Diamond-Blackfan Anemia - He thisen natna hmuh tur awm lo tak hi a awlsam zawngin i zir ang u

I fa chu a chau reng a, a sen hle tih i hre ngai em? A châng chuan naupang tê tête chu chutiang chiah chu a ni tih kan ngaihtuah ṭhin a, mahse a hnung lamah hian kan hriat loh chhan a awm mai thei. Vawiin hian chutiang thisen dinhmun hmuh tur awm lo, mahse hriat a pawimawh em em chu kan sawi dawn a ni. Hei hi Diamond-Blackfan Anemia (DBA) an ti a.

A awlsam zawngin, Diamond-Blackfan Anemia (DBA) hi eng nge ni?

Hei hi thisen natna hmuh tur awm lo tak a ni. A awlsam zawngin, thil thleng chu kan ruhro hian thisen sen (red blood cells) a siam chhuak thei lo tihna a ni. I hria em, heng thisen sen (red blood cells) te hi kan taksa pum puia oxygen phurtu an ni. Chuvangin an tlin loh chuan taksa hnathawh danah nghawng lian tak a nei a ni.

DBA hi genetic disorder a ni a . Chumi awmzia chu he natna hi genes inthlak danglamna avanga lo awm a ni tihna a ni. Heng genetic changes te hian symptoms nasat zia a hril a ni. Khawvel puma naute 500,000 zinga pakhat velin a nghawng a ni. Nun chhung zawnga awm thei ni mah se, enkawlna dik tak a awm chuan a natna lan chhuah dan chu thunun theih a ni a, nun ṭha tak a awm thei bawk .

He natna lan chhuah dan engte nge ni?

DBA vei zawng zawng deuhthaw hian thisen tlakchhamna (anemia) an nei vek. Chu chu thisen tlakchham tihna a ni. Mi thenkhat tan chuan a natna lan chhuah dan hi a na lo thei hle a, thenkhat tan chuan a na hle thei bawk. Simptom langsar tak tak hmuh theih te hi han thlir ila.

Engvangin nge hetiang hi a lo awm?

Kan taksaa thil awm zawng zawng, kan genes tia koh, thupek petu lehkhabu a awm ang tih han ngaihtuah teh. Thisen sen siam dan tur thupek pe thei gene chi khat a awm a, chu chu ``ribosomal protein genes.`` tih a ni.`` DBA hi chu genes-a danglamna a awm chuan a lo awm thin. Tichuan thisen zam (red blood cells) siam dan hi a thleng dik lo.

DBA vei zinga 10% leh 25% inkar chuan an nu leh pate hnen atangin an rochun thin. Mahse, a tam zâwk chuan, chhûngkaw zînga tumahin an la neih ngai loh natna lo awm thar a ni .

Hei hian harsatna dang a thlen thei em?

Ni e, DBA hian hriselna lama harsatna dang engemaw zat a thlen thei a ni.

Doctor-te hian engtin nge hei hi an hmuh?

I doctor-in he natna hi a rinhlelh chuan test eng emaw zat an kalpui ang a, a finfiah ang.

• Complete Blood Count (CBC): Hei hian thisen chhunga thisen sen, thisen var, platelet leh hemoglobin (hemoglobin) awm zat a enfiah thin. DBA-ah chuan thisen sen leh hemoglobin level a hniam hle.
• Reticulocyte Count: Reticulocyte hi thisen sen thar, puitling lo tak tak a ni. Chung zinga a tlem chuan ruhro (bone marrow)-in thisen sen (red blood cells) a siamnaah harsatna a awm tihna a ni.
• Bone Marrow Aspiration leh Biopsy: Hei hian ruhro sample te tak te lak a, microscope hmanga enfiah a ni. Hei hian thisen sen siam chhuah tlahniam a awm leh awm loh hriat theih nan a pui thei a ni.

Enkawlnate chu engte nge ni?

DBA hi enkawlna kawng hrang hrang a awm a. Damlo dinhmun a zirin doctor chuan a enkawl dan tur dik ber a ruat ang.

1. Corticosteroids: Hengte hi steroid chi hrang hrang damdawi a ni. Heng damdawi te hian ruhro (bone marrow) a tichak a, thisen sen (red blood cells) siamchhuahna a tipung bawk. Results hi kar hnih atanga kar li chhungin hmuh tur a awm tlangpui.

2. Thisen pek: A awlsam zawngin thisen pek. Damlo hnena thisen petute hnen atanga thisen sen pek hian rang takin taksaa thisen sen zat a siam tha thei a ni. Hei hi enkawlna a ni a, a tlangpuiin natna hmuhchhuah a nih veleh tan a ni.

3. Stem Cell Transplant: Hei hi he natna hi tihdam kim theihna awmchhun a ni . Mahse, hei hi a buaithlak deuh a, a hlauhawm hle a, nunna atana hlauhawm tak a ni. Chuvangin doctor-te chuan damlo tinte chu mimal takin an ngaihtuah a, a tul tak tak a nih chuan he enkawlna hi an hmang chauh thin.

Nakinah eng nge thleng dawn? Dam rei zawng hi engtin nge ni ang?

Enkawlna mumal leh damdawi lam enkawlna mumal tak an neih chuan DBA vei te hian nun tha tak an hmang thei a ni. Mahse, hei hi dam chhung enkawl ngai dinhmun a ni. Kum 25 an tlin hnuah chuan harsatna lian tham a thlen theihna a sang deuh thei a ni.

Dam rei zawng chungchang erawh chu mi hrang hrangah a inang lo. A natna lan chhuah dan nasat dan leh enkawlna dawn danah a innghat. Zirna hrang hrangah chuan enkawl a nih chuan damlo 75% vel chu kum 50 thleng an dam tih a tarlang . Mahse, hei hi natna hmuh tur awm lo tak a nih avangin data a tlem hle. I doctor chauh hian i dinhmun chungchangah thu dik ber a pe thei che a ni.

Engtikah nge Doctor ka hmuh ang?

Nang emaw i fa emaw hian DBA i nei a nih chuan i doctor nena inbiak reng a pawimawh hle. Clinic appointment ruat tawhte chu tlawh loh ngai suh.

Tin, i natna lan chhuahna a zual zel nia i hriat chuan, entirnan, i chau zual thut emaw, natna i vei fo emaw chuan , i doctor hnenah hrilh nghal rawh.

Emergency-ah eng nge tih tur?

A hnuaia chhinchhiahna te hi a lo awm a nih chuan damdawi in hnai ber Emergency Department (ETU) pan nghal tur a ni.

• A rilru a na
• Pum natna nasa tak
• Lu na tak tak emaw, hriatna hloh emaw (stroke chhinchhiahna a ni thei) .
• Thunun theih loh thisen chhuak

Mi tam takin an hriat ngai loh DBA ang damchhung dinhmun nen nun hi thil awlsam a ni lo. A bîk takin i fa a nghawng hunah chuan a buaithlâk thei hle. Mahni chauhvin i inhria mai thei. Mahse, nangmah chauh i ni lo tih hre reng ang che. I doctor leh medical team te chuan i mamawh tanpuina, thurawn leh enkawlna ruahmanna an pe ang che.