Mhlawumbi ukhe weva nge-"anemia" okanye "anemia" akunjalo? Ngamafutshane, kuxa imizimba yethu ingavelisi iiseli ezibomvu zegazi ezaneleyo okanye iiseli ezibomvu zegazi esinazo zingasebenzi kakuhle. Iiseli ezibomvu zegazi zezona zithwala ioksijini emizimbeni yethu yonke. Ngoko ke xa ziphantsi, sinokuziva sidiniwe kwaye sidiniwe ngalo lonke ixesha. Ezi seli ezibomvu zegazi ziveliswa kumongo wethambo, izicubu ezithambileyo, ezithambileyo ngaphakathi kwamathambo ethu. I-Diamond-Blackfan Anemia (DBA) luhlobo olukhethekileyo lwe-anemia oluvela xa umongo wethambo ungakwazi ukuvelisa iiseli ezibomvu zegazi ezaneleyo.
Yintoni ebangela iDiamond-Blackfan Anemia (DBA)?
I-Diamond-Blackfan Anemia, ngamanye amaxesha ebizwa ngokuba yi-"acquired pure red cell aplasia", idla ngokuchongwa ngoogqirha ngaphambi kokuba umntwana abe nonyaka omnye ubudala. Isizathu esiphambili soku kukutshintsha, okanye utshintsho, kwiijini ezizizinto ezakha imizimba yethu.
Khumbula, ngamanye amaxesha esi siphene semfuza sinokufunyanwa kumama okanye kutata. Kodwa oko akusoloko kunjalo. Ezinye ii-genes zabantwana nazo zinokutshintsha ngokuzenzekelayo ngaphandle kwesizathu. Uninzi lwabantwana abane-DBA banale meko ngenxa yotshintsho olutsha lwemfuza. Ke ngoko, njengabazali, akukho mfuneko yokukhathazeka ngokungeyomfuneko ngale nto. Nangona unobangela othile wemfuza ufunyenwe phantse kwisiqingatha sezigulane ze-DBA, kwabanye abantu, unobangela othile wale meko awukafunyanwa.
Zithini iimpawu ze-DBA?
Abantwana abane-DBA banokufumana iimpawu ezininzi eziqhelekileyo zezinye iintlobo ze-anemia. Oko kukuthi,
- Ukudinwa rhoqo
- Ulusu olukhanyayo
- Ukuziva ubuthathaka
Ukongeza kwezi mpawu ziqhelekileyo, abanye abantwana abazalwa bene-DBA banokuba neempawu ezithile zangaphandle ebusweni nasemizimbeni yabo. Makhe sijonge ukuba ziintoni.
| Ilungu lomzimba | Iimpawu ezibonakalayo |
|---|---|
| Intloko | Ukuba nentloko encinci kuneqhelekileyo. |
| Ubuso | Umgama okhulayo phakathi kwamehlo nempumlo ethe tyaba. |
| Iindlebe | Iindlebe ezimbini ezincinci ezibekwe ngaphantsi kunesiqhelo. |
| Umhlathi | Ukuba nomhlathi omncinci ongezantsi. |
| Intamo | Intamo emfutshane, enentambo. |
| Amagxa | Iibhleyidi ezincinci zamagxa. |
| Izandla | Izithupha ezimile ngendlela engaqhelekanga. |
| Umlomo | Ukuba nencakuba okanye umlebe oqhekekileyo. |
Ukongeza, i-DBA inokubangela iingxaki zezintso , iziphene zentliziyo , kunye neengxaki zamehlo ezifana ne -cataracts kunye ne -glaucoma . Amakhwenkwe anokuba nesiphene sokuzalwa esibizwa ngokuba yi-hypospadias, apho umngxuma womchamo ungekho kwincam yepenis.
Indlela yokubona ngokuchanekileyo esi sifo?
Ugqirha wakho angenza iimvavanyo ezininzi ukuze abone ukuba umntwana wakho une-DBA. Abantwana abane-DBA baneeseli ezibomvu zegazi ezisezantsi, kodwa iiseli ezimhlophe zegazi kunye neeplatelet ziqhelekile.
Eyona nto ibalulekileyo kukungabi naxhala xa ubona ezi mpawu, kodwa kukubona ugqirha ofanelekileyo ngokukhawuleza. Uza kwenza uvavanyo olufunekayo aze akunike ulwazi oluchanekileyo.
Ezi zezinye zezona zilingo ziphambili ezenziwa ngugqirha.
| Uvavanyo | Ijonge ntoni? |
|---|---|
| Inani elipheleleyo legazi (i-CBC) | Izinto ezininzi zilinganiswa egazini, njengenani leeseli ezibomvu zegazi, iiseli ezimhlophe zegazi, iiplatelets, iproteni ethwala ioksijini i-hemoglobin, kunye nomthamo weeseli ezibomvu zegazi (i-hematocrit) . |
| Inani lee-reticulocyte | Oku kulinganisa inani leeseli ezibomvu zegazi ezingavuthwanga, okanye ezisandula ukwakheka. Oku kunokubonisa ukuba umongo wethambo uvelisa iiseli ezibomvu ngokufanelekileyo na. |
| Ukujonga inqanaba le-eADA | Malunga nama-80% abantu abane-DBA banezinga eliphezulu le-enzyme ebizwa ngokuba yi-erythrocyte adenosine deaminase (eADA) . Le yindlela olu vavanyo olujonge ngayo. |
| Amanqanaba e-hemoglobin yosana olusandul' ukuzalwa | Olu luhlobo lwe-hemoglobin olunazo iintsana esibelekweni. Lufanele lunciphe emva kokuzalwa. Nangona kunjalo, abantwana abane-DBA basenokuba namanqanaba aphezulu njengoko bekhula. |
| Ukufunxwa komnkantsha wethambo kunye ne-biopsy | Kuthathwa inani elincinci leeseli kunye nolwelo kumongo wethambo kusetyenziswa inaliti kwaye kuvavanywa. Abantwana abane-DBA baneeseli ezibomvu zegazi ezimbalwa kakhulu ezisempilweni kumongo wabo wethambo. |
| Uvavanyo lwe-genetic | Olu vavanyo lwezakhi zofuzo ezinxulumene ne-DBA okanye ezinye izifo ze-anemia ezizuzwe ngabazali. |
Ziziphi ezinye iingxaki ezinokwenzeka ngenxa ye-DBA?
Abantu abane-DBA basengozini enkulu kancinci yokuhlaselwa zizifo ezithile kunye neemeko ezithile. Ezi ziquka umhlaza wegazi kunye nomongo wamathambo (i-acute myeloid leukemia), umhlaza wamathambo (i-osteosarcoma), kunye nezinye izifo apho umongo wamathambo ungenakwenza iiseli zegazi eziphilileyo (i-myelodysplastic syndrome). Kodwa ungakhathazeki ngale nto. Oogqirha bahlala bejonga oku, ngoko ke ungathatha amanyathelo afanelekileyo ezonyango ngexesha elifanelekileyo.
Zithini iindlela zonyango zoku?
Le yeyona nxalenye ibalulekileyo nethuthuzelayo. Abantwana abafunyaniswe bene-DBA banokuphila ubomi obude nobunempumelelo ngonyango lwezonyango. Abanye abantwana baya kuphila ubomi obupheleleyo, nto leyo ethetha ukuba iimpawu ziyanyamalala kangangexesha elithile.
Iindlela ezimbini eziphambili zonyango ezisetyenziswayo ngamayeza e-corticosteroid kunye notofelo-gazi.
- Amayeza e-corticosteroid: Amayeza afana ne-`(Prednisone)` avuselela umongo wethambo aze awuncede ukuba wenze iiseli ezibomvu zegazi ezingaphezulu.
- Utofelo-gazi: Olu lukhetho olufanelekileyo ukuba amayeza e-steroid awasebenzi, okanye ukuba i-anemia inzima. Oku kuquka ukunika umntwana igazi okanye iiseli ezibomvu zegazi ezivela kumntu ophilileyo.
- Ukufakelwa kweseli ye-bone marrow/stem: Olu lolona nyango lonyango kuphela lwe-DBA. Nangona kunjalo, luyingozi kancinci. Kwakhona, ukufumana umntu oza kunikela naye ngamanye amaxesha kunokuba nzima.
Kubalulekile ukuxoxa ngazo zonke ezi ndlela zonyango, izinto ezilungileyo nezingalunganga, ngokucacileyo nogqirha wakho, uze ukhethe okona kufanelekileyo kumntwana wakho.
Umyalezo Wokuya Ekhaya
- I-Diamond-Blackfan Anemia (DBA) yimeko engaqhelekanga yemfuza ebantwaneni apho umongo wethambo ungakwazi ukuvelisa iiseli ezibomvu zegazi ezaneleyo.
- Kunye neempawu eziqhelekileyo ezifana nokudinwa rhoqo kunye nokuphaphatheka, esi sifo sinokubangela ezinye iimpawu zangaphandle ebusweni nasemzimbeni.
- Ugqirha angasixilonga ngokuchanekileyo esi sifo ngovavanyo lwegazi kunye novavanyo lomongo wethambo.
- Unyango olufana namayeza e-steroid kunye notofelo-gazi lunokunceda abantwana baphile ubomi obude nobusempilweni. Ukufakelwa komongo wethambo lolona nyango luphela olunyangayo.
- Ukuba unesikrokro nokuba ncinci kangakanani na sokuba umntwana wakho unale mpawu, musa ukoyika kwaye ubone ugqirha wabantwana ngoko nangoko. Ukuxilongwa ngokukhawuleza kunye nonyango kubaluleke kakhulu.
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