He kal natna hmuh tur awm lo tak hi i hria em? (Atypical Hemolytic Uremic Syndrome - a HUS) a ni a, a rilru a hah lutuk a, a rilru a buai em em bawk a.

I kal chhunga thisen kalna kawng te tak te chhungah thisen zam (blood clots) lo awmin, i kal (kidney)-a thisen kalna tur a tikhawlo a nih chuan eng nge thleng ang? Ngaihtuah a hlauhawm khawp mai, a ni lawm ni? Chu chu vawiina kan sawi tum tak chu a ni. Hei hi Atypical Hemolytic Uremic Syndrome, a tawi zawngin aHUS an ti a. Dam theih ni lo mah se, enkawl dan dik tak hmangin a enkawl tha thei a ni. Chuvangin a chanchin hi i zir belh leh ang u.

Atypical leh Typical HUS hi eng nge an danglamna?

HUS tih hming pu condition i hre tawh mai thei. "Typical HUS" kan ti a. A tlangpuiin E. coli ang chi bacteria infection vang a ni a, chu chuan riltam a thlen thin. Mahse vawiin a kan sawi tum condition, aHUS hi chu a danglam hle.

He natna hi aHUS tia koh hi kum eng pawhah a awm thei. Naupang tê têah chuan mipa leh hmeichhia a nghawng vek a ni. Amaherawhchu, an kum a upat deuh deuh chuan hmeichhiate hian he natna hi an vei chak zawk a ni. Hei hi a chhan chu naupai hi natna thlentu lian tak a nih theih vang a ni. A tlem em avangin zirchianna thenkhatah chuan mi maktaduai khat zinga pahnih chauhvin he natna hi an vei tih an sawi.

Engvangin nge aHUS hi a lo awm?

A awlsam zawngin, aHUS vei tam tak chuan an taksaah hian genetic change, a nih loh leh gene mutation an nei a. I taksaah hian he natna thlen thei ‘button’ awm ang maiin han ngaihtuah teh. Mahse chu button on mai mai chuan natna chu a thlen nghal mai lo. ‘Turn’, emaw activate tur chuan a pawn lam thil a mamawh a ni. Hengte hi ‘triggers’ kan ti thin.

Trigger lian ber berte chu:

• Naupai lai
• Hri kai theihna thenkhat
• Ngawt
• Damdawi thenkhat
• Organ transplant pakhat a ni
• Hun rei tak chhunga damdawi lama harsatna thenkhat (systemic sclerosis emaw malignant hypertension emaw ang chi) .

Eng nge a lan chhuah dan?

aHUS symptoms hi trigger a awm hnuah a lang nghal tlangpui. Flare-ups hi a na lo thei a, a na thei bawk. A nasat loh chuan kal (kidney) hi a nghawng vak lo thei a, mahse a nasat chuan kal (kidney failure) a thlen thei a ni .

A tir lama i hriat theih tur symptoms:

• Taksa na leh hah lutuk
• A tlangpuiin hrisel lohna neia inhriatna

Hei hi natna zual zel a ni. Chumi awmzia chu hun kal zelah symptoms a na zual tihna a ni a, a bik takin a hmaa hriat chhuah a nih loh chuan a na zual zel tihna a ni. aHUS hian i kal (kidney) a nghawng ber. Mahse, taksa peng dang, thluak, thin, lung leh thinlung te pawh hi thisen kalna kawng a inkharkhip avangin a nghawng thei bawk.

Engtin nge natna hi kan hriat theih ang?

I chhungte zingah tumahin he natna hi an la vei lo a nih chuan aHUS hriatchhuah a harsa deuh thei. A tlangpuiin nephrologist emaw hematologist emaw hi he thil hi hriat tur tha ber a ni. A chunga table-a thil tarlan te hi a bik takin an ngaihven ang a, test tul tak tak atan an refer ang che.

Enkawlna kawng hrang hrangte chu engte nge ni?

Vanneihthlak takin aHUS damdawi pawmpui pahnih a awm a.

• `Eculizumab (Soliris) tih a ni
• `Ravulizumab-cwcz (Ultomiris)` tih a ni

Heng pahnih hi damdawi chi khat `monoclonal antibodies` an tih zinga mi an ni. Taksa immune system peng pakhat an control a, thisen zam a titawp bawk.

Damdawi ``Eculizumab`` tih hian i thisenah platelet leh red blood cells a tipung thei a ni. If treatment is started as soon as possible , he damdawi hian kal chhiatna thenkhat pawh a tidanglam thei bawk.

Heng damdawi pahnih hi injection hmanga pek a ni. Anni hian side effect tenau te, lu na, flu ang chi natna, pum na, leh taksa na te an thlen thei a ni. A pawimawh ber chu heng damdawi ei hian natna khirh tak tak, meningitis (meningococcal disease) ang chi a awm theihna a awm. Chuvangin i doctor chuan chutiang tur chuan shot la turin a hrilh ang che a, chubakah natna kai loh nan antibiotics a pe thei bawk ang che.

Heng damdawi bakah hian plasma therapy pawh hman theih a ni. Hei hian damlo chu mi hrisel hnen atanga lak plasma (thisen tui tak) pek emaw, damlo plasma lakchhuah a, plasma hrisel hmanga thlak (plasma exchange) emaw a huam a ni.

Heng enkawlna zinga pakhat mah kidney-in a chhan loh chuan a hnuhnung ber chu kidney dialysis emaw kidney transplant emaw a ni .

Harsatna leh emergency awm thei te

Kidney failure bakah hian aHUS hian harsatna dang a thlen thei bawk.

He natna leh rilru hriselna nena khawsa

Hetiang natna rei tak nena kan nun hian lungngaihna leh lungkhamna hi thil pangngai tak a ni. I sualna a ni lo. Chuvangin i rilru hriselna bakah i taksa hriselna pawh ngaihtuah a pawimawh .

• I medical team-te tanpuina dil rawh: Doctor, nurse, leh social worker-te chuan i mamawh rilru lam puihna i hmuh theihna hmunte chu an kaihruai thei che a ni.
• Counsellor hmu rawh: Licensed counselor nena inbiakna chuan i phurrit tihziaawmna leh he dinhmun hmachhawn tur hian i mamawh rilru chakna a siam thei a ni.
• Mahni in enkawl rawh: Mut tawk rawh. Stress tihziaawmna tur kawng zawng rawh. Kidney-friendly diet ei rawh. Exercise tam thei ang ber nei rawh. Heng thilte hian i hriselna zawng zawngah nghawng lian tak a nei thei a ni.
• I hmangaih takte nên sawipui rawh: Hemi chungchâng hi chhûngte leh ṭhiante nêna sawipui chuan thlamuanna nasa tak a pe ang che.