Olina ebizibu bino ku mawuggwe go? Ka twogere ku LAM (Lymphangioleiomyomatosis) .

Wali owuliddeko ku bulwadde buno nga bulina erinnya eddene ennyo eriyitibwa LAM (lymphangioleiomyomatosis)? Oyinza okusanga erinnya eryo nga lyewuunyisa. Naye bulwadde obutatera kulabika nga bukosa amawuggwe. Leero, tugenda kukyogerako mu ngeri ennyangu, mu ngeri gy’osobola okutegeera. Teweeraliikiriranga, buli kimu tujja kukinnyonnyola.

Ddala LAM (Lymphangioleiomyomatosis) kye ki?

Mu ngeri ennyangu, LAM bulwadde obutatera kulabika obuleetera ebizimba ebijjudde amazzi okukola munda mu mawuggwe go. Kino kiyinza okwonoona amawuggwe go. Era oluusi kiyinza okuvaako ebizimba mu nsigo n’enkola y’amazzi. Teebereza ekyandibaddewo singa obutoffaali bw’ebinywa ebiseeneekerevu mu mubiri gwo butandika okukula mu bwangu obutafugibwa. Ekyo kye kibaawo wano. Kiva ku nkyukakyuka mu buzaale.

Embeera eno eyitibwa LAM (eyatulwa “limf-AN-gee-oh-ly-oh-my-oh-muh-TOH-sis”) , esinga kukwata bakyala . Butera okukwata abakyala abali wakati w’emyaka 20 ne 40, oba abakyala abayise mu myaka gy’obuvubuka nga banaatera okuva mu nsonga. Olw’ensonga eno, abasawo balowooza nti obusimu obuyitibwa estrogen oba okubeerawo kwa nnabaana buyinza okubaako kye bukola ku nkula y’ebizimba bino.

Bika ki ebikulu ebya LAM?

Waliwo ebika bya LAM ebikulu bibiri:

1. TSC-LAM: Abakyala abamu balina embeera y'obuzaale eyitibwa ``Tuberous Sclerosis Complex - TSC''. Singa bakulaakulanya LAM, eyitibwa TSC-LAM.

2. Sporadic LAM: Kino kibaawo mu ngeri ya kimpowooze, ekitegeeza nti enkyukakyuka ebaawo mu buzaale mu ngeri ey’ekifuulannenge mu bulamu. Ekikulu kiri nti ekika kino ekya Sporadic LAM tekiyisibwa mu baana bo.

Obulwadde buno obuyitibwa LAM butera kutya?

LAM mu butuufu bulwadde obutatera kulabika nnyo . Kiteeberezebwa nti abakyala nga omu ku buli 140,000 ayinza okuba n’obulwadde buno. Wabula wakati wa 30% ne 80% ku bakyala abalina ``Tuberous Sclerosis`` bayinza okuba ne LAM.

Bubonero ki obulaga obulwadde bwa LAM?

Omuntu alina LAM ayinza okufuna obubonero nga:

• Obuzibu mu kussa (dyspnea) : Oluusi kino kiyinza okweyongera mpolampola.
• Okuwuuma ddoboozi lya kuwuuma nga liva mu kifuba .
• Obulumi mu kifuba .
• Okukolola .
• Oluusi wabaawo omusaayi oba chyle (`(chyle)` - amazzi g’amazzi agava mu nkola y’okugaaya emmere) agafuluma n’omusulo .

Tokitwala nti olina LAM olw’okuba olina obubonero buno, kuba era buyinza okuba obubonero bw’endwadde endala ez’amawuggwe. N’olwekyo, kikulu okunoonya amagezi mu basawo.

Lwaki LAM eno ebaawo? Kiki ekivaako?

Ekisinga okuvaako LAM y’enkyukakyuka mu buzaale bubiri mu mubiri gwaffe obuyitibwa `(TSC1)` ne `(TSC2)`. Ensengekera zino ebbiri ziringa obuzaale obuziyiza ebizimba obuziyiza ebintu ng’obutoffaali bwa kookolo okukula mu mubiri gwaffe mu ngeri eteetaagisa. Kale, bwe wabaawo ekikyamu mu bumu ku buzaale buno obubiri, obutoffaali obwo obw’ebinywa ebiseeneekerevu bwe nnayogeddeko emabegako butandika okukula mu ngeri etafugibwa. N’ekyavaamu, kino kiri nti:

• Ebizimba by’amawuggwe (pulmonary lymphangioleiomyomatosis) bikula.
• Ebizimba (angiomyolipomas) bitondebwa mu nsigo.
• Ebizimba bitondebwa mu nkola y’amazzi (lymphatic system).

Omuntu afuna atya LAM?

Waliwo engeri bbiri omuntu gy’ayinza okukulaakulanyaamu LAM. Ekimu kwe kusikira obuzaale obulema okuva ku muzadde alina ekikyamu mu buzaale bwa `(TSC1)` oba `(TSC2)`. Obusika buno buvaamu embeera `(Tuberous Sclerosis)`, era nga nayo ejja n'obulabe bw'okufuna LAM. Abakyala abasoba mu 30% abalina `(Tuberous Sclerosis)` balina LAM.

Engeri endala eri nti enkyukakyuka mu ggiini ya `(TSC2)` ebaawo mu ngeri eyeetongodde, awatali kakwate konna ak’obuzaale. Ekituufu ekivaako kino tekimanyiddwa. LAM ekula mu ngeri eno eyitibwa Sporadic LAM. Mu mbeera eno, obubonero obulala obwa `(Tuberous Sclerosis)` tebulabika.

Ani ali mu bulabe obw’amaanyi obw’okulwala LAM?

Wadde nga kino kyewuunyisa, LAM esinga kubeera mu bakyala . Abasajja batono bokka be bazuuliddwa n’obulwadde bwa LAM n’okutuusa kati. Omusajja omu yekka y’abadde agambibwa okuba n’obulwadde bwa LAM obw’ekiseera ekigere.

Ate era, LAM eyinza okuba ey’amaanyi ennyo ng’oli lubuto ne ng’okozesa eddagala eririmu estrogen .

Bizibu ki ebiyinza okuva mu LAM?

Ekizibu ekisinga okutawaanya LAM ye pneumothorax . Okufaananako bbaatule bw’ekutuka, empewo ekulukuta okuva mu mawuggwe n’ereetera amawuggwe okugwa. Abakyala abasoba mu kitundu abalina LAM bajja kuba n’obulwadde bwa pneumothorax waakiri omulundi gumu mu bulamu bwabwe. Oluusi kino kiyinza okubaawo enfunda n’enfunda. LAM etera okuzuulibwa oluvannyuma lw’ekitundu kino.

Ebizibu ebirala ebiyinza okuvaamu mulimu:

• Okukuŋŋaanyizibwa kw’amazzi agayitibwa chyle okwetoloola amawuggwe (chylothorax).
• Okukuŋŋaanyizibwa kw’amazzi okwetoloola amawuggwe (pleural effusion).
• Okuzibikira enkola y’amazzi (lymphatic system).

Obulwadde bwa LAM buzuulibwa butya?

Okuzuula obulwadde bwa LAM, omusawo ajja kusooka kukukebera n’akubuuza ku bubonero bwo. Olwo bajja kulagira okukeberebwa n’okukebera ebifaananyi okuzuula bino wammanga:

• Laba amawuggwe go bwe gakola obulungi.
• Kebera omukka gwa oxygen mu musaayi.
• Kebera oba waliwo enkyukakyuka yonna mu mawuggwe.
• Kebera olabe oba waliwo obungi obusukka ku bwa bulijjo obwa ``puloteyina'' ezimu mu musaayi.

Olw’okuba obubonero bwa LAM bufaananako n’obw’endwadde endala ez’amawuggwe ezimanyiddwa, oluusi kiyinza okuba ekizibu abasawo okuzuula.

Bikebere ki ebikozesebwa okuzuula obulwadde bwa LAM?

Oyinza okwetaaga okukola ebigezo nga bino:

• Okukebera enkola y’amawuggwe : Bino bikebera engeri amawuggwe go gye gakuyambamu okussa n’okufulumya.
• Okukebera omukka gwa oxygen mu musaayi (`(pulse oximetry)`) : Okukebera okwangu okuzingiramu okusiba ekintu nga clip ku ngalo yo.
• Okukebera ebifaananyi : Mu bino mulimu CT scans (Computed Tomography scans), MRI (Magnetic Resonance Imaging), oba HRCT (High-Resolution Computed Tomography). Sikaani ya HRCT esobola okulaga ekifaananyi ekitegeerekeka obulungi n’ekitundu ekitono ennyo eky’amawuggwe, ne kiba kyangu okuzuula endwadde ezimu ez’amawuggwe.
• Okukebera VEGF-D : Omusawo ayinza okukebera omusaayi okukebera oba ofunye yinfekisoni, engeri ebitundu byo ebirala gye bikolamu obulungi, n’emiwendo gyo egya puloteyina eyitibwa VEGF-D (Vascular Endothelial Growth Factor-D). Abantu bangi abalina LAM balina obutoffaali buno bungi, naye si buli muntu nti alina.
• Okukebera amawuggwe : Kino kizingiramu okuggya akatundu akatono mu mawuggwe n’akakebera mu laboratory. Sampuli eno esobola okutwalibwa oba nga bakozesa bronchoscopy (enkola nga tube erimu kkamera eyisibwa mu mawuggwe) oba nga bakozesa video-assisted thoracic surgery (VATS).

Obulwadde bwa LAM bujjanjabwa butya?

Ebyembi, mu kiseera kino tewali ddagala lya LAM. Wabula eddagala lya sirolimus (era erimanyiddwa nga rapamycin, brand name Rapamune®) liyinza okuyamba okutebenkeza obulwadde buno n’okubutangira okweyongera. Sirolimus esobola okuyamba okukendeeza ku bizimba by’ekibumba (angiomyolipomas), okukendeeza ku bizimba n’amazzi agakuŋŋaanyizibwa mu nkola y’amazzi, n’okuyamba okukendeeza ku bubonero bwo.

Nga obujjanjabi obulala:

• Obujjanjabi bwa oxygen : Eri abo abalina obuzibu mu kussa.
• Ensigo ezigaziya ennyindo ezissa : Zino ziringa ezissa. Ziggulawo emikutu gy’empewo mu mawuggwe ne zikwanguyira okussa.
• Okuddaabiriza amawuggwe : Eno nteekateeka egatta dduyiro, okusomesebwa, n’okubudaabudibwa.
• Okusimbuliza amawuggwe : Ng’ekisembayo eri abo obulwadde bwabwe bwe bugenda mu maaso okutuuka ku mutendera ogw’oku ntikko ennyo.

Ka tuyige n’ebizibu ebiva mu bujjanjabi n’ebizibu ebiva mu bujjanjabi.

Sirolimus oluusi asobola okuleeta okwonooneka okw'amaanyi mu kibumba n'okwongera ku bulabe bw'okukwatibwa yinfekisoni ez'amaanyi . N’olwekyo, kikulu nnyo okukubaganya ebirowoozo n’omusawo ku birungi n’ebibi ebiri mu kukozesa eddagala lino nga tonnasalawo.

Omuntu alina LAM ayinza okusuubira ebiseera eby’omu maaso eby’engeri ki?

LAM eyinza okweyongera okusajjuka okumala ekiseera. Enteekateeka yo ey’obujjanjabi eyinza okukyuka okusinziira ku bujjanjabi ki obukusaanira. Ojja kukolagana n’omusawo wo ow’amawuggwe (omusawo w’amawuggwe) okuzuula engeri esinga obulungi ey’okulondoola embeera yo. Bulijjo ajja kukebera enkola y’amawuggwe go. Enteekateeka yo ey’obujjanjabi ejja kwesigama ku:

• emyaka gyo.
• Embeera yo ey’okuggwaamu amaanyi mu nsonga (oba oyise mu nsonga oba nedda).
• Engeri enkola y’amawuggwe gy’ekendeera amangu.
• Oba enkizi zo oba enkola ya lymphatic ekosebwa.

LAM esaasaana ku sipiidi etya?

Engeri LAM gy'esaasaana amangu eyinza okwawukana okusinziira ku muntu . Waliwo ensonga eziwerako eziyinza okukosa kino:

• Ekika kya LAM ky’olina : Wadde ng’abantu abalina `(TSC-LAM)` balina enkola y’amawuggwe ennungi okusinga abo abalina `(Sporadic LAM)`, omuwendo gw’okukendeera kw’enkola y’amawuggwe okusinga gwe gumu mu byombi.
• Emyaka gyo : Ku bantu abamu, LAM ekoma okusaasaana oluvannyuma lw’okuggwaamu amaanyi mu nsonga, nga estrogen mu mubiri ekendedde.
• Engeri gy’okwatamu obujjanjabi : Eddagala lya Sirolimus lisobola okutebenkeza obulwadde mu bantu bangi.

Ddi lwe kyetaagisa okukyusa amawuggwe?

Abantu bangi abalina LAM (nga 64%) basobola okuwangaala emyaka 20 oba okusingawo oluvannyuma lw’okuzuulibwa nga tebeetaaga kukyusibwa mawuggwe. Kyokka olw’okuba obutoffaali bwa LAM tebutandikira mu mawuggwe, ebizimba bikyayinza okukula ne bwe biba bimaze okusimbibwa amawuggwe .

Obulamu obusuubirwa ne LAM bwe buliwa? (Obulamu Obusuubirwa)

Ebbanga ly’osobola okuwangaala ne LAM kisinziira ku buzibu bw’obulwadde n’engeri gye busaasaana amangu. Naye, okutwaliza awamu, embeera nnungi nnyo kati okusinga bwe yali. Abantu abasoba mu 90% abalina LAM balamu nga wayise emyaka 10 bukya bazuuliddwa.

Nze nneerabirira ntya?

Yogerako n’omusawo wo ow’amawuggwe (omusawo w’amawuggwe) ku by’osuubira n’engeri y’okukolamu enteekateeka y’okubeera ne LAM. Laba omusawo mu budde, mira eddagala nga bwe yakulagidde, era tegeeza omusawo wo amangu ago bw’oba ​​olina obubonero obupya, singa obubonero bwo bweyongera okusajjuka, oba bw’oba ​​olina ebizibu ebiva mu bujjanjabi bwo.

Ddi lw’osaanidde okulaba omusawo? Ddi lw’osaanidde okugenda mu ddwaaliro ery’amangu?

Bw’oba ​​olina obuzibu mu kussa, okusesema oba obubonero obulala obweraliikiriza, ka bubeere nga busigala oba bweyongera, kakasa nti ogenda ew’omusawo.

Ddi lw’olina okugenda mu kitongole ekijjanjaba abalwadde ab’amangu (ETU) :

Bw’oba ​​olina obubonero bw’okugwa kw’amawuggwe (pneumothorax) oba obubonero obulala obw’amaanyi, genda mu ddwaaliro mangu:

• Bw’oba ​​olina obuzibu mu kussa.
• Bwoba olina obulumi obw'amaanyi mu kifuba .
• Singa osesema omusaayi .
• Singa olususu, emimwa, n’emisumaali bifuuka bbululu (`(cyanosis)`) .

Bibuuzo ki ebikulu by’olina okubuuza omusawo?

Kiyinza okuba eky’omugaso okubuuza omusawo wo ebibuuzo nga bino:

• Nnina `(Sporadic LAM)` oba `(TSC-LAM)`?
• Bujjanjabi ki bwe nnina?
• Nkozese ntya eddagala lyange?
• Bubonero ki obupya oba obweyongera okusajjuka bwe nsaanidde okwegendereza?
• Nja kwetaaga okusimbibwa amawuggwe?

LAM kookolo?

LAM tetera kutwalibwa nga kookolo . Wabula mu ngeri ezimu yeeyisa nga kookolo asaasaana okuva mu kifo ekimu mu mubiri okudda mu kirala (metastatic cancer). Kino kitegeeza nti obutoffaali butandikira mu kifo kimu ne buyita mu misuwa n’enkola y’amazzi okutuuka mu mawuggwe n’ensigo. Ekika ky’okukula kino oluusi kiyitibwa benign cell metastasis.

Naye bannassaayansi n’okutuusa kati tebamanyi butoffaali buno gye butandikira. Ate era ebizimba bino tebirabika nga bisaasaana nnyo mu bitundu ebirala, gamba ng’ekibumba oba obwongo, nga kookolo. Obutoffaali buno bwe butunuulirwa mu microscope, bufaanana nnyo obutoffaali obwa bulijjo okusinga obutoffaali bwa kookolo.

Olw’obujjanjabi obupya n’okwongera okutegeera LAM, kati abantu bawangaala nga tebakyusiddwa mawuggwe. Naye kitwala ekiseera okumanyiira obulamu buno obupya. Okuyiga ku bulwadde bw’obulamu bwo bwonna kiyinza okuwulira ng’ettaka likutuse okuva wansi w’ebigere byo. Naye ttiimu yo ey’abasawo – ne ttiimu yo ey’obuyambi obw’obuntu – basobola okukuyamba okudda ku bigere.

N’ekisembayo, kiki ky’olina okujjukira

LAM eyinza okuba obulwadde obusomooza, naye si ggwe wekka. Olw’okukulaakulana mu sayansi w’obusawo, waliwo engeri nnyingi ez’okuddukanyaamu obulwadde buno. Ekisinga obukulu kwe kusigala ng’olina endowooza ennungi, goberera amagezi g’omusawo, era toggwaamu ssuubi. Yogerako n’omusawo wo n’abaagalwa bo ku bikweraliikiriza n’okutya. Jjukira nti waliwo abantu bangi abasobola okukuyamba mu lugendo luno.

` LAM, obulwadde bw’amawuggwe, obulwadde obutatera kulabika, obuzibu mu kussa, sirolimus, okukyusa amawuggwe, enkyukakyuka mu buzaale