Cishe uke wezwa nge-"anemia" noma "anemia" akunjalo? Kalula nje, yilapho imizimba yethu ingakhiqizi amangqamuzana abomvu egazi anele noma amangqamuzana abomvu egazi esinawo engasebenzi kahle. Amangqamuzana abomvu egazi ayizithwali eziyinhloko zomoya-mpilo emizimbeni yethu yonke. Ngakho-ke uma ephansi, singazizwa sikhathele futhi sikhathele ngaso sonke isikhathi. Lawa maseli abomvu egazi akhiqizwa emnkantsheni wamathambo, okuyizicubu ezithambile, ezithambile ngaphakathi kwamathambo ethu. I-Diamond-Blackfan Anemia (DBA) uhlobo olukhethekile lwe-anemia oluvela lapho umnkantshe wamathambo ungakwazi ukukhiqiza amangqamuzana abomvu egazi anele.
Yini ebangela i-Diamond-Blackfan Anemia (DBA)?
I-Diamond-Blackfan Anemia, ngezinye izikhathi ebizwa ngokuthi "i-acquired pure red cell aplasia", ivame ukuhlonzwa odokotela ngaphambi kokuba ingane ibe nonyaka owodwa ubudala. Imbangela eyinhloko yalokhu izinguquko, noma izinguquko, ezakhiweni zofuzo eziyizakhi zomzimba wethu.
Khumbula, ngezinye izikhathi lesi sici sofuzo singazuzwa kumama noma kuyise. Kodwa lokho akunjalo ngaso sonke isikhathi. Ezinye izakhi zofuzo zezingane nazo zingashintsha ngokuzenzekelayo ngaphandle kwesizathu. Iningi lezingane ezine-DBA linalesi simo ngenxa yokuguquka kwezakhi zofuzo okusha. Ngakho-ke, njengabazali, asikho isidingo sokukhathazeka ngokungadingekile ngalokhu. Nakuba imbangela ethile yezakhi zofuzo itholakale cishe kwengxenye yeziguli ezine-DBA, kwabanye abantu, imbangela ethile yalesi simo ayikatholakali.
Ziyini izimpawu ze-DBA?
Izingane ezine-DBA zingase zithole izimpawu eziningi ezivamile zezinye izinhlobo ze-anemia. Okusho ukuthi,
- Ukukhathala okuqhubekayo
- Isikhumba esikhanyayo
- Ukuzizwa ubuthakathaka
Ngaphezu kwalezi zici ezivamile, ezinye izingane ezizalwa zine-DBA zingase zibe nezici ezithile zangaphandle ebusweni nasemizimbeni yazo. Ake sibheke ukuthi ziyini.
| Ingxenye yomzimba | Izici ezibonakalayo |
|---|---|
| Ikhanda | Ukuba nekhanda elincane kunelivamile. |
| Ubuso | Ibanga elikhudlwana phakathi kwamehlo nekhala eliyisicaba. |
| Izindlebe | Izindlebe ezimbili ezincane ezibekwe phansi kunokuvamile. |
| Umhlathi | Ukuba nomhlathi omncane ongezansi. |
| Intamo | Intamo emfushane, enezintambo. |
| Amahlombe | Amahlombe amancane. |
| Izandla | Izithupha ezimile ngendlela engavamile. |
| Umlomo | Ukuba nomlomo oqhekekile noma oqhekekile. |
Ngaphezu kwalokho, i-DBA ingabangela izinkinga zezinso , ukukhubazeka kwenhliziyo , nezinkinga zamehlo njenge -cataracts kanye ne -glaucoma . Abafana nabo bangaba nokukhubazeka kokuzalwa okubizwa ngokuthi i-hypospadias, okuyilapho imbobo yomchamo ingekho esicongweni sepenis.
Ungasibona kanjani lesi sifo ngokunembile?
Udokotela wakho angenza izivivinyo eziningana ukuze anqume ukuthi ingane yakho ine-DBA yini. Izingane ezine-DBA zinezibalo eziphansi zamaseli abomvu egazi, kodwa amaseli amhlophe egazi kanye nama-platelet ajwayelekile.
Into ebaluleke kakhulu ukungathuki uma ubona lezi zimpawu, kodwa ukubona udokotela ofanelekayo ngokushesha okukhulu. Uzokwenza izivivinyo ezidingekayo futhi akunikeze ulwazi olunembile kakhulu.
Lezi ezinye zezivivinyo eziyinhloko ezenziwa udokotela.
| Ukuhlolwa | Ifunani? |
|---|---|
| Inani Eliphelele Legazi (i-CBC) | Izinto eziningi zilinganiswa egazini, njengenani lamaseli abomvu egazi, amaseli amhlophe egazi, ama-platelet, i- hemoglobin yeprotheni ethwala umoya-mpilo, kanye nomthamo wamaseli abomvu egazi (i-hematocrit) . |
| Inani lama-reticulocyte | Lokhu kulinganisa inani lamaseli abomvu egazi angakavuthwa, noma asanda kwakheka. Lokhu kungabonisa ukuthi umnkantsha ukhiqiza amaseli abomvu ngendlela efanele yini. |
| Ukuhlola izinga le-eADA | Cishe abantu abangu-80% abane-DBA banamazinga aphezulu e-enzyme ebizwa ngokuthi i-erythrocyte adenosine deaminase (eADA) . Yilokhu okufunwa yilolu vivinyo. |
| Amazinga e-hemoglobin yengane esanda kuzalwa | Lolu uhlobo lwe-hemoglobin olunalo izingane esibelethweni. Kufanele lunciphe ngemva kokuzalwa. Kodwa-ke, izingane ezine-DBA zingase zibe namazinga aphezulu njengoba zikhula. |
| Ukufuthwa komnkantsha kanye ne-biopsy | Inani elincane lamaseli noketshezi kuthathwa emnkantsheni kusetshenziswa inaliti bese kuhlolwa. Izingane ezine-DBA zinamaseli abomvu egazi ambalwa kakhulu anempilo emnkantsheni wazo. |
| Ukuhlolwa kofuzo | Lokhu kuhlola izakhi zofuzo ezihlobene ne-DBA noma ezinye izifo ze-anemia ezizuzwe kubazali. |
Yiziphi ezinye izinkinga ezingaba khona ngenxa ye-DBA?
Abantu abane-DBA basengozini enkulu kancane yokuthola izifo nezimo ezithile. Lokhu kufaka phakathi umdlavuza wegazi kanye nomnkantsha wamathambo (i-acute myeloid leukemia), umdlavuza wamathambo (i-osteosarcoma), kanye nezinye izifo lapho umnkantsha wamathambo ungakwazi khona ukwenza amangqamuzana egazi anempilo (i-myelodysplastic syndrome). Kodwa ungakhathazeki ngalokhu. Odokotela bahlala beqapha lokhu, ngakho-ke ungathatha izinyathelo zezokwelapha ezidingekayo ngesikhathi esifanele.
Yiziphi izindlela zokwelapha lokhu?
Lena yingxenye ebaluleke kakhulu neduduzayo. Izingane ezitholakale zine-DBA zingaphila impilo ende nephumelelayo ngokwelashwa. Ezinye izingane ziyalulama ngokuphelele, okusho ukuthi izimpawu ziyanyamalala isikhathi esithile.
Izindlela ezimbili zokwelapha eziyinhloko ezisetshenziswayo imithi ye-corticosteroid kanye nokumpontshelwa igazi.
- Imithi ye-Corticosteroid: Imithi efana ne-`(Prednisone)` ivuselela umongo wamathambo futhi iyisize ukuthi yenze amaseli egazi abomvu engeziwe.
- Ukumpontshelwa igazi: Lena inketho enhle uma imithi yama-steroid ingasebenzi, noma uma i-anemia inzima kakhulu. Lokhu kuhilela ukunikeza ingane igazi noma amangqamuzana abomvu egazi avela kumnikeli onempilo.
- Ukufakelwa kweseli lomnkantsha/i-stem: Lena ukuphela kwendlela yokwelapha i-DBA. Kodwa-ke, kuyingozi kancane. Futhi, ukuthola umuntu onikelayo ofanelanayo kungaba nzima ngezinye izikhathi.
Kubalulekile ukuxoxa ngazo zonke lezi zindlela zokwelapha, izinzuzo nezingozi zazo, ngokucacile nodokotela wakho, bese ukhetha okulungele ingane yakho.
Umlayezo Wokuya Nawe Ekhaya
- I-Diamond-Blackfan Anemia (DBA) yisimo esingavamile sofuzo ezinganeni lapho umongo wethambo ungakwazi ukukhiqiza amangqamuzana abomvu egazi anele.
- Kanye nezimpawu ezivamile njengokukhathala okuvamile kanye nokuphaphatheka, lesi sifo singadala nezinye izici ezithile zangaphandle ebusweni nasemzimbeni.
- Udokotela angakwazi ukuxilonga lesi sifo ngokunembile ngokuhlolwa kwegazi kanye nokuhlolwa komnkantsha.
- Ukwelashwa okufana nemithi yama-steroid kanye nokumpontshelwa igazi kungasiza izingane ziphile impilo ende nenempilo. Ukufakelwa umnkantsha wethambo kuwukuphela kokwelashwa okwelaphayo.
- Uma usola ngisho nokuncane ukuthi ingane yakho inalezi zimpawu, ungesabi futhi ubone udokotela wezingane ngokushesha. Ukuxilongwa okusheshayo kanye nokwelashwa kubaluleke kakhulu.
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