Grand Budapest Hotel: Inclusion Body Myositis

INCLUSIOTOSIS(IBM):

IBM is a rare inflammatory muscle disease that slowly progresses weakness and eventually the wasting away of both distal and proximal muscles. Muscles in the legs and arms are most obvious areas of the wasting away of muscle tissue. The disease slowly progresses and is diagnosed as a chronic, degenerative neuromuscular disease in which inflammatory cells invade muscle tissue causing progressive muscle weakness and wasting. IBM differs from other types of Myositis in that there is currently no known effective treatment for the disease. (B)

IBM is known to cause slow, progressive asymmetrical weakness and atrophy of the muscles of the wrists and fingers, muscles in the front of the thigh, and muscles that lift the front of the foot. Patients who have the disease can gradually lose the ability to walk, experience falls and injuries, lose hand function, and have swallowing difficulties. Some patients also experience muscle pain. The first muscles affected in Inclusion Body Myositis are usually those of the wrists and fingers, and the muscles at the front of the thigh. The muscles that lift the front of the foot also may be affected.Muscle weakness is often asymmetrical, more prominent on one side than the other. (B)

 TYPES OF IBMs:

There are two forms of IBMs- inflammatory and non-inflammatory. Inflammatory IBM is the most common form of IBM called 'Sporadic Inclusion Body Myositis' (sIBM). It is classified as an Idiopathic Inflammatory Myopathy, an inflammatory muscle disease with no known cause. It is also considered an Acquired Myopathy. This means that it is not caused by genetic inheritance.

It is the most common inflammatory muscle disease in adults older than 50 and is found in men more often than in women. Symptom onset occurs gradually, typically over a period of months or years, most often after the age of 50. It is not uncommon for people with IBM to become wheelchair bound within 10 to 15 years of disease onset. The cause of inflammatory myopathies is unclear. For some reason, in the case of sIBM, the body’s immune system turns against itself, referred to as an autoimmune response, and damages muscle tissue. The cause of the progressive muscle degeneration that occurs in IBM is unclear as well. (C)

Inflammatory cells invading muscle tissues is one characteristic of IBM, but the disease is distinct from other inflammatory myopathies in that muscle degeneration also occurs. Inclusion Body Myositis is named for the clumps of discarded cellular material — the “bodies” — that collect in the muscle tissues. These are apparent on the pathology reports for a muscle biopsy.

Inherited Inclusion Body Myositis (hIBM) is a disease which is genetically inherited, often appearing in other family members, and frequently starts with muscle weakness in early adulthood, occasionally as early as the teen years. This form of the disease is not categorized as an Acquired Myopathy or an Idiopathic Inflammatory Myopathy. The early symptoms include index finger weakness, difficulty walking, tripping and loss of balance. The disease typically progresses to the hands, shoulders, and neck. In this form of the disease, organs are rarely affected, nor are the muscles in the face and eyes, those used for breathing, digestion, and the heart. (C)

Each case of IBM may be quite different from others.  There have been cases where people originally diagnosed with Polymyositis (PM) but were not responding to standard treatments later found they actually had IBM or a non-inflammatory Myopathy such as a form of Muscular Dystrophy.the lack of attention given to neurological conditions and mental health, but it is important to reiterate the scale of the challenge cancer poses to health and social care.

Cancer remains the leading cause of death in the UK, and 2.5 million people are currently living with a cancer diagnosis; this is due to rise to 4 million by 2030. More people than ever before survive in the long term, but often in poor health, requiring extensive support for the rest of their life.ut it would be a mistake to address any long-term condition in isolation.

It is often not as clearcut as a person solely living with cancer or another serious illness. We estimate that currently 1.8 million people with cancer are living with at least one other long-term condition such as dementia or heart disease. Mental health is another important example of this; depression and anxiety often go hand in hand with serious illnesses including cancer.

STUDIES:

Participating in research studies is essential to discovering new, more effective tests and treatments for different conditions. It is very important to have knowledge of rare diseases like myositis and its forms. ( dermatomyositis, Inclusion-Body Myositis, Juvenile Myositis, Polymyositis). The different types of studies conducted on myositis are the following.

BYM338:

Novartis is enlisting sites to test this drug (which has been given breakthrough status) for inclusion-body myositis. Recruitment has begun in Houston, Texas and Phoenix, Arizona. Other sites selected but not recruiting as yet are in Orange, California; Miami, Florida; Kansas City, Kansas; Baltimore, Maryland; Boston, Massachusetts; Columbus, Ohio; Portland, Oregon; and Dallas, Texas. In addition, there are five sites in Italy and one in Denmark. 

The NIH Twin-Sib Study:

 risk factors and mechanisms for the development of systemic autoimmune diseases in adults and children. This study evaluates same-gender siblings within four years of age where one has been diagnosed within the last four years with rheumatoid arthritis, polyarticular juvenile idiopathic arthritis, lupus, systemic sclerosis or polymyositis/dermatomyositis/inclusion body myositis, and the other has no autoimmune disease.

The MYORISK Study: 

to determine if those with myositis, and particularly those with anti-synthetase syndrome (myositis with frequent interstitial lung disease and arthritis), have experienced different environmental exposures before disease onset than other polymyositis/dermatomyositis patients and healthy controls. Adult or juvenile polymyositis/dermatomyositis patients diagnosed within the last two years are eligible for enrollment.

The Myositis in Military Personnel Study:

compares those who developed any form of myositis during U.S. active duty service, with matched persons who did not develop an autoimmune disease during active duty to assess factors that might have led to the development of their myositis.

*These studies involve a single visit to the NIH Clinical Center in Bethesda, MD, the NIEHS Clinical Research Unit in Research Triangle Park, NC, a collaborating center, or a local physician's office to complete patient/parent and physician questionnaires and a blood draw. MYORISK patients will also collect a home dust sample. Patients may undergo a more thorough clinical evaluation if they enroll at the NIH Clinical Center. Compensation for enrollment will be $100 for each patient.

RESEARCH:

Similar to Parkinson’s disease, Inclusion Body Myositis (rare condition where the muscles waste away), affecting mainly older people, though there are other forms of myositis that are found in younger people. There is currently no treatment, and cuts to social services and the health service mean access to support such as physiotherapy, occupational therapy and speech therapy is spread very thin. (A)

Awareness of myositis among professionals is also very low, so patients have to become noisy advocates working through charities, in this case the Muscular Dystrophy Association, or individually. The cost to the health service through emergency hospital admissions, bed-blocking and severe disability is high, and access to specialist services is minimal.(A)

Increasing the profile of neurological conditions should be a much higher priority, not least because I have been told that the incidence of diagnosed cases is rising, perhaps because some people are being “cured” of higher-profile conditions such as cancer. It can be tiring having to tell every professional you meet the name of your illness and then explain what it is. (A)

Links:
(A)http://www.theguardian.com/society/2016/may/09/living-with-an-illness-thats-barely-understood
(B)https://understandingmyositis.org/types-of-myositis/inclusion-body-myositis/?gclid=CIP_-Nzt18wCFVZahgodd30BbQ
(C) http://www.myositis.org/explore-research/clinical-trials

*Please note! These are not my images! They were found on various tumblr sites! Please let me know if any are yours so that I can give you credit for them. Thanks so much & enjoy~
screenshots by:  https://film-grab.com/2014/07/10/the-grand-budapest-hotel/