Yu urine dak? Yu taya ɔltɛm? I kin bi PNH (Paroksismal Nɔkchɔnal Ɛmoglobinuria)!

Yu kin gɛt chenj bak na yu urine kɔlɔ we yu wek na mɔnin, we kin tɔn dak rɛd, brawn, ɔ sɔntɛnde blak? Ɔ yu kin fil taya bad bad wan ɛn taya fɔ natin ɔl di de? Dɛn tin ya kin tan lɛk nɔmal tin dɛn we wi nɔ kin pe atɛnshɔn to bɛtɛ na wi layf ɛvride. Bɔt sɔmtɛm, dɛn smɔl smɔl sik ya kin nid fɔ pe atɛnshɔn smɔl, bikɔs dɛn kin de ayd wan wɛl bɔdi prɔblɛm we nɔ kin apin so ɔltɛm bɔt we kin rili siriɔs. Tide wi go tɔk bɔt wan pan dɛn kayn sik ya we gɛt fɔ du wit blɔd, PNH, ɔ `(Paroxysmal Nocturnal Hemoglobinuria)`. Pan ɔl we di nem kin tan lɛk se i kɔmplikt smɔl, lɛ wi ɔndastand am simpul wan.

Wetin na PNH? Lɛ wi ɔndastand am simpul wan

So, wetin na ``Paroxysmal Nocturnal Hemoglobinuria'' ɔ PNH? Fɔ tru, na wan sik we nɔ kin apin na di blɔd we nɔ kin apin so ɔltɛm. If yu luk wetin di nem min:

• "Paroxysmal" min wan wan, we kin apin wan wan.
• "Nocturnal" min se i gɛt fɔ du wit di nɛt.
• "Hemoglobinuria" na di prεsεns fכ wan bכdi protin we dεn kכl ``Hemoglobin`` insay di urine. Dis ``Hemoglobin`` na in de mek wi bכdi luk rεd. So, we dis kכmכt wit di urine, di urine de kכla dak.

Fɔ tɔk am simpul wan, PNH na wan sik we yu imyun sistɛm kin atak ɛn pwɛl yu yon rɛd blɔd sɛl dɛn . dכkta dεn kכl dis ``Hemolysis`` כ di brok dכn fכ di rεd bכdi sεl dεm. Wan pan di men sayn dɛm fɔ dis na dak, rɛd/brawn urine we yu de pis na nɛt ɔ na ali mɔnin.

Bɔt dis dak urine na jɔs wan sayn fɔ PNH. If dɛn nɔ trit dis sik fayn, i kin mek pɔsin gɛt siriɔs sik lɛk anemia , kidni sik we nɔ de dɔn , ɔ blɔd kin klɔt na di blɔd vesel . Laki se naw, fayn fayn mɛrɛsin dɛn de we de mek PNH nɔ pwɛl di blɔd sɛl dɛn.

Udat go ebul fɔ divɛlɔp dis?

PNH na sik we nɔ kin bɔku. Statistikin sho se ɛvri ia, na lɛk 6 pan ɛvri wan milyɔn pipul dɛn kin jɔs gɛt dis sik. Bɔku tɛm, dɛn kin si dis sik pan man ɛn uman we ol bitwin 30 ɛn 40. Bɔt dɛn kin tɔk bak se uman dɛn kin gɛt PNH smɔl pas man dɛn.

כda tin na dat sכmtεm pipul dεm we gεt כda bon mכro sik dεm, fכ egzampl, kכndyushכn lεk `(Aplastic Anemia)` כ `(Myelodysplastic Syndrome)`, kin gεt PNH bak leta.

Wetin na di difrɛns bitwin Ɛmoglobinuria ɛn Ɛmaturia ?

Yu kin fil kɔnfyus smɔl we yu yɛri dɛn tu wɔd ya ( kɔnfyushɔn) .- konfyushɔn, dis na Tamil, a fɔ avɔyd am. Mek a rifrayz: Yu kin kɔnfyus smɔl we yu yɛri dɛn tu wɔd ya.) Yu kin kɔnfyus smɔl we yu yɛri dɛn tu wɔd ya. Bikɔs pan ɔl tu di kes dɛm, wetin kin apin na dat wan luk lɛk blɔd de na di urine.

• Ɛmaturia na we di rɛd blɔd sɛl dɛn de na yu urine.
• εmoglobinuria (dis na wetin wi de si na wi PNH) na di prεsεns fכ wan protin we dεn kכl εmoglobin insay di urine. We di rɛd blɔd sɛl dɛn brok, dis ɛmoglobin de kɔmɔt ɛn ad am to di urine.

Fɔ tɔk am simpul wan, wan de go to di wan ol sɛl dɛn, ɛn di ɔda wan de go to di rɛd tin we de kɔmɔt we di sɛl dɛn brok.

Wetin kin mek pɔsin gɛt PNH? Wetin mek dis kin apin?

di men tin we de mek PNH na difεkt na wan jin na wi bכdi. dis jin dεfεkt de afekt di wok we tu kayn bכdi sεl dεm, rεd bכdi sεl dεm εn pletlεt dεm de wok. Dis kin stat wan chenj wae kin kam wit prɔblɛm wan afta di ɔda, sɔmtɛm kin ivin gɛt tin dɛm wae kin mek pɔrsin in layf de pan denja.

Ɔl dis kin bigin na wi bon mɛro . Yu no, di bon mɛro tan lɛk di blɔd faktri na wi bɔdi. Dis na di say we dɛn kin mek stem sɛl dɛn. Leta dɛn stem sɛl dɛn ya kin bi rɛd blɔd sɛl, wayt blɔd sɛl, ɛn pletlɛt.

insay PNH, wan mכtayshכn כ chenj na wan jin we dεn kכl ``PIGA`` de apin insay wan stεm sεl. dis mכtayt stεm sεl de divayd εn prodyuz mכr sεm sεm sεl dεm we nכ de wok fayn. Dɔn dɛn stem sɛl dɛn ya we nɔ fayn kin mek rɛd blɔd sɛl dɛn ɛn pletlɛt dɛn we nɔ fayn.

Aw dis chenj we di jɛnɛtiks chenj kin afɛkt di rɛd blɔd sɛl dɛn?

Tink bɔt am dis we: wi imyun sistɛm tan lɛk wi kɔntri in difɛns fɔs. wan pan in spεshal fכs dεm dεn kכl am di `Kכmpliment Sistem` . Dɛn wok na fɔ fɛt di jem ɛn infɛkshɔn dɛn we de kam insay di bɔdi. כltu, sכmtεm di protin dεm we de insay dis `(Complement System)` kin bigin fכ atak wi כwn hεlty rεd bכdi sεl dεm bay mistek.

Nɔmal, wɛlbɔdi rɛd blɔd sɛl gɛt wan prɔtin we tan lɛk shild we de protɛkt am fɔ protɛkt am frɔm dɛn kayn atak dɛn ya. di `PIGA` jin we wi bin dכn tכk bכt de εp fכ mek dis protεktiv shild. so, we di `PIGA` jin dεn mכt, i nכ kin mek dis protεktiv shild fayn fayn wan. Dɔn, na dis kin apin:

1. Atak εn Brekdaun: we yu ``Kכmpliment Sistem`` atak dεn rεd bכdi sεl dεm ya we nכ de fכ difεns, dεn sεl dεm de izi fכ brok dכn εn brok dכn. כl di ``hεmoglobin`` we de insay di rεd bכdi sεl dεm – dat na di rεd protin we de kכri כksijεn – de kכmכt insay di bכdi. Dɔktɔ dɛn kin kɔl dis ``Fri Ɛmoglobin`` .

2. If di klin sistɛm nɔ wok:nכmal wan, wi bכdi gεt wan tin we dεn kכl ``Haptoglobin'' we de trap εn pul dis fri εmoglobin. Bɔt na PNH, di nɔmba fɔ di rɛd blɔd sɛl dɛn we dɔn brok so bɔku dat dis tim we de klin nɔ ebul fɔ kip am.

3. Naytric Oxide Deficiency: Di bɔdi de yuz ɔda we fɔ manej dis ɛmoglobin we pasmak, ɛn dat na kemikal we dɛn kɔl nitric oxide. Bɔt dis sɛf kin dɔn kwik kwik wan. We yu bɔdi nɔ gɛt bɛtɛ naytrik ɔksayd, yu kin gɛt siriɔs kramp we kin mek yu fil pen wantɛm wantɛm na yu bɛlɛ mɔsul dɛn, yu it (di tiub we de kɔmɔt na yu trot to yu bɛlɛ), ɛn yu bak mɔsul dɛn.

4. Anemia: Na wan say, we rɛd blɔd sɛl dɛn dɔn pwɛl, na di ɔda say, bɔku prɛshɔn de pan di bon mɛrɔ fɔ mek nyu rɛd blɔd sɛl dɛn. Bɔt we dɛn nɔ ebul fɔ mek dɛn di sem rit we dɛn de pwɛl dɛn, di nɔmba fɔ di rɛd blɔd sɛl dɛn na di bɔdi kin go dɔŋ. Dis na wetin wi kɔl anemia .

5. Kidni damej: Dis εmoglobin we pasmak de damej di kidni dεm bak. Dɛn se pipul dɛn we gɛt PNH kin gɛt siks tɛm mɔ pan di sik we dɛn kin gɛt na dɛn kidni sik .

6. Dak urine: Fɔ dɔn, dis fri ɛmoglobin de afɛkt di urine bak. Bɔrku pipul dɛm wae gɛt PNH kin notis dak urine wae dɛn de pis na nɛt ɔr na ali mɔnin. dis na biכs di kכnsantreshכn כf εmoglobin na di urine de inkrεs, we de tכn di urine dak.

Fɔ tɔk am simpul wan, bikɔs ɔf di difrɛns we de na di `PIGA` jin, di prɔtɛktiv shild fɔ di rɛd blɔd sɛl dɛn de lɔs, ɛn di imyun sistɛm insɛf de pwɛl dɛn, we de mek wan chenj prɔblɛm.

Aw dis chenj na di jɛnɛtiks kin afɛkt di pletlɛt dɛn?

Plɛtlɛt na ɔda impɔtant kayn sɛl na wi blɔd. Dɛn kin ɛp fɔ stɔp blɔd bay we dɛn kin mek blɔd klot. di mכtεshכn na di PIGA jin de afekt di stεm sεl dεm we de mek rεd bכdi sεl dεm εn di stεm sεl dεm we de mek pletlet dεm.

Dis kin mek di pletlɛt dɛn we nɔ fayn kin mek mɔ blɔd klɔt pas aw i kin bi . Dis min se blɔd kin klɔt na say dɛn we dɛn nɔ nid am, insay di blɔd vesel dɛn. dis dεn kכl am `(Trombosis)` (trombosis). Dis kin rili denja, bikɔs if dɛn blɔd ya kin go na di bren, at, ɔ lɔng, dɛn kin mek pɔsin in layf de pan denja.

Wetin na di sayn dɛm fɔ PNH?

Pan ɔl we dɛn pul di nem PNH frɔm wan sayn (dak urine), bɔku pipul dɛn we gɛt PNH kin go to dɔktɔ fɔs bikɔs dɛn kin taya pasmak ɛn nɔ kin taya ɛn dat kin mek i nɔ izi fɔ dɛn fɔ du dɛn tin dɛn we dɛn kin du ɛvride . Dis na di sayn wae de prɛzɛnt pasmak.

Ɔda sayn dɛn kin bi:

• I nɔ izi fɔ blo (`(Dyspnea)`) .
• Kidni prɔblɛm (e.g., yu nɔ de pis, yu leg dɛn de swɛlin) .
• I nɔ kin izi fɔ swɛla it (Dysphagia) .
• Di Spasm dɛn we de na di Ɛsophageal
• Bɛlɛ de pen
• Bak pen
• Man seksual disfכnkshכn (`(Erectile Dysfunction)`)

If yu gɛt wan ɔ mɔ pan dɛn sik ya, mɔ wit di chenj we yu de chenj yu urine kɔlɔ, i go fayn fɔ mek yu go to dɔktɔ fɔ advays.

Aw dɔktɔ dɛn kin no se yu gɛt PNH? (Diagnosis) .

We yu go to dɔktɔ, dɛn go aks yu bɔt yu sik ɛn chɛk yu. Dɔn, if dɛn sɔprayz se yu gɛt PNH, dɛn kin du sɔm tɛst fɔ kɔnfɔm am.

Di men wan dɛn pan dɛn tin ya na:

• Kɔmplit Blɔd Kɔnt (CBC w/diff): Dis de chɛk fɔ ɔda blɔd prɔblɛm dɛn, lɛk anemia ɛn smɔl pletlɛt kɔnt (thrombocytopenia).
• Besik Mεtabolik Panεl (BMP): Dis de chεk fכ di kidni fכ wok εn di simptom dεm fכ krεse sik.
• Urinalysis: Dis kin no tin dεm lεk di prεsεns fכ hεmoglobin na di urine (Hemoglobinuria) εn di εksyכs ayron dεposishכn (Hemosiderosis).
• rεtikulכsayt kכnt: rεtikulכsayt dεm na rεtikulכsayt rεd bכdi sεl dεm we nכ machכ. Dɛn kin kɔnt dɛn tin ya fɔ si if di bon mɛro de mek wɛlbɔdi rɛd blɔd sɛl dɛn.
• Haptoglobin Test: Dis protin de wok fɔ pul dɔti tin dɛn we de kɔmɔt na di rɛd blɔd sɛl dɛn we dɔn brok. If di blɔd sɛl smɔl, dat kin sho se di rɛd blɔd sɛl dɛn dɔn brok pasmak.
• Laktat Dihaydrojenεz (LDH) tεst: LDH na εnzym we de insay rεd bכdi sεl dεm. If dis bɔku na di blɔd, dat de sho se dɛn de pwɛl di rɛd blɔd sɛl dɛn.
• Liva Fכnshכn Tεst: Dis tεst de mכsu di lεvεl כf bilirubin we di rεd bכdi sεl dεm brok dכn.

bay di rizulyt fכ dεn tεst ya, if di dכkta gεt כda saspekshכn bכt PNH, i kin כda fכ spεshal blכd tεst we dεn kכl ``Flow Cytometry.'' Dis na wetin kin kכrekt fכ no di rεd blכd sεl dεm we gεt PNH.

Wetin na di tritmɛnt dɛm fɔ PNH?

Trade, dɛn bin de tek PNH as sik we rili denja ɛn we at fɔ trit. Da tɛm de, di men tritmɛnt na fɔ transfyushɔn blɔd ɔltɛm fɔ trit anemia. Bɔt pipul dɛn we gɛt PNH kin liv fɔ 10 to 22 ia afta dɛn dɔn no se dɛn gɛt di sik. di onli we fכ kכmplit kכl PNH na fכ du wan allojenik stεm sεl transplant . Ɛn dat nɔto sɔntin we ɔlman go ebul fɔ du.

Bɔt, di tin dɔn rili difrɛn naw!

Laki, naw, dɛn gɛt rili ifɛktiv, targeted therapies fɔ PNH. dεn kכl dεn tin ya kompliment inhibito dεm.wetin dεn dכg ya de du na fכ stכp di ``Complement System`` fכ atak εn pwεl di rεd bכdi sεl dεm.

Stɔdi dɔn sho se di wan dɛn we gɛt PNH we gɛt dɛn nyu tritmɛnt ya kin liv nɔmal layf, jɔs lɛk pɔsin we nɔ gɛt PNH. Dis na rili big tin we dɛn dɔn du!

Ɛni bad tin de we di tritmɛnt kin du?

Yes, dis `(Complement Inhibitors)` dεm kin gεt sכm sayd ifekt dεm. Fɔ ɛgzampul, sɔm pipul dɛn kin gɛt tin dɛn lɛk ed we de at ɛn nɔs. Bɔt di gud nyus na dat bɔku pan dɛn sayd ɛfɛkt ya kin stɔp insay sɔm dez afta dɛn bigin fɔ trit am. Yu dɔktɔ go tɛl yu mɔ bɔt dis.

Yu tink se dɛn kin ebul fɔ mek dɛn nɔ gɛt PNH? Yu tink se dɛn kin mɛn am?

Bikɔs PNH na bikɔs ɔf wan jenɛtik muteshɔn, no we nɔ de fɔ mek i nɔ apin.

pan tritmεnt, nyu ``Complement Inhibitor`` dεm kin stכp di damej to rεd blכd sεl dεm εn pletlet dεm we PNH kin mek. Dis min se dɛn kin ebul fɔ kɔntrol di sik. Bɔrku PNH pasɛnt kin nid ɔda tritmɛnt fɔ kɔndishɔn lɛk anemia. as wi bin dכn tכk, pan כl we i posibul fכ ``Stem Sεl Transplant``, na tritmεnt we rili kכmpleks. Wit di mɛrɛsin wae de naw, dɛn kin ebul fɔ kɔntrol di sik fayn fayn wan ɛn pipul dɛn kin liv nɔrmal layf.

Aw yu kin tek kia ɔf yusɛf as pɔsin we gɛt PNH?

Ivin wit tritmɛnt, i rili impɔtant fɔ mek yu tek step fɔ mek blɔd nɔ klɔt if yu gɛt PNH. Fɔ ɛgzampul:

• If pɔsin we gɛt PNH gɛt fɔ du ɔpreshɔn , i kin mek di blɔd klɔt ɛn blɔd pasmak we dɛn de du di ɔpreshɔn. So, dɔktɔ dɛn kin tek tɛm mɔ bɔt dis.
• Bεlε de mek uman dεm we gεt PNH gεt hεlth risk, fכ dεn sεf εn fכ dεn pikin we dεn bכn yet. So, i impɔtant fɔ de ɔnda spɛshal dɔktɔ in sɔpɔt bifo ɛn ɔl di tɛm we yu gɛt bɛlɛ.

Yu dɔktɔ go advays yu bɔt di chenj dɛn we yu nid fɔ mek na yu layf, di tin dɛn we yu nid fɔ pe atɛnshɔn to ( atɛnshɔn , dis na Sanskrit/Malayalam, a fɔ avɔyd am. Mek a rifrɛz: chenj dɛn we yu nid fɔ mek na yu layf, di tin dɛn we yu nid fɔ pe atɛnshɔn to.) chenj dɛn we yu nid fɔ mek na yu layf, di tin dɛn we yu nid fɔ pe atɛnshɔn to. I rili impɔtant fɔ fala da advays de.

Fɔ dɔn, di tin we impɔtant pas ɔl (Take-Home Message) .

PNH, ɔ ``Paroxysmal Nocturnal Hemoglobinuria'', na wan sik we nɔ kin apin so ɔltɛm bɔt we kin rili siriɔs. Arawnd 20 ia bifo dis tɛm, pipul dɛn we gɛt dis sik nɔ bin gɛt bɛtɛ tritmɛnt ɛn dɛn nɔ bin shɔ bɔt dɛn tumara bambay.

Bɔt tide, di tin dɔn rili difrɛn! Tɛnki fɔ nyu, mɔdan tritmɛnt dɛm, pipul dɛm wae gɛt PNH kin kip dɛn blɔd sɛl dɛm, avɔyd siriɔs prɔblɛm dɛm, ɛn liv ful, wɛl bɔdi layf.

If dɛn no se yu gɛt PNH, nɔ panik. Yu dɔktɔ go ɛksplen di tritmɛnt dɛn we go fayn fɔ yu. Pan ɔl we tritmɛnt nɔ kin mɛn PNH ɔltogɛda, i kin ɛp fɔ mek yu nɔ gɛt siriɔs wɛlbɔdi prɔblɛm we di sik kin kam wit ɛn ɛp yu fɔ liv fayn layf. Di tin we impɔtant pas ɔl na fɔ go to dɔktɔ kwik kwik wan if yu gɛt sɔm sayn dɛn ɛn fala di tritmɛnt jɔs lɛk aw dɛn tɛl yu fɔ du am.

` PNH, Paroxysmal Nocturnal Hemoglobinuria, Blɔd Sik, Ɛmoglobinuria, Anemia, Blɔd Klɔt, Kidni Sik