Edin no yɛ den kakra sɛ wobɛkenkan, ɛnte saa? "Nsu a ɛma mogya tu". Ɔhaw biara nni ho, momma yɛmfrɛ no LAM kɛkɛ. Eyi yɛ ahurututu mu yare a ɛntaa nsi koraa a ɛka mmea kɛse. Ntease wom sɛ wobɛbɔ hu bere a wote din a ɛte saa no. Nanso mma ɛnhaw wo. Wɔ saa asɛm yi mu no, yɛbɛka nea LAM yɛ, nea enti a ɛba, ne ayaresa ahorow a ɛwɔ hɔ ma no ho asɛm wɔ ɔkwan a ɛyɛ mmerɛw na ɛyɛ adamfofa su so.
Dɛn na LAM kyerɛ ara kwa?
Sɛ yɛbɛka no tiawa a, LAM yɛ bere a ntini a ɛyɛ mmerɛw nkwammoaa a ɛnyɛ ne kwan so a ɛwɔ w’ahurututu mu no fi ase nyin a wuntumi nni so. Te sɛ akisikuru nketenkete. Saa nkwammoaa yi bom yɛ nneɛma nketenkete a ɛte sɛ ahurututu a wɔfrɛ no cysts wɔ w’ahurututu mu. Bere kɔ so no, bere a saa akisikuru yi nyin no, ebetumi asɛe ahurututu mu ntini a ɛte apɔw na ama ayɛ den sɛ wɔbɛhome.
Ɛnyɛ ahurututu mu nkutoo na tebea yi wɔ. Ɛtɔ da bi a, akisikuru nso tumi ba asaabo ne ntini a ɛma mogya tu no mu. Nea ɛde eyi ba titiriw ne awosu mu nkwaadɔm a ɛhwɛ nkwammoaa a ɛwɔ yɛn nipadua mu nyin so no mu nsakrae.
Ɛho hia titiriw sɛ yɛkae sɛ saa yare yi taa ba mmea a wɔadi fi mfe 20 kosi 40, kyerɛ sɛ, wɔ mmerantebere ne mmabaabere ne bratwa ntam. Enti, nnuruyɛfo gye di sɛ estrogen hormone no ne no wɔ biribi yɛ.
LAM ahorow titiriw abien na ɛwɔ hɔ.
Wobetumi akyekyɛ LAM yare no mu ayɛ no ahorow atitiriw abien, na nsonsonoe kakra wɔ abien no ntam.
| LAM type | Nkyerɛkyerɛmu a ɛnyɛ den |
|---|---|
| TSC-LAM na ɛwɔ hɔ | Eyi yɛ LAM bi a ɛba mmea a wɔwɔ awosu mu yare bi a wɔfrɛ no tuberous sclerosis mu. Eyi kyerɛ sɛ ɛba sɛ aduruyɛ mu tebea foforo bi fã. |
| LAM a ɛba bere ne bere mu | Saa ɔkwan yi fi awosu mu nsakrae a ɛba kwa (mutation) a ɛba wɔ nkwa mu. Ɛnyɛ nea wonya fi awo mu. Enti sɛ wowɔ saa yare yi a, ɛho nhia sɛ wohaw wo ho sɛ wo mma de yare no bɛma obi. |
Dɛn ne LAM yare no ho sɛnkyerɛnne?
Esiane sɛ LAM ho sɛnkyerɛnne te sɛ ahurututu mu nyarewa afoforo a ɛtaa ba te sɛ ayamtu nti, ɛtɔ mmere bi a ebetumi agye bere kakra ansa na wɔahu. Eyinom ne sɛnkyerɛnne atitiriw.
- Ɔhome a ɛyɛ den (ɔhome a ɛyɛ den): Ɛwom sɛ mfiase no ɛnyɛ nea wohu de, nanso bere kɔ so no, saa ɔhaw yi betumi ayɛ kɛse. Ebetumi ayɛ nea ɛka ho ne sɛ wobɛte ɔbrɛ nka bere a wonam kwan tiaa mpo, anaasɛ wobɛhome bere a woreforo antweri no.
- Wheezing: Nnyigyei a ɛyɛ brɛoo a efi koko mu ba.
- Koko mu yaw: Ebia koko mu yaw a emu yɛ den mpofirim bɛba.
- Ɔfe: Ɔfe a ɛyɛ kusuu a ɛkɔ so tra hɔ.
- Mogya a wɔfe anaa ‘chyle’: Chyle yɛ nsuo a ɛfiri yɛn aduane mu a ɛfiri yɛn aduane mu. Ne kɔla yɛ nufusu.
Dɛn nti na saa LAM yi ba? Dɛn na ɛde ba?
Sɛnea yɛadi kan aka no, nsakrae a ɛba awosu mu nkwaadɔm mu na ɛde eyi ba. Awosu mu nkwaadɔm ahorow abien na ɛwɔ yɛn nipadua mu a wɔfrɛ no TSC1 ne TSC2 . Eyinom te sɛ ‘awɛmfo’ a wɔhwɛ nkwammoaa a ɛwɔ yɛn nipadua mu no nyin so. Wɔfrɛ wɔn ‘tumor suppressor’ awosu mu nkwaadɔm. Ɛne sɛ, saa awosu mu nkwaadɔm yi adwuma ne sɛ ɛbɛma nkwammoaa no mu apaapae a ɛho nhia na ayɛ akisikuru.
Obi a ɔwɔ LAM no wɔ sintɔ, anaasɛ nkwaadɔm mu nsakrae, wɔ saa awosu mu nkwaadɔm yi biako mu, TSC1 anaa TSC2. Afei saa ‘awɛmfo’ no nyɛ adwuma yiye. Ne saa nti, nkwammoaa a ɛyɛ mmerɛw no fi ase kyekyɛ a wontumi nni so, na ɛyɛ akisikuru ahorow a yɛadi kan aka ho asɛm no.
- Ahurututu mu ntini a ɛyɛ mmerɛw
- Asaabo mu akisikuru (angiomyolipomas) .
- Lymphatic system mu akisikuru
Dɛn ne nsɛnnennen a ebetumi afi yare yi mu aba?
Ɔhaw a ɛtaa ba na ɛyɛ hu sen biara wɔ LAM ho ne ahurututu a abubu (pneumothorax) . Fa no sɛ akisikuru bi a ɛte sɛ ahurututu mu a ɛpae mpofirim, na ɛma mframa tu kɔ ahurututu no ne koko fasu no ntam. Eyi ma ahurututu no hwe ase. Mmea a wɔwɔ LAM no mu bɛboro fã behyia saa tebea yi anyɛ yiye koraa no pɛnkoro wɔ wɔn asetra mu. Mpɛn pii no, wodi kan hu LAM bere a ahurututu a abubu aba no.
Ebia nsɛnnennen afoforo nso wɔ hɔ:
- Nsu a ‘chylous’ a ɛboaboa ano wɔ ahurututu no ho (chylothorax) .
- Nsu afoforo a ɛboaboa ano wɔ ahurututu no ho (pleural effusion) .
- Ntini a ɛma mogya tu no siw kwan
Ɔkwan bɛn so na oduruyɛfo hu LAM?
Sɛ oduruyɛfo no tie wo yare no ho sɛnkyerɛnne na wahwehwɛ wo mu awie no, sɛ oduruyɛfo no susuw sɛ saa yare yi a, ɔbɛhyɛ sɛ wɔnyɛ nhwehwɛmu pii de si so dua.
| Sɔhwɛ | Dɛn na wode eyi yɛ? |
|---|---|
| Ahurututu mu dwumadi ho sɔhwɛ ahorow | Ɛsusuw sɛnea w’ahurututu yɛ adwuma yiye ne mframa dodow a wubetumi akɔ mu na woafi mu. |
| Pulse Oximetry a Wɔde Yɛ Adwuma | Wɔde biribi te sɛ clip bata wo nsateaa ho na wɔhwɛ oxygen dodow a ɛwɔ wo mogya mu. |
| Mfoninitwa (scans) . | CT scan , titiriw HRCT (High-Resolution CT) scan, tumi hu saa akisikuru nketewa a ɛwɔ ahurututu no mu no pefee. |
| VEGF-D mogya mu nhwehwɛmu | VEGF-D yɛ protein a ɛwɔ nipadua no mu. Mpɛn pii no, protein yi dodow dɔɔso wɔ nnipa a wɔwɔ LAM mogya mu. Eyi boa paa wɔ yare no a wobehu mu. |
| Ahurututu mu nipadua mu nhwehwɛmu | Sɛ nhwehwɛmu afoforo ntumi nkyerɛ sɛ wɔahu yare no a, woyi ntini ketewaa bi fi ahurututu no mu na wɔde afiri a wɔde hwɛ nneɛma nketenkete hwehwɛ mu. Wobetumi de ɔkwan bi te sɛ ahurututu mu nhwehwɛmu anaa VATS ayɛ eyi . |
Dɛn ne ayaresa ahorow a wɔde sa LAM?
Nea edi kan koraa no, wonnya nnyaa LAM aduru biara.Nanso mma ɛnhaw wo. Mprempren ayaresa ahorow a etu mpɔn wɔ hɔ a ebetumi aboa ma wɔadi yare yi so, atew yare no ho sɛnkyerɛnne so, na amma ansɛe.
Ayaresa titiriw ne aduru bi a wɔfrɛ no sirolimus . Wɔsan frɛ no rapamycin. Saa aduru yi yɛ adwuma denam ntini mu nkwammoaa a ɛnyɛ ne kwan so a egyae nyin no so. Eyi betumi ama yare no agyina pintinn na ama akisikuru a ɛwɔ asaabo ne ntini a ɛma mogya no mu no so atew.
Bio nso, egyina wo tebea so no, wo duruyɛfo betumi akamfo ayaresa afoforo te sɛ:
- Oxygen ayaresa: Sɛ oxygen dodow a ɛwɔ mogya no mu no sua a.
- Nnuru a ɛma ahurututu mu trɛw a wɔhome: Ɛtew ɔhome a ɛyɛ den so.
- Ahurututu mu ahosiesie: Apɔw-mu-teɛteɛ ne asetra mu afotu a ɛhyɛ ahurututu mu den.
- Ahurututu a wɔde si ananmu: Ɔkwan a etwa to a wobetumi afa so bere a yare no mu yɛ den yiye na wontumi mfa ayaresa afoforo nsiw ano no.
Nea ɛho hia: Sirolimus betumi de nneɛma bɔne aba, te sɛ asaabo a ɛsɛe ne asiane a ɛkɔ soro sɛ obenya ɔyare mmoawa. Enti, wo ne wo duruyɛfo nkasa fa mfaso ne ɔhaw ahorow a ɛwɔ aduru yi so ho ansa na woafi ase.
Nneɛma a ɛsɛ sɛ wuhu bere a wo ne LAM te no
LAM yɛ tebea a ɛtra hɔ kyɛ. Enti, ɛho behia sɛ wo ne w’ahurututu ho oduruyɛfo no yɛ adwuma yiye. Ɛsono sɛnea yare no kɔ so, kyerɛ sɛ, ahoɔhare a yare no kɔ so no, gu ahorow wɔ onipa biara mu. Egyina nneɛma pii so:
- Egyina LAM ko a wowɔ so.
- Wo mfe (wɔ nnipa binom fam no, yare no gyae sɛ ɛyɛ den bere a woagyae brayɛ akyi).
- Sɛnea wo nipadua yɛ n’ade wɔ ayaresa ho.
Nsuro sɛ wɔbɛma w’ahurututu mu. Nnipa dodow no ara (bɛyɛ 64%) renhia ahurututu mu nipadua foforo kosi mfe 20 anaa nea ɛboro saa wɔ bere a wɔahu sɛ wɔwɔ yare no akyi. Esiane ayaresa foforo nti, mprempren nnipa a wɔte LAM nkwa nna akɔ soro kɛse. Nnipa bɛboro 90% da so te ase wɔ mfe 10 akyi wɔ bere a wɔahu sɛ wɔwɔ yare no akyi.
Bere a ɛsɛ sɛ wohwehwɛ aduruyɛ mu afotu
Sɛ wo ne yareɛ te sɛ yei te ase a, ɛho hia paa sɛ wohwɛ wo nipadua so.
| Sɛ wowɔ saa sɛnkyerɛnne ahorow yi a, kɔ wo duruyɛfo nkyɛn: | |
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| Kɔ ETU (Emergency Treatment Unit) ntɛm ara sɛ wowɔ saa sɛnkyerɛnne ahorow yi a: | |
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So LAM yɛ kokoram?
Eyi yɛ ɔhaw ma nnipa pii. Wɔntaa mmu LAM sɛ kokoram . Nanso, ɛwɔ su ahorow bi a ɛte sɛ kokoram. Sɛ nhwɛso no, ebetumi ayɛ te sɛ nea nkwammoaa atrɛw afi nipadua no afã afoforo akɔ ahurututu ne asaabo mu. Nanso, sɛ wɔde afiri a wɔde hwɛ nneɛma nketenkete hwɛ saa nkwammoaa yi a, ɛnte sɛ kokoram nkwammoaa, na mmom ɛte sɛ nkwammoaa a ɛteɛ. Afei nso, wɔntrɛw nkɔ akwaa te sɛ mmerɛbo anaa amemene mu.
Ɛyɛ ade a ɛfata sɛ wobɛte nka sɛ wɔatetew fam afi w’ase bere a wuhu sɛ wowɔ yare a ɛte sɛɛ a ɛbɛtra wo nkwa nna nyinaa mu no. Nanso ɛnyɛ wo nkutoo na wowɔ. Sɛ wo duruyɛfo, w’abusuafo, ne wo nnamfo boa wo a, wubetumi agyina tebea yi ano.
Nkrasɛm a Wɔde Kɔ Fie
- LAM yɛ ahurututu mu yare a ɛntaa nsi koraa a ɛtaa ba mmea so.
- Nsɛnkyerɛnne titiriw ne ɔhome a ɛyɛ den, ɔfe, ne koko mu yaw.
- Eyi fi awosu mu nsakrae bi, na ɛnyɛ wo mfomso.
- Ɛwom sɛ wonnya ayaresa koraa de, nanso wobetumi de nnuru te sɛ sirolimus adi yare no so yiye.
- Kɔ so ne wo ɔhome ho ɔbenfo no nni nkitaho daa.
- Wɔ tebea horow a egye ntɛmpɛ te sɛ koko mu yaw a emu yɛ den anaasɛ ɔhome a ɛyɛ den kɛse mu no, kɔ ETU ntɛm ara.
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