10/11/2019 Nursing Care and Considerations of Patients with Amyotrophic Lateral Sclerosis (ALS) 40th Annual Neurorehabilitation Conference on Traumatic Brain Injury, Stroke and Other Neurological Disorders Saturday & Sunday, November 16 & 17, 2019 Hyatt Regency Cambridge 575 Memorial Drive • Cambridge, MA encompasshealth.com/braintreerehab Vincent M. Vacca, Jr., MSN, RN 1 Conflicts of interest – Real or Perceived • NONE 2 Amyotrophic Lateral Sclerosis (ALS) Objectives: 1. Following this presentation the learner will be able to list three signs and symptoms of motor neuron dysfunction found in ALS. 2. Following this presentation the learner will be able to explain three diagnostic tests utilized to establish diagnosis of ALS. 3. Following this presentation the learner will be able to describe three essential nursing interventions guided by best practices and care of patients with ALS. 3 1 10/11/2019 The median age of ALS onset is 55 years • ALS affects about one in every 300 people. • About 30,000 currently with 5,000 new cases per year. • ALS is sporadic in 90% of cases and show familial inheritance in the remaining 10%. • Estimates of the median survival of people with ALS vary between 27 and 64 months. • Death from ALS is primarily pulmonary. 4 ALS is the most frequent motor neuron disorder in adults • Due to degeneration of upper and lower motor neurons in spinal and bulbar myotomes. • Most studies show male predominance with a gender ratio of 3:2, but gender differences are age related. • Age of onset tends to be later in females than in males (68.4 years vs. 61.5 years). • Limb-onset ALS is more frequent in males and bulbar onset predominates in females in all age groups. 5 There are multifactorial pathogenic processes underlying ALS • which are not yet fully determined. • Cellular events: • Oxidative stress, • Mitochondrial dysfunction, • Excitotoxicity (Glutamate), • Protein aggregation, • Impaired axonal transport, • Neuroinflammation, and • Dysregulated RNA signaling, • All can contribute to ALS. 6 2 10/11/2019 Upper & Lower Motor Neurons • Clinical findings indicate where damage occurs: UMN v. LMN. • UMN disease/lesion/disorder results in increased muscle tone/reflexes. • Signs of UMN lesion: Weakness/Spasticity/Hyper-reflexia/ + Babinski/Clasp Knife. • LMN lesion results in decreased/absent muscle tone/reflexes. • Signs of LMN lesion: Weakness/Spasticity/Reduced tone & reflexes/fasiculations. 7 8 ALS – Lou Gehrig’s Disease •Disease is motor not sensory. •May not see all the UMN & LMN signs but should see a mix of both. 9 3 10/11/2019 ALS is now universally recognized as a complex multisystem disorder • Approximately 15% of ALS patients have overt frontotemporal dementia (FTD), • and an additional 30–40% may have less severe cognitive impairment, that manifests largely as frontal lobe executive dysfunction. 10 Convincing evidence supports that ALS is a multisystem disease •not limited to motor areas alone but involving extra-motor areas as well and causing a variable degree of: •Cognitive, •Behavioral, •Dys-autonomic symptoms. 11 ALS symptoms usually begin in the distal limb or bulbar musculature, • and typically spread, causing progressive muscle weakness and eventually leading to death by respiratory insufficiency. • The median survival from symptom onset is 2–3 years, but there are great individual variations: (1) body region of onset (bulbar vs. spinal); (2) relative mix of UMN and LMN involvement; (3) progression rate; (4) age of onset, and (5) the presence of extra-motor symptoms. 12 4 10/11/2019 ALS • Will see ‘generalized’ weakness particularly - limb muscles. • The extra-ocular muscles/movements (EOMs) are preserved. • Many other cranial nerves affected leading to a ‘nasal speech’ pattern/swallowing impairment. (Bulbar) 13 Myelin sheath of nerves: • Oligodendrocytes produce myelin. • Myelin facilitates signal transmission through the nerves at a rapid rate so we can respond to our environment in a split second. • In ALS oligodendrocytes degenerate and myelin is abnormal. 14 S&S – Bulbar & Limbs • Voluntary muscle weakness, stiffness, twitching, cramps, spasticity, difficulty with posture, muscle atrophy, stiffness, slurred/nasal speech, dysphagia, chewing. • Usual onset is in arms/hands – will have difficulty writing or buttoning a shirt/tying a shoe for example. • If legs – awkward gait, stumbling, tripping. • Later stages: dyspnea & dysphagia. • Difficulties with Speaking/Swallowing & Breathing – eventual respiratory failure. • Poor prognosis and decreased survival time correlate with the nutritional status of patients with ALS. • If speech related symptoms appear first it’s called Bulbar onset ALS. • If limbs first – it’s Limb onset ALS. 15 5 10/11/2019 Weight loss and malnutrition are two common features of ALS • associated with a poor prognosis. • There is evidence suggesting that increased energy intake and a higher body mass index (BMI) of 30–35 may slow ALS progression and alter the clinical course of the disease. • Alter food consistency, integrate postural advice such as tucking the chin and flexing the neck when eating, consume oral nutrition supplements, and enteral or parenteral nutrition. • The value of EN likely exceeds that of maintaining BMI, studies have shown that EN can significantly decrease anxiety related to choking and, improve quality of life. 16 Review: Executive dysfunction is the most commonly cited cognitive impairment in ALS • Executive function encompasses several higher-order processes: • planning, • organization, • goal-directed activity, • working memory, • initiation, • behavioral regulation, and inhibitory control, • situation-appropriate decision-making on the basis of projected positive and negative outcomes in novel, complex or ambiguous situations. • Verbal fluency impairment has been consistently reported in ALS. • Language dysfunction is increasingly recognized as a core feature of ALS and has been consistently detected in patients without executive dysfunction. 17 Adherence to treatment - cognitive dysfunction; • compliance with assistive devices, • participation in clinical trials, • making informed financial and end-of-life decisions, • choices in participating in non-licensed treatments are just some of the aspects of a patient journey which may be significantly affected by cognitive or behavioral deficits. • Cognitive impairment in ALS is widely regarded as a negative prognostic indicator and linked to reduced survival. • Neuropsychological deficits in ALS are thought to be associated with increased caregiver burden and reduced quality of life. • The recognition of the far-reaching effects of neuropsychological deficits on nearly all aspects of ALS care, caregiver support, resource allocation, and prognosis, led to the inclusion of specialist neuropsychologists as core members of ALS multidisciplinary teams worldwide. 18 6 10/11/2019 ALS survival • ~50% live up to 3 years after diagnosis. • ~10% live > 10 years after diagnosis. • Can affect anyone regardless of race/gender/ethnicity. • Does not affect senses of touch, smell, hearing, vision and taste. • No apparent risk factors – 90-95% occurs randomly. • 5-10% genetic. • Death is usually caused by respiratory failure - loss of motor neurons supplying innervation to the diaphragm and chest wall muscles. • The rate of decline of Forced Vital Capacity (FVC) predicts survival of patients with ALS. 19 ALS diagnosis • Helpful to also ask a family member about some of the changes as the patient may not notice it as keenly as another. • Ex. Have your muscles shrunk or your voice changed. • Early diagnosis is challenging as the signs may be minimal. • The changes with ALS are consistent and progressive. • There are not ‘good’ days or ‘bad’ days. 20 ALS is a deadly disease – accurate diagnosis essential. • Electromyogram (EMG) is an accurate & confirmatory test for ALS done while patient is alive. • Patients/families need to understand the diagnosis. • End-of-life planning becomes essential. 21 7 10/11/2019 Early diagnosis offers the best prognosis for a longer, quality life while living with the disease. • Many medications are used to relieve symptoms but there are only 2 pharmacologic agents indicated for the management of ALS. • For 2 decades, riluzole had been the mainstay of disease-modifying therapy, but in 2017, edaravone became the second agent approved in the management of patients with ALS. • The mechanism of either agent is not well known. • Riluzole is thought to reduce damage to motor neurons through an inhibitory effect on glutamate release. • Edaravone is thought to act as a neuroprotective agent that prevents oxidative stress damage as a free radical scavenger. 22 • Muscle biopsy to investigate muscles in more detail. ALS Diagnosis • Electromyography (EMG) to measure electrical activity of muscles during contraction and relaxation. • No Specific test – like a biomarker. • Cortical thickness seems to be the • MRI – Can rule out herniated disc most promising neuroimaging (cervical), spinal cord tumors etc…w/ biomarker of ALS. similar symptoms. • Quantitative iron imaging is a very • Nerve conduction studies (NCS) – promising biomarker in ALS. measure ability of nerves to send impulses to muscles. • Diagnostic testing is done to exclude other neurologic • Genetic testing. conditions that have some • Electrical impedance myography - can similarity to ALS. measure changes in affected muscles – can also be used predict spread to other muscles. 23 Potential ALS mimickers include • cerebral lesions, • skull