Stability and change in the psychological adjustment of mothers of children and adolescents with cystic fibrosis and sickle cell disease

Found moderate stability in the classification of maternal adjustment in two longitudinal studies of mothers of children and adolescents with cystic fibrosis and sickle cell disease. In terms of the transactional stress and coping model, stable poor maternal adjustment was associated with higher levels of appraisal of daily stress and palliative coping and low levels of family supportiveness. With initial levels of maternal adjustment, demographic parameters, and follow-up interval controlled, concurrent levels of daily stress accounted for significant portions of variance in maternal adjustment at follow-up for both illness groups. In addition, illness severity, child psychological adjustment, and family conflict added significant increments to maternal adjustment at follow-up in the cystic fibrosis group. Findings are discussed in terms of a basis for subsequent intervention studies to enhance the adjustment of mothers of children with chronic illness.     

Role of child and maternal processes in the psychological adjustment of children with sickle cell disease.

In this study, 64% of children aged 7–12 yrs with sickle cell disease were found to have a parent-reported behavior problem, and 50% met the criteria for a Diagnostic and Statistical Manual of Mental Disorders-III-Revised (DSM-III-R) diagnosis based on a structured clinical interview of the child. Internalizing types of behavior problems and diagnoses were the most frequent. Support was provided for a transactional stress and coping model in delineating the processes associated with child adjustment. In particular, maternal anxiety accounted for 16–33% of the variance in mother-reported internalizing and externalizing behavior problems, respectively, and child pain-coping strategies accounted for 21% of the variance in child-reported adjustment problems. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Psychological adjustment of adolescents with sickle cell disease: relations with demographic, medical, and family competence variables

OBJECTIVE: Investigate the hypothesis that family competence in addressing challenges associated with sickle cell disease (SCD) contributes to adolescents' adjustment. METHOD: During routine clinic appointments, 80 adolescents (M age = 14.4 years) and their parents independently completed the Self-Report Family Inventory (SFI), which assesses family competence, and measures of adolescent adjustment problems. Information related to disease severity was obtained from clinic files. RESULTS: Regression analyses controlling for demographic and medical variables revealed that higher family competence was associated with fewer internalizing and externalizing behaviors by the adolescent; these relations were particularly true for younger adolescents and for girls. Parental reports of somatic complaints in girls were predicted by parental ratings of family competence. DISCUSSION: Interventions for adolescents with SCD should be family-centered and should focus on strengthening the family's ability to manage stressors associated with parenting an adolescent with a chronic illness.     

Psychological adjustment of mothers of children and adolescents with sickle cell disease: the role of stress, coping methods, and family functioning

Assessed the psychological adjustment of 78 mothers of children and adolescents (7-17 years of age) with sickle cell disease. Support was provided for a transactional stress and coping model in delineating the processes associated with maternal adjustment. In particular, poor maternal adjustment was associated with use of palliative coping methods and high levels of stress related to daily hassles. Variables of the model accounted for 55% of the variance in maternal psychological distress.     

Stress, coping, and psychological adjustment of adults with sickle cell disease.

Examined a transactional model of psychological adjustment to chronic illness with 109 African-American adults with sickle cell disease (SCD). Good psychological adjustment was associated with lower levels of perceived daily stress and stress regarding SCD illness tasks, higher efficacy expectations, less use of palliative coping methods, less use of negative thinking/passive adherence pain-coping strategies, and family functioning characterized by high levels of support and low levels of conflict and control. Overall, the underlying stress and coping conceptual model accounted for 44–50% of the variance in psychological adjustment. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Psychological and academic adjustment in adopted children.

Evaluated psychological and academic adjustment of 130 6–11 yr old adopted children and 130 age-matched nonadopted children using the Hahnemann Elementary School Behavior Rating Scale and a child behavior profile. Results show that adopted Ss were rated higher in psychological and school-related behavior problems and lower in social competence and school achievement than were nonadopted Ss. It is suggested that although the results support the position that the risk is greater for adopted children to develop emotional and school-related problems, the data should not be overinterpreted because the majority of adopted Ss were within the normal range of behavior. (50 ref) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Prothrombotic changes in children with sickle cell disease: relationships to cerebrovascular disease and transfusion

Vascular occlusion has a central role in the pathophysiology of sickle cell disease (SCD) and, although there is little evidence that thrombosis alone is responsible, patients with sickle cell disease are known to have an ill-defined but increased thrombotic risk. The most serious complication of this in childhood is stroke which occurs in 7-10% of children and a further 14% have asymptomatic cerebrovascular disease (CVD) on imaging. We have performed a comprehensive profile of coagulation inhibitors and markers of thrombin generation in 96 children (83 nontransfused [NTx] and 13 transfused [Tx]) with steady-state SCD and 18 healthy sibling controls. The levels of protein S (free and total) and heparin cofactor II were reduced in both the NTx and Tx groups compared to controls and protein C and APC resistance ratios were reduced in the NTx group only. Antithrombin levels were not different from controls. Thrombin-antithrombin complexes and prothrombin fragment F1+2 were increased in both patient groups. In the NTx subgroups with or without CVD there were no differences for any of the parameters measured except for lower haemoglobin levels and higher white cell counts in those with asymptomatic CVD. We conclude that children with SCD have a reduction in levels of the majority of the coagulation inhibitors and increased thrombin generation in the steady-state and these are only partially reversed by transfusion. However, these abnormalities do not appear to play a primary role in the development of cerebrovascular disease.     

Nephrotic syndrome in Nigerian children with homozygous sickle cell disease

Two hundred and seventy two children with the nephrotic syndrome were seen and followed up at the Paediatric Renal Unit of the University of Nigeria Teaching Hospital, Enugu over 12 1/2 year period, between June 1983 and December 1995. Of these, there were nine (3.3%) children with homozygous sickle cell disease (SS). This latter group had a mean age of 9.6 +/- 3.2 years, male to female ratio of 1:2 and serum cholesterol of 3.13 +/- 1.48 mmol/l at the time of diagnosis. The mortality rate was 55.6% in these sicklers, death occurring within one and a half to five years of diagnosis. Causes of death were attributable to sickle cell disease in 60%, renal diseases in 20%, and other causes in 20%. Renal biopsy in two of the sicklers showed membrano-proliferative glomerulonephritis (MPGN) and focal glomerulosclerosis (FGS) respectively. Homozygous sickle cell disease does seem to predispose to the development of the nephrotic syndrome and those that do develop nephrotic syndrome exhibit some special characteristics, when compared to non-sicklers with nephrotic syndrome. These include older age of onset of the nephrotic syndrome, reverse sex ratios, lower serum cholesterol, higher mortality rate and sickle cell complications rather than chronic renal failure as the major cause of death.     

Protein C activity in Gabonese children with sickle cell disease

Protein C levels were determined in 40 Gabonese children with sickle cell disease, in the steady state and during vasoocclusive crisis. In comparison with 40 healthy controls matched for age and sex, there was a significant decrease in protein C activity in the patients, although no difference was found between protein C levels in the steady state and during crisis.     

Mortality of children with sickle cell disease in a pediatric department in Central Africa

BACKGROUND: Sickle cell disease is a serious public health problem in Gabon with a relatively high mortality rate. PATIENTS AND METHODS: Charts of 23 children (nine boys, 14 girls) who died of complications from sickle cell anemia in the department of pediatrics of Owendo Pediatric Hospital (Libreville, Gabon), from January 1, 1990 through December 31, 1992, were analysed retrospectively. RESULTS: Approximately two-thirds of the children (60.9%) were under 5 years of age. The great majority of patients were from low socio-economic standard families. Of 319 deaths observed during the study period, 23 were due to sickle cell disease-associated complications, for an overall mortality rate of 7.2% and a related mortality of 3.6%. Commonest causes of deaths were severe anemia (11 cases, i.e., 47.8%), which affected predominantly the younger patients between 6 months and 5 years (eight cases), infections (30.4%) and blood transfusion complications (21.7%). CONCLUSION: To decrease these mortality rates, appropriate health supervision and well-designed preventive strategies are needed.     

Reversible splenic hypofunction in hypertransfused children with homozygous sickle cell disease

Functional hyposplenism, as documented by technetium 99 metastable sulfur colloid spleen scan and increased pocked erythrocyte count (also known as a pit count), is well described in children under 2 years of age with homozygous sickle cell anemia. We evaluated the clinical course and splenic function of 16 patients with sickle cell anemia (ages 3 to 20 years) on a hypertransfusion program for more than 6 months following a cerebrovascular accident. Patients were followed with simultaneous spleen scan and pitted erythrocyte count using direct interference contrast microscopy. Pit counts were taken prior to each transfusion and hemoglobin S level maintained at less than 20%. With the exception of two patients, splenic function was recovered only in those patients who were younger than 10 years of age at the time transfusion was initiated. There were no serious bacterial infections or other complications of sickle cell anemia documented in the hypertransfused group. Based on our results and the literature review, we conclude that some patients with sickle cell anemia receiving intensive hypertransfusion therapy for a cerebrovascular accident recover a normal splenic phagocytic function. Age and level at which the hemoglobin S is maintained are important factors in reestablishing splenic phagocytic function.     

Depiction of pain in the self-drawings of children with sickle cell disease

In an attempt to explore the perception of pain in children, 30 children with sickle cell disease were asked to make two drawings; one of themselves and one of themselves in pain. It was hypothesized that the cognitive ability and the emotional state of the child would be affected by the pain experience. Children and their parents were interviewed on the incidence of pain and on the child's control over it. The cognitive ability of the child was related to the kind of control he/she exercised over the pain. An analysis of the drawing concerning their thematic representations, colour and size is also presented. The mental age of the child dropped in the drawing of pain as compared to the non-pain drawing but it was found to be irrelevant to the kind of control the child exercised on the pain.     

Adjustment of children of depressed mothers: A 10-month follow-up.

There is considerable concern about the adjustment of children of depressed mothers. Earlier, the specificity of these difficulties to maternal depression was studied. Child adjustment difficulties were evident in 7- to 13-yr-old children of both depressed and nondepressed psychiatric patient mothers but not in the children of community mothers or nondepressed medical patient mothers (C. M. Lee and I. H. Gotlib, 1989). This report describes a 10-mo follow-up of 44 of these Ss. Despite the alleviation of maternal depressive symptomatology, children in the 2 psychiatric groups continued to manifest adjustment difficulties that were evident to both clinicians and mothers. These findings suggest that the adjustment difficulties found in the children of psychologically distressed mothers do not abate within the 1st yr after the mothers' recovery. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Psychological adjustment of children with mild and moderately severe asthma

In chronic hepatitis B virus (HBV) infection seroconversion from hepatitis B e antigen (HBeAg) to hepatitis B e antibody (HBeAb) may be followed either by remission of the disease with low-level viraemia, or by continuing inflammation with high-level viraemia. In both situations the virus may acquire a mutation in the precore sequence which prevents it from encoding HBeAg. We now show that the number of amino acid substitutions in the HBV core is low in viral sequences from patients with HBeAg positive chronic liver disease and HBeAg negative HBeAb positive patients in remission, but the frequency of substitutions is high in HBeAg, negative HBeAb positive patients with active liver disease. Furthermore we show that these substitutions cluster in the promiscuous CD4+ T-helper-cell epitope and in HBV core/e antibody binding determinants, but are not found in regions recognized by major histocompatability complex (MHC) restricted cytotoxic T lymphocytes. Sequential viral sequences from patients before and after HBeAg/HbeAb seroconversion shows that core mutations arise either at the same time or after the precore stop mutation which prevents the virus from encoding HBeAg. These results are consistent with the hypothesis that after clearance of HBeAg, mutations in regions of the virus recognized by CD4+ helper T cells and B cells allow persistence of the HBe negative virus in HBeAb positive patients with viraemia and active hepatitis.     

Stability and change in retrospective reports of childhood experiences over a 5-year period: Findings from the Davis Longitudinal Study.

The paths via which childhood experiences influence well-being in adulthood are not well defined because most research relies on retrospective reports. This study examined the influence of demographic characteristics and current mood states on the reliability of reports of childhood experiences. The Child Experiences Scale (CES) was administered in 1996 and 2001 to participants in the Davis Longitudinal Study (N = 571; age range 22–61 years). Responses showed moderate to high cross-time reliability. Males were slightly more likely to change their responses. The influence of mood states was weak and more evident for global ratings of childhood than for specific experiences. These findings support the use of retrospective reports of childhood. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Sickle cell disease pain in children and adolescents: change in pain frequency and coping strategies over time

Examined 9-month follow-up data obtained from children and adolescents with sickle cell disease (SCD) and their parents participating in a longitudinal study of pain coping strategies. Of 87 subjects completing the baseline assessment of pain coping strategies, 70 (80%) of their parents completed a structured pain interview assessing their child's health care use and activity reduction during painful episodes over the follow-up period. Regression analyses controlling for age and pain frequency revealed that baseline Coping Attempts were associated with higher levels of school, household, and social activity during painful episodes. Baseline Passive Adherence was associated with more frequent health care contacts during the subsequent 9 months. Increases in Negative Thinking over time were associated with further increases in health care contacts during the follow-up period. Comparing pain coping strategies assessed at baseline to pain coping strategies measured at follow-up revealed that pain coping strategies were relatively stable over time for younger children but changed more for adolescents.     

Encephaloduroarterio-synangiosis in a child with sickle cell anemia and moyamoya disease

We report a black girl with sickle cell anemia. On prophylactic exchange transfusion protocol, she experienced cerebrovascular accidents at 3 and 3.5 years of age, both associated with transient right hemiparesis. At 7.5 years of age, she presented with a partial motor seizure and a left hemiparesis. A cerebral angiogram demonstrated stenosis at the origins of both middle and anterior cerebral arteries bilaterally with extensive basal collateralization. She underwent uncomplicated bilateral encephaloduroarteriosynangiosis (EDAS) procedures using both superficial temporal arteries. At age 9 years, the patient presented with a severe headache and tunnel vision secondary to a stenosis of both posterior cerebral arteries. She underwent bilateral EDAS procedures using both occipital arteries. No complication was encountered. Postoperative cerebral angiogram demonstrated impressive neovascularity at the sites of all four EDAS procedures. Different treatment options of moyamoya disease are discussed.     

Psychological symptoms in methadone maintenance patients: Prevalence and change over treatment.

Methadone maintenance patients in three cities were interviewed twice using the Addiction Severity Index. Of the subjects followed, 35.4% reported having experienced recent psychological symptoms. No relation was found between the length of time in treatment at the first interview and psychiatric severity. In addition, over a 1-year period, the treatment that subjects received from the methadone programs had no impact on psychological symptoms. (PsycINFO Database Record (c) 2010 APA, all rights reserved)