Awunankinga yini nawe yokuyeka ukopha? Ake sifunde nge-hemophilia!

Wake wezwa ngesifo esibizwa ngokuthi i-Hemophilia ? Ungase uzizwe wesaba kancane uma uzwa leli gama. Kodwa ungakhathazeki, ngoba lesi yisifo esingavamile kakhulu. Namuhla, sizoxoxa ngokuthi iyini i-hemophilia, ukuthi ikhula kanjani, yiziphi izimpawu, nokuthi ikhona yini indlela yokwelapha, konke ngendlela elula ongayiqonda.

Kuyini i-hemophilia?

Kalula nje, i-hemophilia isifo segazi esidluliselwa ngofuzo esenzeka ezinganeni. Lokhu kusho ukuthi igazi lakho alijiki kahle. Lokhu kungabangela ukopha ngokweqile, noma ngisho nokuminyana okuncane.

Cabanga ngakho, kunezinhlobo ezikhethekile zamaprotheni emizimbeni yethu ezisiza ekuqhekekeni kwegazi. Lezi sizibiza ngokuthi 'izici zokugoba' . Zifana namasosha amancane asebenza ukuvimba ukopha. Uma uba ne-hemophilia, umzimba awukhiqizi ngokwanele enye noma ngaphezulu yalezi 'zici zokugoba'. Bese igazi ligoba kancane bese wopha kakhulu.

Kunezinhlobo eziningana eziyinhloko ze-hemophilia. Lesi simo singaba sibi kakhulu, siphakathi nendawo, noma sibe sincane, kuye ngokuthi zingakanani izinto ezibangela ukujiya kwegazi egazini lakho.

Izindaba ezinhle ukuthi kukhona ukwelashwa kwalokhu. Odokotela bazama ukubuyisela izinto ezibangela ukujiya kwegazi emzimbeni. Okwamanje akukho ukwelashwa kwe-hemophilia. Kodwa-ke, abantu abanalesi simo bavame ukuphila isikhathi eside njengabanye ngokwelashwa okufanele. Ososayensi bacwaninga nezindlela ezintsha, njengokwelashwa ngezakhi zofuzo , ukuze babone ukuthi kungelapheka yini.

Ingabe i-hemophilia ingakhula ngemva kokuzalwa?

Yebo, kungenzeka. Kodwa akuvamile kakhulu. Lokhu kubizwa ngokuthi 'i-acquired hemophilia'. Lokho kusho ukuthi ayizuzwa njengefa. Okwenzekayo lapha ukuthi amaprotheni okuzivikela emizimbeni yethu , abizwa ngokuthi ama-antibodies , ngezinye izikhathi ahlasela ngephutha enye yezinto zethu zokujiya kwegazi. Lawa ma-antibodies ahlasela ngendlela engafanele abizwa ngokuthi 'ama-autoantibodies' .

Ingabe i-hemophilia iyisifo esivamile?

Cha, akunjalo nhlobo. Lesi yisifo esingavamile kakhulu. Ngokwezibalo zase-United States (CDC Agasti 2022), abantu ababalelwa ku-33,000 lapho bane-hemophilia. Ivame ukuthinta amadoda. Akuvamile ukuthi abesifazane babe nezimpawu ezifana namazinga aphansi okujiya kwegazi kanye nokopha kakhulu ngesikhathi sokuya esikhathini.

Yiziphi izinhlobo ze-hemophilia?

Kunezinhlobo ezintathu eziyinhloko ze-hemophilia:

• I-Hemophilia A: Lolu uhlobo oluvame kakhulu. Lubangelwa ukuntuleka kwe-blood clotting factor number 8, eyaziwa nangokuthi i-factor VIII., lapho umzimba ungakutholi ngokwanele. Cishe abantu abayi-10 kwabayi-100,000 bangaba nalokhu.
• I-Hemophilia B: Lokhu kubangelwa ukuntuleka kwe-blood clotting factor 9, noma i-factor IX . Cishe abantu abathathu kwabayi-100,000 banalolu hlobo.
• I-Hemophilia C: Ebizwa nangokuthi ukuntuleka kwe-factor XI , lokhu akuvamile kakhulu, kuthinta cishe umuntu oyedwa kwabayisigidi.

Ingabe i-hemophilia iyisifo esibi kakhulu?

Yebo, ngezinye izikhathi lokhu kungaba yingozi. Abantu abane-hemophilia enzima bangopha igazi ngendlela esongela ukuphila, esiyibiza ngokuthi 'ukopha igazi.' Kodwa isikhathi esiningi baphuma igazi emisipheni noma emalungeni.

Ziyini izimpawu ze-hemophilia?

Izimpawu ezibaluleke kakhulu ukopha okungavamile noma ukushaqeka ngokweqile.

Izimpawu ezivame ukubonakala

Abantu abane-hemophilia bangaba nemivimbo emikhulu ngisho nangezingozi ezincane. Lokhu kusho ukuthi igazi liyavuza ngaphansi kwesikhumba.

• Mhlawumbe ngemva kokuhlinzwa, ngemva kokukhishwa kwezinyo, noma ngisho nokusikwa nje komunwe , ukopha kungahlala isikhathi eside ngendlela engavamile.
• Ukopha ekhaleni kungavela ngokuzumayo ngaphandle kwesizathu.

Ukuthi unemivimbo/ukopha okungakanani kuncike ekutheni une-hemophilia embi, ephakathi nendawo, noma encane.

• Abantu abane -hemophilia enzima bangase bophe njalo ngaphandle kwesizathu esibonakalayo.
• Abantu abane -hemophilia ephakathi bangase bophe isikhathi eside ngokungavamile uma besengozini enkulu.
• Abantu abane -hemophilia encane bathola ukopha okungavamile kuphela ngemva kokuhlinzwa okukhulu noma ingozi enkulu.

Kungase kube nezinye izimpawu:

• Ubuhlungu bamalunga obubangelwa ukugeleza kwegazi emzimbeni. Izindawo ezinjengamaqakala, amadolo, izinqe, namahlombe zingase zibe buhlungu, zivuvuke, noma zizwe zishisa uma uzithinta.
• Ukopha ebuchosheni. Lokhu kuyinto engavamile kakhulu kubantu abane-hemophilia enzima. Uma unokopha ebuchosheni, ungase ube nekhanda elibuhlungu eliqhubekayo, umbono ofiphele, noma ukozela okukhulu . Uma une-hemophilia, kufanele ubone udokotela ngokushesha uma uhlangabezana nanoma yiziphi zalezi zimpawu.

Izimpawu ezinganeni nasezinganeni ezincane

Ngezinye izikhathi, i-hemophilia itholakala lapho ingane yomfana isokwa futhi yopha igazi ngaphezu kokuvamile. Ngezinye izikhathi, izingane zibonisa izimpawu ezinyangeni ezimbalwa ngemva kokuzalwa. Izimpawu ezivame kakhulu yilezi:

• Ukopha:Izingane zingaphuma igazi emlonyeni wazo uma ziluma ithoyizi elincane.
• Amaqhubu avuvukele ekhanda: Izinsana nezingane ezincane zingase zibe namaqhubu amakhulu, ayindilinga (amaqanda e-goose) uma zishaya amakhanda azo.
• Ukukhala njalo, ukungahlaliseki, kanye nokungathandi ukukhasa noma ukuhamba: Lokhu kungenzeka uma igazi lingene emisipheni noma emalungeni. Indawo ingase izwe imihuzuko, ivuvukele, ishise uma uyithinta, noma ingane ingase izwe ubuhlungu ngisho noma ithintwa kancane.
• Ama-Hematoma: I-'hematoma' iqoqo lamahlule egazi aqoqana ngaphansi kwesikhumba ezinganeni nasezinganeni ezincane. Lezi zinhlobo zama-hematoma zingavela futhi ngemva komjovo.

Ziyini izimbangela ze-hemophilia?

Lezi 'zici zokujiya' zenziwa ngezakhi zofuzo ezithile emzimbeni wethu. Ku-hemophilia ezuzwe njengefa, izakhi zofuzo ezifundisa ukukhiqizwa kwalezi 'zici zokujiya' ziyashintshashintsha, okusho ukuthi ziyashintsha. Lezi zakhi zofuzo eziguquliwe zingenza 'izici zokujiya' ezingalungile noma zingakhi 'izici zokujiya' ezanele. Kodwa-ke, cishe ama-20% eziguli ze-hemophilia azenzekela, okusho ukuthi angasithola lesi sifo ngisho noma kungekho muntu emndenini oke waba naso ngaphambili.

I-hemophilia izuzwa kanjani njengefa?

Zombili izinhlobo ze-hemophilia A kanye ne-B ziyizifo ezihlobene nocansi. Zizuzwa njengefa ngendlela ehlobene nocansi ehlobene no-X. Ake sibone ukuthi lokho kwenzeka kanjani:

• Sonke sithola isethi eyodwa yama-chromosome kumama wethu kanye nesethi eyodwa yama-chromosome kubaba wethu. Uma uthola i-chromosome X kumama wakho kanye ne-chromosome X kubaba wakho, uyintombazane. Uma uthola i-chromosome X kumama wakho kanye ne-chromosome Y kubaba wakho, ungumfana. Kalula nje, umama uhlala enika ingane yakhe i-chromosome X. Ubulili bakho bunqunywa ukuthi ubaba ukunika i-chromosome X noma Y.
• Uma owesifazane enesici esakhiweni sezakhi zofuzo esenza lesi 'sici sokugoba' kwelinye lama-chromosome akhe amabili e-X, angase abe ngumthwali we- hemophilia, kodwa angase angakhombisi izimpawu ngoba unezakhi zofuzo ezinempilo kwenye i-chromosome yakhe ye-X.
• Uma umama othwala i-chromosome X enephutha njengalena enengane yesilisa, kunethuba elingu-50% lokuthi ingane izozuza i-chromosome X enephutha. Uma lokho kwenzeka, ingane yesilisa izoba ne-hemophilia.
• Uma lowo mama enendodakazi, kunethuba elingu-50% lokuthi ingane izozuza ifa le-chromosome X enephutha. Kodwa-ke, angase angakhombisi izimpawu ngoba izuza i-chromosome X enempilo kuyise. Khona-ke leyo ndodakazi nayo iyoba ngumthwali.

Lokho kusho ukuthi owesifazane ozuza i-chromosome X enephutha uba ngumthwali we-hemophilia. Angase angabi nazimpawu, kodwa angadlulisela lesi sifo ezinganeni zakhe. Ingane ngayinye anayo inethuba elingu-50% lokuthola i-hemophilia njengefa.Izingane zesilisa zinamathuba amaningi okubonisa izimpawu uma zithola i-hemophilia njengefa, ngoba azitholi i-chromosome X enempilo kuyise.

Ingabe amantombazane nawo athola izimpawu ze-hemophilia?

Yebo, kungenzeka. Kodwa izimpawu zivame ukuba zincane. Isibonelo, owesifazane ophethe i-hemophilia gene angase angabi nezinto ezanele zokujiya kwegazi ezivamile. Uma lokhu kwenzeka, angase athole ukopha okukhulu kakhulu ngesikhathi sokuya esikhathini, akhubeke kalula, ophe kakhulu ngemva kokubeletha, noma ophe emalungeni akhe, okubangela izinkinga emalungeni.

Odokotela bakuthola kanjani ukuthi une-hemophilia?

Udokotela uzoqala ngokukubuza umlando ophelele wezokwelapha bese ekwenza ukuhlolwa ngokomzimba. Uma unezimpawu ze-hemophilia, uzophinde akubuze ngomlando womndeni wakho. Bangenza izivivinyo ezifana nalezi:

• Ukubalwa Kwegazi Okuphelele (i-CBC): Lokhu kwenzelwa ukubheka izinhlobo zamaseli egazini.
• Ukuhlolwa Kwesikhathi Se-Prothrombin (PT): Lokhu kungahlola ukuthi igazi lakho liqina ngokushesha kangakanani.
• Ukuhlolwa Kwesikhathi Se-Thromboplastin Okuyingxenye (PTT): Lokhu okunye ukuhlolwa okulinganisa isikhathi esithathwayo ukuze igazi lijiye.
• Ukuhlolwa kwe-Specific clotting factor(s): Lokhu kuhlolwa kwegazi kulinganisa amazinga e-specific clotting factor, njenge -factor VIII kanye ne-factor IX .

Ziyini lezi zici zokwakheka kwamahlule?

Izinto ezivimba igazi yizinto ezisiza ukulawula ukuphuma kwegazi. Odokotela bahlukanisa i-hemophilia njengethambile , ephakathi nendawo, noma enzima ngokusekelwe enanini lalezi zinto ezivimba igazi egazini lakho:

• Abantu abanezici zokujiya kwegazi eziphakathi kuka-5% no-30% wamazinga ajwayelekile bane- hemophilia encane.
• Abantu abanezici zokujiya kwegazi eziphakathi kuka-1% no-5% wamazinga ajwayelekile bane -hemophilia ephakathi.
• Abantu abanezici zokujiya ezingaphansi kwe-1% ezivamile bane -hemophilia enzima.

Yiziphi izindlela zokwelapha i-hemophilia?

Odokotela belapha i-hemophilia ngokwandisa amazinga ezici zokujiya kwegazi eziphansi noma ngokushintsha izici zokujiya kwegazi ezingekho. Lokhu kubizwa ngokuthi ukwelashwa kokushintshana .

Kulolu hlobo lokwelashwa kokushintsha, unikezwa izinto ezibangela ukujiya kwegazi ezenziwe nge-plasma yomuntu (ama-plasma agxilile) noma 'izici ezibangela ukujiya kwegazi' eziphinde zahlanganiswa . Ngokuvamile, abantu abane-hemophilia enzima kuphela abadinga lolu hlobo lokwelashwa kokushintsha igazi njalo. Abantu abane-hemophilia encane kuya kwephakathi banikezwa lolu hlobo lokwelashwa uma sebezohlinzwa. Banikezwa imithi yokwelapha i-antifibrinolytics.Imithi evimba ukuqhekeka kwegazi inganikezwa.

Lezi zinto ezigxilile egazini zenziwa ngegazi lomuntu elinikelwe. Ziyahlanzwa futhi zihlolwe ukuze kuncishiswe ingozi yokudlulisela izifo ezithathelwanayo njenge -hepatitis kanye ne-HIV . Lezi zinto ezithathelwanayo zinikezwa njengokufakwa kwe-intravenous (IV) .

Uma une-hemophilia enzima futhi uphuma igazi njalo, udokotela wakho angase akunikeze ukwelashwa okubizwa ngokuthi 'i-prophylactic factor infusions' ukuze kuvinjelwe ukuphuma kwegazi.

Ingabe kukhona izinkinga ekwelashweni?

Abanye abantu abathola lokhu kwelashwa kokufaka esikhundleni bakha ama-antibodies , abizwa ngokuthi ama-inhibitor , ahlasela izinto ezibangela ukujiya kwegazi abanikezwa zona. Odokotela basebenzisa inqubo ebizwa ngokuthi i-Immune Tolerance Induction (ITI) ukwelapha lokhu. I-ITI ihilela ukubanika imijovo yansuku zonke yezinto ezibangela ukujiya kwegazi ukuze kwehliswe amazinga ezithibela igazi. Lokhu kungaba ukwelashwa kwesikhathi eside, futhi abanye abantu bangase badinge lokhu kwelashwa izinyanga noma ngisho neminyaka.

Ingabe i-hemophilia ingavinjelwa?

Cha, akunakwenzeka. Uma une-hemophilia futhi unezingane, udokotela wakho angancoma ukuhlolwa kofuzo . Ngaleyo ndlela, wena nezingane zakho ningathola ukuthi kungenzeka yini ukuthi zidlulisele i-hemophilia esizukulwaneni esilandelayo.

Luhlobo luni lwethemba okufanele umuntu one-hemophilia abe nalo?

Uma une-hemophilia, ungase udinge ukwelashwa impilo yakho yonke. Ukuthi udinga ukwelashwa okungakanani kuncike ohlotsheni lwe-hemophilia onayo, ubukhali bayo, nokuthi ngabe uba 'nama-inhibitor' noma cha.

Ingakanani iminyaka yokuphila yomuntu one-hemophilia?

Ngokusho kwe-World Federation of Hemophilia, isikhathi sokuphila sendoda ene-hemophilia sifushane ngeminyaka eyi-10 kunesomuntu ongenayo i-hemophilia, ngokusho kwedatha ka-2012 evela kwi-World Federation of Hemophilia. Kodwa-ke, bathi izingane ezine-hemophilia ezixilongwa futhi zelashwe zisencane zinesikhathi sokuphila esijwayelekile .

Kodwa akubona bonke abantu abafanayo. Okuyiqiniso komunye umuntu kungase kungabi iqiniso komunye. Uma wena noma ingane yakho ine-hemophilia, buza udokotela wakho ukuthi yini ongayilindela. Uyazi kangcono isimo sakho/sengane yakho kanye nempilo yayo yonke.

Ngizinakekela kanjani?

Abantu abane-hemophilia bangadinga ukunakekelwa okuqhubekayo kwezokwelapha ukuze bavimbele ukuphuma kwegazi. Kungase kudingeke bayeke eminye imisebenzi kanye nemithi. Kodwa-ke, kunezinto eziningi ongazenza ukuze ulawule umthelela we-hemophilia empilweni yakho.

Izinto okungafanele zenziwe

Izinto okulula ukuthi abanye bazishaye, bawe, noma bakhubeke zingadala izinkinga ezinkulu kubantu abane-hemophilia. Kufanele bagweme imisebenzi ebabeka engcupheni enkulu yokuwa noma ukushaywa kanzima. Izibonelo:

• Ukudlala imidlalo efana nebhola, i-hockey, kanye ne-rugby.
• Ukuhlanganyela ezintweni ezifana nesibhakela kanye nokulwa.
• Ukugibela isithuthuthu.
• Ukuskeyitha.

Eminye imidlalo, njengebhola likanobhutshuzwayo, i-basketball, kanye ne-baseball, nayo inengozi enkulu yokulimala. Uma ufuna ukudlala le midlalo, khuluma nodokotela wakho ngezinto ezifana nokusebenzisa izinto zokuzivikela.

Imithi engalungile ukuyithatha

Iziqeda-buhlungu ezifana ne-aspirin, i-ibuprofen, ne-naproxen zivimbela ukujiya kwegazi. Futhi, ukuthatha izidakamizwa zokulwa nokuvuvukala kwegazi ezifana ne-heparin noma i-warfarin akuwona umqondo omuhle.

Izinto ongazenza ukuze uphile impilo engcono

Kunezinto eziningi ongazenza ukuze unciphise umthelela we-hemophilia empilweni yakho:

• Qala inqubo yokuzivocavoca: Ungase ukhathazeke ngokulimala ngenkathi uvivinya umzimba. Kodwa-ke, khuluma nodokotela wakho ngezindlela zokuhlala umatasa ngenkathi unciphisa ingozi yokopha.
• Lawula ukucindezeleka : I-Hemophilia yisimo esihlala isikhathi eside, futhi kungadingeka wenze umzamo owengeziwe wokusilinganisela nemithwalo yemfanelo yomndeni wakho kanye nomsebenzi.
• Gcina impilo enhle yamazinyo: Ukuxubha, ukuxubha nge-floss, nokubona udokotela wamazinyo njalo kunganciphisa ingozi yokudinga ukwelashwa kwamazinyo okungabangela ukopha. Ungakhohlwa ukutshela udokotela wakho ngesimo sakho.
• Gcina isisindo esinempilo: Uma unenkinga yokuhambahamba ngenxa yokopha kwangaphakathi kanye nomonakalo wamalunga, ukulawula isisindo sakho kuzosiza.
• Yazisa labo abaseduze nawe: Uma une-hemophilia enzima, ungase ube nezigameko zokopha ezingazelelwe, noma ngabe uphuza imithi. Yazisa umndeni wakho ngalokho okufanele ukwenze uma lokhu kwenzeka kuwe. Uma ingane yakho ine-hemophilia, yazisa abanakekeli bayo kanye nabasebenzi besikole ngalokho okufanele bakwenze uma ingane yakho yopha.

Kufanele ngimbone nini udokotela?

Uma ubona noma yiziphi izinguquko emzimbeni wakho, njengokuphuma kwegazi ngokweqile noma ukuklwebheka, bona udokotela.

Nini ukuya eGumbini Eliphuthumayo

Kufanele uye ekamelweni lezimo eziphuthumayo ngokushesha uma ubhekene nalezi zimo:

• Uma uphethwe yikhanda elibuhlungu noma unesizungu, lezi zimpawu zingase zibonise ukuthi uphuma igazi ebuchosheni bakho.
• Uma unamajoyinti avuvukile kanye/noma abuhlungu, futhi ungenayo imithi yokufaka esikhundleni se-factor.

Yimiphi imibuzo okufanele ngiyibuze udokotela?

Uma wena noma ingane yakho nitholakala ninesifo se-hemophilia, ungase ufune ukubuza udokotela wakho imibuzo efana nale:

• Uhlobo luni lwe-hemophilia enginalo/ingane yami?
• Yikuphi ukwelashwa okuncomayo?
• Iyini imiphumela emibi yokwelashwa?
• Ingabe mina/ingane yami kuzodingeka ngithole ukwelashwa nsuku zonke?
• Yimiphi imisebenzi engalungile kimi/enganeni yami ukuyenza?
• Yimiphi imithi engalungile ukuthi mina/ingane yami ngiyiphuze?
• Yini engingayenza ukuze ngilawule umthelela lesi simo esinawo empilweni yami/yengane yami?

Umlayezo Wokuya Nawe Ekhaya

I-Hemophilia iyisifo segazi esingavamile, esizuzwe njengefa. Singaba nomthelela omkhulu empilweni yakho. Abantu abane-hemophilia bangadinga ukunakekelwa kwezokwelapha impilo yonke. Abazali bezingane ezine-hemophilia bangase bakhathazeke ngokuvikela izingane zabo ekulimaleni, kanye nokuzifundisa ukuphila ne-hemophilia.

Njengamanje, odokotela abakwazi ukwelapha i-hemophilia ngokuphelele. Kodwa-ke, bangakunikeza ukwelashwa ukuvimbela noma ukunciphisa izimpawu ze-hemophilia. Bangancoma nezinyathelo ongazithatha ukuze unciphise umthelela we-hemophilia empilweni yakho yansuku zonke. Into ebaluleke kakhulu ukuhlala uzolile futhi ulandele imiyalelo kadokotela wakho ngokucophelela.