Obravnava novorojenčka z bulozno epidermolizo

diplomsko delo

Tamara Fijolič (Avtor), Metka Skubic (Recenzent), Petja Fister (Mentor), Olga Točkova (Komentor)

Povzetek

Uvod: Bulozna epidermoliza (lat. epidermolysis bullosa, EB) je dedna skupina genetskih bolezni, povezanih s krhkostjo kože. Ob minimalnih mehanskih poškodbah ali pa spontano zaradi krhkosti kože nastanejo na mestu poškodbe mehurji, erozije in brazgotine. Bolezen je posledica mutacij različnih genov z zapisom za različne beljakovine, ki povezujejo plasti kožnih celic. Bolezen je klasificirana v štiri osnovne tipe: EB simpleks, junkcijska EB, distrofična EB in Kindlerjev sindrom. Namen: Namen diplomskega dela je predstaviti zdravstveno obravnavo in nego novorojenčka z EB, zato smo diplomsko nalogo poglobili v to področje, saj to pomembno vpliva na kakovost življenja kasneje v otroštvu in odrasli dobi. Pomembno je, da so starši otrok z EB in zdravstveni delavci seznanjeni s primernimi in dostopnimi informacijami. Prav tako je delo namenjeno širši skupnosti ljudi. Metode dela: V diplomski nalogi smo uporabili deskriptivno metodo s sistematičnem pregledom literature. Pri iskanju smo uporabili časovni okvir in sicer od leta 2010 do 2021. Zbiranje podatkov je potekalo s pregledom in kritičnim izborom strokovne in znanstvene literature v slovenskem in angleškem jeziku. Rezultati: Zdravljenje ran pri novorojenčkih je drugačno kot pri otrocih ali odraslih in zahteva posebno nego. Koža je fiziološko in razvojno drugačna, zato mora biti načrt oskrbe individualen glede na starost, znake bolezni, zaplete in glede na bolnikove prioritete. Navadno rane bolnika očistimo in oskrbimo enkrat na dan oziroma po potrebi. Nego izvajamo na mehki podlagi in porabimo tehniko obračanja in dviga (angl. roll and lift). Vsak pritisk na kožo, na primer; drgnjenje z brisačo ali plenico, lahko izzove nastanek novega mehurja. Še posebej občutljive so roke, komolec, zapestje, noge in kolena. Mehurje dosledno prediramo, saj se drugače razširijo. Pomembne so prehranske potrebe novorojenčkov z EB, ki so večje kot potrebe zdravih otrok, zaradi hitrega obnavljanja kože in hkrati potrebe po povečanem vnosu kalorij. Razprava in zaključek: Osnovni načeli oskrbe vseh bolnikov z EB sta preprečevanje nastanka mehurjev in preprečevanje sekundarnih okužb. Zaradi redkosti bolezni se večina družin novorojenčkov znajde v situaciji z malo ali nič izkušnjami kadar se sooča z EB bolezen. Ne glede na podtip je vsem bolnikom z EB skupno to, da na koži nastajajo mehurji in so zato na njej stalno prisotne rane. Pri bolnikih s to izčrpavajočo boleznijo je treba upoštevati posebne vidike gostitelja, procesa celjenja in rane, da bi proces celjenja olajšali.

Ključne besede

diplomska dela;babištvo;bulozna epidermoliza;novorojenčki;oskrba mehurja;nega otroka;

Podatki

Jezik:	Slovenski jezik
Leto izida:	2022
Tipologija:	2.11 - Diplomsko delo
Organizacija:	UL ZF - Zdravstvena fakulteta
Založnik:	[T. Fijolič]
UDK:	618.2/.7
COBISS:	129723139
Št. ogledov:	15
Št. prenosov:	2
Ocena:	0 (0 glasov)
Metapodatki:

Ostali podatki

Sekundarni jezik:	Angleški jezik
Sekundarni naslov:	Management of a newborn with bullous epidermolysis
Sekundarni povzetek:	Introduction: Epidermolysis bullosa (lat. epidermolysis bullosa, EB) is a group of inherited genetic disorders associated with skin fragility. Minimal mechanical trauma or spontaneous skin fragility can lead to blistering, erosions and scarring. The disease is caused by mutations in different genes that code for different proteins that connect the layers of skin cells. The disease is classified into four basic types: EB simplex, junctional EB, dystrophic EB and Kindler syndrome. Purpose: The aim of this thesis is to present the medical management and care of the newborn with EB, and we have therefore delved into this area, as it has a significant impact on the quality of life later in childhood and adulthood. It is important that parents of children with disabilities and health professionals provided with appropriate and accessible information. The thesis will also be aimed for a wider community of people. Methods: In this thesis we used a descriptive method with a systematic literature review. The timeframe used for the search was 2010 to 2021. The data collection was carried out by reviewing and critically selecting the professional and scientific literature in Slovenian and English. Results: Wound healing in newborns is different that in children or adults so that is why it requires special care. Skin is physiologically and developmentally different, so the care plan needs to be individualised according to the age, signs of disease, complications and the patient's priorities. Patient wounds are usually cleaned and treated once a day or as needed. The treatment is carried out on a soft surface using a rolling and lifting technique. Any pressure on the skin, for example; rubbing with a towel or nappy can provoke the formation of a new blister. Particularly sensitive are hands, elbows, wrists, feet and knees. We are consistently over-penetrating blisters, because otherwise they spread. The nutritional needs of newborns with EB are higher than those of healthy children, due to rapid skin repair and the need for increased calorie intake. Discussion and conclusion: The basic principles of care for all EB patients are to prevent blistering and to prevent secondary infections. Due to the rarity of the disease, most families of newborns find themselves with little or zero experience when faced with EB disease. Regardless of the subtype, all EB patients have in common blistering of the skin, which result in persistent wounds. Specific aspects of the host, the healing process and the wound need to be taken into account in patients with this debilitating disease to facilitate the healing process.
Sekundarne ključne besede:	diploma theses;midwifery;epidermolysis bullosa;newborns;blister care;baby care;
Vrsta dela (COBISS):	Diplomsko delo/naloga
Študijski program:	0
Komentar na gradivo:	Univ. v Ljubljani, Zdravstvena fak., Oddelek za babištvo
Strani:	32 str.
ID:	17111289