Ngaba wakha waziva ngathi wena okanye umntu kusapho lwakho uphuma igazi ngendlela engalawulekiyo xa unesilonda esincinci? Okanye ngaba ufumana amabala amakhulu aluhlaza emzimbeni wakho nasemva kokwenzakala okuncinci? Musa ukuzithatha njengeziqhelekileyo ezi zinto. Kuba, oku kungaba luphawu lwesifo esibizwa ngokuba yi-hemophilia . Ungakhathazeki, siza kuthetha ngayo yonke into ngokulula namhlanje.
Ngamafutshane, yintoni i-hemophilia?
I-Hemophilia yimeko yemfuza echaphazela indlela amahlwili egazi ethu aphuma ngayo. Ngokwesiqhelo, xa senzakele, ukopha kuyayeka emva kwexesha elithile. Oku kungenxa yokuba iiproteni ezikhethekileyo egazini lethu (ezibizwa ngokuba zizinto ezibangela ukuqhekeka kwegazi ) zisebenza kunye ukwenza ihlwili legazi. Nangona kunjalo, abantu abane-hemophilia abanayo enye yezi proteni ezinceda ihlwili legazi. Yiyo loo nto ukopha kungayeki lula xa senzakele.
Esi asisosifo esosulelayo. Sisifo semfuza, oko kuthetha ukuba sidluliselwa kwizizukulwana ngezizukulwana .
Kukho iintlobo ezimbini eziphambili ze-hemophilia.
| Uhlobo lwe-hemophilia | Inkcazo |
|---|---|
| I-Hemophilia A | Olu lolona hlobo luxhaphakileyo. Malunga nama-80% ezigulane ezine-hemophilia zinalolu hlobo. Lubangelwa kukunqongophala kweproteni iFactor VIII, efunekayo ukuze igazi lijiye. Ubunzima besifo buxhomekeke kwinqanaba apho le nto incitshiswa khona. |
| I-Hemophilia B | Oku kunqabile kancinci. Malunga nama-20% ezigulana zilolu hlobo. Oku kubangelwa kukunqongophala kweFactor IX, efunekayo ukuze igazi lijiye.Oku kungaba ncinci, phakathi, okanye kube nzima kuxhomekeke kubungakanani bezinto. |
Ukongeza, kukho uhlobo olungaqhelekanga kakhulu olubizwa ngokuba yi-hemophilia C. Lubangelwa kukungabikho kweFactor XI.
Zithini iimpawu ze-hemophilia?
Iimpawu ziyahluka ngokuxhomekeke kubukhali bemeko. Ngamanye amaxesha ukopha kunokubakho ngaphandle kwesizathu esicacileyo. Ezi mpawu zinokubonakala lula, ingakumbi kubantwana abancinci .
Iimpawu ezibonakalayo kubantwana abancinci nakwiintsana:
- Ukopha entloko xa ezalwa: Abanye abantwana basenokuba nokopha entloko xa bezalwa.
- Ukudumba kwamalungu xa uqala ukuhamba: Ufanele ukhathazeke ukuba amalungu omntwana wakho, anjengamadolo neengqiniba, avuvukile kwaye aluhlaza okwesibhakabhaka kwangoko nje ukuba aqale ukuhamba okanye ukukhasa.
- Amabala amakhulu aluhlaza okwesibhakabhaka nokuba avela kwinxeba elincinci: Nokuba iqhuma elincinci linokubangela ukuba kuvele amabala amakhulu aluhlaza okwesibhakabhaka emzimbeni.
- Ukopha rhoqo kweempumlo nokopha iintsini: Ukopha rhoqo kwiintsini ngexesha lokukhuhla amazinyo okanye xa uhlamba amazinyo.
- Igazi kumchamo okanye kwindle.
Umntu one-hemophilia encinci usenokungabonakalisi zimpawu de abe mdala. Le meko ngamanye amaxesha ifunyanwa xa kukho ukopha okugqithisileyo ngexesha lenkqubo encinci, njengokukhupha izinyo.
Esi sifo sivela njani?
Oku kungabonakala kunzima kancinci, kodwa kulula ukukuqonda. I-Hemophilia sisifo semfuza . Oku kuthetha ukuba sibangelwa sisiphene kwiimfuza ezivela kumama okanye kutata.
- Ukusuka kumama ukuya kunyana: Ijini enesiphene ebangela i-hemophilia ikwi-chromosome X. Umntwana oyinkwenkwe (unyana) ufumana i-chromosome X kumama kunye ne-chromosome Y kutata. Ngoko ke, ukuba umama unesiphene kwenye yee-chromosomes zakhe ze-X, kukho amathuba angama-50% okuba unyana wakhe afumane i-chromosome X enesiphene kuye. Ukuba oko kuyenzeka, unyana uya kuba ne-hemophilia.
- Abafazi (iintombi) baba ngabathwali: Umntwana obhinqileyo ufumana ii-chromosomes ezimbini ze-X (enye evela kunina, enye evela kuyise). Nangona efumana i-chromosome ye-X enesiphene kunina, ngesiqhelo akafumani esi sifo ngenxa ye-chromosome ye-X ephilileyo evela kuyise. Nangona kunjalo, baba ngabathwali besifo . Oku kuthetha ukuba banokudlulisela esi sifo kubantwana babo.
- Ukuba utata une-hemophilia: Utata one-hemophilia akasoze adlulisele esi sifo koonyana bakhe kuba oonyana bazuza i-chromosome Y kuphela kuyise wabo. Nangona kunjalo, ekubeni zonke iintombi zakhe zizuza i-chromosome X enesiphene kuye, zonke ezo ntombi ziya kuba ngabathwali besifo.
Ngamanye amaxesha, umntwana unokufumana esi sifo ngokuguquka okuzenzekelayo, nokuba akukho mntu kusapho une-hemophilia.
Ungasixilonga njani esi sifo? (Ukuxilongwa)
Le meko idla ngokufunyaniswa emva nje kokuzalwa. Ingafunyaniswa ngokujonga imbali yosapho, okanye ukuba umntwana uphuma igazi ngendlela engaqhelekanga. Ingafunyaniswa nangokuthatha isampuli yegazi kwi-umbilical cord emva kokuba umntwana ezelwe.
Ukuba wena okanye umntwana wakho unale mpawu, bonana nogqirha ngokukhawuleza. Ugqirha uza kuqala abuze ukuba kukho umntu kusapho lwakho onale ngxaki. Emva koko baza kwenza uvavanyo lwegazi.
- Uvavanyo lokuqunjelwa kwegazi: Olu vavanyo lujonga ukuba igazi lakho liqunjelwa ngokukhawuleza kangakanani na.
- Uvavanyo lweFactor: Ukuba ingxaki ifunyenwe, olu vavanyo lunokuchonga uhlobo lwe-hemophilia onayo (A okanye B) kunye nokuba inzima kangakanani.
| Ubunzima besifo | Amanqanaba e-Clotting Factor xa ethelekiswa namanqanaba aqhelekileyo |
|---|---|
| Ubuthambileyo | 5% ukuya kuma-40% |
| Iphakathi | 1% ukuya kwi-5% |
| Inzima kakhulu | Ngaphantsi kwe-1% |
Zithini iindlela zonyango?
Akukho nto yokoyika, kukho unyango olusebenzayo kakhulu lwe-hemophilia namhlanje. Injongo ephambili kukubuyisela i-blood clotting factor engekhoyo emzimbeni.
- Ukutshintshwa kweClotting Factor: Olu lolona nyango luphambili. I-Factor VIII inikwa ngemithambo yegazi kwi-hemophilia A kwaye i-Factor IX inikwa ngemithambo yegazi kwi-hemophilia B. Oku kungenziwa esibhedlele, okanye ungaqeqeshwa ukukwenza ekhaya.
- Unyango lwehomoni:I-Desmopressin liyeza elinikwa ukunyanga i-hemophilia A engembi kangako. Isebenza ngokuvuselela umzimba ukuba ukhuphe i-Factor VIII. Ifumaneka njengenaliti okanye njenge-spray yempumlo.
- Ii-Anticoagulants: La mayeza asebenza ngokuthintela ukuqhekeka kwegazi ekunyibilikeni ngokukhawuleza. Asetyenziswa kwiimeko ezifana nokukhupha amazinyo.
- Unyango Lomzimba: Ukopha rhoqo kwamalungu kunokubangela ukuba angakwazi ukuhambahamba. Unyango lomzimba lunokunceda ukubuyisela ukusebenza kwamalungu.
Iingxaki ezinokwenzeka ngenxa ye-hemophilia
Ukuba unyango olufanelekileyo alufumaneki, ezinye iingxaki zinokubakho, ingakumbi kwiimeko ezinzima ze-hemophilia.
- Ukonakala kwamalungu: Ukopha rhoqo kwamalungu afana namadolo, iingqiniba, nasemaqakaleni kunokubangela ukuba abe nokukhubazeka okungapheliyo, abe buhlungu, kwaye kube nzima ukuwashukumisa.
- Ukopha igazi engqondweni: Le yeyona ngxaki iyingozi kakhulu. Ukopha igazi engqondweni kunokubakho ngenxa yokwenzakala entloko okanye, kwezinye iimeko ezinzima, ngaphandle kwesizathu esicacileyo. Oku kunokubangela ukukhubazeka okusisigxina okanye nokufa. Ukuba wenzakele kakhulu entloko, yiya kwiSebe lezeMpilo eziNgxamisekileyo (ETU) esibhedlele ngoko nangoko.
- Ubunzima bokuphefumla: Ukuba kukho ukopha emqaleni, ukudumba kunokubangela ubunzima bokuphefumla.
Uninzi lwezi ngxaki zinokuthintelwa ngonyango olufanelekileyo nolufike ngexesha elifanelekileyo. Ngoko ke, kubaluleke kakhulu ukulandela imiyalelo kagqirha wakho ngokuchanekileyo.
Ukuba uceba ukuba nomntwana kwaye unembali yentsapho ye-hemophilia, ungafumana ingcebiso nge-genetic. Oku kunokukunceda ufumanise ukuba ungumthwali we-hemophilia kwaye yintoni ingozi yakho kumntwana wakho. Kunokwenzeka nokukhetha i-embryo esempilweni kwaye uyifake kwisibeleko sakho ngeendlela ezifana nokuchumisa kwi-vitro.
Umyalezo Wokuya Ekhaya
- I-Hemophilia sisifo semfuza esichaphazela inkqubo yokujiya kwegazi kwaye sidluliselwa kwizizukulwana ngezizukulwana. Asisisifo esosulelayo.
- Ukuba uneempawu ezinje ngokopha kakhulu ngenxa yokwenzakala okuncinci, ukukrweleka rhoqo, okanye ukudumba kwamalungu , funa ingcebiso kagqirha ngoko nangoko .
- Namhlanje, kukho unyango olusebenzayo kakhulu lwesi sifo. Ngonyango olufanelekileyo, ungaphila ubomi obuqhelekileyo.
- Ukuba ubetheke kakhulu entloko, kubalulekile ukuba uye kwiYunithi yoNyango oluNgxamisekileyo (ETU) yesibhedlele ngoko nangoko.
- Ukuba kukho imbali ye-hemophilia kusapho lwakho, kuya kuba bubulumko ukufuna iingcebiso ngemfuza ngaphambi kokuba ucebe ukuba nabantwana.
💬 Comments (0)
Akukho zimvo zithunyelweyo okwangoku. Faka uluvo lwakho apha okokuqala.
Faka uluvo lwakho