Caring for Children with Chronic Conditions and Their Families

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chapter 20 Caring for Children with Chronic Conditions and Their Families

Richard B. Goldbloom

The secret of the care of the patient is in caring for the patient.

FRANCIS PEABODY

Providing the best possible care for a child with a chronic illness or disability calls for special skills if you are to be of maximum assistance to the child and family. While writing this chapter, I realized that those best equipped to speak with authority on this topic are the parents of children with chronic conditions and sometimes the youngsters themselves. Their years of firsthand experience, often dealing with multiple caregivers in and out of the hospital, have left them with vivid memories of the clinical encounters that served them well and those that left them unhappy or dissatisfied. I have quoted the following people directly whenever it seemed appropriate:

• Andrea is now an intelligent, articulate young woman, who has become a highly successful cattle breeder. She spent much of her childhood in hospital with severe, disabling arthritis, chronic uveitis, and, more recently, a rare, poorly understood muscle disorder. Both Andrea and her mother Ann have strong views about the clinical skills that make for effective, sensitive care.

• Carol and John Young: Years ago, Carol Young was a dedicated volunteer at a pediatric hospital. She began to care for Michael, an abandoned, desperately ill 3-month-old boy. Michael was blind from birth and had severe, chronic chloride-losing diarrhea. Carol and her husband John adopted Michael when he was 15 months old. Michael suffered from recurrent bouts of severe dehydration and was hospitalized repeatedly. Eventually his kidneys failed, and he received two successive renal transplants, both of which were rejected. Michael was kept alive with hemodialysis and long-term peritoneal dialysis, the latter carried out mainly at home by Carol and John, for 3 years. Despite superb care from his parents, supported by several specialists, Michael died when he was 11 years old.

• Donna and Ian Thompson: The Thompsons have raised two children, Robbie and Jane, both of whom were affected by cystic fibrosis. Both died as young adults. The Thompsons accumulated more than two decades of experience with health professionals and the health care system. They have been outspoken, effective advocates, nationally and internationally, to improve the care of children and adults with cystic fibrosis.

First Disclosure of the Diagnosis to Parents

The basic competencies required for initial disclosure of the diagnosis of a chronic condition are essentially the same, whether the child happens to have cystic fibrosis, a congenital anomaly, cerebral palsy, Down syndrome, diabetes, or any other serious chronic condition. Physicians and students vary considerably in their level of comfort when faced with the task of disclosing to parents a serious diagnosis and its implications. Some cannot wait to get the interview over with, therefore without realizing it, talk too fast, allowing little time for interruptions or questions. Others betray their personal discomfort by talking around the real issues. From a parent’s perspective, however, nothing makes the interview more unsatisfying and unhelpful than haste or circumlocution.

Carol Young: “Not every doctor has the comfort to sit down and talk to parents…but honesty up front is the most important thing.”

Donna Thompson (recalling how she and Ian first learned that their child had cystic fibrosis): “He [the doctor] had an intuitive sense of how much we could absorb at one time, so he had a paced way of delivering the information.”

It is virtually impossible for a clinician to disclose, and for parents to absorb, the full impact and implications of a diagnosis of a serious, chronic condition in their child. A good listener should sense when parents have had as much information as they can handle at an individual session. Usually, several sessions are needed for them to achieve full comprehension and a level of acceptance.

Several careful investigations have identified the specific factors that determine the parents’ level of satisfaction or dissatisfaction when they recall how their child’s diagnosis was first disclosed to them. Their observations provide a very clear picture of “do’s” and “don’ts.” The key components that determined whether parents remembered that conversation with appreciation or with rancor were:

2. Honesty without abruptness

3. Emphasizing the child’s positive qualities

Promptness

Some parents have complained that “Everyone knew the diagnosis before we did.” Even when the news is bad, parents want to be told as soon as possible. When you cannot immediately establish a definitive diagnosis, parents usually accept the situation if you give them justifiable reasons for the delay. They simply want to know as soon as possible whether you have any concerns about their child, even if you are not yet sure of the exact nature of the condition.

Honesty without abruptness

Parents want the truth, but everyone needs time to absorb it and to come to terms with disturbing news. Parents who were dissatisfied with the manner in which their child’s diagnosis was disclosed to them have often pinpointed the abruptness with which the information was delivered or the apparent lack of compassion on the part of the messenger.

Emphasizing the positive

It is a lot easier for a parent to absorb and accept distressing news about a child when it is presented in a context that not only includes but also emphasizes the child’s positive attributes. The physician who opens the conversation “cold” with the bad news or focuses overwhelmingly on the negative or difficult aspects of the child’s condition may inadvertently seem, in the eyes of the parents, to devalue the child. Such a message is guaranteed to induce anger, depression, or both.

Variations in individual styles of delivering bad news may reflect personality differences among caregivers. Each of us has our own unique personal endowments of optimism and pessimism, and the balance between the two may condition both our style of communicating bad news and the parents’ reactions to hearing it. To illustrate this point, I sometimes ask medical students to indicate through role-play how they would deal (in actual language) with the following not-so-hypothetical situation:

“You have just carried out your initial examination of a newborn boy and discover that he has several major congenital anomalies. You consult an authoritative reference book on dysmorphic syndromes and learn that this particular condition carries a 5% risk of recurrence in subsequent children. What exactly do you say to the parents when they ask you about the recurrence risk?”

Some students reply that they would “tell them the truth,” meaning that they would inform the parents that there was a 5% risk that the condition would reappear in a subsequent child. By contrast, others say they would begin by telling the parents that there is a 95% chance that any subsequent child would be completely normal, only then indicating the 5% recurrence risk. Mathematically, both approaches are equally accurate, but the impacts of the two styles of communication on the parents can be diametrically opposite.

By the same token, when starting an interview for the initial disclosure of a child’s chronic condition, be it Down syndrome, a dysmorphic syndrome, or any other chronic condition, always open the conversation with comments that highlight the child’s positive attributes. Also, you should always, but always, refer to the child by name, never as he, she, or the baby. For example, you could start by saying, “I’ve examined Mary Ann. She is a lovely girl and her general health appears to be good. But she does have a problem I’m concerned about.” At this point, as you reveal the specific reason for your concern, the conversation should immediately become a two-way interchange, during which you pause regularly to encourage the parents to ask questions. Never allow your disclosure to turn into a speech.

Always tell parents any bad news about their child’s health against a background of the child’s positive attributes.

Conducting the first interview

On the general principles outlined earlier, the following essential ingredients will make the first disclosure of a child’s chronic condition the least traumatic and as helpful as possible for the parents:

1. Both parents should be present. If only one parent can be present, a close relative or valued friend can provide important comfort and support. Other health care personnel should be present only if they have a specific role to play in the child’s care.

2. Everyone should be seated. Do not sit behind a desk if you can avoid it.

3. The child should be present as well, unless she or he is too ill.

4. The setting should ensure comfort and privacy (turn off your pager!).

5. Open the conversation with a positive statement about the child, and balance the positive and negative elements of the child’s present and future condition throughout the discussion. (For goodness sake, do not ask the parents whether they have noticed anything odd about their child!)

6. Avoid medical jargon. Encourage the parents to stop you if you use any words they do not understand. When parents nod assent, do not assume that they have understood you. Toward the end of the interview, always ask them to describe what they understand about the child’s condition and the plan for investigating and managing it. Doing so gives you a clear picture of how well you have explained the issues and offers a chance to correct any misunderstandings.

7. Always take the initiative in encouraging and facilitating consultation with experts, either for confirmation of the diagnosis or for help with management. Parents appreciate this approach enormously.

8. Whenever possible, offer the parents correct and easily understood printed information or reliable Internet sources concerning the child’s condition. It often helps to put them in contact with other parents or support groups who are experienced in dealing effectively with children who have the same condition.

9. Try to sense, from both verbal and nonverbal cues, when the parents have had enough for one session. All parents have limits of tolerance for how much bad news or complex information they can absorb at one sitting. Often, full comprehension and acceptance requires several interviews.

10. Always conclude the interview by establishing a time and place for an early follow-up meeting with the parents—within 24 hours for a serious condition, but certainly within a few days. You could also take the initiative to telephone them later in the day to see whether they have further questions. Most parents do think of additional questions they want to ask; encourage them to keep a written list of concerns to bring to your next meeting.

11. Finally, let them have some private time together at the conclusion of the interview.

Parental dissatisfaction with the initial disclosure interview is not inevitable. Nor is some initial anger and resentment toward the messenger who delivers the bad news. On the contrary, when you carry out the interview according to these principles, most parents remember the experience with appreciation, despite its upsetting content. Above all, you must help them feel that they have a crucial role to play in ensuring their child the best possible future.

Not surprisingly, parents’ initial reactions to disclosure of a child’s diagnosis sometimes resemble parental responses to bereavement, with mixtures of shock, disbelief, denial, and anger. Many parents search for causes or try to assign blame, often to themselves (see discussion of hidden agendas in Chapter 1). As with most clinical encounters, it is as important for you to understand the parents who are receiving the information as it is to be an authority on the child’s disease.

Special Skills for Continuing Care

Encouraging shared labor

Chapter 1 discussed the key role of sharing labor among family members as a determinant of emotional strength and resilience in families. Sharing the chores of everyday life is doubly important when the family includes a child with special needs. You can offer valuable guidance in ensuring that both parents, and often older siblings as well, share the responsibilities for the ordinary tasks of daily living and for the extra care that the child requires.

As often as possible, involve both parents when you discuss the child, and encourage them to share the responsibilities for the tasks of daily care. Shared labor is a key contributor to every family’s emotional strength and stability, but especially when the family includes a child with special needs.

Too often, health professionals unwittingly focus their attention on one parent only—more often the mother—thereby contributing to parental exhaustion and possibly increasing family tensions. Involving both parents as therapeutic partners best serves the child and family together.

John Young: “Often, doctors talk to mothers rather than to fathers. But both parents need to be partners with the doctor when there are decisions to be made.”

It is important to assess the parents’ level of satisfaction with the quality of their child’s care at regular intervals. The level of parental satisfaction is often a reflection of the quality of the child’s care.

Respecting parents’ knowledge and experience

Health professionals can learn a great deal about disease processes and principles of treatment from a combination of study and clinical experience. But no amount of either can confer the special, intimate knowledge that parents acquire about their own unique child. No one knows that child better than the parents.

Carol Young: “Most parents of chronically ill children learn so much about how to care for their child, how to give medications, and how to carry out complicated treatments, like home peritoneal dialysis and tracheostomy care, or how to help their child get through uncomfortable procedures. Sometimes they’re better at it than the professionals.”

Ian Thompson: “You can’t help but become pretty well informed—and you learn that there are times when the doctors are wrong and the parents are right.”

Recognizing emotional overload, exhaustion, and the need for respite

Parents rarely complain of “burnout” or ask for respite time, even when they have become totally exhausted by the daily demands of caring for a chronically ill or disabled child. Perhaps some believe that a confession of exhaustion would be interpreted as an admission of failure to fulfill their responsibilities. It is therefore essential that all health professionals involved in the child’s care are sensitive to verbal and, especially, nonverbal signals that parents may be nearing “the end of their rope.” Even the healthiest parents with the healthiest offspring need regular respite from each other and from their children. That need is magnified when the family includes a youngster who requires considerable additional care. Therefore, find out what parents do as a couple for regular diversion and recreation, and how often. Determining the family’s external support systems (family members and close friends) is another key element in evaluating parental access to respite in practical terms.

Ian Thompson: “Some parents simply won’t leave their children. But all parents need periodic respite, right from Day 1. Those breaks are good for the children as well as the parents. On a day-to-day basis, parent respite includes simple things, like trying to avoid having to wake children during the night to give medications.”

Evaluating Quality of Life in Children with Chronic Conditions

Because survival rates for many chronic conditions have improved dramatically over recent decades, many caregivers have turned their attention to evaluating and improving the quality of life (often abbreviated as QOL) in such children. In this context, quality of life refers to the area of the child’s or adolescent’s functioning directly affected by the disorder or by its treatment.

In developing the clinical skills necessary for assessing QOL in a child with a chronic disorder, you must consider four specific domains:

• The disease, its signs and symptoms

• Functional status, which consists of cognitive function, self-help, mobility, physical activity, leisure activities, and academic performance

• Psychosocial adjustment, including behavioral problems, anxiety, and depression

• Social adaptation, such as peer relationships and interactions with family members, teachers, and health professionals

In addition, it may be important to evaluate the child’s overall satisfaction with life and, for an adolescent, the level of satisfaction with personal appearance.

Periodic, systematic assessment of the specific elements that determine the quality of a child’s life can be a valuable way to determine overall adjustment and to identify the unmet needs of a child with a chronic, disabling condition.

Three generic parent rating scales have been widely used to develop an overall view of a child’s general health and adjustment. They include the following:

• The Child Health Questionnaire (CHQ): A 50-item questionnaire that assesses 14 health domains in children age 5 years or older. It has established validity and reliability. http://www.healthact.com/chq.html

• The Child Behavior Checklist (CBCL): A parent rating scale that is valid and reliable for children ages 4 to 18 years. It includes a social competence scale and a behavioral problem scale. http://www.aseba.org/products/cbcl6-18.html

• Children’s Depression Inventory (CDI): A well-validated instrument that has been widely used to assess depression in children with chronic illness. http://www.mentalhealthpromotion.net/?i=promenpol.en.toolkit.326

Various questionnaire instruments have been developed to evaluate QOL in children or adolescents with specific chronic disease states, such as asthma, epilepsy, cystic fibrosis, diabetes, and malignant diseases. These instruments can provide clinically useful information, either as research tools to evaluate changes in management or as ways to follow the progress of individual children over time. Examples of such instruments:

• Pediatric Oncology Quality of Life Scales (POQOLS): Consists of separate scales for physical functioning, emotional distress, and treatment-related adjustment. http://www.medscape.com/medline/abstract/ 19437363

• Diabetic Quality of Life Instrument: Designed for adolescents. http://www.proqolid.org/instruments/diabetes_quality_of_life_measure_dqol

• Cystic Fibrosis Problem Checklist: http://www. marchofdimes.com/professionals/14332_1213.asp

• Asthma Problem Behavior Checklist: http://www.aseba.org/products/cbcl6-18.html

• Child Epilepsy Questionnaire: http://www.special- kidsspecialhelp.org/assets/pdf/szparentquestionnaire.pdf

Even if you do not use such instruments in the routine care of children with chronic conditions, you may find it valuable to become familiar with the questions they ask. Awareness of the principal domains that you must evaluate to develop a clear picture of the quality of a child’s life provides a knowledge base that will help you be of maximum benefit to the child and family. It is not enough to rely simply on common sense and intuition. The best form of intuition is the kind based on a thorough familiarity with existing knowledge.

Physical Examination of a Child with a Chronic Condition

Andrea Crowe: “When a doctor who comes to examine me is someone I don’t already know, I like him (or her) to come in, sit down, tell me his name and just talk to me, even if it’s only for a minute or two. When he starts to examine me, I want him to respect my personal space. And when he’s finished, he shouldn’t just get up and leave. He should tell me what he’s found and not simply write it down in the chart. Sometimes I wonder exactly what they’re writing about me!”

Andrea also believes that the doctors who come to see her should read her chart beforehand. “Sometimes it’s as if they haven’t read it at all,” she reports.

Remember that children have big ears and long antennae, and that these receptors are especially well developed in youngsters who have spent significant time in the hospital. The unfortunate modern practice of conducting ward rounds largely in hospital corridors, poring over medical records, and discussing patients in hushed tones, is a practice guaranteed to generate anxiety in the children and parents who observe but cannot listen to or participate in those discussions.

Carol Young: “Chronically ill children grow up very quickly in some ways. They have big ears, and when they hear you talking, they don’t always know if you’re talking about them, or about someone else.”

The detailed systematic physical examination of various systems is described in other chapters. One issue, however, deserves comment. In children with chronic conditions, it is often advisable to reorder the sequence of the examination so as to leave for the last examination of the systems most affected by the child’s condition and the procedures that may cause the child discomfort. Explain to older children what you are doing at every step. Tell them what you have found, and comment on normal findings and areas of improvement with approval. They usually greatly appreciate this approach.

Transitional Care

Too often, transferring responsibility for the care of an adolescent with a long-standing chronic condition from pediatric to adult specialists and facilities can cause the patient and the parents a lot of anxiety and discomfort. Such problems should be avoidable to a large extent. For many families, years of close association with and dependency on trusted pediatric health professionals and facilities have built a strong sense of confidence. Not surprisingly, many families feel anxious and destabilized by the prospect of change.

Many pediatric health centers have adopted a flexible policy about age limits for adolescents with chronic conditions. Ideally, the decision to transfer care to professionals who deal primarily with adult patients should be made jointly by the patient, the parents, and the caregivers. Once the decision is made, there should be as much overlap as possible between the two sets of caregivers, through mechanisms such as the following:

1. Having physicians and other professionals see the adolescent and family together

2. Cross-over visits between institutions to ensure that the transition will be as comfortable as possible for the adolescent or young adult and the family

Some health professionals who deal exclusively with adults take an approach that suggests a belief that people who have reached the age of majority should stand on their own feet. Irrespective of the patient’s age, however, chronic disabling illness makes all patients more dependent on others, most often on family members. Although self-sufficiency is a virtue that should be encouraged, the need for family-centered care does not disappear on a particular birthday. Effective transition from pediatric to adult care calls for special skills and sensitivity and for extremely close collaboration among all people concerned over a considerable time.

Donna Thompson: “It takes time to adapt to a new doctor, and when they have different views about treatment, it tends to shake your confidence.”

Effective transitional care requires that the new caregivers maintain regular contact with the parents. The transition to adult care should be made as gradually as possible, rather than as a sudden break.

Summary

Children with different chronic conditions and their families experience many common stresses, despite differences in the disease process, degree of incapacity, and prognosis.

From the moment that the diagnosis of a child’s chronic, disabling condition is first disclosed to the parents, you need special clinical skills to help the family come to terms with the child’s condition and to set the stage for optimum management and support. The specifics of what works best for sensitive, effective first disclosure of the child’s diagnosis are well established. Caregivers must appreciate that many parents of children with chronic, disabling conditions need regular periods of respite, especially in situations that put extra-heavy demands on them for the child’s care. Acquiring the skills to evaluate the quality of the child’s life can improve overall care and help you assess progress. All health professionals should cultivate a healthy respect for the knowledge, experience, and technical skills acquired by parents of chronically ill or disabled children. The physician’s role can then change, as it should, from that of the traditional, authoritarian prescriber of treatment to the far more valuable role of therapeutic partner.

Suggested readings

Baird G., McConachie H., Scrutton D. Parents’ perception of disclosure of the diagnosis of cerebral palsy. Arch Dis Child. 2000;83:475-480.

Janse A.J., Sinnema G., Uiterwaal C.S.P.M., et al. Quality of life in chronic illness: Children, parents, and paediatricians have different, but stable perceptions. Arch Dis Child. 2008;97:1118-1124.

Steinbeck K.S., Brodie L., Towns S.J. Transition in chronic illness: who is going where? J Paediatr Child Health. 2008;44:478-482.

Suris J.C., Michaud P.A., Akre C., et al. Health risk behaviors in adolescents with chronic conditions. Pediatrics. 2008;122:e1113-e1118.

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