Omwana wo omuto alina obubonero buno? Ka twogere ku bulwadde bwa Wiskott-Aldrich Syndrome.

Omwana wo omuto alwala buli kiseera? Oluusi alina ebizibu by’olususu nga eczema, avaamu omusaayi mungi ne bwe guva ku kiwundu ekitono, oba bulijjo anyiga buli wamu? Wadde nga bino biyinza okulabika ng’ebya bulijjo, oluusi wayinza okubaawo embeera y’obuzaale etali ya bulijjo emabega wa kino gye tutawuliddeko nnyo. Emu ku mbeera ezo ye Wiskott-Aldrich Syndrome. Katukwogere ku nsonga eno mu bujjuvu katono leero.

Kino obulwadde bwa Wiskott-Aldrich Syndrome kye ki?

Mu ngeri ennyangu, eno mbeera ya buzaale etali ya bulijjo nnyo . Okusinga kukosa abaserikale b’omubiri gw’omwana wo n’enkola y’obutoffaali obumu mu musaayi. Kino kiyinza okuleeta obubonero obuwerako omulundi gumu. Mu bino mulimu:

• Eczema: Embeera y’olususu ereeta ebitundu by’olususu ebikalu n’okusiiwa.
• Okubulwa abaserikale b’omubiri: Obutoffaali obweru obulwanyisa endwadde mu mubiri tebukola bulungi.
• Okuvaamu omusaayi n’okunyiga: Ne bw’okwatako akatono kiyinza okuvaako omusaayi, n’okunyiga kuyinza okubaawo mu bifo olw’okukaluubirirwa okuzimba.

Embeera eno oluusi eyinza okuvaako ebizibu ebiyinza okutta omuntu. Era kiyinza okukendeeza ku bulamu bwo. Kyokka, n’obujjanjabi obuliwo kati, obulabe buno busobola okukendeezebwa ennyo .

Embeera eno ya bulijjo etya?

Kino mu butuufu tekitera kubaawo nnyo . Okusinziira ku bibalo, kiteeberezebwa nti ku buli bukadde bw’abalenzi basatu bokka be bakwatibwa obulwadde bwa Wiskott-Aldrich Syndrome. Ne mu nsi nga Amerika, abantu abalina embeera eno tebawera 5,000. Kisinga kukwata balenzi , era tekitera nnyo nnyo abawala okukifuna.

Obuzibu obukwatagana ne WAS kye ki?

Omusawo wo ayinza n’okuyita Wiskott-Aldrich Syndrome ng’obulwadde obukwatagana ne WAS. Kino kitegeeza embeera ezikosa abaserikale b’omubiri olw’enkyukakyuka mu ggiini ya WAS. Okugeza, X-linked thrombocytopenia nayo buzibu obukwatagana ne WAS.

Naye embeera eyitibwa ``congenital neutropenia'' ereetebwa enkyukakyuka y'obuzaale ey'enjawulo, etalina kakwate na buzaale bwa ``WAS''. Abasawo embeera eno bagitegeera oluvannyuma lw’omwana okufuna obulwadde bwa bakitiriya obw’amaanyi.

Bubonero ki obulaga obulwadde bwa Wiskott-Aldrich Syndrome?

Twagamba dda nti waliwo obubonero busatu obukulu obulaga embeera eno. Ka tuzitunuulire mu bujjuvu katono .

• Eczema: Kino kireetera olususu okukala ennyo n’okusiiyibwa ., oluusi kiyinza okufuuka ekimyufu n’okufuuka ekikuta. Kiringa eczema abaana abato abamu gye bafuna naye kino kiyinza okuba eky’amaanyi katono.

• Okubulwa abaserikale b’omubiri: Embeera eno ebaawo ng’obutoffaali obweru obuyamba okukuuma emibiri gyaffe nga mulamu tebukola bulungi. Kino kikendeeza ku busobozi bw’omubiri okulwanyisa endwadde . Oluusi abaserikale b’omubiri basobola n’okutandika okulumba omubiri gwabwe. Kino kiyinza okuvaako okukwatibwa yinfekisoni enfunda eziwera, n’embeera ng’endwadde z’enkizi, okuzimba emisuwa (okuzimba emisuwa), okukendeera kw’omusaayi (omuwendo gw’omusaayi omutono), leukemia (ekika kya kookolo w’omusaayi), oba lymphoma (ekika kya kookolo w’ennywanto).

• Obuzibu bw’okuvaamu omusaayi (Microthrombocytopenia): Kino kye kiseera omuwendo gw’obutoffaali obuyitibwa platelets, obuyamba omusaayi okuzimba, bwe gukendeera oba ne gufuuka omutono ennyo . Mu butuufu buno bwe butoffaali obuyamba okukomya okuvaamu omusaayi. Kale bino bwe biba wansi, ne bwe bisalako akatono kiyinza okuvaako omusaayi ogutakoma. Kino kiyinza okuvaako okunyiga , okuvaamu omusaayi mu nnyindo, oluusi ekiddukano okuvaamu omusaayi, okuvaamu omusaayi wansi w’olususu (purpura), n’obutontome obutono obumyufu (petechiae) ku lususu.

Totya bulwadde buno olw’okuba olina obubonero buno bumu oba bubiri. Naye ebintu bino bwe bibaawo, kiba kirungi n’ogenda ew’omusawo.

Abaana abawere abalina obulwadde bwa Wiskott-Aldrich syndrome obusinga okuba obw’amaanyi (okubulwa emirimu) nabo bayinza okulaga obubonero nga:

• Eczema
• Okubulwa abaserikale b’omubiri
• Okusannyalala okw’amaanyi
• Obulwadde bw’ekifuba

Oluusi, mu mbeera z’obusimu obuzaaliranwa obuva ku buzaale bwa WAS obukola obulungi, yinfekisoni ez’amaanyi eza bakitiriya oba myelodysplastic syndrome (obulwadde bw’obusigo bw’amagumba) busobola okubaawo.

Kiki ekivaako kino?

Ekisinga okuvaako obulwadde bwa Wiskott-Aldrich Syndrome y’enkyukakyuka mu buzaale oba enkyukakyuka mu buzaale bwa WAS . Ensengekera eno eya WAS esangibwa ku mukono omumpi ogwa X chromosome yaffe. Ensengekera eno ekola puloteyina eyitibwa Wiskott-Aldrich syndrome. Puloteeni eno eri mu butoffaali bwaffe bwonna obw’omusaayi.

Puloteeni eno eya Wiskott-Aldrich syndrome eyamba obutoffaali bwaffe okunywerera ku butoffaali obulala n’ebitundu by’omubiri nga buyita mu ``kunywerera.'' Okunywerera kuno kukulu nnyo kubanga kuyamba abaserikale baffe abaserikale okuwangula abalabe nga bakitiriya ne akawuka akayingira mu mubiri.

Kale, bw’oba ​​olina enkyukakyuka mu buzaale mu buzaale bwo obwa `WAS`, obutoffaali bwo obw’omusaayi tebujja kusobola kwekwata bulungi ku butoffaali n’ebitundu ebirala. Kino kijja kulemesa abaserikale b’omubiri okukola obulungi omulimu gwabwe. Ekyo kye kivaako obubonero bw’obulwadde bwa Wiskott-Aldrich Syndrome.

Mu congenital neutropenia, enkyukakyuka mu buzaale eyimiriza entambula y’ebika by’obutoffaali bibiri, neutrophils ne monocytes, ne kiremesa abaserikale b’omubiri okufulumya obutoffaali buno okulwanyisa yinfekisoni.

Kino kintu ekiva mu milembe?

Yee, eno mbeera eyinza okusikira. Kisikira mu nkola ya ``X-linked recessive``. Kino kitegeeza nti omwana alina okufuna enkyukakyuka mu buzaale okuva mu bazadde bombi.

Tusikira chromosomes zaffe ez’okwegatta okuva mu bazadde baffe. Ensajja zirina chromosome emu eya `X` ne chromosome emu eya `Y`. Enkazi zirina ensengekera za `X` bbiri. Obulwadde buno bukosa nnyo abalenzi, kubanga enkyukakyuka eno ey’obuzaale ekosa enkola ya chromosome yaabwe yokka eya `X`.

Wadde ng’obulwadde buno bulina enkola y’amaka, abalwadde abasoba mu 30% ku bonna babufuna ``de novo'' , ekitegeeza nti buva ku nkyukakyuka empya mu buzaale ebaawo nga bafunye olubuto.

Kino okitegeera otya?

Omusawo atera okuzuula obulwadde bwa Wiskott-Aldrich Syndrome ng’akyali muwere oba mu buto . Omwana akeberebwa era ne bakola ebigezo ebyetaagisa. Obubonero obusooka obw’embeera eno buyinza okuba omusaayi mu musulo, okuvaamu omusaayi ogutali gwa bulijjo oba okunyiga. Omusawo ayinza okukola ebigezo bino wammanga okukakasa embeera eno:

• Okubala omusaayi mu bujjuvu (CBC) .
• Okukebera omusaayi gw’obuzaale
• Okusiiga omusaayi ogw’okumpi

Kino bwe kitategeerwa mu buwere, obubonero bweyongera okweyoleka mu buto.

Singa omwana wo alaga obubonero obulaga nti abaserikale b’omubiri banafuye, gamba ng’okukwatibwa yinfekisoni enfunda eziwera, kiyinza okwetaagisa okukeberebwa ebirala ng’akyali muto. Kino kiri bwe kityo kubanga omubiri gw’omwana guyinza obutasobola kulwanyisa bulungi bakitiriya ne virus, era guyinza obutaddamu bulungi kugema ebimu.

Omusawo ayinza okukebera omusaayi okukebera oba omubiri gw’omwana wo gukola obutoffaali obuziyiza endwadde oluvannyuma lw’okugema. Antibodies zino ze zikola abaserikale b’omubiri okukuuma endwadde ez’amaanyi. Okugatta ku ekyo, okukeberebwa omusaayi okulala kujja kukolebwa okukebera obutoffaali obweru obw’omwana wo naddala T-cells ne immunoglobulins (nga nabwo biyamba okukola antibodies).

Bujjanjabi ki obukolebwa ku kino?

Obulwadde bwa Wiskott-Aldrich Syndrome busobola okujjanjabibwa mu ngeri zino wammanga:

• Okujjanjaba yinfekisoniEddagala eritta obuwuka oba eddagala eritta akawuka.
• Antibody (immunoglobulin) infusions ziweebwa okuzzaawo antibodies ezibadde ziweddewo.
• Okuteekebwamu omusaayi-platelet olw’ebizibu by’okuvaamu omusaayi.
• Eczema esobola okujjanjabibwa n’eddagala erissiddwa ku mubiri n’eddagala erifukirira eritali ku ddagala (OTC) .

Singa omwana wo afuna obulwadde oba yinfekisoni, kiyinza okuba eky’amaanyi era n’okutta obulamu bwe . Okusobola okukuuma obulamu bw’omwana wo, osobola n’okugezesa obujjanjabi buno:

• Stem cell transplant : Kino kiyinza okuba nga kye kisinga obulungi mu kiseera kino.
• Gene therapy (kino kikyali mu mutendera gw’okunoonyereza).

Omusawo w’omwana wo ajja kwogera naawe ku ngeri zino ez’obujjanjabi era ategeke obujjanjabi okusobola okutuuka ku birungi omwana wo n’okutumbula omutindo gw’obulamu bwe.

Omwana wange bw’aba alina embeera eno, kiki kye nsuubira?

Singa omwana wo alina obulwadde bwa Wiskott-Aldrich Syndrome, omusawo wo ajja kukola enteekateeka y’obujjanjabi okuyamba okutangira ebizibu ebiyinza okutta obulamu bwe kubanga abaserikale b’omubiri be tebakola bulungi. Oluvannyuma lw’okuzuula obulwadde buno, oyinza okusindikibwa okubudaabudibwa ku buzaale . Kino kiyinza okukuyamba ggwe n’ab’omu maka go okumanya ebisingawo ku mbeera eno n’obujjanjabi obuliwo.

N’endwadde z’abaana eza bulijjo nga enkoko ziyinza okuleeta obuzibu obw’amaanyi mu bulamu bw’omwana wo. Olw’okuba abaserikale b’omubiri si ba maanyi ng’abaana abalala ab’emyaka gye, ayinza okwetaaga okugenda ew’omusawo amangu ago. Okujjanjaba endwadde ne yinfekisoni nga bukyali kiyinza okukuyamba okufuna ebirungi.

Omusawo ayinza okukugamba obutawa mwana wo ddagala lya kawuka ka nnamu (nga essasi lya ssennyiga) kubanga ekika ky’akawuka ekiramu kiyinza okulwaza omwana wo. Omwana wo ne bw’afuna eddagala erigema, liyinza obutakola bulungi. Omwana wo bw’alwala akawuka, okujjanjaba amangu n’eddagala eriweweeza ku kawuka ebiseera ebisinga kukola bulungi. Naye n’endwadde eza bulijjo zisobola okuleeta ebizibu.

Omwana wo ayinza okwetaaga okwekebejjebwa kookolo buli kiseera obulamu bwe bwonna kubanga ali mu bulabe obw’okufuna ebika bya kookolo ebimu naddala mu musaayi oba lymphoma .

Waliwo eddagala erijjuvu eriwonya kino?

Yee, okusimbuliza obutoffaali obusibuka (era ekiyitibwa okukyusa obusigo bw’amagumba) kuyinza okuwonya obulwadde bwa Wiskott-Aldrich Syndrome.Ebika by’okusimbuliza bino biggyawo obutoffaali obusibuka mu mubiri obukola omusaayi ne buteekebwamu obutoffaali obusibuka obulungi. Olw’okuba obulwadde bwa Wiskott-Aldrich Syndrome buleetera obutoffaali bw’omusaayi obutali bwa bulijjo, okusimbuliza obutoffaali obusibuka mu musaayi kuyinza okukyusa obutoffaali obwo n’obutoffaali obulamu obulungi era obukola.

Obulwadde bwa Wiskott-Aldrich Syndrome bufa?

Obulwadde bwa Wiskott-Aldrich Syndrome buyinza okutta omuntu . Emabegako kyategeezebwa nti abaana abatalina kusimbulizibwa butoffaali busimbuddwa bawangaala emyaka nga 15. Obubonero obuva ku yinfekisoni, okuvaamu omusaayi mu bwongo bw’omwana, yinfekisoni ez’amaanyi oba kookolo buyinza okutta obulamu, era okufa kuyinza okubaawo amangu.

Kyokka, olw’okukulaakulana kwa ssaayansi w’obusawo, obujjanjabi obuliwo kati busobozesezza okulongoosa obulamu bw’omwana okutwalira awamu.

Kino kisobola okuziyizibwa?

Eno mbeera ya buzaale era tesobola kuziyizibwa . Bw’oba ​​oteekateeka okuzaala era ng’oyagala okumanya akabi k’olina okuzaala omwana alina embeera y’obuzaale, yogerako n’omusawo wo ku kukebera obuzaale .

Nsaana okulaba omusawo w’omwana wange ku ssaawa mmeka?

Omwana wo bw’aba mulwadde era ng’azuuliddwa ng’alina obulwadde bwa Wiskott-Aldrich Syndrome, tuukirira omusawo we mu bwangu . Olw’okuba abaserikale b’omubiri baabwe tebakola bulungi, bayinza okufuna yinfekisoni emirundi mingi. Omusawo wo asobola okujjanjaba obulwadde oba yinfekisoni, oba okuziyiza ebizibu ebiyinza okutta omuntu.

Laba omusawo wo singa omwana wo alina ekimu ku bino wammanga:

• Omusaayi mu musulo (Bloody diarrhea) .
• Okuvaamu omusaayi mu nnyindo emirundi mingi
• Ebitundu ebinene ebirimu ebiwundu
• Enkyukakyuka mu lususu lwabwe
• Yinfekisoni eziddirira (e.g. ekizimba eky’amaanyi oba obulwadde bwa thrush, yinfekisoni za bakitiriya) .

Bibuuzo ki bye nnina okubuuza omusawo?

Osobola okubuuza omusawo ebibuuzo nga bino:

• Omwana wange awangaala ki?
• Bintu ki bye nsobola okukozesa okujjanjaba omwana wange obulwadde bw’okusannyalala?
• Waliwo ebizibu byonna ebiva mu bujjanjabi bw’oteesa?
• Omwana wange asaanira okusimbuliza obutoffaali obusibuka mu mubiri?

Njawulo ki eriwo wakati w’obulwadde bwa Wiskott-Aldrich Syndrome ne Ataxia-Telangiectasia?

Wiskott-Aldrich Syndrome ne Ataxia-Telangiectasia zombi mbeera za buzaale ezikosa enkola y’abaserikale b’omubiri.. Wabula obulwadde bwa ataxia-telangiectasia buva ku nkyukakyuka mu ggiini ya ATM. Kikosa entambula yo n’okukwatagana kwo. Era kireetera emisuwa okulabika nga zig-zag ku lususu lwo.

Ebintu ebikulu by’olina okujjukira

Okuzuula nti omwana wo alina embeera y’obuzaale etali ya bulijjo ejja okumukosa obulamu bwe bwonna kiyinza okukuzitoowerera. Kiyinza okukuwuniikiriza. Naye teweeraliikiriranga. Omusawo w’omwana wo ajja kukubuulira ebisingawo ku mbeera eno n’engeri gy’oyinza okuyambamu omwana wo awaka. Abaserikale b’omubiri gw’omwana wo bayinza obutakola bulungi, n’olwekyo bayinza okulwala emirundi mingi.

Kikulu okwerabirira ng’olabirira omwana wo. Abazadde n'amaka bangi bafuna okubudaabudibwa n'obuwagizi bungi nga boogera n'omubuulirizi ku bulamu bw'obwongo .

Olw’enkulaakulana mu bujjanjabi obuliwo kati, abaana abalina embeera eno kati basobola okubeera n’obulamu obujjuvu era obw’essanyu. Kale sigala ng’oli mugumu.

` Obulwadde bwa Wiskott-Aldrich, endwadde z’obuzaale, abaserikale b’omubiri, eczema, okuvaamu omusaayi, okukyusa obutoffaali obusibuka, obulamu bw’abaana