Jacqui, Louise, Shay & Brendon

Motor Neurone Disease - Pain and Associated Psychological Perspectives in Terminal Illness

The following presentation will focus on the management of pain and its psychological effects on MND sufferers in the last 6-months (as determined clinically) before death.

Section 1 - Defining Terminal Illness

Terminal illness is not a medical diagnosis, but is a term made popular in the 20 th century to describe a disease that has no known cure and is expected to result in the eventual death of the sufferer. Typically an illness will be classed as terminal if death is expected to occur within 6-months of the diagnosis of the illness. There is therefore no particular ailment that can be classed as terminal, but rather certain diseases may be considered terminal at a particular point in its progression within the sufferer. Once a disease has been diagnosed as terminal, treatment efforts are usually halted and multi-disciplinary palliative care is put in place. This provides pain relief and other appropriate measures to ensure as much comfort and well-being in the end stages of life. Many (if not most?) terminal illnesses result in a degree of associated pain that may be progressive as the patient enters the final stages of the illness. This creates a distinct and special relationship between the patient’s physical symptoms and psychological well-being as the individual attempts to deal with pain whilst knowing that death is inevitable.

Section 2 - Definition of Pain

What is pain? - Pain is an unpleasant sensory and emotional experience associated with actual or potential tissue damage or described in terms of such damage. It implies a monistic view of the experience of pain and is inappropriate to encumber it with concepts of pain behaviour. Physicians recognise the individuals report because pain is subjective and it varies both with the physical state of the individual and his/her state of mind (Merskey, 1991).

Types of pain include:

Acute Pain: Commonly associated with a specific injury that signifies injury or damage has occurred. This pain type can last from seconds to six months (Smeltzer & Brunner, 2010).

Chronic Pain: Constant pain that persists beyond expected healing time and can seldom be attributed to a specific cause or injury. This pain lasts for six months or longer (Marcus, 2009).

Referred: Pain that occurs from deep structures such as joints, muscles, ligaments, tendons, viscera and is difficult to locate as opposed to superficial pain. (Lautenbacher & Fillingim, 2004).

Radiating: Radiating pain “moves” from its point of origin to other parts of the body meaning that tissue injury at one site can produce pain in a region separate and distinct from that involved in the injury (Knight & Draper,2008)

Sensation thresholds appear to be universal in that despite cultural/ethical differences in background, sensation is reported at the same level cross-culturally. However, pain perception may differ across cultures with pain tolerance often notably different depending on cultural background. This indicates that pain is a highly personalised experience within individuals with variations reported across cultures, (Zatzick & Dimsdale, 1990).

Section 3 - Defining Motor Neurone Disease

Motor Neurone Disease (MND), a progressive neurodegenerative disease, is a group of neurobiological disorders that afflict the upper and lower motor neurones, leading to the attrition of muscles. Damage and death of the motor neurones is the underlying cause of the disease. When motor neurons fail to give out signals, muscles cease to respond and the process of muscle wasting begins, (atrophy). The result is a loss of limb movement and difficulties with speech, swallowing and breathing. There is no known cure or cause. The disease was first characterised in 1874 by Jean-Martin Charcot who named the illness Amyotrophic Lateral Sclerosis (ALS), a term which classifies the most common form of the illness and is often used synonymously with MND (McLeod & Clark, 2007). It is relatively rare with approximately 5000 cases in the UK at any one time (Moore, McDermott & Shaw, 2008).

Only 5% of known cases of MND have been traced back to family history, with the other 95% being sporadic, meaning that no genetic evidence exists for the onset of the disease. Length of life from first diagnosis is typically 2 – 5 years; however there are many well known deviations from this. Stephen Hawking, perhaps the most well-known case, has lived with the disease for over 40-years. The most important risk factor for MND is age with the peak incidence between 50 – 70 years of age. Men are more likely to develop MND than women with a ratio of 3:2, (Ng, Khan & Mathers, 2009).

The disease is associated with varying degrees of pain in sufferers, and as with any terminal illness, may impact heavily on the psychological well-being of patients.

Section 4 - Pain associated with MND - Physical and Psychological

Non-motor disturbances (NMDs) affect most patients with MND. These include a range of difficulties (e.g. fatigue, sleep disorders, constipation) with pain, anxiety and depression featuring prominently. In one survey, palliative care staff members ranked pain as the number one physical problem in patients with a variety of terminal illnesses. Anxiety and depression were ranked first and second in the list of psychological difficulties, (Stiel, et al. 2011).

Physical

Physical pain in MND can be subdivided into a number of specific pain areas. These pain categories include muscle cramps, spasticity, constipation, spasms, skin pressure and musculo-skeletal pain. Each of these categories presents different challenges for the sufferers of MND and their carers. Common issues and treatments are shown in the diagram below:

Management of Physical Pain in MND

According to the Motor Neurone Disease Association, the only available treatment for MND is Riluzole (Rilutek). In a trial by Miller, Mitchell, Lyon & Moore (2007), Riluzole taken 100mg daily, was

found to be a reasonably safe form of treatment and almost certainly prolongs median survival of an MND/ALS patient by approximately 2 – 3 months. There are a number of specific drug trials that are currently underway, however much of the previous clinical trials that have been carried out have proved to be largely disappointing.

Pharmacological management of pain in MND can incorporate the use of medication that is non-steroidal anti-inflammatory (NSAID), especially if an active inflammatory process like arthritis or tenosynovitis is evident. Taking prescribed amounts of acetaminophen, about 1000mg every 6 hours, along with NSAID or by itself if NSAIDs are intolerable may help. For pain alleviation, tricyclic antidepressants and anti-epileptic drugs such as Neurontin or Gabapentin can be useful. Neurontin may also help with relief of spasms (DeLisa, Gans & Walsh, 2005). Some reports have suggested however, that Gabapentin can be ineffective in the control of pain and may also produce side effects such as memory and attention impairments along with diminished cognitive ability. Earlier research suggested that Gabapentin was well tolerated in most patients with possible side effects being mainly transient and occurring during the titration phase, (Serpell, 2002). However, more recent research reported that the adverse effects of gabapentin were more prevalent, suggesting that adverse effects may be dose dependent with motor/ambulatory effects recorded even in doses too low to produce significant therapeutic effects on pain, (30 mg/kg). Doses of gabapentin needed to produce significant therapeutic effects (100 mg/kg) also significantly increased deficits in cognitive functions. Although it was noted that adverse side effects may subside after about ten days in some patients, for others the adverse reactions to the medication became greater with repeated dosing, (Lindner, Bourin, Chen, McElroy, Leet, Hogan, Stock & Machet, 2006). The side effects often noted in current medications result in much trial-and-error treatment of pain which in turn may lead to negative thinking and depression.

Another method of managing pain associated with MND is through the use of Marijuana (Cannabis). It has been noted to aid in muscle relaxation, stimulation of appetite, bronchodilation, sleep induction, saliva reduction and analgesia. Several cannabinoids have been shown to have strong antioxidative and neuroprotective effects that lengthen the endurance of the neuronal cell. Special care must be taken by physicians with regards to medicinal usage of marijuana for MND patients, and they must also have an understanding and follow local and state laws concerning its use (Delisa et. al., 2005).

Physical disability in patients leads to activity limitations, this can be characterised by utilising physical performance measures. One such measure is the Physical Functioning Scale (PFS) of the 36 item short form health survey (SF-36) (Bohannon & DePasquale, 2010).

The physical aspects of MND frequently receive the majority of attention with psychosocial aspects afforded secondary important (McLeod & Clarke, 2007).

Psychological

Physicians and others involved in the care of patients with MND need to be aware that depression associated with pain is a significant problem irrespective of the level of physical disability (Tedman, Young & Williams, 1997).

In terminal illness depression may be a reaction to pain and other symptoms of the illness. However it may also be a comorbidity of the condition. Distinguishing between a reactive feeling of sadness and a treatable depressive illness remains a dilemma (Lloyd-Williams & Riddleston, 2002). This issue causes difficulties with diagnosing depression in terminal illness and as a result, under-diagnoses and under-treatment have been noted, (Stiel, et al. 2011).

Further issues of cultural restraint and relative levels of care also make psychological pain more difficult to determine than physical pain, and therefore treat. The diagram below shows the impacts of individual differences on levels of psychological pain and associated treatments:

Measurement of Depression associated with pain

Beck Depression Index BDI) – a widely used scale that measures the severity of depression – a short form of the BDI (BDI-SF) consists of 13 items – has been used in a study of terminally ill patients.

Self-Rating Depression Scale – 20 item self-administered instrument – used in a study which looked at depression in chronic medical diseases

Management of Psychological Pain in MND

Section 5 - Comorbidity Factors (Desire for Death & Suicidal Thoughts)

Hope and hopelessness are important issues for MND patients, with hopelessness contributing significantly to suffering and for some a desire for hastened death (McLeod & Clarke, 2007).

Both physical pain and feelings of hopelessness have been noted as predictors of patient interest in assisted suicide Ganzini, Silveira & Johnston, 2002). To assess the severity of ALS at various stages of progression, the ALS Functional Rating Scale (ALSFRS-R) is available. This assessment covers four domains: bulbar functions, fine motor skills, gross motor skills and respiratory status. In assessing symptoms of depression, patients may be administered the Patient Health Questionnaire (PHQ) which assesses depressive disorders based on criteria in the DSM – IV, (Albert, Whitaker. Rabkin, del Bene, Tider, O’Sullivan & Mitsumoto, 2009).There are few options for MND patients with regard to treatment and they eventually have to deal with decisions to either accept or forgo life-sustaining therapies. The concerns of patients and caregivers bring about fears of pain and suffocation that may manifest as both helplessness and hopelessness. It should be noted however, that feelings of hopelessness may be influenced by religious beliefs and/or cultural factors. For example, if the patient believes in life after death, feelings of hopelessness may be diminished.

With increasing levels of physical pain and comorbidity with psychological factors in dealing with a terminal illness, the risk of suicidal thoughts is a concern.

The desire for escape from intolerable pain is considered to be one of the most frequently reported incentives for suicidal behaviour. For a number of patients, pain may become so severe that it is intolerable and patients consider that their future will be ‘an eternity of suffering, ‘pill after pill after pill’. It has been suggested that the feelings of defeat and entrapment play a vital role in the decision that patients make when the yearning to escape the pain results in the consideration of suicide (Tang and Crane, 2006).

Suicide vulnerability factors (Ganzini, Silveira & Johnston, 2002)

- Fear of losing autonomy- Loss of autonomy- Fear of losing independence- Loss of independence- Perception of being a burden on their families- Depression with a feeling of hopelessness as a consequence of the clinical conditions - Hopelessness in dealing with pain when death is inevitable

Suicide Facts (Maytal and Stern, 2006)

- Suicide act is committed in only a minority of terminally ill patients- Statistically significant association between clinical depression and the desire for hastened

death- 59% of patients who expressed a desire for hastened death had clinical depression- 8% of patients without a desire for hastened death were depressed- Terminally ill patients with a history of depression had vulnerability for a desire for hastened

death even if they did not have active symptoms of mood disorder- Statistically significant association between a history of depression and a desire for a

hastened death

Section 6 - Conclusion

Summary

Motor Neurone disease is the terminal illness reported within this presentation and can be defined as a progressive neurodegenerative disease that results in the loss of limb movement and leads to difficulties when speaking, swallowing and breathing. The statistics of MND have been noted revealing that 95% of cases have no genetic evidence. MND has no known cure with statistics of survival for more than 10 years following diagnosis being extremely uncommon – with exception! Stephen Hawking is a theoretical physicist and cosmologist who was diagnosed with MND when he was 21 and is chronically disabled, but still alive at the age of 69.

Initial reactions on discovering one’s fate can result in significant emotional trauma. The diagnosis of a terminal illness affects not only the patient, who may express a number of different emotions, including disbelief, anger and grief but also those who are close to them, who may feel helplessness, a sense of inadequacy, sorrow and anger that they are going to be parted.

The presentation has also outlined the definition of pain and given consideration to the types of pain that are commonly associated with, not only specific injuries, but also pain that can be experienced whilst enduring the chronic effects of the terminal illness identified within this presentation. Chronic pain can be long lasting and indeed present often through the final stages of terminal illness and can be particularly associated with degenerative conditions, such as the degeneration of the motor neurones. Chronic pain in terminal illness can result in an increased focus on emotions such as sadness and thoughts associated with the pain. There is an increasing recognition of the cognitive and affective dimensions of pain.

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The measurement and management of the physical pain associated with MND has also been considered. The physical symptoms of MND often begin very gradually, with clumsy fingers and a weak grip often being the first symptoms of muscular problems associated with MND. As the disease progresses so do the physical disabilities. The presentation reported the use of Physical performance measures such as the SF36, a short form health survey which can help identify where input is required. A multi-disciplinary approach is usually adopted, with physiotherapy and dietary input as well as pharmacological intervention to target the management of pain.

The measurement and management of the psychological pain associated with MND was also discussed and reported depression associated with pain as being a significant problem of MND. Measurement of depression via the Beck Depression Index (BDI) for severity of depression and the Self Rating Depression Scale used for depression in chronic medical diseases has been able to highlight the requirement of not only a range of pharmacological interventions for depression but also a range of psychological methods of coping with pain.

The presentation then focussed on the co-morbidity factors associated with pain in MND and discussed the possibility of a desire for death and suicidal thoughts as a last resort to be freed from the pointless pain. There is a strong link between physical pain and psychological well-being in terminally ill patients. Coping with pain, whilst knowing that death is inevitable, is often associated with powerful feelings of depression and hopelessness. When an illness is not terminal, fighting the pain, coping and managing, may actually bring about positive feelings of gaining control and putting up a fight, however, for those with a terminal illness it may seem that there is nothing to fight for. This hopelessness and despair may lead to ideas of suicide and the desire for hastened death.

Evaluation & Suggestions

Managing pain in terminally ill patients requires a multi-disciplinary approach. Pharmacological intervention by medical staff is needed but, just as important, are the psychologists, counsellors and caregivers who try to relieve the psychological distress and provide support for the emotional and psychological symptoms associated with terminal illness. It is important to remember that the management of pain in terminal illness is not restricted to physical pain but also includes psychological pain. This comorbidity is the key factor on why it is considered “pain in special circumstances”

Although previous research has failed to understand the causes of MND, current research of stem cells to develop new motor neurons and the identification of faulty genes in the development of MND are underway. A referral to a neurologist is the common pathway for patients diagnosed with MND, future consideration could be given to the pain associated with MND at an earlier stage ensuring that not only pharmacological input for management of pain is prescribed but early intervention of the psychological management of pain should be made available to ensure that patients have engagement in controlling their management of such a debilitating terminal illness.

Final Thoughts

Coping with and managing pain can obviously be regarded as challenging in any situation. However, the feelings of depression and hopelessness associated with terminal illness create a unique circumstance under which individuals need to cope with and manage pain without the positive effects of hope and whilst battling with related negative emotions.

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