Houseman, Gail; Walsh, Susan; Brothers, Allyson; Lyter, Judy; Simmons, Zachary; Klapper, Jennifer
Purpose and Scope of Article
The purpose of this article is to provide education for nurses involved in caring for patients who have both ALS and FTD; the emphasis being on helping the caregiver effectively cope with the symptoms of FTD. Symptoms of both ALS and FTD are presented. Specific behavioral and cognitive symptoms of FTD are noted. Possible nursing interventions and instructions are outlined for both issues.
Researchers in the previous decade have concluded that there is a link between amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). In fact, it is becoming more widely accepted that frontotemporal dysfunction is clinically and pathologically related to ALS in a substantial number of cases (Strong et al., 2009). It is estimated that approximately 10%–15% of people with ALS also have FTLD and that almost half may have a milder form of cognitive impairment (CI; Lomen-Hoerth, Anderson, & Miller, 2002; Lomen-Hoerth et al., 2003). Neuropathological studies show that it is the frontal and temporal lobes that are primarily affected in patients with ALS and cognitive dysfunction, although the pathological findings can be as heterogenous as the clinical symptoms associated with this disorder (Tsermentseli, Leigh, & Goldstein, 2012). Bulbar onset ALS may increase the likelihood of CI and FTLD (Zimmerman, Eslinger, Simmons, & Barrett, 2007). The symptoms of ALS, which involve eventual weakness of all voluntary muscles, may precede the symptoms of FTLD and CI. It is also possible that a person develop CI after the onset of ALS symptoms. For years, it was believed in both the medical and lay communities that, although ALS causes complete paralysis of the body, the mind remains intact. Unfortunately, for people with ALS and their loved ones, this belief is not always true.
The presence of executive function deficits in ALS has been linked to shorter survival, greater noncompliance with treatment recommendations, and heightened caregiver distress (Elamin et al., 2011; Goldstein, Atkins, Landau, Brown, & Leigh, 2006; Olney et al., 2005). For these reasons, cognitive-behavioral impairment in patients with ALS is important to recognize and address. This article will begin with a review of the subtypes of FTLD, along with the symptoms associated with each. We then go on to discuss the ways in which nursing professionals can provide support to patients and families who are facing the co-occurrence of ALS with CI. The supports addressed include providing education, addressing safety concerns, managing difficult behaviors, and supporting the decision-making process throughout the course of the disease.
FTLD is a progressive disorder that involves the degeneration of the frontal and anterior temporal lobes of the brain, resulting in changes in personality and behavior and/or difficulties with speech. FTLD is a broad term that includes three subtypes:
* frontotemporal degeneration (FTD)—characterized by problems with thinking and/or behavior;
* progressive nonfluent aphasia—problems with language including nonfluent speech with grammatical errors, using phrases in place of sentences, and problems with word finding; and
* semantic dementia—problems with language that involves empty speech, impaired understanding of word meaning, and/or ability to recognize objects or faces.
The focus of this article will concern the person who has both ALS and FTD. FTD is the most commonly seen subtype in the ALS population (Neary et al., 1998). It is not clear if a person with ALS can develop more than one subtype of FTLD (Miller et al., 2009). It is important to note that cognitive-behavioral changes in the ALS population occur on a spectrum from mild to those that meet the Neary criteria for FTD (see Table 1). Mild cognitive-behavioral changes may or may not progress into FTD. The two most significant areas of concern for the person with FTD and their caregiver are problems with cognition (otherwise known as ALSci) and behavior (otherwise known as ALSbi or bvFTD), as noted in Table 2.
ALS is a progressive, neuromuscular disorder of both the upper and lower motor neurons, resulting in eventual paralysis of all voluntary muscles, including the muscles involved in speaking, swallowing, and breathing. The average life expectancy after diagnosis is 2–5 years; however, approximately 10% of all people with ALS live greater than 10 years (Mitsumoto, 2001). The usual cause of death is respiratory failure. Average life expectancy for the person with both ALS and FTD is shorter than with ALS alone (Chio et al., 2009). Reasons for this decrease in life expectancy are not clear. There is some suspicion that the person with both ALS and FTD is less likely to utilize noninvasive positive pressure ventilation (BiPAP) or percutaneous endoscopic gastrostomy (PEG). Noncompliance with treatment may also play a factor. The ALS patient with CI may refuse to use necessary equipment, such as a walker or wheelchair, resulting in fatal falls. In addition, people with FTD are at greater risk for injury, secondary to poor insight and lack of judgment.
Implications for the Caregiver
The caregiver of a person with both ALS and FTD has the double challenge of caring for a person with two disabling, progressive illnesses. Caregiver distress has been shown to be directly related to the degree of executive function decline in the ALS patient (Goldstein et al., 2006). Caregivers of the person with both illnesses have been shown to be in the highest risk group of all caregivers for stress. This includes caregivers of people with Alzheimer disease and people with ALS who do not have CI (Riedijk et al., 2006). Although the general public has most likely heard of “Lou Gehrig disease,” FTD is not an illness that receives much media attention. Caregivers most likely will have a lack of knowledge about FTD and its connection with ALS. (Many healthcare workers may not be aware of a relationship between the two illnesses.) Caregivers may not understand the difference between FTD and Alzheimer disease. They may have negative thoughts and feelings about their loved one’s CI—anger at the disease or related to their loved one’s lack of empathy, embarrassment by their loved one’s behaviors, and/or feelings of isolation.
The nurse has the opportunity to support the caregiver in the following domains: providing education, addressing safety and environmental concerns, managing negative behavioral issues, and assisting with decision-making issues. Suggested interventions for each topic are discussed below and are derived from an extensive literature review (using PubMed, MedLine, and Cochrane Library of Evidence-Based Reviews), expert opinion from ALS clinicians and therapists of two ALSA-certified Centers for ALS Care (The ALS Clinic at Penn State Milton S. Hershey Medical Center and the ALS Center at the Penn Comprehensive Neuroscience Center at Pennsylvania Hospital), and 11 ALS-FTD caregiver interviews that were conducted as part of a related study.
Providing Education About FTD
Because not many caregivers will be familiar with FTD, nurses can teach the caregiver about the relationship between ALS and FTD, including typical problematic behaviors or symptoms. It is important for the nurse to emphasize that there are actual brain changes involved in FTD, that these brain changes will most likely worsen over time, and that the person with FTD cannot help his or her problematic behaviors and/or cognitive challenges. Lack of empathy appears to be a particularly disturbing symptom for caregivers to digest. They can be taking care of their loved one 24/7, but it may go unnoticed by the person with both ALS and FTD. Again, educating the caregiver that this symptom is because of an organic change in the brain may help decrease caregiver anger and frustration.
Nurses can also instruct the caregivers regarding the difference between FTD and Alzheimer disease. Many people outside of the medical world lump all diseases related to CI as being “Alzheimer” or “Old-timer” disease. Although both FTD and Alzheimer disease are progressive and result in organic changes in the brain, Alzheimer disease primarily involves problems with memory and usually occurs after age of 65. FTD strikes most people before age of 65 and often results in changes in thinking and behavior.
Nurses can support the caregiver to get support; encourage the caregiver to identify and allow family and friends to help care for the person with both ALS and FTD; and help identify other possible sources of support, for example, use of in-home care, respite care, adult day care, long-term care placement, hospice care. Additional resources may include the following: Family Caregiver Alliance (www.caregiver.org), ALS support groups, FTD support groups, biannual FTD Caregiver Conferences, National ALS Association (www.alsa.org), University of California, San Francisco’s FTD website (http://memory.ucsf.edu/ftd/), and the Association for Frontotemporal Dementia (www.theaftd.org).
Addressing Safety and Environmental Concerns
People with both ALS and FTD are less likely to follow treatment recommendations. Creating a structured, predictable daily routine, one that is individualized to the person’s likes and interests, may help. Nurses can encourage the caregiver to implement changes over time before they are absolutely necessary. In addition, the caregiver should eventually assume responsibility for the following:
* medication management;
* driving—even if driving is not impaired by physical symptoms of ALS, the person with ALS and CI/FTD may not be safe on the roads because of poor judgment, impulsiveness, and problems with anger management;
* managing finances—caregivers may need to freeze financial accounts, run credit checks regularly, disconnect landlines, use one cell phone with a password, and install child controls for TV and the Internet;
* use of power tools and equipment, stoves, and ovens; and
* locking up/keeping out of sight anything that may be harmful such as guns and firearms, medications, unsafe foods (if the person has a problem with binge eating and/or choking), car keys, and doors.
Managing Behavioral Concerns
Nurses can help the caregiver look for patterns in the patient’s behavior or note times of day in which the behavior is more problematic. They can encourage the caregiver to keep a log and evaluate current behavioral interventions that the caregiver is utilizing. They can suggest that the caregiver consider the following potential interventions:
* use a calm voice at all times;
* use simple words, ask yes/no questions;
* limit choices, such as, “we are going to eat now,” instead of asking the loved one if he or she is ready to eat;
* praise desired behavior;
* for food stuffing: supervise eating—make sure the patient swallows all food in the mouth before putting any more in;
* find soothing rituals that help like hand massage, listening to music, time with a pet;
* do not argue or try to reason with the person affected—he or she is unable to reason effectively; change the subject if the patient is getting upset about something in particular;
* find a walking buddy for restless times;
* adjust sound/lighting to limit agitation;
* create picture board if there is trouble in communicating; and
* avoid physical restraint—this may include any object that the affected patient feels is restraining, such as a noninvasive mechanical ventilator (BiPAP) or suction machine.
Assisting With Decision-Making Issues
At some point, most people with ALS (along with their loved ones) need to decide whether or not they want to pursue more aggressive forms of medical treatment, such as percutaneous gastrostomy tube (PEG) placement and, in particular, invasive mechanical ventilation. For the person with both ALS and CI, it is important that these issues be discussed and decided upon early-on during the course of both illnesses, given that most people with both ALS and significant CI or FTD will demonstrate poor judgment. The nurse should encourage the caregivers to openly discuss with their loved one his or her advanced directives. If the person affected elects to pursue invasive medical treatment, then the nurse should ascertain at what point, if ever, the person wishes to be taken off of life-supportive measures. Some people with ALS elect to discontinue tube feeds and/or ask to be weaned off of respiratory devices once they can no longer effectively communicate.
Once a decision has been made regarding advanced directives, the nurse should encourage the appropriate loved one to pursue both a legal power of attorney (for financial concerns) and a medical power of attorney (for healthcare decisions). The medical power of attorney’s job is to understand the patient’s advanced directives and communicate to appropriate healthcare professionals, as needed. An attorney who specializes in elder care, disabilities and estate planning, may be of help in preparing the legal documents.
Of major concern is the person with both ALS and CI who does not have a living will and/or advanced directive. If this person’s judgment is significantly impaired, then the primary caregiver would need to ascertain what their loved one would have chosen if not cognitively impaired and, most likely, seek the legal help of an attorney.
Further Points to Consider and Summary
Caregiver stress and frustration in the face of a chronic, progressive illness with no effective treatment is often high. The combined load of heavy physical care giving needs because of ALS, in combination with the complexity of caring for a loved one with an unfamiliar cognitive decline affecting, among other things, language and overall personality, creates a heavy coping burden on caregivers. As with ALS, there is no cure for FTD, and no medications to slow the progression. Like ALS, the medical treatment of FTD is primarily geared toward symptom management; this may include consultation with a psychiatrist to help manage agitation, irritability, and depression. The losses inherent to FTD challenge common notions of “dementia,” especially the loss of logic, empathy, and appropriate inhibitions, and can be profoundly disturbing for loved ones to experience. This becomes especially confusing when loved ones witness the preservation of other cognitive functions, such as recognition of others, of place, personal desires, and expression of emotions. Support to caregivers of loved ones with ALS/FTD primarily includes the following:
1. Education about the disease process, understanding the organic brain change, and lack of a personal intent of the patient to be hurtful, unsympathetic, or difficult.
2. Managing the most challenging symptoms.
3. Assisting loved ones in setting realistic expectations and goals.
4. Providing on-going emotional support in coping with incremental physical and cognitive losses.
Nursing science on effective patient education indicates that most productive learning occurs with the content offered matches the immediate need: When the information is perceived as relevant. As caregivers experience new challenging situations with their loved ones as the illnesses progress over time, they will gradually be ready to participate in new phases of learning about managing ALS and FTD. The effectiveness of patient (and caregiver) teaching has been shown to improve with more frequent and longer-term contacts with the healthcare professionals providing the teacher (Pignone et al., 2003). The long-term relationship nurses establish with families, in which listening, assessment, and evaluation of the entire system are natural parts of nursing practice, ideally positions nurses to provide on-going, targeted, and meaningful education and support to caregivers. Dunbar-Jacob (2007) notes that, in changing health teaching, it is essential that “help with setting goals and…multiple interventions are necessary (Dunbar-Jacob, 2007). Nurses can help assist families to develop a revolving “tool kit” of unique structural and behavioral approaches to deal with their loved ones’ CI and most challenging symptoms. Once developed, nurses are in a key position to help the caregiver reassess the tool kit and respond to new challenges, with on-going support in creative problem-solving, compassion in the face of the losses they experience, and validation of their success as caregivers in the face of two extremely challenging illnesses.
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Keywords:: amyotrophic lateral sclerosis; behavioral impairment; behavioral management; caregiver; cognitive impairment; frontotemporal dementia