Study backgroundThalassemia is the commonest genetic blood disorder in Malaysia which requires life-long blood transfusions. From a total of 7,984 thalassemia patients in Malaysia, adolescent age group account for the highest number of patients (2,680 patients, 33.57%). In developed countries, the average rate of adherence to long-term treatment among children and adolescents is only 58%. Sub-optimal adherence to iron chelation therapy may impact the outcome and quality of life in these patients. Thus, assessing adherence level and identification of risk factors for non-adherence is essential in optimizing management.ObjectivesTo determine the association between mean serum ferritin level with self-reported level of adherence to iron chelation therapy in transfusion dependent thalassemia (TDT) adolescents in Hospital Tengku Ampuan Afzan (HTAA), Kuantan and Pusat Perubatan Universiti Kebangsaan Malaysia (PPUKM), Cheras; to determine the association between socio-demographic factors and patients’ knowledge on thalassemia and iron chelation therapy with the level of adherence.Materials and methodsThis was a cross-sectional study conducted between 1st March 2019 and 31st March 2020. Data was collected through face-to-face interview by a single interviewer during the thalassemia clinic follow up, with content validated questionnaires. The questionnaires comprised four sections which included socio-demographic data, medication adherence questionnaire, knowledge of disease, and clinical characteristics of the participants.ResultsA total of 70 participants were recruited. Results showed that only 51.4% of participants had good adherence to iron chelation therapy. There was a significant association between monthly household incomes of the family with the level of adherence to iron chelation (p-value 0.006). There was also an association between the mean serum ferritin levels with total Adherence Starts with Knowledge (ASK-12) score (p-value 0.001). However, there was no association between knowledge on thalassemia with the level of adherence.ConclusionAdherence to iron chelation was generally unsatisfactory amongst adolescents with TDT as only 51.4% had good adherence. Low monthly household income of the family may affect adherence to iron chelation therapy in TDT patients. As adherence remains to be an issue amongst adolescent thalassemia patients, management should include regular and objective assessments to address this problem so as to optimize patient outcome.

Introduction: In Malaysia, a course of vaccination DTaP/IPV/Hib was introduced in 2008, replacing the 2006 DwPT-HBV/Hib+OPV vaccines. Severe systemic adverse reactions after diphtheria, tetanus and pertussis vaccination are uncommon. Cardiac complications are rarely reported and is most probably implicated to the pertussis component. We describe a rare case of acute myocarditis that developed 60 hours after DTaP/IPV/Hib vaccination. Case report: A 2-month old infant presented to emergency department after her first diphtheria, tetanus and pertussis vaccination due to severe respiratory distress and cyanosis. She had her BCG and two Hepatitis B vaccinations previously with no major side effects. Parents reported that she was feverish for 48 hours post vaccination with no other associated symptoms. Prior to presentation, she went floppy and was immediately brought to hospital. On arrival, she was tachypnoeic and cyanotic with hypoperfusion and hypotensive. She was also noted to have hepatomegaly. She was grunting and her level of consciousness deteriorated. She was immediately intubated and her first blood gas showed profound metabolic acidosis with pH 6.6, base excess -24mmol/L, lactate 14mmol/L and bicarbonate 4mmol/L. She required fluid boluses and inotrope infusion was commenced. She received antibiotics and sodium bicarbonate to correct her acidosis. Her echocardiography showed global hypokinesia, CK 3018 and positive Troponin. She was treated with immunoglobulin for myocarditis and on high frequency oscillation for 4 days before being extubated on day 11 of admission. All her viral serology and cultures came back negative. Discussion: Cardiac complications after diphtheria, tetanus and pertussis and other vaccinations are exceptionally uncommon. This patient developed sudden onset cardiogenic deterioration after an expected fever-like illness post vaccination. Given her viral screening and cultures were negative, this make acute myocarditis post vaccination a remote possibility. We concur that evaluation of cardiac state should be considered in recently vaccinated infants who manifest with cyanosis, hypoperfusion and drowsiness.