I chhungte zingah "Sickle Cell Disease" nei an awm em? I chhui chiang ang u!

Vawiin hian mi tam zawk tan chuan thil thar deuh, mahse chhungkaw thenkhat nena inhnaih thei tak dinhmun kan sawi dawn a ni. Sickle Cell Disease (SCD) an ti a. He hming hi i lo hre tawh mai thei. A nihna takah chuan kan thisen nena inzawm, chu chu thisen nena inzawm, rochun atanga lo piang natna a ni. Eng nge a nih tak tak, engvangin nge a lo awm tih leh eng thil dang nge tih theih tih hi han thlir ila.

Sickle Cell Disease hi eng nge ni? A awlsam zawngin i hrethiam ang u!

A awlsam zawngin sawi ila, sickle cell disease (SCD) hi kan taksaa thisen sen (red blood cells) te, nu leh pa atanga fate rochun a ni. Hei hi khawvela thisen natna (hereditary blood diseases) awm tam ber zinga mi a ni. Tunah chuan en teh, kan thisen sen chhungah hian protein pakhat hemoglobin tih a awm a. He hemoglobin hi a pawimawh hle. A chhan chu, he protein hian kan taksa pum puiah oxygen a keng tel a ni. Oxygen taxi ang mai a ni. Mi hrisel thisen sen (red blood cells) hi a tlangpuiin a zungbun leh a inthlak danglam thei a ni. Rubber ball te te ang maiin. Chumi avang chuan taksa chhunga thisen kalna kawng te ber ber ( capillaries kan ti) te chu awlsam takin an tlan chhuak thei a, kan taksa peng leh tissue zawng zawngah oxygen an pe thei a ni. Mahse, sickle cell natna vei chunga thil thleng chu he hemoglobin-ah hian inthlak danglamna tlem a awm a ni. He hemoglobin pangngai lo hi hemoglobin S an ti a, hei hian thisen sen chu a zungpui leh a inthlak danglam thei lo va, thlasik ang maia siam, a nih loh leh thla chanve ang maia siam a ni. Chu mai ni lovin, heng cell te hi an khauh hle a, an ben awlsam lo va, an inzawm khawm bawk. Hêng sickle ang maia siam cell-te hian chu thisen kalna kawng tê tête chu an kal tlang chuan eng nge thleng ang tih han ngaihtuah teh? An tang tlat mai! Tichuan thisen kal chu a tibuai ta a ni. Chumi awmzia chu, kan taksa peng leh taksa peng pawimawh ber berte hian oxygen an dawng tlem hle tihna a ni. Hei vang hian harsatna lian tak tak , natna khirh tak, natna chi hrang hrang, taksa peng hrang hrang tihchhiat, leh hnathawh that lohna te a awm thei a ni. Thil dang pakhat chu heng sickle ang chi cell te hi thisen sen pangngai angin an dam rei lo tih hi a ni. An thi thuai thin. Tichuan taksaah hian thisen sen (red blood cells) a tlachham fo thin. Hei hi anemia kan tih chu a ni . Sickle cell natna hi dam chhung zawnga natna a ni. Mahse, lungngai suh, hei hi enkawl dan tur a awm. Heng enkawlna hmang hian a natna lan chhuah dan chu i ti tlem thei a, i dam chhung pawh i ti rei thei bawk.

Sickle cell natna chi hrang hrang a awm em?

Ni e, sickle cell natna chi hrang hrang a awm. Heng chi hrang hrangte hi mi pakhatin a nu leh pa hnen atanga a rochun genes atanga teh a ni.

Hemoglobin SS (HbSS) hmanga enkawl a ni.

Hei hi sickle cell natna chi khat na ber a ni. SCD vei zinga 65% velin he chi hi an nei a ni. Heng mite hian nu leh pa pahnih hnen atangin hemoglobin S gene hi an rochun a ni. Chumi awmzia chu an hemoglobin tam zawk emaw, a zawng zawng emaw chu a pangngai lo tihna a ni. Hei hian chronic anemia an nei thin.

Hemoglobin SC (HbSC) hmanga enkawl a ni.

Hei hi a na lo emaw, a hniam emaw a ni.Level sang taka awm type. SCD vei zinga 25% velin hetiang hi an nei a ni. Heng mite hian nu leh pa pakhat aṭangin hemoglobin S gene an rochun a, nu leh pa dang aṭangin hemoglobin chi dang, hemoglobin C an tih, gene chu an rochun bawk.

Hemoglobin (HbS) Beta Thalassemia atanga lo chhuak a ni

Heng mite hian nu leh pa pakhat aṭangin hemoglobin S gene an rochun a, nu leh pa dang aṭangin beta thalassemia an tih gene pangngai lo an rochun bawk. Hei hi subtype pahnih a awm bawk a:

• “Plus” (HbS beta +): Hei hi SCD vei zinga 8% velin an vei chi a ni a, a tlangpuiin a na lo hle.
• “Zero” (HbS beta 0): Hei hi SCD vei zinga 2% velin a nghawng chi a ni a, hemoglobin SS (HbSS) natna ang maia na thei a ni.

A dang hmuh tur awm lo chi hrang hrang

Hei bakah hian chi dang hmuh tur awm lo tak tak, hemoglobin SD (HbSD), hemoglobin SE (HbSE), leh hemoglobin SO (HbSO) te pawh a awm bawk. Heng mite hian hemoglobin S gene pakhat leh gene danglam dang D, E emaw O emaw an rochun a ni.

Sickle Cell Anemia leh Sickle Cell Disease hi eng nge an danglamna?

Hei hi mi tam tak buaina hmun a ni. Sickle Cell Disease (SCD) hi a chunga kan sawi tak sickle cell natna chi hrang hrang zawng zawng sawina tlangpui a ni. Umbrella ang mai a ni. A chi dang zawng zawng chu chu umbrella hnuaiah chuan an tla vek a ni. Doctor-te chuan "Sickle Cell Anemia" tih thumal hi SCD chi hrang hrang, thisen tlakchhamna thlentu na ber berte tan chauh an hmang thin . Chu chu hemoglobin SS (HbSS) leh hemoglobin beta zero thalassemia an tih chi hrang hrangte a ni. Hrethiam e?

Sickle Cell Trait leh Natna hi eng nge a danglamna?

Mi thenkhat chuan sickle cell trait chauh an nei thin . Hei hian nu leh pa pakhat chauh hnen atangin hemoglobin S gene an rochun tihna a ni . Nu leh pa dang chuan gene hrisel tak a rochun a ni. A tlangpuiin sickle cell trait nei te hian sickle cell natna symptom an nei lo. Mahse, he gene danglam tak hi an fate hnenah an pe chhuak thei a ni. Chumi awmzia chu he gene kengtu an ni tihna a ni. Mahse, a tlem hle a, sickle cell trait nei mite pawhin tui an tlakchham lutuk emaw, exercise nasa tak an tih emaw chuan hriselna lama harsatna an nei thei a ni . Hemi chungchangah hian zirchianna a la kal zel a ni.

Sickle cell natna hi engtiang chiahin nge a hluar?

Zirchiangtute chuan United States-ah ringawt pawh mi 100,000 velin sickle cell natna an vei niin an chhut. African hnam chhuak zingah a tam ber. Hispanic American-te zingah pawh hmuh tur a awm a, Mediterranean, Middle Eastern, Indian leh Asian hnam chhuakte zingah pawh hmuh tur a awm bawk. Sri Lanka ramah pawh hetiang natna vei an awm bawk.

Eng thilin nge sickle cell natna thlen?

Sickle cell natna hi HBB gene an tih gene a genetic mutation avanga lo awm a ni . He HBB gene hian hemoglobin peng khat siamtu a ni. SCD vei te hian mutated HBB genes pahnih an rochun a, chu chuan hemoglobin pangngai lo a siam a – nu leh pa pakhat atanga pakhat. Hei hi autosomal recessive inheritance an ti a. Hei hian SCD vei naupang nu leh pa pahnih hian mutated gene copy khat an keng tel tihna a ni (chu chu sickle cell trait an nei thei tihna a ni). Mahse, chu nu leh pate chuan symptom an nei lo tlangpui.

Tute nge sickle cell disease (SCD) vei theihna sang zawk?

Mi pawl thenkhat chuan he natna hi an vei tam zawk. Anni chu:

• African hnam chhuak (e.g. African American) te.
• South America leh Central America-a Hispanic American-te an ni.
• Mediterranean, Middle Eastern, Indian leh Asian hnam chhuak mite.

Sickle cell natna lanchhuahna chu engte nge ni?

Sickle cell natna lanchhuahna hi naupang thla 5 atanga thla 6 vel a nih atanga a lan tan tlangpui. Heng symptoms te hi mi hrang hrangah a inang lo thei. Mi thenkhat chuan symptoms na lo tak tak an nei a, thenkhat erawh chuan complication na tak tak an nei thei thung. A lan chhuah dan tlangpui chu:

• Natna episode awm fo thin.
• Anemia : Hei hian hah lutuk, vun rawng, leh chak lohna a thlen thei.
• Jaundice : Vun sen leh mit var.
• Kut leh ke na tak a hring chhuak.

Hetiang dinhmun hian eng harsatna nge a thlen?

Sickle cell natna hian taksa peng tam tak a nghawng thei a. Effect thenkhat chu a intan nghal (acute), thenkhat erawh chu rei tak (chronic) a awm thung. Heng harsatnate hi a hmain a intan thei a, dam chhung zawng a daih thei bawk.

Na

Hei hi sickle cell natna avanga harsatna awm tam ber a ni. Sickle ang chi cell te chu thisen kalna kawnga an tang a, thisen kalna tur an tihkhawtlai hian natna hi a awm thin. Natna nasa tak i nei nghal mai thei. Hei hi natna khirh tak, sickle cell harsatna, vaso-occlusive crisis (VOC), emaw vaso-occlusive episode (VOE) te pawh an ti bawk . Heng natna khirh tak takte hi a na lo emaw, na tak emaw pawh a ni thei a, a intan nghal thei a, eng chen pawh a daih thei bawk. A natna hi a tam ber chu rilru, hnungzang, ke leh kut ah a ni. Mi thenkhat chuan chronic pain , chu chu thla ruk aia rei natna tihna a ni.

Thisen tlakchhamna (Anaemia).

Sickle cell natna hian thisen sen (red blood cells) a thih thuai avangin thisen tlakchhamna (anemia) a thlen thin. Anemia chu taksa pum puia oxygen phur thei thisen sen hrisel tak tak awm lohna a ni. Hei hi a niChauhna nasa tak, jaundice, rilru hahdam lohna, luak chhuak, leh lu hah lohna a awm thei.

Acute Chest Syndrome a awm a, a rilru a hah hle

Hei hi nunna atana hlauhawm medical emergency a ni . Lung a tichhe thei a, thawk harsatna a thlen thei a, taksa peng danga oxygen supply a ti tlem thei bawk. He harsatna hi sickle cell-in lung chhunga thisen leh oxygen kal tur a tihkhawtlai hian a thleng thin.

Thisen a chhuak

Sickle cell anemia hian thisen zam a tipung a. Hei hian vein thuk takah thisen zam (deep vein thrombosis - DVT) a awm theihna a tipung a ni. DVT pawh a chhe thei a, lung chhungah a awm thei bawk (pulmonary embolism - PE).

Thai

Sickle cells te chu thluak lam panna thisen kalna kawngah an tang a nih chuan thluaka thisen kal chu a tikhawlo a ni. Thluak hian hna thawk turin oxygen a hmu tlem hle. Hei hian stroke a thlen thei a ni . SCD vei zinga 10% vel chuan clinical stroke an nei ang. Sickle cell anemia vei te hi stroke vei an tam zawk.

Vision lama harsatna awm thei

Sickle cells hian mit chhunga thisen kalna kawng a tikhawlo thei a ni. Hei hi retina ah a thleng tam ber . A chang chuan symptoms a awm lo va, chutah chuan mit a bo nghal thei a, mitdelna nghet tak pawh a thlen thei bawk.

Priapism tih hi a ni

Priapism hi mipa zang chhunga sickle ang chi cell te chu a inkharkhip a, chu chuan erection na tak, na tak ( priapism ) a thlen thin. Natna bakah hian priapism hian chhiatna a thlen thei a, erectile dysfunction a thlen thei bawk . Darkar li aia rei Priapism hi medical emergency a ni.

Organ chhiatna leh hlawhchhamna

Sickle cell natna vei te hian thisen leh oxygen an dawn tlem avangin an thinlung, lung, kal leh taksa peng dangte harsatna tawk thei an ni. SCD hian multi-organ failure a thlen thei .

Sickle cell trait emaw natna emaw hian naupai hi a nghawng em?

Sickle cell natna vei hmeichhia tam tak chuan naupai hrisel tak tak an nei thin. Mahse a risk a sang hle. SCD hian thisen sang, thisen zam, nau pai, nau piang rit lo, leh nau piang hmasa te a tipung thei a ni . Chuvangin doctor thurawn zawm a pawimawh.

Engtin nge sickle cell natna hi hriat theih?

Khawvel ram tam takah ang bawkin Sri Lanka ramah pawh nausen piang thar apiang chu nau piang thar screening neihnaah sickle cell natna an neih leh neih loh endik vek an ni . Hei hian naute kekawr atanga thisen sample te tak te lak a, test a huam a ni. Hei hian natna dang engemaw zat a enfiah bawk. Diagnosis finfiah nan naupang doctor chuan hemoglobin electrophoresis test a ti ang. Tin, sickle cell natna hi naute pian hmain prenatal testing hmangin hriat theih a ni bawk . Heng test hrang hrangte hi:Chung zingah chuan chorionic villus sample lak leh amniocentesis te pawh a tel .

Sickle cell natna hi damdawi tluantling a awm em?

Ni e, bone marrow transplant , stem cell transplant tia hriat bawk hian sickle cell natna a tidam thei a ni. Hei hian donor hrisel, genetically compatible donor (unau ang chi) hnen atanga ruh hrisel tak lak a, damlo chhunga dah luh a huam a ni. Mahse, SCD vei zinga 18% vel chauhvin hetiang match hi an hmu thei a ni. He transplant hi a hlauhawmna leh harsatna a awm bawk. I doctor chuan hei hi a sawipui ang che.

Sickle cell natna enkawl dan tur chu engte nge ni?

Sickle cell natna enkawlnaah chuan damdawi te, thisen pek te, ruhro thlak te, leh gene therapy te a tel a ni . Enkawlna chu antibiotics hmanga tan theih a ni . SCD na tak nei nau piang tharte chu hri kai loh nan ni khatah vawi hnih kum 5 thleng antibiotics pek thin an ni.

Damdawi dang

SCD vei tam tak chuan an natna nasatzia tihziaawm nan leh natna lan chhuah dan enkawl nan damdawi an hmang thin. Chung zinga ṭhenkhat chu:

• Voxelotor: Hei hian thisen sen (red blood cells) te chu sickle ang maia awm leh inzawmkhawm loh nan a veng thei a ni. Hei hian thisen sen thenkhat tihchhiatna a tiziaawm thei a, taksa peng hrang hranga thisen kalna a titha thei a, thisen tlakchhamna (anemia) hlauhawmna a tiziaawm thei bawk.
• Crizanlizumab: He damdawi hian thisen kalna kawng bangah sickle ang maia thisen sen a innghah loh nan a pui a ni . Hei hian thisen kal a titha thei a, inflammation leh natna a tiziaawm thei bawk.
• Hydroxyurea : Hei hian SCD natna chi hrang hrang, natna khirh fo, acute chest syndrome, leh thisen tlakchhamna nasa tak te chu a tiziaawm thei a, a veng thei bawk.
• L-glutamine: Hei hi natna tidamtu a ni. Pain attack i neih zat tihtlem nan a pui thei a ni. Natna tidamtu dangte chu nonsteroidal anti-inflammatory drugs (NSAIDs) leh opiate te an ni .

Thisen pek a ni

I doctor chuan SCD harsatna enkawlna leh venna atan thisen pek thenkhat a rawt thei ang.

• Acute transfusion tih hi: 1.1.Hengte hian thisen tlakchhamna nasa tak thlen thei harsatnate enkawlnaah a pui thin. Doctor-te pawhin stroke, acute chest syndrome, leh organ failure ang chi crise-ah te pawh an hmang thei bawk.
• Red blood cell transfusion: Hengte hian taksaa thisen sen a tipung thei a, sickle ang ni lo, red blood cell pangngai a pe thei bawk.

Stem cell thlak (bone marrow transplant an ti bawk) .

SCD hi stem cell transplant hmangin tihdam theih a ni. Hei hian donor inmil tak (unau ang chi) a mamawh a ni. Nu leh pa emaw, unau thenkhat nena inmil (partially matched siblings) atanga transplant tihchak dan tur zirchianna pawh kalpui mek a ni. I doctor chuan i dinhmun bik a zirin he enkawlna hian a hlauhawmna leh a hlawkna a sawi ang.

Gene Therapy hmanga enkawl a ni

Tunah hian zirchiangtute chuan SCD enkawlna atana gene therapy an zirchiang mek a ni. Hei hian hemoglobin gene pangngai lo siamthat emaw, mi pakhat stem cell-a hemoglobin gene hrisel tak dah emaw a huam a ni. Hemi chungchanga data hmasa berte hi a beisei awm hle. Beiseina chu ni khat chu gene therapy hi SCD enkawlna pangngai a lo ni tawh dawn a ni.

Hei hi ven theih a ni em?

Sickle cell natna hi genetic condition a nih avangin ven theih a ni lo . Naupai i nih chuan i doctor nen genetic testing emaw genetic counseling emaw chungchang sawipui a tha ang . Hei hian nang leh i kawppui nen hian gene hi in nei em tih leh i fate hnena i thlen theihna tur risk chu eng nge ni tih in hriat theih nan a pui ang.

Sickle cell natna vei chuan eng nge a beisei theih ang?

Sickle cell natna vei te hian mipui zawng zawng aiin an dam rei zawk thei a ni. Mahse, SCD enkawlna thar chuan dam rei zawng leh nunphung a tichangtlung hle a ni . Natna hi uluk taka enkawl a nih chuan sickle cell natna vei te chu kum 50 vel an dam thei a ni.

Ka fa hian sickle cell natna a neih chuan engtin nge ka enkawl ang?

I fain sickle cell natna a neih chuan an dinhmun enkawl dan tur tam tak i tih theih a ni:

• I fa chu doctor hnenah hruai fo ang che.
• I fa chu a hun takah vaccine rawt zawng zawng pe vek rawh.
• I fa chu exercise nei reng turin pui la, thinlung hriselna atana tha ei tur ei rawh .
• Natna harsatna a awm chuan i fa chu tui tam tak pe la, nonsteroidal anti-inflammatory drug (NSAID) . In lama natna hi a thunun theih loh chuan i fa chu damdawi inah hruai la, natna tidamtu chak zawk ei tur a ni.

Engtikah nge Emergency Treatment Unit (ETU) ah i kal a ngaih ?

Sickle cell natna hian nunna atana hlauhawm harsatna chi hrang hrang a thlen thei a ni. Nangmah emaw i fain a hnuaia symptoms te hi i neih chuan 911 call emaw, emergency room (ER) hnai ber pan nghal emaw rawh:

• Natna nasa tak a nei.
• Thisen tlakchhamna nasa tak lan chhuah dan: hah lutuk, luak chhuak, leh thawk hah.
• Khawsik chu 101.3 degree Fahrenheit (38.5 degree Celsius) aia sang a ni.
• Mit hmuhna lama harsatna a awm.
• Thâwk harsa.
• Penile erection darkar li aia rei (priapism).
• Acute chest syndrome chhinchhiahna: rilru na, khuh, khawsik.
• Stroke chhinchhiahna: Taksa sir khata chak lohna thut, rilru hahna emaw, hriatna hloh emaw.

Engvangin nge sickle cell natna hian natna a thlen?

A awlsam zawngin, sickle ang maia thisen cell sen chu letter C, a nih loh leh thla (crescent moon) ang mai a ni. I thisen kalna kawng an kal lai chuan an tang a, thisen kalna tur an tikhawlo thin. Chutah chuan a natna chu a lo awm ta a ni. Kawngpuiah traffic jam ang maiin han ngaihtuah teh.

Sickle cell hi autoimmune natna a ni em?

No. Sickle cell natna hian autoimmune diseases -a mizia thenkhat nei mahse, doctor-te chuan SCD hi autoimmune disease-ah an ngai lo. SCD hi genetic condition ah an ngai a .

Sickle cell natna hi dam chhung zawnga natna a ni. Damna famkim pe thei stem cell transplant hi tih theih a ni ngai lo va, a hlauhawm hle bawk. Mahse, a hmaa hriatchhuah leh enkawl hian natna lan chhuah dan a tiziaawm thei a, harsatna awm theihna a ti tlem thei bawk. Damdawi lam enkawlna mumal tak i neih chuan nun famkim, thawkrim takin i nung thei a ni.

A tawp berah chuan thil pawimawh thenkhat hriat reng tur (Take-Home Message) .

Okay, chuvang chuan sickle cell natna chungchang hi kan sawi nasa hle. Thil pawimawh tak tak hriat reng tur thenkhat chu hetiang hi a ni:

• Sickle cell disease (SCD) hi inherited blood disorder a ni a, thisen sen (red blood cells) pianzia a inthlak a, thisen kal danah harsatna a thlen thin.
• Hei hi a chhan ber chu hemoglobin chi danglam tak, hemoglobin S an tih vang a ni.
• Natna te, thisen tlakchhamna te, infection te, taksa peng hrang hrang tihchhiatna te hi a awm fo thin.
• A hmaa hriatchhuah leh enkawl dan dik a pawimawh hle. Nausen piang tharte chu hei hi screening an nei thin.
• Bone marrow transplant hi damdawi ni thei mah se, mi zawng zawng tan a tha lo. Enkawlna dang, damdawi leh thisen pek te pawh a awm bawk.
• Nunphung tihdanglam te, damdawi lam thurawn dik tak zawm te, leh emergency-a thil thleng rang taka hma lain he natna hi i nunpui thei a ni .

Nangmah emaw i hriat miin emaw sickle cell natna chungchanga zawhna i neih chuan doctor hnenah thurawn dil ngei ngei tur a ni. Hlauh tur leh zah tur a awm lo. A pawimawh ber chu inhriattir hi a ni! Sickle cell natna, hemoglobin, thisen sen, thisen tlakchham, genetic natna, natna khirh, sickle cell enkawlna