Day-care management of sickle cell painful crisis in Jamaica: a model applicable ... suitable oral analgesia, adequate education and support, the majority of ...
British Journal of Haematology, 1999, 104, 93–96
Day-care management of sickle cell painful crisis in Jamaica: a model applicable elsewhere? M. A. WA RE , I. H A M B L E TO N , I. O C H AYA AN D G. R. S E R J E A N T MRC Laboratories (Jamaica), University of the West Indies, Kingston, Jamaica Received 20 July 1998; accepted for publication 27 October 1998
Summary. In the U.K. and the U.S.A., painful crises account for 80–90% of sickle-cell-related hospital admissions, with average durations of 5–11 d. In Jamaica, many severe painful crises are managed in a day-care centre. Patients (n¼1160) with homozygous sickle cell (SS) disease aged 18 years and over were registered with the clinic during a 1-year study period. Of these, 216 patients with 476 painful crises attended the day-care facility for a total of 686 d. Most patients (119 or 55·1%) had single crises and for most crises (338 or 71%), patients attended for only 1 d, when they were given bed rest, assurance, rehydration and analgesia. Patients with complicated painful crises were usually referred for admission after initial pain relief and the rest were monitored during the day. In the evening they were given the option of hospital admission or allowed home with oral analgesia. Hospital admission for complicated painful
crises or inadequate pain relief occurred in 42 (8·8%) crises and home management in 434 (91·2%) crises. Of 186 patients initially selecting home management, 20% returned for further day-care and five (2·7%) died during subsequent admission for that painful crisis, one without other known complications, two with acute chest syndrome (one associated with Salmonella septicaemia), another with Salmonella septicaemia, and one with dengue haemorrhagic fever. With suitable oral analgesia, adequate education and support, the majority of severe painful crises in SS disease in Jamaica have been managed on an outpatient basis. This model of patient care may merit assessment in other communities where painful crises are a common clinical problem.
The painful crisis is the most common cause of acute morbidity in sickle cell disease (Baum et al, 1987; Platt et al, 1991). In the U.K., painful crises account for 80–90% of acute hospital admissions in sickle cell patients with a mean duration of 5–10 d (Brozovic & Anionwu, 1984; Brozovic et al, 1987). In the U.S.A., painful crises account for 79–91% of emergency room visits by sickle cell patients and 59–68% of hospitalizations, with average admission durations of 8–11 d (Yang et al, 1997). In Jamaica, the Sickle Cell Unit supervises the management of many patients with sickle cell disease and a median of six patients attend daily with bone pain of sufficient severity to require narcotic analgesia. Protocols for outpatient management in a day-care centre have been developed as an alternative to hospital admission. We present the experience of the day-care model over a 1-year period and suggest that this approach may merit assessment elsewhere.
PATIENTS AND METHODS
Correspondence: Professor Graham Serjeant, MRC Laboratories (Jamaica), University of the West Indies, Kingston 7, Jamaica. q 1999 Blackwell Science Ltd
Keywords: sickle cell, day care, painful crisis.
Patients. The patients attended the Sickle Cell Clinic of the University Hospital, operated by the MRC Laboratories at the University of the West Indies, Kingston, Jamaica. The study was confined to adult patients (18 years or over) with homozygous sickle cell (SS) disease presenting with severe painful crisis between 1 April 1995 and 31 March 1996. None of the patients were on chronic transfusion programmes or hydroxyurea, or had undergone bone marrow transplantation. The diagnosis of SS disease was based on standard criteria (Serjeant, 1992). Severe painful crisis was defined as bone pain of sufficient severity to limit function, require opiate analgesia, and unexplained on any basis other than sickle cell disease. Repeat visits to the day-care centre within 7 d were arbitrarily defined as the same crisis. During the study period, a total of 1160 SS patients aged 18 years and over were enrolled on the clinic register, and 976 of these attended the clinic at least once during the study year. Day-care facility. At the time of the study the day-care centre was a single room with four beds (currently expanded to eight beds) within the Sickle Cell Clinic which operates
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from 8:30 to 16:30, Monday to Friday. The facilities are free and patients are encouraged to attend at any time during these hours. As a result the clinic is the preferred and principle health-care provider for the great majority of sickle cell patients living in Kingston and the surrounding areas. The day-care centre provides oral or intravenous fluids and parenteral pain relief, and is supervised by a full-time nurse and attending physician. The room is kept warm, generally without air-conditioning, blankets are provided, electronic monitoring equipment is kept to a minimum, soft music played, and relatives or friends can attend patients throughout the day. On arrival, patients are seen by the clinic physician and a brief history taken for possible precipitating factors, location and duration of pain, previous analgesia at home, and attendance at other health-care facilities. Physical examination assesses the state of hydration, searches for infection in the upper respiratory tract, urine, osteomyelitis or gallbladder, and other pathology such as acute chest syndrome. Analgesia is based on intramuscular pethidine 50–100 mg, pentazocine 30–60 mg or morphine 5–10 mg, given 2–4-hourly as required. Intramuscular diclofenac sodium 75 mg is commonly added to improve pain relief. Hydration is given orally or intravenously if the patient is dehydrated or vomiting. Patients with underlying pathology were usually referred for admission after initial pain relief, but others were monitored during the day. Prior to closure of the clinic, patients were given the option of hospital referral (if pain control was deemed inadequate) or of returning home with oral analgesia (a 2 d supply of codeine, pethidine, pentazocine or diclofenac) and the reassurance that they could return for further day care if necessary. The private home phone numbers of all Unit doctors are printed on the appointment cards and all are available for consultation.
Table I. Day-care centre attendances for individual painful crises.
Frequency No. of attendances per painful crisis
No.
%
1 2 3 4 5 6 7
338 92 31 8 4 2 1
71·0 19·3 6·5 1·7 0·8 0·4 0·2
Total
476
of day-care attendances for each painful crisis (Table I) showed that 338 (71%) of patients attended for only 1 d, but 15 crises required four or more attendances. Assessment of patients at the end of the day showed that 42/476 (8·8%) painful crises in 32 patients were referred to hospital because of concern about possible underlying pathology or failure of the pain to settle to tolerable levels, whereas in 434 (91·2%) crises, patients chose to return home. Of the 186 patients opting for home management, five deaths (2·7%) were associated with that painful episode (Table II). Two deaths (one from Salmonella septicaemia) had clinical evidence of acute chest syndrome, confirmed at autopsy. Evidence of the acute chest syndrome (dyspnoea, chest pain, clinical evidence of consolidation) occurred in 23 patients, 14 of whom were referred for admission with no mortality and nine were sent home on antibiotics, two (22%) of whom died with pathological evidence of acute chest syndrome.
RESULTS Among the 1160 eligible patients enrolled in the clinic, there were 476 painful crises in 216 adults with SS disease (50% male) accounting for 686 day-care attendances during the study year. Single crises occurred in 119 (55·1%) patients, two crises in 42 (19·4%) patients, three in 21 (9·7%), four in 16 (7·4%), and five or more in 18 (8%) patients. The number
DISCUSSION The rationale for hospital admission of the painful crisis includes concern about the potential severity of the underlying pathology, possible precipitating factors such as infection, and difficulty in rendering adequate pain relief as outpatients. The underlying pathological process appears to
Table II. Deaths in patients discharged from day care.
Age/sex
Clinical features
Relationship to day care
Cause of death
19F
Bone pain, fever
Attended three times in 7 d; referred A&E; died same day
Salmonella septicaemia
19F
Bone pain, fever
Attended 2 d; referred A&E; died 2 d later
Dengue haemorrhagic fever
23F
Bone pain, cough, dyspnoea chest pain
Died 1 d after discharge
Acute chest syndrome; Salmonella septicaemia
35M
Bone pain, dyspnoea, chest pain, abnormal chest X-ray
Attended 2 d; died on third day after discharge
Acute chest syndrome
42M
Bone pain, chest signs
Died 1 d after discharge
No anatomical cause of death
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Day-Care Management of Painful Sickle Crises be avascular necrosis of active bone marrow (Charache & Page, 1967) and the pain is assumed to arise from the increase in intramedullary pressure consequent on the ensuing inflammatory response. Only one patient in the current study died during an apparently uncomplicated painful crisis, and retrospective analysis of 501 deaths in SS disease over the last 35 years revealed that 10 (2%) were associated with uncomplicated painful crises (unpublished observations). This contrasts with experience in the Cooperative Study of Sickle Cell Disease in the U.S.A. (Platt et al, 1994), where 25/209 (12%) of deaths were associated with apparently uncomplicated painful crises. The factors contributing to this difference are unclear but may include more aggressive therapy (Cole et al, 1986). It was a cause of concern that of patients opting for day care, five died, four of whom were subsequently admitted to hospital, but it remains possible that earlier admission might have influenced the outcome. The observation that two of nine patients with evidence of acute chest syndrome sent home on apparently appropriate antibiotic therapy subsequently died implies that this should be a contraindication to home management. Otherwise, our experience suggests that the painful crisis, although causing distress, does not usually warrant admission because of the severity of the underlying pathology. The need for admission is generally based on the perceived inability to provide adequate pain relief as outpatients. Many factors influence the patient’s ability to cope with pain and few objective studies are available but the following statements derive largely from Focus Groups in the U.K. (Streetly et al, 1997). Delays in the provision of analgesia in busy casualty departments contribute to the apprehension and anxiety experienced by patients in severe pain. Rapid and effective analgesia provided by familiar and experienced staff in a specialized unit is likely to allay these tensions. Reassurance that the pathology underlying the painful crisis is not life-threatening, that the pains always resolve, and that the natural history of bone pains is for a progressive improvement and eventual disappearance with advancing age (Serjeant, 1992), are all likely to improve the patient’s ability to cope. A dedicated unit is likely to encourage patients to attend earlier for mild pain when supportive measures may prevent the development of more severe pain. The extended family system in Jamaica whereby most patients have relatives or friends at home also ensures a supportive environment with provision of fluids and other care. It is also important for patients to have appropriate expectations, and ameliorating pain to allow sleep and rest may be achieved by much lower analgesic levels than those necessary for the total abolition of pain. Several protocols support the effectiveness of oral analgesia in the management of severe pain (Friedman et al, 1986; Powers, 1986; Jacobson et al, 1997). With full explanation and discussion of these factors, this study suggests that many Jamaican patients prefer day-care management. It has been argued that the Jamaican form of sickle cell disease is different from that observed in the U.K. and the U.S.A. (Ranney, 1973). Major genetic differences seem unlikely since all three patient populations are of similar West African origin, but there may be important social or q 1999 Blackwell Science Ltd, British Journal of Haematology 104: 93–96
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environmental differences. The importance of skin cooling, which is a major precipitant for bone pain in Jamaica (Redwood et al, 1976; Serjeant et al, 1994), has been questioned in temperate environments (Seeler, 1973; Slovis et al, 1986), yet a clear relationship occurred in other studies (Amjad et al, 1974; Resar & Oski, 1991). It seems likely that the relationship has been obscured by adaptations in microclimate (central heating, warm clothes) rather than being absent. Other potential differences include greater expectations of pain relief, the lack of social support for patients living alone, reluctance to attend emergency rooms where their perceived reception may not be sympathetic, and other attitudes towards health-care providers (Elander & Midence, 1996). It is to be hoped that education of staff and patients about the disease and improving social support for the patient may make home management a preferred and more viable option than prolonged hospital admission. Investigation of the differences in attitudes between U.K. and Jamaican patients and the factors contributing to them is a vital step in finding solutions to the problems and devising more appropriate management for all patients.
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Redwood, A.M., Williams, E.M., Desai, P. & Serjeant, G.R. (1976) Climate and painful crisis of sickle-cell disease in Jamaica. British Medical Journal, i, 66–68. Resar, L.M.S. & Oski, F.A. (1991) Cold water exposure and vasoocclusive crises in sickle cell anemia. Journal of Pediatrics, 118, 407–409. Seeler, R.A. (1973) Non-seasonality of sickle-cell crisis. Lancet, ii, 743. Serjeant, G.R. (1992) Sickle Cell Disease, 2nd edn. Oxford University Press. Serjeant, G.R., De Ceulaer, C., Lethbridge, R., Morris, J.S., Singhal, A. & Thomas, P.W. (1994) The painful crisis of homozygous sickle
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