⚀ 2. Complications and Comorbidities.

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⚁  2.1 Complications.

⚂  2.1.1. Dysphagia: difficulty swallowing.

⚂  2.1.2. IBM and Respiration.

⚂  2.1.3. Peripheral neuropathy.

⚂  2.1.4. Falls.

⚂  2.1.5. Edema.

⚂  2.1.6. Lifestyle.

⚁  2.2 Comorbidities.

  This is the most important section of the website.
≻ Most people with IBM encounter complications.
≻ Some of these are a nuisance, like having excess saliva and mucus.
≻ Others pose a threat to the life of the patient.
≻ Awareness and management of complications is vitally important for both the patient and the doctor.
≻ How complications are managed makes a direct impact on the quality of life and the mortality of the IBM patient.

  Comorbidity describes the situation when a patient has a disease and has been diagnosed with a second or third (or more) other diseases.
≻ It is important for you and your doctor to be aware of other diseases that commonly occur along with IBM in case you develop one of them.
≻ This helps the doctor understand the context of the other disease(s).

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⚂  2.1.1. Dysphagia.

⚃  Dysphagia means difficulty swallowing.
≻ This is a prevalent and severe complication that many, if not most, IBM patients have at one stage in their disease.
≻ Unfortunately, it is connected with the death of many people who have IBM.
≻ It is now recognized that many patients, researchers, and doctors are not aware enough of this complication and are not watching for it.

⚃  Here is a review of the problem: Garand, K. L. (Focht), Malandraki, G. A., & Dimachkie, M. M. (2023). Update on the evaluation and management of dysphagia in sporadic inclusion body myositis. Current Opinion in Otolaryngology & Head & Neck Surgery . https://doi.org/10.1097/MOO.0000000000000922 DOWNLOAD PDF.

⚃  Here is a review of the problem: Ambrocio KR, Garand KLF, Roy B, Bhutada AM, Malandraki GA. Diagnosing and managing dysphagia in inclusion body myositis: A systematic review. Rheumatology (Oxford). 2023 Oct 3;62(10):3227-3244. doi: 10.1093/rheumatology/kead194. PMID: 37115631. DOWNLOAD PDF.

⚃  Here is an excellent overview from the Outlook magazine (2003) (pdf) of the Myositis Association of America.
Here is another version of the same article: Version two (pdf).

⚃  Here is a 2019 article describing the problem (pdf).

⚃  Here is a synopsis of the above article in everyday language (pdf).

⚃ In from 40 to 85% of IBM cases, people will develop weakness in the pharyngeal muscles, the muscles used in swallowing, resulting in dysphagia.

⚃  Dysphagia in IBM is common but under-reported by the vast majority of patients if not specifically asked about.   In practice, three questions reveal problems:
≻ 1). ‘Does food get stuck in your throat?’
≻ 2). ‘Do you have to swallow repeatedly in order to get rid of food?’
≻ 3). ‘Have you choked while eating?’

⚃ This weakness is generally progressive and if present should be evaluated and reviewed for treatment.

⚃ Dysphagia is characterized by food getting stuck “half way down” resulting in choking episodes.
≻ In some cases, during these episodes food gets drawn into the lungs, a situation called aspiration. When this happens, pneumonia often develops.

⚃ In IBM patients, dysphagia is a significant cause of death from respiratory complications associated with aspiration pneumonia.

⚃ To reduce the risk of choking while eating, a few simple rules will help.

⚄ Think about and talk about these issues before you eat: it’s often the case that you don’t expect to choke, and especially if it’s the first time you’ve had real problems, it can be very frightening if you haven’t thought about it and have a plan.

⚄ If you become afraid to eat, tell someone on your medical team because this is a big red flag that needs to be addressed.

⚄ Always have liquid available when you eat.

⚄ Before eating, take a drink to wet your throat.

⚄ Eat slowly and deliberately, concentrating on what you are doing.

⚄ Above all, do not rush and do not talk or attempt to have a conversation while eating.

⚄ Take small bites and chew your food completely.

⚄ Never eat in bed or while reclining.

⚄ Personally, I would suggest never eating alone and alerting your eating partner about what to do if you choke.
≻ This includes how you should signal them if you choke; you may not be able to make even a grunt or any sound, so alert your partner that if you choke, you will shake your head from side to side to signal them.
≻ Have your eating partner know the Heimlich maneuver and how to do it if you are in a wheelchair.

⚄ Some foods may be more difficult for you to swallow; the consistency of certain foods tends to form lumps of food in the throat; for example, many people have trouble eating french fries.
≻ Avoid dry foods; for example, cake is often troublesome.
≻ Learn what foods give you trouble, and either avoid these foods or be extra careful.

⚄ One recommendation is to eat foods in groups; for example, when eating a hamburger, separate the bun from the hamburger and eat them separately.
≻ The consistency of the food is important, especially bread, doughnuts, potatoes and other doughy foods.

⚄ Some small foods often get stuck more easily in your throat; for example, popcorn and rice can be tricky.

⚄ Take smaller quantities of food with each bite and ensure that the food goes down your throat; if necessary, take a small drink after each swallow.

⚄ If you feel that food is not going down or stuck, take a small sip of liquid and swallow. This often loosens and lubricates the food to pass.
≻ Once the food has passed, take a larger drink to clear the throat fully.

⚄ If you are coughing and some air is still passing by, try to take a very small amount of liquid to see if you can get the food down.

⚄ If you choke, the tendency is to panic, and I have found that the calmer you can be, the better.

⚄ If your air is cut off completely, you’ll likely need help getting the plug back up.

⚄ If you are choking, do not worry about the mess: it may be necessary to spit out food on your chest while clearing your throat.
≻ Focus on clearing your airway first and clean up later.

⚄ Watch for signs of chest congestion and coughing after a choking episode.
≻ Let your doctor know what happened; they may want you to be checked with an x-ray.

⚃ How to perform the Heimlich Maneuver on a Person in a Wheelchair

⚄ Step 1 Ask, "Are you choking?". A person who is choking will likely not be able to reply verbally.

⚄ Step 2 Remain calm and encourage the person to do the same. Try speaking to him or rubbing his back or arm to calm him.

⚄ Step 3 Look for signs that the person is suffering from total airway obstruction.
≻ These signs include the victim being unable to make any sounds above a wheeze, the face turning blue and hands clutching the throat in the universal symbol for choking.

⚄ Step 4 Engage the wheelchair's brake if it's not already on, or turn off a power chair.

⚄ Step 5 Try to perform the maneuver from the back, standing behind the person's wheelchair (Image 5a).
≻ If the back of the wheelchair is too high, do the maneuver from standing in front of the person (with him or her still sitting in the chair) (Image 5b).

⚄ Step 6 Lean the person forward, moving her head and torso down at a slight tilt.

⚄ Step 7 Make a fist with one hand.

⚄ Step 8 Place your fist just above the person's navel with your thumb in contact with his body.

⚄ Step 9 Grab hold of your fist firmly with your other hand.

⚄ Step 10 Make a quick in-and-up thrust against the person's diaphragm.
≻ You may need to repeat thrusting several times before the object is expelled.

⚄ Step 11 Repeat until the choking person can breathe, the object is expelled or the person loses consciousness.
≻ If necessary, lay the person flat and do the maneuver as pictured above.

⚄ Step 12 Call for medical help using 911 or another emergency number if necessary.

⚄ The feeling of choking often results in panic, when choking you need to tell yourself to relax and be as calm as possible until the episode can be resolved.

⚄ Sadly, many elderly IBM patients with dysphagia develop malnutrition because they are afraid to eat, or eat foods that are nutritionally inappropriate but are easy to swallow, for example, ice cream.

⚄ If you are an IBM patient and you feel you are developing difficulty swallowing you need to discuss this with your doctor who will refer you to a specialist for evaluation and treatment.
≻ Speech therapists often assess swallowing difficulties and there are tests that can be done, for example a barium swallow.

⚄ Treatment may involve exercises or in some cases an operation on the throat.

⚄ There is a muscle at the top of the esophagus called the cricopharyngeus.
≻ This muscle is normally contracted, closing off the top of the esophagus.
≻ Normally, when a person swallows this muscle momentarily relaxes opening the top of the esophagus allowing food to pass into the stomach.
≻ In some cases, individuals who have trouble swallowing may have a procedure (a myotomy) to disable this muscle, facilitating the passage of food into the stomach.

Image 5a

Image 5b pushing sharply in and up.

Image 5b alternate hand position.

⚃ Other resources (pdf):

⚄ Articles here. 2008 2009 2012 2014 2016

⚄  A 2006 textbook on dysphagia.

⚄  A 2012 textbook on dysphagia.

⚄  A 2015 textbook on dysphagia.

⚄  A 2018 textbook on dysphagia.

⚄  A 2019 textbook on dysphagia.

⚄ Schrey, A., Airas, L., Jokela, M., & Pulkkinen, J. (2017). Botulinum toxin alleviates dysphagia of patients with inclusion body myositis. Journal of the Neurological Sciences, 380, 142-147. https://doi.org/10.1016/j.jns.2017.07.031

⚄ Cox, F. M., Verschuuren, J. J., Verbist, B. M., Niks, E. H., Wintzen, A. R., & Badrising, U. A. (2009). Detecting dysphagia in inclusion body myositis. Journal of Neurology, 256 (12), 2009-2013. Article Here.

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⚂  2.1.2. Respiratory Issues.

⚂  2.1.3. Peripheral neuropathy.

⚃  It is commonly said that IBM does not attack nerves, however, it appears that a type of nerve problem may be a commonly seen complication in IBM.
≻ The cause of these problems is not known and may be independent of the causes of IBM.
≻ Specifically, problems in the nerves, called peripheral neuropathy, are seen in the hands and feet. One recent study (Lee et al., 2020), found peripheral neuropathy in about 35% of IBM cases.

⚃  Problems can be easily measured using nerve conduction studies.
≻ These studies measure the nerve impulse as it travels down the nerve.
≻ Abnormalities seen in the impulses point to disease or breakdown in the nerves, often related to problems in the myelin sheath that surrounds the nerve fibers.
≻ Demyelination is often seen in another nerve disease – multiple sclerosis.

⚃  The study by Lee also showed that the presence of peripheral neuropathy in IBM did not seem to be related to the severity of IBM.
≻ The exact relationship between IBM and the presence of peripheral neuropathy is not known.

⚃  As well, in the Lee study, about 50% of cases showed problems in what are called thermal thresholds - the ability to feel changes in temperature.
≻ This was tested by putting a patch over a muscle in the wrist and one over a muscle in the foot and the temperature of the patch was raised and lowered.
≻ The subjects were asked to push a button when they first notice a temperature change.
≻ Some 50% of the IBM cases showed abnormalities in being able to perceive temperature changes.

⚃  Common symptoms of peripheral neuropathy may include: numbness or tingling of feet or hands, burning sensations, sharp, jabbing, throbbing or burning pain, extreme sensitivity or not being able to feel and problems in coordination (all primarily in the hands or feet).
≻ If you have been diagnosed with IBM and you are experiencing any of these symptoms, you should bring them to the attention of your doctors.

⚃  Lee, J. H., Boland-Freitas, R., Liang, C., Howells, J., & Ng, K. (2020). Neuropathy in sporadic inclusion body myositis: A multi-modality neurophysiological study. Clinical Neurophysiology, xxxx.  https://doi.org/10.1016/j.clinph.2020.07.025

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⚂  2.1.4. Falls.

⚃  Injuries caused by tripping and falling are a major risk in IBM patients.
≻ Some patients benefit from leg or ankle braces (Knee-Ankle-Foot Orthosis - KAFO) or (Ankle-Foot Orthosis - AFO).
≻ A KAFO is a long-leg orthosis that spans the knee, the ankle, and the foot in an effort to stabilize the joints and assist the muscles of the leg.

⚃ Three basic falling scenarios are described.

⚄ 1. Falls caused by toe drop.

⚅ Toe drop is the situation where when taking a step the toe does not rise high enough causing the toe to stub and the individual falls forward onto their knees.
≻ In IBM, this is caused by weakness in the muscles in my leg responsible for lifting the toe.
≻ These falls are often one of the first symptoms noticed by the patient with IBM.
≻ A common example is a person stepping up onto the curb and not having the foot go high enough causing the toe to bump into the curb and tripping.

⚄ 2. Falls caused by losing one's balance.

⚅ These falls are often experienced as in "slow-motion," you start to lose your balance and realize that you're falling but you cannot stop yourself and you slowly fall over "like a tree falling in the forest."
≻ These falls can often result in back and head injuries. Walking on uneven surfaces like lawns is difficult for the IBM patient causing these kinds of falls.

⚄ 3. Falls caused by collapse of the knees.

⚅ In these falls, the knees collapse without warning and you fall straight down, landing on top of your feet.
≻ These types of falls are often experienced instantaneously and you are on the ground before you feel that you are falling.
≻ These falls can easily result in knee and ankle injuries.

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⚂  2.1.5. Edema (swelling).

⚃  Generally speaking, edema is a swelling of tissues caused by a pooling of excess fluid.
≻ It is often associated with people who are stationary for long periods of time causing fluid to accumulate in the ankles and lower legs.
≻ There can be several complications of untreated edema, in the worst case, the fluid can compress the tissues to the point that blood circulation is impaired resulting in damage.
≻ The best treatment for this is prevention through the use of pressure stockings which help reduce the pooling of fluid and keep the circulation moving.
≻ Unfortunately, these stockings are very difficult to put on by yourself when you have weakness in the hands.

⚃  The two gentlemen in these pictures have not had proper management (with the use of pressure stockings) to prevent edema and they now face potentially serious complications.

⚃  Here is an example of a pressure stocking.

⚃  It is important to understand that other medical conditions can also cause edema.
≻ If you have swelling, you should consult your physician to rule out other problems.
≻ Your doctor should also recommend a compression strength; the stockings come in different strengths.
≻ Usually, the doctor will prescribe a stocking; this is better than just going to the drug store and buying one off-the-shelf.

⚃  Edema is made much worse by high salt intake, and generally speaking, salt should be severely limited in your diet.
≻ Flying can also exacerbate edema because of the changes in pressure.

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⚂  2.1.6. Lifestyle Complications.

⚃  Also see the section on practical coping strategies

⚃  It is important to realize that the effects of IBM on one's lifestyle may produce secondary issues.

⚄ For example, as mobility is restricted, one's dietary intake should be reduced to avoid weight gain.

⚄ Restricted mobility, especially when using a wheelchair, may be related to the development of edema in the lower legs should be monitored.
≻ Edema can be prevented or limited by using pressure stockings and salt restriction in one's diet.

⚄ Restricted mobility is also an issue in skincare.
≻ Skin is prone to damage from excess moisture, and hygiene is essential as urine is a major irritant.
≻ Sitting for long periods in one position can cause creases and blisters in the skin.
≻ Finally, attention must be paid to sleeping as remaining in one position for long periods can develop pressure sores.
≻ Pressure sores can result in the breakdown of the skin or, in more serious cases, of underlying muscle tissue.
≻ These skin-related complications can be complicated to treat; therefore, prevention is the best strategy.

⚄ IBM presents a significant risk of developing bedsores.
≻ Pressure sores or bedsores are a serious complication for relatively immobile people.
≻ The major muscle atrophy in the thigh muscles seen in IBM also increases the risk.
≻ Pressure sores have several major aspects.
≻ One is gravity's simple but constant pressure as it pulls the body down.
≻ In cases where it is difficult (or impossible) to move or turn over in bed, pressure sores can rapidly develop (in as little as 12 hours).
≻ In cases where there is significant muscle atrophy, usually, the hip bone pushes down on the thigh muscles (and skin), impairing the circulation and breaking the tissues down.
≻ The key to preventing this is to have a soft mattress and move around enough that a "spot" of damage does not "buildup."
≻ In some cases, a special mattress surface can be used (often a rubber honeycomb type of pad that cushions the "boney spots.")
≻ These sores can also occur if you sit in a chair too long in one position.
≻ Another major issue is shear.
≻ Shear occurs when your body shifts its weight in one direction, but the skin does not move; it adheres to the surface under you.
≻ Sliding down in a bed or chair more than 30 degrees is likely to cause shearing, which stretches and tears cell walls and tiny blood vessels.
≻ Especially affected are areas such as your tailbone, where the skin is already thin and fragile.
≻ In IBM, shear is a problem as it is difficult to turn using the arms, and as the natural tendency is to try to turn over in our sleep, the skin often "sticks" to the covers or mattress, and shear forces are created (the body tilts over, but the skin does not move; it feels like there is a piece of tape holding the skin from moving).
≻ The best solution to this problem may be sleeping with the skin directly on a natural sheepskin.
≻ The best results are with a fairly thick pile (the thicker the cut, the better).
≻ The sheepskin also helps prevent moisture (sweat) buildup, another major factor contributing to skin irritation.
≻ If excessive moisture builds up, especially in the presence of urine or feces, there can be rapid skin irritation.
≻ In cases of IBM, pain perception is usually intact, and you will feel your hip getting sore. Do not ignore these pain signals.
≻ Lack of pain perception and sensation is a major complication in some cases, usually involving spinal cord injuries or disease.
≻ Whatever the cause, an inability to feel pain means you're not aware when you're uncomfortable and need to change your position or that a bedsore is forming; this situation requires active management and careful monitoring.

⚄ See: https://www.mattressadvisor.com/bedsores/.

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⚁  2.2 Comorbidities.

⚂ 2.2.1. Overview:

⚃ Comorbidity describes the situation when a patient has a disease and has been diagnosed with a second or third (or more) other diseases.
≻ These other diseases may occur spontaneously and be independent of each other (diabetes, prostate cancer) or may be causally related (diabetes, hypertension).
≻ There are also other situations; for example, disease one may directly cause disease two.
≻ Another situation is associated risk; we'll use the example of smoking. Many smokers also drink. Therefore, smoking and drinking are correlated risk factors. You may get COPD from smoking and have a higher risk for liver disease from alcohol consumption. Thus, smoking and drinking make it a higher risk that these associated diseases will occur together.
≻ In another example, disease one and disease two symptoms may be due to a third distinct disease (see Valderas, Starfi, & Sibbald, 2009).
≻ Comorbidities are associated with aging; more than 50 percent of older adults show occurrences of three or more chronic conditions (American Geriatrics Society, 2012).

⚃  Naddaf et al. (2021) found that some 36% of IBM patients showed peripheral neuropathy, 6% had Sjogren's syndrome and 10% had a hematologic malignancy.
≻ Some 60% of cases meet the criteria for T cell large granular lymphocytic leukemia (Greenberg et al., 2016).

⚃  Comorbidity is complicated by the lifestyle of IBM patient.
≻ For example, due to their disability, many IBM patients are sedentary – not moving around very much; thus, muscle disuse becomes a factor. Most people gain weight in this situation.
≻ People often consume high-fat, high sodium, low-protein diet and overeat. Thus they gain weight and often have high blood fat levels.
≻ These lifestyle factors put IBM patients at risk for diseases like type II diabetes.
≻ If you have IBM and develop type II diabetes, you now have the burden of both illnesses.
≻ It may be difficult to sort out what symptoms are from which disease because both IBM and type II diabetes are associated with muscle issues.

⚃  A study done by Keshishiana, Greenberg and others in 2018 showed that several diseases are comorbid with IBM.
≻ Patients with IBM are at an increased risk of cardiovascular disease compared to matched non-IBM patients, as indicated by associations with hypertension, diabetes, dyslipidemia, myocardial infarction, and congestive heart failure. Prevalences of hypertension (65.9 percent) and diabetes (25.2 percent) were found.
≻ Other IBM comorbidities may include muscle and joint pain and pulmonary complications.

⚃  It appears that IBM and type II diabetes mellitus both have impacts on muscle.
≻ For example, diabetes affects the muscles’ ability to contract under performance.
≻ It is thought that this may be related to problems with microcirculation of blood through the skeletal muscle fibre. (Frisbee, Lewis, & Wiseman, 2019).

⚃  Chronic diseases such as type II diabetes reduce the oxidative capacity as skeletal muscles.
≻ The increased death of mitochondria (mitophagy) is also related to common diseases symptoms.
≻ For example, factors such as hypoxia, inflammation, the disuse of muscles, and iron deficiencies all contribute to increasing mitophagy.
≻ There is a tight balance between mitochondria dying off and mitochondria being produced.
≻ It is likely that muscle disuse is an important early trigger of mitophagy, probably as part of a normal physiological adaptation to adjust mitochondrial content to the reduced energy demand associated with lower physical activity levels.
≻ Interventions to combat the loss of muscle oxidative capacity targeted directly at mitophagy signaling should be approached with the highest caution.
≻ A reduction in mitophagy will not only rescue healthy mitochondria but result in an increased number of dysfunctional mitochondria as well, which could aggravate the decrease in muscular health even more (Leermakers & Gosker, 2016).

⚃  Type II diabetes is also associated with muscle wasting.
≻ Despite differences in pathogenesis and disease-related complications, there are reasons to believe that some fundamental underlying mechanisms are inherent to the muscle wasting process, irrespective of the pathology.
≻ Recent evidence shows that inflammation, either local or systemic, contributes to the modulation of muscle mass and/or muscle strength via an altered molecular profile in muscle tissue.
≻ However, it remains ambiguous to which extent and via which mechanisms inflammatory signaling affect muscle mass in disease (Dalle & Koppo, 2020).

⚄  American Geriatrics Society Expert Panel on the Care of Older Adults with Multimorbidity. Guiding principles for the care of older adults with multimorbidity: an approach for clinicians. J Am Geriatr Soc. 2012; 60:E1-E25. https://doi.org/10.1111/j.1532-5415.2012.04188.x

⚄  Dalle, S., & Koppo, K. (2020). Is inflammatory signaling involved in disease-related muscle wasting? Evidence from osteoarthritis, chronic obstructive pulmonary disease and type II diabetes. Experimental Gerontology, 137 (April), 110964. https://doi.org/10.1016/j.exger.2020.110964

⚄  Frisbee, J. C., Lewis, M. T., & Wiseman, R. W. (2019). Skeletal muscle performance in metabolic disease: Microvascular or mitochondrial limitation or both? Microcirculation, 26 (5), e12517. https://doi.org/10.1111/micc.12517

⚄  Greenberg, S. A. (2021). Counting People with Inclusion Body Myositis. Neurology, 10.1212/WNL.0000000000011994. https://doi.org/10.1212/WNL.0000000000011994.

⚄  Greenberg, S. A., Pinkus, J. L., Amato, A. A., Kristensen, T., & Dorfman, D. M. (2016). Association of inclusion body myositis with T cell large granular lymphocytic leukaemia. Brain, 139 (5), 1348-1360. http://doi.org/10.1093/brain/aww024.

⚄  Keshishian, A., Greenberg, S. A., Agashivala, N., Baser, O., & Johnson, K. (2018). Health care costs and comorbidities for patients with inclusion body myositis. Current Medical Research and Opinion, 34 (9), 1679-1685. https://doi.org/10.1080/03007995.2018.1486294

⚄  Leermakers, P. A., & Gosker, H. R. (2016). Skeletal muscle mitophagy in chronic disease. Current Opinion in Clinical Nutrition & Metabolic Care, 19 (6), 427-433. https://doi.org/10.1097/MCO.0000000000000319

⚄  Naddaf, E., Shelly, S., Mandrekar, J., Chamberlain, A. M., Hoffman, E. M., Ernste, F. C., & Liewluck, T. (2021). Survival and associated comorbidities in inclusion body myositis. Rheumatology, keab716. https://doi.org/10.1093/rheumatology/keab716.

⚄  Shelly, S., Mielke, M. M., Mandrekar, J., Milone, M., Ernste, F. C., Naddaf, E., & Liewluck, T. (2021). Epidemiology and Natural History of Inclusion Body Myositis: A 40-Year Population-Based Study. Neurology, 10.1212/WNL.0000000000012004. https://doi.org/10.1212/WNL.0000000000012004.

⚄  Valderas, J. M., Starfi, B., & Sibbald, B. (2009). Defining Comorbidity: Implications for Understanding Health and Health Services. Annals Of Family Medicine, 357-363. https://doi.org/10.1370/afm.983