Ngaba nawe unayo imeko apho nokuba inxeba elincinci aliyeki ukopha? (Ukunqongophala kweFactor VII)

Ngaba ngamanye amaxesha ukhe uzive ngathi kuthatha ixesha elide nokuba linxeba elincinci ukuze liyeke ukopha? Okanye ujika ube luhlaza okwesibhakabhaka kwaye ube nemivumbo? Mhlawumbi kukho isizathu sezi zinto esingazaziyo. Yiloo nto ibangela ukungabikho kweFactor VII.

Yintoni iFactor VII Deficiency?

Ngamafutshane, ukungabikho kweFactor VII yimeko yemfuza apho igazi lakho lingajiki kakuhle, oko kuthetha ukuba aliyeki ukopha ngokukhawuleza. Kukho iintlobo ezahlukeneyo zeeproteni egazini lethu ezibizwa ngokuba yi-'blood clotting factors' okanye 'coagulation factors' . I-Factor VII yenye yeeproteni ezinjalo. Zonke ezi proteni zisebenza kunye neeplatelets zakho ukwenza i-blood clot yokuyeka ukopha xa wenzakele. Le nkqubo ifana nokwakha udonga ngezitena nesamente. Ngoko ke, ukuba awunayo eyaneleyo le proteni ibalulekileyo ebizwa ngokuba yiFactor VII emzimbeni wakho, okanye ukuba ayisebenzi kakuhle, kuthatha ixesha elide ukuba igazi lakho lijike kunesiqhelo. Yiyo loo nto ukruquka kwaye wopha ngokulula kunabanye.

Amanye amagama alo mbandela sisifo sika-Alexander kunye nokungabikho kwe-proconvertin .

Ixhaphake kangakanani le ngxaki yokunqongophala kwe-factor VII?

Esi sisifo esingaqhelekanga . Ehlabathini lonke, sichaphazela umntu omnye kwabangama-500,000 . Kukho iingxaki ezininzi zokuqhekeka kwegazi ezizuzwe njengelifa ezithi oogqirha bazibize ngokuba "zingaqhelekanga." Ukungabikho kwe-Factor VII yenye yezona zifo zixhaphakileyo.

Zithini iimpawu zoku?

Ukunqongophala kwe-Factor VII kunokubangela ukopha ixesha elide kunye nokukrweleka okulula. Iimpawu eziphambili ziquka:

• Iintsini eziphuma igazi.
• Ukopha kwempumlo (epistaxis) , kunye nobunzima bokuyimisa.
• Kwabasetyhini, ukopha kakhulu ngexesha lokuya exesheni (i-menorrhagia).
• Igazi kumchamo (i-hematuria) okanye igazi elikwisitulo.
• Ukopha okungapheliyo emva kokwenzakala, utyando, okanye ukuzala.
• Ukopha ngaphakathi emzimbeni (umzekelo, kwinkqubo yesisu, kwizicubu, kwimisipha, kumalungu).
• Ukopha ngaphakathi kwentloko nako kunokubakho. Oku kuyingozi ngakumbi.
• Ukopha okuzenzekelayo kwenzeka xa ukopha ngequbuliso kuqala ngaphandle kwesizathu esicacileyo.

Ubunzima bezi mpawu kunye nexesha ezibonakala ngalo ziyahluka kumntu nomntu. Umzekelo, umntwana onesifo esibi kakhulu seFactor VII unokufunyanwa xa ezalwa. Nangona kunjalo, umntu oneempawu ezingembi kangako usenokungafunyanwa de abe mdala.

Yintoni ebangela ukunqongophala kweFactor VII?

Nangona esi sisifo esidluliselwa ngelifa , kukwakho nendlela esi sifo esinokwenzeka ngayo ebomini.

Ukuba uzalwe une-factor VII deficiency , oko kuthetha ukuba uzuze i-mutation, okanye isiphene, kwi -F7 gene yakho. Le gene ilawula indlela umzimba wakho oyenza ngayo i-factor VII. Ukuze ube ne-factor VII deficiency, kufuneka uyizuze le gene mutation kubazali bobabini be-biological . Oku kubizwa ngokuba yi -autosomal recessive inheritance . Ukuba bobabini abazali banale nguqu, banethuba elingama-25% lokuba nomntwana one-factor VII deficiency.

Ukunqongophala kweFactor VII okuqhubeka ngokuhamba kwexesha kubizwa ngokuba yi -'acquired factor VII deficiency' . Kukho izizathu ezininzi ezinokubakho:

• Isifo sesibindi: Isibindi sakho senza iproteni yeFactor VII. Ukuba unesifo sesibindi esinzima, isibindi sakho sisenokungakwazi ukwenza lo msebenzi ngokufanelekileyo.
• Ukunqongophala kweVithamin K: IVithamin K ibalulekile emzimbeni ukuze wenze iFactor VII. Ukuba awuyifumani ngokwaneleyo, unokufumana ukunqongophala kweFactor VII.
• Amayeza okunciphisa igazi: Le meko inokwenzeka naxa kusetyenziswa amayeza okunciphisa igazi afana neWarfarin (Coumadin®) .
• Iingxaki zeeseli zegazi kunye nomhlaza: Ukungabikho kwe-Factor VII kuye kwaxelwa kubantu abanezifo ezifana ne -aplastic anemia kunye ne -leukemia .

Zithini iingxaki zokungabikho kweFactor VII?

Kwiimeko ezinzima, ukungabikho kweFactor VII kunokukhokelela kwezinye iingxaki zempilo ukuba akunyangwa. Umzekelo:

• I-anemia: Xa igazi elininzi kakhulu liphuma emzimbeni, inani leeseli ezibomvu zegazi linokwehla kakhulu.
• Ukonakala kwamalungu: Xa kukho ukopha ngaphakathi kwelungu, izicwili ezilapho zinokuguga kwaye ilungu lisenokungakwazi ukugoba okanye ukuma ngokufanelekileyo.
• IiHematoma: I-hematoma yingqokelela yamahlwili egazi phantsi kolusu lwakho. Ukuba kuvela i-hematoma enkulu, inokubangela uxinzelelo kumalungu akufutshane kwaye ibangele iimpawu ezibuhlungu.

Okubaluleke kakhulu, ukopha kakhulu kunokuba yingozi ebomini ukuba akunyangwa. Yiyo loo nto kubalulekile ukufuna iingcebiso zonyango ukuba ukopha akuyeki.

Ifunyanwa njani i-Factor VII deficiency?

Ugqirha wakho uza kukubuza ngeempawu zakho nokuba kukho umntu kusapho lwakho okhe wanengxaki yokopha na. Ukungabikho kwe-Factor VII kuxilongwa ngovavanyo lwegazi oluvavanya ukuba igazi lakho liqhekeka kangakanani na.

Ezi mvavanyo ziquka:

• Uvavanyo lwexesha le-Prothrombin (PT/INR): Olu vavanyo lulinganisa ixesha elithathwayo ukuze igazi lijiye ngokusekelwe kumsebenzi we-blood clotting factor ebizwa ngokuba yi-prothrombin. Ukuba une-factor VII deficiency, kuthatha ixesha elide kunesiqhelo ukuba igazi lijiye.
• Uvavanyo lwexesha le-thromboplastin engaphelelanga (PTT): Olu vavanyo lulinganisa ixesha elithathwayo ukuze igazi lijiye ngokusekelwe kumsebenzi wezinto ezibangela ukujiya kwegazi ngaphandle kwe-prothrombin. Iziphumo zolu vavanyo zihlala ziqhelekile kumntu oswele i-factor VII.
• Uvavanyo lweFactor VII: Olu vavanyo lubonisa indlela esebenza ngayo iproteni yeFactor VII.

Iphathwa njani i-factor VII deficiency?

Unyango ludla ngokubandakanya ukongeza i-factor VII esebenza kakuhle egazini lakho. Usenokuthi usebenze nogqirha ogxile kwiingxaki zegazi, obizwa ngokuba yi-hematologist , oza kugqiba ukuba loluphi unyango olufunekayo kunye nokuba uza kuludinga ixesha elingakanani.

Kukho iindlela ezininzi eziphambili zonyango:

• I-Recombinant factor VIIa (NovoSeven®): Eli yeza livunyiwe yi-US Food and Drug Administration (FDA) ukunyanga ukungabikho kwe-factor VII. Lenziwe ngoososayensi kwilebhu. Oku kuthetha ukuba, ngokungafaniyo nezinye iindlela zonyango zokutshintsha i-factor VII, alenziwa ngegazi elinikelweyo ngabantu.
• Ii-Prothrombin complex concentrates (PCC): Olu nyango luqulathe ubuninzi bezinto ezine ezibangela ukugabha igazi, kuquka i-factor VII, ezithathwe kwigazi elinikelweyo.
• Iplasma eqandisiweyo entsha (FFP): Usenokunikwa iplasma ngemithambo ukuze uncede ekwakheni kwakhona iivenkile zakho zeFactor VII.

Ezinye iindlela zonyango:

• Ii-antifibrinolytic agents: La mayeza asebenza ngokuthintela amahlwili egazi ayenzeka emzimbeni wakho ukuba anganyibiliki. Anceda ekuthinteleni ukopha kakhulu ngokukhusela amahlwili egazi asele ekhona emzimbeni wakho. Imizekelo ibandakanya amayeza abizwa ngokuba yi -aminocaproic acid kunye ne-tranexamic acid .
• Iipilisi zokulawula inzala:Ezinye iintlobo zeepilisi zokulawula ukukhulelwa zithintela ukopha kakhulu ngexesha lokuya exesheni.
• Izongezo okanye iinaliti zeVitamin K: Ukuba imeko yakho ibangelwa kukunqongophala kweVitamin K, ukufumana iVitamin K engaphezulu kunokunceda umzimba wakho wenze iFactor VII engaphezulu.

Zithini iingxaki/iziphumo ebezingalindelekanga zonyango?

Unyango olongeza i-factor VII egazini ngamanye amaxesha lunokubangela iingxaki. Nangona kunjalo, kubalulekile ukukhumbula ukuba ugqirha wakho uyazazi ezi ngozi kwaye uthatha amanyathelo okuzikhusela.

Iingxaki ezinokubakho zonyango lweFactor VII zingabandakanya:

• Amahlwili egazi ayingozi: Ukongeza i-factor VII kunokunyusa umngcipheko wamahlwili egazi anokuvala imithambo yegazi. Ugqirha wakho uza kujonga umthamo wamayeza akho ukuze anciphise lo mngcipheko.
• Ukuvezwa kwiintsholongwane: Xa ufumana igazi elinikelweyo, kusoloko kukho umngcipheko wokuvezwa kwiintsholongwane eziyingozi (umz., iintsholongwane okanye iibhaktheriya) egazini. Oogqirha bahlola ngononophelo iimveliso zegazi ukuthintela oku.
• Iimpendulo zotofelo-gazi: Usenokuba ne-allergy kwi-plasma enikelweyo.
• Ukugqithisa kweplasma: I-Factor VII iyaqhekeka ngokukhawuleza kwaye isuswe egazini lakho. Ukuthintela oku, unokufuna inani elikhulu leplasma ukuze ugcine i-factor VII eyaneleyo emzimbeni wakho. Oku kunokubangela ukwanda kweplasma kwinkqubo yakho. Iziphumo zokugqithisa kweplasma zinokubandakanya uxinzelelo lwegazi oluphezulu kunye nokudumba kwemiphunga (pulmonary edema) .

Yintoni endinokuyilindela ukuba ndinesiphene seFactor VII?

Ukunqongophala kwe-Factor VII yimeko ehlala ubomi bonke . Nangona kungekho nyango, ungasebenzisana nogqirha wakho ukuyilawula.

Enye yezona ngxaki zinkulu ekuqinisekiseni ukuba esi sifo siza kuba sibi kangakanani kukuba akunakwenzeka ukuqikelela ubukhali beempawu ngokusekelwe ekubeni amanqanaba akho eFactor VII aphantsi kangakanani. Endaweni yoko, oogqirha basebenza nabantu abanobu buthathaka ngokwabantu ngabanye. Imbono yakho iya kuxhomekeka kwizinto ezikhethekileyo kuwe, ezifana namava akho, yintoni ebangele ukungabikho, indlela osabela ngayo kunyango, kunye nokuba ukopha kwakho kukhulu kangakanani.

Ndingazinyamekela njani?

Kubalulekile ukuxelela bonke ababoneleli bezempilo abakunyangayo ngemeko yakho. Ukuba umntwana wakho une-factor VII deficiency, xelela nabani na omkhathalelayo (kuquka ootitshala kunye nabasebenzi be-daycare).

Buza ugqirha wakho ngezinto onokuzenza ukuze uzinyamekele imihla ngemihla. Usenokucebisa izinto ezifana nezi:

• Ziphephe izinto ezinobungozi obukhulu:Usenokufuna ukuphepha imisebenzi eyonyusa umngcipheko wokwenzakala (umz., imidlalo yokudibanisa umzimba).
• Ziphephe ezinye iindlela zonyango: Kusenokufuneka uhlale kude namayeza anciphisa igazi lakho (umz., i-aspirin , ii-NSAID , ii-SSRI antidepressants ). Usenokufuna ukuphepha nezinye izongezo ezineoyile yentlanzi, i-vitamin E, kunye ne-turmeric, kuba ezi zinokunyusa ukopha.
• Gcina ikopi yesicwangciso sakho sonyango: Kubalulekile ukugcina amaxwebhu avela kwingcali yakho ye-hematologist malunga nendlela omele uphathwe ngayo ziingcali zempilo xa kukho imeko engxamisekileyo.
• Fumana ingcebiso okanye uvavanyo lwemfuza: Ngaphambi kokuba uzame ukuba nomntwana, ungathetha nomcebisi wemfuza ukuze uvavanye umngcipheko womntwana wakho wokufumana ukungabikho kwe-factor VII.

Ndifanele ndimbone nini ugqirha wam?

Ukuba wena okanye umntwana wakho nibona naziphi na iimpawu zokopha okungapheliyo okanye ukuba luhlaza okwesibhakabhaka rhoqo, bonani ugqirha wenu. Ukopha empumlweni kunye nokuba luhlaza okwesibhakabhaka ngamanye amaxesha ziqhelekile. Kodwa ukuba zenzeka ngaphezu kwesiqhelo, inokuba luphawu lwengxaki yegazi.

Ndingaya nini kwiYunithi yoNyango oluNgxamisekileyo (i-ETU) ?

Ukuba ukopha kwakho kuyingozi kwimpilo yakho, yiya kwigumbi likaxakeka. Khangela iimpawu ezifana nezi:

• Ukopha kwempumlo okuhlala iyure nangaphezulu.
• Ukumanzisa iipads okanye iitampon ezingaphezu kwezibini ngeyure kangangeeyure ezimbini nangaphezulu (kubafazi).

Ndingayibuza yiphi imibuzo ugqirha wam?

Nantsi eminye imibuzo onokuyibuza ugqirha wakho:

• Ingaba ukusilela kwam kwiFactor VII kuzuzwe njengelifa okanye kwavela kamva?
• Luhlobo luni lonyango endiludingayo?
• Kuza kufuneka ndifumane unyango ixesha elingakanani?
• Zeziphi iziphumo ebezingalindelekanga ekufuneka ndizazi ngexesha lonyango?
• Zeziphi iinguqu kwindlela yokuphila ekufuneka ndizenze ukuze ndilawule imeko yam?

Ingaba ukunqongophala kweFactor VII kuyafana 'ne-hemophilia'?

Ukunqongophala kweFactor VII kunye ne-hemophilia ziingxaki ezimbini zegazi ezinxulumeneyo, kodwa ezahlukeneyo, ezizuzwe njengelifa. Ukunqongophala kweFactor VII kubangelwa kukunqongophala kweproteni iFactor VII (7), ngelixa i-hemophilia A , uhlobo oluqhelekileyo lwe-hemophilia, ibangelwa kukunqongophala kweproteni iFactor VIII (8).

I-Hemophilia A ixhaphake kakhulu kune-factor VII deficiency, ichaphazela umntu omnye kwabayi-100,000. Ikwaxhaphake kakhulu kumadoda .

Kodwa eyona nto ibalulekileyo kukuba zombini ezi meko zinokubangela ukopha kakhulu, ngoko ke kufuneka zilawulwe kakuhle ngoncedo lukagqirha.

Izinto ezibaluleke kakhulu ekufuneka uzikhumbule

Akukho ndlela yokuthintela ukuzalwa unesifo sokugalela igazi esibizwa ngokuba yi-blood clotting disorder VII deficiency. Kodwa oko akuthethi ukuba wena (okanye umntwana wakho) aninakuphila ubomi obusempilweni nobusebenzayo xa ninesi sifo. Ingcali yakho ye-hematologist inokukunceda uthintele kwaye ulawule ukopha. Inokukunceda wenze isicwangciso sento omawuyenze ukuba wopha kakhulu. Ungakhathazeki, ungaphila ngempumelelo nale meko.

Ukunqongophala kweFactor VII, Ukuvaleka kweGazi, Ukopha, Izifo ezidluliselwa kwiLifa, Isifo sika-Alexander, Ingcali yeHematologist, Izifo zeGazi