I naupang tê chu a chau reng niin a lang em? Chaw ei tam a duh lo em ni? Naupang dang aiin a paler deuh tih a hre tawh em? Chûngte chu thil pangngai angin lang mah se, a châng chuan he thil hnung lamah hian kan hriat tam loh thil awm lo tak a awm thei a ni. Vawiin hian chutiang dinhmun hmuh tur awm lo pakhat chu kan sawi dawn a, mahse uluk taka enkawl a nih chuan hlawhtling takin i nung thei ang. Chu chu Diamond-Blackfan Anemia, a nih loh leh `(Diamond-Blackfan Anemia - DBA)` a ni.
A awlsam zawngin, Diamond-Blackfan Anemia (DBA) hi eng nge ni?
Kan taksa chhungah thisen siamna factory lian tak kan nei ang tih han ngaihtuah teh. Bone marrow kan ti a . He factory hna ber chu thisen zam (red blood cells) siam a ni a, chu chu kan taksa pum puia oxygen phurtu hnathawk tlemte te hi a ni. DBA hi he factory bone marrow an tih hian thisen sen (red blood cells) a siam tling thei lo tihna a ni. Hei hi a tlem hle a, chu chu naupang 500,000 zinga pakhat vel ah he natna hi a awm tihna a ni.
Hei hi genetic disorder a ni a . Chumi awmzia chu kan taksa cell-a genes inthlak danglam vang a ni tihna a ni. Hei hian taksaa thisen sen (red blood cells) a ti tlem phah a ni. Hetiang dinhmun hi anemia , emaw thisen tlakchhamna emaw, thisen tlakchhamna emaw kan ti a. DBA hi dam chhung zawnga awm tur a ni. Mahse lungngai suh, enkawlna tha tak nen chuan mi tam tak chuan an natna lan chhuah dan an thunun thei a, nun tha tak an hmang thei bawk .
DBA vei hian eng symptoms nge a neih?
DBA natna lan chhuah dan hi mi hrang hrangah a inang lo thei. Mi thenkhat chuan symptoms na lo tak tak an nei thei a, thenkhat erawh chuan symptoms na zawk an nei thei thung. A lan chhuah dan bulpui te han thlir ila.
| Symptom a ni | A sawifiahna awlsam tak |
|---|---|
| Chauh fo thin | Taksa cell-te hian oxygen mamawh zat an dawn loh avangin mutthilh i nei fo va, engmah ti thei tur chakna i nei lo. |
| Vun rawng dum (Pallor) . | Thisen rawng sen tur siamtu thisen sen (red blood cells) a tlakchham avangin vun, hmui leh nail te chu a sen hring angin a lang. |
| Lung natna chak tak (Tachycardia) . | Thisen pump chak zawk tur leh taksa mamawh oxygen pe turin thinlung hian nasa zawkin hna a thawh a ngai a ni. |
| Thâwk harsa | A châng chuan, hla vak lo kea kal emaw, stairs-a ka chhoh emaw ang chi hian thawk harsa ka ti a, ka rilru a buai ang maiin ka inhria. |
| Chaw chakna | A bik takin naupang tê tê, ei leh in lama tui vak lote. |
| Lu na leh chak lohna | Taksa leh thluaka oxygen a tlakchham vangin a awm fo thin. |
Engvangin nge hetiang natna hi a lo awm? Eng nge a chhan?
Kan sawi tawh ang khan hei hi genetic disease a ni. Kan taksaah hian thisen zam (red blood cells) siam tura min zirtirtu gene bik a awm a. Chungte chu `(ribosomal protein genes)` an ti a. DBA vei chuan heng genes-ah hian variation emaw mutation emaw a nei a. Chuvang chuan thisen zam (red blood cells) siam dan hi a thleng tha lo hle a ni.
Pawimawh tak chu nu leh pa atanga fate hnena hlan chhawn theih a ni. Mahse hei hi za zela tlemte chauh a thleng a, 10% - 25% vel a ni. Thil tam takah chuan naupang chuan chhungkaw zingah tumahin he natna hi an nei lo a nih pawhin he natna hi a nei thei a, hei hi random genetic mutation vang a ni.
DBA vang hian eng harsatna dang nge awm thei?
DBA hian thisen sen chauh a nghawng lo. A chang chuan hriselna lama harsatna dang pawh a thlen thei bawk. Tin, DBA vei te hian mipui zawng zawng aiin cancer chi thenkhat an vei theihna chance a sang zawk deuh bawk.
| Complication chi hrang hrang | Hrilhfiahna |
|---|---|
| Harsatna awm fo thin | |
| Nau piangsualna | Thinlung, kal, a nih loh leh taksa peng hrang hrangah harsatna engemaw zat a awm thei. |
| Growth a tlahniam zel | Taksa thanlenna hi kum upa lam aiin beisei aia hniam zawk a ni. |
| Thir a tam lutuk | Thisen pek fo hian taksaa iron level chu a tul lovah a tisang thei a, taksa peng hrang hrang, thinlung leh thin te a tichhe thei a ni. |
| Thisen cell dang a tlahniam | A châng chuan thisen cell dang, thisen var (neutropenia) emaw, platelet (thrombocytopenia) emaw pawh a hniam thei bawk. |
| Cancer hlauhawmna (a sang deuh) . | |
| Leukemia natna (Leukemia) a ni | A bik takin thisen cancer chi khat ``Acute myeloid leukemia - AML`` an tih hi a ni. |
| Myelodysplastic syndrome (MDS) natna hi a awm lo. | Thisen natna dang, ruhro hnathawh tichhe thei. |
| Osteosarcoma natna a ni | Cancer natna, ruh chhunga lo awm. |
Engtin nge doctor-te hian he natna hi dik taka an hriat theih?
I fa hian heng symptoms te hi a neih chuan i doctor chuan i fa chu uluk takin a enfiah hmasa ang a, a chipchiar zawngin a zawt ang che. Tichuan, an diagnosis tih finfiah nan test thenkhat an ti mai thei bawk.
- Complete Blood Count (CBC): Hei hian thisen chhunga thisen sen, thisen var, leh platelet awm zat a tehna a ni. DBA vei chuan thisen sen (red blood cell) a hniam hle.
- Reticulocyte count: Reticulocyte hi thisen sen thar lo piang chhuak, puitling kim lo a ni. Number tlem chuan ruhro hian thisen sen thar a siam chhuak tawk lo tihna a ni.
- Bone marrow aspiration leh biopsy: Hei hi natna hriat chian nan test hman ber a ni. Hetah hian ruhro sample tlemte tak tak lak a ni a, a tlangpuiin hip ruh atanga lak a ni a, anesthesia hmangin lak a ni. Chumi hnuah microscope hmangin an enfiah a, thisen sen (red blood cell) siam chhuah zat dik tak chu an hre thei a ni.
DBA enkawl dan tur chu engte nge ni?
DBA hi tihdam kim theih ni lo mah se, natna lan chhuah dan control thei tur leh nunphung tha tak vawng reng thei tur enkawlna tha tak tak a awm.
1. Corticosteroids: Heng damdawi te hian ruhro (bone marrow) a tichak a, thisen sen (red blood cell) tam zawk a siam chhuak thin. He enkawlna hi damlo tam tak tan chuan a hlawhtling hle.
2. Thisen pek: Thisen hniam tak takte chu thisen petu hrisel tak hnen atangin thisen sen (red blood cells) pek an ni. Doctor ten natna an hriat chian veleh he enkawlna hi an tan nghal thei a ni. Hei hian symptoms atanga relief rang tak a pe a ni.
3. Stem cell transplant: Hei hi DBA tidam kim thei damdawi awmchhun a ni. Mahse, hei hi enkawlna buaithlak tak leh risky tak a ni. Hetah hian mi hrisel (a tlangpuiin chhungte) ruhro atanga stem cells te chu damlo chhungah an dah lut thin. Hei hi mi zawng zawng tan thil theih a ni lo. Doctor chuan damlo dinhmun chu uluk takin a zirchiang a, a inmil ber tur a zawng a, ngaihtuahna tam tak a neih hnuah he thutlukna hi a siam thin.
Emergency-ah eng nge tih tur?
Nang emaw i fa emaw hian DBA i nei a nih chuan i doctor nena inbiak reng a pawimawh hle . Appointment neih a pawimawh a, damdawi ei tur pawh a tih angin ei a pawimawh. I natna lan chhuah dan a na zual a nih chuan (entir nan, a tlangpui aia i chau zawk a, natna i vei tam zawk a nih chuan) doctor hnenah hrilh nghal rawh.
Tin, a hnuaia emergency symptoms te hi i neih chuan damdawi in hnai ber Emergency Department (ETU) ah kal nghal rawh .
- A rilru a na hle
- Vawikhatmah pum na na tak
- Lu na tak tak, buaina, emaw tawng harsa (hengte hi stroke chhinchhiahna a ni thei) .
- Thunun theih loh thisen chhuak
DBA ang chi dam chhung leh natna hlauhawm tak nena nun chu, a bik takin i fa a nih chuan a harsa hle thei. Mahni chauhvin a awm thei. Mahse, nangmah chauh i ni lo tih hre reng ang che. I doctor leh medical team te chuan i mamawh tanpuina leh kaihhruaina an pe ang che.
Take-Home Thuchah a ni
- Diamond-Blackfan anemia (DBA) hi genetic natna hmuh tur awm lo tak a ni a, ruhro hian thisen sen (red blood cell) a siam tling thei lo.
- Chaw hah fo, vun rawng hring leh thawk hah lutuk te hi a lan chhuah dan ber a ni.
- Hei hi dam chhung zawnga natna awm ni mahse, steroid damdawi leh thisen pek ang chi enkawlna hmangin hlawhtling takin enkawl theih a ni.
- Doctor nena inbiak reng a, test leh enkawlna \ha tak tak dawng a pawimawh hle.
- Emergency warning signs (ril na na tak, hriatna hloh ang chi) te chu fimkhur la, chutiang a nih chuan damdawi in Emergency Treatment Unit (ETU) pan nghal rawh.
- Hei hi natna hmuh tur awm lo tak a nih avangin inhlat takin i inhria a nih pawhin, nangmah thlawptu tur medical team leh support network an awm tih hre reng ang che.
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