Respiratory Insufficiency in Patients with ALS at Or Near the End of Life

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Amyotrophic lateral sclerosis (ALS) is a devastating motor neuron disease causing progressive paralysis and eventual death, usually from respiratory failure. Treatment for ALS is focused primarily on optimal symptom management because there is no known cure. Respiratory symptoms that occur are related to the disease process and can be very distressing for patients and their loved ones. Recommendations on the management of respiratory insufficiency are provided to help guide clinicians caring for patients with ALS.

Hospice and Palliative Care Feature

The Management of Andrea L. Torres, APN, CNP Respiratory Insufficiency in Patients With ALS at or Near the End of Life

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Copyright © 2012 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. Introduction 2007). By the time most patients are definitively Amyotrophic lateral sclerosis (ALS) is a devastat- diagnosed, they are often already in an advanced ing motor neuron disease characterized by pro- stage of the disease (Wood-Allum & Shaw, 2010). gressive muscle weakness eventually leading to Life expectancy is typically 3-5 years from the paralysis and death. The onset typically occurs onset of symptoms (Elman et al., 2007). in late middle age, with men slightly more af- fected than women (Wood-Allum & Shaw, 2010). Palliative Care Approaches for ALS Patients The majority of cases of ALS have no known Due to the progressive nature of ALS, early pal- cause; about 10% of ALS cases are linked to a fa- liative care is an essential component in the milial trait (Ferguson & Elman, 2007). Treatment treatment plan, and should begin as soon as the is primarily focused on optimal symptom man- diagnosis of ALS is confirmed (Elman et al., 2007). agement and palliative care as the etiology and Palliative care aims to prevent and alleviate suf- pathophysiology remain unknown, and there is fering while improving quality of life for patients no cure for the disease to date (Elman et al., 2007; and their families (Morrison & Morrison, 2006). It Wood-Allum & Shaw, 2010). is a holistic approach to care, tending to the There are many theories about causative fac- physical, spiritual, and psychosocial needs of tors of ALS, such as toxic accumulations of gluta- patients who are living with advanced disease mate, oxidative stress, and genetic links that are (McCluskey, 2007). As ALS progresses into the currently being researched; the evidence sug- terminal phase, patients then qualify for hospice gests it is a polyfactorial, multigenic disease care. (Corcia & Meininger, 2008; Valente & Karp, 2007). In the United States, hospice care is a formal The theory that glutamate toxicity results in neu- and reimbursed program of palliative care that ronal cell death led to the development of rilu- always involves an interdisciplinary team, in- zole, the only medication approved for treatment cluding a physician, nurse, hospice aide, social (Ferguson & Elman, 2007). Although the drug is worker, chaplain, volunteers, and at times other very expensive—costing about $10,000 per year— members of the healthcare team such as physi- this medication slows the disease’s progression cal, occupational, speech, and respiratory ther- and can extend life by at least 2 to 3 months apists. Alternative therapies such as music (Ferguson & Elman, 2007; Miller et al., 2009). therapy, art therapy, and massage are provided The typical disease course varies between to patients. In addition, hospice provides medi- patients, but the most common presentation is a cal equipment, supplies, and medications to unilateral weakness involving one body segment manage symptoms. To qualify for hospice care, that progressively becomes worse over time and physician certification verifying an expected spreads to other areas of the body (Ferguson & prognosis of 6 months or less if the disease runs Elman, 2007). Approximately one-third of ALS its typical course is necessary. The present Medi- patients present with symptoms of difficulty care criteria for hospice care specific to ALS speaking or swallowing known as bulbar dys- patients are listed in Supplemental Digital Con- function (Ferguson & Elman, 2007). Signs and tent 1, http://links.lww.com/HHN/A7. According symptoms of ALS that indicate bulbar weakness to the Medicare criteria, patients must display a include dysarthria, dysphagia, tongue atrophy or rapid progression of the ALS disease process and weakness, drooling, and muscle twitches called have significant nutritional or respiratory im- fasciculations (Ferguson & Elman, 2007). Usually pairment meeting all of the characteristics listed bowel and bladder control and eye movements to be in the terminal stage of the disease process are spared by the disease, but not always (Mitsu- (McCluskey & Houseman, 2004). Medicare’s cri- moto & Rabkin, 2007). teria are viewed as being too stringent by many There is no definitive diagnostic test for ALS; clinicians who work with ALS patients (McClus- diagnosis is based on the loss of both upper and key & Houseman, 2004; Mitsumoto et al., 2005). It lower motor neurons in multiple body segments is recommended that readers check with their (Ferguson & Elman, 2007). The diagnostic pro- fiscal intermediary Medicare administrator con- cess can be a difficult and lengthy process as tractors for any ALS-specific policies. patients undergo multiple diagnostic tests to rule Because the majority of patients with ALS out other disease processes (Ferguson & Elman, die from respiratory failure, it is important for

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Copyright © 2012 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. Due to the progressive nature of ALS, early palliative care is an essential component in the treatment plan, and should begin as soon as the diagnosis of ALS is confirmed. Palliative care aims to prevent and alleviate suffering while improving quality of life for patients and their families. It is a holistic approach to care, tending to the physical, spiritual, and psychosocial needs of patients who are living with advanced disease. As ALS progresses into the terminal phase, patients then qualify for hospice care.

clinicians working with these patients to effec- Diagnostic Testing to Evaluate Respiratory tively manage their respiratory symptoms as the Muscle Strength in Patients With ALS disease progresses. The following literature re- There are a number of tests available to measure view provides evidence-based recommendations inspiratory respiratory muscle function; the for the management of respiratory insufficiency forced vital capacity (FVC) is the most commonly experienced by patients with ALS at and near the used (Heffernan et al., 2006). Using a spirometer, end-of-life. patients are instructed to take the deepest breath possible and then exhale forcefully as long as ALS: Management of Respiratory possible and the volume of air exhaled is mea- Insufficiency sured (Gregory, 2007). Patients with ALS have Overview of Respiratory Insufficiency measurements taken both sitting upright and in Patients with ALS lying supine (Andersen et al., 2007; Gregory, Because the majority of patients with ALS die 2007). An abnormal FVC value is anything less from respiratory failure, interventions to help than 80% of predicted (Gregory, 2007). Another manage respiratory insufficiency and improve method used is the maximal inspiratory pres- control of symptoms related to breathing are sure (MIP). The patient is instructed to inhale crucial (Miller et al., 2009). Respiratory insuffi- against an occluded airway and the pressure is ciency in ALS is caused by the disease process then recorded by a manometer (Gregory, 2007). itself; death of the nerve cells that innervate the An MIP value of less than -60 cm H2O implies respiratory muscles leads to weakness of the inspiratory muscle weakness (Gregory, 2007). muscles of inspiration, expiration, the accessory For a quick reference for diagnostic tools, see muscles, and the upper airway muscles (Gregory, Table 1. 2007). Weakness of the diaphragm, the most im- The MIP value may be difficult to interpret for portant muscle for inspiration, often leads to ALS patients who have severe oral muscle weak- nocturnal hypoventilation in part because the ness because their value may be low simply due supine position during sleep further negatively to difficulty performing the test (Gregory, 2007). impacts a weakened diaphragm (Gregory, 2007). The sniff nasal pressure (SNP) is a newer method Patients with ALS often may report the following to measure inspiratory respiratory muscle strength initial symptoms: morning headaches, orthopnea, that is easier for patients with oral muscle weak- daytime sleepiness, and poor sleep (Gregory, ness to perform (Gregory, 2007). A pressure trans- 2007). As the disease progresses reports of a ducer is inserted into one nostril and the patient weak cough, difficulty blowing the nose and is asked to sniff (Gregory, 2007). The pressure clearing secretions, and dyspnea at rest occur obtained from sniffing correlates well with the because of advanced respiratory musculature transdiaphragmatic pressures and has shown to weakness (Elman et al., 2007; Gregory, 2007). be a more reliable indicator of respiratory func- Signs of respiratory insufficiency that may be tion in patients with ALS (Gregory, 2007; Miller et seen on clinical exam may include tachypnea and al., 2009). An SNP value of less than 65 cm H2O is the use of accessory muscles or paradoxical abnormal indicating diaphragm weakness (Corcia breathing (Elman et al., 2007). & Meininger, 2007). An SNP result less than 40 cm

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Copyright © 2012 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. H2O is a predictor of nocturnal hypoxemia and Additional tests to evaluate respiratory func- mortality in ALS patients (Gregory, 2007). tion in patients with ALS include morning arterial Two tests available to measure expiratory blood gas (ABG) sampling, pulse oximetry, blood muscle function, are the maximal expiratory bicarbonate level, and sleep quality through noc- pressure (PEmax) and the peak cough expiratory turnal oximetry and polysomnography (PSG; flow (PCEF) (Gregory, 2007). The PEmax is mea- Corcia & Meininger, 2007). Abnormal results indi- sured by a pressure transducer while the patient cating respiratory dysfunction include the fol- exhales against an occluded airway (Gregory, lowing: an ABG sampling with the pCO2 greater 2007). The PCEF is measured by having a patient than 45 mm Hg; blood bicarbonate level greater cough into a peak flow meter, and helps to indi- than 30 mmol/L; oxygen saturation levels less cate a patient’s ability to cough and clear secre- than 90% for more than 5 minutes of recording tions (Gregory, 2007). Similar to the MIP, patients time, or less than 88% for 5 consecutive minutes with severe bulbar dysfunction may have low (Corcia & Meininger, 2007). PSG requires patients readings on the PEmax related to difficulty perform- with ALS to sleep overnight in a laboratory, ing the test (Gregory, 2007). When abnormalities which may be difficult and impractical for some in expiratory respiratory muscle function are patients with ALS, but it does provide important detected such as a PCEF below 270 L/min, cough information about sleep fragmentation and hy- assist manual techniques and devices are recom- poventilation for patients that are able to com- mended to promote respiratory secretion clear- plete the test (Gregory, 2007). ance and prevent pulmonary infections (Gregory, It is important to note there is not any one test 2007; Phukan & Hardiman, 2009). These include to predict hypercapnia that is proven more reli- the use of manual abdominal thrusts and a mechani- able than others (Heffernan et al., 2006). A com- cal insufflator-exsufflator machine. It delivers pres- bination of tests tailored to the patient’s needs is surized gas into the lungs to assist with inflation most appropriate to detect early respiratory in- through a tube inserted in the mouth, and then sufficiency (Heffernan et al., 2006). Respiratory the machine exerts negative pressure to assist with function should be evaluated in patients with ALS cough and removal of pulmonary secretions when with the use of both laboratory tests and a clini- activated (Gregory, 2007; Phukan & Hardiman, cal evaluation at a minimum of every 3 months or 2009). High-frequency chest wall oscillation through earlier with any change in symptoms (Corcia & a vest may also be used to mobilize and assist Meininger, 2007; Gregory, 2007). When patients with pulmonary secretion clearance, although the with ALS have abnormal laboratory results American Academy of Neurology reports there is through pulmonary function testing or experi- not strong evidence to support or refute this ence symptoms of respiratory dysfunction, the treatment for ALS patients (Miller et al., 2009). American Academy of Neurology recommends clinicians to initiate noninvasive ventilation (NIV) as a first line of treatment for respiratory insuffi- Table 1. Quick Reference Tool ciency (Miller et al., 2009). NIV is a compact computerized device that assists ventilation using positive pressure in persons with Treatment Options for ALS Patients With respiratory dysfunction. Evidence of Respiratory Insufficiency FVC is a measurement of the amount of air exhaled Treatment options for patients with ALS vary with after maximal inspiration. some being more aggressive than others. When there is evidence of respiratory insufficiency, NIV MIP is a measurement of inhalation against an occluded airway. is a recommended treatment strategy (Andersen et al., 2007; Miller et al., 2009; Mitsumoto et al., SNP is a measurement taken with a pressure trans- 2005). Other treatments related to respiratory ducer in the nostril while person is asked to sniff. function recommended for patients with ALS in-

PEmax is a measurement taken while exhaling against clude cough assist devices (see Figure 1), suction an occluded airway. machinery, flu and pneumococcal vaccines, and aggressive treatment of pulmonary infections PCEF is a measurement of a person’s ability to cough and clear secretions. (Gregory, 2007). Regardless of whether or not a patient uses NIV, discussions about long-term

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Copyright © 2012 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. mechanical ventilation (LTMV) should take place lungs (Mehta & Hill, 2001). There are a few differ- (Phukan & Hardiman, 2009). ent types of noninvasive ventilators, which are LTMV, the most aggressive option for patients used, but bilevel noninvasive ventilators are most with ALS, is not very common in the United States commonly used on patients with ALS (Gregory, (Phukan & Hardiman, 2009). Some of the reasons 2007). A bilevel ventilator is a compact computer- for this are patient concerns related to quality of ized device to mechanically assist ventilation, life, financial cost, and disease progression de- both inspiration and expiration, in patients with spite the use of mechanical ventilation (Elman et al., respiratory dysfunction. Since 2000, noninvasive 2007). On the other side of the spectrum, there positive pressure ventilators have been refined so are patients with ALS who may not wish to use that patients with neuromuscular diseases such any assistive devices or may not be able to toler- as ALS can use them easily (Lechtzin et al., 2004). ate them; these patients should be informed With bilevel NIV, inspiratory positive pres- about the terminal phase of ALS and hospice care sure, expiratory positive pressure, and a backup (Miller et al., 2009; Ritsma et al., 2010). respiratory rate can be set, and oxygen therapy It is crucial that discussions about treatment is rarely needed (Gregory, 2007). It is important options take place before respiratory insuffi- for patients with ALS to select a comfortable ciency occurs so that patients can take time to mask that provides the best seal to prevent air think about their options (Phukan & Hardiman, leaks and increase compliance (Gregory, 2007). 2009). Clear advance directives are essential to ALS patients with upper limb weakness or severe respect patient autonomy and prevent unwanted bulbar weakness often have difficulties tolerat- procedures in the event of a respiratory crisis ing NIV, but should still be offered treatment (Elman et al., 2007). Whatever the circumstance (Miller et al., 2009). Aggressive treatment of sial- may be, because of the progressive disease pro- orrhea, excessive salivation, is important for this cess, there comes a time when NIV is no longer group of patients using anticholinergics or effective in controlling symptoms of respiratory medications with anticholinergic side effects, insufficiency, or a patient may wish to be taken such as hyoscyamine, amitriptyline, glycopyr- off of LTMV. A hospice referral to focus on com- rolate, botulinum toxin injection, and transcuta- fort and symptom control is then recommended neous scopolamine (Elman et al., 2007; Phukan & (Miller et al., 2009). The algorithm for respiratory Hardiman, 2009). management recommended by the American NIV is often used solely at nighttime to help Academy of Neurology is provided in Supplemental with symptoms of sleep-disordered breathing Digital Content 2, http://links.lww.com/HHN/A8 that present with early respiratory insufficiency (Miller et al., 2009). (Elman et al., 2007). As the disease progresses Patients with ALS are generally best managed in specialized multidisciplinary care centers (Wood- Allum & Shaw, 2010). A team of clinicians which typically includes a neurologist, nurse specialist, pulmonologist, respiratory therapist, occupational therapist, dietician, and a speech patholo- gist, all working together to support patients’ needs (Wood-Allum & Shaw, 2010). As the disease progresses and mobility becomes more difficult, specialty clinics offer an overall advantage of one clinic visit for patient convenience with practitio- ners who have expertise in caring for patients with ALS (Wood-Allum & Shaw, 2010).

NIV and Its Role in ALS Noninvasive positive pressure ventilators deliver pressurized gas to the airways through the use of Figure 1. Philips Cough Assist Mechanical a mask (or “interface”) causing an increase in Insufflator-Exsufflator. Image courtesy of transpulmonary pressure and inflation of the Philips Respironics, Murrysville, PA.

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Copyright © 2012 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. and an increase in dyspnea occurs, patients may become more dependent on NIV during the day- Because the majority of patients with time (Elman et al., 2007). With increased use of ALS die from respiratory failure, it is NIV, skin breakdown and nasal stuffiness or dryness can occur (Elman et al., 2007). Alternating important for clinicians working with masks and providing good skin care with occlu- these patients to effectively manage sive dressings are then essential nursing interventions (Elman et al., 2007). A humidified air their respiratory symptoms as the supply may help with nasal dryness or stuffiness disease progresses. (Elman et al., 2007). Heffernan et al. (2006) reviewed several obser- vational studies regarding the use of NIV in pa- sleepiness was assessed using the Epworth tients with ALS; although they were dated from Sleepiness Scale. ABG sampling was performed in the 1990s, their results support the use of NIV in patients who scored greater than 10 points on patients with ALS. A more recent study by Bourke the Epworth Sleepiness Scale or reported morn- strongly supports the use of NIV in patients with ing headaches. An assessment of the facial mus- ALS (Miller et al., 2009). Despite all of the good cles commonly used for eating, swallowing, and evidence on the benefits of NIV in patients with speaking, known as bulbar function, was catego- ALS, to prolong survival, slow the rate of respira- rized using a six-point scale with normal to mod- tory insufficiency, and increase quality of life, it erate bulbar impairment rated (4-6), and severe remains highly underutilized in the United States bulbar impairment rated (0-3). Criteria for en- and around the world (Jackson et al., 2006; Miller trance into the study included orthopnea with a et al., 2009). Females and patients of a low socio- maximum inspiratory pressure less than 60% economic status are less likely to use NIV (Lech- predicted or symptomatic daytime hypercapnia tzin et al., 2004). Some of the proposed reasons measured by the Epworth Sleepiness Scale and for this disparity include a lack of access to mul- ABG. The sample consisted of 41 people with ALS tidisciplinary care, healthcare provider bias, and who met criteria during the surveillance period patient attitudes regarding health beliefs (Lech- from September 2000 to December 2003, and tzin et al., 2004). Some factors associated with were randomly assigned to receive either NIV or increased use of NIV in patients with ALS include standard care. a higher socioeconomic status, more severe After being assigned to either the NIV or the disease, participation in research trials, male standard care group, an initial assessment of gender, and the use of a gastrostomy tube their symptoms, lung function, and quality of life (Lechtzin et al., 2004). took place, and was subsequently assessed at 1 month, 3 months, and then every 3 months The Benefits of NIV in Patients With ALS thereafter. All patients were followed up to death, A randomized controlled trial in the United with the exception of one patient who remained Kingdom explored the effect of NIV on quality of alive beyond 45 months. Multiple instruments life and survival for patients with ALS (Bourke et were used to assess quality of life: the SF36, a al., 2006). All 121 patients with ALS from a single generic instrument that includes questions re- regional center were potentially eligible to be garding general physical and emotional health included in the study. Participants were ex- perception, pain, and energy vitality; the sleep cluded if they had current or past use of NIV, apnea quality of life index (SAQLI); and the were older than 75 years of age, or were unable chronic respiratory disease questionnaire (CRQ). to complete assessment scales because of cog- Baseline ABG and PSG were completed, which nitive impairment or inability to communicate allowed for sleep stage, arousals, apneas, hypop- with the use of an assistive device. neas, and oxygen saturation levels to be recorded. At enrollment and every 2 months thereafter, Patients in both the NIV and the standard care both respiratory symptoms and measurements group were given flu and pneumococcal vaccines including FVC sitting and supine, maximum inspi- and were taught about special cough assist tech- ratory and expiratory pressures, and sniff nasal niques. Education was given to patients in both inspiratory pressure were recorded. Daytime groups about posture during sleep and adjustable

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Copyright © 2012 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. followed by mental health, energy vitality, and general health perception. Palliative care research has shown In this investigation, NIV improves survival that movement of air across the nasal and increases quality of life for patients with passages helps to relieve the sensation normal to moderately impaired bulbar function. Although NIV did not demonstrate to offer a of dyspnea in patients with refractory survival advantage in patients with severe bulbar dyspnea related to a variety of life- impairment, these patients should still be offered NIV therapy to improve symptom control and limiting illnesses. Airflow from fans quality of life (Bourke et al., 2006). Although the or open windows can stimulate small sample size is a limitation to the study, the results indicated very positive benefits overall receptors in the face and nasal for patients with ALS. passages to help decrease the sensation of dyspnea. Dyspnea in the Terminal Phase Patients with ALS are considered to be in the terminal phase of the disease when their life expectancy is less than 6 months (McCluskey, beds were supplied when indicated. All patients 2007). Dyspnea is one of the most distressing had access to palliative care in the terminal symptoms for patients with ALS (Tripodoro & De phase of their illness. Vito, 2008). This troublesome symptom can NIV was initiated in hospital using a bilevel occur either early or late in the course of the pressure support ventilator. Masks were altered disease and is terrifying for both the patient and as necessary to increase compliance. A total of their caregivers (Currow & Abernathy, 2007; Lo 20 patients had normal bulbar function or mild or Coco et al., 2006). A variety of different causes may moderate impairment (12 in the NIV group and 8 contribute to dyspnea in patients with ALS some of in the standard care). There were 21 patients which include weakness of the respiratory mus- with severe bulbar impairment (11 patients in the cles due to the disease process; choking on food, NIV group and 10 in the standard care). The ven- saliva, or liquids; anxiety; exertion; and orthopnea tilator recorded the mean duration of hours used (Tripodoro & De Vito, 2008). Anxiety associated per day, which was 9.3 hours per day in the group with dyspnea needs to be treated aggressively in with better bulbar function compared with the patients with ALS (Elman et al., 2007). poor bulbar group at 3.8 hours per day. In the As the disease progresses NIV may no longer normal and moderate bulbar impairment group effectively relieve symptoms of ALS nor provide who received NIV, the largest benefits were adequate oxygen exchange to manage symptoms shown in quality of life assessment scales and of respiratory insufficiency (Elman et al., 2007). survival. Median survival in the NIV group with Hospice care is then recommended for patients normal and moderate bulbar impairment was 216 who are in the terminal stage of the disease days whereas the group of patients who did not (Elman et al., 2007; Miller et al., 2009). receive NIV with normal and moderate bulbar impairment had a median survival of 11 days. Oxygen Therapy in ALS Patients Although NIV did not show any benefit in sur- In patients with ALS, oxygen therapy alone is not vival in the patients with poor bulbar function, it recommended to treat dyspnea because of a did show increased quality of life based on the potential to increase the arterial pCO2, which following domains listed in the assessment worsens hypoventilation, ultimately contributing scales: the CRQ dyspnea, and SAQLI daily func- to hypoventilatory respiratory failure (Elman et tioning, social isolation, and symptoms. The al., 2007; Gregory, 2007). Oxygen is generally pro- quality of life measures reported in the NIV group vided in the terminal stage of the disease process maintained above 75% of the baseline for most of when symptomatic hypoxia is present (Andersen the follow-up period not dependent on bulbar et al., 2007). Symptoms of hypoxia patients may function. The greatest improvements in quality of experience include complaints of air hunger, life were reported in problems related to sleep, headache, nausea, fatigue, or dizziness. Cyanosis

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Copyright © 2012 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. and rapid breathing are obvious signs of hypoxia Conclusion in patients with ALS in the terminal stage. The diagnosis of ALS is devastating for patients Palliative care research has shown that move- and their loved ones. Because there is no known ment of air across the nasal passages helps to cure, treatment focuses primarily on optimal relieve the sensation of dyspnea in patients with symptom control. There are many bothersome refractory dyspnea related to a variety of life- and distressing symptoms patients with ALS ex- limiting illnesses (Abernathy et al., 2010). Airflow perience, with dyspnea being one of the most from fans or open windows can stimulate re- feared. It is important that clinicians discuss ceptors in the face and nasal passages to help treatment options to manage respiratory insuffi- decrease the sensation of dyspnea (Morrison & ciency early in the course of the disease to assist Morrison, 2006). patients through their disease progression. To respect patient autonomy, early advance direc- Pharmacological Management of tives are essential for patients with ALS before Dyspnea in ALS Patients their disease advances into the terminal stage. According to Miller et al. (2009) there is insuffi- Routine assessments of respiratory function cient evidence to support any specific treatment should be performed every 3 months for patients of dyspnea in ALS patients. Treatment of dyspnea with ALS and should include a history, physical is derived from palliative care literature (Elman exam, and laboratory tests. When signs or symp- et al., 2007; Morrison & Morrison, 2006; Oxberry toms of hypoventilation occur, patients with ALS & Lawrie, 2009; Tripodoro & De Vito, 2008). Opi- should be offered NIV as the first line of treatment. oids are the mainstay of treatment for dyspnea NIV can help to improve symptoms of hypoventi- in patients who are near and at the end-of-life lation and dyspnea for many patients with ALS (Elman et al., 2007; Oxberry & Lawrie, 2009). Opi- who are able to tolerate therapy, and has been oids relieve dyspnea by decreasing the central shown to prolong survival in patients without perception of air hunger (Morrison & Morrison, severe bulbar weakness. As patients enter into the 2006). It is well known that dyspnea has psycho- terminal stage of the ALS disease process, de- logical and emotional components (Morrision & fined by a prognosis of less than 6 months to live, Morrison, 2006). Benzodiazepines such as loraz- hospice care is recommended to provide addi- epam may be helpful to treat the anxiety associ- tional support for the patient and family through ated with not being able to breathe efficiently a multidisciplinary team of clinicians. (Elman et al., 2007). Andrea L. Torres, APN, CNP, is a Critical Care Nonpharmacological Interventions for Nurse in the Medical Coronary Intensive Care Unit, Dyspnea in ALS Patients at Advocate Lutheran General Hospital in Park Nonpharmacological interventions that can be Ridge, Illinois. She graduated with a master’s de- used for the treatment of dyspnea are derived gree from Loyola University Chicago, Chicago, Illinois. from palliative care and critical care since evi- Supplemental digital content is available for dence-based recommendations in ALS literature this article. Direct URL citations appear in the are not available (Bookbinder & McHugh, 2010). printed text and are provided in the HTML and Adjusting patients’ position so they are sitting up PDF versions of this article on the journal’s and leaning forward slightly helps to increase Web site (http://journals.lww.com/homehealth abdominal pressure and improve respiratory carenurseonline). muscular function (Spector et al., 2007). Vibration The author wishes to acknowledge Dr. MariJo of the patient’s chest and electrical stimulation of Letizia for her unconditional support and guidance. leg muscles are recommended to help relieve You are truly an inspiration to all nurses. shortness of breath (Spector et al., 2007). A The author and planners have disclosed that they pursed-lip diaphragmatic breathing technique have no financial relationships related to this article. may be helpful to slow down breathing (Spector Address for correspondence: Andrea L. Torres, et al., 2007). Lastly, psychotherapy or relaxation APN, CNP, 5704 Carol Ave., Morton Grove, IL 60053 techniques such as massage and music therapy ([email protected]). can help relieve the anxiety that often accompa- nies dyspnea (Bookbinder & McHugh, 2010). DOI: 10.1097/NHH.0b013e318246d45a

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