Ngaba nenxeba elincinci aliyeki ukopha? Singathetha nge-hemophilia?

Uyakhumbula xa wawusengumntwana, xa wawuwa uze utyumke idolo okanye ingqiniba, ukopha kwakuye kuphele emva kwexesha elithile? Ayimangalisi loo nto? Xa sisopha, imizimba yethu inenkqubo yokuyimisa, eyinkqubo eyenza ukujiya kwegazi. Kodwa kukho abanye abantu, kwaye le nkqubo yokujiya kwegazi ayisebenzi kakuhle emizimbeni yabo. Kulapho sithetha khona ngemeko ebizwa ngokuba yi-hemophilia . Oku kunokuba yingozi kancinci, kodwa ngokuphathwa ngokufanelekileyo , yimeko enokunyangeka kakuhle.

Yintoni kanye kanye i-hemophilia?

Ngamafutshane, i-hemophilia yimeko apho igazi lethu lingajiyi kakuhle, oko kuthetha ukuba xa sisopha, aliyeki lula . Ngokuyintloko sisifo semfuza , oko kuthetha ukuba sinokudluliselwa kwiimfuza ukusuka kwisizukulwana ukuya kwesinye.

Khawucinge ukuba unenxeba elincinci esandleni sakho. Ngokwesiqhelo, iiproteni ezithile egazini lethu, ezibizwa ngokuba zizinto ezibangela ukuqhekeka kwegazi , zisebenza kunye ukuvala inxeba nokuthintela ukopha. Kufana nokuvala umngxuma eludongeni.

Kodwa kumntu one-hemophilia, enye yezi zinto zokujiya kwegazi ayikho ngokwaneleyo, okanye le nto ayisebenzi kakuhle. Yiyo loo nto inokubangela ukuba baphume igazi ixesha elide nokuba bangenzakele kancinci, okanye bangakwazi ukukrala lula.

Indlela amahlwili egazi alula ngayo

Xa siqala ukopha, kukho amanyathelo aliqela okukuthintela.

1. Into yokuqala eyenzekayo kukuba imithambo yegazi iyaxina. Emva koko inani legazi eliphumayo liyehla kancinci.

2. Emva koko, iiseli ezincinci ezibizwa ngokuba ziiplatelets egazini lethu ziyafika kwindawo yokwenzakala zize zinamathelene, zenze uhlobo oluthile lwedama elincinci.

3. Okulandelayo, izinto ezibangela ukugabha kwegazi endizikhankanyileyo ngaphambili ziyasebenza, zisebenza enye emva kwenye, zenze i-mesh eqinileyo ebizwa ngokuba yi-fibrin , iqinisa ngakumbi umqobo weeplatelet kwaye iyeke ngokupheleleyo ukopha.

Okwenzekayo kwi-hemophilia kukuba kweli nyathelo lesithathu, enye yezinto ezibangela ukujiya kwegazi ayikho, ukuze i-clot enamandla ingakheki.

Ngaba kukho iintlobo ze-hemophilia?

Ewe, kukho iintlobo ezimbini eziphambili ze-hemophilia, kuxhomekeke ekubeni yeyiphi i-clotting factor enqongopheleyo.

I-Hemophilia A

Olu lolona hlobo luqhelekileyo lwe-hemophilia. Lubangelwa kukungabikho kwe-clotting factor VIII eyaneleyo okanye ukungasebenzi kakuhle kwayo.

I-Hemophilia B

Olu hlobo lukwabizwa ngokuba yiChristmas Disease . Lubangelwa kukunqongophala kwe-nine blood clotting factor (Factor IX).Kulungile ukuba akwanelanga, kulungile ukuba ayisebenzi kakuhle.

Iimpawu zeentlobo zombini ziyafana kakhulu. Nangona kunjalo, kubaluleke kakhulu ukuchonga ezi ntlobo zimbini ngokuchanekileyo xa uzinyanga. Kuba unyango kufuneka lunikwe ngokwento ehlileyo.

Ubunzulu bale meko

Ubunzima be-hemophilia bungahluka kumntu nomntu, kuxhomekeke ekubeni ingakanani na into ebangela ukugabha kwegazi engafanelekanga emzimbeni.

• I-hemophilia engephi: Abantu abane-hemophilia engephi banezinto ezinciphisa igazi kancinci kuneziqhelekileyo. Banokopha ixesha elifutshane kuphela ukuba benzakele kakhulu, batyandwe, okanye bakhutshwe amazinyo. Basenokungazi nokuba bane-hemophilia de kwenzeke into embi kakhulu.
• I-hemophilia ephakathi: Aba bantu baneempawu ezimbalwa zokuqunjelwa kwegazi. Banokopha kakhulu ngenxa yokwenzakala okuncinci. Ngamanye amaxesha, banokopha ngaphandle kwesizathu (ukopha okuzenzekelayo).
• I-hemophilia enzulu: Aba bantu banezinga eliphantsi kakhulu lezinto ezibangela ukujiya kwegazi. Banokopha emalungwini nasezihlunwini ngaphandle kwesizathu esicacileyo. Nokuba yinxeba elincinci kakhulu linokubangela ingxaki enkulu.

Kutheni oku kusenzeka? Zithini izizathu?

I-Hemophilia idla ngokuba yimeko yemfuza , oko kuthetha ukuba idluliselwa kubazali ukuya ebantwaneni ngeemfuza.

Indlela evela ngayo kwilifa (ilifa elidityaniswe ne-X)

I-gene ye-hemophilia ikwi-chromosome X. Uyazi, abafazi bane-chromosomes ezimbini ze-X (XX), ngelixa amadoda ene-chromosome enye ye-X kunye ne-chromosome enye ye-Y (XY).

• Umntwana oyintombazana: Ufumana u-X omnye kunina kunye no-X omnye kuyise.
• Umntwana oyinkwenkwe: Ufumana u-X omnye kumama kunye no-Y omnye kutata.

Ngoku, ukuba i-gene enesiphene ebangela i-hemophilia ikwi-chromosome X,

• Ukuba inkwenkwe ifumana elo chromosome X linesiphene kunina, iya kuba ne-hemophilia. Kuba ine-chromosome X enye kuphela. Ukuba kukho isiphene kuyo, akukho X enye yokuyibuyisela.
• Ukuba intombazana ifumana i-chromosome X enesiphene kunina okanye kuyise, kodwa ine-chromosome X ephilileyo, ayiyi kubonisa zimpawu. Kodwa iya kuba ngumthwali . Oku kuthetha ukuba nokuba ayinayo i-hemophilia, inokudlulisela i-gene enesiphene kubantwana bayo. Amaxesha amaninzi, abathwali basetyhini banokubonisa iimpawu ezincinci.

Yiyo loo nto i-hemophilia idla ngokuvela kumadoda .

Khawucinge nje, ukuba umama unesifo se-hemophilia, wonke umntwana oyinkwenkwe ozelweyo unethuba elingama-50% lokufumana isifo se-hemophilia. Ngokufanayo, wonke umntwana oyintombazana unethuba elingama-50% lokuba nesifo se-hemophilia.

Ingaba isebenza kwiintsapho? (Utshintsho oluzenzekelayo)

Akunyanzelekanga ukuba umntu kusapho abe ne-hemophilia. Ngamanye amaxesha, malunga ne-30% yamatyala, i-hemophilia inokwenzeka njengotshintsho oluzenzekelayo kwi-gene. Kwimeko enjalo, akukho mntu kusapho onokuba wayenale meko ngaphambili.

Zithini iimpawu ze-hemophilia? Ifunyaniswa njani?

Iimpawu ze-hemophilia ziyahluka ngokuxhomekeke kubukhali bemeko. Iimpawu eziqhelekileyo ziquka:

• Ukuvela rhoqo kwemivumbo emikhulu nenzulu: Nokuba iqhuma elincinci linokubangela imivumbo emikhulu.
• Ukopha okuqhubekayo ngenxa yokwenzakala, ukukhutshwa kwezinyo, okanye emva kotyando.
• Ukopha empumlweni ngaphandle kwesizathu.
• Iintsini eziphuma igazi.
• Ukopha igazi kwilungu (hemarthrosis): Oku kubuhlungu kakhulu. Ilungu liyadumba, lishushu, kwaye alikwazi ukuhamba kakuhle. Amalungu afana namadolo, iingqiniba, kunye namaqatha achaphazeleka kakhulu. Ekuhambeni kwexesha, oku kunokubangela umonakalo kwilungu.
• Ukopha ngaphakathi kwimisipha: Oku kubangela intlungu kunye nokudumba.
• Ukopha ngomchamo okanye ngendle.
• Kwiintsana: Ngamanye amaxesha uphawu lokuqala lukopha okungapheliyo emva kokwaluka. Okanye emva kokufakwa inaliti, indawo isenokudumba kakhulu kwaye yophe.
• Imeko embi kakhulu, kodwa engaqhelekanga, kukopha kwegazi engqondweni. Oku kunokubangela iimpawu ezifana nentloko ebuhlungu, ukuhlanza, ukozela, kunye nokuxhuzula. Le yimeko engxamisekileyo.

Ugqirha ufumanisa njani ukuba le yi-hemophilia? (Ukuxilongwa)

Ukuba wena okanye umntwana wakho unenye yezi mpawu, eyona nto ingcono onokuyenza kukubona ugqirha. Ugqirha uhlala esenza uxilongo ngezi ndlela zilandelayo:

1. Ukubuza ngembali yosapho: Ukujonga ukuba kukho umntu kusapho one-hemophilia okanye ezinye iingxaki zokopha.

2. Uvavanyo lomzimba lwenziwa: Ukujonga ukuba akukho mivumbo, amalungu avuvukileyo, njl.njl.

3. Uvavanyo lwegazi lwenziwa:

• Uvavanyo lokuhlola: Olu vavanyo lulinganisa ixesha elithathayo ukuze igazi lijiye. Umzekelo, uvavanyo olufana ne -PTT (Partial Thromboplastin Time) kunye ne-PT (Prothrombin Time) . Amaxabiso e-PTT anokwandiswa kwi-hemophilia.
• Uvavanyo lwezinto ezibangela ukugabha: Ezi zisetyenziselwa ukumisela ukuba iFactor VIII kunye neFactor IX azinazo na iintsilelo, kwaye zifikelela kuwuphi umlinganiselo. Oku kusetyenziselwa ukumisela uhlobo kunye nobunzima be-hemophilia.

Nokuba ngexesha lokukhulelwa, ukuba kukho imbali yosapho ene-hemophilia, uvavanyo lunokwenziwa ukuze kubonwe ukuba umntwana unayo na i-hemophilia.

Zithini iindlela zokunyanga oku? (Unyango)

Nangona i-hemophilia yimeko enganyangekiyo, kukho unyango olusebenzayo kakhulu olufumanekayo.Olu nyango lunokunceda ekulawuleni nasekuthinteleni ukopha, kwaye lukuncede uphile ubomi obungcono.

Unyango Lokutshintsha

Olu lolona nyango luphambili. Lubandakanya ukunika umzimba i-blood clotting factor (Factor VIII okanye Factor IX) enganelanga emzimbeni ngemithambo (i-intravenous okanye i-IV infusion). Ezi zinto zenziwe nge-plasma yegazi lomntu okanye ngeteknoloji ye-genetic (iimveliso eziphinda zisetyenziswe).

Olu nyango lwenziwa ngeendlela ezimbini:

1. Unyango lokuthintela: Kule meko, le nto inikwa umzimba izihlandlo ezininzi ngeveki, ngaphambi kokuba kuphume igazi. Oku kunokuthintela ukopha ngequbuliso, ingakumbi emalungeni. Oku kudla ngokwenziwa kubantu abane-hemophilia enzima.

2. Unyango olufunekayo: Kule meko, olu nyango lunikwa kuphela emva kokuba kuqalile ukopha. Olu nyango lusetyenziswa kubantu abane-hemophilia encinci kunye nabo bangekho kunyango lokuthintela.

Amanye amayeza

• I-Desmopressin (DDAVP): Eli liyeza elingasetyenziswa kubantu abane-hemophilia A engephi. Lisebenza ngokukhupha i-Factor VIII egciniweyo emzimbeni. Linokunikwa njenge-spray yempumlo okanye njenge-injection.
• Amayeza okulwa ne-fibrinolytic: Umzekelo , i-tranexamic acid . La mayeza asebenza ngokucothisa ukunyibilika kwehlwili legazi. Aluncedo kwiimeko ezinje ngokukhupha amazinyo kunye nokopha kwempumlo.
• Izibulali-ntlungu: Ungathatha izinto ezifana neparacetamol ukuze uthomalalise iintlungu ezibangelwa kukopha kwamalungu. Nangona kunjalo, amayeza afana ne -aspirin kunye ne -NSAIDs (umz., i-ibuprofen, i-diclofenac) awacetyiswa kubantu abane-hemophilia, kuba anokunyusa umngcipheko wokopha.

Wenza ntoni xa uphuma igazi?

Kukho izinto ezimbalwa ekufuneka uzenze xa uphuma igazi. Khumbula indlela ye-RICE .

• R (Phumla): Phumla ilungu okanye umsipha owonzakeleyo. Yigcine ingashukumi.
• I (Ice): Faka ipakethe yomkhenkce kwindawo eyonzakeleyo malunga nemizuzu eli-15-20, amaxesha amaninzi ngemini. Oku kuya kunciphisa ukudumba kunye nentlungu.
• C (Uxinzelelo): Songa indawo eyonzakeleyo ngebhandishi elastiki ukuze iqine kancinci.
• E (Ukuphakama): Gcina ingalo okanye umlenze owonzakeleyo ungaphezulu kwenqanaba lentliziyo.

Ngelixa usenza ezi zinto, kufuneka uthethe nogqirha wakho kwaye ufumane unyango lweFactor ukuba kuyimfuneko.

Indlela yokuphila ne-hemophilia

Ukuphila ne-hemophilia kunokuba nzima, kodwa ngolawulo olufanelekileyo , ulwazi kunye nenkxaso, ungaphila ubomi obuqhelekileyo nobusebenzayo.

• Inkxaso evela kwiqela loogqirha abaziingcali: Xa unyanga i-hemophilia,Kubaluleke kakhulu ukufumana inkxaso kwiqela eliquka ingcali ye-hematologist, abahlengikazi, iingcali zonyango lomzimba, kunye nabasebenzi bezentlalo. Baza kukunika iingcebiso malunga nonyango, umthambo, kunye nendlela yokuhlala ukhuselekile.
• Zibandakanye kwimisebenzi ekhuselekileyo: Abantu abane-hemophilia kufuneka baphephe imidlalo enokubangela ukwenzakala okukhulu (umz., i-rugby, i-boxing). Endaweni yoko, zibandakanye kwimithambo ekhuselekileyo efana nokudada, ukuhamba, kunye nokukhwela ibhayisekile (unxibe isigcina-ntloko). Thetha nogqirha wezonyango ukuze ukhethe umthambo ofanelekileyo kuwe.
• Gcina impilo yamazinyo intle: Kubaluleke kakhulu ukuthintela ukubola kwamazinyo kunye nesifo seentsini, njengoko kukho umngcipheko wokopha xa kusenziwa iinkqubo ezifana nokukhupha amazinyo. Qhubeka ubona ugqirha wamazinyo.
• Ukuchongwa kwesilumkiso sezonyango: Soloko uphatha isongo okanye ikhadi elibonisa ukuba une-hemophilia. Inokuba luncedo kakhulu xa kukho imeko engxamisekileyo.
• Inkxaso yoluntu: Ukuthetha nabanye abantu abane-hemophilia kunye neentsapho zabo kunye nokwabelana ngamava ngamandla amakhulu. Ukuba kukho imibutho enjalo, yijoyine.
• Ithemba ngekamva: Unyango olutsha lwe-hemophilia luhlala lufunyanwa. Uphando luyaqhubeka nakwizinto ezifana nonyango lwezakhi zofuzo . Ngoko ke singaba nethemba ngekamva.

Umyalezo Wokuya Ekhaya

I-Hemophilia yingxaki yokuqunjelwa kwegazi, kodwa ayisiyonto yokoyika kwaye inokulawulwa kakuhle .

Eyona nto ibalulekileyo kukufuna ingcebiso kagqirha ngokukhawuleza ukuba uneempawu, ufumane uxilongo oluchanekileyo, kwaye ulandele unyango olunikiweyo.

Ukuba wena okanye umntu omaziyo une-hemophilia, khumbula, awuwedwa. Ngolwazi, inkxaso, kunye nonyango olufunayo, nawe ungaphila ubomi obusempilweni nobusebenzayo. Musa ukulahla ithemba!

I- Hemophilia, ukopha, ukujiya kwegazi, izifo zemfuza, iFactor VIII, iFactor IX