ALS Disability Insurance Act

ALS Disability Insurance Act of 2019 (H.R. 1407, S.578)

ALS is a quickly progressing disease. People diagnosed with ALS can’t afford to wait to access the SSDI benefits they desperately need.

Currently, all people living with Amyotrophic Lateral Sclerosis (ALS) who qualify for Social Security Disability Insurance (SSDI) must wait five months before they can begin receiving these benefits.

To address this issue and provide relief for these individuals, Sen. Sheldon Whitehouse (D-RI), Sen. Tom Cotton (R-AR), Rep. Seth Moulton (D-MA), and Rep. Peter King (R-NY) introduced the ALS Disability Insurance Act in February 2019. The legislation would eliminate the five month waiting period for SSDI benefits for people living with ALS so that they can begin receiving these benefits immediately.

Because ALS can progress very quickly, it is essential that diagnosed individuals are able to access SSDI benefits as soon as possible. Congress has recognized the urgent need of the ALS community before when it voted in 2000 to waive the two year waiting period for Medicare eligibility for ALS patients.

The Muscular Dystrophy Association has led the way for innovations in ALS science and care for nearly 70 years. We have dedicated hundreds of millions of dollars to ALS research, and care for tens of thousands of people diagnosed with ALS and their families.

Swim Training Improves Muscle Function in Mouse Model of ALS, Study Shows

I know of several men with Kennedy's Disease (SBMA) who expressed how good they felt and how much better/safer their exercise routine is in the pool. I never tried it. Below is an article on the benefits of swim training in mouse models with ALS. Clink on the header below to read the entire article or go to the bottom of this page and follow the link to the actual published report.

Swim Training Improves Muscle Function in Mouse Model of ALS

... Findings revealed that ALS mice had reduced muscle strength (70% less between 11 and 15 weeks), and showed significant alterations in energy metabolism (30% less citrate synthase activity, and higher activities of cytochrome c oxidase and malate dehydrogenase), and high levels of oxidative stress markers compared with controls.

However, swim training reduced the loss of muscle strength associated with ALS (5% less between 11 and 15 weeks), and increased citrate synthase activity by 26% compared with ALS mice that did not undergo swim training.

According to previous studies, swim training prolongs the lifespan of ALS mice by 10% to 13%. However, “from a clinical point of view, not only prolongation of lifespan, but also sustained functionality and inhibition of muscle waste are critical elements of therapy,” the study said.

“In agreement with previously published data, swim training significantly decreases the reduction in muscle strength clearly visible at the symptomatic stage of ALS, . . . reduces oxidative stress, and improves muscle energy metabolism at terminal stage of the disease,” the researchers stated.

“Our findings indicate that swim training is a modulator of skeletal muscle energy metabolism with concomitant improvement of skeletal muscle function in ALS mice,” they concluded.

Here is the link to the report on the study:  https://www.mdpi.com/1422-0067/20/2/233/htm

Great News - A Treatment for ALS

In case you missed it, the big news this week is the FDA fast-tracked the approval of a treatment for ALS. You can read about it here:  CNN Health and here:  FDAPress Release.

This is fantastic news for those living with ALS.

I have received a few inquiries asking if this drug could be helpful for treating Kennedy’s Disease.

First off, I am not a doctor and not as knowledgeable as some others with KD concerning this topic. What I do know is that ALS is a condition that attacks the upper motor neurons. Kennedy’s Disease attacks the lower motor neurons. For this reason, I do not believe the new drug will be beneficial for those of us living with KD.

So what is the difference between the two upper and lower motor neurons? I found the following explanation to be helpful.

“Upper motor neurons originate in the motor region of the brain stem. They are not responsible for the stimulation of the muscle which is targeted as they do not carry information down to the final common pathway. They work through a neurotransmitter called glutamate which transmits the nerve impulses from upper to lower motor neurons where it is detected by glutamatergic receptors. On the other hand, lower motor neurons receive impulses from the upper motor neurons and connect the spinal cord and brain stem to the muscle fibers. They are the cranial and spinal nerves. They work by making use of the glutamate which is released from the upper motor neurons, and this triggers depolarization in the lower motor neurons. A series of actions occur which end up signalling the muscle to contract. The cell bodies of the lower motor neurons are located in the neuraxis, and their axons leave and synapse with the muscles in the body. On the other hand, the upper motor neurons synapse with the lower motor neurons as they are unable to leave the central nervous system.”

Source:  DifferenceBetween Net

Nonetheless, even if this new treatment is not the answer, it is progress and a help for those living with ALS. 

Kennedy’s Disease and ALS

Even though Kennedy’s Disease (SBMA) is becoming more widely known in the medical community, I still receive calls or emails requesting additional information on the condition after a person has been initially diagnosed with Amyotrophic Lateral Sclerosis (ALS). One major difference between the two conditions is that SBMA effects the lower motor neurons where ALS effects both the upper and lower motor neurons.

Sunday I received such a call. A woman in her late 70s was experiencing swallowing problems and the occasional fall. Her doctor diagnosed the health concern as Familial ALS (FALS). Since she never experienced any symptoms until the past year, she did her own research and came across Kennedy’s Disease. Several symptoms were similar to ALS and a few she felt fit her better. 

The average age of onset for ALS is in the mid-to-late 50s, however, there have been cases where symptoms first appeared in the patient’s 70s. The ALS Association has a good PDF report on the Epidemiology of ALS here.  To learn more about ALS, follow this link to the ALS Association.

I also ran across an interesting 2008 report on how Kennedy’s Disease mimics ALS. The summary is shown below with a link to the actual report. 

Kennedy Disease Mimics Amyotrophic Lateral Sclerosis:
A Case Report
Mu-Hui Fu, Min-Yu Lan, Jia-Shou Liu, Shung-Lon Lai, Shun-Sheng Chen,
and Yung-Yee Chang

Abstract- Kennedy disease (KD) is an X-linked inherited motor neuron disease that is often accompanied by androgen insensitivity. Its estimated incidence in the US is approximately 1 case in 40,000 men. KD has also been reported in individuals of different racial backgrounds, especially in Japanese but the prevalence rate in Taiwan has not been fully investigated. Here we report a case of KD definitely diagnosed by abnormal expansion of a polymorphic tandem cytosine-adenine-guanine (CAG) triplet repeat in the first exon of the androgen receptor gene. The direct genotyping from polymerase chain reaction product is subsequently performed utilizing capillary electrophoresis. The patient’s neurological conditions mimic amyotrophic lateral sclerosis (ALS). Since these two diseases have different etiologies and prognosis, it reminds us the necessity to rule out KD in face with a suspected male case of ALS.

Download the six page report here

ALS Image: ALS Association

KD Image: MDA