Management of amyotrophic lateral sclerosis (ALS) by the family nurse practitioner: A timeline for anticipated referrals

CLINICAL PRACTICE

Management of amyotrophic lateral sclerosis (ALS) by the familynurse practitioner: A timeline for anticipated referralsMelanie Davis, RN, MSN, FNP1 & Jau-Shin Lou,MD2

1School of Nursing, Pacific Lutheran University, Tacoma, Washington2Neurology Department/ALS Center of Oregon, Oregon Health and Science University, Portland, Oregon

KeywordsTreatment; family practice; nurse practitioners;

neuromuscular; neurology; collaboration;

clinical practice guidelines.

CorrespondenceMelanie Davis, RN, MSN, FNP, School of Nursing,

Pacific Lutheran University, Tacoma, WA 97239.

Tel: 503-201-0425;

Fax: 503-494-0966;

E-mail: [email protected]

Received: May 2009;

accepted: December 2010

doi: 10.1111/j.1745-7599.2011.00628.x

Abstract

Purpose: This article reviews characteristics of amyotrophic lateral sclerosis(ALS) and appropriate timing of referrals by the nurse practitioner (NP).Data sources: Selected research and clinical articles.Conclusion: Management of patients with ALS by the NP requires anticipa-tion of needed referrals based on symptom assessment and knowledge of thecommon timeline of ALS progression. Close collaboration with specialists suchas neurologists, pulmonologists, and a palliative care team provides patientsand families with much needed support and improves outcomes.Implications for practice: Anticipating and initiating appropriate and timelyreferrals for patients with ALS may improve quality of life for patients withthis devastating condition.

Nurse practitioners (NPs) are responsible for manage-ment of patients with a wide range of conditions, in-cluding those with amyotrophic lateral sclerosis (ALS).Management of a newly diagnosed patient with ALS iscomplex and requires detailed follow-up through collab-oration with multiple providers. The roles of the NP are tocomplete a comprehensive neurologic exam, to design anindividualized care plan that manages symptoms basedon clinical presentation, to coordinate care with multi-ple specialists, and to seek further outside consultationwhen appropriate (American Academy of Family Physi-cians [AAFP], 1988). Guidelines for the NP regarding ap-propriate timing of referrals are limited. Guidelines mayaid NPs with anticipation and initiation of appropriate re-ferrals and coordination with specialists with the goal ofdecreasing delay of necessary medical treatment. The aimof this article is to discuss the role of the NP when a pa-tient presents with a new diagnosis of ALS.

ALS (also called Lou Gehrig’s or motor neuron disease)is fatal and involves rapid deterioration of upper andlower motor neurons. Muscles controlled by these neu-rons become weak, atrophic, and can present with fas-ciculation. Cognitive function can also be compromised.Annually, over 5000 people are diagnosed with ALS in

the United States. Worldwide, 4–8 in 100,000 people areaffected (Genetics Home Reference, 2007). Age of on-set is between 40 and 60 years of age. Women are af-fected slightly less often than men. Only 5%–10% ofALS is familial, with the remaining 90% of cases consid-ered sporadic (National Institutes of Neurological Diseaseand Stroke [NINDS], 2003). A final diagnosis of ALS maynot occur until a year after onset of symptoms, thereforeshortening the time available to set up necessary inter-ventions. Respiratory failure from progressive paralysis isthe most common cause of death. Management is there-fore supportive and palliative (NINDS, 2003). Introduc-tion of palliative care early after diagnosis will help to ad-dress the patient’s needs by integrating the eight domainsof palliative care.

Pathophysiology and clinical manifestations

Patients with ALS suffer from progressive weaknesswithout sensory symptoms (Rowland & Shneider, 2001).Common presenting signs correspond to early involve-ment of bulbar, cervical, or lumbosacral areas of thecentral nervous system (see Table 1). The time coursecan vary from rapid progression, with patients becoming

464 Journal of the American Academy of Nurse Practitioners 23 (2011) 464–472 C©2011 The Author(s)Journal compilation C©2011 American Academy of Nurse Practitioners

M. Davis & J.-S. Lou ALS referrals by the family nurse practitioner

Table 1 Clinical manifestations of ALS

Bulbar Upper motor neuron Lower motor neuron

� Dysphagia � Hyperreflexia �Weakness� Dysarthria � Spasticity �Muscle atrophy� Sialorrhea � Babinski signs � Fasciculations� Tongue atrophy � Jaw jerk� Tongue fasciculation � Snout reflexes

� Spread of reflexes� Incoordination�Weakness

ventilator-dependent in a few months, to slow progres-sion, with patients living independently more than 10years after diagnosis.

Bulbar signs and symptoms

Bulbar signs and symptoms are caused by the involve-ment of cranial nerve motor nuclei in the medulla, in-cluding VII (facial), IX (glossopharyngeal), and XII (hy-poglossal). Patients present with dysarthria, dysphagia,sialorrhea (drooling), tongue atrophy, and tongue fasci-culation. Difficulty in speaking and swallowing is oftenclosely correlated with reduced vital capacity. Thus, bul-bar involvement carries a poor prognosis. The life ex-pectancy in patients with bulbar manifestations is shorterthan in those who presented with limb onset.

Upper motor neuron signs and symptoms

Upper motor neuron signs and symptoms include hy-perreflexia, spasticity, Babinski signs, jaw jerk, snout re-flexes, spread of reflexes, incoordination, and weakness.Upper motor neuron weakness is different from lowermotor neuron weakness because the former may not beassociated with muscle atrophy. Patients may complainof slowness, inability to control the muscles (incoordina-tion), or stiffness, which is because of spasticity.

Lower motor neuron signs and symptoms

These include weakness, muscle atrophy, and fascicu-lations; they may be focal, multifocal, or diffuse. Fascicu-lations are common and often abundant and widespread,but patients rarely present with fasciculations as an iso-lated symptom. In ALS, fasciculations are accompaniedwith weakness or upper motor neuron signs.

Cognitive changes

A significant number of ALS patients have cognitiveimpairment. Lomen-Hoerth and colleagues (2002, 2003)have examined the relationship between ALS and fron-totemporal dementia. They studied 100 patients with

ALS. Thirty-one of the patients had abnormal wordgeneration, 18 had probable or definite frontotemporaldementia, and 5 patients had possible frontotemporaldementia (Lomen-Hoerth et al., 2003).

Important negatives

Sensory symptoms, bladder and bowel dysfunction, oc-ular palsy, and decubiti are rare. Clinically, ALS is a puremotor neuron syndrome. Furthermore, motor neuronsthat innervate extraocular muscles, the bladder, and analsphincter muscles are unaffected. If sensory symptomsare found, without clear etiology in a patient diagnosedwith ALS, the diagnosis should be reevaluated by the ALSspecialist.

Diagnostic testing

The diagnosis of ALS is primarily based on presentingsymptoms and exclusion of other causes, and diagnosiscan take a long time to establish. While diagnosis remainsuncertain, patients may present to family practice witha possible or probable diagnosis of ALS (NINDS, 2003).Differential diagnoses include human immunodeficiencyvirus (HIV), human T-cell leukemia virus (HTLV), Lymedisease, multiple sclerosis, postpolio syndrome, multifo-cal motor neuropathy, spinal muscular atrophy, trauma,dietary deficiency, or exposure to toxic agents. Confir-matory diagnostic testing for ALS will be performed byspecialists and the role of the family practice clinicianswill primarily be coordination of testing and referrals andsupport for the patient and family. The most importantdiagnostic tests are the electromyography (EMG) and thenerve conduction velocity (NCV) studies. Other studiesthat aid in confirming diagnosis and ruling out otherpossible conditions include magnetic resonance imaging(MRI) and muscle biopsy. In order to enhance clinical andresearch studies in ALS, the Consortium on Clinical Tri-als in Amyotrophic Lateral Sclerosis, a subcommittee ofthe Motor Neuron Diseases Research Group of the WorldFederation of Neurology (WFN), established The El Esco-rial WFN Criteria for the Diagnosis of Amyotrophic Lat-eral Sclerosis (Brooks, 1994). See Table 2 for the criteria.

Etiology

About 10% of ALS patients have been shown to havefamilial genetic abnormalities. About 20% of these pa-tients have mutations in the SOD1 gene. The other 80%include at least 12 other autosomal dominant mutations,2 autosomal recessive, and 1 X-linked inheritance (Rosenet al., 1993; Wijesekera & Leigh, 2009).

Other suggested etiologies include smoking (Armon,2003), environmental causes (Spencer et al., 1987),

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ALS referrals by the family nurse practitioner M. Davis & J.-S. Lou

Table 2 Level of certainty in the clinical diagnosis of amyotrophic lateral sclerosis according toWorld Federation of Neurology El Escorial Criteria (Brooks,

1994)

Level of certainty Clinical features

Clinically definite amyotrophic lateral sclerosis � Clinical evidence alone by the presence of upper and lower motor neuron signs in three

regions.

Clinically probable amyotrophic lateral sclerosis � Clinical evidence alone by upper and lower motor neuron signs in at least two regions with

some upper motor neuron signs necessarily rostral to (above) the lower motor neuron signs.

Clinically probable, laboratory-supported

amyotrophic lateral sclerosis

� Clinical signs of upper and lower motor neuron dysfunction in only one region or upper motor

neuron signs alone in one region.

–And—� Lower motor neuron signs defined by EMG criteria present in at least two limbs, with proper

application of neuroimagingand clinical laboratory protocols to exclude other causes.

Clinically possible amyotrophic lateral sclerosis � Clinical signs of upper and lower motor neuron dysfunction in only one region or upper motor

neuron signs alone in one or more regions.

–Or—� Lower motor neuron signs rostral to upper motor neuron signs and the diagnosis of clinically

probable, laboratory-supported amyotrophic lateral sclerosis cannot be proven by evidence

on clinical grounds in conjunction with electrodiagnostic, neurophysiologic, neuroimaging, or

clinical laboratory studies.� Other diagnoses must have been excluded to accept a diagnosis of “clinically possible”

amyotrophic lateral sclerosis.

Clinically suspected amyotrophic lateral sclerosis � A pure lower motor neuron syndrome, wherein the diagnosis of amyotrophic lateral sclerosis

could not be regarded as sufficiently certain to include the patient in a research study. This

category is deleted from the revised El Escorial Criteria for the Diagnosis of Amyotrophic

Lateral Sclerosis.

viral infection (Berger et al., 2000), lymphoprolifera-tive diseases (Gordon et al., 1997), prior military service(Weisskopf et al., 2005), and being a professional athlete(Chio, Gianmartino, Dossena1, Mutani, & Mora, 2005).

Proposed pathogenesis

Several possible pathogenic mechanisms have beenproposed: immune and inflammatory factors (Krizbaiet al., 1998), free-radical mediated oxidative stress(Aguirre et al., 1998), glutamate excitotoxicity (Rosen-berg, Amin, & Leitner, 1992), neurofilament abnormal-ities (Rao & Weiss, 2004; Wong, He, & Strong, 2000),microglial-mediated neurotoxicity (Zhao et al., 2010), de-ranged retrograde axonal transport (Hafezparast et al.,2003), and peripherin (Robertsonet al., 2003) are be-lieved to play a role in the pathogenesis of ALS. As yet,no definitive cause has been determined.

Treatment

Treatment for ALS is directed at the improvement ofquality of life (QOL), as no cure exists to date. Riluzole(Rilutek, sanofi-aventis, Bridgewater, NJ) decreases neu-ron exposure to glutamate, an excitatory neurotransmit-ter, and is the only medication approved by the Food andDrug Administration (FDA) for ALS treatment (NINDS,

2003). One double-blind international study of 959 pa-tients with ALS found that those who took 100 mg ofriluzole were 35% less likely to die or require a tra-cheostomy within 18 months than the placebo group(Bensimon & Lacomblez, 1996; Miller, Mitchell, Lyon,& Moore, 2008). In some ALS patients, riluzole hasbeen shown to extend life approximately 2 months. Cur-rent treatment guidelines continue to recommend theuse of riluzole (Andersen et al., 2007; Miller et al.,2009). Percutaneous endoscopic gastrostomy (PEG) canbe used to provide adequate nutrition, and noninvasiveventilation (NIV), such as bilevel positive airway pres-sure, can be used as needed to assist respiration. A re-cent study by Lou, Moore, Gordon, and Miller (2009)showed that both PEG and NIV improved patient’s QOL.Other treatments consist of safety precautions, commu-nication interventions, assistive devices, dietary inter-ventions, and symptom management (Lou et al., 2009;NINDS, 2003). Family NPs can play a key role in man-agement of many of these symptoms (see Table 3 fordetails).

Referrals to initiate at first visit

At diagnosis, regardless of rate of decline or symptoms,initiate a referral to neurology, pulmonology, palliativecare, and local ALS resource groups (see Figure 1).

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Table 3 Symptomatic treatments in amyotrophic lateral sclerosisa

Spasticity Laryngospasm Pseudobulbar affect Sialorrhea Dry mouth and thick phlegm

Baclofen, 10–20 mg four

times daily

Dantrolene, 25 mg, three

times daily

Tizanidine, 4 mg, three

times daily

Clonazepam, 0.5 mg three

times daily

Lorazepam, 0.5–1 mg

three times daily

Diazepam, 2.5 to 5 mg

three times daily

Phenytoin, 300 mg four

times daily

These medications may

increase weakness and

decrease pulmonary

function.

Clonazepam, 0.5 mg as

needed

Lorazepam, 0.5–1 mg as

needed

Diazepam, 2.5–5 mg as

needed

Laryngospasm is often

triggered by aspiration,

cough, or

gastroesophageal

reflexes

Amitriptyline, 10–75 mg four

times daily

Fluoxetine, 10–40 mg four

times daily

Sertraline, 50–100 mg at

bedtime

Paroxetine, 10–40 mg four

times daily

Glycopyrrolate, 1–2 mg four

times daily

Amitriptyline, 10–25 mg three

times daily

Diphenhydramine, 25 mg

every 8 h

Oxybutynin, 5 mg three times

daily

Scopolamine, 0.125 mg three

times daily

Trihexyphenidyl, 2 mg twice

daily

Botulinum toxin injection or

parotid or submandibular

glands irradiation may

reduce saliva secretion.

A portable suction device is

also useful

Guaifenesin, 300 mg every 4 h

as needed

Propranolol, 10–40 mg three

times daily

Nebulized acetylcysteine, 10%

solution, 2 mL twice daily as

needed

Dry mouth and thick phlegm

are often caused by

medications for drooling

and depression

Sleep disturbance Pain Fatigue Jaw quivering an clenching Depression or anxiety

Amitriptyline, 10–75 mg

every night

Trazodone, 50 mg every

night

Acetaminophen, 500 mg

three times daily

Non-steroidal

anti-inflammatories

Morphine sulfate, 5–10 mg

twice daily

Amantadine, 100 mg twice

daily

Methylphenidate, 5–10 mg

twice daily

Excessive daytime fatigue can

be because of nocturnal

hypoventilation and a sleep

study is indicated.

Clonazepam, 0.5 mg three

times daily

Lorazepam, 0.5–1 mg three

times daily

Diazepam, 2.5–5 mg three

times daily

Amitriptyline, 10–75 mg daily

Fluoxetine, 10–40 mg daily

Sertraline, 50–100 mg at

bedtime

Paroxetine, 10–40 mg daily

Nasal congestion Cramps Agitation or anxiety Terminal management

Pseudoephedrine, 30–60

mg three times daily

Diphenhydramine, 25–50

mg twice daily

Budesonide nasal spray,

two sprays twice daily

Quinine sulfate, 325 mg

twice daily

Lorazepam, 1–3 mg twice

daily as needed

Diazepam, 25–10 mg twice

daily as needed

Lorazepam, 1–3 mg three

times daily

Morphine sulfate elixir 20

mg/mL, 0.5–2 mg

Morphine subcutaneous

pump at 1–5 mg/h

aAdapted from MedLinkR©Neurology (www.medlink.com) with permission. Accessed on January 10, 2010.

Neurologist

The FNP must establish a close relationship with thepatient’s neurologist for guidance with appropriatemanagement. Patients should be seen by their neurolo-gist approximately every 2–3 months, depending on therate of deterioration (Anderson et al., 2007). A referralmust be made to establish care with a neurologist thatspecializes in neuromuscular disease with training in per-forming EMG and NCV. The neurologist will act as thepoint person to consult regarding ALS and will be able tobest explain the disease process. The neurologist will also

be able to discuss possible research and/or clinical trialsavailable for patient participation.

Pulmonologist

Most patients with ALS die from pulmonary complica-tions because of weakening respiratory muscles. Baselinehistory, physical exam, and spirometry testing are recom-mended along with collaboration with a pulmonologist(Miller et al., 1999, 2009). Spirometry testing is helpfulwith predicting mortality in ALS patients. If spirometrytesting is available on site, forced vital capacity (FVC)

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Figure 1 Anticipated referrals

testing can be used to detect muscle weakness (Fallat,Jewitt, Bass, Kamm, & Norris, 1979). In case of emer-gent hospitalization of the patient, having a baselinerespiratory evaluation available for comparison will bevaluable.

Palliative care

Palliative care is a philosophy of care that focuses on thepatient and family unit with a goal of preventing and re-lieving suffering. Discussion with the patient should high-light the positive purposes of palliative care. If available,palliative care specialists can assist with assessing and co-ordinating the needs of the ALS patient and family basedon eight domains (National Consensus Project ClinicalPractice Guidelines for Quality Palliative Care [NCPQPC],2009). Within a multidisciplinary approach, care of thepatient would include first defining the needs of the pa-tient and family and defining the structure to implementcare and assure that it continues. Physical care encom-passes pain management and physical symptoms. Psy-chological care addresses loss, grief, and other psycho-logical symptoms experienced by the patient as well asfamily. Social systems available to the patient are de-fined. Spiritual and cultural norms for the patient aredefined and integrated into care. Choices should be pro-vided for the patient and family to define the patient’swishes for healthcare interventions surrounding death.Patient designation of property, care giver, and other le-gal aspects of care must also be addressed. These make up

the categories of the eight domains of palliative care (seeFigure 2).

NPs should work with palliative care specialists toestablish a care plan that encompasses the goals andneeds of the patient and family. Information regardingearly establishment of advance directives, orders for life-sustaining treatment, power of attorney, or other forms ofmedical and legal wills in preparation for the end of lifeshould be provided by either the palliative care team or

Figure 2 Eight domains of palliative care. Derived from NCPQPC (2009).

468


the NP. These directives may also help patients and fami-lies to anticipate future needs and make life-changing de-cisions before medical interventions such as NIV and PEGare necessary. NPs should be familiar with their state andlocal government regulations regarding living wills andadvance directives and communicate all options to ALSpatients early after diagnosis. Often the primary caregiveris the spouse of the patient, but family members shouldbe made aware that the patient will likely need 3–4 care-givers working in shifts to reduce caregiver strain andprevent caregiver burnout.

Local resources

Recommending local and national resources can pro-vide the patient and family with much needed supportand specialized services. Registration with both the Amy-otrophic Lateral Sclerosis Association (ALSA) and Mus-cular Dystrophy Association (MDA) should be stronglyrecommended to the patient. While many services over-lap, it is beneficial to register with both associations aseach has unique resources. Videos, books, occupationaltherapy manual, updates on ALS research, and resourcesfor patients are some services offered by ALSA (ALS As-sociation, 2007). The MDA serves patients with a widevariety of neuromuscular diagnoses, including patientswith ALS. Services include some monetary support forclinic visits as well as mobility device loans (MuscularDystrophy Association, 2009). Both ALSA and MDA of-fer support groups, access to healthcare supplies, andother resources. Many local ALSA and MDA chaptershave social work resources available to help patients navi-gate through the complicated familial and social issues as-sociated with the diagnosis of ALS. Another website (pa-tientslikeme.com/als/community) connects patients withothers with ALS and allows them to exchange personalstories and resource information.

Multidisciplinary ALS clinics

Multidisciplinary team centers specifically for patientswith ALS are becoming increasingly available. These cen-ters usually include care teams consisting of neurolo-gists, pulmonologists, speech therapists, occupational andphysical therapists, physiatrists, gastroenterologists, di-eticians, psychologists, palliative care providers, socialworkers, and dentists. If available, use of these centersmay decrease the number of admissions and length ofhospitalization of patients with ALS (Andersen et al.,2007; Chio, Mora, Balzarino & Mutani, 2004). Whenthese centers are available, care of the ALS patient is coor-dinated by the NP with close collaboration with the neu-rologist. Often, patients will be seen in the clinic onceevery 3 months for pulmonary function testing and

neurology follow-up. Based on the patient’s presentingsymptoms or decline, other providers may also consultwith the patient. Recommendations by the multidisci-plinary clinic can then be implemented by the NP. De-pending on the type of patient insurance, referral to anALS center may need to be approved or coordinatedthrough the family practice office.

Refer when symptoms present or routinely

The following lists anticipated referrals that would notbe initiated at the first visit, but instead upon presenta-tion of symptoms or decline in function. Patients will notexperience all symptoms at the same rate or concurrentlyand therefore will need individualized referrals.

Decline in respiratory function

Respiratory failure is the most common cause of deathfrom ALS; therefore, close monitoring of respiratoryfunction and symptoms is necessary. An onset of res-piratory insufficiency may include symptoms of fatigue,shortness of breath, labored breathing, inability to cough,and other presentations. Collecting history, performing aphysical exam, and spirometry testing are recommendedevery 3 months (Miller et al., 1999). FVC tends to de-crease at a consistent individual rate, with most patientsaveraging a decline of 3.5% each month (Schiffman &Belsh, 1993). If the patient is symptomatic, but stable,and the FVC is less than 50% of predicted value, initiatean evaluation by the established pulmonologist immedi-ately. If the patient is symptomatic with an FVC greaterthan 50%, still refer to pulmonology and consider or-dering further testing. Collaborate closely with the pa-tient’s pulmonologist to establish possible testing basedon the individual needs of the ALS patient (Miller et al.,1999).

While use of the FVC for measurement of respiratorydecline has been the gold standard, a retrospective studyof 161 ALS patients in the United States found that uponenrollment, only 8% met Medicare criteria for NIV byFVC while 65% met the criteria by maximum inspiratoryvolume (MIV) measurements. MIV measurement quali-fied ALS patients for treatment more than 4 months ear-lier than FVC (Mendoza, Gelinas, Moore, & Miller, 2007).Trends in the United States may therefore shift to the useof MIV for ALS patients.

Options for ventilator support, including NIV and in-vasive ventilation (IV), can be reviewed with the pa-tient. One study of 64 patients showed longer survivalin patients started on NIV after a drop in 15 periodsof nocturnal desaturation on pulse oximetry comparedto patients started on NIV after detection of diurnal

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respiratory insufficiency (Pinto, de Carvalho, Evangelist,Lopes, & Sales-Luis, 2003). Specific details of NIV and IVshould be discussed with the pulmonologist. If the patientchooses to have IV, complications should be monitored.These may include infection, increased secretions, andtracheoesophageal fistula (Andersen et al., 2007; Leighet al., 2003). Pulmonary symptoms should be managedby the pulmonary specialist. Consult the pulmonologist ifcare becomes more complicated than treating secondarysymptoms or if there is further decline in respiratoryfunction.

Dysphagia and dysarthria

As weakness in muscles of the throat and mouth in-creases, ALS patients may present with difficulty inspeech and swallowing. Often caregivers will noticechanges in the patient’s speech. Patients may noticecough during eating, increased choking, or overall diffi-culty swallowing. This increases risk for infection, aspi-ration, mucus plugs, and atelectasis (Miller et al., 1999).Speech therapy consultation should be initiated immedi-ately along with screening for airway compromise, aspi-ration pneumonia, or nutrition deficits. Speech therapistscan then establish a baseline swallowing ability and workwith the patient to promote safe swallowing.

Speech therapists can also work with patients to en-hance speech and introduce assistive devices as appropri-ate. While most speech deficits are caused by muscle at-rophy, a small number of patients present with languageimpairment. The patient’s ability to communicate withfriends, family, and caregivers largely impacts QOL. Earlyreferral at the first sign of symptoms may provide time forlearning to use equipment and therefore improve successof communication (Cobble, 1998; Murphy, 2004). TheNP must be familiar with common communication de-vices and work to prescribe devices if necessary. As theALS patient’s function declines, collaboration with thespeech therapist, occupational therapist, local resources,social work, palliative care, and others in the multidisci-plinary team will be necessary to assess communicationneeds. Promotion of better communication and educa-tion of healthcare providers may enhance QOL for thepatient and family and prevent poor outcomes.

Nutrition deficits

Monitor weight loss and muscle atrophy by dietary intakerecord, laboratory values, and muscle atrophy. If nutri-tion deficits are present, a referral to a registered dietitianshould be initiated. The patient’s ability to chew, swallow,and breath can affect the energy expenditure required formeal consumption. PEG tube placement can help extend

life and improve nutrition if initiated early after swallow-ing difficulty develops. A recent study showed that theuse of a PEG to provide adequate nutrition improved QOL(Lou et al., 2009). The length of life may be increased bymonths if the PEG is inserted before the patient’s FVC isless than 50% of predicted value (Mathus-Vliegen, Louw-erse, Merkus, Tytgat, & Vianney de Jong, 1994; Mazziniet al. 1995). Another option is percutaneous radiologicgastrostomy, which does not require general anesthesia(Chio et al., 2004).

Mobility and safety

Patients with increased muscle atrophy present withmany changes in mobility. Referral to physical and occu-pational therapy should be initiated at first onset of func-tional decline and increased risk for falls at home. Declineof a patient’s function can occur at a rapid rate. Physicaland occupational therapy interventions and devices maytake significant time to be delivered to the patient. Rampsand other structural changes may need to be built to al-low the patient to continue to remain in the home. Avail-able resources should be considered when planning timefor physical or occupational therapy referrals.

Depression

Depression is common in patients with ALS. Patients withdepression are reported to have decreased QOL (Lou,Reeves, Benice, & Sexton, 2003). Therefore, depressionin ALS patients needs to be treated appropriately. In2008, one study suggested using the ALS-Depression-Inventory (ALI-12), a 12-question screening test, to eval-uate ALS patients for depression. This questionnaire isshorter than traditional questionnaires but equally aseffective in detecting depression in patients with ALS(Hammer, Hacker, Hautzinger, Meyer, & Kubler, 2008).Depression in ALS in most cases can be managed by pri-mary care providers. Some NPs manage depression fre-quently in the primary care setting, so depending ontraining, personal comfort, and state regulations, man-agement of depression can be initiated by the NP. Psy-chiatric referral should be initiated if symptoms cannotbe adequately managed.

Hospital admission

When ALS clinics are not available, or if symptoms be-come emergent, it may be beneficial to admit the pa-tient directly to the hospital for inpatient work-up. Whileat the hospital, the patient can be seen by all necessaryproviders in one visit as well. This is warranted whensymptoms are not stable, such as onset of falls, failure tothrive, inability to swallow, rapid loss of function of limbs,

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or respiratory compromise. Hospitalization will establishcare with multiple providers at one visit, decreasing ne-cessity of multiple individual visits. Contact the patient’sneurologist for guidance as well as any previously estab-lished providers, such as the pulmonologist. If care hasbeen established, be prepared to provide documentationof visits to multiple providers and recommend consultsbased on symptoms.

Complexity of patient

If the ALS patient has co-morbidities that collectively ex-ceed the knowledge and experience of the NP, the ALSpatient should be referred to a primary care physician,internal medicine specialist, or closely monitored by col-laboration of care with a physician. Consultation with theneurologist may also provide insight regarding the patho-physiology of ALS and possible effects on other systems orco-morbidities.

Misdiagnosis or unclear diagnosis

Signs that the diagnosis of ALS may be incorrect mayinclude the presence of sensory signs, sphincter distur-bances, visual disturbances, autonomic features, basalganglia dysfunction, or Alzheimer-type dementia (Li,Day, Alberman, & Swash, 1986). If symptoms that are notcharacteristic of ALS present, or if signs show resolution,refer immediately to neurologist for further evaluation ofthe diagnosis.

NPs should provide anticipatory guidance to the pa-tient with ALS. Working in collaboration with the manyproviders discussed above requires knowledge of eachspecialty in order to effectively communicate expecta-tions of care to the ALS patient. One study reported thatgreater than 44% of physicians spent less than 30 minon discussion at new diagnosis of ALS (Borasio et al.,2001). The NP can reinforce concepts of an ALS diagno-sis while managing care between specialist visits. Effec-tive communication with longer time spent with patientshas been shown to increase patient satisfaction with care(McCluskey, Casarett, & Siderowf, 2004). Involve the pa-tient’s family and support network whenever possible.Investigate what patients already know and what theywant to know. Reinforce that lifespan cannot be predictedwith certainty and some diagnosed with ALS live 10 yearsor longer. Discuss resources and support groups specificfor ALS. Provide patients with hope by reinforcing thatwhile there is no cure at present, research and drug trialsare ongoing and appropriate referrals may be made by thepatient’s neurologist. Afterwards, summarize the discus-sion verbally and in writing. More specific recommenda-tions for discussion by the neurologist are also available(Andersen et al., 2007; Miller et al., 1999).

Suggested research

Guidelines for ALS referrals for the NP have not beenclearly demonstrated. Utilization of NPs in the Ameri-can healthcare system is increasing, and evidence-basedguidelines defining appropriate care for patients with ALSby the NP may help to reduce costs and standardize care.Future research may evaluate ALS patient outcomes asthey relate to referrals initiated by NPs. Updated statisticson the number of NPs managing ALS patient care mayhelp further define effective care by the multidisciplinarycare team.

Summary

ALS is a degenerative disease impacting upper andlower motor neurons with an average mortality in 3–5years after onset of symptoms. Management of ALS bythe NP is complex, involving regular screening for newor changing symptoms and anticipating referrals. Collab-oration from a multidisciplinary care team is ideal. Refer-rals that should occur at first onset of diagnosis include aneurologist, pulmonologist, palliative care specialist, andlocal ALS organizations. Advancement of motor neurondegeneration and resulting symptoms will require regularsurveillance by the NP and the timely initiation of appro-priate referrals.

Acknowledgements

We thank Patsy Maloney, Kimberley Forthofer, HollyHitchcock, Briana Johnson, and Julie Rice.

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Documents

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