Coping with the “New Normal” after an ALS Diagnosis

Everyone responds differently when life throws him or her a curve ball, and an ALS diagnosis might be the fastest curve ball life has to offer.  Some respond by “hitting that ball back” and go on with life fairly quickly, while others may need more time to adjust to the news and come up with a plan.  There is no right or wrong way to feel when faced with this diagnosis.

Most of us have heard about the stages of acceptance, grief and loss.  These stages describe different reactions one might have, including denial, anger, bargaining, depression, and ending with acceptance.  Acceptance does not mean giving up on hopes or dreams.  It should be the first step in making the most of life with ALS.  There is much to be done to help someone live a fuller and enjoyable life.

The following tips, adapted from Neurology Reviews: October 2010, may help someone cope with ALS:

  • Take Time to Adjust. Being diagnosed with ALS is nothing less than shocking.  Take time to absorb the information and understand what to expect.  Allow yourself time to work through emotional reactions, such as denial, anger, sadness and grief.
  • Be Hopeful. Attitude is everything.  Try to remain hopeful. Be positive. Don’t let ALS take away your spirit. Don’t let your illness define who you are.  Try to think of ALS as only one part of your life, not your entire identity.
  • Think Beyond Physical Changes. You can look at ALS as a slow death or as an opportunity to enrich your life and make the most of the time you have: time to foster deeper connections with family and friends and broaden your spiritual awareness.
  • Seek Early Treatment.  Many symptoms can be reduced with simple treatment.  Often these treatments can ease the effects of disease progression.  Ignoring manageable problems can make a difficult situation worse.
  • Take Charge of Your Care. Physicians, other professionals on your healthcare team, and family can help with healthcare decisions, but remember that you are in charge throughout your illness.  Don’t let others dictate your care. If you healthcare provider hasn’t already done so, request that he or she refer you to the nearest ALS clinic.  Your providers will also be able to help you in other ways in the future, such as signing your application for disability and social security benefits.
  • Engage Family and Friends.  Life with ALS can trigger overwhelming emotional reactions.  Keep lines of communication open, so that you are comfortable expressing your feelings. Encourage your family and friends to express their feelings as well.
  • Join a Resource/Support Group. You don’t have to face this alone. You can get a lot of support and useful information from others who have faced this disease. Your family and friends may also benefit from a support group devoted to caregivers.
  • Plan Ahead. Planning for the future allows you to be in control of decisions about your life and your care. Work with your healthcare team and family to formulate plans for life-extending treatments and end-of-life care. Make a living will and discuss it with your family.  See Aging with Dignity for more details on living wills and Five Wishes.  You may also want to consider formalizing advance directives and assigning power of attorney.

The information from this blog post was excerpted from the Living with ALS Resource Guide: After the Diagnosis: Coping with the “New Normal”.

The National ALS Registry

The National ALS Registry is the single largest ALS research project ever created and the only population-based registry in the U.S.  The registry collects information and demographics on people with ALS, connects patients to clinical trials, and funds ALS research.  The purpose of the registry is to learn more about who gets ALS and what causes ALS.  This will hopefully give scientists a more complete understanding of the disease and enable them to find a cure.

Get the Facts- ALS registry

The National ALS Registry also includes a Biorepository that stores samples for future research.

Biorepository

Recently, the Registry was used for the first time to recruit patients and caregivers into a landmark patient-focused drug development survey that captured important information on the burden of ALS across multiple dimensions, including: symptom experience, quality of life, health care resource utilization, and emotional well-being.  Other important advances made possible by the ALS Registry include attaining a 96 percent enrollment rate for registrants to be notified about ALS research opportunities (more than 100,000 such notifications being sent for over 30 clinical trials) and research funding for 13 institutions to examine what factors may contribute to ALS.  ATSDR recently released the third report on the results of the data gathered by the National ALS Registry.  You can read the report here.

If you are interested in joining the National ALS Registry or learning more about the latest news from the Registry, please visit https://www.cdc.gov/als/.

It is vital that the National ALS Registry continues to be fully funded.  Congress has started work on the Fiscal Year (FY) 2019 appropriations process and Representatives Eliot Engel (D-NY) and Peter King (R-NY) are circulating a bipartisan Dear Colleague letter in the House of Representatives (see it here), urging their colleagues to support $10 million in funding for the Registry.  The deadline for this letter has passed, but there is still more you can do to help make sure your Representatives are aware of the importance of issues affecting people with ALS.

Visit the ALS Association’s Advocacy Action Center to learn about the latest updates and news about legislative and regulatory issues being addressed by The ALS Association and to sign up to receive alerts when your action is needed to support people living with ALS.

Living with ALS: Keep Your World Large

By Julia Henderson-Kalb, OTD OTR/L and Elissa Held Bradford, PT, PhD, NCS

What do you want to be able to do in a day? What activities are important to you? Everyone craves activity. It is part of our human experience (1). However, oftentimes when people are diagnosed with a disease like ALS, their world tends to shrink. They might isolate themselves, stay at home the majority of the time, and stop doing the things they love to do in exchange for activities that aren’t very meaningful to them, like watching TV for hours on end. As part of the therapy team at Saint Louis University’s ALS Certified Center of Excellence, one of our priorities is to help people diagnosed with ALS understand how to keep their world LARGE so that they can enjoy meaningful activity for as long as possible.

What causes the world to shrink for someone living with ALS? It might be physical. The loss of mobility due to muscle weakness, balance issues, or low endurance can make it more difficult to get from point A to point B. Getting into and out of a car or using public transportation can be a problem. Once you arrive at your destination, maneuvering around might be challenging for many reasons-if there is a great distance to cover, the terrain isn’t smooth, or there isn’t enough space to move comfortably, to name a few. Loss of fine motor coordination and gross motor coordination can make completing daily activities more complicated.

Other causes of isolation from the world might not be as physically obvious but are no less influential. People living with ALS often experience feelings of depression, loss of control, and loss of “sense of self” when they are having difficulty or no longer able to complete daily activities in the way they once did (2). A common example is no longer going out to eat with family and friends, despite enjoying and participating in this activity frequently previously. When searching out the reason for this change, it is often embarrassment related to challenges with eating such as dropping utensils or coughing a lot. Furthermore, the loss of ability to verbally communicate with others has been found to be linked to a decrease in quality of life (3).

So what can be done to overcome these barriers and keep your world LARGE for as long as possible? There are several strategies to be considered to stay engaged in meaningful activities.

1.    Exercise/Physical Activity

While it might not be right for everyone with ALS, studies have shown that moderate-intensity exercise helps to maintain the strength you already have. If a person with ALS is not active, greater weakness can occur on top of the weakness caused by the disease itself (4). Prior to starting an exercise program, it’s important to talk with your doctor. Referral to a physical therapist or occupational therapist (either at an outpatient clinic or through home health) will ensure that the exercise program is appropriate for your needs and will avoid further damage to your muscles.

2.    Activity/Environmental Analysis

Sometimes the difference between being able to complete a beloved activity or not is simply a matter of changing the approach to how you do it. Sitting rather than standing while cooking can reduce the amount of energy it takes. Moving gardening items to easily accessible locations and creating a raised garden bed can allow you to garden with decreased risk of falling or using too much energy on the task.  An environmental assessment by an occupational or physical therapist can make your home or office safer and more manageable. An occupational therapist can observe you completing a specific task and give you recommendations on how to make the task completion easier.

3.    Equipment

There is a piece of medical equipment for just about every issue you can think of, and that includes helping you complete your desired activities despite most physical limitations. Adaptive equipment can be used to help you do anything from brush your teeth to drive a car. Insurance covers a few items, but many have to be paid for out-of-pocket. It is recommended that you discuss equipment options with an occupational or physical therapist so that you get the items that best fit your needs. The therapist might have an idea of how to contain cost by equipment by using something you already have or can put together at home. If you do need to purchase equipment, it is a good idea to search the internet for best pricing options. The ALS Association also has a loan closet that might have the piece of equipment you want, which can reduce cost.

When walking becomes difficult due to decreased strength, balance, or endurance, mobility devices offer options for moving around the home or in the community in a safe and effective manner. Devices from straight canes to power wheelchairs can make it possible for you to get to where you need to go with a reduced risk of falls and decreased expenditure of energy. It is recommended that you see a physical therapist or occupational therapist in order to determine what type of device is best for your particular need.

4.    Delegation

When in doubt, delegate it out! ALS is a disease that allows you to only have so much energy in your energy bank every day, and once it is gone, it’s gone. Choose the activities that are most important to you and then ask other people to help you with the other tasks. If you really want to go to your grandson’s baseball game but know that you only have so much energy in the day, someone else might need to fold the laundry. If you want to go to lunch with friends, you might need help getting dressed and ready for the day. Consider your body and your energy level. In most cases, your friends and family want to help in any way they can. Use that help as it is offered so that you can do the things that matter most to you. Remember it takes a village. We all have different roles to play at different times in our lives.

5.    Focus on What Matters Most

ALS is a chronic, progressive disease. It changes the way a person is able to move, talk, eat, and even breathe. But it cannot take away who you are as a person and what you mean to those who love you. What matters most is different for every individual. But knowing what that is and really focusing on it can free person from worrying about other things. For example, if time spent with friends and family is what matters most to you, slurring your speech in front of them might seem less important than not seeing them at all. If being out in nature is most important to you, then does it really matter if you walk or ride in a power chair to get there? Letting go of what you can’t do and allowing yourself to focus on doing what you love regardless of your limitations can create a greater sense of control, identity, and appreciation of life (5).

Elissa Held Bradford, and Julia Henderson-Kalb are part of the multidisciplinary care team at the ALS Certified Center of Excellence at Saint Louis University Hospital.


References:

(1) Boyt Schell, B.A., Gillen, G., & Scaffa, M.E. (2014). Willard and Spackman’s occupational therapy (12th ed) Lippincott, Williams, & Wilkins: Philadelphia, PA.

(2) Radomski, M.V. & Trombly Latham, C.A. (2014). Occupational therapy for physical dysfunction (7th ed). Lippincott, Williams, & Wilkins: Philadelphia, PA.

(3) Felgoise, S.H., Zaccheo, V., Duff, J., & Simmons, Z. (2016). Verbal communication impacts quality of life in patients with amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 17, 179–183.

(4) Bello-Haas, V.D. & Florence, J.M. (2013). Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease (review). The Cochrane Collaboration: Johne Wiley & Sons, Ltd. doi: 10.1002/14651858

(5) Foley, G., O’Mahony, P., & Hardiman, O. (2007). Perceptions of quality of life in people with ALS: Effects of coping and health care. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 8(3), 164-169.

Technologies That Make Life Easier for Caregivers

By June Duncan

Assistive technologies for the disabled have come a long way since the advent of automated wheelchairs and hearing aids. Smart technology has opened a new world of possibilities for people with disabilities and those who care for them. Smartphones, tablets, and an ever-growing list of apps are helping the disabled improve mobility, communication capabilities, speech, and vision. One of the greatest benefits is the ability to foster better communication between the disabled and their caregivers. Today, technology enables disabled persons to care for themselves more effectively, which makes things easier on caregivers, who often suffer from fatigue and burnout. Technology also helps give disabled persons more independence and a greater sense of confidence and control over their own environment.

Speech

Tablets have become a valuable tool, helping people with speech disabilities communicate through apps that provide access to an extensive list of words. Augie AAC and Speak for Yourself give people access to more than 13,000 words with the tap of a finger. Both apps transform your tablet into a device for people who have autism, cerebral palsy, and other conditions that constitute a complex communications need. It even aids the development of verbal speech.

Smartphone Navigation

Smartphones today are dynamic tools for communicating and accessing information. Speech recognition technology allows people with visual impairments or learning disabilities to use smartphones. It’s an especially useful tool for people with dyslexia. It reads texts and emails out loud and even allows users to use social media sites like Instagram. Apple has been a pioneer for decades in different forms of smart technology aimed at helping disabled people live more independent lives. Its assistive touch technology helps people with motor-control problems use an iPhone or iPad without having to use their fingers in complex ways to size the screen and adjust touch-based controls (like volume control) more easily. Voice-activated systems can also help visually impaired people control their home entertainment systems.

Notification Light System

One especially useful notification system can be customized to communicate different messages to hearing-impaired individuals through a system of multicolored lights that can alert them, for example, when someone comes to the door, when it’s time to leave for school or work, or when dinner is ready. The system can be integrated with smart home-control technologies like Amazon’s Echo. There is also a two-way communication technology that can translate sign language into text for people who are not hearing impaired, a useful tool for helping caregivers and care subjects communicate.

Cognitive Challenges

Individuals with cognitive challenges and memory impairment sometimes have trouble taking care of themselves, forgetting to take medications or prepare meals. A program called Lively uses sensors to track the daily movements and behavior patterns of cognitively disabled individuals. Caregivers can log into a web page to track their care subject’s movements and activities, making sure he or she is sleeping and eating properly and performing other normal daily activities. Still more sophisticated Internet-based smart technologies allow wheelchair-bound individuals to interact with their surroundings in ways that wouldn’t be possible otherwise. Lights, blinds, thermostats, appliances, sprinklers, and even pet feeders can be controlled with a smartphone, as can door locks and home security systems.

Technology is helping improve the lives of people with many different disabilities. It’s also beneficial to caregivers who live on-site or provide care remotely, protecting loved ones who are highly vulnerable to accidents or mishaps caused by their disabilities. Communication is the key, and there are many apps and systems that can make it easier for individuals with visual and hearing impairments to communicate with their caregivers.


June Duncan is the primary caregiver to her 85-year-old mom and the co-creator of Rise Up for Caregivers, which offers support for family members and friends who have taken on the responsibility of caring for their loved ones.  She is passionate about helping and supporting other caregivers and is currently writing a book titled, The Complete Guide to Caregiving: A Daily Companion for New Senior Caregivers, due out in Winter 2018.

For more information and resources for caregivers, please visit www.alsa-stl.org.

 

Caregiver Confidential: It Takes a Village

While reading online the other day, I came across a popular saying that I hadn’t seen in a while: “It takes a village.” Although familiar with its general meaning, which refers to the communal raising of children, in a broader context, can’t the concept of a “village” also apply to caregiving for a loved one with a terminal illness? Doesn’t it take many individuals—caregivers, medical and social services professionals, friends, and family members, all working together—to provide the best possible care for an ALS patient?

As a caregiver for a loved one with ALS, you constantly interact with a myriad of individuals at various times during the course of the disease. A single caregiver, no matter how dedicated, cannot provide all the requisite care. While acknowledging that you cannot go it alone, it can also be difficult accepting that you need help. After all, who can you trust to perform the vital functions that impact the health and well-being of your loved one? And who wants a parade of strangers coming into one’s home constantly, creating even more stress?

Reflecting on my own experience, I was fortunate that my husband Brian was highly functional for the first 3 years of his disease. During that time, I increasingly helped him with activities of daily living as his ALS progressed, but solo caregiving was manageable. However, I was totally unprepared for what lay ahead when Brian’s breathing and mobility status suddenly changed in October 2008.

As I related in an earlier post, Brian collapsed at home in October 2008 and was rushed to the hospital, spending almost 2 weeks in the ICU. During that time, I had a crash course in use of the BiPAP, along with other caregiving functions. Simultaneously, I was arranging for agency help (4 hours of morning caregiving each day) to start when Brian was discharged. It was a time of tremendous change and transition for Brian, our daughter Leah, and me.

I vividly recall feeling overwhelmed by all the changes that were swirling around me. I felt that my home was turned into a mini-hospital. There was the constant stream of people invading our space: PT, OT, nurses, vendors, a physician who made house calls, and a variety of caregivers, some of whom were not competent or caring. Luckily after some initial missteps, our home care agency sent Michelle and Christina. They were compassionate, patient, and knowledgeable. Michelle took care of another ALS patient in the afternoons, so she was well versed with the unique needs and challenges of the disease. Both caregivers contributed to the quality of Brian’s life and were supportive of both Brian and me. We became a team. Eventually, Christina found another job, but our agency sent another wonderful caregiver, Lilly.

Our village was small. We did not have any family in St. Louis. My brother, who was close with Brian, drove down from Chicago every 3 weeks or so, and Brian’s sister Joyce came every 6 weeks. Our daughter Leah moved home after college graduation to help care for her dad. Many of Brian’s colleagues visited regularly while he could still speak and go without the BiPAP for short periods, and friends and other out-of-town family members came when Brian was feeling well enough. At the end, Michelle and Lilly were there for us, even on the morning of Brian’s passing. They stayed and cared for him until the funeral home arrived.

Michelle, Christina, and Lilly saved my life. They made the unbearable more bearable, the impossible a little easier. They were my village—my rock, my foundation, my family. They treated Brian with dignity and respect, and I am profoundly grateful to them.

Who is or was in your village? What were the biggest challenges you faced as your loved one needed more caregiving assistance? Please share your experiences and thoughts on any aspect of caregiving, current or previous, in the comments section. Let’s continue the conversation.
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Idelle Winer was primary caregiver to her husband Brian, who passed away in 2010 after a 5-year battle with ALS. A retired medical publishing professional, Idelle offers her unique perspective on a variety of caregiving topics in our feature Caregiver Confidential.

Understanding Insurance and Benefits When You Have ALS

Navigating the maze of health insurance is challenging for anyone and can be particularly overwhelming for people with ALS.  We’ve compiled some tips and information that may help save you time and energy spent figuring out the complicated web of insurance benefits.

Questions to Ask Your Health Insurance Provider

A diagnosis of ALS can certainly be overwhelming and many ask, “where do I begin?” What will I need? What does my insurance cover? Below is a list of questions you should ask your insurer:

  • Is there an annual deductible?
  • Is there an annual out-of-pocket expense limit or maximum? If I meet my limit, does my coverage increase and to what extent?
  • Do I have a major medical plan? Is there an annual or lifetime maximum?
  • Do I need to complete any claim forms?
  • Am I subject to pre-existing condition regulations?
  • What does insurance cover/not cover for a person with ALS?
  • Does my plan cover durable medical equipment?
  • Does my plan cover prescription drugs?
  • Does my plan cover home health coverage? Is there a preferred home healthcare agency I must use?

Social Security Disability Insurance (SSDI)

When you are diagnosed with ALS, making a decision to stop working and apply for SSDI may be difficult, but the process for applying for it and Medicare should be relatively easy.  You can file for benefits once you are no longer able to work, or if your current Substantial Gainful Activity (SGA) is below the allotted amount.  The easiest way to file for SSDI is to do so online at http://socialsecurity.gov.  You will need the names and addresses of your doctors, as well as a very brief outline of the work you have done in the last 15 years.  If you are unable to apply online, you can call the Social Security Administration at 1-800-722-1213 or you can apply in person at your local social security office.  Because ALS is on the Social Security Administration’s list of Compassionate Allowances, Social Security will grant you disability status immediately once an application is filed.  There is a full five-month waiting period, so even though Social Security will award disability status immediately, no monetary benefits are paid until after there has been no work for five full months.  Once you are approved for benefits and the full five month waiting period has passed, you will receive monthly monetary payments for you, and in most cases, your minor children and a spouse who cares for your children under age 16.  Your Social Security statement and an on-line benefits estimator can be obtained at http://ssa.gov/myaccount.

Supplemental Security Income (SSI)

Supplemental Security Income provides cash to meet basic needs for food, clothing and shelter to those age 65 and older, blind, or disable who have little or no income.  Individuals and their children may be eligible for SSI even if they have never worked or have an insufficient insured status for SSDI.  SSI is a Federal income supplement program funded by general tax revenues, not Social Security taxes.  Each individual who applies for SSDI is also screened for SSI benefits.  SSI payments may be available during the usual 5-month waiting period before SSDI cash benefits begin. Those eligible for SSI may be automatically eligible for Medicaid benefits.

Medicare and Medicaid

Medicare is a federal health insurance program administered by the Centers for Medicare and Medicaid Services (CMS).  Medicare is available to persons of age 65 and older (and their spouses) and persons eligible for SSDI.  Medicare Part A (hospital insurance) helps pay for care in hospitals as an inpatient, skilled nursing facilities, hospice care, and some home healthcare. This coverage is free to SSDI recipients.  Part B (medical insurance) helps pay for doctors’ services, outpatient hospital care, and some other medical services that Part A does not cover, such as physical and occupational therapy, and some home healthcare. Part B helps pay for these services and supplies when they are medically necessary.  There is a monthly premium for this coverage.  If an individual is also a Medicaid recipient, their monthly premium for Medicare may be paid by their Medicaid benefits.  Please refer to the official Medicare website for the most up-to-date information at http://medicare.gov/Coverage/Home.asp or call 1-800-MEDICARE.  Medicaid provides health coverage for low-income people, families and children, pregnant women, the elderly and people with disabilities.  Federal and state governments jointly fund Medicaid.  For more information on Medicaid contact your state’s Medicaid program.   If you live in Missouri visit https://mydds.mo.gov and if you live in Illinois visit http://illinois.gov/hfs.

For a person with ALS, the most important part of healthcare planning is selecting the best health insurance for their needs.  Discussing health options with an insurance counselor is a great way to think through and plan for your needs to find the most appropriate coverage when you need it.  Your State Health Insurance Counseling Program has expert volunteers who can assist you free of charge.  Go to https://www.medicare.gov/contacts/#resources/ships for more information and as always you can contact The ALS Association St. Louis Regional Chapter through our website or by calling us at 314-432-7257.

The information from this blog post was excerpted from Living with ALS Resource Guide 5: Understanding Insurance and Benefits When You Have ALS.

 

Diagnosing ALS

ALS is very difficult to diagnose — often taking up to a year from when the first symptoms appear until a definitive diagnosis is reached.  Why is diagnosing a person with ALS such a challenging and long process?  Here are a few reasons that can contribute to the delay:

1. There is no clear cause of ALS.

Because there is no clear cause or trigger for ALS, and in most cases, no specific genes, it is diagnosed by asking disease-related questions and taking a history of the patient’s symptoms.  It is very important that a doctor with knowledge of ALS — such as a neurologist or a specialist with experience with ALS — conducts this examination to see the symptoms and clues that need to be observed together to confirm the diagnosis, as well as to rule out other possible conditions.

2. ALS is usually thought of as something else at first.

People with ALS experience a wide variety of symptoms, especially early on in the progression of the disease.  One person may experience muscle twitches or weakness and think at first that they just overexerted themselves at the gym, and another may experience slurred speech and think they may have had a stroke.  The way symptoms present can lead to many areas or specialists before being referred to a neurologist. It’s  typically only when more symptoms appear that ALS is thought of as a possibility.

3. There is no specific diagnostic test for ALS.

An ALS diagnosis is a process of exclusion, which means that doctors have to test for other possible explanations to rule out other diagnoses.  A comprehensive diagnostic workup includes most, if not all, of these procedures:

  • blood and urine studies
  • x-rays, including MRI
  • electrodiagnostic tests including electomyography (EMG) and nerve conduction velocity (NCV)
  • spinal tap
  • muscle and/or nerve biopsy
  • myelogram of cervical spine

When physical and laboratory examinations indicate that a person has weakness caused by the loss of motor neurons, worsening over time and spreading to different parts of the body, along with test results showing a decrease in muscle and nerve function, a diagnosis can be confirmed.  It is always important to get a second opinion, especially when given an ALS diagnosis, because of the wide variety of symptoms and different tests that can be done.

For more information on diagnosing ALS, please see the ALS Association St. Louis Regional Chapter’s website.

Currently, promising research is being done to diagnose ALS earlier and faster with the help of biomarkers. Biomarkers are biological measures that help identify the presence or rate of progression of a disease or the effectiveness of a therapeutic intervention.  Biomarkers can be molecules derived from bodily fluids (blood and cerebrospinal fluid), an image of the brain or spinal cord, or a measure of the ability of a nerve or muscle to process electrical signals.  This research could identify a test that could be done to find an ALS diagnosis earlier in someone’s disease progression, enabling them to start drugs and therapy faster. 

For more information on biomarker research, see The ALS Association’s Research page.