Vpliv bulbarnih in psevdobulbarnih znakov na uporabo neinvazivne umetne ventilacije pri bolnikih z boleznijo motoričnega nevrona

Vehabović, Azra (author), Leonardis, Lea (mentor)

Uvod: Bolezen motoričnega nevrona ali amiotrofična lateralna skleroza (ALS) je hitro napredujoča nevrodegenerativna bolezen, ki prizadene celice motoričnih nevronov centralnega in perifernega živčevja. Posledično se pojavijo mišična šibkost, spastičnost in/ali atrofija skeletnih mišic. Pri nekaterih se bolezen začne z znaki bulbarne okvare in sicer pojavijo se dizartrija, disfagija, neučinkovit kašelj, zaletavanje, laringospazem in aspiracija. V času bolezni se razvije tudi šibkost dihalnih mišic, kar povzroči nezadostno izmenjavo kisika in ogljikovega dioksida. Posledično je potrebna uvedba umetne podpore dihanja, ki se lahko izvaja na dva načina. Z neinvazivno mehansko ventilacijo (NIV) ali invazivno mehansko ventilacijo (IMV). Raziskave so pokazale, da NIV izboljša kakovost življenja in podaljša preživetje bolnikov z ALS. Avtorji v nekaterih člankih navajajo, da NIV in izkašljevalnik slabše prenašajo bolniki z bulbarno obliko bolezni ALS kot ti s psevdobulbarno. Namen: Na podlagi podatkov pridobljenih iz zdravstvenih dokumentacij in spominskih kartic dihalnih aparatov in izkašljevalnikov primerjati vpliv bulbarnih in psevdobulbarnih znakov na uporabo NIV in izkašljevalnika pri bolnikih z ALS. Metode dela: Analizirani so bili podatki iz popisov/spominskih kartic ventilatorjev in izkašljevalnikov 101 bolnika. Demografske podatke smo analizirali z deskriptivno statistiko, posamezne parametre s t-testom, čas preživetja pa smo ponazorili s pomočjo krivulje preživetja po metodi Kaplan-Meier in logrank testom. Rezultati: Vključene bolnike smo razdelili v štiri skupine, glede na bolezenske znake in simptome ob uvedbi NIV. Podrobneje smo analizirali samo dve skupini, v katerih je bilo 48 bolnikov. Prvo skupino je sestavljalo 16 bolnikov z bulbarno (brez psevdobulbarnih znakov) obliko bolezni ALS, med njimi jih je 9 uporabljalo aparat za NIV, 7 jih je odklonilo oziroma ni prenašalo aparata. V drugi skupini s psevdobulbarno obliko (brez okvare spodnjih motoričnih nevronov v bulbarni regiji) ALS bolezni pa je bilo 32 bolnikov, med katerimi jih je 20 uporabljalo NIV, 12 pa jih je NIV odklonilo. Razprava in sklep: Ugotovili smo, da NIV pomembno vpliva na preživetje bolnikov z bulbarno kot psevdobulbarno obliko ALS. Pri psevdobulbarni obliki, pri katerih gre za učinkovito uporabo aparata za NIV (več kot 4 ure/dan), je čas preživetja daljši kot pri bolnikih z bulbarno obliko bolezni. Prav tako je pri bolnikih s psevdobulbarno obliko bolezni uporaba NIV kasneje priporočena kot pri bolnikih z bulbarno obliko bolezni, zaradi počasnejšega napredovanja bolezni. Tudi čas od začetka bolezni do smrti je pri bolnikih s psevdobulbarno obliko bolezni ALS daljši.

• Language: Slovenian
• Keywords: diplomska dela, fizioterapija, amiotrofična lateralna skleroza, okvara zgornjega motoričnega nevrona, okvara spodnjega motoričnega nevrona, neinvazivna mehanska ventilacija
• Work type: Bachelor thesis/paper
• Organization: ZF - Faculty of Health Sciences
• Place of publishing: Ljubljana
• Publisher: [A. Vehabović]
• Year: 2018
• Number of pages: 25 str.
• PID: 20.500.12556/RUL-100592
• COBISS.SI-ID: 5407083
• Publication date in RUL: 31.03.2018

Secondary language

• Language: English
• Title: The impact of bulbar and pseudobulbar signs on the use of non-invasive ventilation in patients with motor neuron disease

Abstract

Introduction: Motor neuron disease or amyotrophic lateral sclerosis (ALS) is a progressive, neurodegenerative disease that affects the motor nervous system. Consequently the weakness, spasticity and/or atrophy of skeletal muscles appear. In some cases, the ALS disease starts with bulbar signs such as dysphagia, disartria, ineffective cough, laryngospazm and aspiration. In time, weaknes of respiratory muscles appears and contributes to various respiratory complications. Because of that, it is necessary to suggest artificial respiration support. There are two types - non invasive mechanical ventilation (NIV) or invasive mechanical ventilation (IMV). Some researches showed, that NIV improves quality of ALS patient lives and has an impact on their survival. There are authors that said, that bulbar patients do not tolerate or tolerate NIV worse than pseudobulbar patients. Purpose: In our research we wanted to compare how bulbar or pseudobulbar patients reacted on NIV, based on their signs and symptoms. Methods: We analyzed some parameters from medical documentations and parameters from memory cards of ventilators for non-invasive ventilation. Demographic datas were analyzed with descriptive statistics, some of the parameters we analyzed with statistic T-test and the survival time of patients of both groups, it is presented with survival curve/graph, where we used the Kaplan-Meier method and logrank test. Results: We divided all patients in four groups, based on theirs signs and symptoms. We analyzed only 48 patients (two groups). In the first group, there were 16 patients, that had bulbar type (without pseudobulbar signs) of ALS disease, 9 of them used NIV, but 7 of them refused or did not tolerate NIV. In the second group, there were 32 patients with pseudobulbar type (without lower motor neuron dissfunction in bulbar region) of ALS. Twenty of those patients used NIV, but 12 of them refused the use of NIV. Discussion and conclussion: We came to the conclusion, that NIV has a significant impact on the time of survival of bouth groups of patients (bulbar/pseudobulbar type of disease). The time of survival was longer in a group of patients with pseudobulbar type than in a group of patients with bulbar type of ALS, if they used NIV more than 4 hours a day. We also discovered, that the use of NIV was recommended later in a group of patients with pseudobulbar type than in a group of patients with bulbar type of disease, because of slower progression of the disease. The time from the beginning of the disease till death, was also longer in a group of patients with pseudobulbar type of ALS disease than in a group of bulbar type.

• Keywords: diploma theses, physiotherapy, amyotrophic lateral sclerosis, upper motor neuron damage, lower motor neuron damage, non-invasive mechanical ventilation