Awunangxaki nawe yokuyeka ukopha? Masifunde nge-hemophilia!

Ngaba wakha weva ngesifo esibizwa ngokuba yiHemophilia ? Usenokuziva usoyika kancinci xa usiva eli gama. Kodwa ungakhathazeki, kuba esi sisifo esingaqhelekanga. Namhlanje, siza kuthetha ngokuba yintoni i-hemophilia, indlela ekhula ngayo, zithini iimpawu, kwaye ingaba kukho unyango, konke oku ngendlela elula onokuyiqonda.

Yintoni i-hemophilia?

Ngamafutshane, i-hemophilia sisifo segazi esidluliselwa kwilifa esenzeka ebantwaneni. Oku kuthetha ukuba igazi lakho alijiki kakuhle. Oku kunokubangela ukopha kakhulu, okanye ukukrweleka okuncinci.

Cinga ngako, kukho iiproteni ezikhethekileyo emizimbeni yethu ezinceda ekuqhekekeni kwegazi. Ezi sizibiza ngokuba 'zizinto ezivala igazi' . Zifana namajoni amancinci asebenza ukunqanda ukopha. Xa unesifo se-hemophilia, umzimba awuvelisi ngokwaneleyo enye okanye ezingaphezulu kwezi 'zinto ezivala igazi'. Emva koko igazi liyawa lincipha kwaye wopha igazi ngakumbi.

Kukho iintlobo ezininzi eziphambili ze-hemophilia. Le meko inokuba qatha, ibe phakathi, okanye ibe ncinci, kuxhomekeke kubungakanani bezinto ezibangela ukujiya kwegazi lakho.

Iindaba ezimnandi zezokuba kukho unyango loku. Oogqirha bazama ukubuyisela izinto ezibangela ukujiya kwegazi emzimbeni. Okwangoku akukho nyango lwe-hemophilia. Nangona kunjalo, abantu abanale meko badla ngokuphila ixesha elide njengabanye xa benonyango olufanelekileyo. Izazinzulu zikwaphanda ngeendlela ezintsha, ezinje ngonyango lwezakhi zofuzo , ukuze zibone ukuba lunganyangeka na.

Ngaba i-hemophilia ingakhula emva kokuzalwa?

Ewe, inokwenzeka. Kodwa ayiqhelekanga kakhulu. Oku kubizwa ngokuba yi-'acquired hemophilia'. Oko kuthetha ukuba ayizuzwa njengelifa. Okwenzekayo apha kukuba iiproteni zokuzikhusela zomzimba wethu , ezibizwa ngokuba zii-antibodies , ngamanye amaxesha zihlasela ngempazamo enye yezinto zethu zokugalela igazi. Ezi antibodies zihlasela ngendlela engafanelekanga zibizwa ngokuba yi -'autoantibodies' .

Ingaba i-hemophilia sisifo esiqhelekileyo?

Hayi, akunjalo konke konke. Esi sisifo esingaqhelekanga kakhulu. Ngokwezibalo eMelika (CDC Agasti 2022), malunga nabantu abangama-33,000 apho bane-hemophilia. Ihlala ichaphazela amadoda. Amaxesha amaninzi, abafazi banokufumana iimpawu ezifana namanqanaba aphantsi okujiya kwegazi kunye nokopha kakhulu ngexesha lokuya exesheni.

Ziziphi iintlobo ze-hemophilia?

Kukho iintlobo ezintathu eziphambili ze-hemophilia:

• I-Hemophilia A: Olu lolona hlobo luxhaphakileyo. Lubangelwa kukungabikho kwe-blood clotting factor number 8, ekwaziwa ngokuba yi -factor VIII., xa umzimba unganelanga. Malunga ne-10 kwi-100,000 yabantu banokuba nale.
• I-Hemophilia B: Oku kubangelwa kukunqongophala kwe-blood clotting factor 9, okanye i-factor IX . Malunga nabantu abathathu kwi-100,000 banalo olu hlobo.
• I-Hemophilia C: Ikwabizwa ngokuba yi-factor XI deficiency , oku kunqabile kakhulu, kuchaphazela malunga nomntu omnye kwisigidi.

Ngaba i-hemophilia sisifo esibi kakhulu?

Ewe, ngamanye amaxesha oku kunokuba yinto enzulu. Abantu abane-hemophilia enzima bangopha igazi ngendlela esongela ubomi, esiyibiza ngokuba 'yi-hemorrhage.' Kodwa uninzi lwexesha baphuma igazi baye kwimisipha okanye amalungu omzimba.

Zithini iimpawu ze-hemophilia?

Iimpawu ezibaluleke kakhulu kukuphuma kwegazi okungaqhelekanga okanye ukopha kakhulu kunye nokukrweleka.

Iimpawu eziqhelekileyo

Abantu abane-hemophilia banokufumana imivumbo emikhulu nokuba kungengozi ezincinci. Oku kuthetha ukuba igazi liyavuza phantsi kolusu.

• Mhlawumbi emva kotyando, emva kokukhupha izinyo, okanye nokuba linxeba nje emnweni , ukopha kunokuhlala ixesha elide ngokungaqhelekanga.
• Ukopha empumlweni kunokubakho ngequbuliso ngaphandle kwesizathu.

Ukuba unemivumbo/ukopha okungakanani kuxhomekeke ekubeni une-hemophilia embi, ephakathi, okanye engephi.

• Abantu abane -hemophilia enzima bangopha rhoqo ngaphandle kwesizathu esicacileyo.
• Abantu abane -hemophilia ephakathi bangopha ixesha elide ngokungaqhelekanga ukuba bakwingozi enkulu.
• Abantu abane -hemophilia encinci bafumana ukopha okungaqhelekanga kuphela emva kotyando olukhulu okanye ingozi enkulu.

Kusenokubakho nezinye iimpawu:

• Intlungu yamalungu ngenxa yokuxinana kwegazi emzimbeni. Iindawo ezinje ngamaqatha, amadolo, izinqe, namagxa zinokuba buhlungu, zidumbe, okanye zivakale zishushu xa uzichukumisa.
• Ukopha ebuchotsheni. Oku kuyinto engaqhelekanga kubantu abane-hemophilia enzima. Ukuba unesifo sokopha ebuchotsheni, usenokuba nentloko ebuhlungu engapheliyo, umbono ofipheleyo, okanye ukozela kakhulu . Ukuba unesifo se-hemophilia, kufuneka ubone ugqirha ngoko nangoko ukuba ufumana naziphi na kwezi mpawu.

Iimpawu kwiintsana nakubantwana abancinci

Ngamanye amaxesha, i-hemophilia ifunyanwa xa umntwana oyinkwenkwe olukile aze ophe igazi ngaphezu kwesiqhelo. Ngamanye amaxesha, abantwana babonisa iimpawu kwiinyanga ezimbalwa emva kokuzalwa. Iimpawu eziqhelekileyo zezi:

• Ukopha:Iintsana zinokopha emlonyeni xa ziluma ithoyizi elincinci.
• Amaqhuma avuvukileyo entloko: Iintsana nabantwana abancinci banokuba namaqhuma amakhulu, angqukuva (amaqanda eehansi) xa bebetha iintloko zabo.
• Ukukhala rhoqo, ukungazinzi, kunye nokungafuni ukukhasa okanye ukuhamba: Oku kunokwenzeka ukuba igazi lingene kwisihlunu okanye kwilungu. Indawo isenokuvakala ngathi ikruqukile, idumbile, ishushu xa uyichukumisa, okanye umntwana unokuva iintlungu nokuba uchukunyiswa kancinci.
• IiHematoma: 'I-hematoma' yingqokelela yamahlwili egazi aqokelelana phantsi kolusu kwiintsana nakubantwana abancinci. Olu hlobo lweehematoma lunokwenzeka nasemva kokufakwa inaliti.

Zithini izizathu ze-hemophilia?

Ezi 'zinto zokuqhina' zenziwe ngeejini ezithile emzimbeni wethu. Kwi-hemophilia yelifa, iijini eziyalela ukuveliswa kwezi 'zinto zokuqhina' ziyatshintshatshintsha, oko kuthetha ukuba ziyatshintsha. Ezi jini zitshintshileyo zinokwenza 'izinto zokuqhina' ezingalunganga okanye zingazenzi 'izinto zokuqhina' ezaneleyo. Nangona kunjalo, malunga nama-20% ezigulane ze-hemophilia zizenzekela, oko kuthetha ukuba zinokuba nesi sifo nokuba akukho mntu kusapho lwakhe owakha wasinaso ngaphambili.

I-hemophilia ifunyanwa njani njengelifa?

Zombini ezi ntlobo ze-hemophilia A kunye ne-B zizifo ezinxulumene nesondo. Zizuzwa njengelifa ngendlela ehambelana ne-X. Makhe sibone ukuba oko kwenzeka njani:

• Sonke sifumana iseti enye yeechromosomes kumama wethu kunye neseti enye yeechromosomes kutata wethu. Ukuba ufumana i-chromosome X kumama wakho kunye ne-chromosome X kutata wakho, uyintombazana. Ukuba ufumana i-chromosome X kumama wakho kunye ne-chromosome Y kutata wakho, uyinkwenkwe. Ngamafutshane, umama uhlala enika umntwana wakhe i-chromosome X. Isini sakho simiselwa kukuba utata ukunika i-chromosome X okanye Y.
• Ukuba umfazi unesiphako kwijini esenza le 'clotting factor' kwi-chromosome enye kuphela kwii-chromosomes zakhe ezimbini ze-X, usenokuba ngumthwali we- hemophilia, kodwa usenokungabonakalisi zimpawu kuba unejini ephilileyo kwenye i-chromosome yakhe ye-X.
• Ukuba umama one-chromosome X enesiphene njengale unomntwana oyinkwenkwe, kukho amathuba angama-50% okuba umntwana azuze ilifa le-chromosome X enesiphene. Ukuba oko kuyenzeka, umntwana oyinkwenkwe uya kuba ne-hemophilia.
• Ukuba loo mama unentombi, kukho amathuba angama-50% okuba umntwana naye azuze ilifa le-chromosome X enesiphene. Nangona kunjalo, usenokungabonakalisi zimpawu kuba uzuze i-chromosome X ephilileyo kuyise. Emva koko loo ntombi naye uya kuba ngumthwali.

Oko kuthetha ukuba umfazi ofumana ilifa le-chromosome X enesiphene uba ngumthwali we-hemophilia. Usenokungabi nazimpawu, kodwa unokudlulisela esi sifo kubantwana bakhe. Umntwana ngamnye anaye unethuba elingama-50% lokufumana i-hemophilia njengelifa.Abantwana abangamakhwenkwe banamathuba amaninzi okubonakalisa iimpawu ukuba bazuze ilifa le-hemophilia, kuba abafumani i-chromosome X ephilileyo kuyise.

Ngaba amantombazana nawo aneempawu ze-hemophilia?

Ewe, inokwenzeka. Kodwa iimpawu zihlala zincinci. Umzekelo, umfazi one-hemophilia gene usenokungabi nazo izinto ezaneleyo zokujiya kwegazi. Ukuba oku kuyenzeka, unokufumana ukopha okungaqhelekanga ngexesha lokuya kwakhe exesheni, akruquke lula, ophe kakhulu emva kokubeleka, okanye ophe kakhulu emalungwini akhe, nto leyo ebangela iingxaki emalungwini.

Oogqirha bayixilonga njani i-hemophilia?

Ugqirha uza kuqala akubuze imbali epheleleyo yezonyango aze akuhlole ngokwasemzimbeni. Ukuba uneempawu ze-hemophilia, uza kubuza nangembali yosapho lwakho. Bangenza iimvavanyo ezifana nezi:

• Ubalo lweGazi olupheleleyo (CBC): Oku kwenzelwa ukujonga iintlobo zeeseli egazini.
• Uvavanyo lwexesha le-Prothrombin (PT): Oku kunokujonga ukuba igazi lakho likhawuleza kangakanani na ukujiya.
• Uvavanyo lwexesha le-Prial Thromboplastin (PTT): Olu lolunye uvavanyo olulinganisa ixesha elithathwayo ukuze igazi lijiye.
• Uvavanyo oluthile lwezinto ezibangela ukugabha kwegazi: Olu vavanyo lwegazi lulinganisa amanqanaba ezinto ezithile ezibangela ukugabha kwegazi, ezifana ne -factor VIII kunye ne -factor IX .

Zithini ezi nqanaba ze-clotting factor?

Izinto ezibangela ukujiya kwegazi zizinto ezinceda ekulawuleni ukopha kwegazi. Oogqirha bahlula i-hemophilia njengencinci , ephakathi, okanye enzima ngokusekelwe kubungakanani bezi zinto ezibangela ukujiya kwegazi egazini lakho:

• Abantu abane-clotting factors ephakathi kwe-5% kunye ne-30% yamanqanaba aqhelekileyo bane -hemophilia encinci.
• Abantu abane-clotting factors ephakathi kwe-1% kunye ne-5% yamanqanaba aqhelekileyo bane -hemophilia ephakathi.
• Abantu abane-clotting factors engaphantsi kwe-1% eqhelekileyo bane -hemophilia enzima.

Ziziphi iindlela zonyango lwe-hemophilia?

Oogqirha banyanga i-hemophilia ngokunyusa amanqanaba ezinto ezibangela ukugabha kwegazi eziphantsi okanye ngokutshintsha izinto ezibangela ukugabha kwegazi ezingekhoyo. Oku kubizwa ngokuba yi-replacement therapy .

Kolu nyango lokutshintshwa, unikwa izinto ezibangela ukugabha kwegazi ezenziwe nge-plasma yomntu (ii-human plasma concentrates) okanye ' ii-recombinant clotting factors'. Ngokwesiqhelo, ngabantu abane-hemophilia enzima kuphela abadinga olu nyango lokutshintshwa rhoqo. Abantu abane-hemophilia engephi ukuya kwephakathi banikwa olu nyango ukuba sele beza kutyandwa. Banikwa ii-antifibrinolytics.Amayeza athintela ukuqhekeka kwegazi ekunyibilikeni nawo anokunikwa.

Ezi zinto zixutyiweyo zegazi zenziwa ngegazi lomntu elinikelweyo. Ziyacocwa kwaye zivavanywe ukunciphisa umngcipheko wokudlulisela izifo ezosulelayo ezifana ne -hepatitis kunye ne-HIV . Ezi zinto zingena endaweni yazo zinikwa njenge- intravenous infusion (IV) .

Ukuba une-hemophilia enzima kwaye wopha rhoqo, ugqirha wakho angakunika unyango olubizwa ngokuba yi -'prophylactic factor infusions' ukuthintela ukopha.

Ngaba kukho naziphi na iingxaki kunyango?

Abanye abantu abafumana olu nyango lokutshintsha baphuhlisa ii-antibodies , ezibizwa ngokuba zii-inhibitors , ezihlasela izinto ezibangela ukugabha igazi abazinikiweyo. Oogqirha basebenzisa inkqubo ebizwa ngokuba yi -Immune Tolerance Induction (ITI) ukunyanga oku. I-ITI ibandakanya ukubanika inaliti yemihla ngemihla yezinto ezibangela ukugabha igazi ukuze banciphise amanqanaba ezi zithinteli. Olu lunyango oluhlala ixesha elide, kwaye abanye abantu banokulufuna olu nyango kangangeenyanga okanye iminyaka.

Ngaba i-hemophilia ingathintelwa?

Hayi, akunakwenzeka. Ukuba une-hemophilia kwaye unabantwana, ugqirha wakho unokucebisa uvavanyo lwemfuza . Ngale ndlela, wena nabantwana bakho ninokufumanisa ukuba banako na ukudlulisela i-hemophilia kwisizukulwana esilandelayo.

Luhlobo luni lwethemba umntu one-hemophilia afanele abe nalo?

Ukuba une-hemophilia, unokufuna unyango ubomi bakho bonke. Ubungakanani bonyango olufunekayo buxhomekeke kuhlobo lwe-hemophilia onayo, ubukhali bayo, kunye nokuba ingaba uphuhlisa okanye awuphuhlisi 'ii-inhibitors'.

Ingakanani iminyaka yobomi bomntu one-hemophilia?

Ngokutsho kweWorld Federation of Hemophilia, ubomi bendoda ene-hemophilia bufutshane ngeminyaka eli-10 kunobendoda engenayo i-hemophilia, ngokutsho kwedatha ka-2012 evela kwiWorld Federation of Hemophilia. Nangona kunjalo, bathi abantwana abane-hemophilia abafunyaniswa benale ngxaki baze banyangwe besebancinci banobomi obuqhelekileyo .

Kodwa ayingabo bonke abantu abafanayo. Oko kuyinyaniso komnye umntu kusenokungabi yinyani komnye. Ukuba wena okanye umntwana wakho une-hemophilia, buza ugqirha wakho ukuba ulindele ntoni. Uyazi kakuhle imeko yakho/yomntwana wakho kunye nempilo yakhe ngokubanzi.

Ndingazinyamekela njani?

Abantu abane-hemophilia banokufuna unyango oluqhubekayo ukuze bathintele ukopha. Basenokufuna nokuyeka eminye imisebenzi kunye namayeza. Nangona kunjalo, zininzi izinto onokuzenza ukulawula impembelelo ye-hemophilia ebomini bakho.

Izinto ekungamele zenziwe

Izinto ezilula kwabanye ukungqubana, ukuwa, okanye ukukhubeka zinokubangela iingxaki ezinkulu kubantu abane-hemophilia. Bafanele baphephe imisebenzi ebabeka emngciphekweni omkhulu wokuwa okanye ukubethwa kabuhlungu. Imizekelo:

• Ukudlala imidlalo efana nebhola ekhatywayo, ihoki, kunye neragbi.
• Ukuthatha inxaxheba kwizinto ezifana nebhokisi kunye nokulwa.
• Ukukhwela isithuthuthu.
• Ukutyibiliza ngebhodi.

Eminye imidlalo, efana nebhola ekhatywayo, ibasketball, kunye nebaseball, nayo inomngcipheko omkhulu wokwenzakala. Ukuba ufuna ukudlala le midlalo, thetha nogqirha wakho ngezinto ezifana nokusebenzisa izixhobo zokuzikhusela.

Amayeza angalunganga ukuwasela

Izithomalalisi zentlungu ezifana ne -aspirin, i-ibuprofen, kunye ne-naproxen zithintela ukujiya kwegazi. Kwakhona, ukuthatha izithomalalisi zegazi ezifana ne-heparin okanye i-warfarin akulunganga.

Izinto onokuzenza ukuze uphile ubomi obungcono

Zininzi izinto onokuzenza ukunciphisa impembelelo ye-hemophilia ebomini bakho:

• Yenza umthambo rhoqo: Usenokuba nexhala lokuba uza kwenzakala ngelixa usenza umthambo. Nangona kunjalo, thetha nogqirha wakho ngeendlela zokuhlala usempilweni ngelixa unciphisa umngcipheko wokopha.
• Lawula uxinzelelo : I-Hemophilia yimeko ehlala ixesha elide, kwaye unokufuna ukwenza umgudu owongezelelweyo wokuyilinganisela kunye noxanduva lwakho losapho kunye nomsebenzi.
• Gcina impilo entle yamazinyo: Ukuxubha amazinyo, ukuwaxuba nge-floss, nokubona ugqirha wamazinyo rhoqo kunokunciphisa umngcipheko wokufuna unyango lwamazinyo olunokubangela ukopha. Ungalibali ukuxelela ugqirha wakho ngemeko yakho.
• Gcina ubunzima obusempilweni: Ukuba unengxaki yokuhambahamba ngenxa yokopha ngaphakathi kunye nomonakalo wamalungu, ukulawula ubunzima bakho kuya kukunceda.
• Yazisa abo bakungqongileyo: Ukuba une-hemophilia enzima, unokufumana ukopha okungalindelekanga, nokuba usela amayeza. Yazisa usapho lwakho ngento omawuyenze ukuba oku kuyenzeka kuwe. Ukuba umntwana wakho une-hemophilia, yazisa abanakekeli bakhe kunye nabasebenzi besikolo ngento omawuyenze ukuba umntwana wakho uyopha.

Ndifanele ndimbone nini ugqirha?

Ukuba ubona naluphi na utshintsho emzimbeni wakho, njengokuphuma kwegazi elininzi okanye ukukrweleka, bona ugqirha.

Ixesha lokuya kwiGumbi loNgxamiseko

Kuya kufuneka uye kwigumbi likaxakeka ngoko nangoko kwezi meko zilandelayo:

• Ukuba uneentloko ezibuhlungu okanye unesiyezi, ezi mpawu zinokubonisa ukuba uphuma igazi engqondweni yakho.
• Ukuba unamajoyinti adumbileyo okanye abuhlungu, kwaye awunayo iyeza lokutshintsha i-factor.

Ndingayibuza yiphi imibuzo ugqirha?

Ukuba wena okanye umntwana wakho nifunyaniswa ukuba ninesifo se-hemophilia, ningathanda ukubuza ugqirha wakho imibuzo efana nale:

• Luhlobo luni lwe-hemophilia endinalo/umntwana wam?
• Loluphi unyango olucebisayo?
• Zithini iziphumo ebezingalindelekanga zonyango?
• Ngaba mna/umntwana wam kuya kufuneka ndifumane unyango yonke imihla?
• Zeziphi izinto ezingalunganga kum/kumntwana wam ukuba azenze?
• Ngawaphi amayeza angalunganga ukuba mna/umntwana wam ndiwasebenzise?
• Ndingenza ntoni ukulawula impembelelo enayo le meko kubomi bam/bomntwana wam?

Umyalezo Wokuya Ekhaya

I-Hemophilia sisifo segazi esingaqhelekanga, esizuzwe njengelifa. Sinokuba nefuthe elikhulu ebomini bakho. Abantu abane-hemophilia banokufuna unyango oluhlala ixesha elide. Abazali babantwana abane-hemophilia banokuxhalabela ukukhusela abantwana babo kwiingozi, kunye nokubafundisa ukuphila ne-hemophilia.

Okwangoku, oogqirha abakwazi ukunyanga i-hemophilia ngokupheleleyo. Nangona kunjalo, banokubonelela ngonyango lokuthintela okanye lokunciphisa iimpawu ze-hemophilia. Banokucebisa namanyathelo onokuwathatha ukunciphisa impembelelo ye-hemophilia kubomi bakho bemihla ngemihla. Eyona nto ibalulekileyo kukuhlala uzolile kwaye ulandele imiyalelo kagqirha wakho ngononophelo.