Abstract | Amiotrofična lateralna skleroza (ALS) je progresivna neurodegenerativna bolest koja zahvaća živčane stanice u mozgu i leđnoj moždini. ALS čini oko 70% svih slučajeva bolesti motornih neurona i jedan od prvih simptoma koji se javlja je progresivna mišićna slabost i atrofija mišića ekstremiteta koje prate bulbarni simptomi. Atrofija mišića se javlja postepeno i na kraju zahvaća sve mišiće. Bulbarni oblik bolesti progredira znatno brže i u roku od 2 do 3 godine dolazi do respiratornih problema i prestanka disanja. Preživljavanje kod spinalog oblika je nešto duže, 3 do 5 godina, dok je prosječna dob u kojoj osobe obole 56 godina. Prevalencija ALS-a u europskoj populaciji i populaciji europskog podrijetla procjenjuje da ima 2.6 – 3 oboljelih od ALS-a na 100 000 ljudi. U 95% slučajeva ALS se pojavljuje sporadično, ali u 5 do 10% postoji pozitivna obiteljska anamneza. Otprilike 70% pacijenata ima spinalni oblik koji zahvaća gornje i/ili donje udove, oko 25% pacijenata ima bulbarni oblik, a ostalih 5% ima izoliranu slabost respiratornih mišića kojeg prate simptomi kao što su dispneja, nesanica, anoreksija i moguća smrt. Uspostavljanje dijagnoze čini kvalitetno uzeta anamneza u kojoj se obuhvaćaju specifična pitanja o simptomima koji se javljaju u pojedinim regijama. Glavna svrha liječenja je prevencija i ublažavanje komplikacija koje se javljaju kod oboljelih i poticanje što veće samostalnosti kod pacijenta u svakodnevnom životu. Najčešće aktualne dijagnoze koje se javljaju su smanjena mogućnost brige o sebi, smanjena prohodnost dišnih puteva i smanjeno podnošenje napora, a potencijalne sestrinske dijagnoze su visoki rizik za pad, visoki rizik dekubitus i visoki rizik za infekciju. Kako bolest progredira sam zdravstvena njega postaje sve kompleksnija i zahtjevnija i zato pristup medicinske sestre / medicinskog tehinčara treba biti prilagođen i individualan svakom pacijentu. Pri izradi plana zdravstvene njege treba se pridržavati načela zdravstvene njege i pristup holistički prema pacijentu uvažavajući njego psihičko i fizičko stanje. |
Abstract (english) | Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord. ALS accounts for about 70% of all cases of motor neuron disease and one of the first symptoms to appear is progressive muscle weakness and atrophy of the muscles of the extremities followed by bulbar symptoms. Muscle atrophy occurs gradually and eventually affects all muscles. The bulbar form of the disease progresses much faster and within 2 to 3 years respiratory problems and cessation of breathing occur. Survival in the spinal form is slightly longer, 3 to 5 years, while the average age at which people get the disease is 56 years. The prevalence of ALS in the European population and the population of European origin is estimated to be 2.6-3 ALS patients per 100,000 people. In 95% of cases, ALS appears sporadically, but in 5 to 10% there is a positive family history. Approximately 70% of patients have the spinal form affecting the upper and/or lower limbs, about 25% of patients have the bulbar form, and the remaining 5% have isolated respiratory muscle weakness accompanied by symptoms such as dyspnea, insomnia, anorexia and possible death. Establishing a diagnosis is made by taking a quality medical history, which includes specific questions about symptoms that occur in certain regions. The main purpose of treatment is to prevent and alleviate complications that occur in patients and to encourage greater independence in the patient's daily life. The most common nursing diagnoses that occur are reduced ability to selfcare, reduced airway patency, and reduced exertion, and potential nursing diagnoses are high risk for falls, high risk for pressure ulcers, and high risk for infection. As the disease progresses, health care itself becomes more and more complex and demanding, and therefore the approach of the nurse should be adapted and individual to each patient. When creating a health care plan, you should adhere to the principles of health care and a holistic approach to the patient, respecting his mental and physical condition. |