Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome

PURPOSE: Our objective was to evaluate L-arginine and nitric oxide metabolite (NOx) levels in children with sickle cell disease (SCD) at steady-state and during vaso-occlusive crisis (VOC). Because alterations in nitric oxide production may have an important role in the pathophysiology of SCD, our second aim was to determine if a relationship exists between these levels and vaso-occlusive crisis (VOC). PATIENTS AND METHODS: Plasma L-arginine and serum NOx levels were examined in 36 patients with SCD with 39 episodes of VOC and 10 children with SCD at steady-state. Daily levels were obtained in children requiring hospitalization. RESULTS: Steady-state L-arginine levels were normal in children with SCD. L-arginine levels were low, however, in children with VOC (37.4 +/- 2.7 vs. 53.6 +/- 4.6 micromol/L; P = 0.008) but returned to baseline during hospitalization. In contrast, NOx levels were normal at presentation but decreased during hospitalization for both patients with VOC and patients with acute chest syndrome (ACS) (21.1 +/- 2.0, 17.4 +/- 2.4, and 12.3 +/- 1.6 micromol/L, respectively; P < 0.05). In the patients with VOC who had ACS develop, L-arginine decreased to the lowest levels at the time of the ACS diagnosis, correlating with decreasing NOx levels. CONCLUSION: These data suggest that there may be a relationship between the L-arginine-nitric oxide pathway and vaso-occlusion in SCD. Low arginine levels during VOC could reflect a state of acute substrate depletion that results in a decrease in nitric oxide production.     

Children with sickle cell disease and human immunodeficiency virus-1 infection: use of inpatient care services in the United States

BACKGROUND: The purpose of this study was to describe hospital use patterns of children with sickle cell disease (SCD) and human immunodeficiency virus type-1 (HIV) infection in the United States. METHODS: Hospital discharges of children with 1 or both of the 2 conditions (SCD and HIV infection) were analyzed using nationally weighted data from the 1994 to 2003 Nationwide Inpatient Databases of the Healthcare Cost and Utilization Project. Demographic and hospital characteristics, length of stay, charges and the most frequent diagnoses and procedures performed during the hospitalization were compared. Multivariate logistic regression was used to analyze the effects of age, sex and HIV infection on number of hospitalizations for selected conditions. RESULTS: There were an estimated 686 hospitalizations of children with SCD and HIV infection in the United States in the 10-year period 1994-2003; these hospitalizations aggregated in the South (78.2%) and their expected payer was mostly Medicaid/Medicare (82.0%). Their average length of stay was longer than that of children with SCD alone (8.0 days vs. 4.3 days, respectively), and the mean charges associated with the hospitalization were also higher ($18,291 vs. $9584). Compared with patients with SCD without HIV, HIV infection conferred a higher risk for hospitalizations for bacterial infections and sepsis (odds ratio 2.75; 95% CI, 1.66-4.6), but less of a risk for vaso-occlusive crises (odds ratio 0.32; 95% CI, 0.22-0.48). Inpatient case-fatality rate of children with SCD and HIV was no different from that of children with SCD alone, but lower than that of the rest of children with HIV infection. CONCLUSIONS: Hospitalized children with SCD and HIV infection have higher odds of infection than those with SCD alone. Their inpatient case-fatality rate is lower than that of children with HIV infection alone. These findings should be considered in designing appropriate interventions for this population.     

Growth hormone response to growth hormone releasing factor in sickle cell disease

Many children with sickle cell disease (SCD) have impaired growth during childhood and adolescence, with patterns of growth consistent with constitutional delay in growth and pubertal development (CDGD). We evaluated the growth hormone (GH) response to a rapid intravenous (i.v.) infusion of growth hormone releasing factor (GRF, 1-44, 1 microgram/kg) in six children with SCD whose growth patterns and bone ages were consistent with CDGD. The peak GH response of the SCD patients to GRF (29.2 +/- 14.3 ng/ml, mean +/- SD, n = 6) was not statistically significantly different from the peak GH response of the control children (29.0 +/- 6.3 ng/ml, mean +/- SD, n = 7). These findings suggest that pituicyte GH response to GRF is intact and is not the cause of the observed impaired growth in patients with SCD.     

Sickle cell anemia from central India: A retrospective analysis

Although sickle cell anemia in India is believed to have a mild clinical presentation, few studies report severe disease in many patients from central India. Hence, we have retrospectively studied 316 children with SCA who were followed up for a period of 5.8±5.7 years. There were 55.4 blood transfusions, 43.3 episodes of vaso-occlusive crises requiring hospitalization, and 108.9 hospitalizations per 100 person years. Ninety six (30%) patients had severe disease whereas 74 patients also fulfilled the criteria for hydroxyurea therapy. Significant proportion of children with sickle cell anemia from central India present with severe clinical presentation and require regular medical attention.     

Sickle cell disease in children in Dakar, Senegal]

AIM OF THE STUDY: To determine the socioeconomic, clinical and biological aspects of sickle cell disease (SCD) in Senegalese children and adolescents, we retrospectively analysed all records of follow-up attending patients in the Albert Royer Children Hospital of Dakar (Senegal). RESULTS: Homozygous sickle cell (SS) was the most frequent genotype (307 cases). Sickle cell hemoglobin C (13 cases) and sickle cell beta-thalassemia (three cases) were uncommon. Patients were aged from five months to 22 years (mean age: eight years). Most of them came from poor families. The mean number of children was five in patients' families, with at least two cases of SCD in 60% of them. Immunization against hepatitis B virus (10.2%), Haemophilus influenzae b (8.4%), Salmonella (8.7%) and Streptococcus pneumoniae (21.4%) was insufficiently performed, because of its relatively high cost. Only 30% of the patients had received a blood transfusion. Painful crises occurred less than three times a year in 74% of the cases. Complications such as acute chest syndrome (1%), stroke (1%), cholelithiasis (9%), meningitis (0.4%), septicemia (2%) and osteomyelitis (6%) were rare. Mean steady state hemoglobin (Hb) and hemoglobin F(HbF) levels were 8.27 +/- 1.36 g/dL and 6.8 +/- 5.9% respectively among SS patients. No correlations were found neither between Hb and HbF nor between these parameters and the frequency of complications. Eleven patients (1.1% per year of follow-up) died, and infection was the main cause of death (73%). CONCLUSION: In comparison with published data, SCD seems to have mild severity in Senegalese children and adolescents in spite of poor follow-up conditions. In addition to genetic factors, environmental factors might have an important role in disease tolerance.     

The economic burden of environmental tobacco smoke in the first year of life.

AIMS: To examine the population impact and economic costs associated with environmental tobacco smoke (ETS) in Chinese infants with non-smoking mothers. METHODS: Prospective, population based birth cohort study in Hong Kong, 1997-98. Main outcome measures were: doctor consultations and hospitalisations; adjusted odds ratios for higher utilisation by service type for each of the ETS exposure variables; corresponding population attributable risks (PARs); and associated extra health care costs. RESULTS: For the 1997 annual birth cohort, ETS exposure through the mother in utero was positively associated with higher consultation (adjusted odds ratio (OR) 1.26) and hospitalisation (OR 1.18) due to any illness. This translated into 7.4% of all inpatient episodes in the first year of life, representing an additional 1581 hospital attendances that cost over 2.1 million US dollars. The corresponding PAR for outpatient services was 7.7%, where the majority was due to respiratory or febrile illnesses, accounting for 0.44 million dollars in extra costs. Postnatal exposure to ETS at home was linked to higher rates of hospitalisations for any illness compared with non-exposed infants (OR 1.12), which led to 662 extra hospitalisations consuming 0.90 million dollars, where the associated PAR was 3.1%. CONCLUSIONS: Assuming that ETS was causally associated with health services use, about 9% of the total direct medical costs in the first year of life can be attributed to passive smoking. The community, as well as the private citizen, needs to be made aware of the costs foregone from exposure to tobacco smoke as well as the potential savings from a smoke-free society.     

Impact of Environmental Tobacco Smoke on Children With Asthma,United States, 2003–2010

ObjectiveGiven widespread interventions to reduce environmental tobacco smoke (ETS) exposure and improve asthma control, we sought to assess the current impact of ETS exposure on children with asthma.MethodsWe analyzed 2003–2010 data for nonsmoking children aged 6 to 19 years with asthma from the National Health and Nutrition Examination Survey. Outcomes (sleep disturbance, missed school days, health care visits, activity limitation, and wheezing with exercise) were compared between ETS exposed children (serum cotinine levels 0.05 to 10 ng/mL) and unexposed children (<0.05 ng/mL) using ordinal regression adjusted for demographic characteristics. We also assessed whether associations were observable with low ETS exposure levels (0.05 to 1.0 ng/mL).ResultsOverall, 53.3% of children aged 6 to 19 years with asthma were ETS exposed. Age-stratified models showed associations between ETS exposure and most adverse outcomes among 6- to 11-year-olds, but not 12- to 19-year-olds. Even ETS exposure associated with low serum cotinine levels was associated with adverse outcomes for 6- to 11-year-olds. Race-stratified models for children aged 6 to 19 years showed an association between ETS exposure and missing school, health care visits, and activity limitation due to wheezing among non-Hispanic white children, and disturbed sleep among non-Hispanic white and Mexican children. Among non-Hispanic black children, there was no elevated risk between ETS exposure and the assessed outcomes: non-Hispanic black children had high rates of adverse outcomes regardless of ETS exposure.ConclusionsAmong children with asthma 6 to 11 years of age, ETS exposure was associated with most adverse outcomes. Even ETS exposure resulting in low serum cotinine levels was associated with risks for young children with asthma.     

Pyridoxal 5'-phosphate levels in children with sickle cell disease

Pyridoxal 5'-phosphate (PLP), the major coenzyme form of vitamin B6, is known to have antisickling properties in vitro. Recently, low plasma PLP levels were reported in a group of adults with sickle cell anemia. We measured the plasma PLP levels in a group of 55 asymptomatic nontransfused children with sickle cell diseases (SCD) to determine the prevalence of low plasma PLP levels in this population. Comparative studies were made with the measurement of PLP in three other groups serving as controls: Group A (black children, n = 36); Group B (white children, n = 37); and Group C (black adults, n = 13). PLP was measured directly in plasma by a radioenzymatic technique. The results of these comparisons showed that there was no statistically significant difference in plasma PLP of black children with SCD (10.7 +/- 10.0 ng/ml) as compared with black control children (group A, 9.0 +/- 12.3 ng/ml). The low plasma levels PLP in these two groups were significantly lower than that of the plasma PLP of white control children (group B, 15.85 +/- 15.92 ng/ml). This data suggest that a high prevalence of low PLP levels exists in black children seen at Grady Memorial Hospital, both with and without SCD.     

Bioelectrical impedance analysis of the body composition of children and adolescents with sickle cell disease

OBJECTIVES: To examine the body composition of children and adolescents with sickle cell disease (SCD) using bioelectrical impedance analysis and to determine if the impedance parameters resistance, reactance, and phase angle are able to distinguish between subjects with SCD and age- and gender matched controls. STUDY DESIGN: Total body resistance and reactance were obtained for a total of 53 subjects with SCD (27 male and 26 female) between 10 and 18 years of age and 49 control subjects (23 male and 26 female). The fat-free mass, body cell mass, phase angle, and capacitance were also determined. Group comparisons were made using the 2-sample t test. RESULTS: Male subjects with SCD had significantly lower fat-free mass (37.5 +/- 8.8 vs 43.9 +/- 12.3 kg, P =.04), body cell mass (17.4 +/- 4.3 vs 21.7 +/- 5.8 kg,P =.005), and body fat (3.7 +/- 2.6 vs 6.6 +/- 4.7 kg, P =.008) compared with controls. No significant differences in any body composition components were found for the female subjects. Both male and female subjects had significantly lower phase angle measurements (P <.001 and.006, respectively) than their respective controls, indicating possible alterations in cell membrane properties because of an imbalance in membrane composition or function. CONCLUSIONS: Bioelectrical impedance analysis can be used to determine body composition differences in children with SCD. The phase angle may provide a useful method to monitor the efficacy of therapeutic interventions in patients with SCD.     

Assessment of cerebral tissue oxygenation in patients with sickle cell disease: effect of transfusion therapy

This study used spatially resolved transcranial near-infrared spectroscopy (NIRS) to compare brain tissue oxygenation in sickle cell disease (SCD) patients with that of healthy children. In addition, NIRS was used to measure the dynamic response of cerebral oxygen balance to erythrocytapheresis. Transcranial NIRS measurements were obtained from 25 children with SCD who were not receiving transfusion or hydroxyurea therapy (NT-SCD). These patients were divided into two subgroups, those with mild (n = 10) or severe (n = 15) SCD symptoms. In addition, NIRS measurements were performed in 16 SCD patients with severe disease maintained on long-term erythrocytapheresis (T-SCD) and in 35 control children. The lowest mean brain tissue oxygen saturation occurred in the NT-SCD subgroup with severe symptoms (48 +/- 9%; P < 0.001 vs. control). NT-SCD patients with mild symptoms had higher saturation (62 +/- 8%; P < 0.001 vs. control), while the highest appeared in the control group (72 +/- 7%). In T-SCD patients, however, brain tissue oxygen saturations were higher than severely symptomatic NT-SCD children and similar to mildly symptomatic NT-SCD children (65 +/- 7%). Non-invasive measurements of brain tissue oxygenation with NIRS revealed that abnormal oxygen saturation levels in SCD patients correlated with the severity of their clinical manifestations. Additionally, cerebral oxygen balance seems to be favorably affected by erythro-cytapheresis.     

Vitamin A status, hospitalizations, and other outcomes in young children with sickle cell disease

OBJECTIVE: To determine the relation of serum vitamin A status to growth, nutritional and hematologic status, and to the number of hospitalizations in children with sickle cell disease-SS (homozygous for the S allele, SCD-SS). STUDY DESIGN: Children (2-9.9 years of age) with SCD-SS were assessed for serum retinol, hemoglobin, hematocrit, reticulocyte count, height, weight, body mass index, and recalled dietary intake. Vitamin A status was defined on the basis of serum retinol concentration as suboptimal (<30 microg/dL) and normal (> or =30 microg/dL). Hospitalizations were determined for 1 year after vitamin A assessment. RESULTS: Mean serum retinol was 26.7 +/- 6.8 microg/dL in 66 subjects (39 girls) and was suboptimal in 66% of children. Compared with those with normal status, children with suboptimal vitamin A had significantly lower body mass index z score (-0.7 +/- 1.0 vs -0.1 +/- 0.6) and hemoglobin (7.9 +/- 1.1 vs 8.5 +/- 1.1), and hematocrit (23.3 +/- 3.0 vs 25.1 +/- 3.8) and significantly more hospitalizations (2.8 +/- 2.0 vs 0.7 +/- 0.8). After adjusting for age and sex, suboptimal vitamin A status was associated with a 10-fold increased risk for hospitalization (OR, 10.5; 95% CI, 2.3, 48.6) and with increased pain (OR,5.3; 95% CI, 1.3, 21.6) and fever episodes (OR, 6.4; 95% CI, 1.7, 24.9) requiring hospitalization. CONCLUSIONS: Suboptimal vitamin A status was prevalent in US children with SCD-SS and was associated with increased hospitalizations and poor growth and hematologic status.     

Exhaled carbon monoxide levels in children with sickle cell disease

It is important to measure the rate of haemolysis in patients with sickle cell disease (SCD) to identify aplastic crises and indirectly assess the rate of vaso-occlusion and sequestration. The aim of this study was to assess whether end-tidal carbon monoxide (ETCOc) levels in children with sickle cell disease (SCD) could be measured reproducibly, reflected haemolysis and whether they were elevated compared to those of similarly aged, ethnic matched children without SCD (controls). ETCOc levels were measured non-invasively in 87 SCD children (age range 2.3–17.6 years) and 26 age and ethnic origin matched healthy controls using an electro-chemical sensor. The within- and between- occasion reproducibilities were assessed in ten and 15 SCD children respectively. ETCOc levels of 15 SCD children undergoing regular transfusions were related to carboxyhaemoglobin, haemoglobin and bilirubin levels. The within and between occasions mean intrasubject coefficients of reproducibility were 5% and 18% respectively. Positive correlations were found between the ETCOc and carboxyhaemoglobin ( P =0.007) and bilirubin ( P =0.02) levels, and a significant negative correlation between the ETCOc and haemoglobin ( P =0.0002) levels. The mean and SD ETCOc levels of the SCD children (4.9 ppm; SD 1.7 ppm) were significantly higher than that of the controls (mean 1.3 ppm; SD 0.4 ppm) (difference between means 3.60; 95% C.I. 2.93–4.28; P <0.0001). Conclusion:These results suggest that measurement of end-tidal carbon monoxide levels is a reliable and useful method to monitor haemolysis in children with sickle cell disease.     

Growth hormone deficiency in patients with sickle cell disease and growth failure

BACKGROUND: Growth disorders are common in children with sickle cell disease (SCD). The etiology for growth disturbances in this population appears to be multifactorial. Recent evidence suggests abnormalities in the growth hormone (GH)/insulin-like growth factor-I (IGF-I) and IGF binding protein-3 (IGFBP-3) axis may play a role. OBJECTIVE: To measure GH levels through provocative stimulation in a group of patients with SCD with growth failure, and to evaluate response to treatment. PATIENTS AND METHODS: Growth records were reviewed of 79 children with sickle cell hemoglobinopathies to identify children with growth failure. GH levels were measured in patients with SCD with and without growth failure using arginine and L-Dopa as provocative stimulation tests. Treatment with GH was offered to GH-deficient children with SCD and these patients were followed longitudinally over 5 years. RESULTS: Of the 79 patients, 13 (16.5%, all SS) had heights less than -2 SD below the mean or a growth velocity < -2 SD below the mean for age. Seven of the 13 children with growth failure participated in this study. Five patients received GH for 3 or more years and demonstrated significant improvement in their height SDS. One of the two who declined treatment was lost to follow-up and the other had significant worsening of height SDS score. CONCLUSION: GH deficiency may be associated with growth failure in some patients with SCD. These patients may benefit from treatment with GH.     

Hodgkin lymphoma in a child with sickle cell anemia

There are reports of patients with sickle cell disease who developed hematological malignancies but the relationship between these malignancies and sickle cell disease (SCD) is not yet defined. The co-existence of a hematological malignancy with SCD poses certain challenges for the management of each condition. We describe a 7-year-old boy with sickle cell anemia who developed Hodgkin's lymphoma and the challenges of management. He presented with a 4-year history of bilateral neck swelling and a 2-month history of weight loss and high-grade fever. Histology of a lymph node biopsy was consistent with mixed cellularity Hodgkin's lymphoma. He was treated with five cycles of Cyclophosphamide, Vincristine, Procarbazine and Prednisolone (COPP) and had complete clinical response. Chemotherapy was associated with an increase in frequency of painful crises and complicated by septicaemia. Blood transfusion needs were minimal; apart from the transfusion preceding the first cycle of chemotherapy, there was no need for further transfusion. Myelosuppression was not a problem in the patient; he responded well to antibiotics during the two episodes of septicemia without the use of hemopoetic growth factor. Patients with sickle cell anaemia who develop Hodgkin's lymphoma can be successfully treated with chemotherapy along with supportive management for crises and infections.     

Association between exposure to environmental tobacco smoke (ETS) and breastfeeding behaviour

AIM: A cross-sectional study was conducted to investigate the association between breastfeeding behaviour and exposure to environmental tobacco smoke (ETS). METHOD: Questionnaires were collected from 552 women. Blood and urine specimens were taken from part of the population at the time of delivery. The study population was classified into two groups: those exposed to ETS and those unexposed, based on self-reports from the subjects involved in the study. Cotinine levels in the urine and blood specimens were analysed by HPLC-UV under strict quality control procedures. RESULTS: There was a significant negative association between the exposure to ETS at home or in the workplace and the prevalence of breastfeeding in each of the 6 months following delivery using multiple logistic regression adjusted for other covariates. The cotinine levels in the urine and blood were dose-dependent, but not significantly so. However, women with lower cotinine levels had a higher probability of breastfeeding than those with higher levels. CONCLUSION: Women who are exposed to ETS have a low likelihood of breastfeeding. It is necessary for the government to regulate ETS in public areas and confined spaces in order to reduce the levels of ETS that women are exposed to.     

Side effects following COVID-19 vaccination in pediatric patients with sickle cell disease

Vulnerable patient populations have seen decreased rates of vaccination against SARS-CoV2-19 (COVID-19) due to hesitancies and distrust, magnified by a paucity of data for certain populations. The rate of COVID-19 vaccination in children with sickle cell disease (SCD) remains low despite the risk for severe complications, resulting in continued infections and hospitalizations from COVID-19. We sought to describe vaccine reactions, including vaso-occlusive crises, emergency department visits, and hospitalizations, in children with SCD. Our findings will start to provide the necessary vaccine side effect data to inform patients, caregivers, and clinicians considering the COVID-19 primary vaccination series.     

Impact of acute chest syndrome on lung function of children with sickle cell disease

OBJECTIVE: To test the hypothesis that children with sickle cell disease (SCD) who experienced an acute chest syndrome (ACS) hospitalization episode would have worse lung function than children with SCD without ACS episodes. STUDY DESIGN: Forced expiratory volume in 1 second (FEV(1)); forced vital capacity (FVC); FEV(1)/FVC ratio; peak expiratory flow (PEF); forced expiratory flow at 25% (FEF(25)), 50% (FEF(50)), and 75% (FEF(75)) of FVC; airway resistance (Raw); and lung volumes were compared in 20 children with ACS and 20 aged-matched children without ACS (median age, 11 years; range, 6 to 16 years). Fourteen age-matched pairs were assessed before and after bronchodilator use. RESULTS: The mean Raw (P = .03), TLC (P = .01), and RV (P = .003) were significantly higher in the group with ACS than in the group without ACS. There were no significant differences in the changes in lung function test results in response to bronchodilator administration between the 2 groups, but the children with ACS had a lower FEF(25) (P = .04) and FEF(75) (P = .03) pre-bronchodilator use and a lower mean FEV(1)/FVC ratio (P = .03) and FEF(75) (P = .03) post-bronchodilator use. CONCLUSIONS: Children with SCD who experienced an ACS hospitalization episode had significant differences in lung function compared with those who did not experience ACS episodes. Our results are compatible with the hypothesis that ACS episodes predispose children to increased airway obstruction.     

Early blood transfusions protect against microalbuminuria in children with sickle cell disease

BACKGROUND: Microalbuminuria (MA) is an early indicator for glomerulopathy in sickle cell disease (SCD). PROCEDURE: We reviewed the medical records of asymptomatic patients ages 4-20 with sickle hemoglobinopathies, who were screened for MA in order to find out its prevalence and risk factors. RESULTS: Nineteen of 120 (15.8%) screened patients had MA detected by spot urine (mean albumin absolute value 6.95 +/- 0.56 mg/dl) and abnormal albumin to creatinine ratios (79.8 +/- 0.62 mg/g creatinine). Twenty four-hour urine collections confirmed 57% of MA cases by spot urine. There was no difference in hyperfiltration between positive and negative patients. From the MA-positive patients, 15 had SS (16.8% of SS group) and 4 had SC (18% of SC group). Nineteen percent of children 10 years of age or older had MA, as compared to 8% of the younger children (P = 0.018), demonstrating that increasing age is a risk factor for MA. There was a positive correlation between MA and acute chest syndrome. Young age at start of chronic transfusions was inversely related to MA and therefore renoprotective (P = 0.03). We did not see a protective effect in the group of patients taking hydroxyurea for a relatively short time, mean age at start of treatment 12 +/- 5 years; however the sample was small. CONCLUSIONS: We conclude that: (1) children with sickle cell hemoglobinopathies 10 years or older should be screened for MA and (2) chronic transfusions starting at an early age may be renoprotective.     

Effect of hydroxyurea in sickle cell anemia: a clinical trial in children and teenagers with severe sickle cell anemia and sickle cell beta-thalassemia.

This study evaluated the efficacy of hydroxyurea treatment in the prevention of vaso-occlusive crises among children and teenagers with severe sickle cell anemia and sickle cell beta-thalassemia. Nineteen children and young adults with severe sickle cell disease were enrolled to the hydroxyurea treatment trial. The incidence of vaso-occlusive crises, acute chest syndrome, hemolytic crises, splenic sequestration episodes, blood transfusions, and hospital days in the 2 years before hydroxyurea (HU) treatment were compared with the same parameters in the first 2 years of treatment. The patients received a mean dose of 21.3 mg/kg/day daily and were treated during a mean period of 40.3 +/- 14 months (range 20 to 68 months). Significant increases were observed after 1 month in the Hgb, MCV, MCH, and MCHC levels and were more notable after 3 months. The increase in the Hgb F level became important after 3 months of HU therapy and was highly significant (p < .001) beyond 6 months. No differences were observed in the RDW, reticulocyte count, Hgb S, and Hgb A2. Severe neutropenia was observed in one case. A decrease in the frequency of vaso-occlusive crises, acute chest syndrome, hemolytic crises, blood transfusions, and days spent in the hospital was demonstrated during the HU treatment period compared to the same period before. The clinical and laboratory response to HU was dramatic in severely affected sickle cell anemia (SCA) patients. The response to HU in children and teenagers with severe sickle cell anemia is similar to the response in adults, and no severe adverse effects were observed.     

Caregiving time in sickle cell disease: psychological effects in maternal caregivers

Background

Providing home care for a child with a chronic illness can be stressful for the family. The purpose of this paper is to examine patterns of caregiving and the associated psychological impact on maternal caregivers of children with sickle cell disease (SCD).

Procedure

Fourteen maternal caregivers of children with SCD were interviewed as part of a larger study of maternal caregivers of children with chronic illness. Forty‐four caregivers of children with HIV and 36 caregivers of healthy children were included as comparison groups. Interviews included questions regarding amount of time spent providing care for the child (technical care, non‐technical care, health care management), hospitalization, emergency room visits, illness stigma, and mental health of the caregiver.

Results

Children with SCD had significantly lower functional status and significantly more hospitalizations in the previous 3 months than children with HIV. Caregivers of children with SCD were more likely to work full‐time and had higher incomes than caregivers of children with HIV. The three caregiving groups did not differ significantly on amount of total care, although caregivers of children with SCD and caregivers of children with HIV both reported significantly more time spent in technical care than caregivers of healthy children. Despite lower functional status of the children in the SCD group, when group comparisons on caregiving time variables were adjusted for child's functional status, the differences between groups increased. This appeared to be due to the fact that caregivers in the HIV group spent more time in all caregiving categories except skin, crisis, and other care. In terms of caregiver mental health, caregivers of children with HIV and SCD had significantly higher depressive mood scores than caregivers of healthy children but the groups did not differ on caregiving burden.

Conclusions

The perceived care burden of caregivers of children with SCD may be related to the unpredictable nature of the crisis care they provide. Additional attention is warranted to developing adequate resources for caregivers of children with SCD to mitigate the stress of unexpected crises. Pediatr Blood Cancer 2007;48:64–71. © 2006 Wiley‐Liss, Inc.