MULTIDISCIPLINARY APPROACH TO PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS

Amiotrofična lateralna skelroza (ALS) je neuromuskularna bolest i najčešći je oblik bolesti motornog neurona. Incidencija u Europi je 2.16/100 000 stanovnika. Češće se javlja kod muškog spola. Može biti sporadična i nasljedna. Najčešće se javlja u srednjoj i starijoj životnoj dobi. Od postavljanja dijagnoze do smrti bolesnika prođe 3-5 godina. Simptomi bolesti odgovaraju simptomima oštećenja gornjeg i donjeg motoneurona. Točan uzrok bolesti je nepoznat. Povezuje se s genetskim čimbenicima, ekscitotoksičnom hipotezom, oksidativnim stresom, okolišnim čimbenicima, imunološkim promjenama i deficitom neurotrofičnih faktora. Ne postoji specifičan biomarker, pa se dijagnoza postavlja na temelju kliničke slike. Terapija bolesti je simptomatska. Specijalizirane klinike za ALS i multidisciplinarni pristup liječenja pokazuju višestruku korist. U multidisciplinarnom timu su neurolog, liječnik obiteljske medicine, medicinska sestra, fizioterapeut, logoped, dijetetičar, pulmolog, gastroenterolog, socijalni radnik. U multidisciplinarnom pristupu centar interesa je bolesnik i obitelj koja je aktivni sudionik u liječenju. Dokazano je kako ti bolesnici imaju veću kvalitetu života, dulje očekivano trajanje života i bolji mentalni status. Važno je od samog početka bolesti uključiti obitelj bolesnika. Medicinska sestra, kao dio multidisciplinarnog tima, ima zadatak provoditi zdravstvenu njegu, educirati bolesnika i njegovu obitelj i pomoći u olakšavanju simptoma bolesti. Standardi njege bolesnika s ALS su postavljeni od strane specjaliziranih ALS klinika.Amyotrophic lateral sclerosis (ALS) is a neuromuscular disease. It is the most common form of motor neuron disease. Motor neuron diseases are caused by degeneration and death of motor neurons. Incidence in Europe is 2.16 / 100 000 inhabitants. ALS often occurs in male gender. It can be sporadic and inherited. ALS most commonly occurs in middle and older age. It lasts 3-5 years from diagnosis to death. Symptoms of the disease correspond to the symptoms of damage of upper and lower motor neurons. The exact cause of the disease is unknown. It is linked to genetic factors, excitotoxic hypothesis, oxidative stress, environmental factors, immune changes and deficit of neurotrophic factors. There is no specific biomarker, so the diagnosis is based on a clinical presentation. Therapy is symptomatic. Specialized clinics for ALS and a multidisciplinary approach to treatment shows multiple benefits. The multidisciplinary team is formed of neurologist, general practitioner, nurse, physiotherapist, speech therapist, dietician, pulmonologist, gastroenterologist and social worker. In a multidisciplinary approach, the center of interest is a patient and the patient and family are active participants in the treatment. It has been proven that these patients have a higher quality of life, longer life expectancy and better mental status. It is important to include a patient's family from the very beginning of the illness. The nurse as part of a multidisciplinary team has the task of conducting health care, educating the patient and his family, and helping to ease the symptoms of the disease. The standard of care for patients with ALS was set by the ALS specialized clinic. There is no register of patients in the Republic of Croatia, nor specialized clinics for the treatment of ALS