Ese inkari zawe zijimye? Ese uhora unaniwe? Bishobora kuba ari PNH (Paroxysmal Nocturnal Hemoglobinuria)!

Ese hari n'ihinduka ry'ibara ry'inkari zawe iyo ukangutse mu gitondo, ukagira umutuku wijimye, umukara, cyangwa rimwe na rimwe umukara? Cyangwa wumva unaniwe cyane kandi unaniwe nta mpamvu umunsi wose? Ibi bishobora gusa n'ibintu bisanzwe tutitaho cyane mu buzima bwacu bwa buri munsi. Ariko, rimwe na rimwe ibi bimenyetso bito bikenera kwitabwaho gato, kuko bishobora kuba bihishe indwara idasanzwe ariko ikomeye. Uyu munsi tugiye kuvuga kuri imwe muri izo ndwara zifitanye isano n'amaraso, PNH, cyangwa `(Paroxysmal Nocturnal Hemoglobinuria)`. Nubwo izina rishobora kumvikana nk'irigoye gato, reka tubisobanukirwe mu buryo bworoshye.

PNH ni iki? Reka tubisobanukirwe mu buryo bworoshye

None se, "Paroxysmal Nocturnal Hemoglobinuria" cyangwa PNH ni iki? Mu by'ukuri ni indwara idakunze kugaragara mu maraso. Iyo urebye icyo izina risobanura:

• "Paroxysmal" bivuze ikintu gitunguranye, kibaho mu buryo butunguranye.
• "Ijoro" bivuze isano n'ijoro.
• "Hemoglobinuria" ni ukuba hari poroteyine yo mu maraso yitwa "Hemoglobin" mu nkari. Iyi "Hemoglobin" ni yo ituma amaraso yacu asa n'umutuku. Rero, iyo ibi bisohowe mu nkari, inkari ziba zijimye.

Mu magambo make, PNH ni indwara aho ubudahangarwa bw'umubiri bwawe bwibasira uturemangingo tw'amaraso dutukura kandi tukanangiza utunyangingo tw'amaraso dutukura . Abaganga babyita "Hemolysis" cyangwa "ivunika ry'utunyangingo tw'amaraso dutukura". Kimwe mu bimenyetso by'ingenzi by'ibi ni inkari zijimye, zitukura/zitukura iyo unyara nijoro cyangwa mu gitondo cya kare.

Ariko, iyi nkari yijimye ni ikimenyetso kimwe gusa cya PNH. Iyo iyi ndwara idavuwe neza, ishobora gutera indwara zikomeye nka kubura amaraso , indwara zidakira z'impyiko , cyangwa kuzura kw'amaraso mu mitsi . Ku bw'amahirwe, ubu hari imiti ikora neza ibuza PNH kwangiza uturemangingo tw'amaraso.

Ni nde ushobora guteza imbere ibi?

PNH ni indwara idakunze kugaragara cyane. Dukurikije imibare, abantu bagera kuri 6 kuri miliyoni 1 basanzwe barwara iyi ndwara buri mwaka. Akenshi, iyi ndwara igaragara ku bagabo n'abagore bari hagati y'imyaka 30 na 40. Ariko kandi, bivugwa ko abagore bafite amahirwe make yo kurwara PNH kurusha abagabo.

Ikindi kintu ni uko rimwe na rimwe abantu bafite izindi ndwara z'umusemburo w'amagufwa, urugero nk'indwara nka `(Aplastic Anemia)` cyangwa `(Myelodysplastic Syndrome)`, bashobora no kurwara PNH nyuma.

Ni irihe tandukaniro riri hagati ya Hemoglobinuria na Hematuria ?

Ukumva urujijo gato iyo wumvise aya magambo abiri ( urujijo)- urujijo, iki ni igitamili, nkwiye kucyirinda. Reka nongere mbisubiremo: Ushobora kujijinganya gato iyo wumvise aya magambo abiri.) Ushobora kujijinganya gato iyo wumvise aya magambo abiri. Kuko muri ibyo byombi, ikibaho ni uko haba hasa n'amaraso mu nkari.

• Hematuria ni ukuba hari uturemangingo dutukura mu nkari zawe.
• Hemoglobinuria (ibi nibyo tubona muri PNH yacu) ni ukuba hari poroteyine yitwa hemoglobin mu nkari. Iyo uturemangingo dutukura tw’amaraso twangiritse, iyi hemoglobin irarekurwa ikongera ikajya mu nkari.

Mu magambo make, imwe ijya mu turemangingo twose, indi ijya mu kintu gitukura gisohoka iyo uturemangingo twangiritse.

Ni iki gitera indwara ya PNH? Kuki ibi bibaho?

Impamvu nyamukuru ya PNH ni inenge iri muri gene mu mubiri wacu. Iyi ntege nke ya gene igira ingaruka ku mikorere y'ubwoko bubiri bw'uturemangingo tw'amaraso, uturemangingo dutukura n'uturemangingo tw'amaraso. Ibi bitangira urukurikirane rw'ibintu biganisha ku bibazo kimwe kimwe, rimwe na rimwe ndetse n'ibintu bishobora gushyira ubuzima mu kaga.

Byose bitangirira mu musokoro wacu. Urabizi, umwokoro w'amagufwa ni nk'uruganda rw'amaraso mu mubiri wacu. Aha niho uturemangingo tw'amaraso turema. Utwo turemangingo tw'amaraso nyuma tukavamo uturemangingo tw'amaraso dutukura, uturemangingo tw'amaraso twera, na plaquettes.

Muri PNH, impinduka cyangwa impinduka muri gene yitwa ``PIGA`` biba mu karemangingo kamwe k’umubiri. Aka karemangingo k’umubiri kahindutse gacikamo ibice kagakora utunyangingo tw’umubiri dufite inenge nk’izo. Utu tunyangingo tw’umubiri dufite inenge hanyuma tugakora uturemangingo tw’amaraso dutukura n’udupira tw’amaraso dufite inenge.

Ni gute iri hinduka ry’uturemangingo rigira ingaruka ku turemangingo dutukura tw’amaraso?

Tekereza kuri ubu buryo: ubudahangarwa bwacu bw'umubiri bumeze nk'ingabo z'igihugu cyacu. Imwe mu mbaraga zayo zidasanzwe yitwa 'Ubudahangarwa bw'umubiri '. Akazi kabo ni ukurwanya mikorobe n'indwara zinjira mu mubiri. Ariko, rimwe na rimwe poroteyine zo muri 'Ubudahangarwa bw'umubiri' zitangira kwibasira uturemangingo tw'amaraso dutukura dufite ubuzima bwiza mu buryo butari bwo.

Uturemangingo dutukura dusanzwe kandi dufite ubuzima bwiza dufite poroteyine imeze nk'igikingirizo kugira ngo ikingire ibitero nk'ibyo. Gene ya `PIGA` twavuze haruguru ifasha mu gukora iyi mashini irinda. Rero, iyo gene ya `PIGA` yahindutse, ntabwo ishobora gukora iyi mashini irinda neza. Noneho, dore uko bigenda:

1. Igitero n'Isenyuka: Iyo "Sisitemu yawe y'Ubwuzuzanye" yibasiye izi selile zitukura zidafite ubwirinzi, izo selile zirasenyuka kandi zigasenyuka byoroshye. "Hemoglobini" yose iri mu selile zitukura - ni ukuvuga poroteyine itukura itwara ogisijeni - irekurwa mu maraso. Abaganga bayita "Hemoglobini y'ubuntu" .

2. Kwangirika kwa sisitemu yo gusukura:Ubusanzwe, imibiri yacu ifite ikintu cyitwa "Haptoglobin" gifata kandi kigakuraho iyi hemoglobine y’ubuntu. Ariko muri PNH, umubare w’uturemangingo dutukura tw’amaraso twangiritse uba uri hejuru cyane ku buryo iri tsinda rishinzwe gusukura ridashobora gukomeza.

3. Kubura Okiside ya Nitrike: Umubiri ukoresha ubundi buryo bwo gucunga iyi hemoglobine irenze urugero, ari yo shimi yitwa nitrike oxide. Ariko na yo irashira vuba. Iyo umubiri wawe udafite nitrike oxide ihagije, ushobora guhita ugira ububabare bukomeye mu mitsi yo mu gifu, mu muhogo (umuyoboro uva mu muhogo ujya mu gifu), no mu mitsi yo mu mugongo.

4. Anemia: Ku ruhande rumwe, iyo uturemangingo dutukura tw’amaraso twangiritse, ku rundi ruhande, hari igitutu kinini ku musokoro w’amagufwa kugira ngo hakorwe uturemangingo dushya dutukura tw’amaraso. Ariko, iyo tudashobora kuremwa ku gipimo kimwe n’uko twangiritse, umubare w’uturemangingo dutukura tw’amaraso mu mubiri uragabanuka. Ibi nibyo twita anemia .

5. Kwangirika kw'impyiko: Iyi hemoglobin irenze urugero inangiza impyiko. Bivugwa ko abantu barwaye PNH bafite ibyago byikubye inshuro esheshatu byo kurwara indwara zidakira z'impyiko .

6. Inkari zijimye: Amaherezo, iyi hemoglobine yigenga inagira ingaruka ku nkari. Abantu benshi barwaye PNH bashobora kubona inkari zijimye iyo binyara nijoro cyangwa mu gitondo cya kare. Ibi biterwa nuko ingano ya hemoglobine mu nkari yiyongera, bigatuma inkari zihinduka umwijima.

Mu magambo make, bitewe n'inenge iri muri gene ya `PIGA`, ubwirinzi bw'uturemangingo dutukura tw'amaraso burabura, kandi bugasenywa n'ubudahangarwa bw'umubiri ubwabwo, bigatera uruhererekane rw'ibibazo.

Ni gute iri hinduka rya gene rigira ingaruka ku miyoboro ya platelets?

Platelets ni ubundi bwoko bw'ingenzi bw'uturemangingo mu maraso yacu. Bifasha guhagarika kuva amaraso binyuze mu gukora ibice by'amaraso. Impinduka mu ngirabuzimafatizo ya PIGA igira ingaruka ku turemangingo fatizo dukora uturemangingo dutukura tw'amaraso ndetse no ku turemangingo fatizo dukora uturemangingo fatizo.

Ibi bituma plaquettes zifite inenge zigira ibice byinshi by'amaraso kurusha uko bisanzwe . Ibi bivuze ko ibice by'amaraso bishobora kubaho ahantu bidakenewe, mu mitsi y'amaraso. Ibi byitwa `(Thrombose)` (thrombose). Ibi ni bibi cyane, kuko iyo ibi bice by'amaraso bigeze mu bwonko, mu mutima, cyangwa mu bihaha, bishobora gushyira ubuzima mu kaga.

Ni ibihe bimenyetso bya PNH?

Nubwo izina PNH rikomoka ku kimenyetso kimwe (inkari zijimye), abantu benshi barwaye PNH babanza kujya kwa muganga kubera umunaniro ukabije kandi uhoraho utuma bigorana gukora imirimo yabo ya buri munsi . Iki ni cyo kimenyetso gikunze kugaragara.

Ibindi bimenyetso bishobora kuba birimo:

• Kugorwa no guhumeka (`(Dyspnea)`)
• Ibibazo by'impyiko (urugero: kugabanuka k'inkari, kubyimba amaguru)
• Kugorwa no kumira ibiryo (Dysphagia)
• Kubabara mu nda
• Kubabara mu gifu
• Kubabara umugongo
• Ubumuga bw'abagabo mu mibonano mpuzabitsina (`(Ubumuga bwo guhagarara k'uburemba)`)

Niba ufite kimwe cyangwa byinshi muri ibi bimenyetso, cyane cyane hamwe n'ihinduka ry'ibara ry'inkari, ni byiza cyane gushaka inama kwa muganga.

Ni gute abaganga basuzuma PNH? (Isuzuma)

Iyo ugiye kwa muganga, bazakubaza ibimenyetso byawe kandi bagusuzume. Hanyuma, niba bakeka ko ufite PNH, bashobora kugukorera ibizamini kugira ngo bemeze ko ari yo.

Iby'ingenzi muri ibyo ni ibi bikurikira:

• Umubare wuzuye w'amaraso (CBC w/difference): Ibi bisuzuma ibindi bibazo by'amaraso, nko kubura amaraso n'umubare muto wa platelet (thrombocytopenia).
• Igice cy'ibanze cya Metabolic Panel (BMP): Iki gice gisuzuma imikorere y'impyiko n'ibimenyetso by'indwara zidakira z'impyiko.
• Isesengura ry'inkari: Ibi bishobora kugaragaza ibintu nko kuba hemoglobin iri mu nkari (Hemoglobinuria) no gushyira icyuma gikabije mu nkari (Hemosiderosis).
• Umubare wa reticulocytes: Reticulocytes ni uturemangingo tw'amaraso dutukura tudakuze. Utwo tubarwa kugira ngo harebwe niba umwongo w'amagufwa urimo gukora uturemangingo tw'amaraso dutukura dufite ubuzima bwiza.
• Ikizamini cya Haptoglobin: Iyi poroteyine ishinzwe gukura imyanda mu turemangingo dutukura tw’amaraso twangiritse. Urugero ruri hasi rushobora kugaragaza ko uturemangingo dutukura tw’amaraso twangiritse cyane.
• Ikizamini cya Lactate Dehydrogenase (LDH): LDH ni enzyme iboneka mu turemangingo dutukura tw'amaraso. Ingano nyinshi y'ibi mu maraso igaragaza ko uturemangingo dutukura tw'amaraso turimo kwangirika.
• Ikizamini cy'imikorere y'umwijima: Iki kizamini gipima urugero rwa bilirubin iyo uturemangingo dutukura tw'amaraso twangiritse.

Hashingiwe ku bisubizo by'ibi bizamini, niba muganga afite ibindi bikeka kuri PNH, ashobora gutegeka ko hakorwa ikizamini cyihariye cy'amaraso cyitwa ``Flow Cytometry.'' Ibi nibyo bishobora kumenya neza uturemangingo dutukura dufite PNH.

Ni ubuhe buryo bwo kuvura PNH?

Mu bihe byashize, PNH yafatwaga nk'indwara iteje akaga cyane kandi igoye kuvura. Muri icyo gihe, uburyo bw'ingenzi bwo kuvura bwari uguterwa amaraso buri gihe kugira ngo havurwe amaraso make. Ariko, abantu barwaye PNH bakundaga kubaho imyaka 10 kugeza kuri 22 nyuma yo kumenyekana. Uburyo bwonyine bwo gukira burundu PNH kwari uguterwa uturemangingo tw’umubiri two mu bwoko bwa allogeneic . Kandi ibyo si ibintu buri wese yakora.

Ariko, ibintu biratandukanye cyane ubu!

Ku bw'amahirwe, ubu hari uburyo bwiza cyane bwo kuvura PNH. Ubu buryo bwitwa inhibitor zuzuzanya.Icyo iyi miti ikora ni ukubuza "Sisitemu y'Ubwuzuzanye" kwibasira no gusenya uturemangingo dutukura tw'amaraso.

Ubushakashatsi bwagaragaje ko abarwayi ba PNH bahabwa ubu buvuzi bushya bashobora kubaho ubuzima busanzwe, kimwe n'umuntu udafite PNH. Iki ni ikintu gikomeye cyane!

Ese hari ingaruka mbi zikomoka ku buvuzi?

Yego, iyi miti (igabanya ubukana bw'ibiyobyabwenge) ishobora kugira ingaruka mbi zimwe na zimwe. Urugero, hari abantu bashobora kugira ibintu nk'umutwe n'isesemi. Ariko, inkuru nziza ni uko ingaruka nyinshi zigabanuka mu minsi mike uhereye igihe batangiriye kuvurwa. Muganga wawe azakubwira byinshi kuri ibi.

Ese indwara ya PNH ishobora kwirindwa? Ese ishobora kuvurwa?

Kubera ko PNH iterwa n'impinduka mu ngirabuzimafatizo, nta buryo bwo kuyirinda.

Ku bijyanye n'ubuvuzi, imiti mishya ya "Complement Inhibitor" ishobora guhagarika kwangirika kw'uturemangingo dutukura tw'amaraso n'udupira duterwa na PNH. Ibi bivuze ko iyi ndwara ishobora kugenzurwa. Abarwayi benshi ba PNH bashobora gukenera ubundi buvuzi ku ndwara nka anemia. Nkuko byavuzwe mbere, nubwo "Stem Cell Transplant" ishoboka, ni uburyo bugoye cyane bwo kuvura. Hamwe n'imiti iriho ubu, iyi ndwara ishobora kwitabwaho neza kandi abantu bakabaho ubuzima busanzwe.

Ni gute wiyitaho nk'umuntu urwaye PNH?

Nubwo wavurwa, ni ingenzi cyane ko ufata ingamba zo kwirinda kubyimba kw'amaraso mu gihe ufite PNH. Urugero:

• Iyo umuntu urwaye PNH agomba kubagwa , hari ibyago byo kubyimba kw'amaraso no kuva amaraso menshi mu gihe cyo kubagwa. Kubwibyo, abaganga babyitondera cyane.
• Gutwita biteza ibibazo ku buzima bw'abagore barwaye PNH, haba kuri bo ndetse no ku mwana uri mu nda. Kubwibyo, ni ngombwa kuba munsi y'ubugenzuzi bw'inzobere mbere yo gutwita no mu gihe cyose cyo gutwita.

Muganga wawe azakugira inama ku mpinduka ugomba gukora mu mibereho yawe, ibintu ugomba kwitondera ( kwitonda , iki ni Sanskrit/Malayalam, ngomba kubyirinda. Reka nongere mbivuge: impinduka ugomba gukora mu mibereho yawe, ibintu ugomba kwitondera.) impinduka ugomba gukora mu mibereho yawe, ibintu ugomba kwitondera. Ni ingenzi cyane gukurikiza iyo nama.

Amaherezo, ikintu cy'ingenzi cyane (Ubutumwa bwo kujyana mu rugo)

PNH, cyangwa "Paroxysmal Nocturnal Hemoglobinuria", ni indwara idakunze kugaragara ariko ishobora kuba ikomeye mu maraso. Mu myaka igera kuri 20 ishize, abarwayi bari bafite uburyo buke bwo kuvurwa kandi nta hazaza heza hazwi.

Ariko muri iki gihe, ibintu biratandukanye rwose! Kubera uburyo bushya bwo kuvura bugezweho, abantu barwaye PNH bashobora kubungabunga uturemangingo tw’amaraso yabo, bakirinda ingorane zikomeye, kandi bakabaho ubuzima bwuzuye kandi buzira umuze.

Niba urwaye PNH, ntugatinye. Muganga wawe azagusobanurira uburyo bwiza bwo kuvura. Nubwo kuvura PNH bidakiza burundu, bishobora kugufasha kwirinda ibibazo bikomeye by'ubuzima biterwa n'iyi ndwara no kugufasha kubaho ubuzima bwiza. Icy'ingenzi ni ukujya kwa muganga vuba bishoboka niba ufite ibimenyetso no gukurikiza uburyo bwo kuvura nk'uko byategetswe.

PNH , Hemoglobinuria yo mu ijoro, Indwara z'amaraso, Hemoglobinuria, Anemia, Kuziba kw'amaraso, Indwara z'impyiko