Shapiro, B. S., Dinges, D. F., Orne, E. C., Ohene-Frempong, K., & Orne, M. T. Recording of crisis pain in sickle cell disease. In D. C. Tyler & E. J. Krane (Eds.), Pain in children (Advances in pain research and therapy, Vol. 15). New York: Raven Press, 1990. Pp.313-321.
*Multidisciplinary Pain Management Program, Division of General Pediatrics and Department of Anesthesiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104; †Unit for Experimental Psychiatry, The Institute of Pennsylvania Hospital, and University of Pennsylvania School of Medicine; and **Comprehensive Sickle Cell Center, Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104
Sickle cell disease results from an inherited defect of hemoglobin structure. It is the most common serious genetic disorder found in blacks (1). In the United States alone, about 1500 babies with sickle cell disease are born each year (2). The disease produces a variety of acute and chronic problems, as early as six months of age, and lasts throughout adult life (1,3). There is no known cure for this illness; rather, therapy consists of close monitoring and supportive medical and psychosocial care (3).
The most common cause of morbidity in sickle cell disease is vaso-occlusive crisis (VOC), which consists of intermittent, usually unpredictable, and repetitive attacks of mildly to intensely acute pain. The pain may be anywhere in the body, may be localized or generalized, lasts from minutes to weeks, and is followed by a return to the patient's baseline status (1,3,4). Due to poorly understood variability, that is at least partially genetic, about 20% of patients with sickle cell disease experience about 70% of all episodes of VOC pain. Some of these patients may have more than 40 VOC episodes in a year (1,5,6). Thus, a subset of patients who have an increased propensity to repeated and frequent episodes of VOC pain can be identified.
VOC pain is a major cause of ongoing morbidity among people with this chronic illness. In the United States and in Great Britain, VOC pain is the most common reason for emergency room and inpatient admission of patients with sickle cell disease (7,8). Additionally, the pain often results in absence from school or work and interferes with other important activities of daily living. Although there has been considerable investigation of the physiologic and molecular aspects of the disease, to our knowledge there have only been two published studies in which the incidence of VOC pain, not limited to
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those episodes for which hospital care was sought, was assessed in a prospective fashion (6,9). There have been no published studies of the extent to which the pain interferes with school and work attendance.
Patients with this rather unique chronic pain syndrome, which is characterized by unpredictable, intermittent, and recurrent episodes of what can be intense pain, may experience significant depression, psychosocial dysfunction, and inability to pursue an independent and productive lifestyle (10-15). It is notable that the unpredictability and intensity of the pain are reminiscent of the learned helplessness model in animal behavior (16). Further study of the natural history of VOC pain is needed in order to understand the manner and extent to which the pain affects the daily lives and functioning of the patients.
ASSESSMENT OF VOC PAIN
The treatment of VOC is essentially the same now as it was 20 years ago; it consists of hydration, rest, peripherally acting analgesics, opioid analgesics, and, in rare cases, transfusion (1,3,4,17,18). The use of a multidisciplinary program utilizing accepted pharmacologic principles of pain control, close outpatient follow-up, cognitive-behavioral interventions, and environmental support, has been shown to be efficacious (5). Controlled studies of various interventions are necessary in order to decrease the dysfunction associated with this problem.
The tailoring of appropriate interventions for VOC pain, and the conducting of controlled studies of such interventions, rests upon the ability to accurately record outcome measurements, such as amount of crisis pain experienced and extent of functional disability. However, as yet there has been no system developed for the accurate home recording of VOC pain and its impact on daily functioning. In-patient and emergency room admission are frequently used as outcome criteria; as such, these criteria represent behavior that may be only partially related to VOC pain intensity, frequency, and impact on the activities of daily living. Although it is typical for patients to be asked at clinic visits the number of VOC episodes experienced since the last clinic visit, the memory for pain and medication usage has been shown to be poor, especially for periods of time greater than 1 to 2 weeks (19-21). Further, the memory report may be affected by the current state of the patient (22). Thus, retrospective pain reports are far from ideal as outcome criteria for clinical studies or as data for clinical decisions. Clearly a system is needed that permits an assessment of pain and function temporally close to its occurrence in the patient's everyday life. Development of such a system was the goal of this study of VOC pain in children.
We report here the successful use of a home diary system for self-reporting of VOC pain presence and intensity, as well as activities of daily living (e.g.,
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school attendance, going outside, and sleep) in a group of children and adolescents with sickle cell disease and a history of frequent VOC pain.
METHODS
Patients
Fifteen children participated in the study. They were patients at the Sickle Care Center of The Children's Hospital of Philadelphia and had been diagnosed with a sickle hemoglobinopathy (Hemoglobin SS or SC). The patients ranged in age from 6 to 16 years (mean = 11.3 years). Each had a history of more than three episodes of VOC over the preceding year. The Committee for the Protection of Human Subjects of The Children's Hospital of Philadelphia approved the study. Before entry, informed consent was obtained from the parents and assent was obtained from the patients.
All data recorded here were gathered during the baseline period of an ongoing study of the efficacy of self-hypnosis versus education in modifying the experience and effect of VOC pain on the activities of daily living. The study involved a longitudinal modified two-period cross-over design, where baseline data were collected over a 4-month period, and then subjects were randomly assigned to a treatment or control condition.
The Diary
We developed a daily pain diary for use by children and adolescents with sickle cell disease and frequent VOC, based on a system devised for sleep and circadian rhythm research (23). Each 5-by-7-inch diary covers 1 week, and consists of two pages for each day; one page is to be completed shortly after awakening in the morning (i.e., the A.M. page), and one before bed at night (i.e., the P.M. page). On the morning (A.M.) page the patient is asked whether he or she has crisis pain now. If the child circles "yes," he or she then rates the pain intensity on a 10-cm (100-mm) horizontal visual analog scale (VAS) (24,25) anchored by "none" and "the worst." The child is also asked whether he or she slept well ("yes" or "no") and rates the length of sleep on a 4-cm VAS anchored by "not much" and "a lot."
On the night (P.M.) page the child is again asked about the presence and intensity of VOC pain for that day; about the presence and intensity of "other pain" (noncrisis pain), using the same format; about the number of "Tylenol" and "codeine" pills taken during the preceding 24 hr; and whether the child went outside that day and/or attended school that day.
The diaries are printed in an attractive format on heavyweight yellow and blue paper. A cover page is personalized with the child's name, the dates of the diary week, and a statement that this is a confidential record. A back page
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thanks the patient for completing the diary and contains a reminder to put the diary in the mail. The pages are bound together with a black plastic spiral so that they lie flat for ease of completion.
Procedures
After enrollment in the study, the patients were instructed in completing the daily diaries. For children under the age of 10, parents were asked to participate in helping the child remember these activities. Over this age, parents were asked to regard the activities as the child's responsibility. Additionally, it was stressed to the parents and the patients that since one of the many problems faced by patients with sickle hemoglobinopathies and frequent VOC is interference with attendance at school and work, all appointments would be made at a time not conflicting with these activities. It was also emphasized that participation in the study involved a responsibility to fill out and return the daily diaries.
To foster completion of the diary, we applied special procedures for mailback and follow-up that we had successfully used in long-term autorhythmetric studies of mood (23). Each week the patient received by mail the diary for the following week, along with a preaddressed, stamped envelope for return when the diary was completed. Patients were instructed to mail the diary at the end of each weekly cycle. Additionally, weekly phone calls were made to the patients and/or parents. These calls were used to give encouragement and immediate feedback regarding pain and activities reported in the diary from the preceding week.
RESULTS
We evaluated a grand total of 1,144 expected diary days from 15 patients. For patients who continued in the study for the baseline data accrual, the length of time for data accrual ranged from 3 months (for patients enrolling later) to 6 months. Two of the 15 patients dropped out of the study after 6 and 10 weeks, saying that they did not want to complete the diaries. Interestingly, these two children had the highest incidence of days with VOC pain (63% and 76%). Table 1 summarizes the extent to which patients complied with the
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protocol and completed the twice-daily diaries. Of 1,144 diary days tabulated, 1,031 days were completed, giving a compliance of 90%.
As shown in Table 2, the children recorded VOC pain in the diaries. On average, 1 in 4 diary days involved VOC pain, although, as expected, there was considerable variability among patients in the incidence of days with VOC (ranging from 6% to 76%). Of the 247 days with VOC, 20% involved inpatient hospitalization. The ratings for VOC pain were much higher than those for "other" pain on VOC pain days. On the 784 VOC-pain-free days, each subject averaged 8.2 days with "other" pain ratings exceeding 0. The mean "other" pain ratings on these days was 36 (SD = 24.1), which is comparable to the mean VOC morning and night ratings on VOC pain days. (One patient had severe aseptic necrosis of the hip.)
VOC pain greatly affected school attendance and time spent outdoors, as is evident in Table 3. Patients did not attend school on 48% of days with VOC pain (SD = 26%) and did not go outside on 42% of the days with VOC pain (SD = 38%). Sixty-two percent of days of school missed because of VOC pain were spent at home and not in the hospital. School absenteeism involved almost 1 in 5 days on days without VOC pain. This high base rate of absenteeism could not be accounted for by the occurrence of "other" pain on those days, as only 28% of the VOC pain-free days involved "other" pain ratings greater than 0.
Similar analyses were conducted on the quality ("good" versus "bad") and quantity (length) of sleep on nights prior to and following VOC pain days, relative to nights following pain-free days. (Sleep data for nights after pain-
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free days are normal and do not differ from that for nights prior to pain-free days.) Table 4 displays these data.
Sleep was much more likely to be disturbed on nights before (36%) and after (42%) VOC days relative to pain-free days. This trend was also reflected in sleep length.
DISCUSSION
The daily diary system appears to be a viable method for gathering data about pain in children with sickle cell disease. Since a large number of VOC episodes are managed at home and appear to affect activities such as school attendance, investigations considering inpatient data will study only one part of the total problem of VOC pain. A system for gathering data that more closely reflects the actual functional impact of the pain is of major importance in gaining a better understanding of this poorly defined pain problem.
Within clinical settings, the number of emergency room and inpatient hospitalizations is often used as an indication of disease severity and to determine the need of medical and/or psychosocial intervention. However, a population of patients whose activities of daily living and development are severely affected by repeated VOC, but who do not come to the hospital for treatment of the pain, may not be targeted for intervention.
Clinical decisions are sometimes based on the memory of the pain. An example would be the decision to place a patient with intermittent priapism, who manages most pain episodes at home, on transfusion therapy. Since, as
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discussed before, the memory of pain incidence and severity may not be accurate, a diary system such as this could be used in gathering data on a short-term individualized basis, in order to make more rational management decisions.
The diary data suggest that VOC pain has a significant impact on school attendance. However, since the time is not missed all at once, the patients may not be eligible for home tutoring during their absences. Many patients in this study reported substantial difficulty arranging for teachers and guidance counselors to gather make-up work. This may result in school difficulties and/or failure, which can only negatively affect the appropriate psychosocial development of these children, and may decrease the child's ability to cope with future pain episodes. This issue of school absence may need to be addressed in public policy decisions (26).
In this context, the amount of school missed on days without pain is of concern. Patients with sickle cell disease may miss school for a variety of reasons. These include infections, clinic visits, and other disease-related problems. Additionally, the child who is behind in schoolwork because of VOCrelated absences may be reluctant to return to school, or parents may be pulled by the child's illness and pain into an overprotective role. The reason for these unexplained absences needs to be clarified.
Children consistently reported in the diary that VOC pain adversely affected sleep quality and quantity. This disturbance is consistent with what is known about the effect of other organically based pain on sleep (27). It is especially disturbing because the quality of sleep very much affects the quality of awake functioning (28). Thus, the sleep disturbance secondary to the pain may itself render activities of daily living, including school, even more difficult for these children. It remains to be determined what role the sleep disturbance plays in further exacerbating the pain experienced of the child.
The diary system we developed and tested appears to yield a surprisingly high rate of compliance. This is within a population of patients whose pain has been poorly studied. The 90% compliance achieved during this baseline period cannot be attributed solely to any one factor, however, because a combination of methods was used in the overall system to enhance compliance. Others have also discussed the importance of some of these aspects (29-31). These include the utilization of an easy-to-use, readily accessible, and attractive diary; personalization of the diary and mailing sytem; ongoing feedback concerning what was reported in the diary; social reinforcement and encouragement; and an established physician-patient relationship, including regular weekly telephone follow-up.
Studies are underway to establish the validity of the diary system. This work includes cross-referencing subjective reports of school absence with school records and comparing parent and patient reports of pain and school absence on the weekly phone calls with the diary reports and medical records. If the
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system is shown to be valid, then it may be possible to space phone calls at longer intervals, using them only to enhance compliance when needed. This would decrease the cost and time required to gather outcome criteria.
The impact of keeping diaries on the skills of coping with pain also needs to be examined. As well as being a promising instrument for the assessment of chronic recurrent pain, the pain diary, at least in the abstract, offers potential advantages in helping the patient better manage his or her pain. When reviewed with the patient and family at the time of an acute pain episode, the diary may reinforce that life overall is not as bad as it may seem in the present. It makes the patient and the parents partners with the medical team and gives them an active rather than a passive role in managing the disease. Last, the mere act of objectively assessing one's pain and other parameters within a communicative framework may make less necessary and/or attractive the acting out of pain via behavior. Although this approach has been recommended by other authors in the assessment and treatment of other chronic and recurrent pain syndromes, it has never been critically examined as far as its effect on coping skills (25,32-34).
Overall, given the prevalence of sickle cell disease and the disabling nature of VOC pain, it is important to assess the efficacy of various treatments intended to help relieve the pain of children and adults with this disease. To this end, the diary appears to he a promising and viable technique.
ACKNOWLEDGMENT
This research was supported in part by grant MH19156 and grant MH44193 from the National Institute of Mental Health and in part by a grant from the Institute for Experimental Psychiatry Research Foundation.
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