Kodi inunso simuvutika kusiya kutuluka magazi? Tiyeni tiphunzire za matenda a hemophilia!

Kodi mudamvapo za matenda otchedwa Hemophilia ? Mungamve mantha pang'ono mukamva dzinali. Koma musadandaule, chifukwa uwu ndi matenda osowa kwambiri. Lero, tikambirana za hemophilia, momwe imakulira, zizindikiro zake, komanso ngati pali chithandizo, zonsezi m'njira yosavuta yomwe mungamvetse.

Kodi hemophilia ndi chiyani?

Mwachidule, hemophilia ndi matenda obadwa nawo a magazi omwe amapezeka mwa ana. Izi zikutanthauza kuti magazi anu sagwirana bwino. Izi zingayambitse kutuluka magazi ambiri, kapena ngakhale kuvulala pang'ono.

Taganizirani izi, pali mapuloteni apadera m'matupi athu omwe amathandiza magazi kuundana. Timatcha izi 'zinthu zoundana' . Ali ngati asilikali ang'onoang'ono omwe amagwira ntchito yoletsa kutuluka kwa magazi. Mukayamba kudwala hemophilia, thupi silipanga zokwanira za 'zinthu zoundana' izi. Kenako magazi amaundana pang'ono ndipo mumatuluka magazi ambiri.

Pali mitundu ikuluikulu ya hemophilia. Matendawa akhoza kukhala aakulu, apakati, kapena ofatsa, kutengera kuchuluka kwa zinthu zomwe zimayambitsa magazi kuundana.

Nkhani yabwino ndi yakuti pali njira yochizira izi. Madokotala akuyesera kubwezeretsa zinthu zomwe zimapangitsa kuti magazi azitsekeka m'thupi. Pakadali pano palibe mankhwala a hemophilia. Komabe, anthu omwe ali ndi vutoli nthawi zambiri amakhala ndi moyo wautali ngati ena omwe ali ndi chithandizo choyenera. Asayansi akufufuzanso njira zatsopano, monga jini therapy , kuti aone ngati zingatheke kuchiritsidwa.

Kodi hemophilia imayamba kubereka?

Inde, zimatha kuchitika. Koma sizichitika kawirikawiri. Izi zimatchedwa 'acquired hemophilia'. Izi zikutanthauza kuti sizitengera cholowa. Chomwe chimachitika apa ndichakuti mapuloteni oteteza thupi lathu , otchedwa ma antibodies , nthawi zina amaukira molakwika chimodzi mwazinthu zomwe timapanga magazi. Ma antibodies amenewa omwe amaukira molakwika amatchedwa 'autoantibodies' .

Kodi hemophilia ndi matenda ofala?

Ayi, ayi konse. Uwu ndi matenda osowa kwambiri. Malinga ndi ziwerengero ku United States (CDC Ogasiti 2022), anthu pafupifupi 33,000 kumeneko ali ndi hemophilia. Nthawi zambiri imakhudza amuna. Nthawi zambiri, akazi amathanso kukhala ndi zizindikiro monga kuchepa kwa magazi m'magazi komanso kutuluka magazi ambiri panthawi ya msambo.

Kodi mitundu ya hemophilia ndi iti?

Pali mitundu itatu ikuluikulu ya hemophilia:

• Hemophilia A: Mtundu uwu ndi wofala kwambiri. Umayambitsidwa ndi kusowa kwa chinthu cholimbitsa magazi nambala 8, chomwe chimadziwikanso kuti factor VIII., pamene thupi silikukwanira. Pafupifupi anthu 10 pa anthu 100,000 akhoza kukhala ndi vutoli.
• Hemophilia B: Izi zimachitika chifukwa cha kusowa kwa chinthu cholimbitsa magazi chotchedwa factor 9, kapena factor IX . Pafupifupi anthu atatu pa anthu 100,000 ali ndi mtundu uwu.
• Hemophilia C: Imatchedwanso kuti factor XI deficiency , izi sizichitika kawirikawiri, ndipo zimakhudza pafupifupi munthu m'modzi pa anthu miliyoni.

Kodi hemophilia ndi matenda oopsa?

Inde, nthawi zina izi zimakhala zoopsa kwambiri. Anthu omwe ali ndi vuto la magazi ochulukirapo amatha kutuluka magazi m'njira yomwe ingawopseze moyo, yomwe timaitcha kuti 'kutuluka magazi.' Koma nthawi zambiri magazi amatuluka m'minofu kapena m'malo olumikizirana mafupa.

Kodi zizindikiro za hemophilia ndi ziti?

Zizindikiro zofunika kwambiri ndi kutuluka magazi ndi mabala osazolowereka kapena ochulukirapo.

Zizindikiro zomwe zimawoneka kawirikawiri

Anthu omwe ali ndi vuto la hemophilia amatha kuvulala kwambiri ngakhale chifukwa cha ngozi zazing'ono. Izi zikutanthauza kuti magazi akutuluka pansi pa khungu.

• Mwina pambuyo pa opaleshoni, pambuyo pochotsa dzino, kapena ngakhale kudula chala , kutuluka magazi kumatha kwa nthawi yayitali kwambiri.
• Kutuluka magazi m'mphuno kungachitike mwadzidzidzi popanda chifukwa.

Kuchuluka kwa mabala/kutuluka magazi komwe muli nako kumadalira ngati muli ndi hemophilia yoopsa, yapakati, kapena yocheperako.

• Anthu omwe ali ndi vuto lalikulu la magazi m'thupi (hemophilia) amatha kutuluka magazi pafupipafupi popanda chifukwa chomveka.
• Anthu omwe ali ndi vuto la kuchepa kwa magazi m'thupi (hemophilia) amatha kutuluka magazi kwa nthawi yayitali ngati achita ngozi yaikulu.
• Anthu omwe ali ndi hemophilia yochepa amatuluka magazi osazolowereka akachitidwa opaleshoni yayikulu kapena ngozi yayikulu.

Pakhoza kukhalanso zizindikiro zina:

• Kupweteka kwa mafupa chifukwa cha magazi omwe amasonkhana m'thupi. Malo monga akakolo, mawondo, chiuno, ndi mapewa amatha kupweteka, kutupa, kapena kumva kutentha akakhudza.
• Kutuluka magazi muubongo. Izi zimachitika kawirikawiri kwa anthu omwe ali ndi vuto la hemophilia yoopsa. Ngati muli ndi vuto la magazi muubongo, mutha kukhala ndi mutu wopweteka nthawi zonse, kusawona bwino, kapena kugona tulo tofa nato kwambiri . Ngati muli ndi vuto la hemophilia, muyenera kuonana ndi dokotala nthawi yomweyo ngati mukukumana ndi zizindikiro izi.

Zizindikiro mwa makanda ndi ana aang'ono

Nthawi zina, hemophilia imapezeka mwana wamwamuna akadulidwa ndipo amatuluka magazi ambiri kuposa masiku onse. Nthawi zina, ana amasonyeza zizindikiro miyezi ingapo atabadwa. Zizindikiro zodziwika kwambiri ndi izi:

• Kutuluka magazi:Makanda amatha kutuluka magazi mkamwa mwawo akaluma chidole chaching'ono.
• Matumbo otupa pamutu: Makanda ndi ana aang'ono amatha kukhala ndi matumbo akuluakulu ozungulira (mazira a atsekwe) akagunda mitu yawo.
• Kulira pafupipafupi, kusakhazikika, komanso kusafuna kukwawa kapena kuyenda: Izi zitha kuchitika ngati magazi alowa mu minofu kapena malo olumikizirana mafupa. Malowa angamve ngati ali ndi mabala, kutupa, kutentha kwambiri akakhudza, kapena mwana angamve kupweteka ngakhale atakhudzidwa pang'ono.
• Ma hematoma: 'Hematoma' ndi gulu la magazi omwe amaundana pansi pa khungu mwa makanda ndi ana aang'ono. Mitundu iyi ya ma hematoma imathanso kuchitika pambuyo pa jakisoni.

Kodi ndi zinthu ziti zomwe zimayambitsa hemophilia?

'Zinthu zoundana' izi zimapangidwa kuchokera ku majini ena m'thupi lathu. Mu hemophilia yobadwa nayo, majini omwe amaphunzitsa kupanga 'zinthu zoundana' izi amasintha, kutanthauza kuti amasintha. Majini osinthika awa amatha kupanga 'zinthu zoundana' zolakwika kapena osapanga 'zinthu zoundana' zokwanira. Komabe, pafupifupi 20% ya odwala hemophilia amabadwa mwangozi, zomwe zikutanthauza kuti amatha kudwala matendawa ngakhale palibe aliyense m'banjamo amene wakhalapo nawo kale.

Kodi hemophilia imabadwa bwanji?

Mitundu yonse iwiri ya hemophilia A ndi B ndi matenda okhudzana ndi kugonana. Amabadwa nawo mwanjira yolumikizana ndi X. Tiyeni tiwone momwe zimachitikira:

• Tonsefe timapeza seti imodzi ya ma chromosome kuchokera kwa amayi athu ndi seti imodzi ya ma chromosome kuchokera kwa abambo athu. Ngati mwapeza X chromosome kuchokera kwa amayi anu ndi X chromosome kuchokera kwa abambo anu, ndinu mtsikana. Ngati mwapeza X chromosome kuchokera kwa amayi anu ndi Y chromosome kuchokera kwa abambo anu, ndinu mnyamata. Mwachidule, amayi nthawi zonse amapatsa mwana wawo X chromosome. Kugonana kwanu kumadalira ngati abambo anu akupatsani X kapena Y chromosome.
• Ngati mkazi ali ndi vuto mu jini lomwe limapanga 'clotting factor' iyi pa imodzi mwa ma chromosome ake awiri a X, akhoza kukhala ndi vuto la hemophilia, koma sangasonyeze zizindikiro chifukwa ali ndi jini yathanzi pa chromosome yake ina ya X.
• Ngati mayi amene ali ndi chromosome ya X yolakwika ngati iyi ali ndi mwana wamwamuna, pali mwayi wa 50% kuti mwanayo adzalandira chromosome ya X yolakwika. Ngati zimenezo zitachitika, mwana wamwamunayo adzakhala ndi vuto la magazi lochepa.
• Ngati mayiyo ali ndi mwana wamkazi, pali mwayi wa 50% kuti mwanayo adzalandira chromosome ya X yolakwika. Komabe, sangasonyeze zizindikiro chifukwa cholandira chromosome ya X yathanzi kuchokera kwa abambo ake. Kenako mwana wamkaziyo adzakhalanso ndi kachilomboka.

Izi zikutanthauza kuti mkazi amene walandira chromosome ya X yolakwika amakhala ndi vuto la haemophilia. Mwina sangakhale ndi zizindikiro, koma akhoza kupatsira matendawa kwa ana ake. Mwana aliyense amene ali naye ali ndi mwayi wa 50% wolandira haemophilia.Ana aamuna amakhala ndi mwayi waukulu wowonetsa zizindikiro ngati alandira hemophilia, chifukwa salandira chromosome ya X yathanzi kuchokera kwa abambo awo.

Kodi atsikana nawonso amakhala ndi zizindikiro za hemophilia?

Inde, zimatha kuchitika. Koma zizindikiro zake nthawi zambiri zimakhala zochepa. Mwachitsanzo, mayi amene ali ndi jini la hemophilia sangakhale ndi zinthu zokwanira zolimbitsa magazi. Ngati izi zitachitika, akhoza kutuluka magazi ambiri nthawi ya msambo, kuvulala mosavuta, kutuluka magazi ambiri atabereka, kapena kutuluka magazi m'malo olumikizirana mafupa, zomwe zimayambitsa mavuto a mafupa.

Kodi madokotala amazindikira bwanji hemophilia?

Dokotala adzakufunsani kaye mbiri yonse ya matenda anu ndikukuyesani. Ngati muli ndi zizindikiro za hemophilia, adzakufunsaninso za mbiri ya banja lanu. Akhoza kuchita mayeso monga:

• Kuchuluka kwa Magazi Okwanira (CBC): Izi zimachitika kuti ziwone mitundu ya maselo m'magazi.
• Kuyesa kwa Prothrombin Time (PT): Izi zingathandize kuona momwe magazi anu amaundana mofulumira.
• Kuyesa kwa Partial Thromboplastin Time (PTT): Iyi ndi njira ina yoyezera nthawi yomwe magazi amaundana.
• Mayeso enieni a magazi oundana: Mayeso a magazi awa amayesa kuchuluka kwa magazi oundana, monga factor VIII ndi factor IX .

Kodi kuchuluka kwa zinthu zotsekeka magazi m'thupi ndi kotani?

Zinthu zotsekereza magazi ndi zinthu zomwe zimathandiza kuchepetsa kutuluka kwa magazi. Madokotala amagawa hemophilia kukhala yofatsa, yapakati, kapena yoopsa kutengera kuchuluka kwa zinthu zotsekereza magazi m'magazi mwanu:

• Anthu omwe ali ndi zinthu zotsekereza magazi pakati pa 5% ndi 30% ya kuchuluka kwa magazi m'thupi amakhala ndi hemophilia yocheperako.
• Anthu omwe ali ndi zinthu zotsekereza magazi pakati pa 1% ndi 5% ya kuchuluka kwa magazi m'thupi amakhala ndi hemophilia yocheperako.
• Anthu omwe ali ndi zinthu zosakwana 1% zomwe zimayambitsa magazi kuundana amakhala ndi vuto lalikulu la magazi kuundana.

Kodi mankhwala a hemophilia ndi otani?

Madokotala amachiritsa matenda a hemophilia mwa kuwonjezera kuchuluka kwa zinthu zotsekereza magazi zomwe zimakhala zochepa kapena kusintha zinthu zotsekereza magazi zomwe sizikupezeka. Izi zimatchedwa chithandizo chobwezeretsa magazi .

Mu chithandizo cholowa m'malo ichi, mumapatsidwa zinthu zoundana zopangidwa kuchokera ku plasma ya anthu (ma plasma a anthu) kapena zinthu zoundana zomwe zimatchedwanso 'clotting factors'. Nthawi zambiri, anthu omwe ali ndi hemophilia yayikulu okha ndi omwe amafunikira chithandizo cholowa m'malochi nthawi zonse. Anthu omwe ali ndi hemophilia yocheperako mpaka yocheperako amapatsidwa chithandizochi ngati akufuna kuchitidwa opaleshoni. Amapatsidwa mankhwala oletsa fibrinolytics.Mankhwala omwe amaletsa magazi kuundana kuti asasungunuke angaperekedwenso.

Zinthu zimenezi zimapangidwa kuchokera ku magazi a anthu omwe aperekedwa. Zimayeretsedwa ndi kuyesedwa kuti zichepetse chiopsezo chofalitsa matenda opatsirana monga chiwindi ndi HIV . Zinthu zimenezi zimaperekedwa ngati njira yolowetsera magazi m'mitsempha (IV) .

Ngati muli ndi vuto la magazi ochulukirapo ndipo mukutuluka magazi pafupipafupi, dokotala wanu angakupatseni mankhwala otchedwa 'prophylactic factor infusions' kuti mupewe kutuluka magazi.

Kodi pali zovuta zilizonse pa chithandizochi?

Anthu ena omwe amalandira chithandizo cholowa m'malochi amapanga ma antibodies , otchedwa inhibitors , omwe amalimbana ndi zinthu zotsekereza magazi zomwe amapatsidwa. Madokotala amagwiritsa ntchito njira yotchedwa Immune Tolerance Induction (ITI) pochiza izi. ITI imaphatikizapo kuwapatsa jakisoni wa zinthu zotsekereza magazi tsiku lililonse kuti achepetse kuchuluka kwa zinthu zotsekereza magazi. Imeneyi ikhoza kukhala njira yochizira nthawi yayitali, ndipo anthu ena angafunike chithandizochi kwa miyezi kapena zaka.

Kodi hemophilia ingapewedwe?

Ayi, sizingatheke. Ngati muli ndi vuto la hemophilia ndipo muli ndi ana, dokotala wanu angakulangizeni kuti muyesedwe majini . Mwanjira imeneyi, inu ndi ana anu mutha kudziwa ngati angapereke hemophilia kwa mbadwo wotsatira.

Kodi munthu wodwala matenda a hemophilia ayenera kukhala ndi chiyembekezo chotani?

Ngati muli ndi vuto la hemophilia, mungafunike chithandizo chamankhwala kwa moyo wanu wonse. Kuchuluka kwa chithandizo chomwe mukufuna kumadalira mtundu wa hemophilia yomwe muli nayo, kuopsa kwake, komanso ngati mumakhala ndi 'zoletsa' kapena ayi.

Kodi munthu amene ali ndi vuto la hemophilia amakhala ndi moyo wautali bwanji?

Malinga ndi bungwe la World Federation of Hemophilia, nthawi ya moyo ya mwamuna yemwe ali ndi hemophilia ndi yochepa ndi zaka 10 kuposa ya mwamuna yemwe alibe hemophilia, malinga ndi deta ya 2012 kuchokera ku bungwe la World Federation of Hemophilia. Komabe, iwo amati ana omwe ali ndi hemophilia omwe amapezeka ndi kuchiritsidwa ali aang'ono amakhala ndi nthawi ya moyo wabwinobwino .

Koma si onse omwe ali ofanana. Chomwe chili choona kwa munthu wina sichingakhale choona kwa wina. Ngati inu kapena mwana wanu ali ndi vuto la hemophilia, funsani dokotala zomwe mungayembekezere. Iye amadziwa bwino momwe mwana wanu/wanu alili komanso thanzi lake.

Kodi ndimadzisamalira bwanji?

Anthu omwe ali ndi vuto la hemophilia angafunike chithandizo chamankhwala nthawi zonse kuti apewe kutuluka magazi. Angafunikenso kusiya zochita zina ndi mankhwala. Komabe, pali zinthu zambiri zomwe mungachite kuti muchepetse mavuto omwe hemophilia imakumana nawo pa moyo wanu.

Zinthu zosayenera kuchita

Zinthu zomwe ena savutika nazo kugwa, kugwa, kapena kupunthwa zingayambitse mavuto aakulu kwa anthu omwe ali ndi vuto la hemophilia. Ayenera kupewa zinthu zomwe zimawaika pachiwopsezo chachikulu chogwa kapena kumenyedwa kwambiri. Zitsanzo:

• Kuchita masewera monga mpira wamiyendo, hockey, ndi rugby.
• Kuchita nawo zinthu monga nkhonya ndi kumenyana.
• Kukwera njinga yamoto.
• Kusewera pa skateboard.

Masewera ena, monga mpira wamiyendo, basketball, ndi baseball, nawonso ali ndi chiopsezo chachikulu chovulala. Ngati mukufuna kusewera masewerawa, lankhulani ndi dokotala wanu za zinthu monga kugwiritsa ntchito zida zodzitetezera.

Mankhwala omwe si abwino kumwa

Mankhwala opha ululu monga aspirin, ibuprofen, ndi naproxen amaletsa magazi kuundana. Komanso, kumwa mankhwala oletsa magazi kuundana monga heparin kapena warfarin si lingaliro labwino.

Zinthu zomwe mungachite kuti mukhale ndi moyo wabwino

Pali zinthu zambiri zomwe mungachite kuti muchepetse zotsatira za hemophilia pa moyo wanu:

• Yambani kuchita masewera olimbitsa thupi: Mungakhale ndi nkhawa kuti mungavulale mukuchita masewera olimbitsa thupi. Komabe, lankhulani ndi dokotala wanu za njira zopitirizira kuchita masewera olimbitsa thupi pamene mukuchepetsa chiopsezo chanu chotuluka magazi.
• Kusamalira kupsinjika maganizo : Hemophilia ndi vuto lomwe limakhalapo kwa moyo wonse, ndipo mungafunike kuyesetsa kuti muligwirizanitse ndi banja lanu komanso maudindo anu kuntchito.
• Sungani thanzi labwino la mano: Kutsuka mano, kutsuka mano ndi floss, komanso kupita kwa dokotala wa mano nthawi zonse kungachepetse chiopsezo chofuna chithandizo cha mano chomwe chingayambitse kutuluka magazi. Musaiwale kuuza dokotala wanu za vuto lanu.
• Khalani ndi kulemera kwabwino: Ngati mukuvutika kuyenda chifukwa cha kutuluka magazi mkati ndi kuwonongeka kwa mafupa, kuwongolera kulemera kwanu kungakuthandizeni.
• Dziwitsani anthu omwe ali pafupi nanu: Ngati muli ndi vuto la magazi ochulukirapo, mungakumane ndi matenda otuluka magazi mwadzidzidzi komanso osalamulirika, ngakhale mutamwa mankhwala. Dziwitsani banja lanu zomwe mungachite ngati izi zitakuchitikirani. Ngati mwana wanu ali ndi vuto la magazi ochulukirapo, dziwitsani osamalira ake ndi ogwira ntchito kusukulu zomwe mungachite ngati mwana wanu watuluka magazi.

Kodi ndiyenera kupita kwa dokotala liti?

Ngati muwona kusintha kulikonse m'thupi lanu, monga kutuluka magazi kwambiri kapena kuvulala, pitani kwa dokotala.

Nthawi yoti mupite ku Chipinda cha Zadzidzidzi

Muyenera kupita kuchipatala mwamsanga ngati izi zikuchitika:

• Ngati mutu wanu ukupweteka kwambiri kapena mukumva chizungulire, zizindikirozi zingasonyeze kuti mukutuluka magazi mu ubongo wanu.
• Ngati muli ndi mafupa otupa komanso/kapena opweteka, ndipo mulibe mankhwala osinthira a factor.

Ndi mafunso ati omwe ndiyenera kufunsa dokotala?

Ngati inu kapena mwana wanu mwapezeka ndi matenda a hemophilia, mungafune kufunsa dokotala wanu mafunso monga awa:

• Kodi ine/mwana wanga muli ndi matenda a hemophilia otani?
• Kodi mumalimbikitsa chithandizo chanji?
• Kodi zotsatirapo zake ndi ziti?
• Kodi ine/mwana wanga adzayenera kulandira chithandizo tsiku lililonse?
• Ndi zochita ziti zomwe sizili bwino kwa ine/mwana wanga kuchita?
• Ndi mankhwala ati omwe si abwino kwa ine/mwana wanga kumwa?
• Kodi ndingachite chiyani kuti ndithetse mavuto omwe vutoli likukumana nawo pa moyo wanga/wa mwana wanga?

Uthenga Wopita Nawo Kunyumba

Matenda a magazi otchedwa hemophilia ndi matenda osowa kwambiri, obadwa nawo m'magazi. Angakhudze kwambiri moyo wanu. Anthu omwe ali ndi matenda a magazi otchedwa hemophilia angafunike chithandizo chamankhwala kwa moyo wawo wonse. Makolo a ana omwe ali ndi matenda a magazi otchedwa hemophilia angade nkhawa zoteteza ana awo ku ngozi, komanso kuwaphunzitsa kukhala ndi matenda a magazi otchedwa hemophilia.

Pakadali pano, madokotala sangathe kuchiza matenda a hemophilia kwathunthu. Komabe, angapereke chithandizo choletsa kapena kuchepetsa zizindikiro za hemophilia. Angakulimbikitseninso njira zomwe mungachite kuti muchepetse zotsatira za hemophilia pa moyo wanu watsiku ndi tsiku. Chofunika kwambiri ndikukhala chete ndikutsatira malangizo a dokotala wanu mosamala.