I fa chu a tlu fo em? A nih loh leh, a tân tho leh, stairs lawn, tlan, a nih loh leh tlân harsa i ti em? A châng chuan hengte hi naupang tê tête an lo puitlin chhoh ruala an chunga thil thleng pangngai a ni. Mahse, a chang chuan an hnunglam ruh (muscle)-ah hian ngaihven ngai harsatna a awm thei. Chuvang chuan natna pawl khat, zawi zawiin taksa ruh tichak lo thei tur kan sawi dawn a ni.
Muscular Dystrophy hi eng nge ni?
A awlsam zawngin sawi ila, muscular dystrophy hi natna pawl hming tlangpui a ni a, hun kal zelah kan taksa ruhte a tichak lo va, a inthlak danglam theihna a ti tlem a ni. Hei hi a chhan ber chu kan taksa ruh hrisel taka awmtirtu genes-a chhiatna vang a ni.
Mi thenkhat chuan an naupan lai atang tawhin he natna hi an vei thei a, thenkhat erawh chuan kum sawm emaw kum sawm leh panga emaw an tlin hma emaw, puitling thleng pawhin natna lanchhuahna an nei lo thei bawk.
He natna hian mi tin a nghawng dan a inang lo. Natna chi hrang hrangah a innghat. Mi tam tak tan chuan hun kal zelah an natna chu a zual zauh zauh thei a ni. Mi ṭhenkhat phei chuan kea kal theihna emaw, thusawi thiamna emaw pawh an hloh thei a ni. Mahse, chutiang chu mi zawng zawngah a thleng lo . Mi thenkhat chu kum tam tak chhung chu nun pangngai takin an hmang a, natna lan chhuah dan na lo tak tak an nei bawk. Chuvangin hlauhthawn hmasak a ngai lo.
Hei hi eng chi hrang hrang nge ni ber?
Muscular dystrophy hi chi 30 chuang a awm a. Mahse kan hmuh tam ber chi main type 9 a awm. A chi tin hi a thlentu gene, a nghawng ruh, natna lan chhuah tan kum leh natna a zual zel danah a inang lo.
| Natna Hming | Thuziak tawi te te |
|---|---|
| Duchenne taksa ruh natna (DMD) a awm a. | Hei hi a tam ber chi a ni. Mipa naupang a nghawng ber. Kum 3-5 inkar atanga tan a ni. |
| Becker taksa ruh natna (muscular dystrophy) a ni | Duchenne nen a inang a, mahse a symptoms chu a na lo hle. Hei hi mipa naupangah pawh a tam zawk. Kum 11-25 inkar ah hian symptoms a lang thin. |
| Myotonic muscular dystrophy a awm a, a rilru a hah hle | Puitling zinga a tam ber chi. A symptom ber chu taksa ruh (muscle) a inzawm hnua a tih hahdam theih lohna hi a ni. Hmeichhia leh mipa a nghawng thei a ni. Kum 20 chho atang hian a intan tlangpui. |
| Congenital muscular dystrophy a awm thin a ni | Pian tirh atangin emaw, pian hnu rei vak loah emaw a intan thin. |
| Limb-girdle taksa peng hrang hranga natna (muscular dystrophy) a awm | Hip leh shoulder vel muscle te a nghawng a ni. Tleirawl emaw kum 20 emaw atang pawhin a intan thei. |
| Facioscapulohumeral taksa peng hrang hranga natna (muscular dystrophy) a awm | Hmai, kekawrte leh kut chunglam ruhte a nghawng a ni. Kum tleirawl atanga puitling kum 40 hnuai lam thlengin a nghawng thei a ni. |
| Distal muscular dystrophy a awm a, a rilru a hah hle | Kut, ke, kut leh ke ruhte a nghawng a ni. Kum 40-60 inkar atanga tan a ni tlangpui. |
| Mit lam natna (Oculopharyngeal muscular dystrophy) a ni | Kum 40 leh 50 chho atang khan a intan a. Hmai, kawng leh ke ruh a tichak lo a, mitmeng a tla (ptosis), ei harsa (dysphagia) a thlen bawk. |
| Emery-Dreifuss taksa ruh natna (muscular dystrophy) a ni | Mipa naupang a nghawng tam ber a, kum 10 vel atanga tan a ni a, taksa ruh chak lohna bakah hian thinlung lam harsatna a awm thei bawk. |
Engvangin nge hetiang natna hi a lo awm? (A chhan) .
He natna hi rochun a ni thei a, a nih loh leh nangmah emaw, i fa emaw-ah emaw i chhungte zingah tu mahin an neih loh avangin a lo awm hmasa thei a ni. Hei hi genes a chhiatna avanga lo awm a ni .
Ngaihtuah teh, heng genes te hian kan taksa chhunga cell te chu hna hrang hrang thawk tura an mamawh protein siam turin an zirtir a. Chaw ei tur recipe ang mai a ni. Gene a dik lo a nih chuan cell-te chuan protein dik lo, an mamawh aia protein tlem zawk emaw, protein chhia emaw an siam thei a ni.
Muscular dystrophy vei te hian taksa ruh hrisel leh chakna siamtu protein siamtu gene-ah chhiatna an nei a. Entirnan, Duchenne leh Becker-a te hian protein pakhat dystrophin an tih chu a siam chhuak tlem hle. He dystrophin protein hian taksa ruh a tichak a, chhiatna lakah a veng bawk. He protein hi a bo chuan taksa ruhte chu awlsam takin a chhe a, a chak lo bawk.
Engtin nge kan hriat theih ang? (Natna)
He natna chi hrang hrang chhinchhiahna hi naupan lai emaw, tleirawl emaw atang hian a lang tan thin. A tlangpuiin, naupang he natna vei hian a hnuaia chhinchhiahnate hi a lantir thei a ni:
- Tlu fo thin
- Taksa chak lohna hriatna
- Muscle a ti na hle
- Thut atanga tho harsa, stairs chhoh, tlan, emaw, zuang emaw
- Tiptoes-a kal emaw, waddling emaw
Nu leh pa kan nih angin kan fate chetna, a bik takin an inkhelh dan hi kan ngaihven a pawimawh hle. Thil danglam bik i hriat chuan Doctor biak hreh suh.
Chu bakah, mi thenkhat chuan natna lan chhuah dan te pawh an nei thei bawk:
- Scoliosis natna (Scoliosis) a ni
- Mitmeng a tla thla
- Lung lam harsatna a awm
- Thâwk harsa emaw, ei tur ei harsa emaw
- Mit lama harsatna nei
- Hmai ruh chak lohna
Engtin nge doctor hian hei hi a nemnghet? (Diagnosis) tih a ni.
Doctor chuan i fain taksa ruh chak lohna a neih leh neih loh enfiah nan test eng emaw zat a ti ang. A hmasain general physical exam an ti ang a, i chhungkaw medical history an zawt ang che a, i fa symptoms te an zawt ang.
Chumi hnuah chuan hetiang test engemaw zat hi tih theih a ni a, chu chuan taksa chak lohna thlen thei dinhmun dang chu paih chhuah a ni thei a, natna hi chiang taka hriat theih a ni bawk.
| Test Hming | A awlsam zawngin, hei hi i tih dan a ni... (Simple Explanation) |
|---|---|
| Thisen test a ni | Muscle a chhiat huna thisena enzyme thenkhat awm khawm level chu enfiah thin a ni. |
| Electromyography (EMG) hmanga enfiah a ni. | Electrode te te te chu taksa peng hrang hrangah dahin, taksa peng hrang hrangte electrical activity chu an teh thin. |
| Muscle biopsy hmanga tih a ni | Muscle te tak te tak te chu needle hmangin an la a, microscope hmangin an enfiah thin. Hei hian eng protein nge bo emaw, chhia emaw tih a hriat theih. |
| Electrocardiogram (EKG/ECG) hmanga tih a ni. | He heart rate leh rhythm hi a hrisel em tih enfiah nan a electrical signal te chu an teh thin a ni. |
| Genetic test neih a ni | Thisen sample test hian eng gene dik lo nge muscle chak lohna thlentu tih chiang takin hriat theih a ni. |
Enkawlnate chu engte nge ni?
Tunah hian muscular dystrophy hi damdawi a la awm lo . Mahse, natna lan chhuah dan control thei, nun awlsam zawk, leh i fate nunphung tha ber pe thei tur enkawlna tam tak a awm. I fain a natna chi hrang hrang a zirin i doctor chuan enkawlna dik ber a rawn rawt ang che.
- Physical therapy: Exercise leh stretch hrang hrang hian taksa ruh a tichak a, a inthlak danglam thei bawk.
- Occupational therapy hmanga enkawlna: 1.1.Naupang chu a theih ang anga nitin hna (e.g., incheina, ziak) tih dan tur zirtir an ni. Wheelchair leh cane ang chi hmanrua hman dan zirtir an ni bawk.
- Speech therapy: I hmai emaw, i kawr ruh emaw chak lohna avanga tawng harsatna i neih chuan tih dan awlsam tak tak kan zirtir ang che.
- Damdawi: 1.1.
- Damdawi bik, `Eteplirsen (Exondys 51)` ang chi, DMD chi thenkhat tan dystrophin siam chhuah tipungtu.
- Muscle spasm tihziaawmna damdawi.
- Lung lam harsatna nei tan thisen sang damdawi.
- Steroids `Prednisone` ang chi hian taksa ruh chhiatna a ti hniam thei. Mahse, hengte hian side effect a nei a, doctor thurawn hnuaiah chauh hman tur a ni .
- Surgery: Spinal stenosis leh heart problems ang chi complication te tan surgery neih a ngai thei.
Naupang enkawl huna ngaihtuah tur thilte
Fate chakna a tlahniam zauh zauh a, naupang dang angin an tlan leh inkhelh theih loh chuan nu leh pate tan lungngai hi thil pangngai tak a ni. Mahse, he harsatna karah pawh hian i fa chu hlim taka nung turin i pui thei a ni.
- Ei leh in tha pe rawh: Ei leh in inthlau tak hian i fa chu a taksa rihna hrisel tak a neih theih nan a pui thin. Hei hian thawk harsatna ang chi thil a tiziaawm thei a ni.
- Active takin awm rawh: Swimming ang chi exercise low-impact hian taksa ruh a tichak thei a ni. Hemi chungchang hi i physical therapist zawt rawh.
- Assistive devices hmang rawh: I fa chu a tul chuan wheelchair, crutches, etc. hmang turin fuih rawh.
- Chak takin awm rawh: He zinnaah hian i rilru hahna, lungngaihna leh thinrimna i neihte chu chhungte, ṭhiante emaw, counselor emaw hnenah sawipui rawh. Tin, hetiang natna vei naupang nu leh pa dangte nena support group neih hi chakna nasa tak a ni thei bawk.
He natna hian mi zawng zawng a nghawng dan inang vek lo. Naupang thenkhat chuan taksa ruh chakna an hloh thuai thin. Mi dangte chuan an hloh rang zawk mai thei. Chuvang chuan i fa doctor nen zalen taka a dinhmun chungchang sawipui a, a tana inhmeh ber tur enkawlna ruahmanna siam hi a pawimawh hle.
Take-Home Thuchah a ni
- Muscular dystrophy hi genetic disease a ni a, hun kal zelah taksa ruh a tichak lo hle.
- Naupangte natna lan chhuah hmasak ber chu tlu fo te, thawh harsat te, kekawr bul atanga kal te a ni thei.
- He natna hi chi hrang hrang a awm avangin, dik taka hriat theih nan damdawi lam thurawn lak a pawimawh hle.
- Damdawi famkim awm lo mah se, taksa lam enkawlna, damdawi leh enkawlna dangte chuan natna lan chhuahna chu a thunun thei a, nunphung ṭha tak a vawng reng thei a ni.
- Naupang leh chhungkaw tan rilru lam puihna leh boruak tha tak siam a pawimawh hle. I doctor nen harsatna i neih chuan sawiho rawh.











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