Wetin na dis strenj kayn anemia ɛn kidni prɔblɛm? (Atypical Hemolytic Uremic Syndrome - aHUS) Lɛ wi tɔk!

Yu kin imajin wetin go apin if wi yon difɛns sistɛm, di imyun sistɛm, instead fɔ protɛkt wi frɔm sik, bigin fɔ atak wi yon bɔdi? Dat na wan ɔnaful, bɔt sɔm kayn rare, kɔndishɔn wae wi go tɔk bɔt tide na aHUS, ɔr Atypical Hemolytic Uremic Syndrome . Dis na we wi blɔd vesel dɛn kin pwɛl, smɔl smɔl blɔd kin kam, ɛn di blɔd we de flɔ to impɔtant ɔgan dɛn lɛk di kidni kin stɔp. Bɔku tɛm dis sik kin kɔmɔt frɔm wan jenɛtik muteshɔn .

Dɔktɔ dɛn kin no se yu gɛt aHUS we yu gɛt ɔl dɛn tri tin ya togɛda:

• Microangiopathic hemolytic anemia : Dis min se yu rεd bכdi sεl dεm de pwεl tu kwik, so yu bכdi nכ de ebul fכ mek nyu sεl dεm fכ tek in ples.
• Trombocytopenia : Dis na we di nɔmba fɔ di pletlɛt dɛn we de ɛp di blɔd de go dɔŋ, we de ɛp fɔ mek di blɔd klɔt.
• Akyu kidni injuri : Dis na wan kayn kidni we nɔ de wok fayn, bɔt sɔntɛnde i kin rivɛns.

Dɛn tri tin ya kin apin wan tɛm, ɔr ɔda sik kin mek dɛn wɔs.

Wetin na di difrɛns bitwin rɛgyula HUS ɛn dis aHUS?

Trade, dɛn bin sheb dis sik we dɛn kɔl HUS (Hemolytic Uremic Syndrome) to tu men kayn.

• Tipik HUS (diarrhea-associated HUS): Bɔku tɛm dis kin kam wit dayarɛa. Na wan kayn baktri we dɛn kɔl E. coli kin mek am. Sɔm pipul dɛn kin kɔl am bak STEC-HUS .
• Di aHUS we wi de tɔk bɔt tide (Atypical HUS): Dis nɔto di kayn dayarɛa we dɛn kin si. Bɔrku tɛm, lɛk af pan pipul dɛm wae kin gɛt aHUS, na bikɔs ɔf wan chenj na dɛn jɛnɛtiks, wan chenj na dɛn jɛnɛtiks . Sɔntɛnde, dɔktɔ dɛn kin kɔl dis kɔndishɔn kɔmplimɛnt-mɛdiet trombotik maykroangiopathy (CM-TMA) .

Wetin na di patikyula trig dɛm fɔ aHUS?

Fɔ tɔk am simpul wan, nɔto ɔlman we gɛt jɛnɛtik muteshon go gɛt aHUS. Bɔku tɛm, ɔda wɛlbɔdi prɔblɛm dɛn kin mek i apin, ɔ i kin mek i wɔs . Tink bɔt dɛn tin ya:

• Di tɛm we uman gɛt bɛlɛ
• Kansa
• Difrɛn infɛkshɔn dɛn
• Sɔm mɛrɛsin dɛn, mɔ di wan dɛn we de tek kimotɛrapi , di mɛrɛsin dɛn we de mek di bɔdi nɔ gɛt bɛtɛ blɔd , di wan dɛn we de mek di blɔd tan , di tin dɛn we dɛn kin yuz fɔ mek uman nɔ gɛt bɛlɛ , ɛn sɔm mɛrɛsin dɛn we kin mek pɔsin nɔ gɛt inflammatory .

Wetin na di sayn dɛm fɔ aHUS?

Naw lɛ wi luk us sayn dɛm pɔrsin wae gɛt aHUS kin sho. Yu kin gɛt sɔm pan dɛn tin ya, ɔr yu kin gɛt sɔm kayn sik wae de kam smɔl smɔl.

• Fɔ fil taya ɔltɛm (Fatigue) .
• Pale skin (Pallor) we de mek yu bɔdi wam.
• Nɔs ɔ vɔmit
• Di urine we de kɔmɔt na di bɔdi dɔn go dɔŋ
• Blɔd we de na di urine
• Ay blɔd prɛshɔn (Hypertension) .
• I nɔ izi fɔ blo (Dyspnea) .
• Ɛdima
• Kɔnfyushɔn , we pɔsin nɔ ebul fɔ mɛmba
• Fiva

Bɔrku pipul nɔr kin gɛt ɔl dɛn sik ya wan tɛm. Sɔntɛnde, dɛn kin kam smɔl smɔl, smɔl smɔl. Yu kin fil lɛk se yu dɔn de sɔfa wit sɔntin fɔ sɔm tɛm, bɔt yu nɔ ebul fɔ no wetin i bi. Dɛn nyurolɔjik simptom ya, na di kɔnfyushɔn we a bin dɔn tɔk bɔt, nɔ kin apin to ɔlman, i nɔ kin apin smɔl.

Wetin rili de mek aHUS?

di men tin we de mek aHUS na di chenj na wi DNA (jεnεtik mכtεshכn) , כ di fכmeshכn fכ כtoantibodi dεm we de atak wi כwn sεl dεm agens di ‘kכmpliment protin dεm’ na wi imyun sistεm . Dɛn chenj ya we kin apin na wi jɛnɛtiks kin kɔmɔt frɔm wi mama ɛn papa, ɔ dɛn kin apin bay wilful. dis jεnεtik mכtεshכn dεm de afekt di fכnshכn fכ di protin dεm we dεn kכl ‘kכmpliment fכktכ dεm’ .

Tink bɔt dɛn ‘kɔmplimɛnt factors’ ya as smɔl sojaman dɛn we de ɛp wi imyun sɛl dɛn. lεk fכ sizin it, dεn protin dεm ya de fכn di bad bad jεm dεm (lεk baktri dεm εn vayrus) εn mak dεm so dat כda imyun sεl dεm go ‘it’ dεm. di wan dεm we dεn kin afekt pas כl na aHUS na di kompliment fכktכ dεm H, I, 3, εn B.

fכ egzampl, di wok we kompliment fכktכ H de du na fכ protεkt hεlty sεl dεm na wi bכdi εn fכ mek כda kompliment fכktכ dεm nכ de aktibכt we dεn nכ nid dεm. naw imajin wetin go apin if dis kompliment fכktכ H nכ de wok fayn (dat na if mכtεshכn de na di CFH jin) ? Wi sɛl dɛn nɔ kin gɛt di prɔtɛkshɔn we dɛn nid. Dɔn na di tin dɛn we kin apin:

• Wi yon imyun sɛl dɛn kin mistek atak ɛn pwɛl di sɛl dɛn we de layn insay wi blɔd vesel dɛn (endothelial cells) .
• Plɛtlɛt dɛn kin gɛda rawnd dis damej ɛn mek blɔd klɔt. Dis kin mek di blɔd vesel dɛn blok.
• Dis kin mek blɔd nɔ go na wi ɔgan dɛn, ɛn dis kin mek dɛn pwɛl ɛn dɛn nɔ kin wok fayn igen. Na di kidni dɛn kin afɛkt pas ɔl.
• Dɛn blɔd ya kin pwɛl ɔda blɔd sɛl dɛn we de pas insay dɛn, ɛn dis kin mek pɔsin gɛt wan sik we dɛn kɔl ɛmolaytik anemia .

Aw yu go no if yu gɛt aHUS?

Dɔktɔ go ɔltɛm no se yu gɛt aHUS bay di sayn dɛm we yu gɛt ɛn di rizɔlt fɔ di tɛst dɛm we de chɛk yu blɔd kɔnt ɛn aw yu ɔgan dɛm de wok. Dɛn go du tɛst bak fɔ mek shɔ se yu nɔ gɛt ɔda sik dɛn we de mek yu gɛt di sem kayn sik (lɛk di STEC-HUS we wi bin dɔn tɔk bɔt).

Us tɛst dɛn kin yuz fɔ no if pɔsin gɛt aHUS?

Yu kin nid fɔ du tɛst dɛn lɛk dɛn wan ya:

• Blɔd tɛst: Kɔmplit Blɔd Kɔnt (CBC) , tɛst fɔ di kidni fɔ wok, ɛn ɔda tin dɛn.
• Jεnεtik tεst : Dis de chεk fכ jεnεtik mכtεshכn.
• Stul test : Chɛk fɔ infɛkshɔn lɛk E. coli.
• Kidni bayɔpsi : Fɔ tek smɔl pat pan di kidni fɔ ɛgzamin.
• Imej tɛst : lɛk ɔltra saund ɔ CT skan .

Aw dɛn kin trit aHUS?

Di tritmɛnt fɔ aHUS dipen pan di ɔndalayn kɔz ɛn aw di kɔndishɔn siriɔs. If yu gɛt wan kayn aHUS we nɔ izi fɔ yu, yu dɔktɔ kin wach yu ɛn gi yu sɔpɔt kia , lɛk fɔ gi yu blɔd ɔ fɔ gi yu ay blɔd prɛshɔn mɛrɛsin, if nid de.

Bɔt if yu gɛt siriɔs aHUS, yu kin nid tritmɛnt lɛk dis:

• Therapeutic plasma exchange : Insay dis, dɛn kin pul di plasma pat pan di blɔd ɛn gi nyu plasma.
• Rituximab ɔ ɔda mɛrɛsin dɛn we dɛn kin yuz fɔ trit ɔtoimyun sik dɛn.
• Dayalaysis : Na we fɔ klin di blɔd we di kidni dɛn nɔ de wok fayn.
• Kidni transplant : Dɛn kin jɔs du dis pan di kes dɛm we rili siriɔs.

Fɔ di wan dɛn we gɛt di ‘complement’ gene mutations we wi bin dɔn tɔk bɔt , bɔku tɛm dɔktɔ dɛn kin yuz wan mɛrɛsin we dɛn kɔl eculizumab fɔ trit aHUS. Eculizumab de wok bay we i de blok sɔm kɔmplimɛnt protin dɛm, we de stɔp yu imyun sistɛm fɔ pwɛl yu yon blɔd vesel dɛm.

Ɛni bad tin de we di tritmɛnt kin du?

We yu de yuz Eculizumab , yu kin gɛt smɔl smɔl protɛkshɔn frɔm meningococcal ɛn nyumococcal vaccines. So, if i pɔsibul, yu dɔktɔ kin advays yu fɔ tek dɛn vaysin ya bifo yu bigin fɔ tek Eculizumab .

Wetin di fiuja go gɛt fɔ pɔsin we gɛt aHUS?

If dɛn no dis sik ɛn trit am kwik, dɛn kin ebul fɔ manej di kɔndishɔn lɛk akyu kidni injuri we aHUS kin mek. Yu kin gɛt sɔm tɛm wae yu nɔr gɛt ɛni sik. Bɔt if ɔda tin trigɛt aHUS bak, i kin bi layf pan denja bak.

Trade, lɛk af, ɔ 50%, pan pipul dɛm wae gɛt aHUS kin gɛt ɛnd-stej kidni sik (ESKD) , we min se dɛn kidni nɔ kin wok fayn atɔl.

Bɔt gud nyus de! Nyu stɔdi dɛn sho klia wan se di risk fɔ ESKD dɔn ridyus bad bad wan pan pipul dɛn we gɛt aHUS tank to di tritmɛnt we dɛn gi Eculizumab .

Wetin na di risk fɔ day bikɔs ɔf aHUS?

Siriɔs kes dɛm fɔ aHUS, lɛk we di kidni nɔ wok ɔ we ɔda pat dɛn kin pwɛl, kin rili siriɔs. Insay dɛn kayn siriɔs kes ya, di risk fɔ day de we na lɛk 15%.

Yu tink se dɛn kin ebul fɔ stɔp aHUS?

Bikɔs bɔku tɛm na bikɔs ɔf di chenj dɛn we de apin na di jɛnɛtiks, bɔku tɛm dɛn nɔ kin ebul fɔ stɔp aHUS. Bɔt if yu de pan denja fɔ gɛt siriɔs sik, yu kin ebul fɔ avɔyd sɔm pan di tin dɛn we kin mek yu gɛt dis sik .

Aw a kin kia fɔ misɛf?

If dɛn dɔn no se yu gɛt aHUS, tɔk to yu dɔktɔ bɔt aw fɔ avɔyd tin dɛn we go mek di sik wɔs. E fayn bak fɔ no de sayn dɛm wae de sho se pɔrsin gɛt siriɔs sik.

Ustɛm a fɔ go to dɔktɔ?

If yu dɔn de fil lɛk yu taya ɔr taya fɔ lɔng tɛm – lɛk se yu gɛt sik we nɔr go dɔn – tɔk to yu dɔktɔ. Dɔn bak, tɔk bɔt ɛni ɔda sayn we yu dɔn gɛt dis biɛn tɛm, lɛk we yu chenj di we aw yu de tɔylɛt.

Ustɛm a fɔ go na Imejɛnsi Tritmɛnt Yunit (ETU) ?

If yu gɛt dɛn bad bad sayn ya, go na imejensi rum wantɛm wantɛm:

• Di urine we de kɔmɔt kin ridyus bad bad wan.
• Kɔnfyushɔn ɔ chenj di we aw pɔsin de tink.
• I de swel bad bad wan.
• I nɔ kin izi fɔ yu fɔ blo bad bad wan.

Us kwɛstyɔn dɛn a fɔ aks di dɔktɔ?

I go fayn fɔ aks yu dɔktɔ kwɛstyɔn dɛn lɛk dɛn wan ya:

• Aw siriɔs mi sik de?
• Wetin mek dis bin de wɔs?
• A go ebul fɔ mek tin dɛn lɛk dis nɔ apin tumara bambay?
• Us sayn dɛn a fɔ luk fɔ?
• Ustɛm a fɔ kam si yu bak?

Aw kכmכn dεn kכl dis kכndyushכn aHUS?

aHUS na tin we rili rare.Wan sik we pɔsin kin gɛt we i de mɛn pipul dɛn. I kin afɛkt lɛk wan pan ɛvri fayv ɔndrɛd tawzin pipul dɛn. I kin bɔku smɔl pan big pipul dɛn pas pikin dɛn.

Fɔ dɔn, tin dɛn we wi fɔ mɛmba

We yu no se yu ɔ yu pikin gɛt wan sik we dɛn kɔl jenɛtik, dat kin chenj yu layf. aHUS na siriɔs sik we kin mek blɔd klɔt ɛn pwɛl di ɔgan dɛn. Bɔt di we aw dɛn jɔs dɔn mek wan mɛrɛsin we dɛn kɔl eculizumab dɔn rili ɛp fɔ mek pipul dɛn we gɛt aHUS liv bɛtɛ. If dɛn dɔn no se yu gɛt aHUS, tɔk to yu dɔktɔ bɔt aw fɔ avɔyd di tin dɛn we kin mek yu sik ɛn ridyus yu risk fɔ gɛt siriɔs sik. I kin ɛp yu fɔ ɔndastand wetin yu nid fɔ du fɔ gɛt wɛlbɔdi.

` aHUS, kidni sik, blɔd klot, imyun sistɛm, jɛnɛtik muteshɔn, anemia, Eculizumab