Yu gɛt kɔndishɔn bak we ivin smɔl kɔt nɔ de stɔp fɔ blɔd? (Faktɔ VII Difisiɛns) .

Sɔntɛnde yu kin fil lɛk se i kin tek lɔng tɛm fɔ mek ivin smɔl kɔt fɔ lɛ i stɔp fɔ blɔd? Ɔ yu jɔs de tɔn blu ɛn brus? Sɔntɛm rizin de biɛn dɛn tin ya we wi nɔ no bɔt. Na dat Factor VII defisiɛns bi.

Wetin na Factor VII Defisiɛns?

Fɔ tɔk am simpul wan, Factor VII deficiency na wan jenɛtik kɔndishɔn we yu blɔd nɔ de klɔt fayn, we min se i nɔ de stɔp fɔ blɔd kwik kwik wan. difrεn kayn protin dεm de na wi bכdi we dεn kכl ‘blood clotting factors’ כ ‘coagulation factors’ . Factor VII na wan pan dɛn kayn prɔtin dɛn de. Ɔl dɛn prɔtin ya de wok togɛda wit yu pletlɛt dɛn fɔ mek blɔd klɔt fɔ stɔp fɔ blɔd we yu wund. Dis prɔses tan lɛk fɔ bil wɔl wit brik ɛn simen. So, if yu nɔ gɛt dis impɔtant prɔtin we dɛn kɔl Factor VII na yu bɔdi, ɔ if i nɔ de wok fayn, i kin tek lɔng tɛm fɔ mek yu blɔd klot pas aw i kin tek. Dis na di rizin we mek yu brus ɛn blɔd izi pas ɔda pipul dɛn.

Ɔda nem dɛn fɔ dis na Alɛgzanda in sik ɛn prokɔnvɛtin dɛfisiɛns .

Aw kɔmɔn dis factor VII dɛfisiɛns?

Dis na rili wan tin we nɔ kin apin so ɔltɛm . Ɔlsay na di wɔl, i kin afɛkt lɛk wan pan ɛvri 500,000 pipul dɛn . Bɔrku blɔd klɔt disɔda wae dɛn kin gɛt frɔm dɛn mama ɛn papa we dɔktɔ dɛn kin kɔl "rare." Factor VII deficiency na wan pan di kɔmɔn pan dɛn sik ya we nɔ kin bɔku.

Wetin na di sayn dɛm fɔ dis?

If yu nɔ gɛt Factor VII, i kin mek yu blɔd fɔ lɔng tɛm ɛn i kin izi fɔ mek yu brus. Di men sayn dɛm wae de sho se yu gɛt dis sik na:

• Di blɔd we de kɔmɔt na di gɔm.
• Nos blɔd (epistaxis) , ɛn i nɔ kin izi fɔ stɔp am.
• Fɔ uman dɛn, bɔku blɔd kin kɔmɔt we dɛn de gɛt mɔnt (menorrhagia).
• Blɔd we de na di urine (hematuria) ɔ blɔd we de na di stɔl.
• Kɔntinyu fɔ blɔd afta i wund, ɔpreshɔn, ɔ bɔn pikin.
• di bכdi de bכn insay di bכdi (fכ egzampl, na di gεstrointestinal sistεm, di tisu dεm, di mכsul dεm, di jכint dεm).
• Blɔd we de kɔmɔt insay di kraynia kin apin bak. Dis na tin we siriɔs smɔl.
• Blɔd kin kɔmɔt bay insɛf we i bigin fɔ blɔd wantɛm wantɛm fɔ no rizin.

Aw dɛn sik ya kin tranga ɛn wae dɛn kin apia kin difrɛn frɔm wan pɔrsin to ɔda pɔrsin. fכ egzampl, dεn kin no se pikin we gεt siriכs Factor VII dεficiency we dεn bכn am. Bɔt, pɔrsin wae gɛt dis sik nɔr kin teik pɔrsin wae gɛt dis sik te i dɔn ol.

Wetin kin mek Factor VII nɔ de?

Pan ɔl we dis na mɔ sik we pɔsin kin gɛt frɔm in mama ɛn papa , wan we de bak we dis sik kin apin we pɔsin de liv.

If dɛn bɔn yu wit factor VII deficiency , dat min se yu dɔn gɛt wan muteshon, ɔ difrɛns, na yu F7 jin . dis jin de kכntro aw yu bכdi de mek fכktכ VII. Fɔ mek yu nɔ gɛt factor VII, yu fɔ gɛt dis jin muteshon frɔm di tu mama ɛn papa we bɔn yu . dis dεn kכl am כtosom rεsεsiv inhεritεns . if dεn tu mama εn papa gεt dis mכtεshכn, dεn gεt bכt 25% chans fכ gεt pikin we gεt fכktכ VII dεfisiεns.

di fכktכ VII dεfisiεns we de divεlכp ova tεm dεn kכl am ‘akwyayz fכktכ VII dεfisiεns’ . Bɔku tin dɛn de we kin mek dis apin:

• Liva sik: Yu liva de mek di Factor VII protin. If yu gɛt siriɔs liva sik, yu liva nɔ go ebul fɔ du dis wok fayn fayn wan.
• Vitamin K we nɔ de: Vitamin K impɔtant fɔ mek di bɔdi mek Factor VII. If yu nɔr gɛt inof pan am, yu kin gɛt Factor VII deficiency.
• Mεdikeshכn dεm we de mek yu blɔd tan: Dis kכndyushכn kin apin bak we yu de yuz mεdikeshכn dεm we de mek yu blɔd tan lɛk Warfarin (Coumadin®) .
• Blɔd sɛl dizayd ɛn kansa: Dɛn dɔn ripɔt se Factor VII deficiency pan pipul dɛm wae gɛt sik dɛm lɛk aplastic anemia ɛn leukemia .

Wetin na di kɔmplikɛshɔn dɛm we Factor VII dɛfisiɛns kin gɛt?

If i siriɔs, di Factor VII we nɔ gɛt bɛtɛ wɛlbɔdi kin mek i gɛt ɔda wɛlbɔdi prɔblɛm if dɛn nɔ trit am. Fɔ ɛgzampul:

• Anemia: We bɔku blɔd de kɔmɔt na di bɔdi, yu rɛd blɔd sɛl kin go dɔŋ bad bad wan.
• di joyn dεm we dεn pwεl: We bכdi de bכn insay di jכint, di tisu we de de kin west εn di jכint nכ kin bεnd כ strεt fayn fayn wan.
• Ɛmatoma: Ɛmatoma na di blɔd we de klɔt ɔnda yu skin. If big ɛmatoma fɔm, i kin put prɛshɔn pan di ɔgan dɛn we de nia am ɛn mek yu gɛt sayn dɛn we de mek yu fil pen.

Di tin we impɔtant pas ɔl na dat, we pɔsin de blɔd bad bad wan, i kin mek i day if i nɔ trit am. Na dat mek i impɔtant fɔ go to dɔktɔ if di blɔd nɔ stɔp.

Aw dɛn kin no se Factor VII defisiɛns?

Yu dɔktɔ go aks yu bɔt yu sik ɛn if ɛnibɔdi na yu famili gɛt histri bɔt blɔd disɔda. Dɛn kin no se yu nɔ gɛt Factor VII wit blɔd tɛst we de chɛk aw yu blɔd kin klɔt fayn fayn wan.

Dɛn tɛst ya na:

• di tεst fכ di prכthrombin tεm (PT/INR) tεst: dis de mכsu aw lכng i tek fכ mek di bכdi kכlכt bay di aktiviti fכ wan bכdi kכlכt fכktכ we dεn kכl prothrombin. If yu gɛt factor VII dɛfisiɛns, i kin tek lɔng pas aw i kin tek fɔ mek blɔd klɔt.
• pat pan tromboplastin tεm (PTT) tεst: dis de mכsu di tεm we i tek fכ blכd kכlכt bay di aktiviti fכ kכlכt fכktכ dεm we nכto prothrombin. Di rizulyt fɔ dis tɛst kin bi nɔmal pan pɔsin we gɛt factor VII dɛfisiɛns.
• Factor VII assay: dis tεst de sho aw di Factor VII protin de wok.

Aw dɛn kin trit di factor VII dɛfisiɛns?

Bɔku tɛm, di tritmɛnt kin min fɔ ad factor VII we de wok fayn fayn wan to yu blɔd. I go mɔs bi se yu go wok wit dɔktɔ we spɛshal pan blɔd dizayd, we dɛn kɔl hematologist , we go disayd us tritmɛnt yu nid ɛn aw lɔng yu go nid am fɔ.

Bɔku men we dɛn de fɔ trit pɔsin:

• Rikombinant factor VIIa (NovoSeven®): Dis mɛrɛsin na di US Food and Drug Administration (FDA) dɔn gri fɔ trit factor VII we nɔ de. Na sayɛnsman dɛn we de na lɛbɔtri mek am. Dis min se, difrɛn frɔm ɔda factor VII riplesmɛnt tritmɛnt dɛm, dɛn nɔ de mek am wit blɔd we dɛn gi frɔm pipul dɛm.
• Prothrombin complex concentrates (PCC): Dis tritmεnt gεt hכy kכnsantreshכn fכ fכ klot fכktכ dεm, inklud fכktכ VII, we dεn tek frכm blכd we dεn gi.
• Fresh frozen plasma (FFP): Dɛn kin gi yu plasma bak insay yu bɛlɛ fɔ ɛp fɔ bil yu Factor VII stoa dɛn bak.

Ɔda tritmɛnt dɛn:

• Antifibrinolytic agents: Dɛn mɛrɛsin ya de wok bay we dɛn de stɔp di blɔd we de kam na yu bɔdi fɔ mek i nɔ sɔlv. Dɛn kin ɛp fɔ mek yu nɔ blɔd pasmak bay we dɛn de protɛkt di blɔd we dɔn ɔlrɛdi de na yu bɔdi. Sɔm ɛgzampul dɛn na drɔgs dɛn we dɛn kɔl aminocaproic acid ɛn tranexamic acid .
• Pils dɛn we dɛn kin yuz fɔ kɔntrol pikin dɛn:Sɔm kayn bɔn kɔntrol pils kin mek yu nɔ gɛt bɔku blɔd we yu de gɛt mɔnt.
• Vitamin K supliment ɔ injɛkshɔn: If yu sik na bikɔs yu nɔ gɛt vaytamɛn K, fɔ gɛt mɔ vaytamɛn K kin ɛp yu bɔdi fɔ mek mɔ factor VII.

Wetin na di kɔmplikɛshɔn/sayd ɛfɛkt dɛm fɔ di tritmɛnt?

Tritmɛnt dɛn we de ad factor VII to di blɔd kin mek sɔm tɛm dɛn gɛt prɔblɛm dɛn. Bɔt i impɔtant fɔ mɛmba se yu dɔktɔ no bɔt dɛn bad tin ya ɛn i de du tin fɔ mek yu nɔ gɛt dɛn.

Di kɔmplikeshɔn dɛn we kin apin we Factor VII tɛrapi kin bi:

• Blɔd we de klɔt bad bad wan: If yu ad factor VII, i kin mek yu gɛt mɔ blɔd we kin blok di blɔd vesel dɛn. Yu dɔktɔ go wach yu mɛrɛsin doz fɔ ridyus dis risk.
• Fɔ gɛt di sik we de mek pɔsin gɛt sik: We dɛn de gɛt di blɔd we dɛn dɔn gi, i kin ɔltɛm gɛt risk fɔ mek i gɛt bad bad sik dɛn (e.g., vayrɔs ɔ baktri) na di blɔd. Dɔktɔ dɛn kin tek tɛm luk di tin dɛn we dɛn mek wit blɔd fɔ mek dis nɔ apin.
• Transfyushɔn riakshɔn: Yu kin gɛt alɛji fɔ plasma we dɛn gi yu.
• Plasma ɔvalod: Factor VII kin brok tu kwik ɛn pul am na yu blɔd. Fɔ mek dis nɔ apin, yu kin nid bɔku bɔku plasma fɔ kip inof factor VII na yu bɔdi. Dis kin mek di plasma na yu sistɛm go ɔp. di ifekt dεm we plasma ovalod kin inklud hεy blכd prεshכn εn swεla na di lכng dεm (pulmonary edema) .

Wetin a kin ɛkspɛkt if a gɛt Factor VII dɛfisiɛns?

Factor VII deficiency na wan sik we de apin fɔ ɔl yu layf . Pan ɔl we no mɛrɛsin nɔ de, yu kin wok wit yu dɔktɔ fɔ mɛn am.

Wan pan de big chalenj fɔ no aw di sik go tranga na dat i nɔr pɔsibul fɔ no aw di sik go siriɔs bay aw yu Factor VII lɛvɛl smɔl. Bifo dat, dɔktɔ dɛn kin wok wit pipul dɛn we gɛt dis prɔblɛm fɔ dɛnsɛf. Di we aw yu go si tin go dipen pan tin dɛn we yu nɔ go si, lɛk di ɛkspiriɛns we yu gɛt, wetin mek yu nɔ gɛt di sik, aw yu de du tritmɛnt, ɛn aw yu blɔd kin rili bad.

Aw a kin kia fɔ misɛf?

E fayn fɔ tɛl ɔl di wɛl bɔdi biznɛs pipul dɛm wae de trit yu bɔt yu sik. If yu pikin gɛt factor VII dɛfisiɛns, tɛl ɛnibɔdi we de kia fɔ am (inklud ticha ɛn daycare wokman dɛm).

Aks yu dɔktɔ bɔt tin dɛn we yu kin du fɔ kia fɔ yusɛf ɛvride. I kin rikɔmɛnd tin dɛn lɛk:

• Nɔ du tin dɛn we gɛt bɔku prɔblɛm:Yu kin want fɔ avɔyd tin dɛn we de mek yu gɛt mɔ injuri (e.g., kɔntakt spɔt).
• Nɔ tek sɔm mɛrɛsin dɛn: Yu kin nid fɔ de fa frɔm mɛrɛsin dɛn we de mek yu blɔd tan (e.g., aspirin , NSAIDs , SSRI antidipreshan ). Yu kin want bak fɔ avɔyd supamakit dɛn we gɛt fish ɔyl, vaytamɛn E, ɛn tumara, bikɔs dɛn tin ya kin mek bak blɔd bɔku.
• Kip wan kɔpi fɔ yu tritmɛnt plan: I impɔtant fɔ kip di dɔkyumɛnt frɔm yu ɛmatɔlɔjis bɔt aw di wɛlbɔdi biznɛs pipul dɛn fɔ trit yu if ɛnitin apin.
• Gɛt jenɛtik kɔyl ɔ tɛst: Bifo yu tray fɔ bɔn pikin, yu kin tɔk to jenɛtik kɔyl fɔ asɛs yu pikin in risk fɔ gɛt inhɛrit factor VII dɛfisiɛns.

Ustɛm a fɔ go to mi dɔktɔ?

If yu ɔ yu pikin notis ɛni sayn we de sho se i de blɔd ɔltɛm ɔ i de blu ɔltɛm, go to yu dɔktɔ. Nos blɔd ɛn blu wan wan tɛm na nɔmal tin. Bɔt if dɛn kin apin pas aw i kin apin, dat kin bi sayn fɔ se pɔsin gɛt blɔd prɔblɛm.

Ustɛm a fɔ go na di Imejɛnsi Tritmɛnt Yunit (ETU) ?

If yu blɔd de ambɔg yu wɛlbɔdi, go na di imejensi rum. Luk fɔ sayn dɛn lɛk dɛn wan ya:

• Nos blɔd we kin las fɔ wan awa ɔ mɔ.
• Wet pas tu pad ɔ tampon pan awa fɔ tu awa ɔ mɔ (fɔ uman dɛn).

Us kwɛstyɔn dɛn a fɔ aks mi dɔktɔ?

Na sɔm kwɛstyɔn dɛn we yu kin aks yu dɔktɔ:

• Mi Factor VII deficiency na hereditary ɔ i bin divɛlɔp leta?
• Us kayn tritmɛnt a nid?
• Aw lɔng a go gɛt fɔ gɛt tritmɛnt?
• Us sayd ɛfɛkt dɛn a fɔ no bɔt we a de du tritmɛnt?
• Us chenj dɛn we a nid fɔ chenj mi layf fɔ mek a ebul fɔ kɔntrol mi sik?

Factor VII deficiency na di sem wit ‘hemophilia’?

Factor VII deficiency ɛn hemophilia na tu tin dɛn we gɛt fɔ du wit blɔd we gɛt fɔ du wit dɛnsɛf, bɔt we difrɛn, we dɛn kin gɛt frɔm dɛn mama ɛn papa. Fכktכ VII dεfichεshכn de kכz fכ di dεfisit fכ di protin Factor VII (7), we εmofilia A , di tכp we kכmכn fכ εmofilia, de kכz fכ difεshכn fכ di protin Factor VIII (8).

Ɛmofilia A kin bɔku pas fɔ gɛt factor VII, i kin afɛkt lɛk wan pan ɛvri 100,000 pipul dɛn. I kin bɔku bak pan man dɛn .

Bɔt di tin we impɔtant pas ɔl na dat, dɛn tu tin ya kin mek pɔsin blɔd pasmak, so dɛn nid fɔ kɔntrol dɛn fayn fayn wan wit dɔktɔ in ɛp.

Di tin dɛn we impɔtant pas ɔl we wi fɔ mɛmba

Nɔ we nɔ de fɔ mek dɛn nɔ bɔn am wit di blɔd klɔt disɔda factor VII dɛfisiɛns. Bɔt dat nɔ min se yu (ɔ yu pikin) nɔ go ebul fɔ liv wɛl, aktif layf wit dis sik. Yu dɔktɔ we de mɛn yu blɔd kin ɛp yu fɔ mek yu nɔ blɔd ɛn kɔntrol yu. I kin ɛp yu bak fɔ mek plan fɔ wetin fɔ du if yu blɔd bɔku. Nɔr wɔri, yu kin liv fayn fayn wan wit dis kɔndishɔn.

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