Yu gɛt dɛn prɔblɛm ya wit yu lɔng? Lɛ wi tɔk bɔt LAM (Lymphangioleiomyomatosis) .

Yu dɔn ɛva yɛri bɔt dis sik wit wan nem we rili lɔng we dɛn kɔl LAM (lymphangioleiomyomatosis)? Yu go si se di nem strenj. Bɔt na sik we nɔ kin apin so ɔltɛm we kin afɛkt di lɔng dɛn. Tide, wi go tɔk bɔt am simpul wan, di we we yu go ɔndastand. Nɔ wɔri, wi go ɛksplen ɔltin.

Wetin rili na LAM (Lymphangioleiomyomatosis)?

Fɔ tɔk am simpul wan, LAM na sik we nɔ kin apin so ɔltɛm we kin mek sist dɛn we ful-ɔp wit wata insay yu lɔng dɛn. Dis kin pwɛl yu lɔng dɛn. Sɔntɛnde i kin mek bak sist na yu kidni ɛn limfatik sistɛm. Imajin wetin go apin if di smɔl smɔl mɔsul sɛl dɛn na yu bɔdi bigin fɔ gro wantɛm wantɛm we yu nɔ ebul fɔ kɔntrol am. Na dat de apin na ya. Na bikɔs ɔf di chenj dɛn we de apin na di jɛnɛtiks.

Dis sik we dɛn kɔl LAM (dɛn kin kɔl am “limf-AN-gee-oh-ly-oh-my-oh-muh-TOH-sis”) , kin afɛkt uman dɛn mɔ . I kin afɛkt uman dɛn we ol bitwin 20 ɛn 40 ia, ɔ uman dɛn we dɔn go tru di tɛm we dɛn de bɔn pikin ɛn we dɔn nia fɔ lɛ dɛn nɔ gɛt bɛlɛ. Bikɔs ɔf dis, dɔktɔ dɛn kin tink se di ɔmon we dɛn kɔl ɛstrojen ɔ di uterus we de de kin gɛt sɔntin fɔ du wit di we aw dɛn tumbu ya de gro.

Wetin na di men kayn LAM?

Tu men kayn LAM de:

1. TSC-LAM: sכm uman dεm gεt wan jεnεtik kכndishכn we dεn kכl ``Tuberous Sclerosis Complex - TSC''. If dɛn divɛlɔp LAM, dɛn kin kɔl am TSC-LAM.

2. Sporadic LAM: Dis kin apin randomly, we min se muteshon de apin na wan jin at random we i de liv. Di impɔtant tin na dat dis kayn Sporadic LAM nɔ de pas to yu pikin dɛn.

Aw kɔmɔn dis sik we dɛn kɔl LAM?

LAM na rili sik we nɔ kin bɔku . Dɛn se na lɛk wan pan ɛvri 140,000 uman dɛn kin gɛt dis sik. כltu, bitwin 30% εn 80% pan di uman dεm we gεt ``Tuberous Sclerosis`` kin gεt LAM.

Wetin na di sayn dɛm fɔ LAM sik?

Pɔsin wae gɛt LAM kin gɛt sɔm kayn sik lɛk:

• I nɔ kin izi fɔ yu fɔ blo (dyspnea) : Sɔntɛnde dis kin go ɔp smɔl smɔl.
• Wiz na sawnd we de kɔmɔt na di chɛst .
• Chɛst pen .
• Kɔf .
• sכmtεm bכdi כ chyle (`(chyle)` - limfatik fכluid frכm di dijestiv sistεm) de kכmכt wit di mכkus .

Nɔ tink se yu gɛt LAM jɔs bikɔs yu gɛt dɛn sik ya, bikɔs dɛn kin bi sayn bak fɔ ɔda sik dɛn na di lɔng. So, i impɔtant fɔ go to dɔktɔ fɔ advays.

Wetin mek dis LAM kin apin? Wetin na di kɔz?

di men tin we de mek LAM na di mכtayshכn dεm na tu jin dεm na wi bכdi we dεn kכl `(TSC1)` εn `(TSC2)`. dis tu jin dεm lεk tumor suppressor jin dεm we de stכp tin dεm lεk kεnsar sεl dεm fכ gro we nכ nid fכ gro na wi bכdi. So, if bad tin de na ɛni wan pan dɛn tu jin ya, dɛn smol mɔsul sɛl dɛn we a bin dɔn tɔk bɔt kin bigin fɔ gro we a nɔ ebul fɔ kɔntrol. Dis dɔn mek dis na:

• di lכng tכmכro dεm (pulmonary lymphangioleiomyomatosis) de divεlכp.
• di tכmכro dεm (angiomyolipomas) de fכm na di kidni dεm.
• di sist dεm de fכm na di limfatik sistεm.

Aw pɔsin kin gɛt LAM?

Tu we de we pɔsin kin gɛt LAM. wan na bay we yu inhεrit di difεkt jin frכm wan mama εn papa we gεt difεkt na di `(TSC1)` כ `(TSC2)` jin. dis inhεritεshכn de rεsult in di kכndishכn `(Tuberous Sclerosis)`, εn wit am de kam di risk fכ divεlכp LAM. Mɔ pas 30% pan di uman dɛn we gɛt `(Tuberous Sclerosis)` gɛt LAM.

di כda we na fכ mכtεshכn na di `(TSC2)` jin de apin insεf, we nכ gεt εni hεridita kכnεkshכn. Wi nɔ no di rayt tin we mek dis apin. di LAM we de divεlכp dis we dεn kכl am Sporadic LAM. insay dis kes, di כda simptom dεm fכ `(Tuberous Sclerosis)` nכ de sho.

Udat de pan ay risk fɔ gɛt LAM?

Pan ɔl we dis kin mek wi sɔprayz, LAM kin apin mɔ pan uman dɛn . Na wan wan man nɔmɔ dɛn dɔn no se gɛt LAM te tide. Na wan man nɔmɔ dɛn dɔn ripɔt se i gɛt sporadik LAM.

Dɔn bak, LAM kin tranga mɔ we uman gɛt bɛlɛ ɛn we i de yuz mɛrɛsin we gɛt ɛstrojen .

Wetin na di pɔsibul kɔmplikeshɔn dɛm fɔ LAM?

di komplikashכn we kכmכn fכ LAM na nyumothorax . Lɛk aw balyɔn de bɔs, di briz kin lik kɔmɔt na di lɔng dɛn ɛn mek di lɔng dɛn fɔdɔm. Mɔ pas af pan di uman dɛn we gɛt LAM go gɛt at le wan ɛpisod fɔ nyumothorax insay dɛn layf. Sɔntɛnde, dis kin apin bak ɛn bak. Bɔku tɛm dɛn kin no LAM afta dis ɛpisod.

Ɔda prɔblɛm dɛn we kin apin na:

• di akyumyuleshכn fכ wata we dεn kכl chyle rawnd di lכng dεm (chylothorax).
• Fluid akyumyuleshכn rawnd di lכng dεm (pleural effusion).
• Obstrukshכn fכ di limfatik sistεm.

Aw dɛn kin no bɔt di sik we dɛn kɔl LAM?

Fɔ no if yu gɛt LAM, dɔktɔ go fɔs chɛk yu ɛn aks yu bɔt di sik dɛn we yu gɛt. Dɔn dɛn go ɔda tɛst ɛn imej tɛst fɔ no dɛn tin ya:

• Si aw yu lɔng dɛn de wok fayn fayn wan.
• Chek di ɔksijɛn lɛvɛl na di blɔd.
• Chek fɔ ɛni chenj na di lɔng dɛn.
• chεk fכ si if i pas nכmal כmכnt dεm fכ sכm ``protein'' dεm na di bכdi.

Bikɔs di sayn dɛm fɔ LAM tan lɛk ɔda kɔmɔn lɔng sik dɛm, sɔntɛnde i kin at fɔ mek dɔktɔ dɛn no if i gɛt am.

Us tɛst dɛn kin yuz fɔ no LAM?

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

• Lכng fכnshכn tεst : dεn ya de chεk aw yu lכng dεm de εp yu fכ brith insay εn kכmכt.
• bכdi כksijεn lεvεl tεst (`(pulse oximetry)`) : Na simpul tεst we involv fכ ataya sכmtin lεk klip pan yu finga.
• Imej tɛst : Dɛn tin ya na CT skan (Computed Tomography scans), MRI (Magnetic Resonance Imaging), ɔ HRCT (High-Resolution Computed Tomography) skan. HRCT skan kin mek yu si klia pikchɔ bɔt ivin wan rili smɔl say na di lɔng, we kin mek i izi fɔ no sɔm sik dɛn we de na di lɔng.
• VEGF-D test : Yu dɔktɔ kin du blɔd tɛst fɔ chɛk if yu gɛt infɛkshɔn, aw yu ɔda ɔgan dɛn de wok fayn, ɛn di lɛvɛl we yu gɛt wan prɔtin we dɛn kɔl VEGF-D (Vascular Endothelial Growth Factor-D). Bɔrku pipul dɛm wae gɛt LAM gɛt ay lɛvɛl pan dis prɔtin, bɔt nɔto ɔlman gɛt am.
• di lכng bayopsi : Dis na fכ tek sכm sכm tisu frכm di lכng εn tεst am na labכtכri. dis sεmpl dεn kin tek am bay brכnkoskopi (na we dεn kin pas tכb wit kεmεra insay di lכng) כ bay vidio-asist tכraks כpεrayshכn (VATS).

Aw dɛn kin trit LAM sik?

I sɔri fɔ no se naw, no mɛrɛsin nɔ de fɔ LAM. Bɔt di mɛrɛsin we dɛn kɔl sirolimus (we dɛn kin kɔl bak rapamycin, brand nem Rapamune®) kin ɛp fɔ mek di sik stebul ɛn mek i nɔ wɔs. Sirolimus kin ɛp fɔ shrink di kidni tumor (angiomyolipomas), ridyus di tumor ɛn fluid we de bɔku na di limfatik sistɛm, ɛn ɛp fɔ ridyus yu simptom dɛm.

As ɔda tritmɛnt dɛn:

• Ɔksijɛn tɛrapi : Fɔ di wan dɛn we gɛt prɔblɛm fɔ blo.
• Inhaled bronchodilators : Dɛn tin ya tan lɛk inhala. Dɛn kin opin di say dɛn we di briz de blo na di lɔng dɛn ɛn mek i izi fɔ blo.
• Pulmonary rehabilitation : Dis na program we de jɔyn ɛksɛsayz, ɛdyukeshɔn, ɛn advays.
• Lɔng transplant : As di las tin fɔ di wan dɛn we dɛn sik dɔn go bifo te i rich wan stej we dɔn rili go bifo.

Lɛ wi lan bak bɔt di sayd ɛfɛkt ɛn kɔmplikeshɔn dɛm wae de kam wit tritmɛnt.

Sɔntɛnde, sirolimus kin mek yu kidni pwɛl bad bad wan ɛn i kin mek yu gɛt siriɔs infɛkshɔn mɔ ɛn mɔ . So, i rili impɔtant fɔ tɔk to yu dɔktɔ bɔt di gud ɛn bad tin dɛn we yu go du fɔ yuz dis mɛrɛsin bifo yu disayd fɔ du sɔntin.

Us kayn fiuja pɔsin we gɛt LAM kin ɛkspɛkt?

LAM kin wɔs as tɛm de go. Yu tritmɛnt plan kin chenj dipen pan us tritmɛnt fayn fɔ yu. Yu go wok wit yu pulmonologist (pulmonologist) fɔ no di bɛst we fɔ wach yu kɔndishɔn. I go de chɛk aw yu lɔng dɛn de wok ɔltɛm. Yu tritmɛnt plan go bi bay:

• yu ej.
• Yu menopausal status (if yu dɔn go tru menopause ɔ nɔ go tru).
• Aw kwik di wok we di lכng de du kin dכn.
• If yu kidni ɔ yu limfatik sistɛm dɔn afɛkt.

Aw fast LAM de skata?

Aw kwik LAM de skata kin difrɛn frɔm wan pɔsin to ɔda pɔsin . Bɔku tin dɛn de we kin afɛkt dis:

• di kayn LAM we yu gεt : pan כl we pipul dεm we gεt `(TSC-LAM)` gεt rili bεtε lכng fכnshכn pas dεn wan dεm we gεt `(Sporadic LAM)`, di rεt we di lכng fכnshכn de dכn bכku pan dεn tu.
• Yu ej : Fɔ sɔm pipul dɛn, LAM kin stɔp fɔ skata afta dɛn dɔn menɔpauz, we di ɛstrojen lɛvɛl na di bɔdi kin go dɔŋ.
• Aw yu kin ansa to tritmɛnt : Di mɛrɛsin we dɛn kɔl Sirolimus kin mek di sik stebul pan bɔku pipul dɛn.

Ustɛm i nid fɔ transplant pɔsin in lɔng?

Bɔrku pipul dɛm wae gɛt LAM (lɛk 64%) kin liv 20 ia ɔr mɔr afta dɛn dɔn no bɔt dɛn sik wae nɔr nid fɔ gɛt lɔng transplant. כltu, biכs di LAM sεl dεm nכ de stat na di lכng, di sist dεm kin stil divεlכp ivin afta dεn transplant di lכng .

Wetin na di layf we pɔsin kin liv wit LAM? (Layf Ɛkspɛkt)

Aw lɔng yu kin liv wit LAM kin dipen pan aw di sik kin tranga ɛn aw i kin skata kwik kwik wan. Bɔt, ɔltogɛda, di tin dɔn bɛtɛ naw pas aw i bin de trade. Mɔr pas 90% pan di pipul dɛm wae gɛt LAM de alayv 10 ia afta dɛn dɔn no se dɛn gɛt dis sik.

Aw a kin kia fɔ misɛf?

Tɔk to yu pulmonologist (pulmonologist) bɔt wetin fɔ ɛkspɛkt ɛn aw fɔ mek plan fɔ liv wit LAM. Si yu dɔktɔ di rayt tɛm, tek yu mɛrɛsin lɛk aw dɛn tɛl yu, ɛn tɛl yu dɔktɔ wantɛm if yu gɛt ɛni nyu sayn, if yu sik de wɔs, ɔ if yu gɛt sayd ɛfɛkt frɔm yu tritmɛnt.

Ustɛm yu fɔ go to dɔktɔ? Ustɛm yu fɔ go na say usay dɛn de kia fɔ pipul dɛn we gɛt prɔblɛm?

If i nɔ izi fɔ yu fɔ blo, yu de kɔf, ɔ ɔda tin dɛn we de mek yu wɔri, ilɛksɛf i de kɔntinyu ɔ i de wɔs, mek shɔ se yu go to dɔktɔ.

Ustɛm fɔ go na di Imejɛnsi Tritmɛnt Yunit (ETU) :

If yu gɛt sayn fɔ se yu lɔng dɔn fɔdɔm (nyumothorax) ɔ ɔda siriɔs sayn dɛn, go na ɔspitul wantɛm wantɛm:

• If yu gɛt prɔblɛm fɔ blo.
• If yu gɛt siriɔs pen na yu chɛst .
• If yu kɔf blɔd .
• If di skin, di lip, ɛn di nel dɛn tɔn blu (`(cyanosis)`) .

Wetin na di impɔtant kwɛstyɔn dɛn we wi fɔ aks di dɔktɔ?

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

• A gɛt `(Sporadik LAM)` ɔ `(TSC-LAM)`?
• Us tritmɛnt opshɔn dɛn a gɛt?
• Aw a fɔ yuz mi mɛrɛsin?
• Us nyu ɔr sik dɛn we de wɔs a fɔ luk fɔ?
• A go nid fɔ transplant mi lɔng?

LAM na kansa?

Dɛn nɔ kin tek LAM as kansa . Bɔt pan sɔm we dɛn i kin biev lɛk kansa we kin kɔmɔt na wan ples na di bɔdi to ɔda ples (mɛtastatik kansa). dis min se di sεl dεm de stat na wan ples εn travul tru di bכdi vεsul dεm εn di limfatik sistεm to di lכng εn kidni dεm. dis kayn we fכ gro sכmtεm dεn kin kכl am benign sεl mεtastas.

Bɔt sayɛnsman dɛn stil nɔ shɔ usay dɛn sɛl dɛn ya bigin. Dɔn bak, i tan lɛk se dɛn tumbu ya nɔ kin go ɔlsay na ɔda pat dɛn lɛk di liva ɔ di bren lɛk kansa. We yu luk dɛn sɛl dɛn ya wit maykroskɔp, dɛn kin tan lɛk nɔmal sɛl dɛn pas kansa sɛl dɛn.

Tɛnki fɔ nyu tritmɛnt ɛn ɔndastandin bɔt LAM mɔ, pipul dɛn de liv lɔng naw we dɛn nɔ transplant dɛn lɔng. Bɔt i kin tek tɛm fɔ ajɔst to dis nyu layf. We yu lan bɔt wan sik we yu go gɛt fɔ yu layf, yu kin fil lɛk se dɛn dɔn kɔt di grɔn ɔnda yu fut. Bɔt yu mɛdikal tim – ɛn yu pasɔnal sɔpɔt tim – kin ɛp yu fɔ tinap bak pan yu fut.

Fɔ dɔn, wetin fɔ mɛmba

LAM kin bi sik we nɔ izi fɔ yu, bɔt nɔto yu wan de. We di tin dɛn we dɛn dɔn lan bɔt mɛrɛsin dɔn go bifo, bɔku we dɛn de fɔ mɛn dis sik. Di tin we impɔtant pas ɔl na fɔ de tink gud wan, fala yu dɔktɔ in advays, ɛn nɔ giv ɔp. Tɔk to yu dɔktɔ ɛn di wan dɛn we yu lɛk bɔt di tin dɛn we de mɔna yu ɛn di tin dɛn we yu de fred. Mɛmba se bɔku pipul dɛn de we go ɛp yu fɔ du dis waka.

` LAM, lכng sik, rεre sik, brith difεlεns, sirolimus, lכng transplant, jεnεtik mכtεshכn