Chest congestion fo, taksa rihna sang lo... Hei hi Cystic Fibrosis (CF) a ni em? Hei hi i fimkhur ang u.

I fa te hian hnar a tikhawlo reng em? Eng ang pawhin ei tur pe mah se, an hriat loh pawhin an lo thau deuh thin? A chang chuan thawk harsatna nei ang maiin a lang em? Nu leh pate hian heng symptoms te hi an hmuh hian hlauhna an nei hi thil pangngai a ni. Hengte hi Cystic Fibrosis (CF) tih natna lanchhuahna a ni thei a, chu chu kan hre tam lo mai thei a, mahse hriat a pawimawh hle. Hemi chungchang hi hlau lo leh awlsam takin vawiin hian i sawi dawn nia.

Cystic fibrosis (CF) hi eng nge ni tak tak?

A awlsam zawngin, hei hi nu leh pate aṭanga rochun genetic disease a ni . Kan taksa cell kal tlanga chi (chloride) leh tui kal dan tur control tu gene bik a awm a. Hei hi CFTR (cystic fibrosis transmembrane conductance regulator) gene an ti a . Chuvangin, CF natna hi he gene-ah hian chhiatna emaw, mutation emaw a awm chuan a lo awm thin.

He chhiatna avang hian eng nge thleng? A tlangpuiin kan taksaa thil chhuak, mucus leh pus , te hi a hring a, a hnim hring bawk. Mahse CF vei taksaah chuan heng secretion te hi a thim hle a, a thim hle a, a sticky hle bawk .

He mucus thuk tak hi lungah a lo awm khawm hian eng nge thleng tih han ngaihtuah teh. Thâwk a harsa a, natna hrik te chu awlsam takin an tang a , natna hrik a awm fo bawk . Hun a kal zel a, hei hian lungte nasa takin a tichhe thei a, thawk lama harsatna a thlen thei a, thihna thlengin a thlen thei a ni.

Tin, heng secretion thuk tak takte hian pancreas ducts te pawh a block bawk. Tichuan kan ei tur digestive enzymes te chu a chhuak lo . Chuvang chuan ei leh in lama harsatna leh than chak lohna a awm thin. Hei bakah hian thin natna, zunthlum (`Cystic Fibrosis-Related Diabetes` - CFRD) leh nau neih lama harsatna te pawh a awm thei bawk.

Mahse, lungngai suh. Tunah chuan enkawlna hmasawn tak tak avang hian CF vei dam rei zawng chu kum sawm tam tak chhungin a pung ta a ni.

He natna lan chhuah dan engte nge ni?

CF natna lan chhuah dan hi mi hrang hrangah a inang lo thei. Natna nasat dan azirin an danglam bawk. A lan chhuah dan bulpui te han thlir ila.

A tlangpuiin cystic fibrosis a awm thin

Hei hi CF chi khat na zawk a ni. Nakin lawkah symptoms a lang chhuak thin. A chang chuan taksa peng pakhat chauh a nghawng thin.

Eng thilin nge CF hi a thlen?

Kan sawi tawh ang khan hei hi CFTR an tih gene a chhiatna avanga lo awm a ni. Naupangin he natna hi a vei theih nan chuan naupang chuan a nu leh pa hnen atangin a gene chhia copy a dawng tur a ni .

Engtin nge natna hi hriat chhuah a nih?

He natna hi hriat hmasak a pawimawh hle a, a chhan chu chutiang a nih chuan enkawl tan thuai theih a ni a, nakin lawkah harsatna awm thei te pawh a tlem thei ang. Sri Lanka-ah chuan tunah hian damdawi-in ṭhenkhat chuan naupang an pian laiin hetiang hi an enfiah mek a ni.

Natna hriat theihna atana test hman berte chu:

• Sweat test: Hei hi CF hriat theihna atana test rintlak ber a ni.He painless test hian i thawmhnaw chhunga chi (chloride) awm zat a teh thin. A zat chu a tlangpui aia tam a nih chuan CF chhinchhiahna a ni.
• Thisen test: Hei hian thisenah chemical `Immunoreactive Trypsinogen (IRT)` an tih level a test thin. He level hi CF vei zingah a sang hle.
• Genetic testing (DNA test): Hei hian `CFTR` gene-a chhiatna awm leh awm loh direct-in a enfiah thin.

Heng test bakah hian i doctor chuan test dang a rawn rawt thei a, chu chuan a natna hriat chian nan leh harsatna a awm leh awm loh a zawng thei bawk. Entirnan, chest X-ray, pulmonary function test (PFTs), leh sputum leh stool test te.

Enkawlnate chu engte nge ni?

CF hi natna khirh tak a nih avangin enkawlna hi specialist team hrang hrangin an pe a, chung zingah chuan pulmonologist, physiotherapist leh nutritionist te pawh an tel a ni. Enkawlna tum ber chu lung chhunga mucus tihfai te, natna hrik laka invenna te, ei leh in mamawh pek te hi a ni.

Damdawi chi hrang hrang

I doctor chuan damdawi a pe thei a, chungte chu:

• Antibiotics: Lung natna veng leh enkawl.
• Mucus-thinning damdawi: Hengte hi inhaler emaw nebulizer emaw hmanga pek thin a ni. Thick mucus tihthianghlim nan an pui a, khuh pawh a awlsam phah bawk.
• Bronchodilators: Thlawhna kawng a tizau a, thawk a ti awlsam bawk.
• Pancreatic enzyme supplement: Hengte hi chaw ei leh snack apiangah ei tur a ni. Digestion leh nutrient absorption-ah an pui thin.
• CFTR modulators: Hengte hi damdawi class thar ber leh tangkai ber berte an ni. Heng damdawi te hian natna thlentu direct-a awm `CFTR` protein chhia chu a hnathawhna tur a siam tha leh thin. Hei hian mucus a ti thim thei a, lung hnathawh a ti tha a, khuh a ti tlem thei a, taksa rihna pawh a ti tlem thei bawk. `(Trikafta, Kalydeco, Orkambi)` te hi heng ruihhlo chi hrang hrang thenkhat an ni.

Airway Clearance Techniques (ACT) hman dan tur te chu 1.1.

Hengte hi nitin, ni khatah vawi hnih tal tih tur a ni. Anni hian lung chhunga mucus thick tak tak awm chu an tizau a, an paih chhuak thei bawk.

• Chest physical therapy: Mi pakhatin hnungzang leh rilru a rhythmically tap tir a, mucus a tizau.
• The Vest: Hei hi machine bik nena inzawm vest a ni. A hak chuan high-frequency vibrations chuan rilru a tap a, chu chuan action a siam a, mucus a tizau bawk.
• PEP devices: `(Flutter, Acapella)` ang chi device te tak te hmanga thawk chhuah hian lungah pressure a siam a, mucus a tifai thei bawk.

Surgery neih a ni

Complication thenkhatah chuan operation a ngai thei bawk.

• Lung transplant: Natna a nasat em em a, lung a thawh that loh zawng zawng deuhthaw a nih chuan a tawp ber.
• Liver transplant: Liver a chhiat nasat chuan.
• Thil dang: Hnar polyp lakchhuah te, chaw ei tur pe turin chaw kawng chhunga chaw pekna tube dah te ang chi te.

CF avanga harsatna awm thei te

CF hi uluk taka enkawl a nih loh chuan harsatna chi hrang hrang a awm thei a ni.

• Lungs: Thlawhna kawng a zau reng (`Bronchiectasis`), lung a tlakbuak (`Pneumothorax`), natna khirh tak tak a awm.
• Pancreatitis: CF kaihhnawih zunthlum (`CFRD`).
• Liver: Thin hliam (Cirrhosis) a awm.
• Intestinal: Intestinal obstruction, colon cancer vei theihna a sang.
• Reproductive system: Hmeichhiaah mipa nau pai theih lohna leh nau neih tlai.
• Ruh: Ruh a hring (Osteoporosis).

Zawhna zawh fo thin (FAQs) .