I-Hemophilia yintlupheko yokuphuma kwegazi . Umntu one-hemophilia ulahleka umbandela wegazi kufuneka aphephe igazi elidala ukuphuma kwegazi.
Iimpawu
Ngezinye izihlandlo abantu abane-hemophilia babizwa ngokuthi "i-bleeders ezamahala" kuthetha ukuba baphuma ngokulula. Ngokuxhomekeka kubunzima, ukuphuma kwamanzi kungenzeka ngokukhawuleza (ngaphandle kokulimala) okanye emva kokuhlinzwa okanye ukuxhatshazwa.
Iimpawu ziquka:
- Iipumphaza ezide
- Ukuhlanjululwa kwizinsini
- Imivalo emikhulu
- Ukuphuma ngexesha elide emva kokuhlinzwa okanye ukukhwela, kuquka ukusoka
- Ukuvuvukala kwamalungu amaninzi (amahlombe, amajolo, amadolo, amaqaqa) ukusuka ekuphumeni kwegazi
- Ubuhlungu obuphuma ekuphumeni kwi-joint okanye imisipha
Ngubani Osemngciphekweni?
Amadoda azalwe kwiintsapho ezinembali ye-hemophilia kwezinye izalamane zisengozini. Ukuqonda ifa le-hemophilia, kufuneka sikhulume i-genetics encinane. Abesilisa bane-X ye-chromosome evela kumama wabo kunye ne-Y chromosome evela kubaba. Abafazi badla i-X ye-chromosome evela kubo bobabini nonina. Isiphako se-hemophilia sitholakala kwi-X ye-chromosome, oomama abathintekayo (abathwali be-disorder) badlulisela le mpahla yemfuza kubantwana babo; eli libizwa ngokuba yi-X edibeneyo. Ngenxa yokuba zine-X ze-chromosomes, iintombi azichaphazelekayo (kodwa zinokuba ziimeko ezingaqhelekanga).
Ukuxilongwa
I-Hemophilia icingelwa xa inkwenkwe okanye umntu egazini ebonakala enzima.
Kufunyaniswa ngokulinganisa iimeko ze-coagulation (iiprotheni ezifunekayo ukuvala igazi).
Ugqirha wakho uya kuqala ngeemvavanyo zelabhoratri ukuvavanya yonke inkqubo ye-coagulation. Ezi zibizwa ngokuba yixesha le-prothrombin (PT) kunye nexesha elithile le-thromboplastin (PTT). Kwi-hemophilia, i-PTT ide.
Ukuba i-PTT iyancipha (ngaphezu kwoluhlu oluqhelekileyo), ukusilela kwe-coagulation ingaba yeso sizathu. Emva koko ugqirha wakho uya kulandelelanisa iziganeko zokuquba (iiprotheyini egazini eziyeka ukuphuma kumzi) 8, 9, no-11. Ngaphandle kokuba kukho imbali yentsapho ecacileyo, ngokuqhelekileyo zonke izinto ezi-3 zivivinywa ngexesha elifanayo. Olu vavanyo lunika ugqirha wakho ipesenti yomsebenzi wento nganye, ephantsi kwe-hemophilia. Ukuxilongwa kungaqinisekiswa ngovavanyo lwemizimba.
Iintlobo
I-Hemophilia inokuhlatyathwa yi-coagulation into engekhoyo.
- I-Hemophilia Iziphumo ezivela ekusileleni kwinqanaba 8.
- I-Hemophilia B (ebizwa nangokuthi isifo seKrisimesi) kubangelwa ukusilela kwinqanaba 9.
- I-Hemophilia C (ebizwa nangokuthi i-Rosenthal syndrome) ibangelwa kukusilela kwinqanaba le-11.
I-Hemophilia ikwahlulwa kwakhona ngumlinganiselo we-coagulation factor found. Ingxaki encinci ye-coagulation enayo, mhlawumbi uya kuphuma igazi.
- Ubumnene: 6 - 40%
- Elingqinelanayo: 1-5%
- Ikhulu: <1%
Unyango
I-Hemophilia iphathwa nge-concentrates. Ezi zinto zijoliswe kwi-vein (IV). I-Hemophilia inokuphathwa ngeendlela ezimbini ezahlukeneyo: ngokufunwa kuphela (xa ukuphuma kwamagqabha ephuma) okanye iprophylaxis (ukufumana into enye, kabini, okanye kathathu ngeveki ukukhusela iziphazamiso zokuphuma kwegazi).
Indlela ophathwa ngayo iyanqunywa ngamanyathelo amaninzi kubandakanya ubunzima be-hemophilia yakho. Ngokuqhelekileyo, abantu abane-hemophilia emninzi banokuphinda baphathwe ngokufunwa njengoko bancipha kakhulu. Ngenhlanhla, uninzi lwamayeza e-hemophilia lulawulwa ekhaya. Abazali banokufunda indlela yokulawula abantwana babo ngokusebenzisa imfumba ekhaya okanye umhlengikazi wekhaya unokulawula loo nto. Abantwana abane-hemophilia nabo banokufunda indlela yokulawula i-concentrating focus to themselves, ngokuqhelekileyo ngaphambi kokuba babe ngabaselula.
Nangona oko kugxininiswa kunyango olukhethiweyo, olu unyango alufumaneki kuwo onke amazwe.
I-Hemophilia nayo ingaphathwa ngeemveliso zegazi. I-Factor 8 iyakwazi ukuphathwa nge-cryoprecipitate (i-plasma ifomu). I-plasma entsha efakwe efrijini ingasetyenziselwa ukuphatha i-factor 8 ne-factory 9.
Kwizigulane ezichaphazelekileyo, unyango olubizwa ngokuba yi-desmopressin acetate (i-DDAVP) lunokulawulwa nge-vein okanye i-nasal spray. Ivuselela umzimba ukuba ukhulule izitolo zezinto ezi-8 ukukunceda ekupheleni kwegazi.