Sickle cell disease (SCD) is an autosomal recessive disorder characterized by production of abnormal hemoglobin S and is associated with high morbidity and mortality. Information about the prevalence ...of SCD in Saudi Arabia is patchy and probably underestimated, but studies have reported that SCD is a relatively common genetic disorder in this part of the world. The prevalence of SCD in Saudi Arabia varies significantly in different parts of the country, with the highest prevalence is in the Eastern province, followed by the southwestern provinces. The reported prevalence for sickle-cell trait ranges from 2% to 27%, and up to 2.6% will have SCD in some areas. Clinical and hematological variability exists in SCD in Saudi Arabia with two major phenotypes: a mild phenotype and a severe phenotype. Further studies on the prevalence, molecular and clinical epidemiology of SCD may help predict disease severity and risk stratification of patients to determine whether to receive early intensive care or continued symptomatic care.
Introduction
The development of cancer programs in developing countries to meet the standards observed in high‐income developed countries is not well documented.
Methods
An analysis of patient care ...of children with acute lymphoblastic leukemia (ALL) at the Princess Noorah Oncology Center over 25 years was performed. A number of improvements were introduced over time including optimizing the cancer care delivery culture, improving access to care, optimizing supportive care, and refining diagnostic and therapeutic capabilities. Outcomes were evaluated over three time periods (period 1, 1989–2000; period 2, 2001–2007; and period 3, 2008–2014). These findings were compared with results of collaborative clinical trials (CCT).
Results
Of the 686 patients treated, the five‐year overall survival (OS) rate improved from 74% ± 2.7% in period 1 to 89.5% ± 2.3% in period 3 (P < 0.0001). Among all patients, the five‐year cumulative incidence of death decreased from 26% in period 1 to 10.5% in period 3 (P < 0.0001). This decrease was mainly due to reduction in the incidence of death from disease (P < 0.00001). In contrast, significant improvement in T‐cell ALL survival outcomes (P < 0.0001) was observed overtime (from period 1 to period 3) as a result of significant reduction in both toxic deaths (P = 0.005) and disease‐related deaths (P = 0.001).
Conclusions
Survival outcomes in Saudi Arabia have improved with five‐year OS rate now approaching 90%. However, further population‐based research is needed to understand variance in outcomes from those reported by CCT groups.
Sickle cell disease (SCD) is a genetic disorder, characterized by hemolytic anemia and vaso‐occlusive crises (VOCs). Data on the global SCD impact on quality of life (QoL) from the patient viewpoint ...are limited. The international Sickle Cell World Assessment Survey (SWAY) aimed to provide insights into patient‐reported impact of SCD on QoL. This cross‐sectional survey of SCD patients enrolled by healthcare professionals and advocacy groups assessed disease impact on daily life, education and work, symptoms, treatment goals, and disease management. Opinions were captured using a Likert scale of 1‐7 for some questions; 5‐7 indicated “high severity/impact.” Two thousand one hundred and forty five patients (mean age 24.7 years standard deviation (SD) = 13.1, 39% ≤18 years, 52% female) were surveyed from 16 countries (six geographical regions). A substantial proportion of patients reported that SCD caused a high negative impact on emotions (60%) and school achievement (51%) and a reduction in work hours (53%). A mean of 5.3 VOCs (SD = 6.8) was reported over the 12 months prior to survey (median 3.0 interquartile range 2.0‐6.0); 24% were managed at home and 76% required healthcare services. Other than VOCs, fatigue was the most commonly reported symptom in the month before survey (65%), graded “high severity” by 67% of patients. Depression and anxiety were reported by 39% and 38% of patients, respectively. The most common patient treatment goal was improving QoL (55%). Findings from SWAY reaffirm that SCD confers a significant burden on patients, epitomized by the high impact on patientsʼ QoL and emotional wellbeing, and the high prevalence of self‐reported VOCs and other symptoms.
Background
Childhood cancer is a highly curable disease when timely diagnosis and appropriate therapy are provided. A negative impact of the coronavirus disease 2019 (COVID‐19) pandemic on access to ...care for children with cancer is likely but has not been evaluated.
METHODS
A 34‐item survey focusing on barriers to pediatric oncology management during the COVID‐19 pandemic was distributed to heads of pediatric oncology units within the Pediatric Oncology East and Mediterranean (POEM) collaborative group, from the Middle East, North Africa, and West Asia. Responses were collected on April 11 through 22, 2020. Corresponding rates of proven COVID‐19 cases and deaths were retrieved from the World Health Organization database.
Results
In total, 34 centers from 19 countries participated. Almost all centers applied guidelines to optimize resource utilization and safety, including delaying off‐treatment visits, rotating and reducing staff, and implementing social distancing, hand hygiene measures, and personal protective equipment use. Essential treatments, including chemotherapy, surgery, and radiation therapy, were delayed in 29% to 44% of centers, and 24% of centers restricted acceptance of new patients. Clinical care delivery was reported as negatively affected in 28% of centers. Greater than 70% of centers reported shortages in blood products, and 47% to 62% reported interruptions in surgery and radiation as well as medication shortages. However, bed availability was affected in <30% of centers, reflecting the low rates of COVID‐19 hospitalizations in the corresponding countries at the time of the survey.
Conclusions
Mechanisms to approach childhood cancer treatment delivery during crises need to be re‐evaluated, because treatment interruptions and delays are expected to affect patient outcomes in this otherwise largely curable disease.
The response to the coronavirus disease 2019 (COVID‐19) pandemic has led to significant alterations in access to care for children with cancer. Interventions are needed to mitigate the effects on life‐threatening diseases requiring immediate and uninterrupted therapy, such as childhood cancer.
Background
There are a limited number of studies that address non-neutropenic fever episodes in children with cancer, and no standard approach exists.
Method
We opt to retrospectively analyze the ...efficacy of the current clinical approach for management of non-neutropenic fever episodes and the associated risk factors among children with cancer at the Princess Noorah Oncology Center from May 2016 through December 2017.
Results
A total of 480 non-neutropenic fever episodes were identified in 131 children, of which 62 episodes were triaged as high-risk non-neutropenic fever and 418 as low-risk non-neutropenic fever. Of those 480 non-neutropenic fever, 361 episodes (75.2%) were associated with the presence of central venous catheters. The overall failure rate of ceftriaxone mono-therapy was observed in 75.6% (11.7% in high-risk non-neutropenic fever with a mean C-reactive protein level of 21.1 (±23.2) mmol/L and 63.9% in low-risk non-neutropenic fever with a mean C-reactive protein level of 17.6 (±53.9) mmol/L). The overall bacteremia rate was 14.4%. The type of organisms isolated was mainly high-risk organisms in 59 non-neutropenic fever episodes (85.5%), OR 1.78 (95% CI: 0.45–7.04) p = 0.41. Of note, all bacteremia were associated with the presence of central venous catheter (100%). Of all the examined risk factors of outpatient treatment failure in low-risk non-neutropenic fever, only prolonged fever of more than three days were significantly associated with bacteremia OR 8.107 95% CI: 1.744–37.691, p = 0.008. Noteworthy is that almost 43% of non-neutropenic fever episodes were associated with respiratory symptoms. This study provides a baseline for future prospective research assessing the pattern of non-neutropenic fever by focusing on associated risk factors.
The prevalence of obstructive sleep apnea syndrome (OSAS) is elevated in some high-risk populations. Children with sickle cell disease (SCD) are known to have many comorbidities, including OSAS. The ...objectives of this study were to assess the prevalence of and risk factors for OSAS among children with SCD in two major tertiary health care facilities in Jeddah, Saudi Arabia. This multicenter cross-sectional study took place in two major tertiary health care facilities—King Abdulaziz University Hospital and King Khalid National Guard Hospital, Jeddah, Saudi Arabia. Children with SCD who were admitted between January 2010 and December 2017 were enrolled. The Pediatric Sleep Questionnaire (PSQ) was used to screen for OSAS. Data were collected from 150 children with SCD aged between 2 and 18 years. Eighty-five percent of the children had sickle cell anemia (SCA) with HbSS, and the rest had sickle beta-thalassemia (HbS/ß-thalassemia). Based on the PSQ, 33 of the 150 (22%) children had OSAS (score ≥ 7). The average score on the PSQ was 3.8/22 (± 3.8). A history of adenotonsillar hypertrophy was found to be a significant risk factor in bivariate and multivariate analyses aOR 5.5; 95% CI 1.84–16.35 (
P
< 0.001). The odds of having OSAS were ninefold higher in children who had periodic limb movements than in those who did not after adjustment 95% CI 1.75–48.03 (
P
< 0.001). OSAS is a highly prevalent disease among children with SCD. Many factors were associated with OSAS in the bivariate analysis, including nationality, education level, a history of adenotonsillar hypertrophy, and a history of periodic limb movements.
Objectives
Sickle cell disease (SCD) is an inherited disorder that causes lifelong complications, substantially impacting the physical and emotional well‐being of patients and their caregivers. ...Studies investigating the effects of SCD on quality of life (QOL) are often limited to individual countries, lack SCD‐specific QOL questionnaires, and exclude the caregiver experience. The SHAPE survey aimed to broaden the understanding of the global burden of SCD on patients and their caregivers and to capture the viewpoint of healthcare providers (HCPs).
Methods
A total of 919 patients, 207 caregivers, and 219 HCPs from 10, 9, and 8 countries, respectively, answered a series of closed‐ended questions about their experiences with SCD.
Results
The symptoms most frequently reported by patients were fatigue/tiredness (84%) and pain/vaso‐occlusive crises (71%). Patients' fatigue/tiredness had one of the greatest impacts on both patients' and caregivers' QOL. On average, patients and caregivers reported missing 7.5 days and 5.0 days per month, respectively, of school or work. HCPs reported a need for effective tools to treat fatigue/tiredness and a desire for more support to educate patients on long‐term SCD‐related health risks.
Conclusions
The multifaceted challenges identified using the SHAPE survey highlight the global need to improve both patient and caregiver QOL.
Background
Delayed access to cancer care has been associated with childhood cancer death. Improving timely access to cancer care is the first important step in the cancer treatment journey. We ...introduced an electronic referral system (e‐RS) to improve timely access to cancer care. This study aimed to assess the impact of implementing an e‐RS on timely access to cancer care.
Methods
A retrospective cross‐sectional study of pediatric oncology patients selected through a consecutive nonprobability sampling technique was performed to determine the turnaround time (TAT) of children with cancer diagnosed 12 months before and after implementation of the e‐RS. TAT was defined as time in hours from referral to approval for admission.
Results
Of the 326 pediatric oncology patients diagnosed between January 2014 and December 2015, 59.9% were male and 40.1% were female. Median age for both sexes was 5.0 years (interquartile range IQR: 2.5‐9.0 years). Among these, 98.2% were Saudi nationals. Hematological malignancies accounted for 50.6% of referrals and 16.6% had lymphoma. The median TAT of the manual referral system (m‐RS) and e‐RS was 18 h (IQR: 2‐25 h) and 2 h (IQR: 1‐16 h; P = .0001), median length of hospital stay during first admission was 11 days versus 9 days (P = .14), and death events occurred in 11 patients versus zero patients referred using the m‐RS versus e‐RS (P = .003), respectively.
Conclusion
The introduction of an e‐RS was associated with more rapid processing of pediatric patients for cancer treatment and fewer patient deaths during the initial evaluation and treatment during that time period.
•Incidence of childhood acute lymphoblastic leukemia (cALL) varies between countries.•Incidence of cALL was 2.35 in Saudi Arabia (SA) compared to 4.1 in USA per 100,000.•The percent annual increase ...in the incidence of cALL was 4.58 % in SA vs. 0.7 % in USA.•Improved reporting, and timely access-to-care may influence trends in incidence in SA.
Introduction. The incidence of childhood acute lymphoblastic leukemia (cALL) varies between countries likely as a result of competing risks including infections, access-to-care, socioeconomic influences, and/or ethnicity. However, little is known about disease burden in high-income Arab countries offering free-of-charge healthcare. The hypothesis was that, due to population characteristics (young age, high fertility and parental consanguinity rate), the incidence of cALL in Saudi Arabia is equal or higher than that observed in high-income Western countries.
Methods. Saudi databases were used to calculate the incidence of cALL from 2001 to 2014. Incidence trends over time of children with ALL, 14-years of age or younger, were analyzed and compared with those reported in USA.
Results. The age-adjusted incidence over the years was lower in Saudi Arabia compared to USA. However, the incidence trend of cALL in Saudi Arabia was increasing at a rate higher than that observed in USA (p < 0.001). The overall incidence of cALL in Saudi Arabia increased from 1.58/100,000 in 2001 to 2.35/100,000 population in 2014. The median annual increase was 4.58 %. The incidence in males increased from 1.88 to 2.71/100,000, and from 1.21 to 1.86/100,000 population in females.
Conclusions. The reported incidence of cALL in Saudi Arabia is rapidly increasing. The increasing trend may reflect evolving socioeconomic structure, improved access-to-cancer care, and improved diagnosis/ reporting capacity. This highlights the need for better understanding of cALL causes and the need for the formation of separate national pediatric cancer registries in different countries to monitor childhood cancer incidence trends.
Background
The frequency of pathogenic/likely pathogenic (P/LP) germline mutations in cancer‐related genes among children with cancer in highly consanguineous populations is not well studied.
Methods
...Whole‐exome sequencing of germline DNA was performed in 60 children with acute leukemia. We used the St. Jude Pediatric Cancer Variant Pathogenicity Information Exchange (PeCanPIE) data portal for the classification of germline variants by the St. Jude Medal Ceremony pipeline.
Results
Fifty‐seven patients had acute lymphoblastic leukemia (ALL) and three patients had acute myeloid leukemia. Parental consanguinity was present in 27 (45%) patients. All patients were of Arab ancestry. Three patients (5%) had a history of cancer in their siblings. Five patients (8.3%) had P/LP germline mutations in cancer‐related genes. Three patients with B‐ALL had heterozygous pathogenic mutations in TP53, BRCA1, and BRCA2; one patient with B‐ALL had homozygous pathogenic mutation in PMS2; and one patient with T‐ALL had LP homozygous mutation in AK2 that was associated with reticular dysgenesis. Among patients who had history of parental consanguinity, three (11%) had P/LP germline mutations compared with two (8%) in the absence of parental consanguinity. Fourteen (23%) patients had gold medal variants in cancer‐related genes, 13 were heterozygous, and one was homozygous. Silver medal variants were present in 35 (58%) patients; all were heterozygous except one homozygous.
Conclusions
Children with acute leukemia in Saudi Arabia had low frequency of P/LP mutations in cancer‐related genes despite the high rate of consanguinity. Larger studies using whole‐genome sequencing are needed to further explore the heritability of childhood leukemia.
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