa ushakeje igihe kinini
• Umuhogo ubabara utakiza

Bamwe mu bantu bagira kandi ibimenyetso by'indwara ziterwa na systeme y'ubwugarizi, aho sisteme yabo y'ubwugarizi itera ibibazo ku bice byiza by'umubiri wabo. Ibi bishobora kugaragara nk'ububabare bw'ingingo, ibibyimba ku ruhu, cyangwa ibibazo by'impyiko cyangwa umwijima.

Mu bihe bitoroshye, ushobora kwandura indwara zidasanzwe ziterwa na mikorobe zisanzwe zitatera indwara ku bantu bazima. Izi ndwara ziterwa n'ubudahangarwa bw'umubiri zishobora kwibasira ibihumekero, ubwonko, cyangwa indi mirengwe y'ingenzi, kandi akenshi zisaba ubuvuzi bwihariye.

Ubwoko bw'ubudahangarwa bw'umubiri bw'ibanze ni bwoki?

Hari ubwoko burenga 400 bw'ubudahangarwa bw'umubiri bw'ibanze, buri bwoko bugira ingaruka ku bice bitandukanye by'ubudahangarwa bw'umubiri. Ubusanzwe bugabanyijemo ibice bikuru bitewe n'ibice by'ubudahangarwa bw'umubiri bitakora neza.

Ubwoko busanzwe harimo:

• Ubuke bw'antikorora: Umubiri wawe ntukora imwe imwe ya poroteyine zirwanya indwara zitwa antikorora.
• Ubuke bw'uturemangingo T: Uturumangingo tw'amaraso yera duhuza imikorere y'ubudahangarwa bw'umubiri ntidukora neza.
• Ubuke bw'ubudahangarwa bw'ibanze buhuriweho: Antikorora n'uturemangingo T byombi bigira ingaruka.
• Ubuke bw'ibintu byongerera imbaraga antikorora: Poroteyine zifasha antikorora kurwanya indwara zirabura.
• Indwara z'uturemangingo phagocyte: Uturemangingo turya mikorobe mbi ntibishobora gukora akazi kabo neza.

Bimwe mu byamamare harimo ubuke bwa antikorora busanzwe (CVID), bugira ingaruka ku gukora antikorora, n'ubudahangarwa bukomeye bw'umubiri (SCID), bugira ingaruka kuri antikorora n'uturemangingo T byombi. Buri bwoko bufite ibimenyetso n'uburyo bwo kuvura byabyo.

Ni iki giterwa n'ubudahangarwa bw'umubiri bw'ibanze?

Ubudahangarwa bw'umubiri bw'ibanze biterwa n'impinduka mu mimerere y'impyiko zigira ingaruka ku iterambere n'imikorere y'ubudahangarwa bw'umubiri. Izi mpinduka z'impyiko zibaho kuva ku ivuka, niyo mpamvu bitwa “ibanze” - ikibazo nyamukuru, atari ikintu giterwa n'ubundi burwayi cyangwa ubuvuzi.

Ubusanzwe bibaho kubera impinduka mu mimerere y'impyiko imwe gusa igenzura imikorere y'ingenzi y'ubudahangarwa bw'umubiri. Izi mpinduka zishobora kuvanwa ku babyeyi cyangwa zikabaho zitewe n'impanuka mu iterambere rya mbere.

Uburyo bwo kwandura indwara bitandukanye bitewe n’uburwayi ubwo ari bwo:

• Iherereye kuri X: Impinduka y’imiterere y’impyiko iri kuri chromosome X, bityo bikagira ingaruka cyane ku bahungu
• Iherereye kuri Autosomal recessive: Ukeneye kopi ebyiri z’imiterere y’impyiko yahindutse, imwe iva kuri buri mubyeyi
• Iherereye kuri Autosomal dominant: Ukeneye kopi imwe gusa y’imiterere y’impyiko yahindutse kugira ngo ugire ubwo burwayi

Rimwe na rimwe, impinduka y’imiterere y’impyiko iba ku nshuro ya mbere kuri wowe, bivuze ko ababyeyi bawe badafite ubwo burwayi ariko wowe ubufite. Ibi bita impinduka nshya cyangwa ihinduka ry’umwanya.

Ni ryari ukwiye kubona muganga kubera Primary Immunodeficiency?

Waganiriza muganga wawe niba ugira ubwandu kenshi kurusha uko bisanzwe kuri wowe. Jya witondera imiterere- ese ugira pneumonia buri mwaka, cyangwa ibikomere bito bifata ibyumweru kugira ngo bikire?

Shaka ubufasha bw’abaganga niba ugira ibi bikurikira:

• Amazimwe arenga 4 mu mwaka umwe
• Amazimwe akomeye abiri cyangwa arenga mu mwaka umwe
• Pneumonia ebyiri cyangwa zirenga mu mwaka umwe
• Ubwandu budakira imiti isanzwe yo kuvura ubwandu
• Ubwandu butasanzwe cyangwa ubwandu buri ahantu hatasanzwe
• Amateka y’umuryango afite Primary Immunodeficiency

Ntugatege amatwi niba ugize ibimenyetso bikomeye nko kugira ikibazo cyo guhumeka, umuriro mwinshi udatakaza, cyangwa ibimenyetso by’ubwandu bukomeye. Ibi bibujijwe bikeneye ubufasha bw’abaganga vuba.

Niba uhangayikishijwe n’uburyo ubwandu bwawe bumeze, tanga uganire n’umuganga wawe usanzwe. Bashobora gusuzuma ibimenyetso byawe bakakwerekeza kwa immunologist (inzobere mu ndwara zifata ubudahangarwa bw’umubiri) niba bibaye ngombwa.

Ni ibihe bintu byongera ibyago bya Primary Immunodeficiency?

Ikintu gikomeye cyongera ibyago bya Primary Immunodeficiency ni ukugira abagize umuryango bafite ubwo burwayi. Kubera ko izi ari indwara zikomoka ku mpyiko, zishobora gukwirakwira mu muryango, nubwo uburyo bwo kwandura butandukanye.

Ibindi bintu byongera ibyago byawe birimo:

• Kuba umuhungu (ubwoko bumwe na bumwe busanzwe cyane mu bahungu kubera gakondo ya X-linked)
• Kugira ababyeyi bafitanye isano (byongera amahirwe yo kugira ibibazo bya genetique byihishe)
• Kuba mu matsinda amwe n'amwe y'abantu aho impinduka za genetique runaka zikunze kugaragara
• Kugira ibindi bibazo bya genetique bigira ingaruka ku iterambere ry'umubiri w'ubudahangarwa

Ni ngombwa gusobanukirwa ko ntushobora gukumira ubudahangarwa bw'ibanze binyuze mu guhindura imibereho kuko biterwa na gene zawe. Ariko, kumenya amateka y'umuryango wawe bishobora gufasha abaganga kumenya ibimenyetso hakiri kare no gutangira kuvura vuba.

Ibibazo bishoboka by'ubudahangarwa bw'ibanze ni ibihe?

Iyo ubudahangarwa bw'ibanze budakurikiranwe neza, bushobora gutera ibibazo bikomeye bigira ingaruka ku bice byinshi by'umubiri wawe. Ikibazo nyamukuru ni uko indwara zishobora kuba zikomeye kandi zigakwirakwira mu ngingo z'ingenzi.

Ibibazo bisanzwe birimo:

• Indwara z'ibihaha zidakira zishobora gutera ibibazo by'ibihaha bidakira
• Indwara z'umuyoboro w'igogorwa zigatuma umuntu atagira imirire ihagije akaba atakaza ibiro
• Ibibazo by'umwijima cyangwa impyiko biturutse ku ndwara zisubiramo
• Indwara zifata umubiri w'ubudahangarwa aho umubiri w'ubudahangarwa utera ibindi bice by'umubiri
• Ibyago byiyongereye byo kurwara kanseri, cyane cyane lymphoma
• Ibibazo by'ubukure mu bana kubera indwara zidakira

Bamwe mu bantu barwara bronchiectasis, uburwayi aho inzira z'umuhogo mu bihaha ziba zaguka burundu kandi zigakomereka kubera indwara zisubiramo. Ibi bishobora gutuma guhumeka bigorana kandi bisaba kuvurwa buri gihe.

Mu bihe bitoroshye, ubwoko bukomeye bw'ubudahangarwa bw'ibanze bushobora kuba bwangiza ubuzima ntibuvuwe vuba. Ariko, kubera ubufasha bw'abaganga no gukurikiranwa, ibibazo byinshi bishobora gukumirwa cyangwa bigacungwa neza.

Ubudahangarwa bw'ibanze bupimwa gute?

Kumenya indwara y’ubudahangarwa bw’umubiri (primary immunodeficiency) bisanzwe bitangira muganga akubajije ibibazo birambuye ku mateka y’indwara wanduye n’amateka y’ubuzima bw’umuryango wawe. Azashaka kumenya ubwoko, umubare, n’uburemere bw’indwara wanduye.

Muganga wawe ashobora gutegeka ko ukorwa ibizamini by’amaraso kugira ngo barebe ibice bitandukanye by’ubudahangarwa bwawe:

• Igipimo cy’amaraso cyuzuye (Complete blood count) kugira ngo barebe urwego rw’uturemangingabo tw’amaraso yera
• Igipimo cy’imbere y’umubiri (Immunoglobulin levels) kugira ngo bapime imiti yica mikorobe
• Ibizamini byo kureba uko umubiri wakira inkingo (Antibody response tests) kugira ngo barebe uko wakira inkingo
• Igipimo cy’uturemangingabo twa T na B n’ibizamini by’imikorere yabyo
• Igipimo cy’ibintu by’ubudahangarwa (Complement levels) kugira ngo bapime aya maporoteyine akomeye y’ubudahangarwa

Niba ibizamini by’ibanze bigaragaza ikibazo, muganga wawe ashobora kugutegeka gukora ibizamini bya gene kugira ngo bamenye impinduka z’imiterere ya gene ziterwa n’iyi ndwara. Ibi bishobora gufasha kumenya neza ubwoko bwa primary immunodeficiency ufite.

Rimwe na rimwe abaganga bapima n’abantu bo mu muryango kugira ngo bamenye uko indwara iragwa kandi bamenye abandi bashobora kuba barayanduye. Igikorwa cyo kumenya indwara gishobora gutwara igihe, ariko kumenya neza indwara ni ingenzi kugira ngo uvurwe neza.

Ubuvuzi bw’indwara y’ubudahangarwa bw’umubiri (Primary Immunodeficiency) ni iki?

Ubuvuzi bw’indwara y’ubudahangarwa bw’umubiri bugamije gukumira indwara no gusubiza ibice by’ubudahangarwa bw’umubiri biba biba byabuze. Ubuvuzi buhabwa umuntu biterwa n’ubwoko bw’indwara y’ubudahangarwa bw’umubiri afite n’uburemere bw’iyi ndwara.

Uburyo nyamukuru bw’ubuvuzi burimo:

• Ubuvuzi bwo gusimbuza immunoglobulin: Inyitiro zisanzwe z’antibody zikomoka ku batanze amaraso bazima
• Antibiyotike zirinda: Imiti ifatwa buri munsi yo kwirinda indwara ziterwa na bagiteri
• Imiti irwanya fungi: Yo kwirinda indwara ziterwa na fungi mu bihe bimwe na bimwe
• Ubuvuzi bwa interferon: Ku bwoko bumwe na bumwe bwa immunodeficiency
• Gusimbuza umwanya w’amasogwe y’igitsina: Ku ndwara zikomeye za immunodeficiency
• Ubuvuzi bwa gene: Uburyo bushya bwo kuvura zimwe mu ndwara

Ubuvuzi bwa immunoglobulin ni bwo bukunzwe gukoreshwa mu kuvura ubuke bw’antibody. Uzajya uhabwa izo nyitiro binyuze mu mutsi (IVIG) cyangwa munsi y’uruhu (SCIG) buri cyumweru bike. Abantu benshi basanga ubuvuzi buragabanya cyane umubare w’indwara.

Ku ndwara zikomeye, gusimbuza umwanya w’amasogwe y’igitsina bishobora gukiza indwara binyuze mu gusimbuza sisitemu yawe y’ubudahangarwa itagikora neza n’iy’umuntu muzima. Ariko, ibi bifite ibyago byinshi kandi bikunze gukoreshwa gusa ku ndwara zihitana ubuzima.

Uko wakwita ku buzima bwawe iwawe mugihe ufite Primary Immunodeficiency?

Kwita ku buzima bwawe iwawe mugihe ufite Primary Immunodeficiency bisobanura gufata ingamba zo kwirinda indwara mugihe ugerageza kugira ubuzima busanzwe. Gukora isuku neza birakomeye cyane igihe sisitemu yawe y’ubudahangarwa ikeneye ubufasha bwiyongereye.

Dore ingamba nyamukuru zo kwita ku buzima bwawe iwawe:

• Koga intoki kenshi n’amazi n’isabune byibuze amasegonda 20
• Kwirinda ahantu hahuriye abantu benshi mu gihe cy’icyorezo cya grippe cyangwa izindi ndwara
• Kwirinda inkingo zose zisabwa (ariko wirinda inkingo zikora)
• Kurya indyo yuzuye kugira ngo ubuzima bwawe bube bwiza
• Kuryama bihagije kugira ngo sisitemu yawe y’ubudahangarwa ikore neza
• Kunywa amazi ahagije
• Kwirinda abantu barwaye igihe bishoboka

Niba uhabwa imiti igabanya ubukana bw’indwara mu rugo, menya neza ko usobanukiwe uburyo bwo guterwa inshinge neza n’uburyo bwo kubika imiti. Komereza hafi ubushyuhe bwo gupima (thermometer) kandi menya igihe ugomba guhamagara muganga kubera umuriro cyangwa ibindi bimenyetso.

Tegura gahunda y’ibikorwa hamwe n’itsinda ry’abaganga bawe kugira ngo muhagarike kwandura hakiri kare. Ibi bishobora kuba birimo kugira imiti igabanya ubukana bw’indwara (antibiotics) cyangwa kumenya igihe ugomba gushaka ubuvuzi bwihuse.

Wategura gute uruzinduko kwa muganga?

Gutegura neza uruzinduko rwawe kwa muganga bituma muganga asobanukirwa neza uko uhagaze, kandi bikaba byiza mu gihe cyanyu hamwe. Tangira wandike amateka yawe y’indwara z’ubwandu uko bishoboka kose.

Zana amakuru akurikira:

• Urutonde rw’indwara zose z’ubwandu wahuye nazo, harimo amatariki n’uburyo wavuye.
• Imiti n’ibindi byongerwamo ubu ukoresha.
• Amateka y’ubuzima bw’umuryango wawe, cyane cyane ibibazo by’ubudahangarwa bw’umubiri.
• Ibibazo ku ndwara yawe n’uburyo bwo kuyivura.
• Ibisubizo by’ibizamini bya vuba cyangwa impapuro z’ubuvuzi.
• Amakuru y’ubwishingizi n’amafishi yo kwerekezwa aho bikenewe.

Andika ibibazo byawe by’ingenzi mbere, kuko gahunda zishobora kumvikana nk’izihuse. Ntugatinye kubabaza icyakubangamiye, harimo ingaruka mbi z’imiti cyangwa guhindura imibereho.

Tegereza kuzana umuntu wo mu muryango wawe cyangwa incuti izagufasha kwibuka amakuru yavuzwe mu gihe cy’uruzinduko. Bashobora kandi kugutera inkunga mu gihe cy’uruzinduko rushobora kuba rukomeye.

Ni iki cy’ingenzi cyo kumenya ku buke bw’ubudahangarwa bw’umubiri?

Ubuke bw’ubudahangarwa bw’umubiri ni uburwayi bushobora kuvurwa neza bumenyekanye kandi bukavurwa. Nubwo bivuze ko ubudahangarwa bwawe bukenera ubufasha bwiyongereye, abantu benshi bafite izi ndwara bashobora kubaho ubuzima buzuye kandi buhamye bafite ubuvuzi bukwiye.

Ikintu cy’ingenzi ni ukumenya imiterere y’indwara zikunze kugaragara cyangwa zikomeye, no gushaka ubuvuzi. Kugirwaho isuzumwa hakiri kare no kuvurwa birashobora gukumira ingaruka zikomeye kandi bikarushaho kunoza imibereho yawe.

Wibuke ko kuba ufite ubumuga bw’umubiri budasanzwe ntibivuze ko uri umuntu udakomeye cyangwa ko ugomba kuba mu kato. Ufite ingamba zikwiye n’ubuvuzi, ushobora gukora, gukora ingendo, kugira imibanire, no gukurikira intego zawe kimwe n’abandi.

Komereza gufatanya n’abaganga bawe, ukore gahunda yawe y’ubuvuzi, kandi ntutinye kuvugana nabo igihe ufite impungenge. Ikipe yawe y’abaganga iriho kugufasha gucunga iyi ndwara neza.

Ibibazo bikunze kubaho ku bijyanye n’ubudahangarwa bw’umubiri budasanzwe

Ese ubumuga bw’umubiri budasanzwe bushobora gukira?

Ubwoko bwinshi bw’ubudahangarwa bw’umubiri budasanzwe ntiburakirwa, ariko bushobora gucungwa neza cyane hakoreshejwe ubuvuzi bukwiye. Kugira igura ry’amasogwe y’inyuma bishobora gukiza bimwe mu bwoko bukomeye, kandi ubuvuzi bw’imiterere y’imyororokere bugaragaje icyizere ku ndwara zimwe na zimwe. Ariko kandi, abantu benshi babaho ubuzima busanzwe bafite ubuvuzi bukomeza aho gushaka gukira.

Ese ubumuga bw’umubiri budasanzwe bwandura?

Oya, ubumuga bw’umubiri budasanzwe ntibwandura. Ni indwara y’imyororokere wavukanye, si ikintu ushobora kwandura cyangwa gutwara abandi mu mibanire isanzwe. Ariko kandi, abantu bafite ubumuga bw’umubiri budasanzwe bashobora kurushaho kwandura indwara z’abandi.

Ese nashobora kubyara abana mfite ubumuga bw’umubiri budasanzwe?

Yego, abantu benshi bafite ubumuga bw’umubiri budasanzwe bashobora kubyara abana. Ariko kandi, kubera ko izi ari indwara z’imyororokere, hari amahirwe yo gutwara iyi ndwara ku bana bawe. Inama ku bijyanye n’imyororokere irashobora kugufasha kumva ibyago no gusesengura ibintu nko gupima imyororokere mu gihe cyo gutwita.

Ese ubumuga bwanjye bw’umubiri budasanzwe buzarushaho kuba bubi uko igihe kigenda?

Ibi biterwa n’ubwoko bwa primary immunodeficiency ufite. Zimwe muri izo ndwara ziguma zimeze kimwe ubuzima bwose iyo uvuwe neza, izindi zishobora gukura cyangwa guhinduka uko iminsi igenda. Kujya gukurikiranwa n’abaganga bawe buri gihe bituma umenya impinduka zose kandi bagahita bahindura uburyo bwo kuvura uko bikenewe.

Mbese, umuntu ufite primary immunodeficiency ashobora gukora ingendo?

Yego, abantu benshi bafite primary immunodeficiency bakora ingendo mu mutekano usesuye iyo babiteguye neza. Ugomba kuvugana n’abaganga bawe ku bijyanye n’igihe cyo kuvurwa, gutwara imiti, no kwitwararika cyane mu duce tumwe na tumwe. Hari ibihugu bisaba inkingo zihariye cyangwa kwitwararika cyane.