Yu smɔl pikin kin sik ɔltɛm? I kin bi XLA (X-Linked Agammaglobulinemia). Mek wi tok!

Pan ɔl we sɔm tɛm wi kin tink se na nɔmal tin fɔ mek wi smɔl pikin dɛn sik bɔku tɛm, fɔ sɔm pikin dɛn dis kin bi siriɔs prɔblɛm smɔl. Ɛspɛshali if bɔbɔ de sɔfa ɔltɛm wit baktriyal infɛkshɔn, i kin bi bikɔs ɔf wan jenɛtik kɔndishɔn we nɔ kin apin so ɔltɛm. Tide wi go tok abaut jos dis kain kondishon.

Wetin na XLA (X-Linked Agammaglobulinemia)?

Okay, so wetin na XLA (X-linked agammaglobulinemia)? Fɔ tɔk am simpul wan, na wan sik we pɔsin kin gɛt frɔm in jɛnɛtiks . Wi bɔdi gɛt wan rili impɔtant sojaman dɛn fɔ fɛt sik, ɛn dat na wi imyun sistɛm . Wan spɛshal kayn sɛl na dis sistɛm we dɛn kɔl B-sɛl . na dεn B-sεl dεm ya de mek protin dεm we dεn kכl antibodi dεm fכ fכt sik we wi bכdi sik. Dɛn antibodi ya de wok lɛk sojaman dɛn we de difend wi kɔntri.

Pɔsin we gɛt XLA (X-Linked Agammaglobulinemia) nɔ de mek dɛn B-sɛl ya fayn fayn wan. Ɔ dɛn kin prodyuz smɔl pan dɛn. So, wetin kin apin we yu nɔ ebul fɔ mek antibodies? Yu kin sik rili izi ɛn yu kin sik bɔku tɛm. Dɔn bak, di tisu dɛn na wi bɔdi we gɛt fɔ du wit di imyun sistɛm, lɛk di limf no dɛm , di tɔnsil dɛm, ɛn di adenoyd dɛm, nɔ kin divɛlɔp fayn, ɛn sɔntɛnde dɛn nɔ kin fɔm atɔl. Dis sik kin mɔs si pan bɔy pikin dɛn . Wi go tɔk bɔt di rizin fɔ dis leta.

Dis kכndyushכn, we dεn kכl XLA (X-Linked Agammaglobulinemia), dεn kin kכl am כda nem dεm bak:

• Bruton in agammaglobulinemia
• Agammaglobulinemia we dɛn bɔn wit
• Hypogammaglobulinemia we gɛt di sik

Bɔt dɛn kin yuz di nem Hypogammaglobulinemia bak fɔ ɔda sik we fiba dis. Dat na CVID (Kɔmɔn Variable Immunodeficiency) . CVID (Common Variable Immunodeficiency) nɔ kin rili bad lɛk XLA (X-Linked Agammaglobulinemia), ɛn dɛn kin no am mɔ we pɔsin big. Bɔt dɛn kin no pikin dɛn we gɛt XLA (X-Linked Agammaglobulinemia) bifo dɛn ol wan ia ɔ we dɛn rili yɔŋ.

Wetin na di difrɛns bitwin XLA (X-Linked Agammaglobulinemia) ɛn SCID (Sivɛri Kɔmbayn Immunodefisiɛns)?

Naw yu kin de wɔnda if dis na XLA (X-Linked Agammaglobulinemia) ɔ di siriɔs kɔmbayn imyunodefisiɛns kɔndishɔn we yu go dɔn yɛri bɔt we dɛn kɔl SCID (Severe Combined Immunodeficiency) . Nɔ, smɔl difrɛns de bitwin dɛn tu. insay XLA (X-Linked Agammaglobulinemia) , di B-sεl dεm we wi bin tכk bכt fכs na dεn kin afekt mεntal. insay SCID (Severe Combined Immunodeficiency) , di T-sεl dεm de afekt.כda imכtant kayn imyun sεl we dεn kכl T-sεl dεm (sכmtεm B-sεl dεm kin afekt bak). Dɛn tu tin ya na jenɛtik kɔndishɔn we kin mek di imyun sistɛm wik ɛn bɔku tɛm kin mek pɔsin gɛt sik. Bɔt di men difrɛns bitwin dɛn tu na di kayn sɛl we dɛn afɛkt.

Aw kɔmɔn tin fɔ XLA (X-Linked Agammaglobulinemia)?

Dis sik we dɛn kɔl XLA (X-Linked Agammaglobulinemia) nɔ kin rili apin . Dat min se nɔto sik we bɔku pipul dɛn kin gɛt. Bɔt, lɛk aw wi bin dɔn tɔk, i kin apin mɔ pan bɔy pikin dɛn . Fɔ sho se na lɛk wan pan ɔl tu ɔndrɛd tawzin (200,000) bɔy pikin dɛn kin bɔn wit XLA (X-Linked Agammaglobulinemia).

Wetin na di sayn dɛm fɔ XLA (X-Linked Agammaglobulinemia)?

Bikɔs pikin dɛn we gɛt XLA (X-Linked Agammaglobulinemia) nɔ gɛt bɛtɛ imyun sistɛm, bɔku tɛm dɛn limf no dɛn, tɔnsil, ɛn adenoyd dɛn kin smɔl ɔ dɛn nɔ kin de . Dis kin mek dɛn kin gɛt baktriyal infɛkshɔn bɔku tɛm frɔm we dɛn smɔl. Fɔ ɛgzampul:

• Bronchitis : Dis na infεkshכn na di brכnki, di tכb dεm we de go na di lכng.
• Infεkshכn na di ia (Otitis media) : Infεkshכn na di midul ia.
• Sinusitis : Infεkshכn na di sayn dεm we de rawnd di nos.
• Nyumonia : Na bad bad infεkshכn we de afekt di lכng.
• Gastrointestinal infεkshכn : Tin dεm lεk bεlε we de at εn dayariya.

Bɔt i impɔtant fɔ mɛmba se pikin dɛn we gɛt XLA (X-Linked Agammaglobulinemia) nɔ kin gɛt vayral infɛkshɔn (e.g., Cytomegalovirus (CMV) , RSV (Respiratory Syncytial Virus) , ɔ fɔngal infɛkshɔn .Dɛn kin mɔna bɔt baktriyal infɛkshɔn.

Wetin kin mek pɔsin gɛt XLA (X-Linked Agammaglobulinemia)?

As wi bin dɔn tɔk, XLA (X-Linked Agammaglobulinemia) na sik we pɔsin kin gɛt frɔm in jɛnɛtiks . Dis min se pikin kin gɛt am frɔm in mama, in papa, ɔ ɔl tu. Wi gɛt wan jin we dɛn kɔl BTK jin na wi bɔdi. Dis jin de sho wi bɔdi fɔ mek B-sɛl dɛn. Wi no se di B-sεl dεm de mek antibodi dεm εn fכ fɛt sik dεm.

so, if chenj כ mכtεshכn de na dis BTK jin, dεn nכ kin mek B-sεl dεm fayn fayn wan. Dɔn, wetin kin apin na dat pɔsin we nɔ gɛt da jin muteshɔn de nɔ kin ebul fɔ fɛt sik dɛn di we aw dɛn kin fɛt. Na dat mek dɛn kin sik ɔltɛm, ɛn sɔntɛnde dɛn kin ivin gɛt siriɔs sik dɛn we kin put dɛn layf pan denja.

Wetin na ‘X-linked’?

Naw lɛ wi luk wetin ‘X-linked’ min. Wi ɔl kin gɛt wi jin frɔm wi mama ɛn papa dɛn ɔl tu. Dɛn jin ya de pan tin dɛn we dɛn kɔl kromozom . Dɛn jin ya de tɛl wi bɔdi aw fɔ mek di prɔtin dɛn we i nid fɔ wok. Bɔku tɛm, ivin if chenj de na wan jin, di ɔda kɔpi (di wan we kɔmɔt frɔm di ɔda mama ɔ papa) stil de, so di bɔdi kin wok lɛk aw i fɔ wok.

Bɔt di kromozom dɛn we man dɛn gɛt fɔ du mami ɛn dadi biznɛs nɔto di sem. Dɛn gɛt wan X kromozom ɛn wan Y kromozom . So, if mכtεshכn de na wan jin na dis X kromozom, nכ כda kכpi de fכ kכmpεns am. di BTK jin we wi bin tכk bכt de na dis X kromozom. Na dat mek dɛn kɔl am ‘X-linked’. So if bכy pikin gεt mכtεshכn na di BTK jin na in X kromozom, i go divεlכp XLA (X-Linked Agammaglobulinemia).

Girl pikin dɛn gɛt tu X kromozom dɛn. ivin if dεn gεt di mכtεshכn we de mek XLA (X-Linked Agammaglobulinemia) pan wan pan dεn X kromozom dεm, di BTK jin na di כda X kromozom stil de wok fayn fayn wan, so dεn kin mek di nכmba we nid fכ di B-sεl dεm. So dɛn nɔ kin gɛt di sik, bɔt dɛn kin gɛt di sik . Dat min se dɛn kin kɛr di jin we dɛn nɔ sho ɛni sayn.

Wetin na di risk factor dɛm fɔ XLA (X-Linked Agammaglobulinemia)?

So fa, di onli risk factor wae dεn no fכ mek yu gεt XLA (X-Linked Agammaglobulinemia) na fכ famili histri fכ dis kכndyushכn . Dis min se na tin we pɔsin kin gɛt frɔm dɛn gret gret granpa dɛn.

Yu tink se gyal pikin dɛn kin gɛt XLA (X-Linked Agammaglobulinemia)?

Yes, very rarely , uman pikin kin divεlכp XLA (X-Linked Agammaglobulinemia) bak. Bɔt fɔ mek dat apin, di mama ɛn papa dɛn ɔl tu fɔ kɛr wan X kromozom wit di BTK jin we dɔn chenj. dat min se di mama na kכriכg εn di papa gεt XLA (X-Linked Agammaglobulinemia) bak. כltu, uman pikin dεm kin bi di wan dεm we de kכri dis jεnεtik mכtεshכn. Dɔn, ivin if dɛn nɔ gɛt di sik, dɛn kin pas dis jin to dɛn pikin dɛn. If dɛn pikin dɛn de na bɔy pikin, i go mɔs bi se dɛn go gɛt XLA (X-Linked Agammaglobulinemia).

Wetin na di poshubul kɔmplikeshɔn dɛm fɔ XLA (X-Linked Agammaglobulinemia)?

Sɔm pan di prɔblɛm dɛn we kin apin wit XLA (X-Linked Agammaglobulinemia) na:

• Krכnik lכng sik : If yu gεt infεkshכn na di lכng bכku tεm kin pwεl di lכng dεm as tεm de go.
• Infεkshכn we de spre to כda pat dεm na di bכdi : fכ egzampl, infεkshכn kin spre to di bכdi (sepsis) כ di bren (meningitis).
• Dɛn kin tink bak se sɔntɛm sɔm kayn kansa kin bɔku , bɔt dɛn de du mɔ risach bɔt dis.

Aw dɛn kin no se dɛn gɛt XLA (X-Linked Agammaglobulinemia)?

Dɔktɔ kin du bɔku blɔd tɛst fɔ no if yu ɔ yu pikin gɛt XLA (X-Linked Agammaglobulinemia). If dɛn blɔd tɛst ya sho se yu B-sɛl ɔ antibodi dɛn nɔ bɔku, yu dɔktɔ go du yu jenɛtik tɛst . dis de luk fכ chenj dεm na di BTK jin na yu DNA .

Aw dɛn kin trit XLA (X-Linked Agammaglobulinemia)?

I sɔri fɔ no se, no mɛrɛsin nɔ de fɔ XLA (X-Linked Agammaglobulinemia). Bɔt sɔm tritmɛnt dɛn de we kin ɛp fɔ mek dɛn nɔ gɛt siriɔs prɔblɛm dɛn. Di men wan dɛn na:

• Riplesmεnt Immunoglobulin Tεrapi (RIgG) : Dis involv fכ gi antibodi dεm frכm hεlty dכna dεm insay intravenכs (IV). Dɛn kin gi dis tritmɛnt wan tɛm insay di mɔnt . dis de εp fכ kכntrכl di lכw antibodi lεvεl dεm na di bכdi to sכm mak.
• Fɔ trit infɛkshɔn kwik : As dɛn tink se yu ɔ yu pikin gɛt infekshɔn so, yu dɔktɔ go bigin fɔ trit baktriyal infɛkshɔn wit antibayɔtik . I impɔtant fɔ bigin tritmɛnt kwik kwik wan.
• Fɔ avɔyd layf vaysin : Pipul dɛn we gɛt XLA (X-Linked Agammaglobulinemia) nɔ fɔ gɛt layf vaysin . Dɛn vaysin ya kin mek pɔsin gɛt siriɔs sik ɛn kin mek pɔsin in layf de pan denja. Sɔm ɛgzampul dɛn na di MMR vaysin (mizl, mɔmp, rubɛla) , chukchuk - varisɛla vaysin, ɛn ɔral polio vaysin . So, i impɔtant fɔ tɔk to yu dɔktɔ bɔt dis ɛn mek yu no ɔltin bɔt dis.

Wetin pɔsin we gɛt XLA (X-Linked Agammaglobulinemia) fɔ ɛkspɛkt?

Pipul dɛn we gɛt XLA (X-Linked Agammaglobulinemia) go nid fɔ tek mɛrɛsin fɔ di res ɔf dɛn layf . Dis na fɔ ridyus di risk fɔ gɛt sik. Dɛn nid fɔ kɔntinyu fɔ gɛt tayt padi biznɛs wit dɛn dɔktɔ ɛn go fɛn tritmɛnt jɔs afta ɛni sik kam. Yu ɔ yu pikin we gɛt XLA (X-Linked Agammaglobulinemia) kin mis mɔ skul ɛn wok de bikɔs ɔf sik pas di jenɛral pipul dɛn.

Aw lɔŋ pipul dɛm wae gɛt XLA (X-Linked Agammaglobulinemia) kin liv?

Na rili gud tin fɔ no se, wit di divɛlɔpmɛnt fɔ di tritmɛnt we dɛn de yuz , pipul dɛn we gɛt XLA (X-Linked Agammaglobulinemia) na divɛlɔp kɔntri dɛn lɛk Amɛrika de liv te dɛn big . Bɔt na di divɛlop kɔntri dɛn, i stil rili at fɔ no di sik ɛn gɛt tritmɛnt. So, in jεnarכl, dεn kin rεdכks di layf we pikin dεm we gεt XLA (X-Linked Agammaglobulinemia) de liv na dεn kayn kכntri dεm ya. Bɔt na Sri Lanka, gud tritmɛnt dɛn de naw fɔ dɛn kayn sik ya.

Yu tink se dɛn kin mek dɛn nɔ gɛt XLA (X-Linked Agammaglobulinemia)?

If yu de wɔri bɔt XLA (X-Linked Agammaglobulinemia), we min se sɔmbɔdi na yu famili gɛt dis sik, yu kin go to dɔktɔ ɛn gɛt jenɛtik skrinin . Dis kin ɛp yu fɔ no bɔt di jenɛtik kɔndishɔn dɛn we yu kin pas to yu pikin. if yu na di kכriכg fכ di jin mכtεshכn we de mek XLA (X-Linked Agammaglobulinemia), we yu gεt pikin, 50% chans de fכ dat pikin go gεt di mכtεshכn. If da pikin de na bɔy pikin, i kin gɛt XLA (X-Linked Agammaglobulinemia). If yu gɛt XLA (X-Linked Agammaglobulinemia), yu gyal pikin dɛn go bi pɔsin we de kɛr am. Wan pɔsin we de advays yu bɔt yu jɛnɛtiks ɔ di dɔktɔ we ɔda di tɛst go gi yu mɔ advays bɔt dis.

Aw a kin kia fɔ misɛf?

Di bɛst we fɔ kia fɔ yusɛf wit XLA (X-Linked Agammaglobulinemia) na fɔ put yu wɛlbɔdi fɔs . Kip yu dɔktɔ in apɔntinmɛnt dɛn. Lan fɔ no di sayn dɛn we de sho se yu gɛt infekshɔn. Aks yu dɔktɔ wetin fɔ du if yu gɛt sayn dɛn we de sho se yu gɛt infekshɔn.

Ustɛm a fɔ go to dɔktɔ?

I bɛtɛ fɔ tɔk to dɔktɔ pan kes dɛn lɛk dis:

• If yu pikin sik afta i dɔn gɛt layf vaysin (e.g. MMR vaysin, chikinpoks vaysin).
• If yu pikin kin gɛt baktriyal infɛkshɔn bɔku tɛm .
• If yu kin gɛt baktriyal infɛkshɔn bak ɔltɛm (as dis kin bi wan kɔndishɔn lɛk CVID, we kin afɛkt big pipul dɛn bak).

Di dɔktɔ go ebul fɔ tɛl yu if dɛn nid fɔ chɛk dis mɔ ɔ nɔ nid fɔ du am.

Us kwɛstyɔn dɛn a fɔ aks mi dɔktɔ?

I go fayn fɔ mek yu aks kwɛstyɔn dɛn lɛk dis we yu go si yu dɔktɔ:

• Us sayn dɛm fɔ infekshɔn a fɔ luk fɔ?
• Wetin a fɔ du if a tink se mi ɔ mi pikin gɛt infɛkshɔn?
• Wetin na di tritmɛnt dɛn we yu kin gɛt?
• Aw ɔltɛm mi ɔ mi pikin nid tritmɛnt?

Na nɔmal tin fɔ mek yɔŋ pikin dɛn sik bɔku tɛm. Bɔt if yu pikin de gɛt baktriyal infɛkshɔn bɔku tɛm, ɔda tin kin de biɛn am. Tɔk to yu dɔktɔ bɔt ɛnitin we de mɔna yu. Fɔ gɛt diagnosis ɛn tritmɛnt kwik na di bɛst we fɔ gɛt di bɛst rizɔlt.

If yu de liv wit XLA (X-Linked Agammaglobulinemia) – wan jenɛtik kɔndishɔn we yu bɔdi nɔ de mek B-sɛl dɛn fayn fayn wan – fala yu dɔktɔ in instrɔkshɔn dɛn ɛksaktɔli. I rili impɔtant fɔ ebul fɔ no di sayn dɛm we de sho se yu gɛt infekshɔn so dat yu go gɛt tritmɛnt kwik kwik wan.

Di tin dɛn we impɔtant pas ɔl we wi fɔ mɛmba na dis atikul

Okay, so na sɔm tin dɛn ya yu nid fɔ mɛmba frɔm wetin wi dɔn tɔk bɔt bɔt XLA (X-Linked Agammaglobulinemia):

• Wetin na XLA (X-Linked Agammaglobulinemia)?Wan sik we nɔ kin apin so ɔltɛm we kin afɛkt bɔy pikin dɛn mɔ .
• dis na we di b-sεl dεm na di bכdi nכ de divεlכp fayn , we de mek di antibodi dεm dכn dכn εn baktri dεm de infεkshכn bכku tεm .
• Tin dεm lεk tכnsil εn adenoyd kin shrink כ disappear.
• Pan ɔl we no patikyula mɛrɛsin nɔ de fɔ dis, dɛn kin kɔntrol am fayn fayn wan wit layflɔng tritmɛnt (RIgG) ɛn kwik kwik wan wit antibayɔtik tritmɛnt.
• I nɔ fayn fɔ gi layf vaysin to dɛn pipul ya.
• If yu pikin bɔy pikin kin sik bad bad wan bɔku tɛm, i rili impɔtant fɔ go to dɔktɔ wantɛm wantɛm. If yu no di sik kwik kwik wan ɛn trit yu pikin, dat go ɛp yu fɔ liv in layf we nɔmal.

A op se dis infɔmeshɔn go ɛp yu. If yu gɛt ɛni kwɛstyɔn, nɔ shem fɔ tɔk to yu famili dɔktɔ!

` X-linked agammaglobulinemia, XLA, B-sεl dεm, imyun sistεm, jεnεtik sik dεm, sik we kin sik fכs, bכy pikin dεm, antibodi dεm, BTK jin, RIgG tεrapi