Koj tus me nyuam puas muaj tus mob hnyav no thiab? Cia peb kawm txog SCID (Severe Combined Immunodeficiency)!

Koj tus me nyuam puas mob tas li? Txawm tias mob khaub thuas me me los puas hloov mus ua ib yam dab tsi loj heev? Qee zaum, ua niam txiv, peb txhawj xeeb ntau txog cov no. Hnub no, peb yuav tham txog ib qho mob tsis tshua muaj uas ua rau ntshai thiab tswj tau yog tias koj paub zoo. Nws hu ua SCID (Severe Combined Immunodeficiency). Lub npe suab me ntsis ntshai, tab sis cia peb tham txog nws kom meej thiab yooj yim.

SCID (Severe Combined Immunodeficiency) yog dab tsi? Cia peb nkag siab yooj yim.

Hais yooj yim xwb, SCID yog ib qho mob tsis tshua muaj . Qhov tshwm sim yog tias thaum cov menyuam mos yug los, lawv lub cev tiv thaiv kab mob, uas tawm tsam kab mob, tsis muaj zog heev lossis tsis muaj kiag li. Xav txog peb lub cev tiv thaiv kab mob zoo li ib pab tub rog uas tiv thaiv peb lub tebchaws. Nws yog lub cev no uas tiv thaiv peb los ntawm kev tawm tsam cov yeeb ncuab (piv txwv li cov kab mob) uas los ntawm sab nraud. Yog li, "pab tub rog" no ntawm tus menyuam mos uas muaj SCID tsis muaj zog heev, lossis yuav luag tsis muaj.

Qhov no hu ua tus kab mob tiv thaiv kab mob tsis txaus . Qhov ntawd txhais tau tias nws yog ib yam dab tsi uas muaj thaum yug los. Yog li ntawd, rau cov menyuam mos uas muaj SCID, txawm tias yog ib qho mob me me uas peb feem ntau tsis quav ntsej txog, xws li mob khaub thuas, nyuaj rau kov yeej. Yog tias tsis kho, qhov mob no tuaj yeem ua rau tuag taus hauv ib xyoos lossis ob xyoos. Yog vim li cas nws thiaj li tseem ceeb heev uas yuav tsum paub txog qhov no.

"Combined immune deficiency" yog dab tsi?

Peb lub cev tiv thaiv kab mob zoo li lub zog tiv thaiv hauv peb lub cev. Thaum cov khoom txawv teb chaws, xws li cov kab mob , cov kab mob me me , cov pwm , cov protozoa , lossis cov tshuaj lom, nkag mus rau hauv lub cev, lub zog tiv thaiv no paub lawv thiab mus tawm tsam. Cov tub rog tseem ceeb hauv kev sib ntaus sib tua no yog cov qe ntshav dawb . Ntawm lawv , cov lymphocytes yog cov tshwj xeeb. Muaj peb hom lymphocytes:

• Cov T-cell
• Cov qe ntshav B
• Cov qe ntshav tua neeg ntuj (NK hlwb)

Rau cov menyuam mos uas muaj SCID, cov T-cell no feem ntau tsis txaus lossis tsis muaj kiag li. Vim tias cov B-cell xav tau kev pab los ntawm cov T-cell kom ua haujlwm tau zoo, thaum cov T-cell ploj mus, cov B-cell kuj tsis ua haujlwm tau zoo thiab. Yog vim li ntawd nws thiaj hu ua "ua ke" - vim tias nws yog kev sib xyaw ua ke ntawm ntau hom hlwb tiv thaiv kab mob tseem ceeb.

Muaj ntau hom SCID sib txawv, nyob ntawm seb hom hlwb tiv thaiv kab mob uas tus menyuam ploj lawm. Tab sis txhua hom no puav leej loj ib yam. Cov kws kho mob yuav piav qhia rau koj seb koj tus menyuam muaj hom twg.

SCID feem ntau tshwm sim li cas?

SCID yeej yog ib qho mob tsis tshua muaj.Piv txwv li, nyob rau hauv Tebchaws Meskas, qhov mob no cuam tshuam txog li ib tug ntawm 50,000 tus menyuam mos uas yug txhua xyoo.

Tiam sis thaum koj pom tias koj tus menyuam muaj ib yam dab tsi zoo li no, nws tsis tshua muaj li cas los xij, puas yog? Nws txaus ntshai heev, ua rau mob siab heev. Thaum koj hnov ​​txog ib qho mob zoo li SCID, nws tsis yog ib lo lus xwb, nws dhau los ua qhov tseeb uas muaj kev cuam tshuam loj heev rau koj lub neej.

Cov tsos mob ntawm SCID yog dab tsi?

Qee zaum tus menyuam mos uas muaj SCID yuav tsis muaj cov tsos mob tshwj xeeb thaum xub thawj. Txawm li cas los xij, cov tsos mob feem ntau muaj xws li:

• Tus me nyuam qhov hnyav tsis nce raws li qhov xav tau.
• Mob raws plab ntev.
• Muaj mob ntau zaus, mob hnyav heev.

Qhov no yog vim tus menyuam lub cev tiv thaiv kab mob tsis muaj zog heev, yog li nws tsis tshua muaj lossis tsis teb rau kev mob. Qhov no txhais tau tias koj tus menyuam yuav muaj feem mob ntau dua li lwm tus menyuam. Tsis tas li ntawd, yog tias lawv mob, lawv cov tsos mob yuav hnyav dua li lwm tus.

Cov menyuam mos uas muaj SCID muaj feem yuav kis tau txhua yam kab mob ntau dua. Qhov no suav nrog:

• Cov kab mob bacterial
• Kev kis kab mob
• Cov kab mob fungal
• Cov kab mob parasitic

Txawm hais tias txhua yam kab mob tuaj yeem ua rau mob hnyav, muaj ntau hom kab mob uas feem ntau tshwm sim rau cov menyuam mos uas muaj SCID:

• Cov kab mob poov xab xws li mob thrush lossis mob pob khaus hauv qhov ncauj thiab tus nplaig.
• Mob qaib.
• Cov pob khaus ncig lub qhov ncauj (Kab mob khaub thuas - herpes simplex).
• Kab mob pob ntseg.
• Mob ntsws o.
• Kab mob hlwb (mob hlwb).

Yog koj tus me nyuam tseem muaj cov tsos mob no, koj yuav tsum mus ntsib kws kho mob tam sim ntawd.

Dab tsi ua rau muaj SCID?

SCID yog ib qho kev mob caj ces . Qhov ntawd txhais tau tias nws yog tshwm sim los ntawm kev hloov pauv caj ces , lossis tsuas yog kev hloov pauv hauv peb cov noob caj ces. Cov kws tshawb fawb tau txheeb xyuas ntau tshaj kaum ob qhov kev hloov pauv caj ces uas tuaj yeem ua rau SCID.

Cov kev hloov pauv caj ces no yog tau txais los ntawm niam txiv mus rau cov menyuam. Lawv tuaj yeem tau txais los ntawm autosomal recessive lossis X-linked .

• Autosomal recessive txhais tau hais tias rau ib tug me nyuam kom muaj tus mob no, lawv yuav tsum tau txais cov noob caj noob ces hloov pauv los ntawm lawv niam thiab txiv.
• X-linked SCID yog tshwm sim los ntawm kev hloov pauv caj ces ntawm X chromosome. Feem ntau nws cuam tshuam rau cov tub hluas ntau dua, vim tias cov tub hluas muaj ib qho X chromosome, thaum cov ntxhais muaj ob lub X chromosomes.

Cov teeb meem uas yuav tshwm sim los ntawm SCID yog dab tsi?

Qhov teeb meem loj tshaj plaws thiab txaus ntshai tshaj plaws yog tus menyuam tsis muaj lub cev tiv thaiv kab mob zoo los tawm tsam cov kab mob. Vim tias tus menyuam lub cev tsis tuaj yeem tawm tsam txawm tias yog qhov me me xwb los xij, qhov kev pheej hmoo ntawm kev muaj teeb meem loj heev yog siab dua li lwm tus menyuam.

Txawm tias cov kab mob uas feem ntau tsis loj heev kuj tuaj yeem ua rau muaj teeb meem loj rau lub neej ntawm ib tug me nyuam uas muaj SCID.

Yog tias tus mob no tsis raug kuaj pom thiab kho thaum yug los, SCID feem ntau yuav ua rau tuag taus.

Cov kws kho mob kuaj mob SCID li cas?

Cov kws kho mob kuaj mob SCID los ntawm kev kuaj ntshav . Qhov kev kuaj no yog ua los ntawm kev siv cov ntshav me me los ntawm tus menyuam lub pob taws tom qab yug los.

Qee lub tsev kho mob hauv Sri Lanka kuj ua cov kev kuaj mob no rau cov menyuam mos, tshwj xeeb tshaj yog tias ib tug neeg hauv tsev neeg tau muaj cov mob no ua ntej. Qhov no tseem ceeb heev, vim tias yog tias pom tus kab mob thaum ntxov, kev kho mob tuaj yeem pib ua ntej muaj kev kis kab mob hnyav.

Puas muaj kev kho mob rau SCID?

Yog, muaj kev kho rau SCID. Cov menyuam mos uas muaj SCID yuav tsum tau hloov hlwb pob txha lossis hloov hlwb pob txha sai li sai tau. Qhov no suav nrog kev txhaj cov hlwb pob txha noj qab haus huv los ntawm tus neeg pub rau hauv lub cev ntawm tus menyuam. Cov hlwb noj qab haus huv no pab tus menyuam tsim lub cev tiv thaiv kab mob tshiab, muaj zog dua uas tuaj yeem tawm tsam cov kab mob.

• Cov neeg pub nyiaj zoo tshaj plaws yog cov kwv tij neej tsa uas tsis muaj SCID.
• Yog tias tsis muaj tus neeg zoo li ntawd, cov kws kho mob yuav tshawb xyuas cov npe ntawm cov hlwb noob . Cov no yog cov ntaub ntawv ntawm cov hlwb noob uas pej xeem pub dawb.

Thaum tos kev hloov noob hlwb (stem cell transplant), koj tus menyuam yuav xav tau lwm yam kev kho mob. Piv txwv li:

• Tshuaj tua kab mob - Tiv thaiv cov kab mob bacterial.
• Cov tshuaj tua kab mob - Tiv thaiv cov kab mob viral.
• Cov tshuaj tua kab mob fungal - Tiv thaiv cov kab mob fungal.
• Kev txhaj tshuaj IVIG - Qhov no yog ua los ntawm kev muab cov tshuaj tiv thaiv kab mob los ntawm sab nraud lub cev los tiv thaiv kev kis kab mob.
• Kev kho mob los ntawm noob caj noob ces - Qhov no tseem yog kev kho mob sim, tab sis nws yuav ua tau zoo rau qee hom SCID.

Cov no tsuas yog kev kho mob ib ntus xwb, SCID tsis tuaj yeem kho tau tag nrho. Tib txoj kev los kho SCID yog los ntawm kev hloov noob hlwb.

Thaum tos lub sijhawm hloov cov noob qes (stem cell transplant), tus menyuam yuav tsum tau muab tso rau hauv chav cais kom huv . Qhov no txhais tau tias txhua yam uas nkag mus rau hauv chav menyuam yuav tsum tau ntxuav kom huv. Qhov no yog ua los tiv thaiv tus menyuam ntawm cov kab mob.

Muaj ib txhia neeg hu SCID ua "kab mob menyuam yaus ua npuas" vim tias tus menyuam raug khaws cia rau hauv lub npuas tiv thaiv. Tab sis lub npe no tsis yog qhov tseeb hauv kev kho mob, thiab nws tuaj yeem ua rau tus menyuam thiab niam txiv mob siab. Koj tsis tau ua dab tsi tsis ncaj ncees lawm, thiab koj tus menyuam txoj kev noj qab haus huv tsis yog ib yam dab tsi uas yuav tsum tau saib tsis taus.

Yog tias kuv tus menyuam muaj SCID, kuv yuav tsum xav li cas?

SCID yog ib yam mob uas yuav ua rau tuag taus yog tias tsis kho sai sai. Yog li ntawd, nws yog ib qho tseem ceeb heev uas yuav tsum nrhiav seb tus menyuam puas muaj SCID sai li sai tau. Cov kws kho mob kuaj pom tus kab mob sai li sai tau thiab xa tus menyuam mus rau kev hloov noob hlwb sai li sai tau, qhov zoo dua uas yuav cawm tau tus menyuam txoj sia.

Tej zaum koj yuav tsis tuav thiab puag koj tus menyuam ntau npaum li koj xav tau. Yog tias koj tus menyuam nyob hauv ib qho chaw uas tsis muaj kab mob, koj yuav tsum ua raws li cov cai huv huv kom tiv thaiv koj tus menyuam ntawm cov kab mob. Cov kws kho mob yuav piav qhia txhua yam no rau koj. Lawv kuj yuav qhia koj tias yuav tsum xav li cas ua ntej thiab tom qab hloov cov noob hlwb.

Tus menyuam uas muaj SCID yuav muaj sia nyob ntev npaum li cas?

Yog tsis kho, cov menyuam mos uas muaj SCID feem ntau tuag hauv ib xyoos lossis ob xyoos. Txawm li cas los xij, cov menyuam uas tau hloov cov hlwb stem cell tiav lawm tuaj yeem ua neej nyob li qub. Thaum lawv lub cev tsim kom muaj lub zog tiv thaiv kab mob zoo, feem ntau lawv tsis muaj teeb meem mus sij hawm ntev.

Puas tiv thaiv tau SCID?

SCID yog tshwm sim los ntawm kev hloov pauv caj ces uas peb tsis tuaj yeem tswj tau, yog li ntawd peb tsis muaj dab tsi ua tau los tiv thaiv nws. Yog tias koj muaj keeb kwm tsev neeg ntawm tus mob caj ces no, nrog koj tus kws kho mob tham txog kev sab laj txog caj ces ua ntej muaj menyuam.

Kuv yuav tsum mus ntsib kws kho mob thaum twg?

• Yog tias koj tus menyuam feem ntau mob, lossis muaj cov tsos mob hnyav thaum muaj mob, mus ntsib kws kho mob tam sim ntawd.
• Yog koj twb paub lawm tias koj tus menyuam muaj SCID, qhia rau koj tus kws kho mob tam sim ntawd yog tias koj pom muaj kev hloov pauv hauv koj tus menyuam txoj kev noj qab haus huv (piv txwv li, kub taub hau, hnoos, raws plab). Vim tias koj tus menyuam lub cev tsis tuaj yeem tawm tsam cov kab mob ntawm nws tus kheej, nws yog ib qho tseem ceeb uas yuav tsum muab tshuaj tua kab mob lossis lwm yam tshuaj rau lawv tam sim ntawd.

Kuv yuav tsum nug kuv tus kws kho mob li cas?

Nws yog ib qho ib txwm muaj uas koj yuav xav tsis thoob thiab poob siab thaum koj pom tias koj tus menyuam muaj SCID. Tab sis tsis txhob ntshai nug koj tus kws kho mob txog tej lus nug uas koj muaj. Nws yog ib qho tseem ceeb uas yuav tsum paub meej txog txhua yam lub sijhawm no. Tej zaum koj yuav nug cov lus nug xws li:

• Kuv tus menyuam puas muaj SCID lossis lwm yam mob uas tiv thaiv kab mob tsis zoo?
• Puas yuav tsum tau muab kuv tus menyuam tso rau hauv ib qho chaw cais tawm uas tsis muaj kab mob?
• Thaum twg kuv tus menyuam yuav tsum tau hloov cov hlwb noob (stem cell transplant)?
• Kuv yuav tsum paub txog tej kev hloov pauv lossis tej tsos mob twg ntawm kuv tus menyuam?

Nco ntsoov tias, nws yog ib qho tseem ceeb heev uas yuav tsum npaj kev hloov pauv stem cell rau koj tus menyuam sai li sai tau. Tsis tas li ntawd, koj muaj cai nkag siab txhua yam uas tab tom tshwm sim. Cov kws kho mob yuav piav qhia txhua yam rau koj thiab qhia koj tias yuav tsum xav li cas.

Thaum kawg, cov lus coj mus tsev:

SCID (Kev Tiv Thaiv Kab Mob Hnyav Ua Ke) yog ib qho mob hnyav thiab txaus ntshai heev. Txawm li cas los xij, kev paub thaum ntxov, nrhiav kev pab kho mob sai li sai tau thaum muaj cov tsos mob tshwm sim, thiab tau txais kev kho mob kom zoo tuaj yeem cawm tau tus menyuam txoj sia.

• Kev kuaj mob thaum ntxov: Nws yog ib qho tseem ceeb heev uas yuav tsum txheeb xyuas SCID los ntawm kev kuaj mob menyuam mos lossis sai li sai tau thaum cov tsos mob tshwm sim.
• Kev hloov hlwb ntawm cov noob qes: Qhov no yog kev kho mob tseem ceeb thiab ua tau zoo tshaj plaws rau SCID.
• Ib puag ncig muaj kev nyab xeeb: Nws yog ib qho tseem ceeb los tiv thaiv tus menyuam kom tsis txhob muaj kab mob thaum lub sijhawm kho mob.
• Lub zog ntawm lub hlwb: Qhov no yuav yog lub sijhawm nyuaj ua niam txiv. Nrhiav kev txhawb nqa los ntawm cov kws kho mob, tsev neeg, thiab cov kws pab tswv yim.

Koj tsis yog tib tug xwb. Nrog kev nce qib hauv kev kho mob, cov menyuam yaus uas muaj SCID tam sim no tuaj yeem ua neej noj qab nyob zoo thiab muaj lub neej puv npo. Nyob twj ywm muaj zog thiab muaj kev cia siab.

Kev tiv thaiv kab mob, SCID, cov kab mob thaum yau, cov kab mob caj ces, kev hloov pob txha, kev tiv thaiv kab mob tsis zoo, cov kab mob sib kis