Yu ɔ pɔsin we yu sabi kin sik ɔltɛm? Yu kin sik ɔltɛm, ilɛksɛf na kol, flu, yes infɛkshɔn, ɔ yu lɔng infɛkshɔn? Sɔntɛnde, dip rizin de biɛn dɛn tin ya we wi nɔ kin tink bɔt. Tide wi de tɔk bɔt wan rare, potentially serious genetic condition we dɛn kɔl WHIM syndrome.
Fɔ tɔk am simpul wan, wetin na dis WHIM sindrom?
WHIM syndrome na wan jenɛtik disɔda we de afɛkt wi bɔdi in imyun sistɛm, we de mek di bɔdi nɔ ebul fɔ fɛt sik. I nɔ kin apin so ɔltɛm. Dɛn pul di nem WHIM frɔm di fɔs lɛta dɛn pan di 4 men sayn dɛm fɔ dis sik. Nɔto ɔlman kin gɛt ɔl di 4 sayn dɛm, bɔt na dɛn wan ya kin bɔrku.
Lɛ wi si us sayn dɛn ya we dɛn 4 lɛta ya de tɔk bɔt.
| Lɛta | Minin ɛn simpul ɛksplen |
|---|---|
| W - Wɔt dɛn | Wɔt: Bikɔs dɛn sik pipul ya nɔ gɛt bɛtɛ imyun sistɛm, wan vayrɔs we dɛn kɔl Human Papillomavirus (HPV) kin izi fɔ go insay di bɔdi ɛn mek wɔt na di skin ɛn di say dɛn we dɛn kin bɔn. Dis HPV vayrɔs de mek bak di risk fɔ gɛt sɔm kayn kansa. |
| H - Hypogammaglobulinemia we de mek pɔsin gɛt sik | Low Antibody Levels (Hypogammaglobulinemia): Fɔ tɔk am simpul wan, antibodi dɛn tan lɛk sojaman dɛn we de fɛt di jem dɛn we de kam insay wi bɔdi. Dɛn sik pipul ya gɛt lɔw lɛvɛl pan dɛn antibodi ya na dɛn bɔdi. Dis na bikɔs dɛn nɔ gɛt bɛtɛ wayt blɔd sɛl dɛn we dɛn kɔl B sɛl we de mek antibodi. So, dɛn kin gɛt infɛkshɔn mɔ. |
| I - Infεkshכn dεm | Infεkshכn: Bikɔs ɔf di wik imyuniti, dεn pipul ya kin gεt infεkshכn bכku tεm . Ɛspɛshali, infɛkshɔn na di lɔng, yes, ɛn skin kin bɔku. |
| M - Maylɔkatɛksis | Myelokathexis: Dis na di men ɛn spɛshal prɔblɛm fɔ dis sik. Di wayt blɔd sɛl dɛn we de fɛt di smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl smɔl. I tan lɛk sojaman dɛn we de tren na soja kamp. Bɔt pan pipul dɛn we gɛt dis sik, dɛn wayt blɔd sɛl dɛn de nɔ kin ebul fɔ kɔmɔt na di bon mɛro ɛn go na di say we di sik de. Dɛn kin trɔp insay di bon mɛro. So, we infεkshכn apin, di sεl dεm nכ de kam fכ fכt am. |
I impɔtant fɔ no se nɔto ɔl di wan dɛn we gɛt WHIM sindrom kin gɛt ɔl dɛn 4 sik ya. Sɔm kin gɛt sɔm kayn sik wae nɔr kin pasmak, wae ɔda wan kin gɛt bɔrku sik wae kin kam pan pɔrsin in layf.
Aw Xolremdi (mavorixafor) de wok?
As wi bin dɔn tɔk, dɛn sik pipul ya dɛn wayt blɔd sɛl kin stɔp na di bon mɛro, nɔto so? di rizin fכ dis na di mכtεshכn na di jin we dεn kכl `CXCR4` insay wi bכdi. dis `CXCR4` jin de mek wan spεshal protin we dεn kכl `CXCR4` rεsεpכta. Tink bɔt dis riseptɔ as di get-kipa we di wayt blɔd sɛl dɛn de kɔmɔt na di bon mɛro.
pan pipul dεm we gεt WHIM sεndrכm, biכs fכ dis mכtεshכn na di `CXCR4` jin, dat gad nכ de wok fayn fayn wan. I kin kip di get lɔk. So di wayt blɔd sɛl dɛn nɔ kin ebul fɔ kɔmɔt.
wan nyu dכg we dεn kכl Xolremdi (mavorixafor) de wok bay we i de blכk di CXCR4 rεsεpכta we nכ de wok fayn. I tan lɛk se yu gi di pɔsin we de kia fɔ di get fɔ blo ɛn opin di domɔt. Dɔn, di wayt blɔd sɛl dɛn we bin dɔn trɔp na di bon mɛro kin fri fɔ go insay di blɔd ɛn go usay dɛn nid fɔ fɛt di sik. Dis na di fɔs drɔg we dɛn dɔn gri fɔ insay 2024 fɔ trit di ɔndalayn kɔz fɔ WHIM sindrom.
Aw a fɔ yuz dis mɛrɛsin?
Xolremdi na kapsul we yu kin tek wit yu mɔt wan tɛm insay di de. Dɛn fɔ tek am pan ɛmti bɛlɛ . Di bɛst we fɔ tek am na fɔ fast wan nɛt afta yu dɔn it ɛn tek am fɔs na mɔnin. Yu nɔ fɔ it ɔ drink ɛni ɔda tin fɔ at le 30 minit afta yu dɔn tek di mɛrɛsin. Di doz we yu nid go dipen pan yu wet, so na yu dɔktɔ go disayd.
Aw dɛn bin tɛst di sakrifays we dis mɛrɛsin bin gɛt?
Dɛn dɔn du wan klinik stɔdi fɔ tɛst if dis mɛrɛsin sef ɛn if i wok fayn. I involv 31 pasɛnt dɛn we gɛt WHIM sindrom we bin gɛt kɔnfyus CXCR4 jin muteshɔn.
Na lɛk af pan di wan dɛn we tek pat pan di stɔdi na bin yɔŋ pipul dɛn we ol bitwin 12 ɛn 17. Dɛn bin sheb dɛn to tu grup, dɛn bin gi wan grup di mɛrɛsin we dɛn kɔl Xolremdi, ɛn dɛn bin gi di ɔda grup wan plesibo ɛvride.
di risach pipul dεm bin mεnli luk di nכmba fכ wan kayn wayt bכdi sεl we dεn kכl ``nyutrofil`` insay di bכdi. dεn mεzj aw lכng dis nכmba de na wan sכm sef lεvεl (500 sεl/maykrolit).
Di tin dɛn we dɔn apin kin rili ɛnkɔrej wi:
- Di wan dɛn we tek Xolremdi bin gɛt nyutrofil lɛvɛl we bin de na sef lɛvɛl fɔ lɛk 12 awa ɛvride pas di wan dɛn we nɔ tek am.
- Dɔn bak, di nɔmba fɔ di wan dɛn we kin gɛt tritmɛnt ɛvri ia bin fɔdɔm to lɛs dan tu pan di ia , ɛn fɔ di wan dɛn we nɔ bin gɛt tritmɛnt i bin go ɔp to pas 4 pan ia.
Dɛn rizɔlt ya sho se Xolremdi rili ridyus di risk fɔ gɛt infɛkshɔn bay we i de rilis wayt blɔd sɛl dɛn. Bɔt mɛmba se ivin wit dɛn tɛst ya, di we aw yu bɔdi de ansa kin difrɛn.
Udat nɔ fɔ yuz Xolremdi?
Dis mɛrɛsin nɔ fayn fɔ yuz if yu gɛt siriɔs kidni ɔ liva sik, yu gɛt bɛlɛ ɔ yu de gi pikin bɔbi. Dɔn bak, dis mɛrɛsin kin miks wit sɔm ɔda mɛrɛsin dɛn. So, i impɔtant fɔ tɔk to yu dɔktɔ fɔ disayd if Xolremdi fayn fɔ yu.
Wetin na di drɔg intarakshɔn wit ɔda mɛrɛsin dɛm?
Dis na pat we rili impɔtant. Xolremdi kin mek yu gɛt prɔblɛm we yu kam togɛda wit ɔda mɛrɛsin dɛn we yu de tek.
- If yu tek Xolremdi wit sɔm mɛrɛsin dɛn (e.g., `P-gp` ɔ `CYP3A4` inhibitors) i kin mek di ifɛkt dɛn we Xolremdi gɛt bɔku ɛn i kin mek di risk fɔ gɛt sayd ifɛkt dɛn go ɔp.
- sכm כda mεdikeshכn dεm (e.g. strכng `CYP3A4` inducers) kin ridyus di effektiv we Xolremdi de du.
- כlso, Xolremdi kin inkrεs di ifekt dεm fכ sכm כda mεdikeshכn dεm we yu de tek (`CYP2D6`, `CYP3A4` כ `P-gp` sabstεrayt dεm).
- Na patikyula, yu fɔ avɔyd fɔ tek Xolremdi wit ɔda mɛrɛsin dɛn we kin mek denja chenj na di at rit (`QT prolongation`).
Dis list nɔ kɔmplit. So, bifo yu bigin Xolremdi, mek shɔ se yu tɛl yu dɔktɔ bɔt ɔl di mɛrɛsin dɛn we yu de tek - lɛk di mɛrɛsin dɛn we dɛn kin gi yu ɛn di mɛrɛsin dɛn we yu kin bay na kɔt, vaytamɛn dɛn, ɛbul mɛrɛsin dɛn, ɛn Ayurveda mɛrɛsin dɛn.
Aw fɔ kɔntrol di sayd ɛfɛkt dɛn if dɛn apin?
Lɛk ɛni mɛrɛsin, Xolremdi kin gɛt sɔm sayd ɛfɛkt dɛn. Dɛn tin ya na di wan dɛn we bɔku pipul dɛn kin gɛt.
| Sayd ɛfɛkt we pɔsin kin gɛt | Wetin yu go ebul fɔ du |
|---|---|
| Lɔw blɔd pletlɛt kɔnt (`Thrombocytopenia`) . | Dis kin mek i izi fɔ mek yu brus ɔ blɔd. If dis apin, tɛl yu dɔktɔ wantɛm wantɛm. |
| Skin rash we de kɔmɔt na di skin | Yu kin si sayn dɛm fɔ `pityriasis`, we kin mek yu skin dray ɛn skel. Tɛl yu dɔktɔ bɔt dis. |
| Nɔs blɔd de kɔmɔt | If yu gɛt blɔd na yu nos, sidɔm stret ɛn yuz yu big an ɛn indeks finga fɔ pinch di saf pat na yu nos togɛda. If di blɔd nɔ stɔp afta lɛk 20 minit ɔ if i ebi, go to dɔktɔ wantɛm wantɛm ɔ go to di Imejɛnsi Dipatmɛnt (ETU) we de nia yu. |
| Fɔ vɔmit | If yu de vɔmit, drink bɔku wata. Nɔ it bɔku tin wan tɛm, it smɔl smɔl it dɛn we nɔ gɛt wan bɔt. |
| Diziz we pɔsin kin gɛt | Diziz kin mek pɔsin fɔdɔm. So, avɔyd tin dɛn lɛk fɔ tɔn yu ed wantɛm wantɛm ɔ fɔ grap wantɛm wantɛm we yu sidɔm ɔ ledɔm. |
Dis nɔto ɔl di sayd ɛfɛkt dɛn we pɔsin kin gɛt. If yu gɛt ɛni sayn we de mɔna yu ɔ we de mek yu nɔ fil fayn, mek shɔ se yu tɔk to yu dɔktɔ fɔ advays yu.
Wetin yu nid fɔ no we yu de tek dis mɛrɛsin
Dɛn kin tek Xolremdi as "spɛshal mɛrɛsin." Dis min se i nɔto sɔntin we yu kin bay na rɛgyula famasi. Dɛn mɛrɛsin ya na dia dia mɛrɛsin dɛn we dɛn kin yuz fɔ mɛn sik dɛn we nɔ kin bɔku ɛn we nɔ kin izi fɔ du.
If dɛn gi yu dis mɛrɛsin, yu spɛshal pɔsin we de trit yu go ɛksplen aw fɔ gɛt am ɛn us ɔspitul famasi fɔ gɛt am frɔm. Dis prɔses kin kɔmplikt smɔl, bɔt yu dɔktɔ ɛn di wan dɛn we de wok na di ɔspitul go gi yu di advays we yu nid.
Mɛsej we dɛn kin kɛr go na os
- WHIM syndrome na wan sik wae nɔr kin bɔrku pan jɛnɛtiks wae kin afɛkt di imyun sistɛm, wae kin mek pɔrsin gɛt infɛkshɔn ɔltɛm.
- Xolremdi (mavorixafor) na nyu mɛrɛsin we de trit di rut kɔz fɔ dis sik, we de ɛp fɔ pul wayt blɔd sɛl dɛn we dɔn trɔp na di bon mɛro.
- Dis na kapsul we yu fɔ tek ɛvride we yu ɛmti bɛlɛ. Nɔ it fɔ 30 minit afta yu dɔn tek di pil.
- Fɔ avɔyd di denja sayd ɛfɛkt, i impɔtant fɔ tɛl yu dɔktɔ bɔt ɔl di ɔda mɛrɛsin dɛn (inklud vaytamɛn ɛn tradishɔnal ɛvbɔdi) we yu de tek.
- If yu gɛt bad bad sayd ɛfɛkt, lɛk we yu de blɔd pasmak, kɔl yu dɔktɔ wantɛm wantɛm ɔ go to di Imejɛnsi Dipatmɛnt (ETU) we de nia yu.
- Bikɔs dis na spɛshal mɛrɛsin, yu dɔktɔ go gayd yu fɔ no aw fɔ gɛt am.
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