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Pain

Pain has a physical, psychologic, spiritual and cultural component. Individuals with mitochondrial disease report pain most commonly in the form of headaches, myalgias and neuropathy, and abdominal or chest pain. Cutaneous pain is also reported in association with Raynaud-like changes, especially in the distal extremities. Impaired function in the central nervous system may also cause pain that may be manifested in a number of different ways including painful myoclonus and spasms; impairment in the spino-thalamo-cortical system appears to be a major cause of central pain (Tanaka, 1997). Chronic pain in mitochondrial disease should be well managed as pain is a significant stressor and has the potential to lead to further decline and lactic acidosis (Ross, 2007).

Abdominal pain may come from any number of causes including gastroesophageal reflux, gastric distention with delayed gastric emptying, intestinal and/or colonic dysmotility associated with bloating and/or constipation. Urinary retention with bladder distention can also be a cause of discomfort.

Younger children may have difficulty describing abnormal sensations accurately (like numbness or tingling) and may substitute more familiar terms instead like pain instead (inaccurately).

Even when the etiology of the pain can be determined and appropriate treatment implemented, the discomfort might not be entirely treatable. The focus should be on minimizing triggering factors (such as anxiety or depression) and developing strategies for managing the pain (e.g., coping mechanisms for dealing with chronic discomfort).

Assessment

  1. Newly onset, acute or change in chronic pain should be evaluated.
  2. A useful acronym for evaluating pain N, O, P, Q, R, S, T (Hallenbeck, 2003):

N - Number of pain locations (1 or more locations)?

O - Origin of pain?

P - What palliates and what potentiates the pain?

Q - Quality of pain?

R - Does the pain radiate?

S - Severity and suffering related to pain (on a scale). The patient's pain threshold may be higher if the pain has been prolonged or chronic.

T - Timing and trend of pain?

  1. Headache - see HEADACHE.
  2. Abdominal pain - see ABDOMINAL PAIN.
    1. GI - see VOMITING and CONSTIPATION.
    2. Bladder - see DIFFICULTY VOIDING OR FREQUENCY.
  3. Chest pain -
    1. Heart - see ISSUES AFFECTING THE HEART
    2. Muscle - see myalgias, below.
  4. Myalgias - see LIMB PAIN AND SENSORY ABNORMALITIES
  5. Neuropathy - see LIMB PAIN AND SENSORY ABNORMALITIES
  6. Skin - see AUTONOMIC DYSREGULATION

Recommendations

  1. Non-narcotics should always be considered first; due to the frequent chronicity of the pain, there is a real potential for dependence. Acetylsalicylic acid and ibuprofen should be avoided in those patients with bleeding or bruising problems. Acetaminophen should be used with caution in those patients with active liver disease.
  2. Narcotics have been used safely but should be used with caution particularly with those individuals with trunk muscle weakness and/or with significant gut dysmotility. .
  3. Baclofen has been used successfully for muscle spasms (Smail, 2005).
  4. Regional blocks may be helpful to control pain and to avoid the use of opiates that may worsen pulmonary insufficiency and increase the risk of acidosis (Celebi, 2006; Ross, 2007).
  5. When more common pain interventions have already been tried, consider:
    1. Referral to a neurologist.
    2. Refer to rehabilitation and/or pain management specialist.
    3. Referral to a holistic or naturopathic clinician, or for acupuncture and other non-traditional medicine approaches.
  6. Consider palliative care as appropriate.

References

Celebi N, Sahin A, Canbay O, et al. Abdominal pain related to mitochondrial neurogastrointestinal encephalomyopathy syndrome may benefit from splanchnic nerve blockade. Paediatr Anaesth 2006;16(10):1073-6.

Hallenback JL. Palliative care perspectives. Oxford University Press 2003. 

Ross AK. Muscular dystrophy versus mitochondrial myopathy: The dilemma of the undiagnosed hypotonic child. Pediatr Anesth 2007;17:1-6.

Ben Smail D, Jacq C, Denys P, Bussel B. Intrathecal baclofen in the treatment of painful, disabling spasms in Friedreich's ataxia. Movement Dis 2005;20(6):758-9.

Tanaka S, Osari S, Ozawa M, et al. Recurrent pain attacks in a 3-year old patient with myoclonus epilepsy associated with ragged-red fibers (MERRF): A single-photon emission computed tomographic (SPECT) and electrophysiological study. Brain Dev 1997;19(3):205-8.

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