Xa unenxeba elincinci emzimbeni wakho, okanye xa ukhutshiwe izinyo, ngaba ukopha kuyaqhubeka ngaphandle kokuma? Okanye ngamanye amaxesha uba nemivumbo eluhlaza okwesibhakabhaka emzimbeni wakho ngaphandle kokubetheka naphi na? Ngaba ubona imivumbo emzimbeni wakho wonke, ingakumbi xa umntwana wakho eqala ukukhasa okanye ukuhamba? Ukuba izinto ezinje ziyenzeka, unobangela usenokuba yimeko ebizwa ngokuba yi-hemophilia . Musa ukoyika xa usiva eli gama. Ngonyango olufanelekileyo , ungaphila ubomi obuqhelekileyo nobupheleleyo. Masithethe ngale nto ngokucacileyo nangokulula namhlanje.
Ngamafutshane, yintoni iHemophilia A?
Ngamafutshane, iHemophilia A yimeko apho igazi lethu lingajiki kakuhle. Ngokwesiqhelo, xa sifumana ingozi, igazi lethu liyajikixa ukuze kumiswe ukopha. Oku kuncedwa ziiproteni ezikhethekileyo egazini lethu. Ezi sizibiza ngokuba "zizinto ezijikixayo."
Umntu one-hemophilia A akavelisi ngokwaneleyo i-clotting factor Factor VIII . Ubunzima besifo bumiselwa kumlinganiselo apho le protein ye-Factor VIII incitshiswa khona. Ngoko ke, yahlulwe yangamacandelo amathathu:
- I-hemophilia engephi: Amanqanaba e-Factor VIII akhona ukuya kuthi ga kwinqanaba elithile.
- I-hemophilia ephakathi: Amanqanaba e-Factor VIII aphantsi kakhulu.
- I-hemophilia enzulu: Amanqanaba e-Factor VIII aphantsi kakhulu okanye akakho.
Uninzi lwexesha, esi sisifo semfuza esifumaneka kwiintsapho. Nangona kunjalo, malunga nesinye kwisithathu samatyala, umntu omtsha unokuhlaselwa sisifo ngaphandle kokuba nabani na kusapho abe naso.
Yintoni ebangela i-hemophilia?
Le yinto esiyizuza njengelifa ngee-genes. Cinga ngayo, zonke iimpawu zomzimba wethu zimiselwa zii-genes. Ibhinqa line -chromosomes ezimbini ze-X (XX), ngelixa iduna line-chromosome enye ye-X kunye ne-chromosome enye ye-Y (XY).
Isiphene sejini esinxulumene ne-hemophilia sikwi-chromosome X.
Ngoko ke, ukuba umama "ungumthwali" wale gene inesiphako, uhlala engabonakalisi zimpawu. Kuba enye i-chromosome yakhe ye-X ephilileyo yenza iproteni ye-factor VIII efunekayo. Kodwa ukuba unyana wakhe uzuze le chromosome ye-X inesiphako, loo nyana uya kuba ne-hemophilia. Kuba inkwenkwe ine-chromosome enye ye-X kuphela. Ngenxa yesi sizathu, i-hemophilia ixhaphake kakhulu kubafana kunakumantombazana.
Amaxesha amaninzi, abantu abadala abaneminyaka ephakathi kwama-60 nama-80 banokuba ne-hemophilia. Yenzeka xa amajoni omzimba ehlasela izinto ezibangela ukujiya kwegazi. Inokwenzeka nangexesha lokukhulelwa, xa kukho umhlaza , kunye nezinye iimeko zokuzikhusela komzimba .
Zithini iimpawu ze-hemophilia?
Iimpawu zixhomekeke ekubeni imeko yakho incinci, iphakathi, okanye inzima.
| Inqanaba lesifo | Iimpawu ezibonakalayo |
|---|---|
| I-hemophilia ebuthathaka | Ngokwesiqhelo, ukopha kakhulu kwenzeka kuphela emva kotyando, ukukhutshwa kwamazinyo, ukuzala, okanye ingozi enkulu. Abanye abantu abazi nokuba banako de babe ngabantu abadala. |
| I-hemophilia ephakathi | - Xa ulimele, kukho ukopha okugqithisileyo. - Ngamanye amaxesha ukopha kwenzeka ngaphandle kwesizathu. - Umzimba ugruzuka lula kwaye ujika ube luhlaza okwesibhakabhaka. - Xa ufumana ugonyo, kuthatha ixesha elide ukopha. |
| I-hemophilia enzulu | Ukopha igazi rhoqo kwenzeka nokuba akukho monakalo. Ukopha igazi kunokwenzeka ngakumbi kumalungu nakwimisipha . Oku kubuhlungu kakhulu. |
Iimpawu ezilumkisayo zokuphuma kwegazi ebuchosheni
Ukuba umntu one-hemophilia enzima ufumana nokuba yintlungu encinci entloko, ukopha kungangena engqondweni. Le yingxaki enkulu. Ukuba wenzakele entloko kwaye kukho naziphi na iimpawu ezilandelayo, fowunela ugqirha wakho ngoko nangoko okanye uye kwicandelo elikufutshane le-emergency (ETU).
- Intloko ebuhlungu engapheliyo
- Ukuhlanza
- Ukozela rhoqo okanye ukudinwa kakhulu
- Ubuthathaka ngequbuliso okanye ubunzima bokuhamba
- Umbono ophindwe kabini
- Ukuxhuzula
Indlela yokuxilonga esi sifo?
Ukuba kukho umntu kusapho lwakho one-hemophilia, ungenza ukuba umntwana wakho avavanyelwe esi sifo ngelixa ukhulelwe. Nangona kunjalo, kukho iingozi ezithile ezibandakanyekayo kwezi mvavanyo, ngoko ke kufuneka uxoxe ngazo nogqirha wakho.
Iimeko ezinzima ze-hemophilia kubantwana abancinci zihlala zifunyaniswa kunyaka wokuqala wobomi. Ukuba umntwana wakho uqala ukugoba aze aqalise ukukhasa aze aqale ukuqaphela imivumbo ephakanyisiweyo emzimbeni wakhe, thetha nogqirha wakho ngaloo nto.
Ugqirha angakubuza imibuzo efana nale:
- Zenzeke njani ezi zilonda kunye nokopha igazi?
- Kudala kangakanani ukopha?
- Ngaba kukho amayeza abantwana abafanele bawasebenzise?
- Ngaba kukho umntu kusapho lwakho onengxaki yokujiya kwegazi?
Emva koko, kuya kwenziwa uvavanyo lwegazi oluninzi ukuqinisekisa ukuba uxilongiwe. Ungathunyelwa kwi-hematologist.
| Igama lovavanyo | Ubona ntoni kule nto? |
|---|---|
| Inani elipheleleyo legazi (i-CBC) | Iintlobo zeeseli egazini kunye nenqanaba le-hemoglobin ziyajongwa. Oku kunokunceda ekuqinisekiseni ukuba kukho i-anemia ebangelwa kukopha. |
| Uvavanyo lwe-PT kunye ne-aPTT | Ilinganisa ixesha elithathayo ukuze igazi lijiye. Ixabiso le-aPTT lidla ngokunyuka kwi-hemophilia. |
| Uvavanyo lweFactor VIII kunye neFactor IX | Balinganisa ngqo ukuba ingakanani le protein esegazini. Ukuba i-factor VIII iphantsi, yi-hemophilia A. |
| Uvavanyo lweMfuzo | Utshintsho lwemfuza olubangela esi sifo lunokuchongwa. Kwimeko yomfazi, kunokuqinisekiswa ukuba ngaba unesifo na. |
Iphathwa njani?
Indlela yonyango ixhomekeke kubukhulu besifo, ubudala bakho, kunye neemfuno zakho zobuqu. Injongo ephambili yonyango kukutshintsha iproteni ye-factor VIII engekho emzimbeni. Oku kubizwa ngokuba yi-factor replacement therapy . Nangona oku kungasinyangi ngokupheleleyo isifo, kunokunceda ukulawula ukopha kwaye kukuvumele ukuba uphile ubomi obuqhelekileyo.
Kukho iintlobo ezimbini zonyango:
- Unyango Lokuthintela: Ukufakwa rhoqo kwe-factor VIII ngexesha elicwangcisiweyo ukuthintela ukopha, kunokulinda ukuba kopha . Oku kubaluleke kakhulu kubantu abane-hemophilia enzima.
- Unyango olufunekayo: Ukufumana unyango kuphela xa kukho ukopha .
Kukho iintlobo ezimbini zemithi yokugonya ye-factor VIII:
1. I-Recombinant Factor VIII: Izinto eziveliswa kwilabhoratri kusetyenziswa ubunjineli bemfuza. Le yeyona isetyenziswa kakhulu namhlanje.
2. I-Plasma-derived Factor VIII: I-Factor efunyenwe kwi-plasma yegazi lomntu.
Abantu abane-hemophilia A engembi ukuya kwephakathi banokusebenzisa iyeza elibizwa ngokuba yiDesmopressin (DDAVP) . Oku kusebenza ngokukhupha i-factor VIII egciniweyo egazini.
Ukongeza, amayeza athathwa ngomlomo afana neTranexamic Acid, ethintela ukuqhekeka kwegazi, asetyenziswa nakwiimeko ezifana nokukhupha amazinyo.
Kubalulekile: Ekubeni umntu one-hemophilia esenokufuna utofelo-gazi rhoqo, kubaluleke kakhulu ukugonyelwa iHepatitis A kunye ne-B. Buza ugqirha wakho ngale nto.
Uzilawula njani ubomi bemihla ngemihla?
Xa uphila ne-hemophilia, kufuneka ucinge ngakumbi ngokhuseleko.
- Yiba nomdla: Ukuzilolonga kuyayomeleza imisipha. Imisipha eqinileyo ikhusela amalungu omzimba kakuhle. Nangona kunjalo, kuphephe imidlalo efana neragbi kunye nebhokisi. Ukudada, ukuhamba, kunye nokukhwela ibhayisekile (unxibe isigcina-ntloko) zizinto ezilungileyo. Thetha nogqirha wakho malunga nemisebenzi efanelekileyo kuwe.
- Khusela abantwana: Ukuba unomntwana omncinci, nxiba ii-knee pads, ii-elbow pads, kunye nesigcina-ntloko xa udlala. Zikhusele kwifenitshala ebukhali ekhayeni lakho.
- Impilo yamazinyo:Gcina ucoceko oluhle lwamazinyo. Hlamba amazinyo akho yonke imihla. Oku kunokunceda ukunciphisa isidingo sokukhupha amazinyo kunye nonyango olukhulu lwamazinyo. Ngaphambi kokuba uye kugqirha wamazinyo, qiniseka ukuba umxelele ukuba une-hemophilia.
- Amayeza ekungafuneki uwasebenzise: Iipilisi zokunciphisa iintlungu ezifana ne-aspirin kunye ne-NSAIDs (umz., i-ibuprofen, i-diclofenac) zinokuthintela ukujiya kwegazi ngakumbi. Musa ukuthatha naluphi na unyango ngaphandle kokubonisana nogqirha wakho.
Umyalezo Wokuya Ekhaya
- I-Hemophilia A sisifo semfuza esibangelwa kukunqongophala kweproteni yeFactor VIII, efunekayo ukuze igazi lijiye.
- Oku kubonakala kakhulu kumakhwenkwe, kodwa abafazi banokuba ngabathwali besifo.
- Iimpawu eziphambili kukuphuma kwegazi elininzi nokuba kukwenzakala okuncinci okanye ukukrweleka.
- Ukuba wenzakele entloko kwaye uneempawu ezifana nentloko ebuhlungu, ukuhlanza, okanye ukozela, yingxaki enkulu leyo. Funa ingcebiso kagqirha ngoko nangoko.
- Ngonyango olufanelekileyo (Factor Replacement Therapy) kunye nendlela yokuphila ekhuselekileyo, abantu abane-hemophilia banokuphila ubomi obupheleleyo nobusebenzayo.
- Soloko uxoxa nogqirha wakho ngayo yonke imithi oyithathayo ngaphambi kokuba utyando okanye unyango lwamazinyo.
💬 Comments (0)
Akukho zimvo zithunyelweyo okwangoku. Faka uluvo lwakho apha okokuqala.
Faka uluvo lwakho