(Lesson 4- Table of Contents) (Next) (Glossary)

Immediately after the death of an infant or the diagnosis of a serious genetic disorder is not the time for detailed and extensive discussions because neither parents nor patients are likely to be receptive or comprehending. To know precisely when and how much information to impart, or what referral to make during the grieving period requires some skill and an understanding of the grief process.

Grief is a normal and natural reaction to any type of loss. For the parents of a child with a congenital anomaly or genetic disorder, they may grieve the loss of the dreams they had envisioned for the child. For individuals diagnosed with an adult onset genetic disorder, they may grieve the loss of their health and vitality, or the loss of future dreams.

Grief is an adaptive process that has several components. Harrington defines grief "not as a state or set of symptoms, but a succession of clinical pictures which blend into and replace each other"5. Several researchers, including Elisabeth Kubler-Ross, George L. Engel, John Bowlby and C. Murray Parkes, have described the stages of grief in various ways6,7,8,9.

The four dimensions of the mourning process outlined by Bowlby and Parkes are
(1) shock and numbness, (2) yearning and searching, (3) disorientation and disorganization, and (4) resolution and reorganization8. The four dimensions do not follow a set order and a person may experience feelings from several stages at one time.

Shock and numbness is characterized by stunned feelings, impaired judgment and functioning, and only short periods of concentration. Shock and numbness are the elements of the grief process that are readily visible to society. These are the protective mechanisms people use to help themselves cope with a loss at their own pace. At this stage in the process people may continue to "function" but they are not really able to hear or to feel. Unless establishing a diagnosis or pursuing a test is crucial to the care of your patient at this point in time, a referral for a genetic consultation should be deferred.

The yearning and searching stage of grief is marked by feelings of restlessness, anger, guilt and ambiguity. During the yearning and searching phase, people may withdraw and want to be left alone. In this stage they also tend to ask a lot of questions about the how and why of their situation. Parents may request definite answers about their child's diagnosis, and at the same time point out the normal appearing features they see in an effort to dispute the diagnosis. Parents and other family members may experience intense anger towards caregivers, their partner, themselves and God. Feelings of anger towards a partner or his/her family are common if the condition is inherited. When a child has a congenital birth defect or genetic disorder, feelings of guilt, of "having failed," or of "having caused the problem" are also magnified. These feelings may intensify the disorganization a person feels when going through this stage of the grief process.

The disorientation and disorganization phase of grief is associated with feelings of depression, guilt and unfamiliarity. This is the time when the defect or illness becomes a "reality." At this point, physical and functional problems become more pronounced, and people may neglect their physical and nutritional needs. If the patient or their parents are treated with tranquilizers or sedatives during this stage, they tend to further distort reality and the grief process is slowed down.

The last phase of the grief process is reorganization and resolution. During this phase there is increased energy, increased decision-making abilities and an increased sense of self-confidence. This is a time of acknowledgment, a time of bringing reality into "focus." Although no one ever gets over the loss of the "perfect baby" or the loss of dreams, a person gets through the process.

The intensity of these stages of grief change over time. Research conducted by Davidson demonstrated that the intensity of feelings within each stage rises and falls throughout the first two years following the loss of a loved one10. Important anniversary dates such as the child's birth, mother's day, father's day, Christmas and other family gatherings, can trigger the grief response and accounts for some of these changes. It is also clear that the stages of grief overlap.

According to Moses, "the feelings experienced during the grief process serve a specific function and can be viewed as protective mechanisms for the person experiencing the loss"11. Denial, for instance, is a defense mechanism people use to block the conscious recognition of specific information. Denial allows a patient or parents to "take in" what they are capable of handling mentally, emotionally and physically.

Lubinsky suggests that denial is often confused with disbelief, deferral and dismissal3. People experience disbelief when they cannot make sense of what they are being told. For instance, parents may experience disbelief when they are told their physically normal appearing newborn baby will be mentally retarded. Parents may not appreciate the subtle physical characteristics associated with some genetic conditions or syndromes that allow physicians to make a diagnosis in the newborn period. Recognition of their child's problems may come slowly for these parents. Their disbelief may change to acceptance only when they begin to see that their child is failing to achieve the normal developmental milestones. "Insisting on acceptance can be counterproductive . . . often the family sees this as 'rubbing their noses' in their misfortune. . . . They need time to say good-bye, to let go of one world and move to another"3.

Deferral is another reaction that may be confused with denial. With deferral the parent accepts the clinical findings but seems to ignore the implications. A coping mechanism associated with deferral is to postpone taking action (e.g., not keeping appointments to see specialists for the child's care). Parents may need assistance in prioritizing basic needs for themselves and the child and in mobilizing the available resources. "Deferral centers on practical issues (therefore) interventions must be perceived as useful"3.

Dismissal displaces the focus of the situation from the diagnosis to the "legitimacy of the purveyors"3. Dismissal is often accompanied by anger at health care professionals, frustration and a sense of betrayal. In such cases the patient or parents need space to explore their feelings and to voice opinions without being judged.

THE PROLONGED GRIEF RESPONSE

Although the writings of Kubler-Ross, Engel, and Bowlby and Parkes address the grief process as reaching a resolution or end point, the parents of a child with a congenital anomaly or a person with an adult onset genetic condition (e.g., Huntington disease) do not always know when the end will occur. Simon Olshansky6 first described the prolonged grief response that accompanies the rearing of a mentally retarded child as chronic sorrow. The term "chronic sorrow" has traditionally been used to describe the recurrence of the feelings associated with the grieving process by parents who have children with special needs. This model was expanded by Joan Blaska, who coined the term "cyclical grieving"12 to describe the cyclical nature of the recurrence of the emotions associated with the grieving process. As opposed to always feeling sad, which the term "chronic sorrow" leads us to conclude, families typically move in and out of the "grief loop." Cyclical grieving may occur frequently, or it may hardly occur at all. Generally, when these feelings of grief return, they are shorter in duration and less intense than the initial grief reactions.

Various life events may trigger the grieving experience to reoccur. Critical periods generally fall into two categories: markers in normal development and the identification of a child as "disabled." Examples of the developmental markers include times when peers begin to walk, talk, reach puberty, learn to drive and graduate from high school. Events in which a child is identified as disabled include when a younger sib surpasses him/her developmentally, discussion of placement, unusual behavior or health, discussion of guardianship and addressing long-term care needs. Keep in mind that different events trigger grief reactions for different people. Additionally, parents may not be grieving over the same thing or at the same time. Where one spouse is upbeat, the other may feel downtrodden.

Another model of the prolonged grief process that accompanies the birth of a child with special needs is the pinwheel model of bereavement13. The pinwheel model of bereavement suggests that, with time, an individual reaches out to others, change occurs and life is rejoined after a loss. But, at any time, the circular experience of the loss may be revisited. A person's past history of experiences with crisis and grief is an important determinant of how he/she will proceed through the grief process.

When a person experiences an acute loss, there are several physical symptoms which may occur. These symptoms include sleeplessness, weakness and fatigue, feeling "choked up," a sense of unreality, sighing respirations, loss of appetite and/or gastrointestinal disturbances. Patients or parents need to be told that any or all of these symptoms are a normal occurrence and, unless the insomnia starts to interfere with daily life activities, tranquilizers and sleeping pills should be avoided.

REACTIONS OF OTHER FAMILY MEMBERS

The reactions of sibs to the birth of a child with a birth defect will vary according to their age, developmental stage, and "knowledge" of the family rules. Since children may not be able to verbalize their feelings, they may begin acting out in ways that are out of character. Keep in mind, too, that children who have a congenital anomaly or genetic disorder will frequently strive to be like other children their own age; however, their disorder may make it impossible for them to achieve age appropriate developmental tasks.

When working with sibs, or the children of a patient diagnosed with a genetic disorder, it is of utmost importance to be honest and forthright. They will sense the change in their home environment, their routines and the interactions of the people in their lives. Give them the basic facts in terms and at a level they can understand. Children do not need all of the details, but a simple explanation of what is occurring may alleviate a lot of misconceptions and anxiety.

Since children, especially young ones, may find it difficult to verbalize their feelings, professionals need to anticipate their needs and provide anticipatory guidance to their care providers. Anticipatory guidance should include information about the developmental stages of childhood. Parents should be instructed to talk to their children about what they might be feeling so the children can connect their feelings with words.

Grandparents can be particularly challenged after the birth of a child with a genetic disorder. Not only do they grieve for their grandchild, they also feel the hurt of their son or daughter who is going through these difficult experiences. Sensitivity to these feelings must be incorporated into interactions with the family, not just at the initial time of diagnosis, but throughout the child's life.

(Lesson 4- Table of Contents) (Next) (Glossary)