Ngaba uyayazi iHemophilia C? Makhe sithethe ngale meko ingaqhelekanga!

Ngamanye amaxesha nokuba nenxeba elincinci lithatha ixesha ukuyeka ukopha, akunjalo? Okanye ukhe weva ukuba umntu kusapho lwakho unesifo esinxulumene negazi? Namhlanje siza kuthetha ngesifo segazi esingaqhelekanga, kodwa kubaluleke kakhulu ukwazi ngaso. Oku kubizwa ngokuba yiHemophilia C.

Yintoni iHemophilia C?

Ngamafutshane, i-hemophilia C luhlobo olungaqhelekanga lwe-hemophilia. I-Hemophilia yimeko apho igazi lakho lingajiki kakuhle, oko kuthetha ukuba xa uphuma igazi, liyayeka kancinci okanye aliyeki. Abantu abane-hemophilia C baneprotheyini ekhethekileyo yegazi ebizwa ngokuba yi-clotting factor , ebizwa ngokuba yi -Factor XI , enceda ukujiya kwegazi. Oku kukwabizwa ngokuba yi -Factor XI Deficiency kwaye ngamanye amaxesha kuthiwa yi -Rosenthal syndrome .

Nangona kunjalo, iimpawu ze-hemophilia C zihlala zincinci kunezo zezinye iintlobo ze-hemophilia (A kunye no-B). Nangona kunjalo, abantu abane-hemophilia C banokopha igazi elingaphezulu kunesiqhelo ngexesha lotyando okanye iinkqubo zamazinyo. Oogqirha banyanga oku nge-plasma entsha eqandisiweyo, eyenziwe ngegazi elinikelweyo. Le plasma inceda ukuqhekeka kwegazi.

Ingaba i-hemophilia C yimeko eqhelekileyo?

Hayi, le yimeko engaqhelekanga ngokwenene. Khawuthelekelele, kwilizwe elifana neMelika, malunga nendoda enye kwi-100,000 yamadoda nabafazi iba ne-hemophilia C. Ukuba siyithelekisa nezinye iintlobo ze-hemophilia, i-hemophilia A ichaphazela malunga ne-12 kwi-100,000 yamadoda, kwaye i-hemophilia B ichaphazela malunga ne-4 kwi-100,000 yamadoda. Abaphandi abakaqiniseki ukuba i-hemophilia A okanye i-B ichaphazela kangaphi abafazi.

Yintoni umahluko phakathi kwe-hemophilia A, B, kunye ne-C?

Zonke iintlobo ezintathu ze-hemophilia zizifo zegazi ezizuzwe njengelifa . Oko kukuthi, utshintsho lwemfuza luchaphazela inkqubo yokujiya kwegazi. Kodwa kukho umahluko omncinci phakathi kwezi ntlobo zintathu. Makhe sijonge ukuba zeziphi:

• I-Hemophilia A kunye ne-Hemophilia B zenzeka xa umntu efumana i-gene eguquliweyo komnye wabazali bakhe bemvelo. Nangona kunjalo, kwi-hemophilia C, i-gene engaqhelekanga inokuzuzwa kubazali bobabini .
• Ngokungafaniyo nabantu abane-hemophilia A okanye B, abantu abane-hemophilia C abadli ngokuba neengxaki zokopha ezichaphazela amalungu okanye izihlunu . Lo ngumahluko obalulekileyo.
• Ngokwesiqhelo, iimpawu zabantu abane-hemophilia A okanye B zibonakaliswa ngamanqanaba eeproteni zokugalela igazi, okanye 'izinto ezigalela igazi', egazini labo. Umzekelo, umntu one-hemophilia A enzima uya kuba namanqanaba aphantsi kakhulu aloo factor. Kodwa okumangalisayo kukuba, umntu one-hemophilia C unokuba namanqanaba aphantsi kakhulu aloo factor, kodwa iimpawu zakhe zinokuba zincinci kakhulu .
• I-Hemophilia A kunye ne-B zinokubakho kubantu bazo zonke iintlanga kunye neentlanga. Nangona kunjalo, i-hemophilia C ixhaphake kancinci kubantu beqela lamaJuda ase-Ashkenazi . Oku akuthethi ukuba akukho mntu unokuyivelisa, kodwa ixhaphake kakhulu phakathi kwabo.
• I-Hemophilia A kunye ne-B zixhaphake kakhulu kumadoda kunakwabasetyhini, kodwa i-hemophilia C ichaphazela amadoda nabasetyhini ngokulinganayo .

Zithini iimpawu ze-hemophilia C?

Abantu abane-hemophilia C banokufumana ukopha okungapheliyo nokunganqandekiyo emva kotyando olukhulu, ukwenzakala okukhulu, okanye ukuzala. Nangona kunjalo, abafumani ukopha okuzenzekelayo , apho igazi liqala ukuphuma ngaphandle kwesizathu esicacileyo.

Abantu abane-hemophilia C badla ngokopha ngendlela engaqhelekanga emva kotyando lwamazinyo, ukukhupha amazinyo, okanye ukususwa kweetoni.

Ezinye iimpawu zingabandakanya:

• Ukopha rhoqo ngeempumlo .
• Ukudumba emzimbeni nokuba yinto encinci.
• Igazi kumchamo .
• Ukopha kakhulu emva kokwaluka.
• Imivumbo ebuhlungu nedumbileyo emva kotyando.
• Kwabasetyhini, ukuya exesheni kunokuhlala iintsuku ezininzi, nto leyo ethatha ixesha elide ngendlela engaqhelekanga.

Zithini izizathu ze-hemophilia C?

I-Hemophilia C sisifo segazi esizuzwe njengelifa . Sivela xa ungenayo iproteni yegazi ebizwa ngokuba yiFactor XI , enye yezinto ezili-13 ezibangela ukujiya kwegazi ezinceda ekunciphiseni okanye ekumiseni ukopha. Oku kungenxa yokuba awunayo i -F11 gene echanekileyo.

Ngokwesiqhelo, le gene ye-F11 inemiyalelo yokwenza i-Factor XI. Ukuba le gene iyatshintsha, oko kukuthi, ukuba iba yinto engaqhelekanga, kuvela i-hemophilia C. Le gene engaqhelekanga isenokungabi yi-Factor XI eyaneleyo, okanye isenokungabi yiyo kwaphela.

Amadoda namabhinqa afumana ilifa le-hemophilia C kuphela ukuba abazali bobabini bemvelo badlulisela i-gene eguquliweyo kumntwana wabo . Ukuba umntu ufumana i-gene eqhelekileyo ye-F11 kunye ne-gene engaqhelekanga, loo mntu ungumthwali we-hemophilia C, kodwa akabonisi zimpawu.

Ngoku, jonga into enokwenzeka xa abazali abanesi jini singaqhelekanga benabantwana:

• Abantwana abazelwe ngabazali abanolu tshintsho lwe-F11 gene banethuba elingama-25% lokufumana i-hemophilia C.
• Abantwana abazalwa ngabo bazali banethuba elingama-50% lokuba ngabathwali besifo, oko kuthetha ukuba bathwala kuphela i-gene ngaphandle kwesifo.
• Abantwana babo bazali banethuba elingama-25% lokufumana ilifa le-F11 gene eqhelekileyo.

Oogqirha bayixilonga njani i-hemophilia C?

Ngaba uyazi ukuba oogqirha bayixilonga njani i-hemophilia C? Baza kuqala bakuxilonge ngokwasemzimbeni . Emva koko baza kubuza ngeempawu zakho, ezinje ngokopha empumlweni okanye ukopha emva kokwenza uphando lwamazinyo. Baza kubuza nangembali yosapho lwakho nokuba kukho umntu kusapho lwakho one-hemophilia okanye ezinye iingxaki zegazi . Olu lwazi lubaluleke kakhulu ekuxilongeni isifo.

Ziziphi iimvavanyo eziphambili ezisetyenziswayo ukuqinisekisa ukuxilongwa?

Ugqirha wakho uza kusebenzisa iimvavanyo ezimbini eziphambili ukuqinisekisa ukuba unesifo:

• Uvavanyo lwexesha le-thromboplastin olungasebenziyo (APTT): Olu luvavanyo olusetyenziselwa ukuxilonga i-hemophilia. Luvumela ugqirha wakho ukuba azi ukuba kuthatha ixesha elingakanani ukuba igazi lakho lijiye .
• Uvavanyo lwe-Clotting factor: Olu vavanyo lwegazi lubonisa uhlobo lwe-hemophilia kunye nokuba inzima kangakanani .

Kwezinye iimeko, ugqirha wakho unokucela ezinye iimvavanyo ezimbalwa:

• Ubalo lwegazi olupheleleyo (CBC): Olu vavanyo lulinganisa kwaye lufunda iiseli zegazi.
• Uvavanyo lwexesha le-Prothrombin (PT): Oku kukwajonga ukuba igazi lakho likhawuleza kangakanani na ukujiya.
• Uvavanyo lwe-Fibrinogen: Olu vavanyo lwegazi lulinganisa ubungakanani beprotheyini yegazi ebizwa ngokuba yi-fibrinogen, enceda ukujiya kwegazi.

Kubalulekile ukuqaphela ukuba i-hemophilia C ngamanye amaxesha kunokuba nzima ukuyixilonga. Ngoba? Iziphumo zovavanyo lwegazi, ingakumbi iziphumo zovavanyo lwe-clotting factor, zisenokungahambelani neempawu zesigulana. Umzekelo, uvavanyo lwe-clotting factor lunokubonisa ukuba inqanaba lakho le-factor liphantsi kakhulu, kodwa usenokungabi nazo naziphi na iimpawu. Kwakhona, usenokuba neempawu ze-hemophilia C nokuba inqanaba lakho le-factor liqhelekile. Ngoko ke ugqirha uza kukuqwalasela konke oku aze afikelele kwisigqibo.

Inyangwa njani i-hemophilia C?

Abantu abane-hemophilia C basenokungadingi unyango de benze utyando okanye olunye unyango. Ukuba oko kuyimfuneko, oogqirha basebenzisa 'i-plasma eqandisiweyo entsha', eyenziwe ngegazi elinikelweyo.kunye nendibaniselwano yamayeza athintela ukwahlulwahlulwa kwezinto 'ezibangela ukuqhekeka' ezinikwa njengezithatha indawo. Ukuba abafazi banexesha elinzima ngokungaqhelekanga, elihlala iintsuku ezininzi, oogqirha banokunika iipilisi zokulawula ukuzala .

Ngaba i-hemophilia C ingathintelwa?

Hayi, i-hemophilia C sisifo segazi esidluliselwa kwilifa, ngoko ke asinakuthintelwa . Nangona kunjalo, ukuba wena okanye umntu osapho lwakho unesi sifo, iingcebiso ngemfuza zinokukunceda uqonde umngcipheko wokusidlulisela kwizizukulwana ezizayo.

Yintoni enokulindelwa ngumntu one-hemophilia C?

Uninzi lwabantu abane-hemophilia C baneempawu ezincinci . Azifane zibangele iingxaki ezinkulu zempilo. Uninzi lwabantu alubonakalisi zimpawu de babe ngabantu abadala, kwaye ezo mpawu zihlala zincinci kakhulu.

Nangona kunjalo, usenokuba neengxaki zokopha emva kotyando okanye unyango lwamazinyo. Ukuba une-hemophilia C, kubalulekile ukubuza ugqirha wakho malunga namanyathelo okhuseleko ekufuneka uwathathe . Umzekelo, ukwazisa ugqirha wakho ngaphambi kotyando kunye nokuphepha amayeza athile anyusa ukopha (njenge-aspirin).

Okokugqibela, ezinye izinto ezibalulekileyo ekufuneka uzikhumbule

Kulungile, ngoko ke, kukho izinto ezimbalwa ekufuneka uzikhumbule koko sithethe ngako:

• I-Hemophilia C sisifo segazi esingaqhelekanga kakhulu .
• Ngokwesiqhelo, iimpawu zayo azibalulekanga kangako kwaye azifane zibangele iingxaki ezinkulu zempilo.
• Abantu abaninzi babonisa iimpawu kuphela xa bebadala.
• Nangona kunjalo, ungopha kakhulu kunabanye ngexesha lotyando okanye unyango lwamazinyo , ngoko ke kubalulekile ukwazisa ugqirha wakho kwiimeko ezinjalo.
• Ukuba ufunyaniswe une-hemophilia C, musa ukoyika. Thetha nogqirha wakho ukuqinisekisa ukuba uyayiqonda into ekufuneka uyazi kunye namanyathelo okhuseleko ekufuneka uwathathe .

Khumbula, ukuqonda kakuhle nayiphi na imeko yezonyango lelona nyathelo lilungileyo lokujamelana nayo! Awuwedwa, kwaye iingcebiso zonyango kunye nenkxaso zihlala zifumaneka kuwe.

I- Hemophilia C, ukujiya kwegazi, iFactor XI, isifo semfuza, ukopha, iimpawu, unyango