Thursday, March 3, 2016

Dysphagia Evaluation and Treatment Info for Myositis

Here's another awesome project by my student Ani Haas! 

If you have a patient with Myositis, this info is a great guide for dysphagia therapy with these patients.  Are exercises appropriate?  What is the prognosis?  What compensatory strategies tend to be helpful?  Other considerations.  Let us know in the comments if you found this helpful! 



Myositis

Background:
Myositis is a general term meaning inflammation of the muscles. Inflammatory myopathies are the largest group of potentially treatable myopathies in children and adults.  The disorders are best classified on the basis of distinct clincopathologic features.  To date, there are four subtypes: dermatomyositis, polymyositis, necrotizing autoimmune myositis, and inclusion-body myositis. Correct subtype identification and the distinction of these conditions from other diseases that have similar characteristics are fundamental in differing prognosis and differing responses to therapy.

Symptoms:

People with inflammatory myopathies have increasing difficulty with tasks that require the use of proximal muscles. An example of this is getting up from a chair, climbing stairs, or lifting objects.  People may feel tired after standing or walking, have muscle pain or soreness that lasts for weeks, and may have trouble holding their head up. With certain subtypes, some patients may experience difficulty swallowing, cardiac arrhythmia, ventricular dysfunction, or fever.

Specific Clinical Features:

Dermatomyositis: Affects both children and adults. Early symptoms include skin manifestations, a blue-purple rash with edema accompanying or proceeding muscle weakness. Muscle strength may not be affected and the dermatomyositis may be limited to the skin.

Polymyositis: This subtype is rare, often mis-diagnosed, and will stand-alone.  Proper diagnosis is based on exclusion and is often diagnosed as sub acute proximal myopathy in adults who do not have a rash, family history of neuromuscular disease, exposure to mytoxic drugs like statins, penicillamine, or any facial or extraocular muscle involvement.

Necrotizing Autoimmune Myositis: This subtype makes up almost 20% of all myopathies.  It can occur at any age but is typically seen in adults.  It can occur alone or following a viral infection.  It causes severe weakness and very high creatine kinase levels in the blood.

Inclusion-Body Myositis: Inclusion-Body Myositis (IBM) is the most common and disabling inflammatory myopathy among people 50 years of age or older.  It is thought to be an insidious disease that develops over a period of years.  It often simulates a late-life muscular dystrophy or slowly progressive motor-neuron disease. Early clinical features of IBM include involvement of distal muscles, especially foot extensors and finger flexors, atrophy of the forearms and quadriceps muscles.



Causes:
Myositis can be caused by anything that leads to inflammation of the muscles. Common causes include; inflammatory conditions, viral infections, drugs, injury, or Rhabdomyolysis (a breakdown of muscle tissue that releases a damaging protein into the blood).

Diagnosis of Myositis:
Blood tests: Testing for elevated levels of muscle enzymes, like creatine kinase, which will be present in patients with active disease.
MRI: A MRI is helpful for diagnosis when muscle edema is present, identifying which particular muscles are affected by atrophy.
EMG: By inserting needle electrodes into muscles, a doctor can test the response of muscles to electrical nerve signals. EMG can identify muscles that are weak or damaged by myositis. EMG can also be used to rule out neurogenic conditions and assess disease activity.
Muscle biopsy: This is the most accurate test for diagnosing myositis. A doctor identifies a weak muscle, makes a small incision, and removes a small sample of muscle tissue for testing. Muscle biopsy leads to a final diagnosis in most people with myositis.

Treatment Options:
Currently, there are differing treatment options for specific subtypes:

Treatment of dermatomyositis, polymyositis, and necrotizing autoimmune myositis:
 Once daily doses of oral prednisone, a corticosteroid, is the first-line drug in combating the above inflammatory myopathies. Corticosteroids slow the body's immune system and stop the inflammatory attack on muscle, skin and other body systems. These medicines control the inflammation, ease pain, and increase muscle strength.

Immunosuppressant: Commonly used is methotrexate and azathioprine is often used in combination with prednisone. They may help the patient to taper off prednisone more quickly and avoid some of the unwanted effects.

Intravenous immune globulin (IVIG): IVIG is a blood product derived from human plasma. IVIG is used to boost the body's immune system response, IVIG is usually reserved for cases resistant to other treatments.

Treatment of Inclusion-Body Myositis (IBM):
Due to the slow and progressive course of IBM, the degenerative effects are already advanced by the time the patient seeks medical help. Musculature of the pharynx is at risk for inflammatory myopathic changes, which can cause dysphagia.  Treatment options used to treat other myositis diagnosis have been ineffective in treating IBM; there is no clear treatment option for IBM. Treatment is aimed at delaying the progression of the disease.



Dysphagia in Myositis:
Due to ineffective drug treatment options for IBM, dysphagia is more common in IBM than any other inflammatory myopathy. Dysphagia in patients with IBM contributes to aspiration pneumonia associated respiratory failure, which is the most common cause of death in people with IBM.

Clinical Features of Dysphagia in IBM:
Patients may experience feeling of:
1. Food sticking in throat
2. Coughing during meals
3. Nasal reflux
4. Difficulty with dry foods, solids, and thin liquids most frequently.

Clinical oral exam on patients with IBM typically show normal lingual range of motion, strength, and coordination.  Some patients may exhibit reduced laryngeal elevation

Videofluroscopic abnormalities in patients with IBM included:  
1. Residual pharyngeal pooling  
2. Reduced pharyngeal constrictor contraction
3. Impaired laryngeal elevation
4. Cricopharyngeal (CP) dysfunction noted by poor relaxation and narrowing of upper esophagus
5. Tongue base weakness
6. Reduced laryngeal elevation.
7. Zenker’s Diverticulum

Phases of Swallowing Effected:

Oral Phase: The oral phase of the swallow may be marked by slow bolus delivery due to slowed limb movements. Patients may also exhibit slowed mastication. Patients are at risk for malnutrition because feeding is difficult and lengthy and patients may lose motivation to finish meals.

Pharyngeal Phase: The pharyngeal phase of swallowing is potentially the most affected by IBM. This is because this phase in swallowing relies on skeletal muscles that may be affected by myopathic changes. The upper esophageal sphincter (UES), which allows food to pass into the esophagus may be unable to relax, causing dysphagia.

Swallowing Rehabilitation:
Patients should receive clinical dysphagia evaluations along with videofluoroscopy.  Swallowing rehabilitation can include: diet modifications, feeding strategies, compensatory techniques, and exercises. A combination of oral and enteral feeding may be necessary for some patients. Extreme cases may require exclusive enteral nutrition.


Feeding Strategies:
Feeding strategies include:
1.      Chewing food well
2.      Double swallow
3.      Small bites
4.      Alternating solids and liquids
5.      Upright position during feeding

Compensatory Techniques:
Compensatory techniques include:
1.      Chin tuck
2.      Head turn, trial both sides
3.      Effortful swallow
4.      Mendelsohn maneuver
5.      Supraglottic swallow

Swallow Exercises:
Swallow exercise should be based off of swallow dysfunction noted during videofluoroscopy. Typical exercises implemented include:
1.      Masako (tongue hold)
2.      Mendelsohn maneuver
3.      Falsetto
4.      Supraglottic exercises

Interventional Measures:
Swallow rehabilitation including the above mentioned strategies might not be sufficient in treating some patients with IBM.  Interventional measures include:
1.      Cricopharyngeal myotomy
2.      Pharyngoesophageal dilation
3.      Botulinum injections
4.      Percutaneous endoscopic gastrostomy

Of these interventional measures, cricopharyngeal myotomy is the most well documented surgical technique for alleviating cricopharyngeal spasm or uncoordinated pharyngeal contraction.  The best results are when a long myotomy (6 cm or greater) is created.  This is because it incorporates the cricopharyngeus that extends into the constrictor above and the esophageal musculature below, which is aimed at improving pressure generation and cricopharyngeal opening.

Dysphagia is a progressive condition in patients with IBM.  It often leads to death from aspiration pneumonia. Immunosuppressive therapy has shown not to be beneficial when treating patients with IBM. Treatment of CP dysfunction seems to provide some symptomatic benefit.  Unfortunately, due to the progressive nature of the disease, pharyngeal function seems to worsen and enteral feeding may become the patient’s only option. Until then, feeding strategies, compensatory techniques, swallow exercises, and interventional measures can help in treating dysphagia in patients with IBM.  


References

Dalakas, M. C., Sonies, B., Dambrosia, J., Sekul, E., Cupler, E., & Sivakumar, K. (1997). Treatment of inclusion-body myositis with IVIg: A double-blind, placebo-controlled study. Neurology, 48(3), 712-716.

Dalakas, M. C. (2015). Inflammatory Muscle Disease. Inflammatory Muscle Disease, 372(18).

Houser, S. M., Calabrese, L. H., & Strome, M. (1998). Dysphagia in patients with inclusion body myositis. Laryngoscope The Laryngoscope, 108(7), 1001-1005.

Oh, T. H., Brumfield, K. A., Hoskin, T. L., Kasperbauer, J. L., & Basford, J. R. (2008). Dysphagia in Inclusion Body Myositis: Clinical Features, Management, and Clinical Outcome. American Journal of Physical Medicine & Rehabilitation, 87(11), 883-889. Retrieved February 14, 2016.

Pars, K., Garde, N., Skripuletz, T., Pul, R., Dengler, R., & Stangel, M. (2013). Subcutaneous immunoglobulin treatment of inclusion-body myositis stabilizes dysphagia. Muscle & Nerve Muscle Nerve, 48(5), 838-839.

TMA - The Myositis Association. (n.d.). Retrieved February 28, 2016, from http://www.myositis.org/

Wintzen, A. R., Ambots, G. T., De Bakker, H., Hulshof, J. H., & Padberg, G. W. (1988). Dysphagia in inclusion body myositis: Clinical Features, Management, and Clinical Outcome. Journal of Neurology, 51, 1542-1545.




Monday, July 6, 2015

Speech Therapy Treatment for Myasthenia Gravis




My student Julie Frost did a project where she researched info and treatment considerations for Myasthenia Gravis patients.  I think she did an excellent job!  There are really specific dysphagia guidelines that helped us withe a patient we had on Rehab who was having severe swallow issues, and severe fatigue.  

I think a lot of times these patients are not treated appropriately.  We really should be timing their treatment and meals with their medication dosages, and providing fewer or shorter sessions including co-treats if necessary so that we don't over-fatigue them and hinder their progress instead of helping. And we also need to be careful not to eat up their outpatient benefits of they are not ready yet, are still in a myasthenic crisis.  

Let me know in the comments if you find this helpful or have anything to add.  

Good job Julie! Thanks for letting me share this on the blog!




~Kristin

Background, Causes, and Symptoms
MG - grave muscle weakness

            Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease characterized by varying degrees of weakness of the skeletal (voluntary) muscles of the body without any other signs of neurological deficit. MG may affect any voluntary muscle, including those used to speak, breathe, and swallow, as well as limb, trunk, and eye muscles.
Muscle weakness usually occurs during periods of activity and improves after periods of rest. This waxing-and-waning weakness of muscles, worsening with use and improving with rest, is a hallmark of this particular disease. There typically are periods when you may notice more symptoms (exacerbation), interspersed with periods when symptoms decrease or disappear (remission).
            Aside from generalized muscle weakness, common symptoms include double vision, dysphagia (oral, pharyngeal, and possibly esophageal), dysarthria, trouble with balance, and shortness of breath. A myasthenic crisis occurs when weakness affects the muscles that control breathing, creating a medical emergency. Deterioration can be abrupt and may require the patient to be put on a respirator. This can be triggered by infection, fever, medication side-effects, and emotional stress.
            The general cause of MG is a disruption to the communication between nerves and muscles at the point of the neuromuscular junction. The specific neurotransmitter involved is acetylcholine. Though the exact cause of MG is unknown, it is thought to be related to a thymus gland abnormality, which is a gland located under the breastbone that is important in developing the immune system in early life. MG can occur at any age, but is most common in women under the age of 40 and men over the age of 60. MG is not contagious or fatal, and does not affect life expectancy. It tends to progress slowly and treatment is often highly successful. MG typically reaches maximum severity within the first 5 years after onset, with males having more rapid symptom progression. Most patients with MG improve to live healthy lives with close to normal activity levels.

General Treatment: Medical

Factors that determine medical intervention include severity of weakness, which muscles are affected, patient's age, and co-morbid medical conditions. Medical interventions are as follows:

  • Immunosuppressant Treatments
    • Anticholinesterates: drugs that increase muscle strength and contraction; symptom relief can occur in as little as 15-30 minutes, and last as long as 3 to 4 hours; can be administered 3 to 4 times per day, depending on dose size (mg); side effects can actually cause additional weakness and symptoms similar to those of MG.
    • Azathioprine: long-term immunosuppressive treatment (around 3-12 month activation period) that gradually improves muscle strength and decreases severity of symptoms.
    • Corticosteroid Therapy: improves muscle strength, but also may cause transient deterioration upon initiation of treatment
o    High-dose intravenous immune globulin (IVIG): Can be effective to temporarily modify the immune system and provide the body with normal antibodies from donated blood.
  • Thymectomy
    • Surgical removal of the thymus gland. This procedure takes years to have an effect and is used for long-term control of MG, possibly resulting in complete remission.
    • Effective for symptom relief in patients under 60 years of age who have a tumor or abnormality of the thymus gland.
  • Plasmapharesis
    • Plasma exchange therapy typically involving 3 to 5 exchanges of 1 or 1.5 volumes (based on patient's height and weight) per week until the patient shows satisfactory improvement.  Usually combined with immunosuppressive treatment.
    • Helps to remove antibodies from the body.
  • C-PAP or Bi-PAP
    • Used with patients whose respiratory muscles are too weak to properly function on their own.
    • May be used throughout the day or during sleep as needed, as well as during a myasthenic crisis.
Treatment: Speech-Language Pathology

Voice and Speech:

            Voice problems correlated with myasthenia gravis include vocal fatigue, difficulty controlling pitch, and a monotone voice. The voice problem can stem from poor breath support, or from weakness causing disordered vocal fold movement. Speech disturbances include hypernasality (caused by weak palatal muscles) and dysarthria (particularly slurred speech, caused by articulatory imprecision). Dysarthria is more frequently seen in younger patients diagnosed with MG, whereas dysphonia is more often seen in elderly men with MG. Typically, the symptoms appear and/or worsen with continuing or extended speech.
            The treatment of speech and voice disorders in MG should occur on an individualized basis, and should take into consideration the underlying cause and severity of the problem. Speech and voice treatment should occur in conjunction with medical treatment, with the SLP planning treatment during peak medication times. The focus of speech and voice treatment in individuals with MG should be in providing compensatory strategies and environmental modifications to help increase intelligibility. Additionally, the patient should be educated on vocal hygiene, with specific emphasis on vocal rest.
            A high- or low-tech augmentative and alternative communication (AAC) device may be useful for patients to communicate their wants and needs while resting their voice. Low-tech devices are particularly recommended during myasthenic crises when it is expected that the patient will be weaned off the ventilator in under 2 weeks. During periods of exacerbation or crisis, patients should be taught to communicate using function or content words, thereby decreasing the overall length of their message and resulting fatigue. Treatment may include strengthening exercises, but this is not indicated during a myasthenic crisis or exacerbation (see below for more information). Improvement and prognosis of voice and speech is often related to the treatment of overall MG disease.

Swallowing:

            The impact of MG on swallowing may occur gradually or suddenly. Swallowing muscles may become fatigued during a meal in as little as 1-20 minutes. A FEES examination is recommended for assessment because it can be used over an extended period of time to assess laryngeal fatigue and potential progression of aspiration throughout a meal. This will help ensure that the patient's fatigue does not overwhelm their swallowing safety, and will allow for appropriate recommendations to be made. The SLP should always be sure to take into account the timing of the patient's medication cycle when interpreting assessment results. The SLP should carefully schedule the examination to be at peak performance time (to see the patient's optimal swallow), or at a less peak time (to observe a more realistic situation).  Dysphagia symptoms in individuals with myasthenia gravis include the following:

Oral
·         anterior spillage
·         prolonged and incomplete mastication
·         trouble forming cohesive bolus
·         oral residue
·         decreased posterior lingual bolus propulsion

Pharyngeal
·         delayed onset of laryngeal elevation and epiglottic inversion
·         vallecular residue due to decreased tongue base to pharyngeal wall approximation and decreased pharyngeal contraction (with resulting multiple swallows)
·         decreased overall pharyngeal peak pressure
·         delayed pharyngeal response, possibly causing aspiration before and during the swallow
·         decreased VF closure, aryepiglottic closure/tightening, aryteniod-epiglottic approximation, epiglottic retroversion
·         note: prognosis tends to be poorer in individuals with pharyngeal phase dysphagia

Esophageal
·         the muscles which provide traction force to open the PES may not produce sufficient force to pull the PES open
·         decreased hyoid elevation and excursion, resulting in decreased PES opening
·         prolongation in mean duration of the peristaltic wave in the upper esophagus
            Treatment for dysphagia should be individualized and based on the underlying cause and severity of the swallowing problem. It should be noted that there is a moderate risk of silent aspiration in this population, and decreased pulmonary function can lead to a myasthenic crisis. Signs and symptoms of aspiration (coughing, throat clearing) should be minimized at all costs to reduce the risk of fatigue in the respiratory muscles. If these signs and symptoms are present during treatment, allow the patient time to rest and recover muscle function before proceeding. 

Exercise
o    exercise is generally not recommended due to patient fatigability and energy expenditure, particularly during a myasthenic crisis or exacerbation. However, an active strengthening program may be implemented during periods of stability or remission, considering that medical treatments are stable and well-managed.
o    consistent exercise will elevate the patient's baseline functional capacity, and thereby decrease the effect of future MG exacerbations and likelihood of aspiration.
o    low intensity exercise (60% of max or less) is most beneficial to maintain and even regain some muscle strength and function.
o     
    Recommended exercises:
§  Masako: improve bilateral pharyngeal contraction
§  Mendelsohn: improve laryngeal elevation

o    Precautions:
§  always closely monitor for immediate during- or post-exercise fatigue, as evidenced by decreased swallowing or speaking function. If fatigue causes functional impairment, reduce the exercise intensity.
§  do not prescribe or practice exercises around meal times.
§  try to complete exercises at peak medication time, near 1.5 to 2 hours post-dosage of anticholinesterase.
§  collaborate and communicate with the patient's other therapists to make sure that he or she is not overwhelmed by the sum of all of the individual areas of therapeutic exercise.
Diet considerations:
o    patient strategies can include:
§  Alternate liquid/(semi)solid swallows to wash thicker material down.
§  Consider diet with softer textures, less chewing required.
§  Multiple swallows per bite to clear residue (use with caution because series of repetitive swallows may be too fatiguing).
§  Eat several small meals throughout the day, rather than 3 large meals.
§  Eat calorie-rich foods as much as possible to ensure adequate nutrition.
§  Crushing or halving pills to reduce swallow effort and risk of aspiration.
o    warm liquids, like coffee, tea or soup, can relax swallowing muscles and further exacerbate swallowing difficulties. Ice chips can be a great alternative.
o    very hot, spicy, and dry foods may trigger MG symptoms.
o    introduce potassium-rich foods if a deficiency is the source of muscle weakness.
o    consider patient quality of life when making diet recommendations. Limiting diet in patients who already find eating to be a chore may lead to more negative reactions toward food.
o    if the patient has moderate to severe dysphagia, an NG or PEG tube may be indicated as primary or secondary means of nutrition and hydration. 

Positioning
 o    a properly-fitted removable cervical collar can help support the patient's neck and reduce muscle fatigue.
o    sitting fully upright will reduce the risk of nasal regurgitation.
o    when considering compensatory swallowing maneuvers, always try strategies that require less effort first before those that require more effort (e.g., chin tuck before supraglottic swallow).
o    effective minimal-effort strategies
§  head turn: decreases pharyngeal pooling by narrowing pharyngeal area to compensate for lack of muscle constriction.
§  have the patient turn to the weak side (if unilateral weakness) to divert the bolus to contralateral stronger side .
§  head tilt: toward stronger side (if unilateral weakness) to compensate for oral and/or pharyngeal weakness.
§  chin tuck: compensate for reduced bolus control with aspiration before or during the swallow.

Timing
 o    be sure that the patient has adequate rest time before meals or PO trials, and avoid conversation during these times.
o    time the patient's meals around their medication cycles (especially those that are intended to improve muscle function).
o    due to the fact that muscle weakness usually occurs during periods of activity and improves after periods of rest, the patient should have a rest break between each therapy session.
o    patients benefit from shorter treatment sessions with frequent rest breaks.


Neuromuscular Electrical Stimulation (NMES)

o    aim to strengthen muscles without causing fatigue.
o    since not all motor end plates are affected to the same degree in MG, some motor units (ones that are less affected) may have greater capacity for improvement than others.
o    limited research base for NMES in this population. The following are recommended guidelines:
§  Initiate a conservative exercise program at moderate exercise intensities while closely monitoring for fatigue and functional decline.
§  Add electrotherapy to facilitate the process if no progress is made but only if no functional declines were observed.
§  If still no progress is made and/or functional declines are observed, stop electrotherapy.
o    functional decline with NMES will be noticeable during or immediately after the very first session. If this is observed, NMES should not be considered as a therapeutic option for that patient.
            Dysphagia may persist when other clinical symptoms have improved. It is important for patients to have their swallowing function re-assessed every 3 months, even when in a long-term remission period. This helps to manage potential aspiration before it adversely affects pulmonary function and creates a possible exacerbation of MG symptoms. The patient should be extensively educated on signs and symptoms of aspiration and beginning signs of pneumonia, in order to ensure on-going self-monitoring skills. The SLP should also make it a focus to reduce the patient's stress and anxiety as much as possible during his or her stay in the hospital, due to the impact of increased stress and emotionality on a myasthenic crisis. This can be achieved through education, collaboration, and building rapport.



References:
            Duhon, K. & Tompkins, S. Myasthenia Gravis: Treatment of Swallowing Disorders (Retrieved from http://www.ucs.louisiana.edu/~ncr3025/roussel/codi531/MG2.htm)
Grob, D., Arsura, E. L., Brunner, N. G., & Namba, T. (1987). The course of myasthenia gravis and therapies affecting outcome. Ann N Y Acad Sci. 505:472–499. doi: 10.1111/j.1749-6632.1987.tb51317.x. 

Howard, J. F. Jr., Ed. (2008). Myasthenia Gravis: A manual for the health care provider. Myasthenia Gravis Foundation of America. (Retrieved from: http://www.myasthenia.org/LinkClick.aspx?fileticket=S472fPAE1ow%3D&tabid=69)

Juan, H.-C., Tou, I., Lo, S.-C., & Wu, I.-H. (2010). Efficacy of postural techniques assessed by videofluoroscopy for myasthenia gravis with dysphagia as the presenting symptom: a case report. Journal of Medical Case Reports, 4, 370. doi:10.1186/1752-1947-4-370 (Retrieved from http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3009659/ )

Myasthenia Gravis Foundation of America, Inc. (www.myasthenia.org)

            Patient Education Institute, Inc. (2012). Myasthenia Gravis. National Library of Medicine. (Retrieved from http://www.nlm.nih.gov/medlineplus/tutorials/myastheniagravis/nr239106.pdf)

Schwartz, D.C., Waclawik, A.J., Ringwala, S.N., & Robbins, J. (2005). Clinical utility of videofluorography with concomitant Tensilon administration in the diagnosis of bulbar myasthenia gravis. Dig Dis Sci.;50:858–861. doi: 10.1007/s10620-005-2653-2. 

Vital Stim Therapy System (2009). Guidance from the literature: Myasthenia Gravis (Retrieved from http://www.vitalstim.com/uploadedFiles/Health_Professionals/Certified_Provider_Resources/Disease_Specific_Information/MG_111309.pdf)