Amachiza amatsha nge-cystic fibrosis

I-cystic fibrosis (i-cystic fibrosis) yenye yezona zifo eziqhelekileyo eziza kubantwana. Ngenxa yokungahambi kakuhle kweengcambu, ukuthuthwa kweeon kwiimbrane zeseli kuphazamiseka, okukhokelela ekuphazamiseni imisebenzi yamalungu amaninzi. I-Cystic fibrosis iyisifo esiphezulu se-autosomal, oko kukuthi, ukwenzela ukuba ibonakalise, umntu kufuneka azuze iikopi ezimbini zeentsholongwane eziphosakeleyo, omnye kumzali ngamnye. Iziyobisi ezintsha ze-cystic fibrosis ziya kunceda kule meko.

Ukuxilongwa kwe-cystic fibrosis

Iimpawu kunye nezibonakaliso zesifo zizodwa, kodwa ziyahlukahluka ngokuxhomekeka kobunzima bekhosi.

Ezi ziquka:

■ Ukungapheli komsebenzi we-pancreatic (kubonwe kwi-85% yezigulane);

■ ukungasebenzi kwempomnari kunye ne-bronchiectasis (ukuhluthwa okungaqhelekanga kwe-bronchial), ephuhliswa ngenxa yokuqokelelwa kwe-mucus yokunamathela kwindlela yokuphefumula;

■ Ukungasebenzi kwamagciwane okuchaphazelekayo okubandakanya umsebenzi osebenzayo ongenakho ukusebenza ekukhohliseni isisindo kunye nokulahleka kwesisindo.

Kwintsapho enye, ubunzima bomonakalo emaphaphu kubantwana bungahluka, kodwa ukungasebenzi kwepakreatic kwiimeko ezininzi kunomlinganiselo ofanayo. Ukusuleleka kwe-Lung yinto ebangela ukuba ukufa kwezigulane eziphethwe yi-cystic fibrosis. Ngokuqhelekileyo oku kungenxa yokusuleleka kwintsholongwane engagunyazisiyo kunyango. Ukuqokelela kwe-viscous mucus kwi-airways kudala izimo ezifanelekileyo zokuphuhliswa kwama-microorganms. Abantu abaphethwe yi-cystic fibrosis ngokukhethekileyo banokutheleleka kwintsholongwane yebhaktiriya uPseudomonas aeruginosa. Amaseli omphunga aphilileyo akwazi ukumelana nezilwanyana ezincinci. Kwizigulane ezine-cystic fibrosis, lo msebenzi unzima, obangela ukuba kubekho ukukhuselwa kwezifo ezingapheliyo.

Unyango lwe-cystic fibrosis

Ukuphucula iindlela zonyango lwe-cystic fibrosis, kubandakanya i-antibiotic kunye ne-physiotherapy, enjongo yokucima imiphunga ye-mucus, inyuse ukuhlala kwithuba lokuphila kwegulane ukuya kwiminyaka engama-30. Uninzi lwezigulane ezine-cystic fibrosis ezingenamandla. Isizathu sokungabikho kwindoda kungabikho kokuzalwa okungabikho kwe-vas deferens, iidrophu apho umdlwane uvela khona kumathambo kwi-urethra. Kubasetyhini, ukungabikho kwengqondo kuhambelana nobukho be-mucus obungavamile kwi-cervix. Nangona kunjalo, okwangoku izigulane zinokubanceda abantwana ngokuncedisa ukuchithwa kwempahla. Phakathi kwabameli be-European white race, omnye wabantu abangama-25 ngumphathi we-cystic fibrosis gene. Ekubeni lo mzimba upheleleka, kufuneka uzuze kubazali bobabini ngokubonakalisa iimpawu zesifo. Phakathi kwabameli be-European white race, umphathi we-genetic defect ye-cystic fibrosis inabantu abangaba-1 kwabangama-25. Abantu abanjalo babizwa ngokuba yi-heterozygous. Abanalo imiqondiso yeklinikhi yesifo kunye nomngcipheko wokuphuhlisa i-cystic fibrosis. Ngaloo manani, amathuba abo bobabini abalingani bebini baya kuba ngabaphathi be-gene defective ba-1: 400 (oko kukuthi, i-pair e-400). Umphathiswa ngamnye unomngcipheko we-50% wokudlulisela umthwalo wokuguqula umntwana ngamnye. Xa bobabini abalingani bebini bezithuthi, umntwana ngamnye unesimo esicacileyo somngcipheko wokufumana ilifa elingenampembelelo.

■ Umngcipheko we-cystic fibrosis ngenxa yelifa lezinto eziphilayo eziphosakeleyo zi-1: 4.

■ Umngcipheko wokuba ngumthwali wesini esingenasiphelo xa uzuza ifa elilodwa kunye nesinye isifo esiqhelekileyo ngu-1: 2.

■ Ilungelo lokudla ilifa ezimbini zeengqungquthela eziqhelekileyo kwaye uhlale ungathintekiyo nge-gene defective-1: 4.

Abantu abathile abazuze iimbumba ezimbini eziphosakeleyo zibizwa ngokuba yi-homozygous, kwaye abo bazuze i-gene gene (hereditary gene) bahlobo lwe-heterozygous, okanye abathwali. Abathwali banomngcipheko wokuba nomntwana ogulayo ukuba iqabane lawo liphethe i-gene defective. Abantu abangekho abathwali be-gene bazingekho kwisichengeni sokuphuhlisa eso sifo kwizingane zabo ezizayo. Amaqabane, apho ngamnye umphathiswa, anokuba ne-1: 4 yokuba banomntwana ogulayo. Ubunzima besifo buyahlukahluka ngokubanzi. Uninzi lwezigulane zifunyaniswa phambi kokuba unyaka omnye, kodwa uhlobo oluthile lwesifo luyafumaneka kwixesha eliphakathi, ngamanye amaxesha ngephene, xa lihlolwa ngenxa yokungabi nantlungu. Umxholo wetyuwa okhuphukisayo kwesikhumba unokusebenza njengesalathisi sokuxilongwa kwe-cystic fibrosis. Uvavanyo lwangoku "oluninzi" luyinkqubo elandelelweyo yindlela esetyenziswe ngaphambili ngababelethisi abakhohlise ebunzini lentsana ukuba bafumane isalathisi ephezulu ngokungavamile. Kwaye kwaye kwaziwa ukuba inqanaba lesityuwa ephezulu libonakaliso lokungenakusebenza kwamapulmonari. I-Cystic fibrosis yenye yezona zifo eziqhelekileyo ezizimeleyo zezifo ezivela kwi-Europe yaseYurophu kwaye zenzeke ngokomyinge kwi-1 kwabantwana abangama-400 abazalwa. Akunabo bonke amaqela anamaqela anjalo aphezulu. Ngokomzekelo, kubameli beSpanishi okanye i-Latino imvelaphi, iziganeko zingama-1 500 abantwana abatsha, kunye nabaseAfrika nabase-Asiya, abangaphantsi kwe-1 kwimeko engama-50,000 abantwana abazalwa. Uninzi lwamacandelo eentlanga ezifundiswayo lunemivuzo engaphantsi kwamaqela abameli be-European white. Nangona kunjalo, kunzima ukuchaza kwangaphambili izinga lokudabuka kwabantu abaxubekileyo. Phantse i-25% yabemi baseMntla Yurophu bayithwala i-genetic defective ye-cystic fibrosis. Ngokomzekelo, e-UK, isifo senzeke kumntwana ongu-1 ngokuzalwa kwabantwana abangama-4 000 (kuquka nabantwana bezinye iintlanga, ngaphandle kobumhlophe).