Ukuphononongwa kweentlobo, iimpawu, ukuxilongwa kunye nokunye
I-Thalassemia yintlungu ye-hemoglobin eyenza i-hemolytic anemia . I-Hemolysis yigama lokuchaza ukutshatyalaliswa kweeseli ezibomvu zegazi. Kubantu abadala, i-hemoglobin yenziwe ngamaketanga amane-iintsimbi ezimbini ze-alpha kunye neetambo ezimbini ze-beta.
Kwi-thalassemia awukwazi ukwenza i-alfabhe okanye i-beta yeetyali ngexabiso elaneleyo ukwenza umongo wakho wethambo ungakwazi ukwenza iiselom zegazi ezibomvu ngokufanelekileyo.
Iibomvu zegazi ezibomvu ziyabhujiswa.
Ngaba Kuninzi Uhlobo LwaseThalassemia?
Ewe, kukho iintlobo ezininzi ze-thalassemia, kuquka:
- Beta thalassemia phakathi
- I-beta enkulu ye-thalassemia (ukuxhomekeka kwegazi)
- Isifo seHemoglobin H (ifom ye-alpha thalassemia)
- I-Hemoglobin H-rhoqo i-Spring (uhlobo lwe-alpha thalassemia, ngokubanzi kunzima kune-hemoglobin H)
- I-Hemoglobin E-beta thalassemia
Ziziphi iimpawu zeThalassemia?
Iimpawu ze-thalassemia zixhomekeke kakhulu kwi-anemia. Ezinye iimpawu zihambelana ne-hemolysis kunye nokuguquka komongo wethambo.
- Ukukhathala okanye ukhathala
- Isikhumba esinamabala
- Ukuqhwala kwesikhumba (i-jaundice) okanye amehlo (i-icterus ye-sclerus) - i-bilirubin (i-pigment) ikhishwa kwiiseli ezibomvu zegazi xa zichithwa
- I-spleen (splenomegaly) ephakanyisiweyo - xa umongo weethambo ungenako ukwenza amangqamuzana obomvu egazi, isebenzisa ipeni ukwenza ezinye iiseli zegazi ezibomvu
- I-Thalassemic facies - ngenxa ye-hemolysis kwi-thalassemia, umongo wethambo (indawo apho kusetyenziswa khona iiseli zegazi) iya kwi-overdrive. Oku kubangela ukukhulisa emathanjeni ebusweni (amaninzi ebunzini nasemathambo amathambo). Le ngxaki inokuthintela unyango olufanelekileyo.
I-Thalassemia ichongwa njani?
EUnited States ngokuqhelekileyo izigulane ezichaphazelekayo zixhaphazwa ngokuqhelekileyo ngeprogram yokuzalwa yokuzalwa . Izigulane ezichaphazelekayo zingadluliselwa kwisithuba esilandelayo xa i-anemia ibonakaliswa kwibala elipheleleyo legazi (CBC). I-Thalassemia ibangela i-anemia (i-hemoglobin ephantsi) kunye ne-microcytosis ( umthamo we-corpuscular volume ).
Uvavanyo oluqinisekisiweyo lubizwa ngokuba yi-hemoglobinopathy work-up okanye i-hemoglobin electrophoresis. Olu vavanyo luchaza iintlobo ze-hemoglobin onayo. Kwimntu omdala ongenaye i-thalassemia, kufuneka ubone kuphela i-hemoglobin A ne-A2 (abadala). Kwi-beta thalassemia intermedia kunye nenkulu, unokuphakama okukhulu kwi-hemoglobin F (fetal), ukuphakama kwe-hemoglobin A2 kunye nokunciphisa okuninzi kwisixa se-hemoglobin A eyenziwe. Isifo se-alpha thalassemia siboniswa ngobuninzi be-hemoglobin H (intlangano ye-4 ye-beta eetyuneni endaweni ye-2 alpha kunye ne-2 beta). Ukuba uvavanyo ludideka, ukuhlolwa kofuzo kungathunyelwa ukuqinisekisa ukuxilongwa.
Ngubani Osemngciphekweni weThalassemia?
I-Thalassemia iyimeko ezuze ilifa. Ukuba ngaba bobabini abazali banomzila we- alpha thalassemia okanye uhlobo lwe-beta thalassemia, banalo ithuba elilodwa lokuba nomntwana onesifo se-thalassemia. Umntu unesibeleko se-thalassemia okanye isifo se-thalassemia-oku akunako ukutshintsha. Ukuba unomthamo we-thalassemia, kufuneka ucinge ukuba iqabane lakho livavanywe ngaphambi kokuba nabantwana ukuhlola umngcipheko wokuba nomntwana nge-thalassemia.
Thalassemia ithathwa njani?
Izinyango zokwelapha zisekelwe kwinqanaba le-anemia:
- Ukuqwalasela okukufutshane: Ukuba i-anemia i-mildem-moderation kwaye ibekezeleke-ugqirha wakho unokukubona rhoqo ukuze ahlole ukubala kwakho kwegazi.
- Amachiza: Amachiza afana ne-hydroxyurea, okwandisa umveliso wakho we-hemoglobin fetal, asetyenziswe kwi-thalassemia eneempembelelo ezahlukeneyo.
- Ukumpontshelwa ngegazi: Ukuba i-anemia yakho yomelele kwaye ibangela iingxaki (umzekelo, i-splenomegaly ephawulekayo, i-thalassemic facies), unokufakwa kwiprogram engapheliyo yokumpontshelwa igazi. Uya kufumana ukumpontshelwa kwegazi nganye kwiiveki ezintathu ukuya kwezine ngomgudu wokuba ithambo lakho lomnatha lenze amangqamuzana egazi abomvu ngokusemandleni.
- Umnkantso wamathambo (okanye i-stem cell) yokufakelwa kwe-transplantation: Ukutshintshwa kwezityalo kunokunyanga. Iziphumo eziphambili zihambisana nabaxhasi bobabini abazalwana. Umntakwabo ogcweleyo unokufumana ithuba elilodwa lokuhambelana nomnye umntakwabo.
- I-Iron Chelation: Izigulane ezifumana igazi elingapheliyo zifumana insimbi eninzi kwigazi (insimbi ifumaneka ngaphakathi kweeseli zegazi ezibomvu). Ukongezelela, abantu abane-thalassemia banomlinganiselo omkhulu wegazi ekudleni kwabo. Le meko ebizwa ngokuba yintsimbi ephezulu okanye i-hemochromatosis yenza ukuba insimbi ifakwe kwezinye iifomthi kwisibindi, intliziyo, kunye ne-pancreas, kubangela ukulimala kwezi zitho. Oku kungaphathwa ngamachiza abizwa ngokuba yi-iron chelators anceda umzimba wakho ukulahla insimbi engaphezulu.