Progeria: yintoni, iimpawu kunye nonyango

Umxholo

• Iimpawu eziphambili
• Unyango lwenziwa njani

I-Progeria, ekwabizwa ngokuba yi-Hutchinson-Gilford Syndrome, sisifo esinqabileyo semfuza esibonakaliswa kukuguga okukhawulezileyo, malunga namaxesha asixhenxe ngaphezulu komgangatho oqhelekileyo, ke, umntwana oneminyaka eli-10 ubudala, umzekelo, ubonakala ngathi uneminyaka engama-70 ubudala.

Umntwana onesifo sengqondo uzalwa kubonakala ngathi uqhelekile, mncinci kancinci kwiminyaka yakhe yokukhulelwa, nangona kunjalo njengoko ekhula, ngesiqhelo emva konyaka wokuqala wobomi, ezinye iimpawu ziyavela ezibonisa ukwaluphala ngaphambi kwexesha, oko kukuthi, iprogeria, enjengeenwele ukulahleka, ukulahleka kwamafutha angaphantsi kunye noshintsho lweentliziyo. Kuba sisifo esibangela ukuguga ngokukhawuleza komzimba, abantwana abane-progeria banomyinge wokuphila iminyaka eli-14 kumantombazana kunye neminyaka eli-16 kubafana.

IHutchinson-Gilford Syndrome ayinalo unyango, nangona iimpawu zokuguga zibonakala, ugqirha wabantwana unokucebisa unyango olunceda ukuphucula umgangatho wobomi bomntwana.

Iimpawu eziphambili

Ekuqaleni, iprogeria yayingenazo iimpawu okanye iimpawu ezithile, nangona kunjalo, ukusukela kunyaka wokuqala wobomi, ezinye iinguqu ezicebisa isifo zinokuqwalaselwa kwaye kufuneka ziphandwe ngoogqirha babantwana kwiimviwo. Ke, ezona mpawu ziphambili zokwaluphala ngaphambi kwexesha zezi:

• Ukulibaziseka kophuhliso;
• Ubuso obuncinci ngesilevu esincinci;
• Imithambo ivela esikhumbeni kwaye inokufikelela kwi-nasal septum;
• Intloko inkulu kakhulu kunobuso;
• Ukulahleka kweenwele, kubandakanya iinkophe kunye namashiya, kuqheleke kakhulu ukugcina ukulahleka kweenwele zizonke kwiminyaka emi-3;
• Ukulibaziseka okukhawulezileyo ekwindla nasekukhuleni kwamazinyo amatsha;
• Amehlo aphuma kunye nobunzima bokuvala iinkophe;
• Ukungabikho kokuvuthwa ngokwesondo;
• Utshintsho lwentliziyo, olufana noxinzelelo lwegazi kunye nokusilela kwentliziyo;
• Ukuphuhliswa kweswekile;
• Amathambo aethe-ethe ngakumbi;
• Ukudumba kumalungu;
• Ilizwi eliphakamileyo;
• Ukuncipha kokuva.

Ngaphandle kwezi mpawu, umntwana one-progeria unamajoni omzimba aqhelekileyo kwaye akukho kubandakanyeka kwingqondo, ke ukukhula kwengqondo kwengqondo yomntwana kuyagcinwa. Ukongeza, nangona kungekho phuhliso lokuvuthwa ngokwesondo ngenxa yotshintsho lwehomoni, ezinye iihormoni ezichaphazelekayo kwimetabolism zisebenza ngokuchanekileyo.

Unyango lwenziwa njani

Akukho ndlela ikhethekileyo yonyango yesi sifo kwaye, ke, ugqirha ucebisa ezinye zonyango ngokweempawu ezivelayo. Phakathi kweendlela zonyango ezisetyenziswayo zezi:

• Ukusetyenziswa kwemihla ngemihla kweasprini: ivumela ukugcina igazi libhitya, kuthintela ukwakheka kwamahlwili anokubangela ukubetha kwentliziyo okanye ukubetha;
• Iiseshoni zePhysotherapy: banceda ekunciphiseni ukudumba kwamalungu kunye nokomeleza izihlunu, kuthintelwe ukwaphuka ngokulula;
• Utyando: zisetyenziselwa ukunyanga okanye ukunqanda iingxaki ezinzulu, ngakumbi entliziyweni.

Ukongeza, ugqirha unokuyalela amanye amayeza, njenge-statins ukunciphisa i-cholesterol, okanye ii-hormone zokukhula, ukuba umntwana utyebe kakhulu, umzekelo.

Umntwana one-progeria kufuneka alandelwe ziingcali zempilo ezininzi, kuba esi sifo siphela sichaphazela iinkqubo ezininzi. Ke, xa umntwana eqala ukuba neentlungu ezidibeneyo kunye nezihlunu, kufuneka abonwe ngugqirha wamathambo ukuze acebise amayeza afanelekileyo kwaye anike isikhokelo ngendlela yokuwonga amalungu, ukuthintela ukonyuka kwe-arthritis kunye ne-osteoarthritis. I-cardiologist kufuneka ihambisane nomntwana ukususela ngexesha lokuxilongwa, kuba uninzi lwabathwali besifo bafa ngenxa yeengxaki zentliziyo.

Bonke abantwana abane-progeria kufuneka babe nokutya okukhokelwa yingcali yesondlo, ukunqanda i-osteoporosis kangangoko kunokwenzeka kunye nokuphucula imetabolism. Ukuziqhelanisa nokusebenza komzimba okanye umdlalo ubuncinci kabini ngeveki kuyacetyiswa, njengoko kuphucula ukujikeleza kwegazi, komeleza izihlunu, kuphazamisa ingqondo kwaye ngenxa yoko umgangatho wobomi bosapho.

Ukucebiswa ngugqirha wezengqondo nako kunokuba luncedo emntwaneni ukuba asiqonde isigulo sakhe kwaye nakwiimeko zoxinzelelo, ukongeza ekubeni kubalulekile kusapho.