So wowɔ saa ɔhaw ahorow yi wɔ w’ahurututu ho? Momma yɛnka LAM (Lymphangioleiomyomatosis) ho asɛm .

So woate yare yi a edin tenten mmom a wɔfrɛ no LAM (lymphangioleiomyomatosis) ho asɛm pɛn? Ebia wubehu sɛ edin no yɛ nwonwa. Nanso ɛyɛ yare a ɛntaa nsi a ɛka ahurututu. Ɛnnɛ, yɛbɛka ho asɛm tiawa, wɔ ɔkwan a wobɛte ase so. Mma ɛnhaw wo, yɛbɛkyerɛkyerɛ biribiara mu.

Dɛn ankasa ne LAM (Lymphangioleiomyomatosis)?

Sɛ yɛbɛka no tiawa a, LAM yɛ yare a ɛntaa nsi a ɛma akisikuru a nsu ahyɛ mu ma ba w’ahurututu mu. Eyi betumi asɛe w’ahurututu. Ɛtɔ mmere bi nso a ebetumi ama w’asaabo ne w’asaabo mu ntini ayɛ kurukuruwa. Susuw nea ɛbɛba sɛ ntini a ɛyɛ mmerɛw a ɛwɔ wo nipadua mu no fi ase nyin mpofirim a wuntumi nni so. Saa na ɛkɔ so wɔ ha. Ɛyɛ awosu mu nsakrae na ɛde ba.

Saa tebea yi a wɔfrɛ no LAM (a wɔbɔ no “limf-AN-gee-oh-ly-oh-my-oh-muh-TOH-sis”) , ka mmea kɛse . Ɛtaa ka mmea a wɔadi fi mfe 20 kosi 40, anaa mmea a wɔakɔ mmerantebere ne mmabaabere mu na wɔrebɛn bratwa. Esiane eyi nti, nnuruyɛfo susuw sɛ ebia estrogen hormone anaa awotwaa a ɛwɔ hɔ no ne akisikuru yi nyin wɔ biribi yɛ.

Dɛn ne LAM ahorow atitiriw?

LAM ahorow atitiriw abien na ɛwɔ hɔ:

1. TSC-LAM: Mmea binom wɔ awosu mu tebea bi a wɔfrɛ no ``Tuberous Sclerosis Complex - TSC''. Sɛ wɔyɛ LAM a, wɔfrɛ no TSC-LAM.

2. Sporadic LAM: Eyi ba kwa, a ɛkyerɛ sɛ nkwaadɔm mu nsakrae bi ba wɔ awosu mu abɔde bi mu kwa wɔ nkwa mu. Adeɛ a ɛho hia ne sɛ wɔmfa saa Sporadic LAM yi nkɔma wo mma.

Saa yare yi a wɔfrɛ no LAM abu so dɛn?

LAM yɛ yare a ɛntaa nsi koraa ankasa . Wobu akontaa sɛ mmea 140,000 biara mu bɛyɛ biako betumi anya saa yare yi. Nanso, mmea a wɔwɔ ``Tuberous Sclerosis`` no mu 30% kosi 80% betumi anya LAM.

Dɛn ne LAM yare no ho sɛnkyerɛnne?

Obi a ɔwɔ LAM betumi anya yare no ho sɛnkyerɛnne te sɛ:

• Ɔhome a ɛyɛ den (dyspnea) : Ɛtɔ mmere bi a eyi betumi akɔ soro nkakrankakra.
• Ahomegye yɛ nnyigyei a ɛyɛ abɛn a efi koko mu ba .
• Koko mu yaw .
• Ɛwa .
• Ɛtɔ mmere bi a mogya anaa chyle (`(chyle)` - nsu a ɛma mogya tu a efi aduan mu) fi adi ne nsu a ɛyɛ mmerɛw no ba .

Mfa no sɛ wowɔ LAM esiane sɛ wowɔ saa sɛnkyerɛnne ahorow yi ara kwa nti, efisɛ ebetumi ayɛ ahurututu mu nyarewa afoforo nso ho sɛnkyerɛnne. Enti, ɛho hia sɛ wohwehwɛ aduruyɛfo afotu.

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

Nea ɛde LAM ba titiriw ne nkwaadɔm mu nsakrae a ɛba awosu mu nkwaadɔm abien a ɛwɔ yɛn nipadua mu a wɔfrɛ no `(TSC1)` ne `(TSC2)` mu. Saa awosu mu nkwaadɔm abien yi te sɛ awosu mu nkwaadɔm a esiw akisikuru ano a ɛma nneɛma te sɛ kokoram nkwammoaa nnyin wɔ yɛn nipadua mu a ɛho nhia. Enti, sɛ sintɔ bi wɔ awosu mu nkwaadɔm abien yi mu biara mu a, saa ntini a ɛyɛ mmerɛw nkwammoaa a madi kan aka ho asɛm no fi ase nyin a wontumi nni so. Ne saa nti, eyi ne:

• Ahurututu mu akisikuru (ahurututu mu lymphangioleiomyomatosis) sɔre.
• Akisikuru (angiomyolipomas) ba asaabo no mu.
• Cysts ba wɔ lymphatic system no mu.

Ɔkwan bɛn so na obi nya LAM?

Akwan abien na obi betumi afa so anya LAM. Biako ne sɛ wobenya awosu mu abɔde a ɛtɔ sin no afi ɔwofo bi a ɔwɔ sintɔ bi wɔ `(TSC1)` anaa `(TSC2)` awosu mu abɔde mu. Saa agyapade yi de tebea `(Tuberous Sclerosis)` ba, na asiane a ɛne sɛ wobenya LAM no ka ho. Mmea a wɔwɔ `(Tuberous Sclerosis)` no mu bɛboro 30% wɔ LAM.

Ɔkwan foforo ne sɛ nkwaadɔm mu nsakrae a ɛba `(TSC2)` awosu mu abɔde mu no ba ara kwa, a awosu mu abusuabɔ biara nni mu. Wonnim nea ɛde eyi ba ankasa. LAM a ɛnyini saa kwan yi so no, wɔfrɛ no Sporadic LAM. Wɔ saa tebea yi mu no, `(Tuberous Sclerosis)` ho sɛnkyerɛnne afoforo no mpue.

Hena na ɔwɔ asiane kɛse mu sɛ obenya LAM?

Ɛwom sɛ eyi yɛ nwonwa de, nanso LAM taa ba mmea so . Mmarima kakraa bi pɛ na wɔahu sɛ wɔwɔ LAM de besi nnɛ. Wɔabɔ amanneɛ sɛ ɔbarima biako pɛ na ɔwɔ LAM a ɛba bere ne bere mu.

Afei nso, LAM betumi ayɛ kɛse bere a wɔyem ne bere a wɔde nnuru a estrogen wom redi dwuma no .

Dɛn ne nsɛnnennen a ebetumi afi LAM mu aba?

LAM ho haw a ɛtaa ba ne ahurututu mu yare . Te sɛ balloon a ɛpae no, mframa fi ahurututu no mu na ɛma ahurututu no bubu. Anyɛ yiye koraa no, mmea a wɔwɔ LAM no mu bɛboro fã benya ahurututu mu yare pɛnkoro wɔ wɔn nkwa nna mu. Ɛtɔ mmere bi a, eyi betumi asi mpɛn pii. Wɔtaa hu LAM wɔ saa adeyɛ yi akyi.

Nsɛnnennen afoforo a ɛde ba no bi ne:

• Nsu a wɔfrɛ no chyle a ɛboaboa ano twa ahurututu no ho hyia (chylothorax).
• Nsu a ɛboaboa ano wɔ ahurututu no ho (pleural effusion).
• Ntini a ɛma mogya tu no siw kwan.

Ɔkwan bɛn so na wohu LAM yare no?

Sɛnea ɛbɛyɛ na wɔahu LAM no, oduruyɛfo bi bedi kan ahwehwɛ wo mu na wabisa wo sɛnkyerɛnne ahorow ho asɛm. Afei wɔbɛhyɛ sɛ wɔnyɛ sɔhwɛ ne mfoniniyɛ mu sɔhwɛ ahorow de ahu nea edidi so yi:

• Hwɛ sɛnea w’ahurututu reyɛ adwuma yiye.
• Hwɛ oxygen dodow a ɛwɔ mogya no mu.
• Hwɛ sɛ nsakrae biara aba ahurututu no mu anaa.
• Hwɛ sɛ ``protein'' bi wɔ mogya no mu sen dodow a ɛfata anaa.

Esiane sɛ LAM ho sɛnkyerɛnne te sɛ ahurututu mu nyarewa afoforo a ɛtaa ba nti, ɛtɔ mmere bi a ebetumi ayɛ den ama nnuruyɛfo sɛ wobehu.

Nhwehwɛmu bɛn na wɔde hu LAM?

Ebia ɛho behia sɛ woyɛ sɔhwɛ ahorow te sɛ eyinom:

• Ahurututu mu dwumadi ho nhwehwɛmu : Eyinom hwɛ sɛnea w’ahurututu boa wo ma wohome na wufi mu.
• Mogya mu oxygen dodow nhwehwɛmu (`(pulse oximetry)`) : Sɔhwɛ a ɛnyɛ den a ɛfa biribi te sɛ clip a wode bɛbata wo nsateaa ho.
• Mfoninitwa nhwehwɛmu : Eyinom bi ne CT mfonini (Computed Tomography scans), MRI (Magnetic Resonance Imaging), anaa HRCT (High-Resolution Computed Tomography) mfonini. HRCT scan betumi ama ahurututu no fã ketewaa bi mpo ho mfonini a emu da hɔ, na ama ayɛ mmerɛw sɛ wobehu ahurututu mu nyarewa bi.
• VEGF-D nhwehwɛmu : Ebia wo duruyɛfo bɛyɛ mogya mu nhwehwɛmu de ahwɛ sɛ ɔyare mmoawa bi wɔ wo mu, sɛnea w’akwaa afoforo reyɛ adwuma yiye, ne protein bi a wɔfrɛ no VEGF-D (Vascular Endothelial Growth Factor-D) dodow a wowɔ. Nnipa pii a wɔwɔ LAM wɔ protein yi pii, nanso ɛnyɛ obiara na ɔwɔ saa.
• Lung biopsy : Eyi hwehwɛ sɛ woyi ntini ketewaa bi fi ahurututu no mu na wɔsɔ hwɛ wɔ aduruyɛdan mu. Wobetumi afa ahurututu mu nhwehwɛmu (ɔkwan a wɔfa so de afiri a wɔde mfoninitwa afiri wom kɔ ahurututu no mu) anaasɛ video-assisted thoracic surgery (VATS) so afa saa nhwɛsode yi.

Ɔkwan bɛn so na wɔsa LAM yare no?

Nea ɛyɛ awerɛhow no, mprempren wonni aduru biara a wɔde sa LAM. Nanso, aduru sirolimus (a wɔsan frɛ no rapamycin, edin a wɔde frɛ no Rapamune®) betumi aboa ma yare no agyina na asiw ano. Sirolimus betumi aboa ma asaabo mu akisikuru (angiomyolipomas) so atew, atew akisikuru ne nsu a ɛboaboa ano wɔ ntini mu no so, na aboa ma wo sɛnkyerɛnne ahorow no so atew.

Sɛ́ ayaresa afoforo no:

• Oxygen therapy : Wɔde ma wɔn a ɛyɛ den ma wɔn sɛ wɔbɛhome.
• Nnuru a ɛma ahurututu mu trɛw a wɔhome : Eyinom te sɛ nea wɔde home. Wobue mframa kwan a ɛwɔ ahurututu no mu na ɛma ɛyɛ mmerɛw sɛ wɔbɛhome.
• Ahurututu mu ahosiesie : Eyi yɛ nhyehyɛe a ɛka apɔw-mu-teɛteɛ, nhomasua, ne afotu bom.
• Ahurututu a wɔde si ananmu : Sɛ́ ɔkwan a etwa to ma wɔn a wɔn yare no akɔ anim akodu baabi a ɛkɔ akyiri yiye no.

Momma yensua nsunsuanso bɔne ne nsɛnnennen a ɛwɔ ayaresa mu nso ho ade.

Ɛtɔ mmere bi a sirolimus betumi ama asaabo asɛe kɛse na ama asiane a ɛwɔ hɔ sɛ wobenya nyarewa a emu yɛ den no yɛ kɛse . Enti, ɛho hia yiye sɛ wo ne wo duruyɛfo susuw mfaso ne ɔhaw ahorow a ɛwɔ aduru yi a wode bedi dwuma mu ho ansa na woasi gyinae.

Daakye bɛn na obi a ɔwɔ LAM betumi ahwɛ kwan?

LAM betumi asɛe kɛse bere a bere kɔ so no. Ebia wo ayaresa nhyehyɛe no bɛsakra agyina ayaresa a ɛfata wo so. Wo ne wo ahurututu ho ɔyaresafo (ahurututu ho oduruyɛfo) bɛyɛ adwuma de ahu ɔkwan a eye sen biara a wobɛfa so ahwɛ wo tebea no. Ɔbɛhwɛ sɛnea w’ahurututu yɛ adwuma daa. Wo ayaresa nhyehyɛe no begyina:

• wo mfe a woadi.
• Wo bratwa tebea (sɛ́ ebia woafa brayɛ mu anaasɛ woankɔ mu).
• Sɛnea ahurututu mu dwumadi so tew ntɛmntɛm.
• Sɛ́ ebia w’asaabo anaa w’asaabo nhyehyɛe no aka wo.

LAM retrɛw ntɛmntɛm dɛn?

Sɛnea LAM trɛw ntɛmntɛm no betumi ayɛ soronko wɔ onipa biara ho . Nneɛma pii wɔ hɔ a ebetumi aka eyi:

• LAM ko a wowɔ : Ɛwom sɛ nnipa a wɔwɔ `(TSC-LAM)` no ahurututu mu dwumadi ye sen wɔn a wɔwɔ `(Sporadic LAM)` de, nanso sɛnea ahurututu mu dwumadi so tew no yɛ pɛ kɛse wɔ abien no nyinaa mu.
• Wo mfe : Wɔ nnipa binom fam no, LAM gyae trɛw bere a wɔagyae brayɛ akyi, bere a estrogen dodow a ɛwɔ nipadua no mu so tew no.
• Sɛnea woyɛ w’ade wɔ ayaresa ho : Aduru Sirolimus betumi ama yare no agyina wɔ nnipa pii mu.

Bere bɛn na ɛho hia sɛ wɔde ahurututu ma obi foforo?

Nnipa pii a wɔwɔ LAM (bɛyɛ 64%) betumi atra ase mfe 20 anaa nea ɛboro saa wɔ bere a wɔahu sɛ wɔwɔ yare no akyi a enhia sɛ wɔde ahurututu foforo ma. Nanso esiane sɛ LAM nkwammoaa mfi ase wɔ ahurututu no mu nti, akisikuru no da so ara tumi nyin bere a wɔde ahurututu foforo ahyɛ obi foforo mpo akyi .

Dɛn ne nkwa nna a wɔhwɛ kwan wɔ LAM ho? (Nkwa Nkwa Ho Nkwa) .

Bere tenten a wubetumi ne LAM atra ase no gyina sɛnea yare no mu yɛ den ne sɛnea ɛtrɛw ntɛmntɛm so. Nanso, ne nyinaa mu no, tebea no ye kɛse mprempren sen sɛnea na ɛte kan no. Nnipa a wɔwɔ LAM bɛboro 90% te ase wɔ mfeɛ 10 akyi wɔ berɛ a wɔahu sɛ wɔanya bi no.

Mɛyɛ dɛn ahwɛ me ho?

Wo ne wo ahurututu ho ɔyaresafo (ahurututu ho oduruyɛfo) nkasa mfa nea wobɛhwɛ kwan ne sɛnea wobɛyɛ nhyehyɛe a wode bɛtra ase ne LAM ho. Hwɛ wo duruyɛfo no bere ano, nom wo nnuru sɛnea wɔakyerɛw ama wo no, na sɛ wunya yare no ho sɛnkyerɛnne foforo bi, sɛ wo sɛnkyerɛnne no mu yɛ den, anaasɛ sɛ wunya ɔhaw ahorow a efi w’ayaresa no mu a, ka kyerɛ wo duruyɛfo ntɛm ara.

Bere bɛn na ɛsɛ sɛ wokɔ oduruyɛfo nkyɛn? Bere bɛn na ɛsɛ sɛ wokɔ baabi a wɔhwɛ wɔn a egye ntɛmpɛ?

Sɛ ɛyɛ den ma wo sɛ wobɛhome, wobɛfefe, anaa sɛnkyerɛnne afoforo a ɛhaw adwene, sɛ ebia ɛkɔ so anaasɛ ɛyɛ kɛse a, hwɛ hu sɛ wobɛkɔ oduruyɛfo nkyɛn.

Bere a ɛsɛ sɛ wokɔ Emergency Treatment Unit (ETU) :

Sɛ wowɔ sɛnkyerɛnne ahorow a ɛkyerɛ sɛ w’ahurututu mu abubu (pneumothorax) anaa sɛnkyerɛnne afoforo a emu yɛ den a, kɔ ayaresabea ntɛm ara:

• Sɛ ɛyɛ den ma wo sɛ wobɛhome a.
• Sɛ wowɔ koko mu yaw a emu yɛ den a .
• Sɛ wofefe mogya a .
• Sɛ honam ani, anofafa, ne nnadewa dan bruu (`(cyanosis)`) .

Nsɛmmisa a ɛho hia bɛn na ɛsɛ sɛ wobisa oduruyɛfo no?

Ebia ɛbɛyɛ nea mfaso wɔ so sɛ wubebisa wo duruyɛfo nsɛm te sɛ eyinom:

• So mewɔ `(Sporadic LAM)` anaa `(TSC-LAM)`?
• Ayaresa ahorow bɛn na mewɔ?
• Ɔkwan bɛn so na ɛsɛ sɛ mede m’aduru no di dwuma?
• Nsɛnkyerɛnne foforo anaa nea ɛreyɛ kɛse bɛn na ɛsɛ sɛ mehwɛ yiye?
• So ɛho behia sɛ wɔde me ahurututu ma obi foforo?

So LAM yɛ kokoram?

Wɔntaa mmu LAM sɛ kokoram . Nanso, wɔ akwan bi so no, ɛyɛ n’ade te sɛ kokoram a ɛtrɛw fi nipadua no mu baabi kɔ foforo (kokoram a ɛtrɛw kɔ mmeae afoforo). Eyi kyerɛ sɛ nkwammoaa fi ase wɔ beae biako na ɛfa mogya ntini ne ntini a ɛma mogya tu no mu kɔ ahurututu ne asaabo mu. Ɛtɔ mmere bi a wɔfrɛ saa onyin yi sɛ benign cell metastasis.

Nanso nyansahufo ntumi nhu baabi a saa nkwammoaa yi fi ase. Afei nso, ɛte sɛ nea saa akisikuru yi ntrɛw nkɔ akwaa afoforo te sɛ mmerɛbo anaa amemene mu, te sɛ kokoram. Sɛ wɔde afiri a wɔde hwɛ nneɛma nketenkete hwɛ saa nkwammoaa yi a, ɛte sɛ nkwammoaa a ɛteɛ sen kokoram nkwammoaa.

Esiane ayaresa foforo ne LAM ho ntease a ɛkɔ soro nti, mprempren nkurɔfo retra ase akyɛ a wɔmfa wɔn ahurututu nsi ananmu. Nanso egye bere ansa na yɛatumi ayɛ nsakrae wɔ asetra kwan foforo yi mu. Sɛ wusua yare a woanya wɔ wo nkwa nna nyinaa mu ho ade a, ebetumi ayɛ te sɛ nea wɔatetew asase no afi wo nan ase. Nanso w’aduruyɛ kuw – ne w’ankasa wo mmoa kuw – betumi aboa wo ma woasan agyina wo nan so.

Awiei koraa no, nea ɛsɛ sɛ yɛkae

LAM betumi ayɛ yare a emu yɛ den, nanso ɛnyɛ wo nkutoo. Esiane sɛ wɔanya nkɔso wɔ aduruyɛ ho nyansahu mu nti, akwan pii wɔ hɔ a wɔfa so di yare yi ho dwuma. Ade a ɛho hia sen biara ne sɛ wobɛkɔ so anya adwempa, di wo duruyɛfo afotu akyi, na mma wo nnyae anidaso. Wo ne wo duruyɛfo ne w’adɔfo nkasa mfa wo haw ne wo suro ho. Kae sɛ nnipa pii wɔ hɔ a wobetumi aboa wo wɔ akwantu yi mu.

` LAM, ahurututu mu yare, yare a ɛntaa nsi, ɔhome a ɛyɛ den, sirolimus, ahurututu mu nsakrae, awosu mu nsakrae