I-Hemophilia yi- disorder ephuma ngegazi . Umuntu onesifo se-hemophilia esweleka isidingo segazi esidinga ukumboza igazi eliholela ekuphumeni okwedlulele ngokweqile.
Izimpawu
Ngezinye izikhathi abantu abane-hemophilia babizwa ngokuthi "ama-bleeders mahhala" okusho ukuthi bavutha kalula. Kuye ngenxa yobunzima, ukuphuma kwamanzi kungenzeka ngaphandle (ngaphandle kokulimala) noma ngemuva kokuhlinzwa noma ukulimala.
Izimpawu zihlanganisa:
- Izinkinga ze-noseble eside
- Ukuhlwitha kusuka ezinsini
- Izibhamu ezinkulu
- Ukusuka isikhathi eside ngemuva kokuhlinzwa noma ukudubula, kubandakanya ukusoka
- Ukuvuvukala kwamajoyina amakhulu (amahlombe, ama-elbows, amadolo, amaqaqa) kusukela ekuphumeni kwegazi ukuya ekuhlanganyeleni
- Ubuhlungu obuphuma ekuphumeni kwegazi kumjoqo
Ubani Osesengozini?
Amadoda azalwa emindeni enomlando we-hemophilia kwezinye izihlobo ayengozini. Ukuze siqonde ifa le-hemophilia, sidinga ukukhuluma i-genetics encane. Abesilisa bane-X chromosome evela kumama wabo ne-Y chromosome evela kubaba wabo. Abesifazane badla i-X chromosome kusuka kokubili uyise nomama. Isici se-hemophilia sitholakala kwi-X chromosome, okusho ukuthi omama (abathwali be-disorder) badlulisela lesi sipho esingokomzimba kumadodana abo; lokhu kubizwa ngefa elixhunywe nge-X. Ngenxa yokuthi banama-chromosomes amabili amabili, amadodakazi ngokuvamile awanakathinteka (kodwa angaba ngaphansi kwezimo ezingavamile).
Ukuxilongwa
I-Hemophilia iyasolakala lapho umfana noma umuntu egazini ebonakala ekhululekile.
Kutholakale ngokulinganisa izici ze-coagulation (amaprotheni ayedinga ukumboza igazi).
Udokotela wakho uzoqala ngezivivinyo zokuhlola ukuhlola lonke uhlelo lwe-coagulation. Lezi zibizwa ngokuthi i-prothrombin time (PT) nesikhathi esithile se-thromboplastin (PTT). E-hemophilia, i-PTT inwetshwa isikhathi eside.
Uma i-PTT ihlala isikhathi eside (ngaphezu kwebanga elijwayelekile), ukuntuleka kwe-coagulation factor kungaba isizathu. Khona-ke udokotela wakho uzohlehlisa izici zokugcoba (amaprotheni egazini ayeka ukuphuma) 8, 9, no-11. Ngaphandle uma kukhona umlando womndeni ocacile, ngokuvamile zonke izici ezintathu zivivinywa ngesikhathi esifanayo. Lezi zivivinyo zinika udokotela wakho amaphesenti omsebenzi wesici ngasinye, esincane e-hemophilia. Ukuxilongwa kungaqinisekiswa ngokuhlolwa kofuzo.
Izinhlobo
I-Hemophilia ingahle ibekwe yi-coagulation ethize engekho.
- I-Hemophilia Imiphumela ngenxa yokuntuleka kwe-factor 8.
- I-Hemophilia B (ebizwa nangokuthi isifo sikaKhisimusi) kubangelwa ukuntuleka kwesigaba 9.
- I-Hemophilia C (ebizwa nangokuthi i-Rosenthal syndrome) imiphumela ngenxa yokuntuleka kwe-factor 11.
I-Hemophilia ingabuye ibekwe yi-coagulation factor etholakalayo. I-factor coagulation encane onakho, cishe kufanele uphephe.
- Ubumnene: 6 - 40%
- Okulinganiselwe: 1-5%
- Okukhulu: <1%
Ukwelapha
I-Hemophilia iphathwa ngokugxila kwezinto. Lokhu kugxila kwezinto kuhlanganiswa nge-vein (IV). I-Hemophilia ingaphathwa ngezindlela ezimbili ezihlukene: ngokufunwa kuphela (lapho ukuphuma kwamaphasethi kwenzeka) noma i-prophylaxis (ukuthola isisodwa kanye, kabili, noma kathathu ngesonto ukuvimbela iziqephu zegazi).
Indlela ophathwa ngayo inqunywe yizinto eziningi ezibandakanya ukuqina kwe-hemophilia yakho. Ngokuvamile, abantu abane-hemophilia emnene banamathuba amaningi okuphathwa ngezinga elidingekayo ngoba banesifo esincane kakhulu. Ngenhlanhla, iningi lama-hemophilia zokwelapha lenziwa ekhaya. Abazali bangafunda indlela yokuphatha izingane zabo nge-vein ekhaya noma umhlengikazi wekhaya angakwazi ukulawula lesi sici. Izingane ezine-hemophilia nazo zingafunda indlela yokuphatha izinto ezigxila kuzo, ngokuvamile ngaphambi kokuba zibe yintsha.
Nakuba i-factor igxile ukwelashwa okhethwayo, le nqubo ayitholakali kuwo wonke amazwe.
I-hemophilia nayo ingaphathwa ngemikhiqizo yegazi. Ukwehluleka kwe-Factor 8 kungaphathwa nge-cryoprecipitate (i-plasma). I-plasma entsha efriziwe ingasetshenziselwa ukwelapha isici 8 kanye nokuntuleka kwemboni 9.
Ngeziguli ezithintekayo kahle, imithi ebizwa ngokuthi i-desmopressin acetate (i-DDAVP) ingalawulwa nge-vein noma spal spray. Ikhuthaza umzimba ukuba ukhulule izitolo ze-factor 8 ukuze usize ekupheleni kwegazi.