Di nem at fɔ rid smɔl, nɔto so? "Limfangioleiomyomatosis" we dɛn kin du. No prɔblɛm, lɛ wi jɔs kɔl am LAM . Dis na wan sik wae nɔr kin bɔrku bɔrku bɔrku bɔrku bɔrku bɔrku bɔrku bɔrku bɔrku bɔrku uman. I ɔndastand se yu de fred we yu yɛri nem lɛk dat. Bɔt nɔ wɔri. Insay dis atikul, wi go tɔk bɔt wetin na LAM, wetin mek i kin apin, ɛn us tritmɛnt dɛn de fɔ am insay wan rili simpul ɛn fayn we.
Wetin LAM jɔs min?
Fɔ tɔk am simpul wan, LAM na we di abnɔmal smol mכsul sεl dεm we de insay yu lכng dεm bigin fכ gro we yu nכ de kכntrכl. Lɛk smɔl smɔl tumbu dɛn. dis sεl dεm kin kכlכp tכgeda εn fכm sכm sכm bכbul lεk strכkchכ dεm we dεn kכl sist na yu lכng. As tɛm de go, as dɛn tumbu ya de gro, dɛn kin pwɛl di wɛlbɔdi lכng tisu ɛn mek i at fɔ blo.
Dis kכndyushכn nכ de כnli di lכng dεm. sכmtεm, tכmכro kin divεlכp bak na di kidni dεm εn di limfatik sistεm. di men tin we kin mek dis apin na we di jin dεm we de kכntrol di sεl dεm na wi bכdi de gro.
I impɔtant mɔ fɔ mɛmba se dis sik kin kam pan uman dɛn we ol bitwin 20 ɛn 40 ia, dat na bitwin di tɛm we dɛn de bɔn pikin ɛn we dɛn dɔn lɛf fɔ gɛt bɛlɛ. So, dɔktɔ dɛn biliv se di ɔmon we dɛn kɔl ɛstrojen gɛt sɔntin fɔ du wit am.
Tu men kayn LAM dɛn de.
LAM sik kin sheb to tu men kayn, wit smɔl difrɛns bitwin di tu.
| LAM tayp | Simpul diskripshɔn |
|---|---|
| TSC-LAM we dɛn kɔl TSC-LAM | dis na wan kayn LAM we kin apin pan uman dεm we gεt jεnεtik kכndyushכn we dεn kכl tuberous sclerosis. Dis min se i kin apin as pat pan ɔda mɛrɛsin sik. |
| LAM we de apin wan wan tɛm | dis kayn de kכz fכ wan random jεnεtik chenj (mכtεshכn) we de apin we i de liv. I nɔto tin we pɔsin kin gɛt frɔm dɛn gret gret granpa dɛn. So if yu gɛt dis kayn, yu nɔ nid fɔ wɔri bɔt yu pikin dɛn we de pas di sik. |
Wetin na di sayn dɛm fɔ LAM sik?
Bikɔs di sayn dɛm fɔ LAM kin rili fiba ɔda kɔmɔn lɔng sik dɛm lɛk asma, sɔmtɛm i kin tek sɔm tɛm fɔ no if i gɛt am. Dis na di men sayn dɛm.
- I nɔ kin izi fɔ blo (dyspnea): Pan ɔl we yu nɔ kin notis am fɔs, dis diskɔmfɔt kin bɔku as tɛm de go. I kin inklud fɔ fil taya ivin we yu de waka fɔ shɔt tɛm, ɔ fɔ blo fɔ blo we yu de klaym stej.
- Wiz: Na saful sawnd we de kɔmɔt insay di chɛst.
- Chɛst pen: Sɔntɛm, yu chɛst kin pen bad bad wan.
- Kɔf: Na dray kɔf we de kɔntinyu fɔ kɔf.
- Kכf bכdi כ ‘chyle’: Chyle na mכkus wata we de kכmכt frכm wi dijestiv sistεm. I gɛt kɔlɔ lɛk milk.
Wetin mek dis LAM kin apin? Wetin na di kɔz?
As wi bin dɔn tɔk bifo tɛm, dis kin apin bikɔs di jin dɛn chenj. tu kayn jin dεm de na wi bכdi we dεn kכl TSC1 εn TSC2 . dis dεm tan lεk ‘guardians’ we de kכntro di growth fכ di sεl dεm na wi bכdi. dεn kכl dεm ‘tumor suppressor’ jin dεm. dat min se di wok we dεn jin dεm ya de du na fכ mek di sεl dεm nכ divayd we nכ nid εn fכ mek tכmכro.
Pɔsin we gɛt LAM gɛt difrɛns, ɔ muteshon, na wan pan dɛn jin ya, we na TSC1 ɔ TSC2. Den dem ‘guards’ no de wok fayn. Dis kin mek di smol mכsul sεl dεm kin bigin fכ sheb we dεn nכ kin kכntro, we kin mek di kayn tכmכro dεm we wi bin dכn tכk bכt.
- Sist dɛn we de na di lɔng
- Tumɔs dɛn na di kidni (angiomyolipomas) .
- Limfatik sistεm tכmכro dεm
Wetin na de prɔblɛm wae kin kam wit dis sik?
di komplikashכn we kכmכn εn denja fכ LAM na di lכng we kכlaps (nyumothorax) . Imajin wan sist we tan lɛk bɔbul na di lɔng we kin bɔs wantɛm wantɛm, we kin mek di briz lik insay di spes bitwin di lɔng ɛn di chɛst wɔl. Dis kin mek di lɔng kin fɔdɔm. Mɔr pas af pan uman dɛm wae gɛt LAM go gɛt dis sik wan tɛm na dɛn layf. Bɔku tɛm, dɛn kin no LAM fɔs we di lɔng we dɔn fɔdɔm apin.
Ɔda prɔblɛm dɛn kin de:
- di akyumyuleshכn fכ ‘chylous’ fכluid rawnd di lכng dεm (chylothorax) .
- כda wata we de kכmכt rawnd di lכng dεm (pleural effusion) .
- Obstrukshכn fכ di limfatik sistεm
Aw dɔktɔ kin no se i gɛt LAM?
Afta i dɔn lisin to yu sayn dɛm ɛn chɛk yu, if di dɔktɔ sɔspɛkt dis sik, i go ɔda fɔ du sɔm tɛst fɔ no if i gɛt am.
| Tɛst | Wetin yu de du wit dis? |
|---|---|
| Test fɔ di wok we di lɔng dɛn de du | I de mɛzhɔ aw yu lɔng dɛn de wok fayn ɛn aw bɔku briz yu kin tek insay ɛn kɔmɔt. |
| Pulse Ɔksimɛtri | Dɛn kin tay sɔntin lɛk klip na yu finga ɛn chɛk di ɔksijɛn lɛvɛl na yu blɔd. |
| Imajin (skan) . | CT skan , mɔ HRCT (High-Resolution CT) skan kin si klia wan dɛn smɔl smɔl sist dɛn de na di lɔng dɛn. |
| VEGF-D blɔd tɛst | VEGF-D na wan prɔtin. di lεvεl dεm fכ dis protin kin hכy na di bכdi fכ pipul dεm we gεt LAM. Dis kin rili ɛp fɔ no di sik. |
| Lung biopsy we dɛn kin du | If ɔda tɛst dɛn nɔ ebul fɔ kɔnfɔm di diagnosis, dɛn kin tek wan smɔl pat pan di tisu frɔm di lɔng ɛn chɛk am ɔnda maykroskɔp. dis kin bi bay we dεn de yuz wan tεknik lεk brכnkoskopi כ VATS . |
Wetin na di tritmɛnt dɛm fɔ LAM?
Fɔs, no mɛrɛsin nɔ de fɔ LAM yet.Bɔt nɔ wɔri. Naw, fayn tritmɛnt dɛn de we go ɛp fɔ kɔntrol dis sik, fɔ ridyus di sayn dɛm, ɛn fɔ mek i nɔ wɔs.
Di men tritmɛnt na wan mɛrɛsin we dɛn kɔl sirolimus . Dɛn kin kɔl am bak rapamycin. Dis dכg de wok bay we i de stכp di mכsul sεl dεm we nכ de gro. dis kin mek di sik stebul εn shrink di tכmכro dεm na di kidni dεm εn di limfatik sistεm.
Apat frɔm dat, yu dɔktɔ kin tɛl yu fɔ du ɔda tritmɛnt dɛn lɛk:
- Ɔksijɛn tɛrapi: If di ɔksijɛn lɛvɛl na di blɔd nɔ bɔku.
- Inhaled bronchodilators: Ridyus di prɔblɛm fɔ blo.
- Pulmonary rehabilitation: Ɛksesaiz ɛn advays bɔt aw fɔ liv we de mek di lɔng dɛn strɔng.
- Lɔng transplant: Na las tin we dɛn kin du we di sik rili bad ɛn ɔda tritmɛnt dɛn nɔ kin ebul fɔ kɔntrol am.
Impɔtant: Sirolimus kin mek yu gɛt sayd ɛfɛkt, lɛk we yu kidni pwɛl ɛn i kin mek yu gɛt infɛkshɔn mɔ ɛn mɔ. So, tɔk to yu dɔktɔ bɔt di gud ɛn bad tin dɛn we dis mɛrɛsin gɛt bifo yu bigin am.
Tin dɛn fɔ no we yu de liv wit LAM
LAM na wan sik we kin de fɔ lɔng tɛm. So, yu go nid fɔ wok klos wit yu pulmonɔlɔjis. Di we aw di sik de go bifo, dat na di spid we di sik de go bifo, kin difrɛn frɔm wan pɔsin to ɔda pɔsin. I dipen pan bɔku tin dɛn:
- I dipen pan us kayn LAM yu gɛt.
- Yu ej (fɔ sɔm pipul dɛm, di sik kin stɔp fɔ wɔs afta yu dɔn menɔpauz).
- Aw yu bɔdi kin biev we yu gɛt tritmɛnt.
Nɔ fred fɔ transplant yu lɔng. Bɔrku pipul dɛm (lɛk 64%) nɔr go nid fɔ gɛt lɔng transplant te 20 ɔr mɔr ia afta dɛn dɔn no bɔt dɛn sik. Tɛnki fɔ nyu tritmɛnt dɛm, di layf we pipul dɛm we de liv wit LAM kin liv naw dɔn bɔku pasmak. Mɔr pas 90% pan di pipul dɛm stil de alayv 10 ia afta dɛn dɔn no se dɛn gɛt dis sik.
Ustɛm fɔ go to dɔktɔ
We yu de liv wit sik lɛk dis, i rili impɔtant fɔ tek kia ɔf yu bɔdi.
| If yu gɛt dɛn sayn ya, go to yu dɔktɔ: | |
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| Go to di ETU (Emergency Treatment Unit) wantɛm wantɛm if yu gɛt dɛn sik ya: | |
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LAM na kansa?
Dis na prɔblɛm fɔ bɔku pipul dɛn. Dɛn nɔ kin tek LAM as kansa . Bɔt, i gɛt sɔm kwaliti dɛn we tan lɛk kansa. Fɔ ɛgzampul, i kin tan lɛk se sɛl dɛn dɔn skata frɔm ɔda pat dɛn na di bɔdi to di lɔng ɛn kidni dɛn. Bɔt we dɛn si dɛn wit maykroskɔp, dɛn sɛl dɛn ya nɔ kin tan lɛk kansa sɛl dɛn, bɔt dɛn tan lɛk nɔmal sɛl dɛn. Dɔn bak, dɛn nɔ kin skata to ɔgan dɛn lɛk di liva ɔ di bren.
Na nɔmal tin fɔ fil lɛk se dɛn dɔn rip di grɔn ɔnda yu we yu kam fɔ no se yu gɛt sik lɛk dis fɔ yu layf. Bɔt nɔto yu wan de. If yu dɔktɔ, yu fambul, ɛn yu padi dɛn sɔpɔt yu, yu go ebul fɔ bia wit dis prɔblɛm.
Mɛsej we dɛn kin kɛr go na os
- LAM na wan sik we nɔ kin bɔku na di lɔng we kin apin mɔ pan uman dɛn.
- Di men sayn dɛm na wae yu nɔr kin ebul fɔ blo fayn, yu kin kɔf, ɛn yu kin gɛt pen na yu chɛst.
- Dis na bikɔs ɔf wan jenɛtik muteshɔn, nɔto yu fɔlt.
- Pan ɔl we dɛn nɔ gɛt kɔmplit mɛrɛsin, dɛn kin kɔntrol di sik fayn fayn wan wit mɛrɛsin lɛk sirolimus.
- Mek yu gɛt kɔntakt ɔltɛm wit yu spɛshal pɔsin we de ɛp yu fɔ blo.
- If yu gɛt imejensi lɛk we yu de fil bad bad wan na yu chɛst ɔ yu at fɔ blo bad bad wan, go na di ETU wantɛm wantɛm.
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