Parenting Stress in Childhood Leukaemia

Parenting stress is the stress level experienced within the role of a parent (HoekstraWeebers et al. 1998). The source of stressors is variable and dependent on the phase of disease and chemotherapy (Sawyer et al. 2000). Failure to cope with these stressors may in turn affect the child’s emotional and social adjustment towards the diagnosis of cancer in addition to poor medical treatment adherence behaviour(Sawyer et al. 1993). The objectives of this study are to determine the level of parenting stress, the risk factors contributing to high parenting stress, and the coping mechanisms used to handle the stress. This single centred, cross-sectional study was done amongst 117 parents at the Paediatric Haematology and Oncology Unit, Universiti Kebangsaan Malaysia Medical Centre (UKMMC) over two years duration. Self-administered questionnaires comprising the Parenting Stress Index/Short Form (PSI/SF) and Coping Inventory for Stressful Situation (CISS) were distributed to parents of children who were 12 years old and below. The mean total parenting stress score amongst parents of children diagnosed with acute leukaemia was 91.5±21.1(95%CI). A total of 27.3% of parents experienced a high total parenting stress score(defined as total PSI score ≥ 75th centile, ie ≥ 103). Task-oriented coping mechanism was used by the majority of parents. Emotion-oriented coping mechanism was the only identifiable risk factor for high parenting stress score following multiple logistic regression analysis. A parent who used emotion-oriented coping mechanism was 7.1 times (95% Confidence Interval 1.2 to 41.4) more likely to have a high parenting stress score compared to a parent who used other coping mechanisms. By identifying these at-risk parents, appropriate counselling and psychological support may be offered early to alleviate the stress as well as assist in the coping and adjustment mechanisms of these parents.

Minimal Residual Disease Status in Childhood Acute Lymphoblastic Leukaemias by Flow Cytometry and Their Clinical and Haematological Features

Residual disease in patients with acute leukaemia indicates unfavorable prognosis. The evaluation of remission using flow cytometry allows a better estimation of minimal residual disease (MRD) after induction chemotherapy in childhood acute lymphoblastic leukaemia (ALL) cases. Patients in morphological marrow remission with presence of blast cells of less than 5%, may still have up to 1010 leukaemic cells. However with flow cytometric analysis, lower levels of the residual leukaemic cells (1 in 104 cells) can be detected and it can be used as a tool to predict relapse. This study compared the presenting clinical and haematological features of children with ALL and their residual disease status determined by flow cytometry. Analysis of their MRD status following remission-induction chemotherapy were done at day-28, week-12 and week-20. The cases were also followed up to five years, to determine their survival status. Their residual disease status by flow cytometric immunophenotyping was also compared with their bone marrow findings morphologically. Thirty-eight cases of precursor B-ALL in pediatric patients from UKM Medical Centre (UKMMC) were analyzed. There was no significant correlation between demographic, clinical and haematological features with MRD status at day-28. However, there was a significant correlation between MRD status by flow cytometry and by morphological marrow examination at week-12. Three cases showed persistent MRD findings until week-20 where two of the cases relapsed and died subsequently. Twenty four patients were still alive after five years of follow up.

Growth velocity in transfusion dependent prepubertal thalassemia patients: results from a thalassemia center in Malaysia.

Growth impairment is commonly seen in children with thalassemia despite regular blood transfusions and desferrioxamine treatments. We investigated the growth velocity of 26 prepubertal patients with beta-thalassemia or HbE-beta thalassemia who were transfusion dependent aged between 2 and 13 years. The prevalence of impaired growth velocity (ie, growth velocity less than the third percentile) amongst the transfusion dependent prepubertal thalassemics was 57.7% compared to 19.2% in the control group. The mean height velocity of the thalassemics was 11.1% less than controls but this difference was not statistically significant (4.23cm/year vs 4.76cm/year, p = 0.08). The mean serum ferritin level of the thalassemics with a height < 3rd percentile was higher compared to those with a height > 3rd percentile (4,567.0 vs 2,271.0, p = 0.01). Our study showed that there was a high prevalence of impaired growth velocity amongst our transfusion dependent prepubertal thalassemics. This highlights the problem of inadequate chelation therapy, and compliance with chelation therapy amongst our patients. This study emphasizes the importance of monitoring growth parameters and optimal iron chelation therapy in these patients.

Prolonged remission after splenectomy for refractory Evans syndrome--a case report and literature review.

We describe a patient with Evans syndrome (autoimmune hemolytic anemia and autoimmune thrombocytopenia) who was refractory to steroids and intravenous immunoglobulin. She responded to splenectomy and has remained in clinical remission for 3 years. In the majority of cases, splenectomy rarely induces a durable remission but it may be beneficial in a small group of patients, hence should be considered as alternative therapy in the management of these patients.

Case report post-splenectomy sepsis in thalassemic patients.

Thalassemia is the commonest hemoglobinopathy in Malaysia. Patients with thalassemia major are transfusion dependent, and a large proportion of them will require splenectomy. As this particular group of patients is immunocompromized, overwhelming sepsis is a recognized complication. We report a series of three patients who all developed intra-abdominal abscesses following splenectomy.

Abnormalities in lung function among multiply-transfused thalassemia patients: results from a thalassemia center in Malaysia.

The aim of this study was to: (1) determine the prevalence and patterns of lung dysfunction among transfusion dependent thalassemics; (2) determine the associated factors that might contribute to this problem. This was a cross-sectional study involving 66 patients with transfusion dependent thalassemia aged 10 years and above. All patients underwent physical examination, standardized pulmonary function tests including spirometry, lung volume, and the carbon monoxide diffusion capacity. A restrictive pattern of lung dysfunction was observed in 22 patients (33.3%) and none showed the presence of obstructive ventilatory impairment. A reduction in the carbon monoxide diffusion capacity (DLCO) was seen 87.9% of the patients, including 7.6% who had evidence of hypoxemia. Ten patients showed a reduction in the FEF25-75% although they did not fulfil the criteria for small airway disease. No correlation was found between lung dysfunction and serum ferritin levels in the patients. Restrictive lung dysfunction and diffusion impairment were the predominant abnormalities found in our cohort of patients.

Short stature and truncal shortening in transfusion dependent thalassemia patients: results from a thalassemia center in Malaysia.

One of the major complications in patients with transfusion dependent thalassemia is growth impairment secondary to iron overload. We studied the growth status in 66 patients with beta-thalassemia major and HbE-beta thalassemia who were transfusion dependent, aged from 2 to 24 years, and 66 controls matched for sex and age. The prevalence of short stature in transfusion-dependent thalassemics was 54.5% compared to 4.5% in control group (p<0.001). Short stature was more prevalent in those above the age of 10 years in this study group (83.3% vs 16.7%). Transfusion dependent thalassemics with short stature were found to have significantly lower mean standing height standard deviation scores (SDS), sitting height SDS and subischial leg length SDS values (p<0.001). There was also a significant difference between the mean sitting height SDS and the mean subischial leg length SDS in our thalassemics with short stature, suggesting that the short stature was due to disproportionate truncal shortening. Serum ferritin levels were significantly higher in transfusion dependent thalassemics who were short compared to those who were of normal height (p = 0.002). However, the mean pre-transfusion hemoglobin levels did not differ significantly between patients with short stature and those with normal height (p = 0.216). The prevalence of short stature also did not differ significantly between those with beta-thalassemia major and those with HbE-beta thalassemia (p = 0.32). This study highlighted the importance of providing optimal treatment in these patients, including monitoring of growth parameters and optimizing iron chelation therapy.