I-Haemophilia

Isifo seHaemophilia sithinta cishe umuntu oyedwa kwabayi-10,000 emhlabeni wonke, okwenza imisebenzi yansuku zonke ibe inselele kulabo abaphila nalokhu. ukuphazamiseka kwegazi. Intuthuko yesimanje yezokwelapha idale izindlela zokwelapha ezimbalwa ezisebenzayo ze-haemophilia ezisiza ukulawula iziqephu zokopha futhi kuvimbele izinkinga. Ukuqonda izimbangela zesifo se-haemophilia, ukuhlonza izimpawu ze-haemophilia, kanye nokwazi ukuthi ungasithola nini isiqondiso sezokwelapha kudlala indima ebalulekile ekulawulweni kwezifo ngempumelelo. Lesi sihloko sihlola lezi zici ezibalulekile futhi sinikeza isiqondiso esisebenzayo kubantu abane-haemophilia.

Iyini i-Haemophilia?

Isifo segazi esingavamile, i-haemophilia ithinta ikhono lomzimba lokwenza amahlule egazi. Lesi simo senzeka lapho igazi lingenawo amaprotheni aqandayo anele, aziwa nangokuthi izici zokujiyisa. Lawa maprotheni asebenza kanye namaplatelet ukuze asize igazi lakhe amahlule futhi ayeke ukopha lapho imithambo yegazi ilimele.

Okulandelayo yizinhlobo eziyinhloko ze-haemophilia:

• I-Haemophilia A: Okubangelwa ukushoda kwe-clotting factor VIII (FVIII), lolu uhlobo oluvame kakhulu lwe-haemophilia
• I-Haemophilia B: Eyaziwa nangokuthi Isifo sikaKhisimusi, umphumela wokuntuleka kwe-clotting factor IX (9)
• I-Haemophilia C: Le haemophilia engavamile kakhulu ibangelwa ukushoda kwe-clotting factor XI (11)

Izingozi Nezimbangela Zesifo I-Haemophilia

Ipulani yofuzo ye-haemophilia ingaphakathi kwezakhi zofuzo ezithile ezilawula ukujiya kwegazi. Ukuguqulwa kwezakhi zofuzo ze-F8 noma ze-F9 kuholela ekukhiqizweni kwezici zokujika ezingavamile noma ezinganele, okuvimbela ukwakheka kwehlule legazi ngendlela efanele. Lezi zakhi zofuzo zinikeza iziyalezo zokwenza amaprotheni abizwa ngokuthi i-coagulation factor VIII kanye ne-IX, abalulekile ekuhlulekeni kwegazi okuvamile.

Abantu abaningi bazuza i-haemophilia ngokusebenzisa isakhi sofuzo esinephutha esiku-X chromosome. Njengoba amadoda elayishwe i-X chromosome eyodwa kuphela, awakwazi ukulungisa umonakalo walolofuzo, okuwenza abe sengozini yokuthola lesi simo. Abesifazane ngokuvamile basebenza njengabathwali, abanofuzo olunesici kodwa bangabonisi zimpawu, nakuba abanye bengase babe nezimpawu zokopha okuncane uma izici zabo zokujiyisa zehla ngokulingene.

Nakuba izimo eziningi zitholwa njengefa, abanye abantu baba ne-haemophilia engenamlando womndeni. Lokhu kwenzeka lapho amasosha omzimba ekhiqiza amaprotheni abizwa ngokuthi ama-autoantibodies ahlasela futhi akhubaze izici ze-antihemophilic, ikakhulukazi i-clotting factor VIII. 

Kunezici ezimbalwa ezingadala leli fomu elitholiwe:

• iziyaluyalu sokuhlasela kwamasosha omzimba
• ukukhulelwa kanye nezimo zangemva kokubeletha
• Cancer kanye nokuphazamiseka kwe-lymphoproliferative
• Imithi ethile kanye nokusabela kwezidakamizwa
• izivikeli mzimba ukuphazamiseka
• Ukuhlukumezeka okukhulu noma ukulimala

Izimpawu ze-Haemophilia

Izimpawu ezivame kakhulu ze-haemophilia zihlanganisa:

• Ukopha kakhulu ngenxa yokusikeka noma ukulimala okuqhubeka isikhathi eside kunokujwayelekile
• Imihuzuko engachazeki ebonakala inkulu futhi ijulile
• Ukopha kwamalungu okubangela ubuhlungu, ukuvuvukala, nokuncipha kokuhamba
• Ukopha kwemisipha okubangela ukuvuvukala nokungakhululeki
• Ukopha isikhathi eside ngemva kwezinqubo zamazinyo noma ukuhlinzwa
• Ukuphuma kwegazi ekhaleni okuzenzakalelayo okunzima ukukumisa

Ubukhulu bezimpawu buhambisana ngokuqondile nenani lezinto zokujiyisa ezikhona egazini. 

• I-Haemophilia Encane: Izinkinga zokopha zenzeka kuphela ngemva kokulimala okukhulu noma ukuhlinzwa
• I-Haemophilia Emaphakathi: Ingase yophe isikhathi eside ngokungavamile kulandela ukulimala okuncane
• I-Haemophilia Enzima: Kungase kuhlotshaniswe nokopha okuzenzakalelayo ngaphandle kwesizathu esibonakalayo

Izinkinga

Ukuphila ne-haemophilia kuletha izinkinga ezimbalwa ezingaba nomthelela omkhulu empilweni yomuntu kanye nezinga lempilo. Lokhu kubandakanya:

• Izinkinga Ezihlangene: Ukopha okuphindaphindiwe emalungeni kuholela ku-hemophilic arthropathy, okubangela ukulimala kwamalunga unomphela ngokuhamba kwesikhathi. Lesi simo sithinta ngokuyinhloko ama-hinge joints futhi singabangela:
  • Ubuhlungu obungapheliyo nokuvuvukala
  • Ibanga elincishisiwe lokunyakaza
  • Ukuncipha kwamandla emisipha
  • Ubunzima ngemisebenzi yansuku zonke
• Ingozi Yokopha Kwengqondo: Enye yezinkinga ezimbi kakhulu ze-haemophilia ukopha ngaphakathi ogebhezini lwekhanda, okudinga ukunakwa ngokushesha. Izimpawu eziyisixwayiso zihlanganisa:
  • Izinwele ezinzima
  • Intamo eqinile
  • Ukudideka noma izinguquko zengqondo
  • Inkulumo emisiwe
  • Izinkinga zembono
  • Ukulahlekelwa kokuxhumana
• Inhibitors: Lawa ma-antibody angenza imiphumela yezinto ezijiyisayo ingabi namandla, yenze izindlela zokwelapha ezijwayelekile zingasebenzi kahle. 
• Ama-pseudotumors: Lawa amachibi egazi ngokuvamile akheka emisipha eduze kwamathambo.

Sifo

Izazi ze-Hematologists zisebenzisa ukuhlolwa kwegazi okuhlukahlukene ukuxilonga i-haemophilia:

• Ukuhlola Ukuhlola: Lokhu kuhlolwa kwegazi kokuqala kunquma ukuthi igazi lijiyisa kahle yini
• Ukuhlolwa kwe-Clotting Factor: Okuphinde kubizwe ngokuthi ama-factor assays, lezi zivivinyo ezikhethekile zikala amazinga nomsebenzi wezinto VIII kanye ne-IX
• Ukuhlolwa kofuzo: Ihlola izakhi zofuzo ezithile ukuze kuqinisekiswe ukuxilongwa kanye nohlobo lwe-haemophilia
• Isikrini sokuvala igazi: Ukuhlolwa okuvamile okutholakala kuzo zonke izibhedlela okungase kuphakamise ukuba khona kwe-haemophilia

Ukwelashwa kwe-Haemophilia

Isayensi yezokwelapha yesimanje inikeza izindlela zokwelapha ezimbalwa ezisebenzayo zokulawula isifo se-haemophilia. 

Izinketho zokwelashwa eziyinhloko ezitholakalayo zihlanganisa:

• I-Clotting Factor Replacement Therapy: Ihlinzeka nge-factor VIII engekho noma i-IX ngemijovo evamile
• I-Bispecific Antibodies: Isebenza njengebhuloho phakathi kwezici zokujiyisa ukuze kuthuthukiswe ukujiya kwegazi
• I-Gene Therapy: Sethula amakhophi asebenzayo ezakhi zofuzo ze-clotting factor ezingekho
• Ama-ejenti okulinganisa kabusha: Isiza ukubuyisela ibhalansi phakathi kwezinto ezilwa ne-clotting kanye ne-clotting

Uzoyibona Nini Udokotela

Abantu abane-haemophilia kufanele bafune usizo lwezokwelapha ngokushesha uma behlangabezana:

• Ikhanda elibuhlungu elibuhlungu ngokuphalaza noma umbono ongenalutho
• Izinguquko ebuntwini noma ukozela okungajwayelekile
• Isisu esinzima noma ukuhlungu obusemhlane
• Igazi emcimbini noma indle
• Ukuqina kwamalunga okuhambisana nobuhlungu nokuvuvukala
• Ubunzima bokukhuluma noma ukushintsha umbono
• Ukulahlekelwa ukusebenzisana nokulinganisela
• Uma ukopha kungayeki phakathi nemizuzu eyi-10 yokufaka ingcindezi. 

Abazali bezingane ezine-haemophilia kufanele baqaphele ikakhulukazi izimpawu ezihlobene namalunga. Izimpawu zokuqala zokopha kwamalunga zihlanganisa:

• Izingane kanye nezingane ziyenqaba ukukhasa noma ukuhamba 
• Ukunwaya noma ukubhavuza esihlokweni
• Ukunyakaza okulinganiselwe noma okubuhlungu
• Ukuvuvukala nokufudumala endaweni ethintekile
• Ukuqina okungajwayelekile noma ukungakhululeki

Prevention

Nakuba i-haemophilia ingenakuvinjelwa ngenxa yemvelo yayo yofuzo, izinyathelo ezifanele zokuvimbela zingasiza ukunciphisa iziqephu zokopha kanye nezinkinga. 

• Ukwelashwa kwe-Prophylactic: Abantu abane-haemophilia kufanele balondoloze amazinga afanele we-clotting factor ngokusekelwe emisebenzini yabo. Amaleveli anconyiwe afaka:
  • Ngaphezu kuka-5% wemisebenzi evamile yansuku zonke
  • Ngaphezulu kuka-15% wemisebenzi yezemidlalo
  • I-20-40% ngaphambi kweseshini ye-physiotherapy
• Ukuphatha Umsebenzi Womzimba: Ukubamba iqhaza okuphephile emisebenzini yomzimba kudinga ukuhlela ngokucophelela nokuhlola ubungozi. Imisebenzi enobungozi obuncane enconywa odokotela ihlanganisa:
  • Ukubhukuda
  • Ukuhamba
  • Ibhayisikili
• Gwema Imidlalo Yokuxhumana: Ubuciko bokulwa, ibhola likanobhutshuzwayo, nehokhi kufanele kugwenywe ngenxa yezingozi ezopha kakhulu.
• Ukunakekelwa Kwamazinyo Okufanelekile: Ukuhlolwa kwamazinyo njalo nokugcina umlomo uhlanzekile kusiza ekuvimbeleni isifo sezinsini nokopha kakhulu phakathi nezinqubo zamazinyo. 
• Ukwelulekwa ngofuzo: Lokhu kusiza abantu ngabanye baqonde amaphethini amafa, benze izinqumo zokuzala benolwazi, futhi balungiselele izinselele ezingaba khona. Ukuze kuvinjwe ukulimala, kufanele kugqokwe igiya lokuvikela elifanele imisebenzi ethile. 
• Nakekela Ngenkathi Uthatha Imithi: Iziguli kufanele zigweme imithi ethinta ukujiya kwegazi, njenge-aspirin nemithi ethile eqeda ukuvuvukala ngaphandle uma kugunyazwe udokotela wazo ngokuqondile.

Isiphetho

Imithi yesimanje iletha ithemba kubantu abaphila ne-haemophilia ngokwelashwa okuthuthukile kanye nezindlela zokunakekela ezibanzi. Ukwelapha ngokushintsha izinto, ukwelapha ngezakhi zofuzo, nezikhungo zokwelapha ezikhethekile kunikeza iziguli izinketho eziningi kunangaphambili. Ukugadwa njalo kwezokwelapha, kuhlanganiswe nezinyathelo zokuvimbela kanye nokulungiswa kwendlela yokuphila, kusiza abantu abaningi abane-haemophilia baphile ukuphila okumatasa, nokwanelisayo. Amathimba okunakekelwa kwezempilo ayaqhubeka nokusebenza ukuthuthukisa ukwelashwa ngenkathi esekela iziguli ekulawuleni lesi simo ngempumelelo.

Imibuzo Evame Ukubuzwa

1. Ingabe abantu bangaba ne-haemophilia?

Nakuba izimo eziningi ze-haemophilia zitholwa ngofuzo, abanye abantu bangathola i-haemophilia kamuva ekuphileni. Leli fomu elingavamile lenzeka lapho amasosha omzimba enza amasosha omzimba ahlasela izici zokujiyisa. Izimo ezithile zingadala i-haemophilia etholakele, okuhlanganisa:

• ukukhulelwa
• iziyaluyalu sokuhlasela kwamasosha omzimba
• Cancer
• Imithi ethile

2. Ungakumisa kanjani ukopha nge-haemophilia?

Ukulawula iziqephu zokopha kudinga isinyathelo esisheshayo kanye nokwelashwa okufanele kwesifo se-haemophilia. Indlela eyinhloko ihilela ukufaka esikhundleni sezici zokujiyiza ezingekho ngokufakwa emthanjeni. Ukusebenzisa ingcindezi eqinile namaphakethe eqhwa kungasiza ekulawuleni ukopha ngenkathi ulinde ukwelashwa kokubuyisela isici ukuze kusebenze ukusikeka okuncane.

3. Ingabe i-haemophilia ibuhlungu?

I-haemophilia ingabangela ubuhlungu obukhulu, ikakhulukazi lapho ukopha kuvela emalungeni noma emisipha. Ukuqina kobuhlungu kuyahlukahluka futhi kuya ngendawo nobukhulu bokopha. Ukwelashwa okuvamile kwe-prophylactic kusiza ukuvimbela iziqephu zokopha okubuhlungu futhi kunciphisa ukulimala kwamalunga kwesikhathi eside.

4. Ingabe i-haemophilia ingelapheka?

Njengamanje, awekho amakhambi e-haemophilia. Nokho, izindlela zokwelapha zesimanje zisiphatha ngokuphumelelayo lesi simo. Ukwelashwa ngofuzo kubonisa imiphumela ethembisayo ezivivinyweni zocwaningo, okungenzeka zinikeze izixazululo zesikhathi eside kwezinye iziguli esikhathini esizayo.

5. Yikuphi ukudla okuvimbela i-haemophilia?

Nakuba kungekho ukudla okuqondile okuvimbela i-haemophilia, imisoco ethile isekela impilo yonke yegazi. Ukudla okunothe nge-iron, uvithamini K, novithamini C kungasiza ekugcineni ukusebenza kahle kwegazi. Lokhu kubandakanya:

• Imifino eluhlaza
• Inyama emnandi
• Izithelo ze-Citrus
• Ukudla okuphelele

6. I-haemophilia itholakala ineminyaka emingaki?

Izigameko eziningi ze-haemophilia zitholakala kusenesikhathi. Ngokwemininingwane yezokwelashwa, izigameko ezinzima zivame ukuhlonzwa phakathi nenyanga yokuqala yokuphila, izimo ezimaphakathi ngezinyanga eziyisishiyagalombili, kanye nezincane kakhulu ngezinyanga ezingama-36. Ezinye izimo ezithambile zingase zingatholakali kuze kube kamuva ekuphileni, ngokuvamile ngemva kokuhlinzwa noma ukulimala.

UDkt. Kunal Chhattani

CARE Medical Team

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