Ulandelelwano lukaPeter Robin

Ulandelelwano lukaPierre Robin (okanye isifo) yimeko apho usana lunomhlathi omncinci kunesiqhelo, ulwimi oluwela umva emqaleni, kunye nobunzima bokuphefumla. Kukho ngexesha lokuzalwa.

Oonobangela ngqo bokulandelelana kukaPierre Robin abaziwa. Isenokuba yinxalenye yeempawu ezininzi zemfuza.

Umhlathi ongezantsi ukhula kancinci phambi kokuzalwa, kodwa ungakhula ngokukhawuleza kwiminyaka embalwa yokuqala yobomi.

Iimpawu zale meko zibandakanya:

• Inkalakahla ecwengileyo
• Inkalaka ephezulu
• Imihlathi incinci kakhulu enesilevu esincinci
• Umhlathi lowo umva kakhulu emqaleni
• Ukuphindaphindeka kosulelo lwendlebe
• Ukuvuleka okuncinci kuphahla lomlomo, okunokubangela ukukrwitshwa okanye ulwelo lubuye luphume ngeempumlo
• Amazinyo avela xa kuzalwa usana
• Ulwimi olukhulu xa lithelekiswa nomhlathi

Umboneleli ngononophelo lwempilo uhlala echonga le meko ngexesha lovavanyo lomzimba. Ukubonisana nengcali yemfuza kunokulawula ezinye iingxaki ezinxulumene nesi sifo.

Thetha nogqirha womntwana wakho malunga neendawo zokulala ezikhuselekileyo. Olunye usana olunokulandelelana kukaPierre-Robin kufuneka lulele esiswini endaweni yomqolo ukukhusela ulwimi lwazo ukuba lungabuyeli kwindlela yomoya.

Kwiimeko eziphakathi, umntwana kuya kufuneka ukuba afakwe ityhubhu ngeempumlo nakwindlela yomoya ukuthintela ukuvalwa kwendlela. Kwiimeko ezinzima, utyando luyafuneka ukuthintela ukubhloka kwindlela yomoya ephezulu. Abanye abantwana bafuna utyando ukwenza umngxunya kwindlela yabo yomoya okanye ukuhambisa imihlathi yabo phambili.

Ukutyisa kufuneka kwenziwe ngononophelo olukhulu ukuthintela ukukrwitshwa kunye nokuphefumla ulwelo kwiindlela zomoya. Umntwana kusenokufuneka ondiswe ngombhobho ukukhusela ukuminxeka.

Ezi zixhobo zilandelayo zinokubonelela ngolwazi oluthe kratya ngokulandelelana kukaPierre Robin:

• Uphando lokuzalwa kwabantwana olunesiphene- www.birthdefects.org/pierre-robin-syndrome
• Isiseko seCleft Palate Foundation-www.cleftline.org
• Umbutho weSizwe woNxunguphalo oluNqabileyo-rarediseases.org/rare-diseases/pierre-robin- kamva

Iingxaki zokukhahlela kunye nokondla zisenokuzihambela zodwa kwiminyaka embalwa yokuqala njengoko umhlathi ongezantsi ukhula uye kubukhulu obuqhelekileyo. Kukho umngcipheko omkhulu weengxaki ukuba iindlela zomoya zomntwana azigcinwanga ekuvaleni.

Ezi ngxaki zinokwenzeka:

• Ubunzima bokuphefumla, ngakumbi xa umntwana elele
• Iziqendu zokumisa
• Ukusilela kwentliziyo
• Ukufa
• Ubunzima bokutya
• Ioksijini yegazi ephantsi kunye nomonakalo wobuchopho (ngenxa yokuphefumla nzima)
• Uhlobo lwexinzelelo lwegazi oluphezulu olubizwa ngokuba yi-pulmonary hypertension

Iintsana ezizalwe zikule meko zihlala zichongwa ekuzalweni.

Fowunela umboneleli wakho ukuba umntwana wakho unezinto eziqengqeleka okanye iingxaki zokuphefumla. Ukuvaleka kweendlela zomoya kunokubangela ingxolo ephezulu xa umntwana ephefumla ngaphakathi. Isenokukhokelela ekubeni kube luhlaza kwesikhumba (cyanosis).

Fowunela kwakhona ukuba umntwana wakho unazo ezinye iingxaki zokuphefumla.

Akukho sithintelo saziwayo. Unyango lunokunciphisa iingxaki zokuphefumla nokukrwitsha.

Isifo sikaPierre Robin; UPierre Robin tata; UPierre Robin anomaly

• Iintsana ezilukhuni kunye nezithambileyo

Dhar V. Syndromes ngokubonakaliswa komlomo. Ku: Kliegman RM, Stanton BF, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 337.

UPurnell CA, uGosain AK. Ulandelelwano lukaPeter Robin. Ku: URodriguez ED, uLosee JE, uNeligan PC, ii-eds. Utyando lweplastikhi: Umqulu wesiThathu: Utyando lwe-Craniofacial, iNtloko kunye neNtamo kunye noPhando lwePlastiki lwabantwana. Ngomhla we-4. IPhiladelphia, PA: Elsevier; I-2018: isahluko 36.