Grief and coping in Charcot-Marie-Tooth disease
by Patsy Levy, M.S.W.

For many people with Charcot-Marie-Tooth disease, the final diagnosis usually follows a process of a long period of physical symptoms, medial visits and tests, accompanied at the same time by slow deterioration of bodily functioning.

Tossed into a sea of anxious questioning, rocked by waves of uncertainty, people with CMT find themselves floundering in an emotional quagmire leading to grief and depression. Phrases such as progressive disease, chronic illness, functional atrophy and hereditary illness create mental images that play across the mind bringing fear, anger, denial and possible disorientation towards a body that has temporarily become a foreign vessel bearing an inner vision of an altered self-reflection.

Being ill is more than having a disease. It is a physical reality that has been created for oneself instead of by oneself, and is thus accompanied by various psychological facts.

The discovery of CMT causes initial crisis leading to varying degrees of emotional upset. Loss of bodily functioning is, in other words, a loss of self. As such, symbolically it involves the loss of a loved object. Concretely in most cases, this has meant the deterioration of the feet and legs of the person who has CMT, a physical and psychological means of mobility, and the person's hands, a physical and symbolic means of caring for oneself and for others. With the news of no cure and the possibility of further complications depending on the progression and severity of the effects of the disease, persons can find themselves in mourning for what has been taken away from them, for an injured self-image, for changes in life goals and existing relationships with others. Usually effective coping skills may suddenly fail or become exhausted in the struggle to adapt to a changed reality with as yet unknown ramifications.

CMT people can experience shock and perhaps denial in the belief that they are ill with a chronic, progressive disease and that the physical results of the illness are permanent. Anger, disorientation, crying, anxiety attacks accompanied by efforts to explain and rationalize what has befallen the person are often expressions of the internal pain of grief being experienced. Many are confronting for the first time with the fact that their bodies have disease and are facing the issue of their own mortality. Chronic grief is a natural chronic emotional response to chronic illness.

Over and Over Again
Because of this, renewed grief can be triggered by a new progression in the disease, by the painful necessity of possible major life changes due to physical inability, and/or by the regression of relationships with significant others due to the increase of tensions and strains maximized by the illness. Even seemingly simple events such as a doctor's casual or impersonal approach to giving treatment, or the glances or verbal reactions of others to a resulting apparent handicap can produce a kaleidoscopic array of depressive feelings. In fact, living in a society that values physical beauty and functioning, career achievement, and efficient coping skills, personal goals and ambitions are often thrown out of balance by illness, creating a trampoline effect of raised feelings of helplessness, overriding anxiety, anger, and at times inestimable sadness.

Society, too, also has formal rituals for grief involving the loss of a loved one. Public sympathy and support spring forth giving comfort to the bereaved. Tolerance of emotional outbursts are accepted and encouragement is given with the acknowledgement that after a period of adjustment life continues.

This, however, is not exactly the case with chronically ill CMT people. There is no formalized ritual for the loss of body function or other effects of the disease. Mourning also has no recognizable end point in CMT but instead there are periods of inactivity mixed with periods of renewed bouts of depression. Support from others may be limited or sporadic, with possible demonstrations of unawareness and seeming insensitivity to the additional psychological pressures and energy it takes for patients to recruit optimum functioning physically and emotionally. They have perhaps taken for granted the freedom of health with which they can function and make choices regarding their everyday lives and future that the chronically ill do not have in quite the same way when facing the reality of a progressive disease.

In fact, a characteristic of CMT is the uncertainty of when, how or to what degree the illness's internal time clock will slow to a rest or speed up the dysfunction. CMT people as a result often feel frustration in learning how to judge the responses of their bodies, how to manage their activities and how to absorb the emotional pain that comes when their bodies fail them.

The complicating factor of CMT being a genetically hereditary disease may also bring with it a burden of guilt for the person with CMT, for other family members who suffer from the disease and a wrestle of conscience of whether or not to have children by prospective parents with CMT. In addition, often well-meaning outsiders are unable to empathize with these issues in conveying their value orientation towards the subject. In the end, persons with CMT must judge for themselves the possible consequences of dealing with a generally hereditary illness while at the same time attempting to make an educated, constructive decision that will leave them with the least emotional baggage necessary to carry around afterwards.

Dealing with CMT involves placing an emphasis on the quality of one's life rather than experiencing frustration concerning the quantity of one's life – quantity meaning in terms of how many accomplishments and ambitions one can achieve within a certain amount of time. CMT people may discover themselves feeling panic and/or restlessness, planning and making life decisions at a much faster rate than they would have ordinarily.

In attempting to assert control over one's reality, instead of pressure being relieved rather the opposite may occur with patients finding themselves under even more tension than before. Fears regarding deterioration and an inability to meet one's needs and desires can create even higher levels of anxiety. For many, activity itself is a sign of being alive. A progressively ill person's basic need for self-validation and self-esteem, although shaken by emotional anxieties, can be formulated into positive actions through the awareness that one must redefine and seek accomplishable goals that can restore self-image and recompensate for lost functioning. Influential in terms of judging self-worth, the work ethic of the surrounding society is usually strong and is considered to be an emotional basic necessity regarding expectations of ourselves and in meeting the expectations of others in order to gain acceptance at home and a work. However, curiously enough, because these are not simple tasks nor are the issues involved easy ones, it is sometimes better for CMT people to slow down their decision making in order to afford themselves the time and patience necessary to cope as successfully as possible in redefining lifestyles and goals. In other words, sometimes by letting go, people can have more control not less over themselves and their lives.

Of course, the situation varies from individual to individual and is especially affected by the severity of the illness and effectiveness of the treatment being received.

People in general, through trial and error, discover successful and unsuccessful coping modes which best help themselves. It is important to become aware of one's limits emotionally between situations and thinking that proved to be sensitive and painful areas and others that are more productive in terms of an emphasis on what one can do, not on what one can't do. Often such variables as age, sex and social situation are additionally influential. For example, adults may grieve more because of their fear of independence on others leading to a loss of certain activities.

With children and adolescents, it may be the worry of social unacceptance by their peer group or by being different from the other kids. A simple query of "How are you?" followed by the usual response of "I'm fine," may seem like a contradiction of terms to a "well" person who views a CMT patient moving past with obvious mobility problems. These are examples of emotional obstacles which many people with CMT must overcome and be re-evaluated in new terms by CMT people to themselves and by those closest to them. Lack of inner reconciliation to these situations can cause even more active efforts at "okayness" or passive avoidance of the issues altogether by ignoring medical follow-ups or by not making use of supportive therapy opportunities.

How much is in your mind?
An important aspect of chronic illness is to be able to differentiate intellectually between being "ill" on a physical level and being "crippled" on an emotional one. In this instance, the grieving process can be an actual help, providing the means for an eventual psychological healing outcome. As a natural human process, it allows people with CMT time for a natural and legitimate response to being chronically ill. Metaphorically, it can be compared to falling to the bottom of an emotional bucket, being mired in depression, until with great emotional energy recruiting old and new coping skills to climb back out to a reattained equilibrium. Depending on the severity and the permanency of the original triggering conditions, each new slide into a grief reaction and the time it takes for emotional resolution is a time-consuming activity that can last from days to months or longer. During these periods, often illness takes on a more dominant aspect coloring the person's self-image, his relationships to others and in fulfilling everyday tasks.

Often at that point relationships that are already strained between family members may break down entirely with those closest to the ill person. While some families respond by growing stronger and more unified in adversity, for others, facing the possibility of a long continuance of a progressive illness, life within the family sphere may alter so radically that all other roles which the person once had may disappear or be replaced by other members. This can develop into an unhealthy depressive situation whereby the ill person's only role is one of being ill and the family's main task becomes one of accommodation to that fact.

In reality, people bring themselves into CMT. They bring with them a history of prior coping skills and personalities which were either operating in positive or negative ways before the onset of the illness. With many people, it is the illness itself which brings a major emotional and spiritual crisis in functioning. With others, the onset of illness can further complicate a poor self-image, adjustments and relationship problems. However, people also carry with them pre-existent strengths into the experience of illness. Many have already faced other obstacles in their lives, the tools of which may help them to overcome the initial adjustment trauma to CMT. One's innate qualities, although seemingly temporarily immobile from shock, anger and depression, have not disappeared. Instead, they may reappear dependent on the individual's choice and motivation to serve him as a means to grow and develop inwardly to become even stronger.

In general, regarding family relationships, a person's former roles, although perhaps submerged at critical times, also do not vanish with illness. The children of a CMT parent will still look to that parent for guidance as a role model, learning all the while tools for the future when they and their children may be confronted with serious illness.

Coping with illness is often a life lesson that is passed down between generations. Married couples also, where one partner has CMT, will seek for the most part to preserve and re-balance their interacting roles in terms of individual and mutual needs and functioning. This can, however, prove to be a rocky process involving feelings of helplessness and grieving by both parties. This can be a painful and stressful time for both, especially if the effects of the illness are seen as threatening dependency issues between the couple. In extreme cases, the result could be divorce and/or desertion. For another couple, a positive resolution could be increased enrichment, closeness, and understanding of each other and of themselves.

How can I help?
Many people with CMT, while feeling injured and in mourning, have difficulty not only in accepting and loving themselves but through their depression and anger at what has happened to them may not believe that anyone else could possibly love them or care for them either. Furthermore, the "well" partner, may feel overwhelmed, experiencing rejection, guilt, and anger in face of the CMT partner's own self struggle, possible withdrawal and emotional outbursts. It is a normal human chain of reaction for couples to fall into when coping with illness. No one is born knowing automatically how to cope with chronic illness, nor are people raised or taught, for the most part, how to deal with the obstacles that illness causes. And yet, couples often express expectations of each other to know what to do and how to respond. While one or both are feeling unsteady and insecure with the situation, each may be seeking reassurance from the other. In actuality, both partners will need emotional space to absorb and negotiate what has occurred. Communication, patience and mutual support are very important at a time when both partners are most probably experiencing the crisis together and apart. But the CMT partner may be so subjectively involved in dealing with being ill that he or she may have very little energy to be able to deal with others during this period. How to adjust to this possibility often depends on what strengths existed in the marital relationship before the illness, what levels of tolerance exist between the couple, how effective each partner's coping skills are, and to what extent they are able to keep their functional roles intact.

One day at a time
The element of time, of just living from day to day, does play a small part in eventual adaptation to illness. In the simplest terms, even most hospitalized patients become involved in and adjusted to daily hospital routine. This does not preclude CMT persons' grieving, but it is indicative of the small steps that are needed to be taken just to start the coping process. Metaphorically, we can ask the question, "How does one eat an apple? Bite by bite," and so patients deal with illness, day by day.

It is very probable that most people with CMT go through the grieving process alone. Because of the illness's chronic character, lifelong management techniques need to be developed concordant with the severity and frequency of the illness's progression in preparation for recurrent periods of depression. For purposes of emotional survival, CMT people must learn to build psychological "corners" that are effective in diffusing the depression and are time limited in content. It is desirable that people with CMT become aware of when they are being emotionally affected by their illness along with a recognition of the ensuing depression. At that point, self-discipline will need to be employed in order to pick an appropriate time and place for the expression of the grieving to allow for functioning on other levels in the meantime.

A time and place to grieve
This could perhaps be during the night's silence when self-reflection occupies one's thought. If the depression is momentarily overwhelming, a written list of sources of satisfaction and meaningfulness may be an important reminder that the illness is not the only thing there is to being a human being. Getting up and changing one's activity or focus to someone or something else can be a positive means of shifting emotional concentration. Do not underestimate the importance and value of people using and experiencing themselves as instruments for interaction, and therefore, self-validation. It is not an easy task. However, CMT people can make a conscious choice for life by taking steps to balance out the periods of darkness.

On a larger scale, living with CMT also involves a process of re-evaluation of personal goals. Grand ambitions for accomplishment in the near or far future may either suddenly and painfully become irrelevant because of physical disability or be moved up on an advanced timetable. As was mentioned previously, illness often narrows a person's perspective to what can be achieved on a day by day basis. In some patients' cases, the progressive severity of the illness may increasingly emphasize the small moments of one's life; the warmth of interaction of being with a loved one or friend, or the accomplishment of a simple activity within one's home Living for today can have a cumulative effect, yielding moments of psychological rest and fulfilment. As a result, contacts with others should be re-evaluated in terms of their meaningfulness and worthwhileness for each individual and acted on.

How a person with CMT involves himself actively is particularly important when it comes to being a participant in one's own medical treatment. It is essential that people with CMT feel a sense of control and input over what is happening to them. On a factual basis, it is necessary for them to know what their exact condition is presently, including a prognosis for the future. It is the patient's right to know. However, often people must learn to be their own best advocate in getting their needs clarified and their questions answered. Sometimes this may come in conflict with the image of being a "good" patient, but often bureaucratic medical systems, including doctors themselves, become inundated, either unable or unwilling to respond to patients on a human level.

Here are some concrete suggestions:
1. The night before a doctor's visit, the person with CMT and their family should list any relevant questions. Doctors often don't inquire into the concrete requirements of patients' everyday living within the home or on the job. As a result, doctors may be unaware or unable to clarify patients' and families' functional expectations. The written list will not only serve as a tool to structure the communication between patient and doctor but will also enable the patient to have some control, particularly if he is feeling anxious.

2. Don't allow yourself to become lost in medical lingo. Many doctors are so accustomed to speaking and thinking in medical terms that they are either unaware of or ignore the patient's inability to understand what is being said. Request that your doctor give an explanation in language that you can understand or, to make it conceptually easier, ask your doctor to sketch a rough diagram of what he is talking about.

3. Remember to pay attention to the implications of treatment and prognosis, particularly in regards to life decisions that you should perhaps be making now rather than later. Consider the effects it may have on other members of your family or on your work environment.

4. When faced with a medical procedure that is causing you anxiety, ask for details regarding the conditions under which it will be given and be clear in your own mind concerning its purpose. Medical procedures can induce high levels of anxiety leaving patients feeling more like helpless victims than active participants. This will give you a chance to prepare yourself emotionally for what lies ahead including tools that might help you at the time of the test: i.e. relaxation techniques during painful and/or difficult procedures.

5. Be sure to take responsibility for finding out about follow-up appointments. Initiate consultation with your doctor in exploring various modes of possible supportive therapy: i.e. maintenance exercises prescribed through physical therapy for muscles which have not yet showed signs of atrophy and are compensating for muscles that are no longer functioning. Some doctors are unaware concerning the broader picture that may lay outside of their area of specialty. People with CMT should always be concerned with measures that can affect optimum daily functioning including referrals for appropriate intervention.

6. Be assertive. It is a sign of realistic acceptance of who you are and that you are still functioning. This is especially important for CMT people who are bedridden and/or hospitalized. Your interaction with others, including medical personnel, is important for you. Remember that although you may no longer have much control over your physical self you, and only you, hold your emotional and spiritual self in your hands.

7. Finally, if you feel you cannot bear the chronic grieving and its accompanying spells of depression alone seek out others who are willing to help you. Besides friends and family, it may mean checking professional and medical resources including your church or synagogue, self-help groups, and counselling professionals. Having CMT entitles you to being a human being in need of support like everyone else.

In conclusion, people with CMT have a long road to travel. But, although their bodies may slowly fail along the way, with the proper emotional and psychological tools, it is possible for them to and their families to continue to function and survive a full life.

Bibliography
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2. Lane, Helen J. Assoc. Professor. Working with Problems of Assault to Self Image and Lifestyle. Social Work in Health Care Vol.1, No.2, Winter 1975-76, pp. 191-198.
3. Stewart, T., M.D. & Shields, Charles, M.D. Grief in Chronic Illness. Arch Phys Med Rehabilitation, July 1985; 66(7): 447-50.
4. Adams, Raymond D. & Victor, Maurice, Principles of Neurology. McGraw Hill Inc. 1985, p.94, p.884, pp.987-988.
5. Charcot-Marie-Tooth – CMT Newsletter, Vol.4, No.3, June 1987 Canada
CMT Newsletter, Vol.3, No.6, Dec.1986 Canada
CMT Newsletter, Vol.4, No.2, Apr.1987 Canada