So yɛbɛka LAM, yare bi a ɛka mmea pɔtee ho asɛm? (Nsu mu ntini a ɛma mogya tu) .

So ɛtɔ mmere bi a wote nka sɛ wo home yɛ wo yaw, wobrɛ, anaasɛ wo koko yɛ wo yaw? Yɛtaa susuw eyinom ho sɛ yare bi a ɛtaa ba te sɛ ayamtu ho sɛnkyerɛnne. Nanso, ahurututu mu yare bi wɔ hɔ a ɛntaa nsi koraa a ɛde saa sɛnkyerɛnne ahorow yi ba, titiriw wɔ mmea mu. Wɔfrɛ no Lymphangioleiomyomatosis. Edin no tenten kakra, enti yɛbɛfrɛ no "LAM" tiawa. Sɛ wohu sɛ ɛyɛ den sɛ wobɛbɔ saa din yi a, susuw ho saa: "Lymph-ang-ge-o-ly-o-myo-ma-to-sis". Ɛwom sɛ ɛyɛ den kakra de, nanso ebetumi ayɛ nea ɛho hia paa sɛ wobɛte yare yi ase wɔ nsɛm a ɛnyɛ den mu.

Sɛ yɛbɛka no tiawa a, dɛn ne LAM?

LAM yɛ tebea a ɛntaa nsi a nkwammoaa a ɛnteɛ nyin wɔ w’ahurututu mu, na ɛyɛ ntini nketenkete. Eyi fi awosu mu nsakrae a ɛma ntini a ɛyɛ mmerɛw a ɛwɔ wo nipadua mu no nyin a wuntumi nni so. Eyi betumi asɛe w’ahurututu. Ɛnyɛ ahurututu no nko, na mmom ɛtɔ mmere bi a asaabo ne ntini a ɛma mogya tu no nso tumi nya saa nyin yi.

Wɔtaa hu saa yare yi wɔ mmea a wɔadi fi mfe 20 kosi 40. Ɛne sɛ, wɔ mmerantebere ne mmabaabere akyi ne ansa na wɔagyae brayɛ. Enti, nnuruyɛfo gye di sɛ ɔbea hormone estrogen di dwuma ma yare yi nyin.

LAM yareɛ ahodoɔ titire mmienu na ɛwɔ hɔ.

Wobetumi akyekyɛ LAM yare no mu ayɛ no ahorow atitiriw abien, na nsonsonoe kakra wɔ abien no ntam.

Dɛn ne LAM yare no ho sɛnkyerɛnne?

LAM ho sɛnkyerɛnne te sɛ ahurututu mu nyarewa afoforo te sɛ ayamtu ne ahurututu mu yare no ara pɛ. Ɛtɔ mmere bi a eyi betumi agye bere kakra ansa na wɔahu yare no. Ɛho hia sɛ wohu eyi.

• Ɔhome a ɛyɛ den (dyspnea): Eyi ne sɛnkyerɛnne titiriw ne nea ɛtaa ba. Ɛwom sɛ mfiase no wobetumi ate nka wɔ mmɔdenbɔ brɛoo nkutoo mu de, nanso bere kɔ so no, tebea yi betumi asɛe kɛse.
• Wheezing: Nnyigyei a ɛyɛ abɛn bere a ɔrehome, a ɛte sɛ obi a ɔwɔ ayamtu.
• Koko mu yaw: Ebia wobɛte ɛyaw yi nka titiriw bere a worehome denneennen no.
• Ɔfe: Ebia ɔfe a ɛyɛ nwunu a ɛkɔ so daa bɛba.
• Mogya a wɔfe anaa ‘chyle’: Chyle yɛ nsuo a ɛfiri yɛn aduane mu a ɛfiri yɛn aduane mu. Ne kɔla yɛ nufusu. Sɛ biribi a ɛte sɛɛ si a, ɛbɛyɛ papa sɛ worenkyɛ koraa .

Dɛn nti na saa LAM yi ba? Dɛn na ɛde ba?

Sɛ yɛbɛka no tiawa a, ade titiriw nti a ɛte saa ne nsakrae (nsakrae) a ɛba wɔ awosu mu nkwaadɔm abien a ɛwɔ yɛn nipadua mu a wɔfrɛ no TSC1 ne TSC2 mu no. Saa awosu abien yi te sɛ yɛn nipadua mu awɛmfo. Wɔn adwuma titiriw ne sɛ wobesiw nkwammoaa bi kwan sɛ wɔbɛkyekyɛ na wɔadɔɔso a ɛho nhia a wonni so. Yɛfrɛ saa awosu mu nkwaadɔm a esiw akisikuru ano.

Susuw ho hwɛ sɛ sintɔ bi wɔ awosu mu nkwaadɔm abien a ɛbɔ ho ban yi biako mu a. Nea ɛba ne sɛ nkwammoaa a ɛyɛ mmerɛw no nnya sɛnkyerɛnne a ɛbɛma wɔaka sɛ, "Ɛyɛ, ɛdɔɔso, gyae mpaapaemu." Enti nkwammoaa no fi ase kyekyɛ a wontumi nni so. Nea afi mu aba ne sɛ:

• Cysts ba wɔ ahurututu no mu.
• Akisikuru (angiomyolipomas) ba asaabo no mu.
• Cysts ba wɔ lymphatic system no mu.

Wubetumi anya saa awosu mu abɔde a ɛnteɛ yi afi w’awofo hɔ (a ɛde tuberous sclerosis ba), anaasɛ wubetumi anya nsakrae wɔ saa awosu mu nkwaadɔm yi mu kwa wɔ wo nkwa nna mu a ntease biara nni mu (a ɛde LAM a ɛba bere ne bere mu ba).

Nsɛnnennen ne asiane ahorow a ɛwɔ LAM yare mu

Ɔhaw a ɛtaa ba na ɛyɛ kɛse sen biara a ɛde LAM ba ne ahurututu a abubu . Wɔ aduruyɛ mu no, wɔfrɛ eyi pneumothorax .Mmea a wɔwɔ LAM no mu bɛboro fã behyia saa tebea yi anyɛ yiye koraa no pɛnkoro wɔ wɔn asetra mu. Ɛtɔ mmere bi a, ebetumi asan aba bio. Nokwarem no, wodi kan hu sɛ mmea pii anya LAM bere a wɔanya ahurututu mu akisikuru na wɔde wɔn akɔ ayaresabea akyi.

Nea ɛka ho no, nsɛnnennen afoforo betumi aba.

Ɔkwan bɛn so na wohu LAM yare no?

Sɛ wo tie wo sɛnkyerɛnne ahorow wie a, sɛ wo duruyɛfo susuw sɛ saa yare yi a, ɔbɛhyɛ sɛ wɔnyɛ nhwehwɛmu mpɛn pii de si so dua.

Nhwehwɛmu ahorow a oduruyɛfo no yɛ no

• Ahurututu mu Dwumadi Ho Sɔhwɛ: Eyi hwehwɛ sɛ wobɛhome na woafa afiri bi a wɔde abɔ afiri mu so. Eyi betumi asusuw sɛnea w’ahurututu reyɛ adwuma yiye ne mframa dodow a ebetumi akura mu.
• Pulse Oximetry: Wɔde afiri ketewaa bi a ɛte sɛ nea wɔde kyekyere wo nsateaa ho bata wo nsateaa ho na ɛkyerɛ oxygen dodow a ɛwɔ wo mogya mu. Eyi yɛ sɔhwɛ a ɛyɛ mmerɛw yiye na ɛnyɛ yaw.
• Mfonini a Wɔde Yɛ Mfonini:
• CT Scan (Kɔmputa so Tomography): .Eyi betumi atwa w’ahurututu ho mfonini a ɛkɔ akyiri wɔ 3D mu. Eyi betumi aboa ma woahu pefee sɛ ebia akisikuru bi wɔ hɔ a ɛfa LAM ho pɔtee anaa.
• HRCT scan (High-Resolution Computed Tomography): Eyi yɛ CT scan no fã a ɛkɔ akyiri. Ebetumi atwa ahurututu no fã ketewaa bi ho mfonini a emu da hɔ yiye. Eyi ho hia yiye ma LAM a wobehu.
• VEGF-D mogya mu nhwehwɛmu: VEGF-D yɛ protein a ɛwɔ yɛn mogya mu. Nnipa pii a wɔwɔ LAM no wɔ protein yi dodow a ɛkɔ soro kɛse wɔ wɔn mogya mu sen sɛnea ɛte daa. Enti mogya mu nhwehwɛmu yi boa paa ma wohu yare no.
• Lung Biopsy: Ɛtɔ da bi a, sɛ nhwehwɛmu afoforo nyɛ nokware 100% a, wo duruyɛfo betumi asi gyinae sɛ obegye ntini ketewaa bi afi w’ahurututu mu akɔyɛ nhwehwɛmu. Wobetumi de ɔkwan bi a wɔfrɛ no bronchoscopy (afiri teateaa a wɔde mfoninitwa afiri hyɛ mframa kwan no mu) anaa VATS (afiri a wɔde video boa ahurututu mu oprehyɛn) adi dwuma de ayɛ eyi .

Dɛn ne ayaresa ahorow a wɔde sa LAM yare no?

Nea edi kan koraa no, wonnya nnyaa LAM aduru biara. Nanso mma ɛnhaw wo. Mprempren ayaresa a etu mpɔn yiye wɔ hɔ a ebetumi adi yare no so, atew yare no ho sɛnkyerɛnne so, na ama yare no akɔ so brɛoo.

Ayaresa titiriw ne aduru bi a wɔfrɛ no sirolimus . Wɔsan frɛ no rapamycin. Saa aduru yi yɛ adwuma denam nkwammoaa a enyin a wontumi nni so no nyin a esiw ano no so. Eyi betumi aboa ma wɔadi yare no a ɛbɛkɔ so asɛe no so kɛse. Ebetumi nso aboa ma akisikuru a ɛwɔ asaabo ne ntini mu no so atew na ama yare no ho sɛnkyerɛnne so atew.

Bio nso, ebia wo duruyɛfo bɛkamfo ayaresa afoforo a egyina wo tebea no so akyerɛ wo.

• Oxygen Ayaresa: Sɛ oxygen dodow a ɛwɔ mogya no mu no sua a, wɔde oxygen nsukorabea ahorow ma ma wɔde di dwuma wɔ fie.
• Nnuru a ɛma ahurututu mu trɛw a wɔhome: Nnuru yi a wɔde ma denam afiri a wɔde home so no betumi atew ahome a ɛyɛ den so.
• Ahurututu mu Nsiesiei: Eyi te sɛ apɔw-mu-teɛteɛ nhyehyɛe titiriw bi a wɔyɛ ma ahurututu no. Ɛboa ma w’asetra tu mpɔn denam ahomegye apɔw-mu-teɛteɛ ne nipadua adamfofa apɔw-mu-teɛteɛ so.
• Ahurututu a Wɔde Si Ahurututu Mu:Sɛ yare no mu yɛ den yiye na wontumi mfa ayaresa foforo biara nsiw ano a, ebia ahurututu a wɔde bɛma obi foforo ne ɔkwan a etwa to.

Yare no kɔ so ntɛmntɛm dɛn?

Eyi gu ahorow wɔ onipa biara mu. Sɛnea LAM kɔ so ntɛmntɛm no gyina nneɛma pii so.

• LAM ko a wowɔ: Ebia nnipa a wɔwɔ LAM a ɛba bere ne bere mu no ahurututu mu dwumadi a ɛba fam kakra sen wɔn a wɔwɔ TSC-LAM no.
• Mfe a woadi: Wɔ mmea binom fam no, bere a wɔagyae brayɛ akyi no, yare no kɔ so gyae anaasɛ ɛbrɛ ase bere a estrogen dodow a ɛwɔ nipadua no mu so tew no.
• Wo mmuae wɔ ayaresa ho: Nnipa pii betumi de nnuru te sɛ Sirolimus adi yare no so yiye.

Tebea no ye kɛse mprempren sen bere a atwam no. Sɛ wɔde nnɛyi ayaresa di dwuma a, nnipa a wɔwɔ LAM no mu bɛboro 90% te ase wɔ mfe 10 akyi wɔ bere a wɔahu sɛ wɔwɔ LAM no. Nnipa pii (bɛyɛ 64%) tumi tra ase mfe 20 anaa nea ɛboro saa a enhia sɛ wɔde ahurututu foforo ma.

Bere a ɛsɛ sɛ wokɔ wo duruyɛfo nkyɛn na wokɔ ETU

Sɛ wo ne LAM te a, ɛho hia paa sɛ wobɛhunu berɛ a ɛsɛ sɛ wohwehwɛ aduruyɛ mu afotuo ne berɛ a ɛsɛ sɛ wokɔ Dwumadibea a Ɛhia Ntɛmntɛm (ETU).

Nnipa binom bisa sɛ ebia LAM yɛ kokoram anaa. Wɔntaa mmu LAM sɛ kokoram. Nanso, wɔ akwan bi so, te sɛ ɔkwan a nkwammoaa no fi baabi foforo ba ahurututu mu no, wɔwɔ su ahorow a ɛte sɛ kokoram. Nanso, sɛ wɔde afiri a wɔde hwɛ nneɛma nketenkete hwɛ saa nkwammoaa yi a, ɛnte sɛ kokoram nkwammoaa.

Ɛyɛ ade a ɛfata sɛ wobɛte ahodwiriw ne ehu nka bere a wuhu sɛ wowɔ yare bi a ɛde wo nkwa to asiane mu te sɛ eyi no. Nanso kae sɛ ɛnyɛ wo nko ara. Sɛ wo nnuruyɛfo, w’abusua, ne wo nnamfo boa wo a, wubenya ahoɔden a wode begyina saa asɛnnennen yi ano.