Uyazazi yini i-Hemophilia C? Ake sixoxe ngalesi simo esingavamile!

Ngezinye izikhathi ngisho nokusikeka okuncane kuthatha isikhathi ukuyeka ukopha, akunjalo? Noma wake wezwa ukuthi othile emndenini wakho unesifo esihlobene negazi? Namuhla sizokhuluma ngesifo segazi esingavamile, kodwa kubaluleke kakhulu ukwazi ngaso. Lokhu kubizwa ngokuthi i-Hemophilia C.

Kuyini i-Hemophilia C?

Kalula nje, i-hemophilia C uhlobo olungavamile kakhulu lwe-hemophilia. I-hemophilia yisimo lapho igazi lakho lingavimbi kahle, okusho ukuthi uma uphuma igazi, liyayeka kancane noma aliyeki. Abantu abane-hemophilia C baneprotheni ekhethekile yegazi ebizwa ngokuthi i-clotting factor , ebizwa ngokuthi i-Factor XI , esiza ukuvimbi kwegazi. Lokhu kubizwa nangokuthi i-Factor XI Deficiency futhi ngezinye izikhathi kuthiwa i-Rosenthal syndrome .

Kodwa-ke, izimpawu ze-hemophilia C zivame ukuba zincane kunezezinye izinhlobo ze-hemophilia (A no-B). Kodwa-ke, abantu abane-hemophilia C bangase bophe igazi elingaphezu kokuvamile ngesikhathi sokuhlinzwa noma izinqubo zamazinyo. Odokotela baphatha lokhu nge-plasma eqandisiwe entsha, eyenziwe ngegazi elinikelwe. Le plasma isiza ukwakheka kwegazi.

Ingabe i-hemophilia C iyisifo esivamile?

Cha, empeleni lesi yisimo esingavamile kakhulu . Cabanga nje, ezweni elifana neMelika, cishe oyedwa kwabesilisa nabesifazane abayi-100,000 uba ne-hemophilia C. Uma siqhathanisa nezinye izinhlobo ze-hemophilia, i-hemophilia A ithinta cishe abayi-12 kwabesilisa abayi-100,000, kanti i-hemophilia B ithinta cishe abayi-4 kwabesilisa abayi-100,000. Abacwaningi abakaqiniseki ukuthi i-hemophilia A noma i-B ithinta kangaki abesifazane.

Uyini umehluko phakathi kwe-hemophilia A, B, kanye ne-C?

Zonke izinhlobo ezintathu ze-hemophilia ziyizifo zegazi ezizuzwe njengefa . Okusho ukuthi, ukuguquka kwezakhi zofuzo kuthinta inqubo yokujiya kwegazi. Kodwa kunezinhlobo ezincane phakathi kwalezi zinhlobo ezintathu. Ake sibheke ukuthi ziyini:

• I-Hemophilia A kanye ne-Hemophilia B zenzeka lapho umuntu ezuza ifa lofuzo oluguqukile komunye wabazali bakhe bemvelo. Kodwa-ke, ku-hemophilia C, ifa lofuzo elingavamile lingazuzwa kubo bobabili abazali .
• Ngokungafani nabantu abane-hemophilia A noma B, abantu abane-hemophilia C abavamile ukuba nezinkinga zokopha ezithinta amalunga noma imisipha . Lona umehluko obalulekile.
• Ngokuvamile, izimpawu zabantu abane-hemophilia A noma B zinqunywa amazinga amaprotheni okujiya kwegazi, noma 'izici zokujiya', egazini labo. Isibonelo, umuntu one-hemophilia A enzima uzoba namazinga aphansi kakhulu aleso sici. Kodwa ngokumangazayo, umuntu one-hemophilia C angaba namazinga aphansi kakhulu aleso sici, kodwa izimpawu zakhe zingase zibe zincane kakhulu .
• I-Hemophilia A kanye ne-B ingavela kubantu bazo zonke izinhlanga kanye nezinhlanga. Kodwa-ke, i-hemophilia C ivame kakhulu kubantu beqembu lohlanga lwamaJuda ase-Ashkenazi . Lokhu akusho ukuthi akekho omunye umuntu ongayithuthukisa, kodwa ivame kakhulu phakathi kwabo.
• I-Hemophilia A kanye ne-B zivame kakhulu kwabesilisa kunakwabesifazane, kodwa i-hemophilia C ithinta kokubili amadoda nabesifazane ngokulinganayo .

Ziyini izimpawu ze-hemophilia C?

Abantu abane-hemophilia C bangase babe nokopha okuqhubekayo, okunganqandeki ngemva kokuhlinzwa okukhulu, ukulimala okukhulu, noma ukubeletha. Kodwa-ke, ababi nokopha okuzenzekelayo , okuyilapho igazi liqala ukugeleza ngaphandle kwesizathu esibonakalayo.

Abantu abane-hemophilia C bavame ukopha ngendlela engavamile ngemva kokuhlinzwa kwamazinyo, ukukhishwa kwamazinyo, noma ukususwa kwamathonsi.

Ezinye izimpawu zingase zihlanganise:

• Ukopha igazi njalo ekhaleni.
• Ukuphuka emzimbeni kusukela entweni encane kakhulu.
• Igazi emchameni .
• Ukopha ngokweqile ngemva kokusoka.
• Imivimbo ebuhlungu nevuvukele ngemva kokuhlinzwa.
• Kwabesifazane, ukuya esikhathini kungahlala izinsuku eziningana, okuhlala isikhathi eside ngendlela engavamile.

Yiziphi izimbangela ze-hemophilia C?

I-Hemophilia C isifo segazi esizuzwe njengefa . Sivela uma untula iphrotheni yegazi ebizwa ngokuthi i-Factor XI , enye yezinto ezingu-13 ezibangela ukujiya kwegazi ezisiza ekunciphiseni noma ekumiseni ukopha. Lokhu kungenxa yokuthi awunayo i -F11 gene efanele.

Ngokuvamile, lesi sakhi sofuzo se-F11 sinemiyalelo yokwenza i-Factor XI. Uma lesi sakhi sofuzo siguquguquka, okungukuthi, uma siba esingavamile, kuvela i-hemophilia C. Lesi sakhi sofuzo esingavamile singase singakhiqizi i-Factor XI eyanele, noma singase singakhiqizi nhlobo.

Bobabili abesilisa nabesifazane bazuza i-hemophilia C kuphela uma bobabili abazali bemvelo bedlulisela i-gene eguquliwe enganeni yabo . Uma othile ezuza i-gene eyodwa evamile ye-F11 kanye ne-gene eyodwa engavamile, lowo muntu ungumthwali we-hemophilia C, kodwa akabonisi zimpawu.

Manje, bheka ukuthi yini engenzeka uma abazali abanalesi sakhi sofuzo esingavamile beba nezingane:

• Izingane ezizalwa abazali abanalolu shintsho lwezakhi zofuzo lwe-F11 zinethuba elingu-25% lokuthuthukisa i-hemophilia C.
• Izingane ezizalwa yilabo bazali zinethuba elingu-50% lokuba ngabathwali balesi sifo, okusho ukuthi zithwala kuphela isakhi sofuzo ngaphandle kwalesi sifo.
• Izingane zalabo bazali zinethuba elingu-25% lokuthola ifa elijwayelekile le-F11.

Odokotela bakuthola kanjani ukuthi une-hemophilia C?

Uyazi ukuthi odokotela baxilonga kanjani i-hemophilia C? Bazoqala bakuhlole ngokomzimba . Bese bebuza ngezimpawu zakho, njengokuphuma kwegazi ekhaleni noma ukuphuma kwegazi ngemva kokuhlolwa kwamazinyo. Bazobuza nangomlando womndeni wakho nokuthi ngabe kukhona yini emndenini wakho one-hemophilia noma ezinye izifo zegazi . Lolu lwazi lubaluleke kakhulu ekuxilongweni kwesifo.

Yiziphi izivivinyo eziyinhloko ezisetshenziswa ukuqinisekisa ukuxilongwa?

Udokotela wakho uzosebenzisa izivivinyo ezimbili eziyinhloko ukuqinisekisa ukuthi unesifo:

• Ukuhlolwa kwesikhathi se-thromboplastin okuyingxenye okusebenzayo (APTT): Lokhu ukuhlolwa okusetshenziselwa ukuxilonga i-hemophilia. Kuvumela udokotela wakho ukuthi azi ukuthi kuthatha isikhathi esingakanani ukuthi igazi lakho lijiye .
• Ukuhlolwa kwe-Clotting factor: Lokhu kuhlolwa kwegazi kukhombisa uhlobo lwe-hemophilia nokuthi lubi kangakanani .

Kwezinye izimo, udokotela wakho angase acele ukuhlolwa okwengeziwe:

• Ukubalwa kwegazi okuphelele (i-CBC): Lokhu kuhlola kulinganisa futhi kufunde amaseli egazi.
• Ukuhlolwa kwesikhathi se-Prothrombin (PT): Lokhu kuhlola nokuthi igazi lakho liqina ngokushesha kangakanani.
• Ukuhlolwa kwe-Fibrinogen: Lokhu kuhlolwa kwegazi kulinganisa inani leprotheni yegazi ebizwa ngokuthi i-fibrinogen, esiza ukujiya kwegazi.

Kubalulekile ukuqaphela ukuthi i-hemophilia C ngezinye izikhathi kungaba nzima ukuyixilonga. Kungani? Imiphumela yokuhlolwa kwegazi, ikakhulukazi imiphumela yokuhlolwa kwe-clotting factor, ingase ingahambisani nezimpawu zesiguli. Isibonelo, ukuhlolwa kwe-clotting factor kungabonisa ukuthi izinga lakho le-factor liphansi kakhulu, kodwa ungase ungabi nazo izimpawu. Futhi, ungase ube nezimpawu ze-hemophilia C noma ngabe izinga lakho le-factor lijwayelekile. Ngakho udokotela uzokucabangela konke lokhu futhi afinyelele esiphethweni.

Iphathwa kanjani i-hemophilia C?

Abantu abane-hemophilia C bangase bangadingi ukwelashwa kuze kube yilapho behlinzwa noma bethola enye inqubo yezokwelapha. Uma lokho kudingeka, odokotela basebenzisa 'i-plasma eqandisiwe entsha', eyenziwe ngegazi elinikelwe.kanye nenhlanganisela yemithi evimba ukuwohloka 'kwezinto ezijiyile' ezinikezwa njengezindlela zokufaka esikhundleni. Uma abesifazane beya esikhathini esinzima ngendlela engavamile, esihlala izinsuku eziningana, odokotela bangase banikeze amaphilisi okuvimbela inzalo .

Ingabe i-hemophilia C ingavinjelwa?

Cha, i-hemophilia C iyisifo segazi esizuzwa njengefa, ngakho-ke asinakuvinjelwa . Kodwa-ke, uma wena noma othile emndenini wakho enalesi simo, ukwelulekwa ngofuzo kungakusiza uqonde ingozi yokudlulisela lesi sifo ezizukulwaneni ezizayo.

Yini umuntu one-hemophilia C angayilindela?

Iningi labantu abane-hemophilia C banezimpawu ezincane . Akuvamile ukubangela izinkinga ezinkulu zezokwelapha. Iningi labantu alibi nezimpawu kuze kube yilapho sebekhulile, futhi lezo zimpawu ngokuvamile zincane kakhulu.

Kodwa-ke, ungase ube nezinkinga zokopha ngemva kokuhlinzwa noma ukwelashwa kwamazinyo. Uma une-hemophilia C, kubalulekile ukubuza udokotela wakho nganoma yiziphi izinyathelo zokuphepha okufanele uzithathe . Isibonelo, ukwazisa udokotela wakho ngaphambi kokuhlinzwa nokugwema imithi ethile ekhulisa ukopha (njenge-aspirin).

Okokugcina, ezinye izinto ezibalulekile okudingeka uzikhumbule

Kulungile, ngakho-ke, kunezinto ezimbalwa okudingeka uzikhumbule kulokho esikhulume ngakho:

• I-Hemophilia C isifo segazi esingavamile kakhulu .
• Ngokuvamile, izimpawu zayo zincane kakhulu futhi azivamile ukubangela izinkinga ezinkulu zempilo.
• Abantu abaningi babonisa izimpawu kuphela lapho sebekhulile.
• Kodwa-ke, ungase wophe kakhulu kunabanye ngesikhathi sokuhlinzwa noma sokwelashwa kwamazinyo , ngakho-ke kubalulekile ukwazisa udokotela wakho ezimweni ezinjalo.
• Uma kutholakale ukuthi une-hemophilia C, ungesabi. Khuluma nodokotela wakho ukuze uqiniseke ukuthi uyaqonda ukuthi yini okudingeka uyazi nokuthi yiziphi izinyathelo zokuphepha okudingeka uzithathe .

Khumbula, ukuqonda kahle noma yisiphi isimo sezokwelapha kuyisinyathelo esingcono kakhulu sokubhekana naso! Awuwedwa, futhi iseluleko sezokwelapha kanye nokusekelwa kuhlala kutholakala kuwe.

I- Hemophilia C, ukujiya kwegazi, i-Factor XI, isifo sofuzo, ukopha, izimpawu, ukwelashwa