Approach to the vaso-occlusive crisis in adults with sickle cell disease

The vaso-occlusive crisis, or sickle cell crisis, is a common painful complication of sickle cell disease in adolescents and adults. Acute episodes of severe pain (crises) are the primary reason that these patients seek medical care in hospital emergency departments. Frequently, however, the pain is incompletely treated. Despite advances in pain management, physicians are often reluctant to give patients adequate dosages of narcotic analgesics because of concerns about addiction, tolerance and side effects. It is important to recognize a pain crisis early, correct the inciting causes, control pain, maintain euvolemia and, when necessary, administer adequate hemoglobin to decrease the hemoglobin S level. The family physician and the hematologist must work together to treat acute pain episodes promptly and effectively, manage the long-term sequelae of chronic pain and prevent future vaso-occlusive crises.     

Blood pressure in acute vaso-occlusive crises of sickle cell disease

OBJECTIVE: We compared blood pressure (BP) in patients with sickle cell disease (SCD)-related crises and black patients without SCD. METHODS: We retrospectively reviewed charts of emergency department (ED) patients with SCD crises in a 2-year period, recording BPs and demographic and SCD data. A cohort of consecutive black patients without SCD was compared. RESULTS: Included were 459 SCD-related visits, 187 by men and 272 by women, representing 106 patients. Women had significantly lower BP than men, diastolic BP was significantly lower in patients with hemoglobin SS disease than in those with hemoglobin SC disease, and systolic BP was significantly lower in patients with bilateral versus unilateral pain. One SCD patient had a history of hypertension. The 125 non-SCD patients, excluding 25 with a history of hypertension, had significantly higher systolic and diastolic BP than patients in SCD crisis. CONCLUSION: No patients seen in SCD crisis were hypertensive. Patients who were female, had SS disease, or had bilateral pain had lower BP. Significantly higher BP and more hypertension occurred in black patients without SCD.     

Acupuncture for pain management in children with sickle cell disease

Pain associated with sickle cell disease (SCD) is frequently treated with opioids which have many side effects. There is a need for adjuvant non-opioid therapies that can improve pain control. Acupuncture, an integrative approach, has been shown to be useful in non-SCD pain conditions but has been used to a very limited extent in SCD. In this report we present a single academic pediatric center experience showing acceptability, feasibility and improved pain experience with adjuvant acupuncture therapy in children with SCD and suggest that acupuncture should be explored as a treatment option for managing pain in SCD.     

Sensitization to acute procedural pain in pediatric sickle cell disease: modulation by painful vaso-occlusive episodes, age, and endothelin-1

The impact of pain early in life is a salient issue for sickle cell disease (SCD), a genetic condition characterized by painful vaso-occlusive episodes (VOEs) that can begin in the first year of life and persist into adulthood. This study examined the effects of age and pain history (age of onset and frequency of recent VOEs) on acute procedural pain in children with SCD. Endothelin-1, a vaso-active peptide released during VOEs and acute tissue injury, and its precursor, Big Endothelin, were explored as markers of pain sensitization and vaso-occlusion. Sixty-one children with SCD (ages 2 to 18) underwent venipuncture at routine health visits. Procedural pain was assessed via child and caregiver reports and observational distress. Pain history was assessed using retrospective chart review. Three primary results were found: 1) younger age was associated with greater procedural pain across pain outcomes; 2) higher frequency of VOEs was associated with greater procedural pain based on observational distress (regardless of age); and 3) age was found to moderate the relationship between VOEs and procedural pain for child-reported pain and observational distress for children 5 years of age and older. Associations between the endothelin variables and pain prior to venipuncture were also observed. PERSPECTIVE: For children with SCD, the child's age and recent pain history should be considered in procedural pain management. The endothelin system may be involved in preprocedure pain, but additional research is needed to understand the role of endothelins in pain sensitization.     

Home treatment of pain for children and adolescents with sickle cell disease.

This ethnographic study examined family caregivers' perceptions of the effectiveness of comfort measures used at home for children with vaso-occlusive episodes (VOE) of sickle cell disease. Interviews were conducted in the hospital while children experienced VOE. Oral analgesics and most nonpharmacologic pain relief methods were rated by caregivers as only partly effective. Major themes that emerged from interview information reflected caregivers' efforts on behalf of their children in avoiding pain episodes, keeping a normal routine, "catching" the pain, getting their minds off the pain, helping the child get through the episode, and staying out of the hospital. Understanding the role of the family caregiver in home settings may assist health care professionals to provide more empathetic care to children with VOE and to their families when children are admitted to the hospital. Findings may encourage professionals to design the most effective methods of home pain management for these families.     

Intensity, location, and quality of pain in Spanish-speaking children with cancer

Spanish speaking children with cancer were asked to describe their pain during the previous week prior to an oncology clinic appointment. Data showed that 41% of the children were experiencing pain and the overall mean pain intensity rating among these children was 5.7 +/- 2.7. Among those children with moderate to severe pain, the most frequently marked locations on the body outline diagram was the abdomen (53.8%), lower back (46.2%), and upper chest (30.8%). The higher percentage of children complaining of abdominal pain may be attributed to the high percentage (63.6%) of children reporting oral chemotherapy at home. Some children experienced pain that was unrecognized and undetected, and therefore were not receiving medications. To minimize the risk of under-treatment of pain, children and parents may be taught to use the Spanish version of the Adolescent Pediatric Pain Tool to communicate the child's pain to clinicians.     

Adenotonsillectomy in children with sickle cell disease

The pediatric patient with sickle cell disease risks having a vasoocclusive episode during adenotonsillectomy under general anesthesia. With proper patient selection and appropriate perioperative management, adenotonsillectomy can be accomplished safely in children with sickle cell disease. We review the management of 10 children with sickle hemoglobinopathies who had adenotonsillectomy. Indications for surgery were recurrent streptococcal infections in four and obstructive sleep apnea in six of these children. No complications resulted from any of these procedures, and the mean length of postoperative hospitalization was 2.4 days. The principal feature of preoperative management was the transfusion of red blood cells to suppress the patient's endogenous erythropoiesis and to reduce the concentration of sickle cell hemoglobin to less than 30%. Though a prospective, multi-institutional clinical trial will ultimately be required to settle the issue of the safest preoperative management of children with sickle cell disease, balancing the risks of transfusion-related complications against anesthesia-related complications, our experience supports the operative safety of hypertransfusion therapy in children with sickle cell disease.     

Laparoscopic splenectomy in children with sickle cell disease

Sickle cell disease is a group of genetic disorders that are characterized by the production of hemoglobin S, anemia, and acute and chronic tissue damage secondary to blockages caused by abnormally shaped (i.e., sickle-shaped) red blood cells. In children, complications of sickle cell disease (e.g., splenic sequestration and splenomegaly) increase the risks for postoperative complications due to the physical stress of the large incisions previously require for splenectomy. An intracorporeal splenic fragmentation technique is now being used to avoid these complications and will be discussed in this article.     

Pain drawings and sickle cell disease pain

This study examined the relationship of pain drawings to somatization in patients with sickle cell disease (SCD). Sixty-nine adult patients with SCD completed a pain drawing in which they shaded in areas of the body in which they experienced pain and also completed the symptom checklist (SCL) 90-R as an index of psychological distress. Analysis of variance indicated that patients who were categorized as having pain drawings with sites inconsistent with expected SCD pain patterns had somatization scores in the clinically significant range. The results suggest that health care professionals who treat SCD patients need to consider pain patterns. In individuals with pain patterns atypical for SCD, the psychological status of the patient may need to be evaluated to facilitate optimal pain management.     

Treatment of cholelithiasis in children with sickle cell disease

Medical and surgical advances have improved the treatment of cholelithiasis (ie, gallstones) in children with sickle cell disease (SCD). Children with SCD have an increased risk of developing pigment gallstones that initially may be asymptomatic but that can lead to acute symptoms of cholelithiasis. An elective laparoscopic cholecystectomy procedure is recommended for pediatric patients with SCD to prevent the risk of requiring an emergency cholecystectomy procedure. The primary benefits of this approach include a shorter hospital stay after surgery, decreased postoperative discomfort, decreased risk of complications, and a quicker return to normal activities. AORN J 77 (June 2003) 1170-1182.     

Changes in sleep, food intake, and activity levels during acute painful episodes in children with sickle cell disease

As part of a larger study that examined pain experience, pain management, and pain outcomes among children with sickle cell disease, functional status (sleep, food intake, and activity levels) was examined during hospitalization for acute painful episodes. Children were asked to rate the amount of pain they experienced as well as the amount of time they slept, the amount of food they ate, and the amount of activity they had everyday. Children reported high levels of pain, which showed only a small decrease throughout hospitalization, and had disrupted sleep and wake patterns, decreased food intake, and decreased activity levels. Nurses need to routinely monitor functional status during acute painful episodes so that strategies to promote adequate sleep, food intake, and activity may be incorporated to minimize long-term negative outcomes in children with sickle cell disease.     

Bacterial blood cultures in children with sickle cell disease

Background

Children with sickle cell disease (SCD) are considered at risk for bacteremia, especially when they present to the emergency department (ED) with fever.

Objective

We aimed to determine the incidence of bacteremia in children with SCD presenting with or without fever to a pediatric ED.

Methods

A retrospective chart review of 692 pediatric ED visits of children with SCD during a 2-year period was conducted.

Results

Seven blood cultures (6 homozygous and 1 heterozygous) had bacterial growth (1.3%; 95% confidence interval, 0.5-2.1), 3 of which were among febrile children (1.7%; 95% confidence interval, 0-3.6). All identified microorganisms are part of the normal skin or oral flora and could represent contamination. None of the patients had growth of the Streptococcus pneumoniae species.

Conclusion

A very low rate of bacterial growth and no S pneumoniae were found. The absence of S pneumoniae in our cohort can be associated with the addition of the 7-valent pneumococcal conjugate vaccine.     

Managing acute and chronic pain in sickle cell disease

Acute painful episodes among patients with sickle cell disease may occur in any body part or several sites simultaneously. The reasons for inadequate pain relief for this patient group are complex. Prompt, accurate pain assessment is the cornerstone of effective pain management and a comprehensive, multidisciplinary team strategy is essential, particularly for managing patients with frequent acute pain or chronic pain.