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.

Review of new product- the Forbrain

I got the opportunity to try a product from Europe called Forbrain.

First of all the company has been so great to work with and so dedicated to their product.  They were also patient with me as I took an ETERNITY to write this review! 
The Fobrain offers auditory feedback via bone conduction!  They call it an "enhanced audio vocal loop."  It's supposed to increase your sound encoding skills.  They say it can improve your comprehension of the info you read aloud, it can help your attention to the task, your encoding it into memory, and your improved speech and voice as you listen to yourself, among other things.  Basically, while you're speaking, you can hear what you're saying more loudly and intensely as it comes back to you.  The "speaker" part of the device rests in front of your ears right on your bones and the sound is vibrated through to your ears.  The company suggested I try it out with patients working on voice, motor speech, and cognition.

I tried the device on myself first, and did some reading out loud. I could definitely hear my speech coming back to me loud and clear, and I could feel the vibrations in my bones. I immediately thought this would be great for those patients with voice issues or dysarthria who are less aware of their speech problems, or who have a hard time hearing the subtle things we are asking them to listen for. Even artic problems could benefit I'm guessing.

Of course, after wearing it myself and doing some reading, it's very hard for me to judged accurately whether or not it improved my attention and memory, etc.  There are some clinical trials going on in Europe right now to test those things.  It will be interesting to see the results.  If the company can still stand to work with me after me being so slow and annoying, I'll keep you posted.  :)

I then tried the device with several of my patients, since it is obviously not harmful, and since any additional potential help to my dysarthria and voice therapy skills is welcome. My patients have all commented that the device is comfortable, seems to be helping.  I did notice with two particularly quiet and dysarthiric patients, their speech did seem to improve mildly after placement of the device.  I think I will hang onto it and continue to use it with patients who need a little something more than traditional tx, or who are not responding to my cues well.  I tried it with a couple of patients working on memory and attention, and again, they said the device seemed helpful in staying on task and remembering what they were reading. Again, I don't see why this couldn't be used by me experimentally to help give patients a "boost."

Several of my colleagues are considering using the device experimentally, and we even considered doing a simple research project and presenting a poster at ASHA.  This could be a future research project!

In conclusion, I think the deice has some great potential.  I love to try to things, and will continue to use it with dysarthria and voice patients who need more help with listening, perceptual skills, changing subtle thinks in their speech, resonance, voice, etc.  And I'll be watching for the results of these trials in Europe.  If you are interested in the device, you can leave a comment below and I'll ask my contact person if they would like to contact you for beta testing, reviewing, research, or purchasing. I don't know if they are currently available for purchase in the US. 

Orientation and calendar task

Ohhh my goodness, I have not blogged in an eternity!!  Sorry.  I had a baby and life got too crazy.  She is an adorable baby!!!  I'll post pictures.  In the mean time, here's a little orientation task I just created since working on orientation can feel kinda...repetitive and boring.  :)

Orientation and calendaring exercise for Inpatient Rehab

• Number each day in this month
• Place a small post-it on today’s square.
• Write the word “today” on the post-it. 
• Are there any holidays (or family birthdays) this month- fill them out.
• What events or appointments are coming this week?  Point to them or fill them in.
• Which is the first day of the week this month?
• Count how many days from today to the end of the month.
• How many Sundays are there in this month?
• On which day of the week does the 14^th fall?
• Which day did you arrive in Rehab?
• How many days have you been here?
• Which day will you be leaving?
• How many days until you discharge?
• How many days (total) will you have been in Rehab by the time of your discharge?
• What season(s) is/are this month? (summer, fall, winter, spring)

For home

Each day:

• Ask a family member or caregiver to join you in doing your orientation exercise. 
• Try to say the date without looking at the calendar.
• Move the “Today” post it forward one day.
• Say the date now, looking at the calendar. 
• Cross of the previous day (all days that have passed should be crossed off).
• Look at upcoming appointments/events.
• Write any to-dos, appointments, events that you learn about today.
• Make any preparations you need to make for those events (e.g., if there is a family birthday tomorrow, do you need to get a gift or card?  If there is a therapy appointment this morning, do you need to shower, dress, etc.?)
• Say the date without looking at the calendar.

Radiation Safety and Guidelines During MBS / VFSS for SLPs and Patients

Hi friends,

I have had some awesome student interns lately.  Each of them completes a student project of some kind.  some of them are so awesome, I have decided to ask the students' permission and post some of them on the blog.  Here is the first of many-- a project by Amelia Bergmann.

Image source: http://lc19.blogspot.com/2010/06/meet-readi-cat-worse-than-drinking.html

The story:
We have had several issues lately regarding confusion about how much radiation is too much, how many frames per second is safe, etc.  Some of the techs in radiology are hesitant to film the studies at 25 frames per second or more, but we feel we can not make adequate measures and judgment calls with anything less.  The alternative with our machines is 12.5 frames/second!  Just terrible.  Jeri Logemann said anything less than 30 is abysmal.  How can we convince the techs that this is not that much more, and is safe for them and the patients (and is it??)?  We didn't really understand ourselves some of the ins and outs of radiation/fluoroscopy safety.  I put this issue on my to do list and my student project list in case anyone was interested, and Amelia took me up on it. She completed this Powerpoint presentation about Fluoroscopy.  I thought she did a fantastic job!  Some of the guidelines are specific to our hospital , so you would have to research your own hospital's guidelines.  Some of the questions Amelia investigated included:

• An MBS/VFSS exam is equivalent to how many hours in the sun?
• How much protection does the lead provide?
• Where do SLP's need to stand in order to avoid radiation scatter during MBS/VFSS?
• What is the pattern of the radiation scatter during the MBS/VFSS?
• How much more radiation is the patient receiving when the frames/second are increased?  
• What are the guidelines from pregnant patients and SLPs/therapists/techs?
• Should we wear lead or radiation-resistant gloves and goggles during MBS/VFSS procedures? 
• What are the radiation dose limits for SLP's and patients?

She interviewed the hospital's radiation physicist, the Radiology PA, looked up hospital and State guidelines and scoured ASHA and research articles.

So check it out!  I have pasted the slides here below.  Amelia and I hope it helps other SLPs with their questions about the mysteries of fluoroscopy.  :)
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Radiation MBS/VFSS Safety Information:

What Is Radiation?

• Energy in the form of particles and waves
• Ionizing: X-rays and gamma rays
• Some x-rays enter body and interact partially, completely, or not at all
• Denser tissue absorbs more radiation than less dense tissue

Local Radiation Regulations (Utah):

• www.radiationcontrol.utah.gov
• Utah law mirrors the federal laws

Radiation Dosage info:

• Radiation is all around us
• Inverse square law (radiation received and distance from source)
• Received through naturally occurring radionuclides (soil, food, water) and through radon indoors
• The type of radiation emitted during fluoroscopy is not the same type of "rays" as the rays of the sun- so telling a patient that an MBS is like __ hours in the sun is not accurate.  They are not comparable. 
• No “zero dose” of radiation exists!

                                                                                                                       (ASHA, 2004)

• Naturally occurring: 300millirems/year
• 300mr added from medical and man-made sources (mostly in latter years of life)    

Radiation Safety Education:

• Education on radiation safety is not standard in graduate programs for speech pathology
• SLP’s responsibility to be aware of guidelines, etc. to reduce radiation exposure to self and patient  

ASHA Guidelines on Radiation;

• Guidelines for Speech-Language Pathologists Performing VFSS:
• Principle of Ethics II Rule B: “Individuals shall engage in only those aspects of the profession that are within their competence, considering their level of education, training, and experience” (ASHA, 2003). Furthermore, individuals should consult state licensure laws regarding speech-language pathologists (SLPs) utilizing the VFSS in their particular state.

Average VFSS/MBS Radiation Exposure:,

• Average: 1 millisievert
• Exposed to 50 millisieverts in first 17 years of life
• Low dose and low risk procedure
• Little radiation exposure and dose = no risk when compared to medical benefit
• Reducing exposure

1) Time that fluoroscopy is on
2) Distance from the source and patient
3) Shielding

Fluoroscopy time during a study:

• Should not exceed 5 minutes (if possible)
• Less time spent in fluoro = less radiation exposure

C Arm:

• The arm is the source of radiation to patient 

Scatter:

• Radiation emitted from the patient (not the direct beam)
• Radiation hits the patient’s tissue, interacts and then changes direction
• Decreased by a factor of 1,000 for every 1 meter in distance

                                                      (P. Jenkins, personal communication, August 13, 2013

• Distance from the beam
• Distance of 6 feet or more from patient:
• Exposure due to scatter and leakage radiation from x-ray tube is so small that wearing apron does not add much protection

                                                                                        (X-ray Radiation Protection Manual, 2010

• SLP can move to the side of the image intensifier, behind radiologist

Fluroscopy Collimation:

• The radiation beam can be collimated to reduce scatter and enhance the contrast of the image
• If radiologist or tech does not collimate, request it                                                                                                        (Kelchner, 2004)
• Collimators can help change the shape (round, square) and size (large, small) of the beam                           
  

Panning:

• A/P view of espohageal phase of swallow
• Increases amount of radiation given off
• Should be kept to minimum
• No need for clinician to be close to patient
• Stay back or step behind the secondary barrier                                                                                      (Kelchner, 2004)
• More radiation required to go through thicker abdominal tissue vs. pharynx, more scatter produced                                                                       (Hayes et al, 2009)

FDA: Radiation Risks:

• Radiation burns and/or radiation-induced cancer
• Probability = very small
•  Radiation risk is usually far less than other risks not associated with radiation, such as anesthesia or sedation, or risks from the treatment itself.
• Fluoroscopy should always be performed with the lowest acceptable exposure for the shortest time necessary

UofU Hospital Guidelines:

• Part of Utah Administration Code R313-15-201
• Occupational dose limit for adults: 5 rem/year (5,000 millirem)
• Non-occupational dose limit (members of the public and non-radiation workers): 0.1 rem/year
• Pregnant worker dose limit: 0.5 rem/9 months

                                                                                     (X-ray Radiation Protection Manual, 2010)

• 1 rem = 1,000 millirems (10 millisieverts)

ALARA: As Low as Reasonably Achievable:

• Risk-benefit approach
• Considers amount of exposure + need for exposure
• If a pt’s radiation exposure is below the limit, should they receive radiation?
• Depends on the need/benefit of the exposure                                                                                (X-ray Radiation Protection Manual, 2010)

                                -Professional risk and benefit weighed differently than patient risk and benefit
                                -No radiation dose limit for patients (MD decides)                                    
                                       (P. Jenkins, personal communication, August 13, 2013)

                                                                                               

Dosimeter badges:

• Utah Division of Radiation Control regulations   
• Requires only those who have a reasonable chance of exceeding 10% of the annual occupational exposure limit (5000 millirem) or those who operate a fluoroscopic x-ray unit
• Worn on collar (or waist or finger, if issued)
• Best legal proof for radiation exposure at work
• Make sure that all radiation exposure is recorded (esp. unanticipated exposure!)
• Good for radiation protection program
• Fee for lost, damaged or “late” badges
• Individual dosimetry report can be requested at any time

                                                                                             (X-ray Radiation Protection Manual, 2010)

Wearing Lead:

• Leads are checked at least once/year
• Not truly lead, but rubber material with some copper and lead interspersed
• 1/2mm of lead-like material = min protection
• 1 lead apron will block 95% of primary beam
• 1 lead apron will block 99% of scatter
• Different organs respond to radiation differently
• Limits:

Head and torso: 5,000mr/year

Eyes: 15,000mr/year

Extremities: 50,000mr/year

Reducing Radiation Exposure

• Know rationale for conducting a study (or repeating one)
• Investigating the appropriateness of referrals ensures that fluoroscopy time is necessary
• Rotating SLPs who do VFSS
• Use FEES when possible if multiple studies will be needed

                                                                                                                     (Kelchner, 2004)

Pregnancy and Radiation Exposure

• National Council on Radiation Protection-no. 116 requirements:
• Dose shall not exceed 5mSv to the fetus during 9 months (0.5 mSv per month)

                                                                                          (X-ray Radiation Protection Manual, 2010)

• 1 millisievert = 0.1 rem
• Limited involvement with close sources of radiation, to the extent practical
• Work in protected areas or wear protective clothing when radiation present
• Wear additional pregnancy dosimeter at waist level (worn under apron)
• Monthly update with DRP

                                                                                           (X-ray Radiation Protection Manual, 2010)

• No justification to wear 2 lead aprons
• If concerned, get thicker lead apron, but this is not really necessary
• Can’t refuse to do radiation-related activities if under radiation limit

                                                                        (P. Jenkins, personal communication, August 13, 2013)

References:

American Speech and Hearing Association. http://www.ASHA.org

“Fluoroscopy Radiation and Safety Training Manual” (n.d.). https://www.case.edu/ehs/Training/RadSafety/fluoro.htm

Hayes et al. (2009). Radiation safety for the speech-language pathologist. Dysphagia, 24: 274-279.

Health Physics Society (2013). Answer to question #10592 submitted to “ask the experts.” https://hps.org/publicinformation/ate/q10592.html

Kelchner, L.N. (2004). Radiation safety during the videofluoroscopic swallow study: The adult exam. Swallowing and Swallowing Disorders, 24-28.

“Radiation Emitting Products” (n.d.) http://www.fda.gov/Radiation-EmittingProducts/RadiationEmittingProductsandProcedures/MedicalImaging/MedicalX-Rays/ucm115354.htm

X-Ray Radiation Protection for Diagnostic X-ray use at the University of Utah Hospitals and Clinics (2010).

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That's it folks.

Kristin

(Note:  I was having some serious trouble with formatting and inserting pictures into this post.  I am aware of some of the inconsistencies in the formatting.  I don't seem to be able to fix it.  Need to take an HTML course I suppose.  :) )