I nervous system tichhe thei natna ALS (Amyotrophic Lateral Sclerosis) chungchang hriat i duh em?

A châng chuan i rilru a buai ang maiin i inhria em, i ke ruhte chu a hlim lo va, i thusawi laiin i thusawi chu a hlimthla ang maiin i inhria em? Hengte hi thil pangngai a ni thei. Mahse, a tlem berah chuan thil pawi zawk chhinchhiahna an ni thei. Vawiin hian condition pakhat kan sawi dawn a, a buaithlak deuh a, mahse a fimkhur a pawimawh hle. Chu chu ALS, a nih loh leh Amyotrophic Lateral Sclerosis .

ALS (Amyotrophic Lateral Sclerosis) hi eng nge ni? A awlsam zawngin i hrethiam ang u!

I thluak leh i ruhro (i ruhro chhunga nerve cord) chu computer control unit ber atan ngaihtuah rawh. Hengte hi i taksa chhunga thil awm zawng zawng thununtu an ni. He system ah hian cell chi hrang hrang `neurons` tih te a awm a. Palai te tak te ang maiin an che thin. Heng nerve cell te hian thluak atanga kan taksa peng leh taksa peng hrang hrangah thuchah an phur a, chutiang ti turin an hrilh thin.

Tunah chuan he natna ALS an tih ah hian thil thleng chu i sawi nerve cells (`neurons`) te kha a chhe vek a ni . A bik takin kan taksa che vel control tu nerve cell te hi hetah hian target ber an ni. Chu tirhkohte chuan an hna an thawk thei lo ang mai a ni. Chuti a nih chuan eng nge thleng? Thluak atanga message lo lut te hi taksa ruh lamah a kal tha lo hle. Zawi zawiin he dinhmun hi a chhe zual sauh sauh a ni .

A tirah chuan i taksa peng hrang hrangah chak lo deuhin i inhria thei a, i taksa ruhte chu a inzawm khawm ang maiin (`muscle twitching`) . Mahni chauha kal te, thil pakhat la tûra kut phar te, chaw ei te, a nih loh leh chiang taka thusawi te chu i harsat mai thei. Hun a kal zel a, taksa ruhte chu hman loh vangin a atrophy (`atrophy`) tan a ni . Machine hman loh leh rust chhuak ang mai a ni. A tawpah chuan he natna hian i thawk dan a nghawng thei a ni. A châng chuan nunna atana hlauhawm tak a ni thei bawk.

Lou Gehrig natna hi i hre tawh ngei ang . Chu chu ALS awmzia chu a ni. Lou Gehrig hi kum 1920 leh 1930 chho vela baseball player lar tak a ni. He natna hi a nei bawk.

America ang ramah pawh statistics atanga a lan dan chuan kum tin mi 5,000 vel chu ALS vei thar an ni. Chuvangin, hei hi a awm vak lo nain, tu pawha lo awm thei dinhmun anga ngaih tur a ni.

Hei hi damdawi a la awm lo . Mahse, lungngai suh. Enkawlna kawng hrang hrangte chu nitin a tha chho zel a ni. Enkawlna dik tak inzawmkhawm chuan natna darh chak lutuk hi thunun theih a ni a, nunphung pawh nasa takin a tichangtlung thei a ni.

ALS hi chi hnih a awm a.

ALS hi a lo awm dan a zirin chi hnih ah then theih a ni:

1. Sporadic ALS: Hei hi a tam ber a ni.. ALS vei zinga 90% vel chu he category-ah hian an tel a ni. Hei hi random occurrence mai a ni. Chu chu thlahtu a ni lo tihna a ni. A lo awm chhan chiang taka sawi a har.

2. Chhungkaw ALS: Hei hi a tlem deuh a, a vei zinga 10% vel a ni. Genetic changes avanga lo awm a ni . Hei hian a awmzia chu nu emaw pa emaw atanga rochun genes chhiatna avanga lo awm a ni tihna a ni.

ALS natna lan chhuahna chu engte nge ni? I han en teh ang.

ALS natna lan chhuah dan hi mi hrang hrangah a inang lo deuh thei a, hun kal zelah a danglam zel bawk. A tir lamah chuan symptom thenkhat chu a fiah lo hle a, hriat theih loh tluk a ni thei.

• Kut, ke leh kawr chhunga thau chak lohna: I dam lo mai mai ang maia inhriatna. Thil chawi emaw, vawn emaw a harsa khawp mai.
• Muscle cramps: Taksa tihkhauh nghal, na tak a ni.
• Kut, ke, kekawrte leh/ emaw lei ruhtea inhnim hring (twitching) hriatna: chhungril atanga inherna te tak te lo chhuak ang maiin.
• Muscle stiffness (`spasticity`): Kawngkhar khauh tak, thinga siam ang maia nghet lo, ben emaw, tihdik emaw harsa ang maia inhriatna.
• Thusawi harsatna: Thusawi thiam lo, thumal dik taka sawi harsa, leh aw inthlak danglam.
• Chaw ei harsa emaw, tui chhuak fo emaw.
• Chau tih hriat reng (chau lutuk).
• Chaw emaw tui emaw ei harsa (dysphagia): Chaw ei kham emaw, ei harsa emaw tih hriatna.
• Nuih emaw, tap emaw ang chi rilru natna lo chhuak nghal mai, thunun theih loh: Lungngaihna nei lovin i tap thei a, a nih loh leh chhan awm lovin i nui thei bawk. Hei hi damdawi lamah chuan `Pseudobulbar affect` an ti bawk.

A tir lamah chuan a lan chhuah dan langsar ber chu kut leh kea chak lohna leh nghet lo, tawng harsa leh ei harsa te hi a ni. Hei hian nitin hna ziak leh ei leh in te tih a ti harsa thei hle. Hun a kal zel a, heng symptoms te hi taksa pum puiah a darh tlangpui. Mahse, heng symptoms te hi engtiang chiahin nge a lo thang chak tih hi mi hrang hrangah a inang lo.

As symptoms worsen , thawk harsa, ding, kal harsa a ni thei. Tin, i taksa rihna tlahniam nghal mai pawh i tawng thei bawk. Heng symptoms te hi i neih a, a zual zel ang tih a nih chuan doctor pan ngei ngei tur a ni . Thâwk harsa i neih chuan , emergency a nih avangin damdawi inah i kal nghal tur a ni.

Engvangin nge he natna ALS an tih hi a lo awm? A chhan chu eng nge ni?

Dik tak chuan zirchiangtute chuan ALS hi eng nge a thlen tih chiang takin an la hre lo a , mahse thil hrang hrang inzawmkhawm a nih an ring.

• Genetics: 1.1.Hereditary ALS chungchang hi kan sawi tawh a. Chuvangin, gene inthlak danglamna thenkhat a tel a ni. Heng genetic changes te hi hereditary ALS patient 70% vel ah hmuh a ni a, sporadic ALS patient 5% leh 10% inkar ah hmuh tur a awm bawk. A bik takin, gene `C9orf72`, `SOD1`, `TARDBP` leh `FUS`-a inthlak danglamna hi a tam ber a ni. Hemi nena inzawm hian gene 40 chuang hmuhchhuah a ni tawh bawk.
• Environment factors: A châng chuan toxins ( lead emaw mercury emaw ang chi), virus ṭhenkhat, emaw taksaa trauma-te hian chanvo an nei nia ngaih a ni a, mahse hengte hi zirchian a la ni reng a ni.

Researcher ten an finfiah theih chu natna hian kan motor neurons a tichhe tih hi a ni . Heng motor neurons te hi nerve cells te an ni a, kan thil tih te, kan thusawi te, chaw ei te, kan taksa peng hrang hrang kan tihchet te, leh thawk te kan thunun a ni.

I thluak leh i taksa ruhte hi telephone line ang maia inzawmna nei niin han ngaihtuah teh. Nerve cell te hian he line hmang hian taksa peng hrang hrangte hnenah thuchah an thawn a, hetiang ti turin, chutiang ti turin an hrilh thin. ALS-ah chuan he telephone line-a signal hi a distort ta a ni . Phone-a reception hloh ang mai a ni. Thluak atanga taksa peng hrang hranga thuchah lo lutte chu a chhe vek a, chiang takin a kal tlang lo. A tawpah chuan he connection hi a bo vek a, call tihtawp ang maiin. Tichuan, chu nerve cell te chuan message thar an thawn thei lo. Chuvang chuan a hmaa ka sawi tawh ang symptoms te kha i nei thin.

ALS hi thlahtu natna a ni em?

ALS chi thenkhat chu genetic a ni a, chu chu thlah khat atanga thlah dangah a kai theih tihna a ni. ALS thlentu genetic change chu i nu leh pate hnen atangin i rochun mai thei. Mahse, he ALS chi (`inherited ALS`) hi a awm vak lo . A tam zawkah chuan genetic changes hi random takin a thleng thin a, chu chu i chhungkuaah hian tumahin he natna hi an la nei lo a nih pawhin a thleng thei tihna a ni.

Tute nge ALS vei theihna chance sang zawk?

Tu pawhin ALS vei thei mahse, mi thenkhat chu midang aiin an hlauhawmna a sang zawk tih hmuhchhuah a ni.

• Kum: Kum 55 leh 75 inkar te hian natna lanchhuahna an nei tam ber.
• Hnam leh chi: Data atanga a lan dan chuan mipa hring (non-Hispanic) te hian ALS an vei tam zawk.
• Sex: Kum 55 hnuai lam ah chuan hmeichhia aiin mipain he natna hi an vei tam zawk.
• Veterans: Zirna thenkhatah chuan sipai service member te hian risk a sang zawk deuh thei tih an sawi. Zirchiangtute chuan hei hi boruak thil (toxin emaw pesticide emaw ang chi) nena inzawmna avang a ni thei niin an ngai.) emaw, hnathawhna hmuna taksa lama accident thleng emaw a ni thei.

ALS natna avanga harsatna awm thei te chu engte nge ni?

He natna lan chhuah dan hi a na zual zel a, vanduaithlak takin dam hun chhung hi a tawi thei a ni . He natna chungchang zir leh nitin a hmachhawn dan hi rilru lamah a harsa hle thei a ni. I rilru a buai a, i bo a, beiseina i nei lo a, i rilru a hah hle bawk. Chumi avang chuan ALS vei nia hriat tam tak chuan rilru lam harsatna, lungngaihna leh lungkhamna te pawh an nei ve bawk .

I taksa hriselna enkawl dan tur doctor leh nurse tam tak an awm a. Mahse i rilru hriselna pawh enkawl a pawimawh bawk . Ṭanpuina i mamawh chuan i medical team emaw, mental health counselor emaw nen inbiakna nei rawh .

Engtin nge doctor-te hian ALS hi dik taka an hriat theih?

Doctor chuan taksa a enfiah hmasa ang che (`physical exam`) , chutah chuan `neurological exam` a ti leh ang. Chu bâkah, ALS i vei leh vei loh hriat nân test dang engemaw zât an nei bawk.

ALS i vei tih i hre nghal thei lo mai thei. Doctor vawi tam tak i pan a ngai mai thei a, specialist engemaw zat i hmuh a ngai mai thei bawk. I doctor chuan i symptoms te chu chhui chian lehzual turin test hrang hrang a ti ang a, i taksa a nghawng dan a enfiah ang. Hei hi a chhan chu natna dang tam tak, ALS ang chi symptom nei an awm vang a ni. Chuvangin, diagnosis dik tak siam theih nan heng test hrang hrang zawng zawng hi tih a pawimawh hle .

ALS hriat theihna atan eng test nge hman thin?

Diagnosis finfiah nan test engemaw zat i tih a ngai mai thei a, chungte chu:

• Thisen test: Damdawi lam thil dang a awm loh nan.
• Zun test: Hengte hi a chhan dang hriat loh nan an ti bawk.
• Electromyogram (EMG): Hei hi taksa ruh hnathawh dan enfiah nan hman a ni. Nerve atanga taksa peng hrang hranga message thawn chhuah dik leh dik loh a enfiah thin.
• Nerve conduction study: Hei hian nerve te hian signal an thawn theih dan a test thin.
• Magnetic Resonance Imaging (MRI): Hei hian i thluak emaw, i ruhro emaw thlalak a la a, chhiatna a awm leh awm loh a en a ni. Tin, natna dang (tumor ang chi) avanga natna lanchhuahna a awm leh awm loh a enfiah thei bawk.

ALS enkawlna tur engte nge ni? Damdawi awm lo mah se, hahdamna a awm em?

Vanduaithlak takin ALS vanga nerve cell tihchhiatna chu tidanglam thei tur damdawi a la awm lo . Mahse, lungngai suh. Enkawlna hian natna lan chhuah dan a tikhawtlai thei a, a theih ang anga i hlim theih nan a pui thei bawk.

I medical team te chuan enkawlna hrang hrang an rawt thei ang:

• Damdawi chi hrang hrang
• Therapeutic method hrang hrang emaw rehabilitation programme hrang hrang
• Nutritional support a ni
• Thâwk lama harsatna awmte tanpui

Natna a zual zel chuan enkawlna chi hrang hrang emaw, enkawlna tam zawk emaw i mamawh thei a ni. Chu bâkah, a rei thei ang bera mahni inrintawkna leh nuam taka nun theih nân ṭanpuitu enkawlna i dawng thei bawk.

ALS damdawi pek thin te

ALS enkawlna atana United States Food and Drug Administration (FDA)-in a pawmpui damdawi chi li a awm a, chungte chu:

• Riluzole: Hei hian motor neurons chhiatna a tihziaawm rin a ni. Thla engemaw zat dam rei theihna a pui thei bawk.
• Edaravone: He damdawi hian taksa ruh hloh zat a ti hniam thei a.
• Sodium phenylbutyrate/taursodiol: Hei hian natna lan chhuahna rate a control thei a ni.
• Tofersen: He damdawi hian nerve cell chhiatna thenkhat a tihziaawm thei. Hei hi i doctor-in SOD1 an tih gene-a inthlak danglamna a hmuh chuan a \angkai thei ang.

Heng damdawi lian ber berte bakah hian i natna lan chhuah dan control thei tur damdawi dang pawh a awm bawk. Entîr nân, taksa ruh tliak, ruh tliak, hmui chhuah tam lutuk, natna, leh rilru lam harsatnate damdawi i hmang thei a ni.

Therapies hrang hrang (`Therapies`) .

I doctor chuan enkawlna kawng hrang hrang emaw, rehabilitation service emaw a rawt thei a, chu chu:

• Physical therapy: Hei hian a rei thei ang bera mahni inrintawkna leh him taka awm turin a pui che a ni. Exercise awlsam tak tak, taksa ruh tichaktu leh hriselna zawng zawng tichaktu a zirtir che a ni.
• Occupational therapy: Hei hian nitin hna thawh tur tih dan tur ruahmanna a zirtir a, chu chu wheelchair emaw brace emaw ang chi assistive devices hmangin a ni.
• Speech therapy: Hei hian him taka ei leh inbiak theihna a siam a ni. Tin, nonverbal communication methods (e.g., signs, writing) a zirtir bawk che a, chu chuan a rei thei ang bera thusawi leh chakna i humhim theih nan a pui bawk.

Nutritional support a ni

ALS vei tan chuan a chang chuan an mamawh ei leh in tur hmuh a harsa thei hle. Ei harsat hian taksa rihna a ti tlahniam thei a, an mamawh vitamin leh minerals hmuh a harsat phah thei bawk.

Dietitian a niChaw ei harsa tak takte ei loh nan leh calorie, fiber leh tui dik tak pe thei tur ei tur ruahmanna siam turin a pui ang che. Nutritional counseling hian ei leh in hrisel tak i neih theih nan a pui thei che a ni. Chaw ei a harsat chhoh zel avangin nutritionist chuan ei dan dang, a remchan dan tur pawh a rawt thei bawk.

A tul chuan feeding tube hman theih a ni. Hei hian ei tur emaw tui emaw lung chhunga lut thei a ti tlem thei a , chu chuan choking emaw pneumonia ang chi natna a thlen thei a ni .

Thâwk lakna support

ALS a zual zel chuan thawk a harsa thei hle. Thil tih dan pakhat chu noninvasive ventilation (NIV) tih a ni. Hei hian thawk lakna tur hnar leh hmui khuh tu mask hman a huam a ni. A tir lamah chuan zanah chauh hman a ni thei a, mahse a hnuah chuan nilengin hman a ngai mai thei.

Hun a kal zel a, i lung chhunga thawk luh leh chhuah theihna tur mechanical ventilation, thawkna machine respirator an tih chu i mamawh mai thei. Thâwk lama harsatna i neih chuan, a bik takin i mut emaw, eng thil pawh i tih laiin, i healthcare team hnenah hrilh rawh. I thawk hahdam theihna tur option hrang hrang an sawipui thei che a ni.

Engtikah nge Doctor i hmuh ang? Heng thilte hi fimkhur rawh

Heng symptoms zinga pakhat tal i neih chuan doctor pan rawh:

• Nitin hna thawh tur i tih harsat nia i hriat chuan .
• Symptoms a na zual deuh niin a lang a nih chuan .
• Mahni chauha i che thei lo dinhmunah i ding a nih chuan.
• Enkawlna hian side effect a neih chuan .

ALS hian thawk harsa a tih theih dan tur kan sawi tawh a. Doctor pan tura fuih tur thawk harsatna chhinchhiahna thenkhat chu hetiang hi a ni:

• Thâwk harsa i tih chuan , chawlh lai pawhin.
• Khuh chu a chak lo ang tih a hriat chuan .
• Thlarau leh lung awlsam taka tihfai theih loh chuan .
• Saliva tam lutuk a awm khawm chuan.
• Khumah i mu thei lo a nih chuan (i thawk hah i tih vang a ni).
• I rilru natna (pneumonia) i neih fo chuan .

Heng symptoms te hian respiratory failure an tih natna a thlen thei a ni . Hei hian i taksa mamawh oxygen i hmuh theih nan oxygen i thawk tling thei lo tihna a ni. Hei hi nunna atana hlauhawm tak a ni . Chuvangin, thawk harsatna i neih chuan emergency room-ah kal nghal rawh .

ALS laka inven dan tur a awm em?

Dik tak chuan ALS hi ven theih a ni.Damdawi finfiah a la awm lo. Hei hi a chhan leh a hlauhawmna hriat chian belh zel a, nakin lawka inven dan tur siam chhuah a nih theih nan zirchianna kalpui mek a ni.

ALS natna hi eng chen nge i dam theih ang? Eng ang thlirna nge ni ang?

ALS vei dam rei zawng chu a natna hmuhchhuah atanga kum thum atanga kum nga chhung a ni . Mahse, mi 30% vel chu kum nga emaw a aia rei emaw an dam nia chhut a ni a, 10% leh 20% inkar chu kum sawm tal an dam nia chhut a ni. I dinhmun hi heng statistics te nen hian a danglam thei . Chuvangin, i dinhmun hriat belh duh chuan i doctor nen inbia ang che.

ALS prognosis hi a tha vak lo a, a chhan chu motor neurons hnathawh a nghawng dan vang a ni. I thlirna chu nerve cells tihchhiatna a thlen rang danah a innghat a ni. He chhiatna hi damdawi engmahin a tidanglam thei lo nain, i doctor chuan a natna lan chhuah dan tihziaawmna tur duhthlan tur a pe thei che a ni.

Tunah hian ALS hi damdawi a la awm lo.

ALS i vei chuan clinical trial-a tel ve i duh mai thei . Heng zirchiannate hian zirchiangtute chu enkawlna thar siam chhuakin, natna hi an hrethiam zawk a ni.

A tawp berah chuan hriat reng tur pawimawh ber chu!

Amyotrophic lateral sclerosis, a nih loh leh ALS i vei tih i hriat chuan zawhna leh rilru natna tam tak i nei thei a ni.

Heti hian i ngaihtuah mai thei, ‘Engvangin nge hetiang hi ka chungah a lo thlen? Eng thilin nge he thil hi a thlen?’ a ti a. I sam hrual te, chaw tui tak ei te, a nih loh leh i hmangaih tak takte biak te ang chi thil awlsam tak i tih theih tawh loh hunah chuan i lungngai a, i lungngai a, i rilru a hah hle thei bawk. Hei hian lungngaihna leh lungkhamna a thlen thei a, a bik takin i natna lan chhuah dan a zual zel chuan.

Khawi hmunah pawh awm la, i rilru put hmang pawh ni se, i medical team te chu i tanpui turin an awm reng a ni . ALS enkawl dan pawh nitin a tha chho zel a, enkawlna thar zirchian leh enchhin mek a ni bawk. Tunah hian damdawi a awm loh laiin, enkawlna awmsa chuan natna lan chhuah dan a tikhawtlai thei a, i hmangaih tak takte nen hun tam zawk i hmang thei bawk. Mahni chauha inhriat ngai suh. Ṭanpuina leh ṭanpuina dil rawh. I chakna hi i thil neih ropui ber a ni.

` ALS, Amyotrophic Lateral Sclerosis, Neuropathy, Muscular Dystrophy, Lou Gehrig’s, Neurons, Motor Neurons, Thlawhna lama harsatna te