Neurofibromatosis Type 2 (NF2) hi eng nge ni? A awlsam zawngin sawi ila.

I taksa hmun thenkhatah hian lump te te te a lo awm em? A nih loh leh hriatna hloh tlem leh luak chhuak i nei em? Hengte hi thil pangngaiah kan ngai a, mahse a chang chuan heng thil hnung lamah hian kan hriat tam loh genetic condition a awm thei. Vawiin hian chutiang natna pakhat chungchang kan sawi dawn a ni. He natna hi Neurofibromatosis Type 2 a ni a, a hming tawi kan hriat vek chu NF2 a ni. Hei hi a buaithlak deuh a, mahse awlsam takin i hrethiam ang u.

A awlsam zawngin NF2 hi eng nge ni?

NF2 hi genetic condition a ni a, kan nervous system a nghawng a ni. Kan taksaa gene pakhat a inthlak avangin kan taksa hian signal dik lo a dawng a, cell tam lutuk a siam chhuak thin. He cell tam lutuk hian a pungkhawm a, tumor a siam thin.

A tha ber chu heng lumps tam zawk hi benign/noncancerous an ni . Chumi awmzia chu taksa peng dangah an darh lo tihna a ni. Mahse, heng lumps lo awmna hmun azirin harsatna hrang hrang kan tawk thei a ni.

Heng bumps lo awm theihna hmun tam ber chu:

• Kan taksa pum puia nerve (peripheral nerves) te.
• Thluak leh luruh inkar (meninges) inkar membrane.
• Spine a ni.
• Thluak leh beng chhungril inzawmkhawmtu, hriatna leh balance-a puitu nerve te.

NF2 hi natna chi khat `neurofibromatosis` an tih zinga mi a ni a, he natna pawl hian kan vun leh nervous system a nghawng ber a ni.

He natna hi engtiang chiahin nge a hluar?

Hei hi natna tlanglawn tak a ni lo. Statistics atanga a lan dan chuan NF2 hian khawvel puma naupang piang 33,000 zinga pakhat vel a nghawng a ni. Neurofibromatosis vei hmuhchhuah zawng zawng zinga 3% vel chu NF2 vei an ni.

NF2 hi eng symptoms nge ni?

NF2 natna lan chhuah dan hi mi hrang hrangah a inang lo thei. I taksaa tumor awmna hmun leh a lian ziaah a innghat a ni. Mi tam tak tan chuan a lan chhuah hmasak ber chu hriatna control tu nerve-a tumor awm vang a ni.

A hnuaia table hian heng symptoms te hi chiang zawkin a hrethiam thei ang.

Naupan lai atanga kum 30 inkar inkarah hian symptoms hi a lang tan fo thin a, mahse, he natna hian kum eng pawha mi a nghawng thei a ni. Puitling hian hriatna lama harsatna an tawk hmasa zawk. Mahse, naupangte hian thluak emaw, ruhro emaw-ah tumor an nei tam zawk. Naupan lai atanga a lan chhuah tan chuan natna hi a na zawk thei tihna a ni.

NF2-ah hian eng chi lumps nge lo awm?

NF2-ah hian nodules chi hrang hrang hmuh tur a awm a. Tlemte han thlir ila.

Engvangin nge NF2 hi a lo awm? Eng nge a chhan?

A chhan ber chu kan taksaa gene `NF2` genetic change vang a ni. He gene hian protein pakhat `merlin` an tih siam nan a pui a ni. He protein hnathawh ber chu tumor (tumor-suppressor) siam chhuah tihtawp a ni.

Chuvangin, `NF2` gene-a inthlak danglamna a awm chuan `merlin` protein hi a siam tha lo hle. Tichuan kan taksaa cell thenkhat chu thunun theih lohvin an inthen darh a, an pung chho tan ta a ni. Hei hi cell tam lutuk te chu an pungkhawm a, tumor an siam dan a ni.

He genetic variation hi kawng hnih in kan hmu thei a ni:

1. Heredity: Nu leh pa zinga pakhatin he gene mutation hi a neih chuan naupangin a rochun theihna chance 50% vel a awm (`autosomal dominant` pattern).

2. Randomly: A chang chuan chhungkaw zingah tumahin he natna hi an nei lo a nih pawhin nau pai lai hian random takin genetic mutation thar a awm thei. Hei hi NF2 vei tam zawkin natna an neih dan a ni.

Tute nge hlauhawma awm? Eng harsatna nge awm thei?

I chhungkuaa mi tu emaw, a bik takin i nu leh pate’n NF2 an neih chuan natna vei theihna i nei bawk.

NF2 vang hian harsatna engemaw zat a awm thei.

• Hriatna hloh vek.
• Mit hmuhna hloh vek.
• Nerve tihchhiat a ni.
• Thluak chhunga fluid awm zat.

A tlem hle a, NF2 nodules thenkhat chu cancer a ni thei a, chuvangin regular medical checkup neih hi a pawimawh hle.

He natna hi engtin nge kan hriat theih ang?

Doctor-in a enfiah ang che a, he natna hi i vei leh neih loh finfiah nan test hrang hrang a kalpui ang che. A hmasain taksa enfiahna an nei ang. I vun inthlak danglamna leh i taksa balance ang chi thil te an en ang. Tichuan i symptoms leh chhungkaw medical history te an zawt ang.

Tin, a hnuaia test te hi tih theih a ni bawk.

• MRI scan: Hei hian i nervous system leh vunah tumor a awm leh awm loh chiang takin a hmu thei.
• Hearing test: I hriatna level enfiah rawh.
• Mit enfiah: Mit (cataract) emaw, mit lama harsatna dang emaw a awm leh awm loh enfiah thin ang che.
• Genetic testing: `NF2` gene ah hian mutation a awm leh awm loh enfiah thin ang che.

NF2 enkawl dan tur chu engte nge ni?

Dik tak chuan NF2 hi damdawi a la awm lo. Amaherawhchu, natna hriatchhuah dan leh enkawl danah nasa takin hma a sawn a. Heng enkawlnate hian natna lan chhuah dan (symptoms) control-na kawngah a pui a, a theih ang anga nun pangngaia i nun theih nan a pui bawk. Mi hrang hrangah enkawl dan a inang lo. I doctor chuan eng enkawlna nge i tan a tha ber tih a rel ang.

A châng chuan i lumps hian harsatna lian tham a thlen lo a nih chuan i doctor chuan enkawl lovin enkawl a tum mai thei. Mahse, natna kal zel dan enfiah nan kum khatah vawi khat tal taksa enfiahna, scan, leh hriatna leh mit test neih a pawimawh hle.

Hei hi enkawltu medical team te hian

NF2 hi taksa system hrang hrang tichhe thei natna a nih avangin doctor team specialty hrang hrang neiin an enkawl a ni. He team-ah hian a tlangpuiin:

• Neuro-oncologists: Doctor, cancer leh nervous system-a tumor hrang hranga specialist te an ni.
• Neurosurgeon: Surgeon, nervous system lama tui tak te.
• ENT surgeon: Beng, hnar leh beng surgeon te.
• Audiologist: Hriatna lama mithiam bik.
• Genetic counselor: Genetic natna chungchanga thurawn pe thin specialist.

Enkawlna hian side effect a nei em?

Ni e, enkawlna dang ang bawkin heng enkawlnate hian nghawng tha lo a nei thei a ni. Enkawlna chi hrang hrang a zirin a danglam thei.

Tumor lakchhuahna tur operation-ah hian hlauhawmna engemaw zat a awm. Heng tumor te hi thluak leh ruhro ang chi hmun sensitive tak takah an awm avangin thisen chhuah, natna hrik kai, nerve tihchhiat te pawh a awm thei. Mahse i surgical team chuan heng hlauhawmnate hi tih tlem tumin theihtawp an chhuah ang.

Chemotherapy leh radiation therapy-ah chuan .

• Ek khal
• Kawthalo
• Chau lutuk
• Sam tlakchhamna
• Chaw chakna
• Nausea leh luak chhuak

Side effects te chu: Enkawl i tan hmain i doctor nen side effect awm thei te sawipui hmasa phawt ang che.

He natna vei dam chhung hi eng nge i sawi theih ang?

NF2 hian mi dam chhung a nghawng dan hi thil tam takah a innghat a ni. Hliam lian tham tak, a awmna hmun leh a natna hmuhchhuah hmasak ber kum te hi a pawimawh ber pawl a ni. A chang chuan a hliam hian symptom a nei lo thei bawk. A châng dangah chuan a lan chhuah dan chu a na hle thei a ni.

A tha ber chu natna hi hriat hmasak a , harsatna a thlen hmain enkawl tan a ni . Tichuan outlook chu a tha zawk hle. I symptoms-in a nghawng dan azirin i doctor chuan prognosis dik zawk a pe thei ang che.

NF2 hi ven theih a ni em?

No. NF2 hi genetic condition a nih avangin ven theih a ni lo. Mahse, chhungkaw history-a he natna hi a awm a, chhungkaw din i tum a nih chuan fa i neih hmain genetic counselling neih hmasak phawt a tha.A hnuaia mite hi en a pawimawh hle: I fain he natna a vei theihna tur hriatthiamna tha zawk i neih theih nan.