The economic impact of ALS

Article abstract-ALS is a w.:ogressive degenerative neuromuscular disease for which there is no known cause, treatment, or cure. The steady dise~se progression of muscle weakness eventually causes paralysis, disabling the patient. Day-to-day patient care and management most frequently fall to family members. The resultant financial burden can be enormous. We review financial issues related to the diagnosis, management of disease progression, and issues of life support. Cost-effective solutions are discussed. It is believed the key to reduction of costs is education of the health care community, patients and families, and third-party payers.
NEUROLOGY 1996;47(8uppl 2):8126-8129

ALS is a progressive degenerative neuromuscular disease for which there is no known cause, treatment, or cure. ALS affects each person individually, both in the area of the body affected and the rate of progression of the disease. Most people (50%) die within 2 to 5 years after the onset of symptoms, although 20% live for 5 years and 10% live for 10 years. A few individuals have been reported to live as long as 20 years. 1 Diagnosis. The economicimpact of ALS may begin long before the actual diagnosis is made. The first signs and symptoms of the disease may be subtle, representing a relatively slowly progressive preclinical stage advancing over months or even years. Early symptoms may include muscle fatigue or reduced endurance, cramps; fasciculations, dysarthria, dysphasia, or shortness of breath.2 Because these can also be symptoms of a number of disorders, many people initially seek evaluation from their family practitioner, believing they have arthritis, a sprain, a pinched nerve, or typical signs associated with "old age." This may lead to patients seeking answers from a number of medical specialists and nontraditional medicine practitioners, resulting in thousands of dollars spent trying to find answers to their progressive disorder. There is no single definitive test to confirm the diagnosis of ALS. For many physicians, the diagnosis remains one of exclusion-a series of clinical and laboratory tests to rule out other diseases or conditions that may mimic ALS. It is not unusual in the United States for a neurologist to expend $10,000 to $20,000 investigating a patient who had presented with ALS in the hope of discovering a disorder that may mimic ALS. Tests include expensive procedures such as MRI; electrophysiologic studies; muscle, nerve, and bone marrow biopsies; and innumerable blood studies (table 1).2
From the National Organization Lake City, UT.

Physicians may be emotionally unprepared to give a patient the diagnosis of ALS, as they believe it is like giving someone a "death sentence." In the process, the physician may unintentionally expand the spectrum of diagnostic tests that prove to be unnecessary or refer the patient to yet another physician. Physicians without the specialized knowledge and experience of ALS may order diagnostic tests that would be .deemed unnecessary by the ALS specialist. As a consequence, the diagnosis is delayed and costs are increased. Patients often report having spent $20,000 ~n advanced diagnostic testing without ever receiving a diagnosis. In the past, delay in obtaining a diagn>sisdid not have any impact on the patient's medical outcome, but that is no longer the case. A delay in initiating drug therapy (Le., riluzole), supportive therapy (physical, occupational, speech, or the emotional therapy of support groups), or a delay in entering a drug study may lead to a worse outcome.3 Equally important is the emotional well-being of the patient and family who are severely stressed as more tests are performed and more physicians are seen without a name being put to the problem. Once a diagnosis is given, the patient and family can begin to deal with the disease. Until that time, they live in doubt and fear with an increasing mistrust of the medical community. Increased use of the World Federation of Neurology EI Escorial Criteria for the Diagnosis of ALS4as the standard diagnostic criteria for ALS should contribute to earlier diagnosis and may eliminate unnecessary and costly testing and provide increased opportunities for psychosocial intervention. Disease management. As the disease progresses, appropriate intervention and assistance play an essential role in enabling the patient to remain as independent as possible and have an optimal quality of
of Neurology (Ms. Forshew), University of Utah, Salt

for Rare Disorders (Ms. K1ein>:lqew Fairfield, CT; and the Department

Presented as a workshop at the Advances in Amyotrophic Lateral Sclerosis meeting, Orlando, FL, January 1996. Sponsored by RhOne-Poulenc Rorer Pharmaceuticals Inc. Address correspondence and reprint requests to Ms. Lynn M. Klein, Executive Vice President, National Organization for Rare Disorders, P.O. Box 8923, New Fairfield, CT 06812-8923. 8126 Copyright 1996 by the American Academy of Neurology

Neurologist Comprehensive history, physical, and clinical neurologic examination Clinical laboratory tests Multiple blood tests Urine studies, including 24-i:l-r, urine Spinal tap Electrophysiology EMG NCV Neuroimaging X-rays

Hospital bed (nonelectric)

Purchase Rental per month

$2,100 $215 $625+ $1,700+ $20,000 $1,500 $110 $95 $85 $400

Wheelchair Nonelectric Electric Patient lift Bedside commode Bath/shower chair Walker Ankle-foot orthotic Neck support collar Suction machine Augmentative communication equipment

Standard High-back/reclining High-back/reclining

$75
$200 $25-$20,000

CT
MRls Neuropathology Muscle biopsy Nerve biopsy Potential cost

life. There are many factors that affect persons with ALS as they attempt to adapt to the physical changes brought on by the disease. These include patients' personal perceptions of their immediate and long-term needs; acceptance of the current and future impact of ALS on their lives; and the drain on personal, social, and financial resources. All must be taken into consideration by members of the health care community when making recommendations to patients and their families.5 Because ALS affects each person differently, recommendations should be made according to individual patient need rather than stage of disease as patients may present with disability at any stage. The progressive nature of physical limitations often requires a wide variety of assistive devices, and careful consideration should be taken to address the patient's short- and long-term needs and to maximize their personal financial resources.5 The expense of essential basic medical equipment and assistive devices can be a major financial burden (table 2), particularly when there are limited or no insurance benefits to cover the cost. Most insurance companies have an annual deductible and a high out-of-pocket expenditure that must be met before major medical coverage takes effect. This out-ofpocket expense can be devastating to a family suddenly faced with the added financial burden of ALS. Medical equipment coverage varies among private insurance, Medicare, and Medicaid. Most--private insurance companies follow Medicare guidelines when determining which equipment will be covered. One example of an ALS patient's need is for communication devices.

Most ALS patients who develop dysarthria need augmentative communication equipment. This equipment may vary greatly in cost, ranging from $25 for a simple eye-blink board to $20,000 for a sophisticated computer system, with a median cost of approximately $6,500 for a basic laptop computer with ALS-specific software. Insurance companies offer minimal or no coverage for communication equipment, and there are few alternative resources available for patients. The ability to continue to communicate one's needs, thoughts, and feelings is a vital component of a person's dignity and quality of life. Patients, caregivers, and their speech therapist and/or social worker must become innovative when seeking funds to obtain communication equipment. Current policy of third-party payers, especially Health Maintenance Organizations (HMOs), often prohibits referral of ALS patients outside of their network. Education of the insurance industry is needed to show that referrals to ALS-specific clinics and specialists can save costs by providing timely diagnosis and coordinated disease management. Nutritional maintenance intervention is a perfect example of how referrals to an ALS-specific clinic with the team approach to disease management can be cost effective. A feeding tube placed early rather than late can mean the difference between an outpatient rather than an inpatient hospital stay. Educational programs for case managers may be an excellent method to affect a change in this policy. Life-support issues. As the disease progresses, so does the cost of care. The two issues that affect survival are the ability to maintain adequate nutrition and respiration. Maintaining adequate nutritional intake often becomes difficult when patients begin to experience dysphagia and weight loss. This necessitates decisions regarding alternative nutrition, including dietary changes, enhanced swallowing techOctober 1996 NEUROLOGY 47(Suppl 2) 8127

PEG tube surgery Costs per month Feeding pump Purchase Rental Food (Ensure, Jevity, Pulmocare; etc.l Supplies (tubing, syringes, etc.) Total cost per month Total cost per year case cost, Los Angeles,_CA, 1995. Source: Faust Medical Suppfy, HoustOn, TX. $700 $220 $250
$30

Bi-Pap/C-Pap Rental/mo Purchase Nasal mask (2 per year

@

$400

$2,000 $60 each) $120 $25

$435

Supplies (tubing, seals, etc) Total cost per month* Total cost per year* Invasive Ventilator Rental/roo Purchase

$500 $6,000

$5,220

* Actual

$800 $8,000-10,000

niques, and feeding tubes. As the muscles of the respiratory system are affected and breathing difficulties are experienced, decisions must be made regarding treatment options. These options may include invasive (tracheostomy) or noninvasive (Bi-Pap or C-Pap) mechanical ventilation or the option of no mechanical ventilation. In most cases, the decisionmaking process includes the patient, family, physicians, and other members of the health care team, meeting as a group to discuss various treatment options. Unfortunately, the financial impact (tables 3 and 4) of implementing these treatments is often not taken into consideration or not appropriately addressed. It is well known that a huge proportion of our nation's health care dollars are spent in the last 30 days of a person's life.6 Patients and families need to be educated so their wishes will be carried out with an emphasis on dignity and respect for the individual, including a realistic appraisal of the family's financial resources and ongoing needs. Similarly, patients and families who do not receive education regarding treatment options and advanced planning often end up making life-support decisions under emergency conditions. Most patients on artificial ventilation did not choose this route. Many of these patients state that if they had been properly educated and had time to discuss this issue with their family and physician, they would not have chosen artificial ventilation.? It is the obligation of health care providers to fully inform patients of the consequences of choosing or not choosing artificial life support. The discussion must include factors of physical well-being, emotional well-being and support, financial resources, family concerns, logistical matters, and quality of life for the patient and the family. The patient's decision and directive regarding life support will guide the recommendations by health care providers regarding the use of emergency medical services. Patients and families need to deCide in advance when and if they will use emergency services. Solutions. Optimal care for individuals with ALS must include early education, intervention, and advanCe planning. Treatment modalities recommended
S128 NEUROLOGY47(Suppl 2) October 1996

Supplies per month Trach tube Tubing Catheters Gloves Trach dressings Trach ties Saline/sterile water Nursing Care - 16 hours/day Total cost per month* Total cost per year $75 $80 $180 $100

$75
$65

$250
$15,000 $16,625 $199,500

and prescribed by health care providers must be thoroughly explained, including the financial impact of such treatments. In addition, there must be increased education of insurance providers, particularly case managers and HMOs, about ALS and the value of disease management using the multidisciplinary team approach. Early intervention and symptomatic disease management through access to ALS specialists, thus reducing complications, may actually save insurance companies money. The recent Food and Drug Administration approval of riluzole and other emerging therapies, along with improved care of patients with ALS, will increase longevity. As patients live longer, the cumulative cost of their care will also increase. The burden of the increased cost will fall to the patients, their families, and to third-party payers. One hope is that earlier intervention will prolong the earlier stage of the disease when cost of care is significantly less and the patient may continue to work. Early intervention includes timely diagnosis without unnecessary diagnostic testing, early prescription of medications, timely guidance to support personnel, and identification of appropriate equipment needs. Education of the patient and professional community about available resources such as the ALS Association, the Muscular Dystrophy Association, and the National Organization for Rare Disorders is a vital component to reducing the financial and emotional

impact of ALS. These and other community resource organizations provide direct patient service programs (equipment loan programs, support groups, medication assistance programs, etc.) that help to maximize financial resources of families. It is incumbent upon health care providers to inform patients and families about these organizations. Summary. The financi~r impact of ALS is enormous and borne by the patient, his or her family, and, ultimately, all of us through third-party payer costs and taxes. Education is the key to decreasing potential costs. This includes education of the physician for timely and cost-effective diagnosis and management, education of th~ patient and family for appropriate decision-making regarding treatment options, education of third-party payers regarding the value of specialists and emerging therapies, and education of patients and the health care community regarding the value of support organizations. Acknowledgments
We gratefully acknowledge the assistance of Mark B. Bromberg, MD, PhD; Alvin H. "Woody" Moss, MD; Edward Anthony Oppenheimer, MD; Mary Beth Parks, RN; for their valuable expertise and assistance; Joy E. Yacolucci for her patient and persistent editing; Faust Medical Supply, Discover Respiratory Health Care for the financial data; The ALS Association, the Muscular Dystro-

phy Association, the National Organization for Rare Disorders for their information and ongoing support to ALS patients and families; and most of all our special thanks and admiration to Wayne and Terri McCullough, who face the challenges of living with ALS every single day.

References
1. Mitsumoto H. Classification and clinical features of amyotrophic lateral sclerosis. In: Mitsumoto H, Norris FH, eds. Amyotrophic Lateral Sclerosis. A Comprehensive Guide to Management. New York: Demos Publications, 1994. 2. Bradley WG. Amyotrophic lateral sclerosis: the diagnostic process. In: Mitsumoto H, Norris FH, eds. Amyotrophic Lateral Sclerosis. A Comprehensive Guide to Management. New York: Demos Publications, 1994:21-28. 3. Belsh JM, Schiffman PL. The ALS patient perspective on misdiagnosis and its reprecussions: an electronic (e-mail) study. Presented at the 6th International Symposium on ALSIMND, October 31, 1994, Dublin. 4. Brooks BR, Sufit RL, DePaul R, Tan Y, Sa..""ljak , Robbins J. M Design of clinical therapeutic trials in amyotrophic lateral sclerosis. Adv NeuroI1991;56:521-546. 5. Casey P. Environmental adaptations. In: Mitsumoto H, Norris FH, eds. Amyotrophic Lateral Sclerosis. A Comprehensive Guide to Management. New York: Demos Publications, 1994: 119-125. 6. Emanuel EJ, Emanuel LL. The economics of dying. N Engl J Med 1994:330:540-544. 7. Moss AH, Casey P, Stocking CB, et aI. Home mechanical ventilation for amyotrophic lateral sclerosis patients: outcomes, costs, and patient, family and physician attitudes. Neurology 1993;43:438-443.