Nursing diagnoses for ALS

Five key nursing diagnoses:

1. Potential for injury related to impaired physical mobility.

2. Impaired urinary elimination related to progressive loss of mobility.

3. Knowledge deficit regarding alternatives for diet, food preparation, and alternative procedures for supplemental feeding.

4. Ineffective airway clearance related to impaired or absent gag reflex, swallowing reflex, or sneeze reflex.

5. Impaired gas exchange related to aspiration secondary to impaired or absent gag, swallowing, and cough reflex.

Nursing Diagnosis	Actual or potential	Related to	Plan and outcome	Nursing intervention
Ineffective airway clearance	Actual	Related to impaired or absent gag, swallowing, or sneeze reflex	Maintain airway patency and adequate ventilation	- Deep breathing and coughing exercises and use of incentive spirometer to encourage lung expansion - Use of aspirator to suction secretions and prevent choking - Chest physiotherapy: percussion, assisted cough and postural drainage when indicated

http://www.alsa.org/als-care/resources/publications-videos/factsheets/nursing-management-in-als.html

Nursing Care for Individuals with ALS

Nursing Care	Details	Nursing diagnoses	Reasoning
1. Assessing muscle weakness	- Assess motor strength; presence of flaccidity or spasticity - Assess presence of contracture	- Potential for injury related to impaired physical mobility - Knowledge deficit regarding prevention of injury while promoting mobility and self-care	A nurse wants to promote daily activity and exercise as tolerated for the individual. This hopes to strengthen unaffected muscle groups without fatiguing affected muscles. ROM exercises can help prevent contracture and pain in joints.
2. Assessing skin status	- Assess skin daily, especially areas susceptible to breakdown - Change patient’s body position every two hours	- Alteration in comfort - Risk for skin integrity	As the patient’s mobility becomes limited, nurses want to maintain skin integrity by changing body position as much as needed, in order to prevent decubitis ulcers.
3. Assessing Urinary function	- Assess urination pattern and patterns of fluid intake - Assess ability to transfer to toilet or commode, or standing ability of a male - Assess for signs and symptoms of UTI	- Impaired urinary function related to progressive loss of mobility	Nurses need to encourage daily fluid intake of 2500 cc’s per day unless contraindicated because of swallowing ability. Staying on top of UTI’s is important to the patient’s comfort and health.
4. Assessing altered bowel function	- Assess bowel pattern (for constipation, diarrhea, impaction) - Assess diet, fluid intake, and swallowing ability - Assess activity level	- Impaired bowel elimination - Progressive loss of mobility - Progressive decline of dietary fiber - Often mild/moderate dehydration - Progressive inability of the trunk muscles to support a normal posture to assist in defaction	Nursing care is focused on preventative measures for bowel dysfunction, like administering stool softeners and promoting fluid intake. Having a daily record of bowel movements is important to identify regular patterns. Providing dietary regarding the importance of fiber, unless contraindicated because of swallowing ability.
5. Assessing nutritional needs	- Measuring height, pre-morbid “usual weight”, current weight, weight gain/loss pattern since onset of disease, and “ideal body weight” - Lab test indicated if patienthas lost 10% or more of body weight in last two months - Assess hydration status by carefully recording intake and output and by test of urine specific gravity	- Risk of inadequate nutritional intake	Being aware of the patient’s previous nutritional status and weight is important in comparing it to their current or progressing states of nutrition, in order to maintain adequate nutritional intake and have goals to put in place for the patient.
6. Assessing dysphagia and management of nutrition	- Assess gag, cough and swallowing reflexes, and chewing - Assess patient’s ability to swallow liquids and solids - Assess weight serially	- Potential for injury - Impaired nutritional status - Knowledge deficit regarding alternatives for diet, food preparation, and alternative procedures for supplemental feeding	Discussing techniques of protecting airway, i.e. sitting up straight, putting chin on chest while swallowing, concentrating while eating,are good preventative measures to keep patient involved and aware of their risk of dysphagia.
7. Assessing respiratory function	- Gather history of subjective symptoms, like SOB in relation to positional changes, fatigue - Assess changes from baseline respiratory rate, depth, pattern, chest expansion - Auscultate decreased breath sounds, presence of extra or adventitious sounds	- Ineffective airway clearances related to impaired/ absence gag reflex, swallowing reflex, or cough/sneeze reflex - Impaired gas exchange related to aspiration secondary to impaired/ absent gag, swallowing, or coughing reflex - Knowledge deficit regarding airway clearance and gas exchange; considerations regarding mechanical life supports	Nurse’s primary goals are maintaining airway patency by using an aspirator to suction secretions and prevent choking, and use of medications. Also maintaining adequate ventilation by cough and deep breathing exercises with an incentive spirometer to encourage lung expansion, using oxygen when ordered.
8. Assessing ability to communicate	- Assess volume and clarity of speech - Assess ability to communicate needs to family/significant others	- Impaired verbal communication related to altered volume of speech, altered clarity of speech, or loss of speech.	Nurses can refer to a speech pathologist for evaluation and intervention.
9. Assessing the patient’s psychological adaptations	- Evaluate the patient and family’s support systems and coping patterns with awareness that with ongoing loss of independence, there will be ongoing grieving by patient and family.	- Ineffective coping strategies - Knowledge deficit regarding alternatives for mobility and comfort - Impaired support system	Nurses need to provide an accepting environment in which the patient and family can share concerns and fears with each other and members of the health care team. Nurses need to help the patient and family anticipate care needs and implications.

http://www.alsa.org/als-care/resources/publications-videos/factsheets/nursing-management-in-als.html

Treatment of ALS

Since there is no cure for ALS today, there are various options of treatment that aim to slow the progression of the disease. These include disease directed therapy, symptom related therapy, and respiratory management that helps the patient maintain the ability to breathe even when their muscles are degenerating.

Disease directed therapy

There is only one drug that has been approved to treat ALS, called Ritulek. Studies on Ritulek have shown that the drug helps protect nerve cells from damage, most likely by reducing the amount of glutamate in the nervous system. Although this drug is meant to help protect the nerve cells from disease damage, once nerve cells have been impaired, this drug does not repair them or gain their function back - it only slows progression of the disease.

Symptom related therapy

As ALS progresses, symptoms worsen. In symptom related therapy, the goal is to increase patients' quality of life by alleviating these symptoms. Due to the fatality of ALS, alleviating the symptoms' impact on the patient's life is important. The most common symptoms include muscle contractions, cramps, stiffness, weakness, fatigue, depression, sleeping, and saliva and drooling. To help improve muscle function before flaccidity takes over, often patients work with physical and occupational therapy. They can begin using walking assistance devices like walkers, and eventually wheel chairs and electrical chairs. 

Respiratory management
In ALS, as muscle degeneration progesses, several problems cause increased difficulty in breathing. 

As this picture indicates, there are several important muscles in the ribcage and diaphragm that help your lungs expand to inhale air, and deflate to exhale. As these muscles weaken, oxygen cannot be adequately exchanged and carbon dioxide builds up in the blood stream, leading to sleepiness and fatigue. Also, without the strength of these breathing muscles, the ability to clear secretions and cough adequately is diminished. This can lead to pneumonia, and even diminish the ability to clear the airway adequately. Treatments for this are directed at decreasing and thinning secretions as well as increasing clearance. 

As the difficulty to breathe progresses, there are options to physically help the patient through ventilation; there is non-invasive ventilation and eventually invasive ventilation if needed. The noninvasive ventilator is triggered and controlled by the patient's own breathing, and reduces the work for the patient since their weakness prohibits them from being able to breath adequately on their own. This improves the exchange of oxygen and carbon dioxide, improving sleep quality. Non-invasive ventilation has also been shown to prolong survival as well as improve the quality of life.

Once a person with ALS becomes ultimately dependent on ventilation to breathe, they will require a tracheostomy to be placed, which is a tube insertion into the airway. This provides more efficient ventilation and better control of airway secretions.
ALS Hope Foundation. (n.d.). Treating ALS. Retrieved February 1, 2015, from https://www.alshopefoundation.org/understanding-als/treatment-als.php

Identifying the Signs and Symptoms of ALS

According to the ALS association, the early signs and symptoms of ALS may be so subtle and slight that they can be overlooked. These early symptoms don't arise in the same order or presentation from patient to patient, and the progression of the disease varies between individuals. One individual might have trouble walking and trip or experience foot-drop. Another may have slurred or altered speech, while another may have muscle weakness in the upper extremities or decreased dexterity in the hands.

Early signs and symptoms of ALS

Muscle weakness is considered a hallmark initial sign of ALS, occurring in approximately 60% of patients. The disease progresses by increasing weakness and paralysis in the muscles throughout the body, and eventually effects speech, swallowing, chewing and breathing.

Because ALS only attacks motor neurons, patients do not experience deficits in the sense of touch, hearing, taste, smell, or sight. 

The video below will show a women's personal story of the early onset of her disease, and the breakdown of symptoms and diagnosis by an MD.

Association, A. (n.d.). Symptoms. Retrieved February 9, 2015, from http://www.alsa.org/about-als/symptoms.html

Diagnosing ALS and learning about the disease process

Today, there is no current test that is done to diagnosis Amyotrophic Lateral Sclerosis, and the disease is very hard to diagnose. Only through a thorough clinical examination and series of diagnostic tests, that help to rule out other diseases that may mimic ALS, can be done to establish the disease.
The presence of upper and lower motor neuron signs is strongly suggestive to ALS. Once these are evaluated by the physician, they can order a series of tests to help eliminate other diseases and further suggest the diagnosis of ALS. Some of these tests are listed below.

• Electrodiagnostic tests including electomyography (EMG) - a special recording technique that detects electrical activity in the muscles
• Nerve conduction velocity (NCV) which measures electrical energy by assessing the nerve's ability to send a signal
• Blood and urine sample studies include high resolution serum protein electrophoresis, thyroid and parathyroid hormone levels and 24-hour urine collection for heavy metals
• Spinal tap
• X-rays, including magnetic resonance imaging (MRI) to get detailed images of the brain and spinal cord. These could reveal evidence of other problems that may be causing the symptoms, such as a spinal cord tumor, a herniated disk in the neck that suppresses the spinal cord, or even a cyst in the spinal cord.
• Myelogram of cervical spine - an imaging test that looks for problems in the spinal canal, including the nerve roots and other tissues.
• Muscle and/or nerve biopsy,
• Thorough neurological examination

There are several diseases that share the same symptoms of ALS and are all treatable. The ALS association recommends that patients diagnosed with ALS receive a second opinion from an "ALS expert" - someone who diagnoses and treats many patients with ALS and has training in this medical specialty.

The ALS Association. (2010, January 1). Diagnosing ALS. Retrieved January 30, 2015, from http://www.alsa.org/about-als/diagnosing-als.html

Etiology of ALS, and putting another face to the name.

In nine out of ten diagnoses of ALS, no clear identifying cause is apparent. Diagnosed patients lack an obvious genetic history, complete with affected family members. Nothing about the way patients live their lives have given scientists and clinicians clues as to what causes ALS. There is only a small, 5-10% of cases of ALS is familial. The term "familial ALS" means that there is more than one occurrence of the disease in the family. 

ALS occurs rarely and spontaneously, and has a median survival of 3 years after diagnosis. This motor neurodegenerative disease progresses in stages. It announces itself with persistent weakness or spasticity in one are or leg, causing difficulty using the affected limb. Some patients' first incidences have occurred with no warning, falling fast face first while walking during the day. Sometimes the motor problems originate in the muscles that control speech and swallowing. Hearing, vision, and touch generally remain normal, but voluntary movements of skeletal muscles can rapidly decline. 

In middle stages of the disease, motor symptoms progress from their starting point to the rest of the body, until it demands to be attended to. Some muscles may become paralyzed, while others are weakened or unaffected. Weakness in swallowing muscles may cause choking or difficulty eating and managing saliva. Weakness in breathing muscles can cause respiratory insufficiency, especially when lying down. Treatment can extend the length and meaningful quality of life for patients.

In the late stages of ALS, the symptoms have progressed immensely, and eventually lead to death. Most voluntary muscles are completely paralyzed. Muscles that help move air in and out of the lungs become diminished, and patients can need ventilatory support. Speech, eating or drinking may become impossible. New technology, like the computer that was talked about in the first post, may be used to help patients communicate through a speaking computer program. 

The end of this post will leave another face to the name of ALS, showing the true progression of this rapid  terminal disease. Steve Gleason, a former NFL football player, has been interviewed about his new journey in living. When Gleason was originally diagnosed, his providers truthfully told him to prepare to die, as this terminal illness has no medical cure. In response, Gleason said that he would instead "prepare to live." 

Stages of ALS. (n.d.). Retrieved January 23, 2015, from http://mda.org/disease/amyotrophic-lateral-sclerosis/signs-and-symptoms/stages-of-als

Epidemiology of ALS

Now that we have a foundational understanding of what Amyotrophic Lateral Sclerosis is, this week we will analyze the frequency and distribution of ALS and learn about the specific populations that this disease effects. Many studies have been done and articles have been written on ALS, but in the last five years, a few resources that had overlapping statistics seemed to be most reliable. 

By learning about the epidemiology of the disease, we can educate more people about its prevalence and help people recognize early symptoms and seek treatment and therapy as early as possible. Knowing which populations the disease is most prevalent in can also give certain people a good idea of who to look out for in regards to ALS.

What is the distribution in the US? 

There is a prevalence of 3.9 per 100,000 persons in the US general population, and an incidence of 1.89 per 100,000 per year are diagnosed. Prevalence and incidence are different from each other, where prevalence pertains to known existing cases of ALS, while incidence pertains to the number of new cases diagnosed each year.

Are certain parts of the population more affected than others? 

ALS occurs throughout the world with no racial, ethnic or socioeconomic boundaries. However, overall, ALS is more common among white males (twice as likely African-Americans), non-Hispanics, and persons aged 60-69 years. 

Males have a higher prevalence rate of ALS than females overall (1.5:1).

Age groups with lowest number of persons with ALS were 18-39 years of age and >80 years. Some physicians report that they are seeing "increasing numbers of younger ALS patients," these reports need more study. 

What is the mortality and morbidity from this disease?

Mean age of onset for sporadic ALS is about 60 years. Paralysis is progressive and leads to death d/t respiratory failure within 2-3 years  for bulbar onset cases and 3-5 years for limb onset cases.

About twenty percent of people with ALS live five years or more and up to ten percent will survive more than ten years and five percent will live 20 years. There are people in whom ALS has stopped progressing and a small number of people in whom the symptoms of ALS reversed.

Studies have shown that 70% to 90% of patients diagnosed as having ALS had this condition recorded on their death certificate.

The ALS Association. (2014, January 1). Epidemiology of ALS and Suspected Clusters. Retrieved January 1, 2015, from http://www.alsa.org/als-care/resources/publications-videos/factsheets/epidemiology.html

Mehta, P., Antao, V., Kaye, W., Sandez, M., & Williamson, D. (2014, July 25). Retrieved January 13, 2015, from http://www.cdc.gov/mmwr/preview/mmwrhtml/ss6307a1.htm

Wijesekera, L. C., & Leigh, P. N. (January 01, 2009). Amyotrophic lateral sclerosis.Orphanet Journal of Rare Diseases, 4.

Meet Stephen Hawking, the man behind "The Theory of Everything"

This past November, "The Theory of Everything" premiered in theaters, a film that moved audiences to tears as they took a journey through the life of Stephen Hawking, a world-renowned theoretical physicist and cosmologist of our time. It was not his baffling genius or ground-breaking research that moved audiences, however; the emotion of the movie was driven by the devastating turn his life took when he was diagnosed with Amyotrophic Lateral Sclerosis (ALS) at the young age of 21.

ALS is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord. The advancement of the disease slowly destroys motor neurons, eventually leading to their death. With compromised motor neurons, the brain's ability to initiate and control movement in the body is lost, and as the disease progresses, individuals can become fully paralyzed.

Hawking, pre-diagnosis of ALS.

 Hawking, years after his diagnosis, physically limited to a motor chair but still able control facial muscles, enabling his speech and various facial expressions.

Today, Hawking is 73 years old, and communicates through a computer speaking device. A small sensor is activated by a muscle in his cheek. This sensor helps him "type" characters and numbers in his keyboard. The incredible computer technology learns to predict words and phrases for Hawking reducing the time and effort it takes for him to communicate. Hawking can give lectures, write chapters of books, and engage in conversations with the help of his technology.

This blog will aim to break down a general understanding of ALS week by week. Although Hawking's condition gives us a small picture to build our understanding of this terminal illness, we must keep in mind that as the longest living survivor of ALS, his condition and survival rate is especially rare. Unfortunately, life expectancy for individuals with ALS averages 2-5 years after the initial diagnosis.