Ingabe ngisho nokusikeka okuncane kwakho akuyeki yini ukuphuma kwegazi? Ake sixoxe nge-hemophilia?

Uyakhumbula ngesikhathi useyingane, lapho uwa futhi ukhubeka idolo noma indololwane, ukopha kwakuvele kume ngemva kwesikhashana? Akumangalisi lokho? Lapho siphuma igazi, imizimba yethu inesimiso sokulimisa, okuyisimiso esenza amahlule egazi. Kodwa kukhona abanye abantu, futhi le nqubo yokuvaleka kwegazi ayisebenzi kahle emizimbeni yabo. Yilapho sikhuluma khona ngesimo esibizwa ngokuthi i-hemophilia . Lokhu kungaba yinto engathí sina, kodwa ngokuphathwa okufanele , kuyisimo esingelashwa kahle.

Kuyini ngempela i-hemophilia?

Kalula nje, i-hemophilia yisimo lapho igazi lethu lingavimbi kahle, okusho ukuthi uma sopha, aliyeki kalula . Ngokuyinhloko yisifo sofuzo , okusho ukuthi singadluliselwa ngezakhi zofuzo kusuka esizukulwaneni kuya kwesinye.

Ake sithi unenxeba elincane esandleni sakho. Ngokuvamile, amaprotheni athile egazini lethu, abizwa ngokuthi izinto ezibangela ukujiya kwegazi , asebenza ndawonye ukuvala inxeba nokuvimba ukuphuma kwegazi. Kufana nokuvala imbobo odongeni.

Kodwa kumuntu one-hemophilia, enye yalezi zinto ezibangela ukujiya kwegazi ayikho ngobuningi obanele, noma leyo nto ayisebenzi kahle. Yileyo inkinga. Yingakho bengopha igazi isikhathi eside ngisho noma benokulimala okuncane, noma bangaphuka kalula.

Indlela amahlule egazi avele ngayo kalula

Uma siqala ukopha, kunezinyathelo eziningana zokukuvimba.

1. Into yokuqala eyenzekayo ukuthi imithambo yegazi iyancipha. Bese inani legazi eliphumayo lehla kancane.

2. Ngemuva kwalokho, amangqamuzana amancane abizwa ngokuthi ama-platelet egazini lethu ayafika endaweni yokulimala futhi anamathele ndawonye, ​​akhe uhlobo lwedamu elincane.

3. Okulandelayo, izici zokujiya kwegazi engizikhulumile ekuqaleni ziyavuselelwa, zisebenza ngokulandelana, zakha i-mesh eqinile ebizwa ngokuthi i-fibrin , iqinisa kakhulu i-platelet barrier futhi imise ngokuphelele ukuphuma kwegazi.

Okwenzekayo ku-hemophilia ukuthi kulesi sinyathelo sesithathu, esinye sezici zokujiya kwegazi asitholakali, ngakho-ke i-clot enamandla ayikhi.

Ingabe zikhona izinhlobo ze-hemophilia?

Yebo, kunezinhlobo ezimbili eziyinhloko ze-hemophilia, kuye ngokuthi iyiphi i-clotting factor entulekayo.

I-Hemophilia A

Lolu uhlobo oluvame kakhulu lwe-hemophilia. Lubangelwa ukungabi ne -clotting factor VIII eyanele noma ukungasebenzi kahle kwayo.

I-Hemophilia B

Lolu hlobo lubizwa nangokuthi iSifo sikaKhisimusi . Lubangelwa ukuntuleka kwe-nine blood clotting factor (Factor IX).Kulungile ukuthi akwanele, kulungile ukuthi akusebenzi kahle.

Izimpawu zazo zombili izinhlobo zifana kakhulu. Kodwa-ke, kubaluleke kakhulu ukuhlonza lezi zinhlobo ezimbili ngokunembile lapho uzelapha. Ngoba ukwelashwa kufanele kunikezwe ngokwesici esehlile.

Ubucayi balesi simo

Ubukhali be-hemophilia bungahlukahluka kumuntu nomuntu, kuye ngokuthi ingakanani into ebangela ukujiya kwegazi ekhona emzimbeni.

• I-hemophilia encane: Abantu abane-hemophilia encane banezici zokujiya kwegazi eziphansi kancane kunezivamile. Bangase bophe isikhathi esifushane kuphela uma benokulimala okukhulu, ukuhlinzwa, noma ukukhishwa kwezinyo. Bangase bangazi nokuthi bane-hemophilia kuze kube yilapho kwenzeka into enkulu.
• I-hemophilia ephakathi nendawo: Laba bantu banezici ezimbalwa zokujiya kwegazi. Bangase bophe kakhulu ngisho noma belimale kancane. Ngezinye izikhathi, bangase bophe ngaphandle kwesizathu (ukopha okungahleliwe).
• I-hemophilia enzima: Laba bantu banamazinga aphansi kakhulu ezici zokujiya kwegazi. Bangaphuma igazi emalungeni nasemisipheni ngaphandle kwesizathu esibonakalayo. Ngisho nokulimala okuncane kakhulu kungabangela inkinga enkulu.

Kungani lokhu kwenzeka? Yiziphi izizathu?

I-Hemophilia ivame ukuba yisimo sofuzo , okusho ukuthi idluliselwa kusuka kubazali iye ezinganeni ngokusebenzisa izakhi zofuzo.

Indlela okuvela ngayo kwifa (ifa elixhunywe ku-X)

I-gene ye-hemophilia ikwi-chromosome X. Uyazi, abesifazane banama-chromosome amabili e-X (XX), kuyilapho abesilisa bene-chromosome eyodwa ye-X kanye ne-chromosome eyodwa ye-Y (XY).

• Ingane yesifazane: Ithola u-X owodwa kunina kanye no-X owodwa kuyise.
• Ingane yesilisa: Ithola u-X oyedwa kumama kanye no-Y oyedwa kuyise.

Manje, uma i-gene enephutha ebangela i-hemophilia iku-chromosome X,

• Uma umfana ethola ifa lelo chromosome X elinephutha kunina, uzoba ne-hemophilia. Ngoba une-chromosome X eyodwa kuphela. Uma kukhona isici kuyo, ayikho enye i-X yokuyibuyisela.
• Uma intombazane izuza i-chromosome X enephutha kunina noma kuyise, kodwa ine-chromosome X enempilo, ngeke ibonise izimpawu. Kodwa izoba yithwali . Lokhu kusho ukuthi noma ingenayo i-hemophilia, ingadlulisela isakhi sofuzo esinephutha kubantwana bayo. Akuvamile kakhulu ukuthi abesifazane abathwala lesi sakhi nabo babonise izimpawu ezincane.

Yingakho i-hemophilia ivame ukwenzeka kwabesilisa .

Cabanga nje, uma umama enesifo se-hemophilia, wonke umntwana wesilisa ozalwa naye unethuba elingu-50% lokuthola i-hemophilia. Ngokufanayo, wonke umntwana wesifazane unethuba elingu-50% lokuba yimthwali.

Ingabe isebenza emindenini? (Ukuguquka okuzenzakalelayo)

Akudingeki ngaso sonke isikhathi ukuthi othile emndenini abe ne-hemophilia. Ngezinye izikhathi, cishe ezimweni ezingama-30%, i-hemophilia ingenzeka njengokuguquka okuzenzakalelayo kwezakhi zofuzo. Kuleso simo, akekho omunye emndenini okungenzeka ukuthi wayenalesi simo ngaphambili.

Ziyini izimpawu ze-hemophilia? Itholakala kanjani?

Izimpawu ze-hemophilia ziyahlukahluka kuye ngokuthi isimo sibucayi kangakanani. Izimpawu ezivamile zifaka:

• Ukuvela njalo kwemivimbo emikhulu nejulile: Ngisho neqhubu elincane lingabangela imivimbo emikhulu.
• Ukopha okuqhubekayo ngenxa yokulimala, ukukhishwa kwezinyo, noma ngemva kokuhlinzwa.
• Ukopha ekhaleni ngaphandle kwesizathu.
• Izinsini eziphuma igazi.
• Ukopha okungena emalungeni (hemarthrosis): Lokhu kubuhlungu kakhulu. Ilunga liyavuvukala, lishise, futhi lingakwazi ukunyakaza kahle. Amalunga afana namadolo, izindololwane, namaqakala avame ukuthinteka. Ngokuhamba kwesikhathi, lokhu kungadala nomonakalo emalungeni.
• Ukopha emisipheni: Lokhu kubangela nobuhlungu nokuvuvukala.
• Ukopha ngomchamo noma ngendle.
• Kwabancane: Ngezinye izikhathi uphawu lokuqala luwukuphuma kwegazi okuqhubekayo ngemva kokusoka. Noma ngemva komjovo, indawo ingase ivuvuke kakhulu futhi yophe igazi.
• Isimo esibi kakhulu, kodwa esingavamile, ukopha kuya ebuchosheni. Lokhu kungabangela izimpawu ezifana nekhanda elibuhlungu, ukuhlanza, ukozela kanye nokuxhuzula. Lesi yisimo esiphuthumayo.

Udokotela unquma kanjani ukuthi lokhu kuyi-hemophilia? (Ukuxilongwa)

Uma wena noma ingane yakho ninezinye zalezi zimpawu, into engcono kakhulu ongayenza ukubonana nodokotela. Udokotela uvame ukwenza ukuxilongwa ngezindlela ezilandelayo:

1. Ukubuza ngomlando womndeni: Ukuhlola ukuthi ukhona yini emndenini one-hemophilia noma ezinye izinkinga zokopha.

2. Kuhlolwa umzimba: Kuhlolwa imivimbo, amalunga avuvukele, njll.

3. Ukuhlolwa kwegazi kwenziwa:

• Ukuhlolwa kokuhlola: Lokhu kulinganisa ukuthi kuthatha isikhathi esingakanani ukuthi igazi lijiye. Isibonelo, ukuhlolwa okufana ne -PTT (Isikhathi se-Thromboplastin Esiyingxenye) kanye ne -PT (Isikhathi se-Prothrombin) . Amanani e-PTT angase ande ku-hemophilia.
• Ukuhlolwa kwezinto ezivala igazi: Lokhu kusetshenziselwa ukunquma ukuthi i-Factor VIII ne-Factor IX azinawo yini umthelela, nokuthi zingakanani. Lokhu kusetshenziselwa ukunquma uhlobo kanye nobukhali be-hemophilia.

Ngisho nangesikhathi sokukhulelwa, uma kunomlando womndeni we-hemophilia, ukuhlolwa kungenziwa ukuze kubonakale ukuthi ingane inayo yini i-hemophilia.

Yiziphi izindlela zokwelapha lokhu? (Ukwelashwa)

Nakuba i-hemophilia iyisimo esingelapheki, kunezindlela zokwelapha eziphumelela kakhulu ezitholakalayo.Lokhu kwelashwa kungasiza ekulawuleni nasekuvimbeleni ukopha, futhi kukusize uphile impilo engcono.

Ukwelashwa Kokufaka Esikhundleni

Lokhu ukwelashwa okuyinhloko. Kuhilela ukunikeza umzimba i-blood clotting factor (Factor VIII noma Factor IX) enganele emzimbeni ngemithambo yegazi (i-intravenous noma i-IV infusion). Lezi zinto zenziwa nge-plasma yegazi lomuntu noma ngobuchwepheshe bezakhi zofuzo (imikhiqizo ephindaphindayo).

Lokhu kwelashwa kwenziwa ngezindlela ezimbili:

1. Ukwelashwa kokuvimbela: Kulokhu, lesi sici sinikezwa umzimba izikhathi eziningana ngesonto, ngaphambi kokuba kuphume igazi. Lokhu kungavimbela ukuphuma kwegazi ngokuzumayo, ikakhulukazi emalungeni. Lokhu kuvame ukwenziwa kubantu abane-hemophilia enzima.

2. Ukwelashwa ngesikhathi sokufunwa: Kulokhu, lesi sici sinikezwa kuphela ngemva kokuba igazi seliqalile ukuphuma. Lokhu kwelashwa kusetshenziselwa abantu abane-hemophilia encane kanye nalabo abangasebenzisi ukwelashwa kokuvimbela.

Eminye imithi

• I-Desmopressin (i-DDAVP): Lona umuthi ongasetshenziswa kubantu abane-hemophilia A encane. Usebenza ngokukhipha i-Factor VIII egciniwe emzimbeni. Unganikezwa njengesifutho sekhala noma njengomjovo.
• Imithi yokulwa ne-fibrinolytic: Isibonelo , i-tranexamic acid . Le mithi isebenza ngokunciphisa ukuqhekeka kwehlule legazi. Iwusizo ezimweni ezifana nokukhishwa kwamazinyo nokopha kwamakhala.
• Ama-Painkiller: Ungathatha izinto ezifana ne-paracetamol ukuze unciphise ubuhlungu obuhambisana nokopha emalungeni. Kodwa-ke, imithi efana ne -aspirin kanye ne-NSAID (isib. i-ibuprofen, i-diclofenac) ayinconywa kubantu abane-hemophilia, njengoba ingandisa ingozi yokopha.

Wenzani uma uphuma igazi?

Kunezinto ezimbalwa okufanele uzenze uma uphuma igazi. Khumbula indlela ye-RICE .

• R (Ukuphumula): Phumuza ilunga noma umsipha olimele. Vimbela ukuthi ungashukumi.
• I (Iqhwa): Faka iphakethe leqhwa endaweni elimele cishe imizuzu eyi-15-20, izikhathi eziningana ngosuku. Lokhu kuzonciphisa ukuvuvukala nobuhlungu.
• C (Ukucindezela): Songa indawo elimele ngebhandeji elinwebekayo ukuze iqine kancane.
• E (Ukuphakama): Gcina ingalo noma umlenze olimele ungaphezulu kwezinga lenhliziyo.

Ngesikhathi wenza lezi zinto, kufanele ukhulume nodokotela wakho futhi uthole ukwelashwa kwe-Factor uma kudingeka.

Indlela yokuphila ne-hemophilia

Ukuphila ne-hemophilia kungaba yinselele, kodwa ngokuphathwa kahle , ukuqwashisa, kanye nokusekelwa, ungaphila impilo evamile nesebenzayo.

• Ukusekelwa yithimba lodokotela abangochwepheshe: Lapho welapha i-hemophilia,Kubaluleke kakhulu ukuthola ukwesekwa eqenjini elihlanganisa udokotela wegazi, abahlengikazi, ochwepheshe be-physiotherapists kanye nabasebenzi bezenhlalakahle. Bazokweluleka ngokwelashwa, ukuzivocavoca, kanye nendlela yokuhlala uphephile.
• Zibandakanye emisebenzini ephephile: Abantu abane-hemophilia kufanele bagweme imidlalo engabangela ukulimala okukhulu (isb., i-rugby, i-boxing). Kunalokho, zibandakanye ekuzilolongeni okuphephile njengokubhukuda, ukuhamba, kanye nokugibela ibhayisikili (unesigqoko). Khuluma nodokotela wezokwelapha ukuze ukhethe ukuzivocavoca okufanele wena.
• Gcina impilo enhle yamazinyo: Kubaluleke kakhulu ukuvimbela ukubola kwamazinyo nesifo sezinsini, njengoba kunengozi yokopha lapho kwenziwa izinqubo ezifana nokukhipha amazinyo. Qhubeka ubona udokotela wamazinyo.
• Ukuhlonza isexwayiso sezokwelapha: Hlala uphethe isongo noma ikhadi elibhalwe ukuthi une-hemophilia. Kungaba usizo kakhulu esimweni esiphuthumayo.
• Ukusekelwa komphakathi: Ukukhuluma nabanye abantu abane-hemophilia kanye nemindeni yabo nokwabelana ngolwazi kuyisici esinamandla kakhulu. Uma kukhona izinhlangano ezinjalo, zijoyine.
• Ithemba ngekusasa: Ukwelashwa okusha kwe-hemophilia kuhlala kutholakala. Ucwaningo luyaqhubeka nezinto ezifana nokwelashwa ngezakhi zofuzo . Ngakho-ke singaba nethemba ngekusasa.

Umyalezo Wokuya Nawo Ekhaya

I-Hemophilia iyinkinga yokujiya kwegazi, kodwa akuyona into okufanele uyesabe futhi ingaphathwa kahle .

Into ebaluleke kakhulu ukufuna iseluleko sezokwelapha ngokushesha uma unezimpawu, uthole ukuxilongwa okunembile, bese ulandela ukwelashwa okunikeziwe.

Uma wena noma othile omaziyo ene-hemophilia, khumbula, awuwedwa. Ngolwazi, ukwesekwa, kanye nokwelashwa okudingayo, nawe ungaphila impilo enempilo nesebenzayo. Ungalilahli ithemba!

I- Hemophilia, ukopha, ukujiya kwegazi, izifo zofuzo, i-Factor VIII, i-Factor IX