Ingaba umntwana wakho unamabala amakhulu abomvu emzimbeni wakhe? Masifunde nge-PHACE Syndrome.

Mama nootata, nisenokuba nexhala kwaye nisoyike xa nibona amabala amakhulu abomvu okanye amaqhuma amancinci emzimbeni womntwana wenu, ngamanye amaxesha ebusweni nasentanyeni. Ngamanye amaxesha ezi zizinto nje eziya kuphiliswa, kodwa kunqabile ukuba zibe luphawu lwemeko engaqhelekanga neyinkimbinkimbi efana nePHACE Syndrome . Ngoko ke, ngaphandle kokuchitha ixesha, masithethe ngokweenkcukacha malunga nokuba yintoni iPHACE Syndrome, ibangelwa yintoni, kunye nento esifanele siyenze ukuba yinto efana naleyo.

Yintoni i-PHACE Syndrome?

Kulungile, masiqale sibone ukuba yintoni i-PHACE syndrome. Ngamafutshane, yimeko engaqhelekanga kakhulu. Oko kuthetha ukuba ayenzeki kuwo wonke umntu. Yimeko yokuzalwa ekhoyo ekuzalweni. Oko kuthetha ukuba yenzeka ngelixa umntwana esesesibelekweni. Le meko inokuchaphazela iindawo ezahlukeneyo zomzimba womntwana. Ichaphazela kakhulu ulusu, ingqondo, intliziyo, imithambo emikhulu kunye namehlo .

Ixesha elininzi, kukho iziphene ezimbalwa kuphela kwindawo nganye kwezi. Abanye abantwana banokubonisa iimpawu ezimbalwa kuphela ezidweliswe ngezantsi. Ezona meko zixhaphakileyo ezibonwa ebantwaneni abane-PHACE syndrome zii -hemangiomas , iziphene ze-cerebrovascular , kunye neziphene zentliziyo . Ezi zinokubonwa ngexesha lokukhulelwa, ekuzalweni, okanye ebuntwaneni.

I-PHACE sisifinyezo seengxaki ezahlukeneyo. Ichaza iingxaki ezinkulu zophuhliso ezinxulumene nesi sifo. Ngamanye amaxesha ibizwa ngokuba yi-PHACES syndrome. I-'S' eyongezelelweyo imele ukuqhekeka kwe-sternal . Oku kuthetha ukuba ithambo eliphakathi kwesifuba, i -sternum, alakhekanga kakuhle, okanye kukho isiphene esithile kuyo.

Zithini iimpawu ze-PHACE syndrome?

Ngoko ke, zithini iimpawu zomntwana one-PHACE syndrome? Oku kunokwahluka kumntwana ngamnye. Abanye abantwana baneempawu ezimbalwa, abanye banezingaphezulu. Kwaye ubukhali beempawu buyahluka kumntwana ngamnye. Makhe sibone ukuba zeziphi iimpawu eziphambili:

• IiHemangiomas: Ezi ziindunduma ezibonakalayo, ezinombala onsundu eluswini. Ziyakhiwa kukukhula kwemithambo yegazi eyongezelelweyo . Zihlala zibonakala entlokweni, ebusweni, entanyeni, emzimbeni, nasezingalweni. Zihlala zinkulu kakhulu kwaye zinokusasazeka. Nangona kunjalo, ngamanye amaxesha ezi hemangiomas zinokuchaphazela nezitho zangaphakathi zomntwana. Ukuba oku kuyenzeka, iingxaki zokubona nokuva zinokubakho.

• Ukuphazamiseka kwengqondo:I-posterior fossa yindawo yengqondo elawula imisebenzi ebalulekileyo efana nokulinganisela, ukulungelelanisa, kunye nokuphefumla. Ukuba kukho into engaqhelekanga kule ndawo, umntwana unokuba neentshukumo ezizenzekelayo kunye nokukhubazeka kwengqondo . Kunokubakho utshintsho kwisakhiwo sengqondo ngokwayo.

• Ukuphazamiseka kwentliziyo: Ezinye iziphene zentliziyo zinokubangela iimpawu ezinje ngokuphefumla kancinci , ubuthathaka bemisipha , uxinzelelo lwegazi oluphezulu , ukuxhalaba rhoqo, kunye nokubila. Ngamanye amaxesha, intliziyo yomntwana ayikakhuli ngokupheleleyo, kwaye kunokubakho umngxuma phakathi kwamagumbi entliziyo (i-ventricular septal defect) . Okanye, kunokubakho ukucutheka kwe-aorta (i-coarctation ye-aorta).

• Ukungasebenzi kakuhle kwemithambo: Ukuba imithambo ethwala igazi iye engqondweni nasentanyeni inciphile okanye inezinye iingxaki, ingqondo isenokungafumani gazi laneleyo. Oku kunokukhokelela kwiimeko eziyingozi ezifana ne -aneurysms , i-seizures , i-stroke , okanye ukulahleka kwemvakalelo kwelinye icala lomzimba.

• Ukungasebenzi kakuhle kwamehlo: Amehlo omntwana, imithambo-luvo yamehlo, okanye imithambo yegazi emehlweni kusenokwenzeka ukuba ayikhulanga kakuhle. Oku kunokubangela ukungaboni kakuhle, ukulahleka kombono , amehlo amancinci ngokungaqhelekanga , iintshukumo zamehlo ezingalawulekiyo, okanye ukugoba kwamehlo .

• Ukungasebenzi kakuhle kwamathambo esifuba: Njengoko bekutshiwo ngaphambili, ithambo lesifuba (i-sternum) eliphakathi kwesifuba somntwana lisenokungabikho okanye lingakhuli kakuhle. Oku kunokubangela ukuba kuvele imifantu, amaqhuma, kunye nemingxuma kuloo ndawo.

Iingxaki ezinokwenzeka ze-PHACE syndrome

Abantwana abane-PHACE syndrome banamathuba amaninzi okuba neengxaki ezithile ngenxa yezi ngxaki kwisakhiwo somzimba wabo kunye nemithambo yegazi. Nazi izinto abazizo:

• Iingxaki zebhalansi
• Iingxaki zamazinyo, umzekelo , i-enamel hypoplasia kunye nokungasebenzi kakuhle kweengcambu zamazinyo.
• Ubunzima bokuginya
• Ukungasebenzi kakuhle kwenkqubo ye-endocrine . Umzekelo, ukwehla komsebenzi we-thyroid kunye nokwehla kwemveliso yeehomoni.
• Intloko ebuhlungu rhoqo, maxa wambi i-migraines .
• Ukukhula okubi
• Ukulibaziseka kokuthetha nolwimi
• Umngcipheko okhulayo wesifo sohlangothi .
• Amalungu okuzala angakhulanga kakuhle

Yintoni ebangela i-PHACE syndrome?

Abaphandi abakayazi kakuhle ukuba kutheni ezinye iintsana zine-PHACE syndrome. Izifundo zibonisa ukuba isenokuba ibangelwa zizinto zemfuza kunye neemeko ezingqongileyo. Nangona kunjalo, utshintsho oluchanekileyo lwemfuza oluyibangelayo alukacaci. Ezinye iingcali zikholelwa ukuba ibangelwa lutshintsho olungaqhelekanga lwemfuza (de novo) olwenzeka ngexesha lokukhulelwa. Oku kuthetha ukuba ayifumaneki njengelifa kubazali.

Ifunyanwa njani i-PHACE syndrome?

Kulungile, ngoko ke oogqirha bayixilonga njani i-PHACE syndrome? Ngokwesiqhelo, ugqirha uya kwenza uvavanyo olucokisekileyo lomzimba aze enze uvavanyo oluthile. Baneendlela ezithile zokuxilonga , eziluluhlu lwezikhokelo. Yileyo ndlela abagqiba ngayo ukuba umntwana une-PHACE syndrome.

Ngamanye amaxesha, oogqirha banokubona imeko ngokujonga iiskeni ozisebenzisayo ngelixa ukhulelwe. Kodwa ngamanye amaxesha, isenokungabonakali de kube semva kokuba umntwana ezelwe, okanye kamva ebuntwaneni.

Rhoqo, ugqirha uqala akhangele i -hemangioma enkulu emzimbeni womntwana, ingakumbi ebusweni, entanyeni, okanye entlokweni. Ukubakho kwe-hemangioma enye kunye nolunye uphawu oluphambili olunxulumene ne-PHACE kunokwanela ukuxilongwa.

Ukongeza, kunokwenziwa uvavanyo oluninzi ukuze kujongwe ngakumbi ingqondo yomntwana, intliziyo, imithambo yegazi kunye namehlo akhe. Olu luvavanyo oluhlala lwenziwa:

• Iiskeni ze-CT
• Ii-Echocardiograms – Oku kujonga ulwakhiwo kunye nomsebenzi wentliziyo.
• Ukuhlolwa kwamehlo – ngugqirha wamehlo.
• Uvavanyo lokuva
• I-MRI scan – Thatha imifanekiso eneenkcukacha, ingakumbi yengqondo kunye nemithambo yegazi.
• Iskeni ye-ultrasound

Iphathwa njani i-PHACE syndrome?

Xa kunyangwa isifo se-PHACE, eyona nto iphambili kukuthintela iingxaki ezinokubakho kunye nokunceda umntwana aphile ubomi obuqhelekileyo neempawu zakhe. Iindlela zonyango ziyahluka ngokuxhomekeke ekubeni leliphi ilungu okanye iindawo zomzimba ezichaphazeleka yimeko.Oko kuthetha ukuba ayinguye wonke umntwana ofumana unyango olufanayo.

Olu hlobo lonyango ludla ngokwenziwa:

• Unyango lwe-laser lwe- hemangiomas eluswini, okanye ukubanika amayeza (umz., ii-beta-blockers ezifana ne-propranolol) ukuze bancitshiswe.
• Ukubonelela ngemfundo ekhethekileyo , unyango lokuthetha, kunye ne-physiotherapy ukunceda ekufundeni esikolweni.
• Ngamanye amaxesha utyando luyadingeka ukuze kulungiswe iziphene entliziyweni, kwimithambo yegazi, okanye engqondweni.
• Ukunika amayeza ahlukeneyo ukulawula isifo sokuwa, iintloko ezibuhlungu, kunye nokungalingani kwamahomoni.
• Ukubonelela ngezixhobo ezixhasa ukulahleka kokuva kunye neeglasi zokulahlekelwa kukubona.

Iintsapho ezininzi zithetha nomcebisi wezemfuza ukuze zifunde ngakumbi ngeendlela zonyango zomntwana wazo, kunye nendlela yokubanceda njengoko bekhula. Oku kunokuba luncedo kakhulu.

Ngaba oku kungathintelwa?

Ngelishwa, akukho ndlela yokuthintela i-PHACE syndrome. Ngenxa yokuba unobangela ochanekileyo awaziwa, akukho nto unokuyenza ukunciphisa umngcipheko wokuba nomntwana onesi sifo.

Kodwa ukuba ufuna ukwazi okungakumbi ngamathuba akho okuba nomntwana onesimo semfuza, ungathetha nogqirha wakho malunga nokufumana uvavanyo lwemfuza .

Kuza kwenzeka ntoni ukuba umntwana wam une-PHACE syndrome? Yintoni onokuyilindela?

Ukuba umntwana wakho unale meko, kuya kufuneka ahlolwe rhoqo ngugqirha wakhe oyintloko kunye neengcali kwiinkalo ezahlukeneyo (umz., ingcali yentliziyo, ingcali yemithambo-luvo, ingcali yamehlo) ukuqinisekisa ukuba iimpawu zakhe azichaphazeli umgangatho wobomi bakhe. Le yimeko efuna ulawulo olude.

Le meko ayinakuchaphazela umntwana kuphela, kodwa nawe kunye nosapho lonke. Ngoko ke, kubaluleke kakhulu ukucinga ngempilo yakho yengqondo. Abanye abantu bafumana isiqabu esikhulu namandla ngokuthetha nogqirha wezempilo yengqondo .

Ngoko ke, lingakanani ixesha lokuphila komntu onesi sifo? Kuxhomekeke ekubeni iimpawu zomntwana zinzima kangakanani na kwaye zichaphazele amalungu omzimba esi sifo.

Abantwana abonzakele kakhulu engqondweni nasentliziyweni banamathuba amaninzi okuba nobomi obufutshane. Nangona kunjalo, ukuba umntwana uneempawu ezincinci kwaye zilawulwa kakuhle, unokuphila ubomi obuqhelekileyo.

Ufanele uye nini kugqirha?

Ukuba umntwana wakho unenye yezi zinto zilandelayo, qiniseka ukuba ubona ugqirha:

• Ukuba unengxaki yokutya okanye ukusela
• Ukuba umntwana akafikeleli kwimigangatho yokukhula efanelekileyo ngokweminyaka yakhe, oko kukuthi, ukuba ukhula kade (umz., akaphakamisi intamo yakhe ngexesha, akahambi, akathethi).
• Ukuba awuphenduli kwizinto ezibonakalayo, oko kukuthi, ukuba ubona into engaqhelekanga emehlweni akho.
• Ukuba ubona ibala elikhulu, elithe tyaba, elibomvu emzimbeni wakho (njenge-hemangioma).

Ufanele umse nini umntwana wakho esibhedlele ngokukhawuleza?

Ukuba ubona into efana nale, thatha umntwana wakho umse kwigumbi likaxakeka elikufutshane ngoko nangoko , okanye utsalele umnxeba ku-1990:

• Ukuba ubonisa iimpawu zestroke (umz. ukugoba ngequbuliso kwelinye icala lobuso, ukungasebenzi kakuhle kwengalo, ukungakwazi ukuthetha).
• Ukuba unesithuthwane.
• Ukuba unengxaki yokuphefumla, ukuba uziva ngathi uyafuthaniseka.
• Ukuba ukubetha kwentliziyo akuhambelani kakuhle, okanye ukuba umntwana ujika abe luhlaza okwesibhakabhaka.

Imibuzo yokubuza ugqirha/nkk.

Ungabuza ugqirha okanye umongikazi womntwana wakho imibuzo efana nale. Le iya kukunceda uqonde imeko:

• Ngaba umntwana wam une-PHACE syndrome ngokwenene? ​​Ngaba kufuneka ndenze naluphi na olunye uvavanyo ukuze ndiqinisekise ukuba ikhona?
• Ngaba umntwana wam udinga utyando ? Ukuba kunjalo, kutheni kwaye nini?
• Ngaba ikho indlela yokususa i-hemangioma yomntwana wam? Ngaba iza kunyangeka ngokupheleleyo?
• Ingaba kukho naziphi na iziphumo ebezingalindelekanga kwezi ndlela zonyango? Zithini?
• Zeziphi izinto ekufuneka sizilumkele ngakumbi kumntwana?
• Ingakanani iminyaka yobomi bomntwana wam?
• Ngaba umntwana uza kukwazi ukuphila ubomi obuqhelekileyo enale meko?

Okokugqibela, into ekufuneka uyikhumbule

Xa ufumanisa ukuba umntwana wakho unesifo esingaqhelekanga kwaye ungazi nto ingako ngaso, ungaziva wothukile kakhulu, ulusizi, kwaye woyika. Kunzima kakhulu ukukholelwa. Oku kunokuba nefuthe elikhulu kuwe nakusapho lwakho ngokweemvakalelo.

Abanye abantwana baphila ixesha elide de babe badala benesi sifo. Nangona kunjalo, kwabanye, ixesha lokuphila linokuncipha ngenxa yeempawu ezinzulu. Ukujoyina iqela lenkxaso, okanye ukuthetha nomcebisi wezemfuza okanye ingcali yezempilo yengqondo , kunokuba ngumthombo omkhulu wentuthuzelo namandla kuwe nakusapho lwakho ngeli xesha. Ngoko ke ungathandabuzi ukufumana olo ncedo.

Khumbula, awuwedwa. Kukho oogqirha, abahlengikazi, kunye nabanye abaninzi abanokukunceda ujongane nale mingeni, bamamele imibuzo yakho, kwaye bakunike ulwazi oludingayo. Ngoko ke, hlala uqinile kwaye uzame ukunika umntwana wakho okona kulungileyo akudingayo.

I - PHACE syndrome, i-hemangioma, izifo zokuzalwa, ukungasebenzi kakuhle kwengqondo, iziphene zentliziyo, iziphene zemithambo yegazi, ukungasebenzi kakuhle kwamehlo, izifo zemfuza, izifo ezingaqhelekanga, impilo yomntwana