Nursing Diagnoses for ALS

5 Nursing Diagnoses for ALS:

1. Potential for injury related to impaired physical mobility
• Pt. has decreased muscle use and therefore they are at more risk for pressure ulcers and also injury from falling.
2. Impaired urinary elimination related to progressive loss of mobility and dehydration
• Pt. has decreased swallowing and loss of mobility which leads to them being unable to drink a lot of fluids to produce enough urine, and also the loss of mobility prevents them from walking to the bathroom and being able to urinate by themselves properly. They may need help going to the bathroom as the disease progresses.
3. Impaired nutritional status related to inability to swallow.
• Pt. has impaired muscle use of throat muscles, therefore they have decreasing ability to swallow and eat food properly, putting them at risk for impaired nutritional status as they are not getting enough of different types of foods as the disease progresses.
4. Ineffective airway clearance related to impaired swallowing, gag reflex, and cough.
• Impaired muscles - abdominal, throat - lead to inability to cough and clear secretions in their throat.
5. Impaired verbal communications related to altered volume of speech, altered clarity of speech and loss of speech.
• Muscles of speech (tongue muscles, mouth muscles, throat muscles) are affected by the degenerative disease, which decrease ability of the patient to use those muscles, which causes garbled speech.

Nursing Diagnoses	Goal	Intervention	Outcome
Potential for injured related to impaired physical mobility.	Promotion of activity and exercise. Promotion of proper positioning to prevent decubitus ulcers.	ROM exercises to prevent contracture & pain in joints. Change positions every 2 hours; after each change of position, check for redness over bony prominences.	Patient will be active for as long as they can, and can remain more comfortable so their joints and muscles die slower. Patient will not get pressure ulcers, since they are immobile, by turning them often you prevent skin breakdown.

If you would like more information on the nursing care and management of patient’s with ALS. I would highly suggest going to this website, as it has a lot of useful information on how to care for these patients safely. It not only provides information on what to assess in these patients, but also how to manage these problems that pop up.

Source: 

Association, A. (2014). Nursing Management in ALS. Retrieved from ALS Association: http://www.alsa.org/als-care/resources/publications-videos/factsheets/nursing-management-in-als.html

Nursing Care of Individuals with ALS

There are multiple steps to the nursing care of patients with ALS.

You want to be very receptive when assessing these patients. Below I’ve created a table on what nurses should assess consistently when their patient has ALS.

Nursing Assessment with ALS
What you Assess	Reasoning
Motor strength	Assessing the motor strength consistently can give the patient and physician a good idea of how fast their disease is progressing and to what degree the disease has progressed to. Assess for presence of spasticity or flaccidity also.
Presence of Contracture	This is a disease of the motor neurons. As the motor neurons are unable to send messages down to the muscle cells. You get contractures, from the muscle being unable to relax, especially when the disease has really progressed and you’re in the end stages.
Skin	You want to especially assess areas that are susceptible to breakdown. These patients’ muscles cells are slowly dying (atrophy) and so they will not be moving as much as the disease progresses. It is very important to assess their skin daily, to prevent ulcers from getting worse or showing up.
Urinary Function	You want to assess their urination pattern and patterns of fluid intake. You also want to assess their ability to transfer to the toilet or stand (for males). This will ensure that the patient is safe when walking. If the patient is unable to walk or transfer safely, it can be dangerous, or we could potentially help them with transfer devices that support them (walker, wheelchair).
Bowel Function	You want to assess their bowel pattern for constipation, diarrhea and impaction. The intestines use muscles to push stool through the body and out. Since ALS is a disease of the muscles, and of them not functioning as well as they are supposed to, then the bowel function will also be altered. The patient can experience some diarrhea and mostly constipation (because they have difficulty pushing the stool out of the body).
Swallowing	As ALS progresses, patients have a hard time swallowing, and experience dysphagia (which is difficulty swallowing). This is because swallowing, much like the intestines, is also moved by muscles in the throat/neck. When the muscles progressively die and start deteriorating, they don’t work as well, therefore the patient has more trouble swallowing. It is very important to assess this frequently as this is a progressive disease, and even though the pt. can swallow currently, they may not be able to in a few weeks or so.

If you would like more information on the nursing care and management of patient’s with ALS. I would highly suggest going to this website, as it has a lot of useful information on how to care for these patients safely. It not only provides information on what to assess in these patients, but also how to manage these problems that pop up.

Source: 

Association, A. (2014). Nursing Management in ALS. Retrieved from ALS Association: http://www.alsa.org/als-care/resources/publications-videos/factsheets/nursing-management-in-als.html

Treatment for ALS

Although there is NO CURE for ALS, there is indeed treatment, to slow the progression of the disease and increase mortality/morbidity. I will now go on the list the different kinds of treatment options there are for someone with ALS.

• There is a medication, one medication, for ALS. Rilutek slows down the progression by decreasing amounts of glutamate in the body. Excess levels of Glutamate are a supposed cause for ALS. 
• Other Medications can relieve symptoms of ALS including:
• Muscle cramps & spasms
• Spasticity
• Constipation
• Fatigue
• Excessive Salivation
• Excessive Phlegm
• Pain
• Depression
• Sleep Problems
• Uncontrollable bursts of crying and laughing
• There have been many advances in respiratory treatment now that increase the morbidity of patients diagnosed with ALS. In the past, patients with ALS have had a hard time surviving for long, due to the weakening of their chest muscles, that make it hard for them to breath as time goes on. In fact, the cause of death in patient's with ALS is usually due to a respiratory problem. Now that ventilation, drugs, and treatment have improved, patients can survive for even longer and live better, more comfortable lifestyles, until their time comes.
• There have also been significant improvements in nutritional care. Very aggressive nutritional supplementation and feeding tubes are used to help patients overcome deficiencies, due to their inability to swallow, chew and eat their food. 

All of these treatments do not cure ALS, but they do increase the quality of life for patients living with the disease. I've included a video I found on Youtube of a Physician discussing tips for the treatment of ALS. What I like about this video is the holistic approach that the Physician decides to take with the treatment of ALS. Most physicians just concentrate on a few of the symptoms and the progression of the disease, but this Physician talks about the life of the patient as a whole, and how the disease affects them.

Sources:

Video Source

Clinic, M. (2014). Amyotrophic Lateral Sclerosis. Retrieved from Mayo Clinic: http://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/basics/treatment/con-20024397

Foundation, A. H. (2012). Treating ALS. Retrieved from ALS Hope Foundation: https://www.alshopefoundation.org/understanding-als/treatment-als.php

Symptoms of ALS

People with ALS are often aware of the signs and symptoms to look out for because most likely someone in their family had experienced the same signs, and it is genetically passed down.

First it starts with muscle weakness in either their arms or legs, or maybe both. For people who don’t realize it’s happening, they think nothing of it, and just think they are being more clumsy than usual.

Picture Source

 The symptoms progress to some twitching or cramping of their muscles, especially those of the hands and feet, leading to impairment of the use of their limbs. They’ll start having symptoms of their respiratory system as the disease starts affecting the motor neurons in their chest muscles. They’ll have difficulty projecting their voice and maybe stumble over their words.

In the advance stages of the disease, the person with ALS will have shortness of breath, which is when the person has difficulty catching a full breath and feels like they aren’t getting enough air. The disease progresses and they eventually have difficulty breathing and swallowing, most people in the end stages of the disease will need a ventilator to help them breathe and maybe a feeding tube or a change in their diet to prevent them from choking on their food.

On the bright side, since ALS only attacks the motor neurons, you still have your sensory skills. You can still feel touch, see, hear, smell, and taste the world just the same as before you had the disease. The only thing that’s changed is your ability to move, breathe, and swallow.

The mean survival time with ALS (or how long it takes the disease to kill you) is about three to five years, but a lot of people survive more than 10 years. The sooner you know the symptoms, the better. There is one drug that slows the progression of ALS, so the sooner you can get on the drug, the more time it can give you with the world. 

Sources:

Association, A. (2010). Symptoms of ALS. Retrieved from ALS Association.

Association, M. D. (2015). Amyotrophic Lateral Sclerosis. Retrieved from Muscular Dystrophy Association: http://mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms

Picture Source: http://mda.org/sites/default/files/body_motor-neurons.jpg

Diagnosis of ALS

There is no one test or procedure used to diagnose ALS. Most of the tests and examinations done when ALS is suspected are just used to rule out other diseases that have the same symptoms as ALS. That is why, it is of the utmost importance that when your physician thinks you may have ALS, it is strongly recommended that you get a second opinion from an ALS expert, to solidify the first opinion.

Most of the procedures done to rule out the other diseases that have similar symptoms to ALS include:

• X-Rays (including MRIs) - this can help determine whether there is a mass or lesion that could be in the spinal area, causing weakness of arm and leg muscles. If there is a mass, it could be blocking motor signals from the actual muscles to the brain, making it difficult to move, and mimicking symptoms of ALS.
• Spinal Tap - this is a procedure in which a large needle is inserted into the lower spinal area, and CSF (or cerebrospinal fluid) is taken out. CSF is made in the brain and circulates the whole spinal cord. Getting a spinal tap can tell you whether the person has an active infection or bacteria.
• Muscle and/or Nerve Biopsy - this procedure is done to take a closer look at the actual muscle and nerve cells in your body. It helps rule out any weird or unusual cells that could be disrupting normal muscle or nerve function.
• Thorough Neurological Examination - this examination goes through a series of tests that tell you how well your nerves are functioning, and can also help the physician keep track of the progression of the disease later on, by testing the nerves and brain function again, and comparing it to these older results.
• Electrodiagnostic Tests: (includes Electromyography and Nerve Conduction Velocity). And electromyography is used to evaluate and record the electrical activity of muscles. The test basically assesses the health of muscle and nerve cells that control the muscle cells, in the body. This can help the physician keep track of the progression of the disease and get a baseline to compare later results to. As ALS is a disease specifically related to the nerve cells that control muscle cells in the body, this test is especially important in helping to rule out other diseases the symptoms could lead to. 

I've included another video that I found very helpful. She does an excellent job of explaining the progression of her symptoms of ALS. Enjoy!

Sources:
Youtube Video: "How My Initial Symptoms Progressed" By: Toochey Films

 Association, A. (2014). Epidemiology of ALS and Suspected Clusters. Retrieved from ALS Association: http://www.alsa.org/als-care/resources/publications-videos/factsheets/epidemiology.html

Pathophysiology of ALS

ALS is the most common neurodegenerative disease of motor neurons. As I've said in my previous posts, there is currently no known cause of ALS.

Disease Progression:  Most people diagnosed with ALS begin showing symptoms that involve movement of their limbs. It starts out as "clumsiness" or stumbling on their feet when they run, this progresses to "slapping gait," which is also known as foot drop. As for the arms, there is reduced dexterity and ability to do activities that involve fine detail and intricate handy work. Hands get cramped and stiff, and it eventually can affect work performance. Later, problems with the throat (which is a lot more debilitating and noticeable) progress to high risk of choking while eating, slurred speech and difficulty talking loudly. ALS patients can also have a hard time with their emotions, such as involuntary laughing or crying, and depression.

As the diseases advances, you see more ALS patients having spasticity, or involuntary tightening of their muscles, which can significantly impair the way they walk and their dexterity. Their muscles have atrophied significantly, and you can start to see it visibly. They also commonly get muscle cramps and contractures due to inability or difficulty moving their limbs.

There are many theories and studies that explore the cause of ALS. One of them suggests that excessive stimulation of glutamate receptors can cause the "excitotoxicity" that happens in ALS. Excitotoxicity is usually controlled by Sodium-dependent glutamate transporters that bind to glutamate and clear it out of the synaptic gap. The one medication that I talked about in my first post "riluzole" actually works through this pathway and gets rid of glutamate. Excitotoxicity causes the death of motor neurons in patients with ALS, and so by getting rid of glutamate (the agent that causes excitotoxicity), you can essentially slow down the progression of the disease.

As you can see in the image above, in a normal person, glutamate removal is regulated and there is not an excessive amount of it in the synaptic cleft (no excitotoxicity would be present here). In the image on the left, the person with ALS has a very excessive amount of glutamate in the synaptic cleft, and this is what causes the cell to have excitotoxicity, and cause death of a motor neuron.

Sources:

Medscape. (2014, May 2). Amyotrophic Lateral Sclerosis. Retrieved from Medscape: http://emedicine.medscape.com/article/1170097-overview

Image Source:

Youtube Video: "Amyotrophic Lateral Sclerosis"

Epidemiology of ALS

Though there is no specific cause for ALS, about 5-10% of the cases are inherited (familial ALS).  As I stated in my previous post, there are many theories on the causes, including occupational exposure, environmental exposure, chemical exposures, trauma, etc.

ALS is known to affect males more than women, especially white males. ALS becomes increasingly more common in people ages 60 or older. This means that a white male that's older than 60 years has an extremely higher risk of getting ALS than a black women in her 40's (Mehta, P).  As ALS is not a condition that is required to be reported by the U.S (Massachusetts is the only state that mandates reporting ALS), we will never know the extent of the disease in the U.S. The main goal of mandatory reporting or registering is to examine specific risk factors for ALS.

Environmental exposures that research has included as making a person of higher risk to developing ALS includes previous exposure to heavy metals (lead) and certain occupations (military service). Most of these risk factor studies use only small sample sizes and therefore have been conducted in limited geographic areas and are not clinically accurate. Other studies show that environmental toxic exposures may work against a background of increased genetic susceptibility to increase one's risk of ALS (LM, N). Evidence regarding a lot of these theories is inconsistent however, and there is still no specific cause or causes of ALS.

The incidence rate of ALS across ALL ages is estimated to be about 2 persons per 100,000 population (Mehta, P). The rate increases to five people per 100,000 population in those ages 70 or above, showing that the prevalence and risk of ALS does increase as one ages. An estimation of about 5,000 people are diagnosed with ALS every year in the U.S. Looking at ALS data across the four national databases, the prevalence rate for whites is two times more than blacks, with whites having a prevalence rate of 4.2 per 100,000 population and blacks only having a rate of 2.0 per 100,000 population. The disease has also found to be more common in non-Hispanics than Hispanics.  According to the ALS Association, some physicians are reporting that they are seeing an increasing number of younger people with ALS than in previous years (Association, A).

There are currently many epidemiological studies being conducted in the U.S., but as physicians are NOT required to report cases of ALS, it is very difficult to investigate the incidence, prevalence and risk factors of ALS.

Life expectancy of people with ALS is 2-5 years from the time of diagnosis. More than half of all patients live more than 3 years after diagnosis (Association, A).

Search Terms: "Epidemiology of ALS," Epidemiology of ALS, NIH"

Sources:

Association, A. (2014). Epidemiology of ALS and Suspected Clusters. Retrieved from ALS Association: http://www.alsa.org/als-care/resources/publications-videos/factsheets/epidemiology.html

LM, N. (1995-1996). Epidemiology of ALS. Clinical Neuroscience.

Mehta, P. (2014, June). Prevalence of Amyotrophic Lateral Sclerosis 2010-2011. Retrieved from Centers for Disease Control and Prevention: http://www.cdc.gov/mmwr/preview/mmwrhtml/ss6307a1.htm

What is ALS?

Amyotrophic lateral sclerosis (ALS) is also referred to as "Lou Gehrig's disease."
It is a very rapidly progressing neurodegenerative disease that affects nerve cells. In simpler terms, this disease causes motor neurons (nerve cells in our body that help us move our muscles) to deteriorate and die. As motor neurons in the body degenerate, the person slowly loses control and function of their muscles, and eventually could become completely paralyzed.

Symptoms that an ALS patient may experience include muscle weakness (as their motor neurons die off), difficulty swallowing, difficulty breathing and difficulty talking. These last three symptoms are due to loss of control of muscles used in breathing (muscles in the chest and diaphragm) and also loss of control of muscles used in swallowing (walls of the pharynx, esophagus). As the disease progresses, the patient is forced to get nutrition through a feeding tube and use ventilation to help them breathe. Loss of control over skeletal muscles causes the fibers to "atrophy" or waste away, and those eventually degenerate also.

ALS is one of the most common neuromuscular diseases worldwide. Within a population of 100,000 people, there are 2 new ALS cases each year. ALS is more common in men than women, white people than black people, and can also be inherited. Being diagnosed with ALS is rare before the age of 40, but it does happen. Most people with the disease live an average of 2-5 years after their first signs of disease and only about 10% of people with ALS survive for 10 years or more.

This disease is fatal, there is no cure and no way to prevent it. Riluzole is the only FDA approved drug available to treat ALS. It slows the progression of ALS, and can only extend survival about 2-3 months.

The cause of ALS is still unknown, but there are a few theories. In the past few decades scientists have discovered that mutations in the gene that produces the SOD1 enzyme were linked with some cases of familial ALS, although it is unclear how the mutations could lead to motor neuron degeneration. Other studies look into environmental factors (exposure to toxins, physical trauma, etc.).  Recently, researchers have started focusing on RNA processing after finding that many genetic risk factors for ALS are involved in this pathway. Although the cause of ALS is still not clear, many researchers seem to agree that the development of the disease could be contributed by a number of processes.

I know you were probably expecting an "Ice Bucket Challenge" video somewhere on this post, as that has been a recent trend on social media sites. Below is my favorite "Ice Bucket Challenge" video, and in my opinion, the only one that really meant something. Fast forward to 2:00 to gain real insight into ALS.

Sources:
 Association, A. (2014). Epidemiology of ALS and Suspected Clusters. Retrieved from ALS Association: http://www.alsa.org/als-care/resources/publications-videos/factsheets/epidemiology.html