Day-care management of sickle cell painful crisis in Jamaica:a model applicable elsewhere?

M. A. WARE, I. HAMBLETO N, I. OCHAYA AND G. R. SERJEANT

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 accountfor 80–90% of sickle-cell-related hospital admissions, withaverage durations of 5–11 d. In Jamaica, many severepainful crises are managed in a day-care centre. Patients(n¼1160) with homozygous sickle cell (SS) disease aged 18years and over were registered with the clinic during a 1-yearstudy period. Of these, 216 patients with 476 painful crisesattended the day-care facility for a total of 686 d. Mostpatients (119 or 55·1%) had single crises and for most crises(338 or 71%), patients attended for only 1 d, when they weregiven bed rest, assurance, rehydration and analgesia.Patients with complicated painful crises were usuallyreferred for admission after initial pain relief and the restwere monitored during the day. In the evening they weregiven the option of hospital admission or allowed home withoral analgesia. Hospital admission for complicated painful

crises or inadequate pain relief occurred in 42 (8·8%) crisesand home management in 434 (91·2%) crises. Of 186patients initially selecting home management, 20% returnedfor further day-care and five (2·7%) died during subsequentadmission for that painful crisis, one without other knowncomplications, two with acute chest syndrome (one asso-ciated with Salmonella septicaemia), another with Salmonellasepticaemia, and one with dengue haemorrhagic fever. Withsuitable oral analgesia, adequate education and support, themajority of severe painful crises in SS disease in Jamaica havebeen managed on an outpatient basis. This model of patientcare may merit assessment in other communities wherepainful crises are a common clinical problem.

Keywords: sickle cell, day care, painful crisis.

The painful crisis is the most common cause of acutemorbidity in sickle cell disease (Baum et al, 1987; Platt et al,1991). In the U.K., painful crises account for 80–90% ofacute hospital admissions in sickle cell patients with a meanduration of 5–10 d (Brozovic & Anionwu, 1984; Brozovicet 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 of8–11 d (Yang et al, 1997).

In Jamaica, the Sickle Cell Unit supervises the manage-ment of many patients with sickle cell disease and a medianof six patients attend daily with bone pain of sufficientseverity to require narcotic analgesia. Protocols for out-patient management in a day-care centre have beendeveloped as an alternative to hospital admission. Wepresent the experience of the day-care model over a 1-yearperiod and suggest that this approach may merit assessmentelsewhere.

PATIENTS AND METHODS

Patients. The patients attended the Sickle Cell Clinic of theUniversity Hospital, operated by the MRC Laboratories at theUniversity of the West Indies, Kingston, Jamaica. The studywas confined to adult patients (18 years or over) withhomozygous sickle cell (SS) disease presenting with severepainful crisis between 1 April 1995 and 31 March 1996.None of the patients were on chronic transfusion pro-grammes or hydroxyurea, or had undergone bone marrowtransplantation. The diagnosis of SS disease was based onstandard criteria (Serjeant, 1992). Severe painful crisis wasdefined as bone pain of sufficient severity to limit function,require opiate analgesia, and unexplained on any basis otherthan sickle cell disease. Repeat visits to the day-care centrewithin 7 d were arbitrarily defined as the same crisis. Duringthe study period, a total of 1160 SS patients aged 18 yearsand over were enrolled on the clinic register, and 976 ofthese attended the clinic at least once during the study year.

Day-care facility. At the time of the study the day-carecentre was a single room with four beds (currently expandedto eight beds) within the Sickle Cell Clinic which operates

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Correspondence: Professor Graham Serjeant, MRC Laboratories(Jamaica), University of the West Indies, Kingston 7, Jamaica.

from 8:30 to 16:30, Monday to Friday. The facilities are freeand patients are encouraged to attend at any time duringthese hours. As a result the clinic is the preferred andprinciple health-care provider for the great majority of sicklecell patients living in Kingston and the surrounding areas.The day-care centre provides oral or intravenous fluids andparenteral pain relief, and is supervised by a full-time nurseand attending physician. The room is kept warm, generallywithout air-conditioning, blankets are provided, electronicmonitoring equipment is kept to a minimum, soft musicplayed, and relatives or friends can attend patients through-out the day. On arrival, patients are seen by the clinicphysician and a brief history taken for possible precipitatingfactors, location and duration of pain, previous analgesia athome, and attendance at other health-care facilities. Physicalexamination assesses the state of hydration, searches forinfection in the upper respiratory tract, urine, osteomyelitis orgallbladder, and other pathology such as acute chestsyndrome. Analgesia is based on intramuscular pethidine50–100 mg, pentazocine 30–60 mg or morphine 5–10 mg,given 2–4-hourly as required. Intramuscular diclofenacsodium 75 mg is commonly added to improve pain relief.Hydration is given orally or intravenously if the patient isdehydrated or vomiting. Patients with underlying pathologywere usually referred for admission after initial pain relief, butothers were monitored during the day. Prior to closure of theclinic, patients were given the option of hospital referral (ifpain control was deemed inadequate) or of returning homewith oral analgesia (a 2 d supply of codeine, pethidine,pentazocine or diclofenac) and the reassurance that theycould return for further day care if necessary. The privatehome phone numbers of all Unit doctors are printed on theappointment cards and all are available for consultation.

RESULTS

Among the 1160 eligible patients enrolled in the clinic, therewere 476 painful crises in 216 adults with SS disease (50%male) accounting for 686 day-care attendances during thestudy year. Single crises occurred in 119 (55·1%) patients,two crises in 42 (19·4%) patients, three in 21 (9·7%), four in16 (7·4%), and five or more in 18 (8%) patients. The number

of day-care attendances for each painful crisis (Table I)showed that 338 (71%) of patients attended for only 1 d, but15 crises required four or more attendances. Assessment ofpatients at the end of the day showed that 42/476 (8·8%)painful crises in 32 patients were referred to hospital becauseof concern about possible underlying pathology or failure ofthe 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, fivedeaths (2·7%) were associated with that painful episode(Table II). Two deaths (one from Salmonella septicaemia) hadclinical 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 ofwhom were referred for admission with no mortality and ninewere sent home on antibiotics, two (22%) of whom died withpathological evidence of acute chest syndrome.

DISCUSSION

The rationale for hospital admission of the painful crisisincludes concern about the potential severity of the under-lying pathology, possible precipitating factors such asinfection, and difficulty in rendering adequate pain relief asoutpatients. The underlying pathological process appears to

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94 M. A. Ware et alTable I. Day-care centre attendances for indivi-dual painful crises.

No. of attendancesFrequency

per painful crisis No. %

1 338 71·02 92 19·33 31 6·54 8 1·75 4 0·86 2 0·47 1 0·2

Total 476

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; Salmonella septicaemiareferred A&E; died same day

19F Bone pain, fever Attended 2 d; referred A&E; Dengue haemorrhagic feverdied 2 d later

23F Bone pain, cough, dyspnoea Died 1 d after discharge Acute chest syndrome; Salmonella septicaemiachest pain

35M Bone pain, dyspnoea, Attended 2 d; died on third day Acute chest syndromechest pain, abnormal chest X-ray after discharge

42M Bone pain, chest signs Died 1 d after discharge No anatomical cause of death

95Day-Care Management of Painful Sickle Crises

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be avascular necrosis of active bone marrow (Charache &Page, 1967) and the pain is assumed to arise from theincrease in intramedullary pressure consequent on theensuing inflammatory response. Only one patient in thecurrent study died during an apparently uncomplicatedpainful crisis, and retrospective analysis of 501 deaths in SSdisease over the last 35 years revealed that 10 (2%) wereassociated with uncomplicated painful crises (unpublishedobservations). This contrasts with experience in the Coop-erative Study of Sickle Cell Disease in the U.S.A. (Platt et al,1994), where 25/209 (12%) of deaths were associated withapparently uncomplicated painful crises. The factors con-tributing to this difference are unclear but may include moreaggressive therapy (Cole et al, 1986). It was a cause ofconcern that of patients opting for day care, five died, four ofwhom were subsequently admitted to hospital, but it remainspossible that earlier admission might have influenced theoutcome. The observation that two of nine patients withevidence of acute chest syndrome sent home on apparentlyappropriate antibiotic therapy subsequently died implies thatthis should be a contraindication to home management.Otherwise, our experience suggests that the painful crisis,although causing distress, does not usually warrant admis-sion because of the severity of the underlying pathology.

The need for admission is generally based on the perceivedinability to provide adequate pain relief as outpatients. Manyfactors influence the patient’s ability to cope with pain andfew objective studies are available but the followingstatements derive largely from Focus Groups in the U.K.(Streetly et al, 1997). Delays in the provision of analgesia inbusy casualty departments contribute to the apprehensionand anxiety experienced by patients in severe pain. Rapidand effective analgesia provided by familiar and experiencedstaff in a specialized unit is likely to allay these tensions.Reassurance that the pathology underlying the painful crisisis not life-threatening, that the pains always resolve, andthat the natural history of bone pains is for a progressiveimprovement and eventual disappearance with advancingage (Serjeant, 1992), are all likely to improve the patient’sability to cope. A dedicated unit is likely to encouragepatients to attend earlier for mild pain when supportivemeasures may prevent the development of more severe pain.The extended family system in Jamaica whereby mostpatients have relatives or friends at home also ensures asupportive environment with provision of fluids and othercare. It is also important for patients to have appropriateexpectations, and ameliorating pain to allow sleep and restmay be achieved by much lower analgesic levels than thosenecessary for the total abolition of pain. Several protocolssupport the effectiveness of oral analgesia in the manage-ment of severe pain (Friedman et al, 1986; Powers, 1986;Jacobson et al, 1997). With full explanation and discussion ofthese factors, this study suggests that many Jamaicanpatients prefer day-care management.

It has been argued that the Jamaican form of sickle celldisease is different from that observed in the U.K. and theU.S.A. (Ranney, 1973). Major genetic differences seemunlikely since all three patient populations are of similarWest African origin, but there may be important social or

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 beenquestioned in temperate environments (Seeler, 1973; Sloviset al, 1986), yet a clear relationship occurred in other studies(Amjad et al, 1974; Resar & Oski, 1991). It seems likely thatthe relationship has been obscured by adaptations inmicroclimate (central heating, warm clothes) rather thanbeing absent. Other potential differences include greaterexpectations of pain relief, the lack of social support forpatients living alone, reluctance to attend emergency roomswhere their perceived reception may not be sympathetic, andother attitudes towards health-care providers (Elander &Midence, 1996). It is to be hoped that education of staff andpatients about the disease and improving social support forthe patient may make home management a preferred andmore viable option than prolonged hospital admission.Investigation of the differences in attitudes between U.K.and Jamaican patients and the factors contributing to themis a vital step in finding solutions to the problems anddevising more appropriate management for all patients.

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