Sɔntɛnde, yu kin fil se yu nɔ de blo fayn, yu kin taya, ɔ yu kin gɛt pen na yu chɛst? Bɔku tɛm wi kin tink bɔt dɛn tin ya as sayn dɛm fɔ wan kɔmɔn sik lɛk asma. Bɔt wan sik de we nɔ kin bɔku na di lɔng we kin mek dɛn gɛt dɛn sik ya, mɔ pan uman dɛn. Dɛn kɔl am Limfangioleiomyomatosis. Di nem long likli, so wi go kol am "LAM" fo shot. If yu si se dis nem at fɔ kɔl, tink bɔt am lɛk dis: "Lymph-ang-ge-o-ly-o-myo-ma-to-sis". Pan ɔl we i kɔmplikt smɔl, i kin rili impɔtant fɔ mek yu ɔndastand dis sik insay simpul wɔd dɛn.
Fɔ tɔk am simpul wan, wetin na LAM?
LAM na wan kayn tin we nɔ kin apin we nɔmal sɛl dɛn kin gro insay yu lɔng dɛn, ɛn mek smɔl smɔl sist dɛn. Dis kin apin bikɔs ɔf di chenj dɛn we de apin na yu jɛnɛtiks we kin mek di smol smol mɔsul sɛl dɛn na yu bɔdi gro we yu nɔ ebul fɔ kɔntrol am. Dis kin pwɛl yu lɔng dɛn. nכto di lכng dεm nכmכ, bכt sכmtεm di kidni dεm εn di limfatik sistεm kin divεlכp dεn growth dεm ya bak.
Dɛn kin no dis sik mɔ pan uman dɛn we ol bitwin 20 ɛn 40. Dat min se afta dɛn dɔn rich di pikin ɛn bifo dɛn dɔn lɛf fɔ gɛt bɛlɛ. So, dɔktɔ dɛn biliv se di uman ɔmon we dɛn kɔl ɛstrojen kin ɛp fɔ mek dis sik bigin.
Tu men kayn LAM sik de.
LAM sik kin sheb to tu men kayn, wit smɔl difrɛns bitwin di tu.
| LAM tayp | Wan simpul ɛksplen |
|---|---|
| TSC-LAM we dɛn kɔl TSC-LAM | Dis gɛt fɔ du wit Tuberous Sclerosis, we na wan sik we de mek pɔsin gɛt jɛnɛtiks. Sɔm uman dɛn we gɛt Tuberous Sclerosis kin gɛt LAM bak. Dis kin bi tin we pɔsin kin gɛt frɔm in mama ɛn papa. |
| LAM we de apin wan wan tɛm | Dis kin bi bikɔs ɔf wan random jɛnɛtik chenj (muteshɔn) we kin apin insay yu layf. I nɔto tin we pɔsin kin gɛt frɔm dɛn gret gret granpa dɛn. So if yu gɛt dis kayn LAM, dɛn nɔ go pas am to yu pikin dɛn. |
De tin wae impɔtant pas ɔl na dat dis sik nɔr kin bɔrku. Statistikin sho se na wan pan ɔl 140,000 uman dɛn nɔmɔ i kin afɛkt. כltu, bitwin 30% εn 80% pan di uman dεm we gεt tuberous sclerosis kin gεt LAM bak.
Wetin na di sayn dɛm fɔ LAM sik?
Di sayn dɛm fɔ LAM rili fiba di wan dɛm fɔ ɔda sik dɛm na di lɔng, lɛk asma ɛn brɔnkayt. Sɔntɛnde, dis kin tek sɔm tɛm fɔ no if yu gɛt dis sik. I impɔtant fɔ no bɔt dis.
- I nɔ kin izi fɔ yu fɔ blo (dyspnea): Dis na di men ɛn kɔmɔn sayn. Pan ɔl we fɔs yu kin fil am we yu tray smɔl, dis sik kin wɔs as tɛm de go.
- Wheezing: Na sawnd we yu de blo we yu de blo, we fiba pɔsin we gɛt asma.
- Chɛst pen: Dɛn kin fil dis pen mɔ we yu de blo dip dip wan.
- Kɔf: I kin gɛt dray kɔf we nɔ de dɔn.
- Kכf bכdi כ ‘chyle’: Chyle na mכkus wata we de kכmכt frכm wi dijestiv sistεm. I gɛt kɔlɔ lɛk milk. If sɔntin lɛk dis apin, i bɛtɛ fɔ lɛ yu nɔ delay atɔl .
Wetin mek dis LAM kin apin? Wetin na di kɔz?
Fɔ tɔk am simpul wan, di men rizin fɔ dis na di chenj dɛn (muteshɔn) we kin apin na tu jin dɛn na wi bɔdi we dɛn kɔl TSC1 ɛn TSC2 . Dɛn tu jin ya tan lɛk di wan dɛn we de gayd wi bɔdi. Dɛn men wok na fɔ stɔp sɔm sɛl dɛn fɔ mek dɛn nɔ sheb ɛn bɔku we dɛn nɔ nid fɔ kɔntrol dɛn. Wi kin kɔl dɛn tumor suppressor jin ya.
Imajin if bad tin bin de na wan pan dɛn tu jin ya we de protɛkt pɔsin. wetin de apin na di smol mכsul sεl dεm nכ de gεt di signal fכ se, "Okay, inof, stכp fכ divayd." So di sɛl dɛn kin bigin fɔ sheb we dɛn nɔ ebul fɔ kɔntrol. Di rizulyt na:
- Sist dεm de fכm na di lכng dεm.
- di tכmכro dεm (angiomyolipomas) de fכm na di kidni dεm.
- di sist dεm de fכm na di limfatik sistεm.
Yu kin gɛt dis fɔlt jin frɔm yu mama ɛn papa (we kin mek yu gɛt tuberous sclerosis), ɔ yu kin gɛt chenj na dɛn jin ya randomly insay yu layf fɔ no rizin (we kin mek yu gɛt LAM wan wan tɛm).
Kɔmplikɛshɔn ɛn risk dɛm fɔ LAM sik
di kכmכn kכmplikεshכn fכ LAM na di lכng we kכlaps , we dεn k כl nyumotכraks .Mɔr pas af pan uman dɛm wae gɛt LAM go gɛt dis sik wan tɛm na dɛn layf. Sɔntɛnde, i kin kam bak. Infakt, bɔku uman dɛn kin fɔs no se dɛn gɛt LAM afta dɛn dɔn gɛt lɛng absɛs ɛn dɛn kin put dɛn na ɔspitul.
Apat frɔm dat, ɔda prɔblɛm dɛn kin apin.
| Kɔmplikɛshɔn | Simpul ɛksplen |
|---|---|
| Lɔng kɔlaps (Nyumotɔraks) . | di sist dεm na di lכng kin bכs, we kin mek di briz kכlekt bitwin di lכng dεm εn di chεst wכl. Dis kin mek i nɔ izi fɔ blo wantɛm wantɛm ɛn i kin mek yu chɛst pen bad bad wan. |
| di akכmyuleshכn fכ di chylous fluid rawnd di lכng dεm (Chylothorax) . | wan milky fluid (chyle) we de kכmכt na di limfatik sistεm de kכlekt rawnd di lכng dεm. Dis kin mek bak i nɔ izi fɔ blo. |
| כda wata we de kכmכt rawnd di lכng dεm (Pleural Effusion) . | di akyumyuleshכn fכ wata dεm we nכto pleura fכluid bitwin di mεmbran dεm we de kכba di lכng dεm. |
Aw dɛn kin no bɔt di sik we dɛn kɔl LAM?
Afta yu dɔn lisin to yu sayn dɛm, if yu dɔktɔ sɔspɛkt dis sik, i go ɔda fɔ du sɔm tɛst fɔ no if yu gɛt am.
Di tɛst dɛn we di dɔktɔ de du
- Lכng Fכnshכn Tεst: Dis min se yu de blo insay εn kכmכt tru wan tiub we kכnekt to mashin. Dis kin mek yu no aw yu lɔng dɛn de wok fayn ɛn aw bɔku briz dɛn kin ol.
- Pulse Oximetry: Na smɔl tin we tan lɛk klip, dɛn kin tay am na yu finga ɛn i kin mɛzhɔ di ɔksijɛn we de na yu blɔd. Dis na tɛst we rili simpul ɛn we nɔ de mek pɔsin fil pen.
- Imej Skan dɛn:
- CT Skan (Kɔmpyut Tomografi): .Dis kin tek ditayla 3D imej dɛm fɔ yu lɔng dɛm. Dis kin ɛp fɔ si klia wan if ɛni sist de we spɛshal fɔ LAM.
- HRCT skan (High-Resolution Computed Tomography): Dis na wan advans we fɔ di CT skan. I kin tek rili klia pikchɔ dɛn fɔ wan rili smɔl pat pan di lɔng dɛn. Dis na impɔtant tin fɔ no bɔt LAM.
- VEGF-D bכdi tεst: VEGF-D na wan protin we de insay wi bכdi. bכku pipul dεm we gεt LAM gεt bכku hכy lεvεl dεm fכ dis protin na dεn blכd pas nכmal. So dis blɔd tɛst kin rili ɛp fɔ no di sik.
- Lɔng Bayopsi: Sɔntɛnde, if ɔda tɛst dɛn nɔ shɔ 100%, yu dɔktɔ kin disayd fɔ tek wan rili smɔl tisu frɔm yu lɔng fɔ tɛst. Dɛn kin du dis bay we dɛn yuz wan tin we dɛn kɔl brɔnkoskɔpi (wan tin tiub we gɛt kamɛra we dɛn put insay di say dɛn we di briz de blo) ɔ VATS (vidio-assisted thoracic surgery) .
Wetin na di tritmɛnt fɔ LAM sik?
Fɔs, no mɛrɛsin nɔ de fɔ LAM yet. Bɔt nɔ wɔri. Naw, tritmɛnt dɛn de we rili fayn we go ebul fɔ kɔntrol di sik, ridyus di sayn dɛm, ɛn slo di sik fɔ go bifo.
Di men tritmɛnt na wan mɛrɛsin we dɛn kɔl sirolimus . Dɛn kin kɔl am bak rapamycin. Dis drɔg de wok bay we i de stɔp di growth fɔ di sɛl dɛn we de gro we dɛn nɔ ebul fɔ kɔntrol. Dis kin ɛp fɔ kɔntrol aw di sik de go bifo mɔ ɛn mɔ. I kin ɛp bak fɔ mek di tכmכro dεm na di kidni εn di limfatik sistεm shrink εn ridyus di simptom dεm.
Impɔtant: Sirolimus kin mek sɔm sayd ɛfɛkt, mɔ di kidni we kin pwɛl ɛn i kin mek i gɛt infɛkshɔn mɔ ɛn mɔ. So, i impɔtant fɔ tɔk bɔt di bɛnifit ɛn di prɔblɛm dɛn we yu gɛt wit yu dɔktɔ bifo yu bigin dis mɛrɛsin.
Apat frɔm dat, yu dɔktɔ kin tɛl yu fɔ du ɔda tritmɛnt dɛn dipen pan yu sik.
- Ɔksijɛn Tɛrapi: If di ɔksijɛn lɛvɛl na di blɔd nɔ bɔku, dɛn kin gi ɔksijɛn tank fɔ yuz na os.
- Inhaled bronchodilators: Dɛn mɛrɛsin ya, we dɛn kin gi tru inhala, kin mek i nɔ izi fɔ blo.
- Pulmonary Rehabilitation: Dis tan lɛk spɛshal ɛksɛsayz program fɔ di lɔng dɛn. I de ɛp fɔ mek yu kwaliti layf bɛtɛ tru ɛksesaiz fɔ blo ɛn ɛksesaiz we fayn fɔ yu bɔdi.
- Transplant fɔ Lɔng:We di sik dɔn rili go bifo ɛn nɔr kin ebul fɔ kɔntrol am wit ɛni ɔda tritmɛnt, fɔ transplant in lɔng kin bi di las tin we dɛn kin du.
Aw kwik di sik kin go bifo?
Dis kin difrɛn frɔm wan pɔsin to ɔda pɔsin. di rεt we LAM de go bifo de dipכnt pan sεvεra fכs tin dεm.
- Di kayn LAM we yu gɛt: Pipul dɛn we gɛt sporadik LAM kin gɛt smɔl smɔl lɔw wok pas di wan dɛn we gɛt TSC-LAM.
- Yu ej: Fɔ sɔm uman dɛn, afta dɛn dɔn menɔpauz, di sik kin stɔp ɔ slo as di ɛstrojen lɛvɛl na di bɔdi de go dɔŋ.
- Yu ansa to tritmɛnt: Bɔrku pipul kin kɔntrol di sik fayn fayn wan wit mɛrɛsin lɛk Sirolimus.
Di tin dɔn bɛtɛ naw pas aw i bin de trade. Wit di mכdan tritmεnt dεm, mכr dan 90% pan di pipul dεm we gεt LAM de alayv 10 ia afta dεn no di sik. Bɔrku pipul (lɛk 64%) kin liv 20 ia ɔr mɔr ɛn nɔr nid fɔ transplant dɛn lɔng.
Ustɛm fɔ si yu dɔktɔ ɛn go na di ETU
We yu de liv wit LAM, i rili impɔtant fɔ no ustɛm fɔ go to dɔktɔ ɛn ustɛm fɔ go na di Imejɛnsi Dipatmɛnt (ETU).
| Chans | Wetin fɔ du |
|---|---|
| Si yu dɔktɔ... | |
| Di kɔmɔn sayn dɛm | If di sayn dɛm lɛk fɔ gɛt prɔblɛm fɔ blo ɛn fɔ kɔf de kɔntinyu ɔ i tan lɛk se i de wɔs. |
| Kwɛstyɔn dɛn bɔt tritmɛnt | If yu gɛt ɛni kwɛstyɔn bɔt di mɛrɛsin we yu de tek ɔ yu de wɔri bɔt di sayd ɛfɛkt dɛn. |
| Go na di Imejɛnsi Tritmɛnt Yunit (ETU) wantɛm wantɛm... | |
| Di sayn dɛn we de sho se pɔsin in lɔng bɔm |
|
Sɔm pipul dɛn kin aks if LAM na kansa. Dɛn nɔ kin tek LAM as kansa. Bɔt pan sɔm we dɛn, lɛk di we aw di sɛl dɛn kɔmɔt ɔdasay to di lɔng, dɛn gɛt tin dɛn we fiba kansa. Bɔt we dɛn luk dɛn sɛl dɛn ya wit maykroskɔp, dɛn nɔ kin tan lɛk kansa sɛl dɛn.
Na nɔmal tin fɔ mek yu fil shɔk ɛn fred we yu kam fɔ no se yu gɛt sik lɛk dis we de mek yu layf de pan denja. Bɔt mɛmba se nɔto yu wangren de. If yu dɔktɔ dɛn, yu famili, ɛn yu padi dɛn go sɔpɔt yu, yu go gɛt trɛnk fɔ bia wit dis prɔblɛm.
Mɛsej we dɛn kin kɛr go na os
- LAM (Lymphangioleiomyomatosis) na wan sik we nɔ kin bɔku na di lɔng we kin afɛkt uman dɛn mɔ.
- I nɔ izi fɔ blo, yu chɛst de pen, ɛn yu de kɔf ɔltɛm na di men sayn dɛm.
- Nyumotɔraks na wan prɔblɛm we kin apin . If yu gɛt bad bad pen na yu chɛst wantɛm wantɛm ɛn i nɔ izi fɔ yu fɔ blo, go na di imejensi rum wantɛm wantɛm.
- Pan ɔl we dis sik nɔ kin wɛl ɔl, mɛrɛsin lɛk sirolimus kin ɛp fɔ kɔntrol di sik fayn fayn wan ɛn alaw yu fɔ liv nɔmal layf.
- Nɔ ɛva fred fɔ tɔk to yu dɔktɔ opin wan bɔt yu sik, tritmɛnt, ɔ ɛnitin we de mɔna yu.
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