Kodi ngakhale kudula pang'ono kwa thupi lanu sikusiya kutuluka magazi? Kodi tikambirane za hemophilia?

Kodi mukukumbukira muli mwana, pamene munagwa ndikubowola bondo lanu kapena chigongono chanu, kutuluka magazi kunkatha pakapita kanthawi? Kodi sizodabwitsa? Tikatuluka magazi, matupi athu ali ndi njira yoletsera kutuluka magazi, yomwe ndi njira yomwe imapangitsa magazi kuundana. Koma pali anthu ena, ndipo njira iyi yotsekereza magazi sigwira ntchito bwino m'thupi lawo. Pamenepo ndi pamene timalankhula za matenda otchedwa hemophilia . Izi zitha kukhala zovuta pang'ono, koma ndi chithandizo choyenera , ndi vuto lomwe lingachiritsidwe bwino.

Kodi hemophilia kwenikweni ndi chiyani?

Mwachidule, hemophilia ndi matenda omwe magazi athu sagwira bwino ntchito, zomwe zikutanthauza kuti tikatuluka magazi, sasiya mosavuta . Ndi matenda a majini makamaka, zomwe zikutanthauza kuti amatha kufalikira kudzera m'majini kuchokera ku mibadwomibadwo.

Tangoganizirani kuti mwadula pang'ono padzanja lanu. Kawirikawiri, mapuloteni ena m'magazi athu, otchedwa zinthu zotsekereza magazi , amagwira ntchito limodzi kuti atseke bala ndikuletsa kutuluka magazi. Zili ngati kumanga dzenje pakhoma.

Koma mwa munthu amene ali ndi hemophilia, chimodzi mwa zinthuzi chimasowa mokwanira, kapena chinthucho sichigwira ntchito bwino. Vuto ndi limenelo. Ichi ndichifukwa chake amatha kutuluka magazi kwa nthawi yayitali ngakhale atavulala pang'ono, kapena amatha kuvulala mosavuta.

Momwe magazi amaundana mosavuta

Tikayamba kutuluka magazi, pali njira zingapo zoletsera kutuluka magazi.

1. Choyamba chomwe chimachitika ndichakuti mitsempha yamagazi imachepa. Kenako kuchuluka kwa magazi omwe amatuluka kumachepa pang'ono.

2. Kenako, maselo ang'onoang'ono otchedwa ma platelet m'magazi athu amabwera pamalo pomwe pavulalapo ndi kumamatirana, ndikupanga mtundu wa dam laling'ono.

3. Kenako, zinthu zotsekereza magazi zomwe ndatchula kale zimayamba kugwira ntchito, zikugwira ntchito imodzi pambuyo pa inzake, kupanga ulusi wolimba wotchedwa fibrin , zomwe zimalimbitsa kwambiri chotchinga cha ma platelet ndikuletsa kutuluka magazi kwathunthu.

Chomwe chimachitika mu hemophilia ndichakuti mu gawo lachitatu ili, chimodzi mwa zinthu zomwe zimayambitsa magazi kuundana chimasowa, kotero kuti magazi kuundana amphamvu sapangidwa.

Kodi pali mitundu ya hemophilia?

Inde, pali mitundu iwiri ikuluikulu ya hemophilia, kutengera ndi chinthu chofooketsa magazi chomwe chilibe mphamvu.

Hemophilia A

Uwu ndi mtundu wofala kwambiri wa hemophilia. Umayamba chifukwa chosakhala ndi clotting factor VIII yokwanira kapena chifukwa chakuti sukugwira ntchito bwino.

Hemophilia B

Mtundu uwu umatchedwanso Matenda a Khirisimasi . Umayambitsidwa ndi kusowa kwa chinthu chachisanu ndi chinayi choyambitsa magazi kuundana (Factor IX).Palibe vuto kuti palibe zokwanira, palibe vuto kuti sizikugwira ntchito bwino.

Zizindikiro za mitundu yonse iwiri zimafanana kwambiri. Komabe, ndikofunikira kwambiri kuzindikira mitundu iwiriyi molondola pochiza. Chifukwa chithandizo chiyenera kuperekedwa malinga ndi chinthu chomwe chachepa.

Kuopsa kwa vutoli

Kuopsa kwa hemophilia kungasiyane malinga ndi munthu, kutengera kuchuluka kwa chinthu chosowa chomwe chimayambitsa magazi kuundana m'thupi.

• Kuchepa kwa magazi m'thupi: Anthu omwe ali ndi kuchepa kwa magazi m'thupi amakhala ndi zinthu zochepa zotsekereza magazi. Amatha kutuluka magazi kwa kanthawi kochepa ngati avulala kwambiri, achitidwa opaleshoni, kapena achotsedwa dzino. Mwina sangadziwe kuti ali ndi kuchepa kwa magazi m'thupi mpaka patakhala vuto lalikulu.
• Matenda a hemophilia apakati: Anthuwa ali ndi zinthu zochepa zotsekereza magazi. Angatulutse magazi ambiri ngakhale chifukwa cha kuvulala pang'ono. Nthawi zina, angatulutse magazi popanda chifukwa (kutuluka magazi mwadzidzidzi).
• Kutaya magazi kwambiri: Anthuwa ali ndi zinthu zochepa kwambiri zotsekereza magazi. Amatha kutuluka magazi m'malo olumikizirana mafupa ndi minofu popanda chifukwa chomveka. Ngakhale kuvulala pang'ono kungayambitse vuto lalikulu.

N’chifukwa chiyani izi zimachitika? Kodi zifukwa zake ndi ziti?

Hemophilia nthawi zambiri ndi matenda a majini , zomwe zikutanthauza kuti amapatsirana kuchokera kwa makolo kupita kwa ana kudzera m'majini.

Momwe zimakhalira kuchokera ku cholowa (cholowa cholumikizidwa ndi X)

Jini ya hemophilia ili pa chromosome ya X. Mukudziwa, akazi ali ndi ma chromosome awiri a X (XX), pomwe amuna ali ndi chromosome imodzi ya X ndi chromosome imodzi ya Y (XY).

• Mwana wamkazi: Amalandira X imodzi kuchokera kwa amayi ake ndi X imodzi kuchokera kwa abambo ake.
• Mwana wamwamuna: Amatenga X imodzi kuchokera kwa amayi ake ndi Y imodzi kuchokera kwa abambo ake.

Tsopano, ngati jini yolakwika yomwe imayambitsa hemophilia ili pa chromosome ya X,

• Ngati mnyamata alandira chromosome ya X yolakwikayo kuchokera kwa amayi ake, adzakhala ndi hemophilia. Chifukwa ali ndi chromosome imodzi yokha ya X. Ngati pali cholakwika pa iyo, palibe X ina yoti imuthandize.
• Ngati mtsikana walandira chromosome ya X yolakwika kuchokera kwa amayi ake kapena abambo ake, koma ali ndi chromosome ya X yathanzi, sadzawonetsa zizindikiro. Koma adzakhala wonyamula . Izi zikutanthauza kuti ngakhale atakhala kuti alibe hemophilia, amatha kupatsira jini yolakwika kwa ana ake. Nthawi zambiri, akazi omwe ali ndi chromosome imeneyi amathanso kusonyeza zizindikiro zochepa.

Ndicho chifukwa chake hemophilia imapezeka kwambiri mwa amuna .

Tangoganizani, ngati mayi ali ndi vuto la haemophilia, mwana wamwamuna aliyense wobadwa naye ali ndi mwayi wa 50% wokhala ndi haemophilia. Momwemonso, mwana wamkazi aliyense ali ndi mwayi wa 50% wokhala ndi vutoli.

Kodi imayenda m'mabanja? (Kusintha kwadzidzidzi)

Sikofunikira nthawi zonse kuti munthu m'banjamo akhale ndi vuto la hemophilia. Nthawi zina, pafupifupi 30% ya milandu, hemophilia imatha kuchitika ngati kusintha kwa majini mwachibadwa. Zikatero, palibe wina m'banjamo amene angakhale ndi vutoli kale.

Kodi zizindikiro za hemophilia ndi ziti? Kodi zimapezeka bwanji?

Zizindikiro za hemophilia zimasiyana malinga ndi kuopsa kwa matendawa. Zizindikiro zodziwika bwino ndi izi:

• Kupezeka pafupipafupi kwa mabala akuluakulu komanso akuya: Ngakhale chiphuphu chaching'ono chingayambitse mabala akulu.
• Kutuluka magazi kosalekeza chifukwa cha kuvulala, kuchotsedwa dzino, kapena pambuyo pa opaleshoni.
• Kutuluka magazi m'mphuno popanda chifukwa.
• Mkamwa wotuluka magazi.
• Kutuluka magazi m'malo olumikizirana mafupa (hemarthrosis): Izi zimapweteka kwambiri. Malo olumikizirana mafupa amatupa, kutentha, komanso sangathe kuyenda bwino. Malo olumikizirana mafupa monga mawondo, zigongono, ndi akakolo nthawi zambiri amakhudzidwa. Pakapita nthawi, izi zingayambitsenso kuwonongeka kwa malo olumikizirana mafupa.
• Kutuluka magazi m'minofu: Izi zimayambitsanso ululu ndi kutupa.
• Kutuluka magazi ndi mkodzo kapena ndowe.
• Kwa makanda: Nthawi zina chizindikiro choyamba chimakhala kutuluka magazi nthawi zonse mutadula. Kapena mutalandira jakisoni, malowo akhoza kutupa kwambiri ndikutuluka magazi.
• Matenda oopsa kwambiri, koma osowa kwambiri, ndi kutuluka magazi muubongo. Izi zingayambitse zizindikiro monga mutu waukulu, kusanza, kugona, ndi khunyu. Izi ndi zadzidzidzi.

Kodi dokotala amazindikira bwanji kuti ichi ndi hemophilia? (Kuzindikira)

Ngati inu kapena mwana wanu muli ndi zizindikiro izi, chinthu chabwino kwambiri choti muchite ndikupita kwa dokotala. Dokotala nthawi zambiri amazindikira matendawa m'njira zotsatirazi:

1. Kufunsa za mbiri ya banja: Kufufuza ngati pali aliyense m'banjamo amene ali ndi vuto la magazi m'thupi kapena matenda ena otuluka magazi.

2. Kuyezetsa thupi kumachitika: Kufufuza ngati pali mabala, kutupa kwa mafupa, ndi zina zotero.

3. Kuyezetsa magazi kumachitika:

• Mayeso oyezera: Izi zimayesa nthawi yomwe magazi amaundana. Mwachitsanzo, mayeso monga PTT (Partial Thromboplastin Time) ndi PT (Prothrombin Time) . Ma PTT values ​​​​angatalikitsidwe mu hemophilia.
• Kuyesa magazi m'thupi: Izi zimagwiritsidwa ntchito kudziwa ngati Factor VIII ndi Factor IX zili ndi vuto lochepa, komanso kuti ndi mpaka pati. Izi zimagwiritsidwa ntchito kudziwa mtundu ndi kuopsa kwa hemophilia.

Ngakhale panthawi ya mimba, ngati pali mbiri ya banja la mwana yemwe ali ndi vuto la hemophilia, mayeso amatha kuchitidwa kuti aone ngati mwana ali ndi hemophilia.

Kodi mankhwala a izi ndi otani? (Chithandizo)

Ngakhale kuti hemophilia ndi matenda osachiritsika, pali mankhwala othandiza kwambiri omwe alipo.Mankhwalawa angathandize kuwongolera ndi kupewa kutuluka magazi, komanso kukuthandizani kukhala ndi moyo wabwino.

Chithandizo Cholowa M'malo

Uwu ndiye chithandizo chachikulu. Umaphatikizapo kupatsa thupi chinthu cholimbitsa magazi (Factor VIII kapena Factor IX) chomwe chilibe mphamvu m'thupi kudzera mu mtsempha (intravenous kapena IV infusion). Zinthuzi zimapangidwa kuchokera ku plasma ya magazi a anthu kapena kudzera mu ukadaulo wa majini (recombinant products).

Chithandizochi chimachitika m'njira ziwiri:

1. Chithandizo choteteza: Pachifukwa ichi, chinthuchi chimaperekedwa m'thupi kangapo pa sabata, magazi asanayambe kutuluka. Izi zitha kupewa kutuluka magazi mwadzidzidzi, makamaka m'malo olumikizirana mafupa. Izi nthawi zambiri zimachitika kwa anthu omwe ali ndi vuto lalikulu la magazi m'thupi.

2. Chithandizo cha pa nthawi yomwe munthu akufuna: Pankhaniyi, chinthuchi chimaperekedwa pokhapokha ngati magazi ayamba kutuluka. Chithandizochi chimagwiritsidwa ntchito kwa anthu omwe ali ndi vuto la kuchepa kwa magazi m'thupi komanso omwe sali pa chithandizo chopewera matenda.

Mankhwala ena

• Desmopressin (DDAVP): Iyi ndi mankhwala omwe angagwiritsidwe ntchito kwa anthu omwe ali ndi vuto la kuchepa kwa magazi m'thupi (hemophilia A). Amagwira ntchito potulutsa Factor VIII yosungidwa m'thupi. Angaperekedwe ngati mankhwala opopera m'mphuno kapena jakisoni.
• Mankhwala oletsa fibrinolytic: Mwachitsanzo , tranexamic acid . Mankhwalawa amagwira ntchito pochepetsa kusungunuka kwa magazi. Ndi othandiza pazochitika monga kuchotsa mano ndi kutuluka magazi m'mphuno.
• Mankhwala ochepetsa ululu: Mungatenge zinthu monga paracetamol kuti muchepetse ululu womwe umabwera chifukwa cha kutuluka magazi m'malo olumikizirana mafupa. Komabe, mankhwala monga aspirin ndi NSAIDs (monga ibuprofen, diclofenac) sakuvomerezeka kwa anthu omwe ali ndi vuto la magazi, chifukwa amatha kuwonjezera chiopsezo cha kutuluka magazi.

Kodi mumatani mukatuluka magazi?

Pali zinthu zingapo zomwe muyenera kuchita mukatuluka magazi. Kumbukirani njira ya RICE .

• R (Kupuma): Pumitsani cholumikizira kapena minofu yovulala. Chitetezeni kuti chisasunthe.
• I (Aisi): Ikani paketi ya ayezi pamalo ovulala kwa mphindi pafupifupi 15-20, kangapo patsiku. Izi zichepetsa kutupa ndi ululu.
• C (Kupsinjika): Manga malo ovulala ndi bandeji yotanuka kuti ikhale yolimba pang'ono.
• E (Kukwera): Sungani mkono kapena mwendo wovulala pamwamba pa mulingo wa mtima.

Mukamachita izi, muyenera kulankhula ndi dokotala wanu ndikupeza chithandizo cha Factor ngati pakufunika kutero.

Momwe mungakhalire ndi hemophilia

Kukhala ndi matenda a hemophilia kungakhale kovuta, koma ndi chisamaliro choyenera , kuzindikira, ndi chithandizo, mutha kukhala moyo wabwinobwino komanso wotanganidwa.

• Chithandizo kuchokera ku gulu la madokotala apadera: Pochiza matenda a hemophilia,Ndikofunikira kwambiri kupeza thandizo kuchokera ku gulu lopangidwa ndi akatswiri a zamagazi, anamwino, akatswiri ochiritsa matenda a minofu, ndi ogwira ntchito zachitukuko. Adzakupatsani malangizo okhudza chithandizo, masewera olimbitsa thupi, komanso momwe mungakhalire otetezeka.
• Chitani zinthu zodzitetezera: Anthu omwe ali ndi vuto la hemophilia ayenera kupewa masewera omwe angayambitse kuvulala kwambiri (monga rugby, nkhonya). M'malo mwake, chitani zinthu zodzitetezera monga kusambira, kuyenda, komanso kukwera njinga (ndi chisoti). Lankhulani ndi katswiri wa zamaganizo kuti akupatseni masewera olimbitsa thupi omwe ali oyenera kwa inu.
• Sungani thanzi labwino la mano: Ndikofunikira kwambiri kupewa kuwola kwa mano ndi matenda a chiseyeye, chifukwa pali chiopsezo chotuluka magazi mukamachita njira monga kuchotsa mano. Pitirizani kukaonana ndi dokotala wa mano.
• Chidziwitso cha chenjezo lachipatala: Nthawi zonse nyamulani chibangili kapena khadi lolembedwa kuti muli ndi hemophilia. Zingakhale zothandiza kwambiri pakagwa ngozi.
• Thandizo la anthu: Kulankhula ndi anthu ena omwe ali ndi vuto la hemophilia ndi mabanja awo komanso kugawana zomwe akumana nazo ndi mphamvu yayikulu. Ngati pali mabungwe otere, lowani nawo.
• Chiyembekezo cha mtsogolo: Mankhwala atsopano a hemophilia akupezedwa nthawi zonse. Kafukufuku akuchitikanso pazinthu monga chithandizo cha majini . Kuti tikhale ndi chiyembekezo cha mtsogolo.

Uthenga Wopita Nawo Kunyumba

Hemophilia ndi vuto la magazi kuundana, koma si chinthu choopa ndipo chingathe kuthetsedwa bwino .

Chofunika kwambiri ndikupempha upangiri wa dokotala mwachangu ngati muli ndi zizindikiro, kupeza matenda olondola, ndikutsatira chithandizo chomwe mwapatsidwa.

Ngati inu kapena munthu amene mumamudziwa ali ndi vuto la hemophilia, kumbukirani kuti simuli nokha. Ndi chidziwitso, chithandizo, ndi chithandizo chomwe mukufuna, inunso mutha kukhala ndi moyo wathanzi komanso wokangalika. Musataye mtima!

Hemophilia , kutuluka magazi, kuundana kwa magazi, matenda a majini, Factor VIII, Factor IX