Sickle cell disease is caused by a mutation in DNA and refers to several different disease processes that are inherited from parents with one or more type of sickle cell trait.1 This mutation affects the way the hemoglobin cells react during oxygen delivery to different parts of the body. In cells that have sickle hemoglobin after oxygen delivery, the cells stick together or are bent, causing them to break apart and potentially block the blood vessel.1 Although there are many different types of sickle cell diseases, they all have similar complications including fever and increased risk of infection, splenic sequestration, acute chest syndrome, pain crises, severe anemia, and stroke.1 Sickle cell disease occurs in one in every 365 black or African-American births and one in every 16,300 Hispanic American births.2 An estimated 300,000 babies are born with sickle cell disease worldwide each year, only 1000 of whom are born in the United States.1,3 In African countries, 10% to 40% of individuals have a sickle cell trait, and approximately 2% of these individuals will develop sickle cell disease. Individuals of almost every background are affected by sickle cell disease.1 Sickle cell disease most commonly occurs in people whose ancestors originate from sub-Saharan Africa, South America, the Caribbean, Central America, Saudi Arabia, India, Turkey, Greece, and Italy.2 The only cure for sickle cell disease is a bone marrow stem cell transplant.1
Pica is a psychological eating disorder that is characterized by the consumption of items that contain no nutritional value.4 Pica in patients with sickle cell disease is documented as a departure from normal development.4 One study performed in the United States found that of 395 study participants with sickle cell disease, 33% had pica behaviors, while another study performed in Belgium found that of 55 study participants with sickle cell disease, 56% had pica behaviors.4,5 This consumption of items in individuals with pica is described as unusual, persistent, and compulsive in nature.5 Most commonly, items ingested during pica behaviors are ice cubes, clay, dried pasta, chalk, starch, paste, cigarette butts, hair, and laundry detergent.6 It is also common for patients with sickle cell disease to ingest paper, fabric, foam (Styrofoam or cushion foam), and dirt.4
Pica is a normal finding in some developmental stages, as this is how many babies and toddlers under the age of 18 months examine the world around them.7 Pica is also found frequently in individuals with developmental disabilities.7 These two populations seem to exist as an exception, as pica in other groups is considered to be a psychological disorder or an indication of a medical condition.4 It is difficult to determine the prevalence of pica due to differences in definitions as well as the stigma associated with the condition that cause it to be under-reported.7
The ingestion of items that are not food causes complications that can be harmful or even fatal. Some of these complications include electrolyte imbalance, metabolic disorders, dental injury, intestinal obstruction or perforation, choking, infection, and poisoning.4-8 Due to the severity of these complications, the goal of any treatment is the reduction of pica behaviors to zero occurrences.8 However, there is no definite treatment for the condition.5
The etiology of pica in patients who have sickle cell disease is not well understood.5 In addition to findings showing that one-third of patients with sickle cell disease develop pica, sickle cell patients who have a history of stroke complications have an even higher prevalence of pica behaviors.4 Parents of children and adolescents with sickle cell disease have found that pica is so frequent that it interferes with the patients’ daily living activities.5 Patients with sickle cell disease and pica were also found to have additional dysfunctional eating patterns.9 Reports indicate that there should be routine screening for pica and dysfunctional eating patterns in children with sickle cell disease.10 Protocols need to be developed and implemented to prevent or discontinue pica behaviors in patients with sickle cell disease.
A documented condition that results in pica is iron deficiency.11 Iron deficiency also occurs in patients with sickle cell disease, especially young patients.12 Patients who experience pica and have sickle cell disease were found to have significantly lower hemoglobin levels compared to those without pica behaviors.5 Patients who receive red cell blood transfusions are usually protected from having low hemoglobin levels, but those who do not receive regular transfusions do not have the same protection.12 In patients with iron deficiency, the most common item consumed during pica behaviors is ice.11 While ice is not a source of iron, it is hypothesized that ice causes a vasoconstrictive response that can result in greater brain perfusion, which can lead to increased alertness and processing speed.11 Patients with iron deficiency often experience sluggishness because of effects resulting in decreased oxygen delivery to the brain.11
Most patients with sickle cell disease will face complications at some point in their lifetime.1 The life expectancy for patients with sickle cell disease is approximately 45 years, compared with 79 years in the general population.1 It is important that providers have an understanding of interventions used for treating pica in pediatric patients with sickle cell disease. Implementing interventions as early as possible can help increase patient life expectancy and quality of life.1 These interventions can be any action taken to prevent or discourage pica behaviors and may include behavioral (managing family stress and learned dysfunctional eating patterns including family history of pica), biological (managing disease severity and nutritional deficits), and environmental interventions (addressing food insecurity and access to ingested items).13 It is equally important for providers to understand the outcomes associated with the interventions used in this patient population.
The purpose of this scoping review is to explore existing literature regarding pica in pediatric patients with sickle cell disease and to identify associated interventions and outcomes. A scoping review was selected for this protocol due to limited research on pica in the sickle cell population. The majority of pica intervention research has been focused on individuals who have developmental disabilities.13 A search in March 2019 of the JBI Database of Systematic Reviews and Implementation Reports, Cochrane Database of Systematic Reviews, MEDLINE, Campbell Systematic Reviews, and PROSPERO revealed no current systematic or scoping reviews on this topic.
What interventions have been used for treating pica in pediatric patients with sickle cell disease?
What outcomes have been measured following interventions for treating pica in pediatric patients with sickle cell disease?
This scoping review will consider studies that include pediatric patients from 18 months to 18 years of age who have been diagnosed with any type of sickle cell disease and who have a history of pica. This study will exclude patients younger than 18 months of age because prior to this age, pica is considered a normal behavior.5 Pediatric patients with sickle cell disease, a history of pica diagnosis, and a diagnosis of developmental disability will be excluded from this review because pica is a common finding in patients with developmental disability, and the interventions and outcomes may differ for this population.8
The scoping review will focus on interventions used to treat pica in pediatric patients with sickle cell disease and the outcomes associated with these interventions. Interventions are defined as any actions taken to prevent or discourage pica behaviors and may include, but will not be limited to, behavioral (managing family stress and learned dysfunctional eating patterns including family history of pica), biological (managing disease severity and nutritional deficits), and environmental interventions (addressing food insecurity and availability to ingested items).13 Outcomes may include, but will not be limited to, reduction in pica behaviors and reduction in adverse medical events related to pica.
This scoping review will consider patients with sickle cell disease and pica in any treatment setting in any geographical location.
Types of sources
This review will consider both experimental and quasi-experimental study designs including randomized and non-randomized controlled trials, before and after studies, and interrupted time-series studies. In addition, analytical observational studies including prospective and retrospective cohort studies, case-control studies, and analytical cross-sectional studies will be considered for inclusion. This review will also consider descriptive observational study designs including case series, individual case reports, and descriptive cross-sectional studies.
Qualitative studies that focus on qualitative data including, but not limited to, designs such as phenomenology, grounded theory, ethnography, qualitative description, action research, and feminist research will also be considered. Text and opinion papers will also be considered for inclusion in this scoping review.
The JBI methodology will be used for this scoping review.14
The search strategy will aim to find both published and unpublished studies. An initial limited search of MEDLINE (PubMed) and CINAHL (EBSCO) will be undertaken to identify articles on the topic, followed by an analysis of the text words contained in the title and abstract, and of the index terms used to describe these articles. This will inform the development of a search strategy including identified key words and index terms, which will be tailored for each information source. A proposed search strategy for PubMed is detailed in Appendix I. The reference lists of all included studies will be screened for additional studies. Studies published in English will be included. Due to limited research on pica in the sickle cell population, all studies will be considered regardless of publication date.
The databases to be searched include CINAHL (EBSCO), Embase (Elsevier), Europe PubMed Central (PubMed), Psychology and Behavioral Sciences Collection (EBSCO), and Scopus (Elsevier).
The search for unpublished studies will include ProQuest Dissertations and Theses (ProQuest) and MedNar (Mednar.com).
Following the search, all identified citations will be collated and uploaded into EndNote V.8 (Clarivate Analytics, PA, USA) and duplicates removed. Titles and abstracts will then be screened by two independent reviewers for assessment against the inclusion criteria for the review. Studies that may meet the inclusion criteria will be retrieved in full and their details imported into JBI System for the Unified Management, Assessment, and Review of Information (JBI SUMARI; JBI, Adelaide, Australia). The full text of selected studies will be retrieved and assessed in detail against the inclusion criteria. Full-text studies that do not meet the inclusion criteria will be excluded, and reasons for exclusion will be provided in an appendix in the final systematic review report. The results of the search will be reported in full in the final report and presented in a PRISMA flow diagram.15 Any disagreements that arise between the reviewers will be resolved through discussion or with a third reviewer.
Data will be extracted from papers included in the scoping review by two independent reviewers using the draft data extraction tool presented in Appendix II. The data extracted will include specific details about the populations, concept, context, and study methods of significance to the scoping review questions and specific objective. Any disagreements that arise between the reviewers will be resolved through discussion or with a third reviewer. Authors of papers will be contacted to request missing or additional data where required. The draft data extraction tool will be modified and revised as necessary during the process of extracting data from each included study. Modifications will be detailed in the full scoping review report.
The extracted data will be presented in diagrammatic or tabular form in a manner that aligns to the objectives and scope of this scoping review. The tables and charts will report on: pica interventions and outcomes, distribution of studies by year or period of publication, countries of origin, and research methods. A qualitative thematic analysis will be undertaken to provide an overview of the literature. A narrative summary will accompany the tabulated and/or charted results and will describe how the results relate to the review objective and questions. The findings will be discussed as they relate to practice and research.
The University of Mississippi Medical Center School of Nursing for its guidance.
This protocol will contribute towards a Doctor of Nursing Practice (DNP) degree for OMC.
Appendix I: Search strategy
- 1. Pediatric OR Adolescen∗ OR Youth OR Child OR children OR “Child”[Mesh] OR “Adolescent”[Mesh]
- 2. “Sickle Cell Disease” OR “Sickle Cell Anemia” OR “Anemia, Sickle Cell”[Mesh]
- 3. Pica OR “Pica”[Mesh] OR Allotriophagy OR Geo
- 4. 1 AND 2 AND 3
Appendix II: Data extraction table
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2. Centers for Disease Control and Prevention. Data & statistics on sickle cell disease
[Internet]. 2019 [cited 2019 Mar 17]. Available from: https://www.cdc.gov/ncbddd/sicklecell/data.html
3. American Society of Hematology. State of sickle cell disease
: 2016 report [Internet]. 2016 [cited 2020 Feb 26]. Available from: http://www.scdcoalition.org/pdfs/ASH%20State%20of%20Sickle%20Cell%20Disease%202016%20Report.pdf
4. Ivascu N, Sarnaik S, McCrae J, Whitten-Shurney W, Thomas R, Bond S. Characterization of pica
prevalence among patients with sickle cell disease
. Arch Pediatr Adolesc Med
2001; 155 (11):1243–1247.
5. Aloni M, Lecerf P, Lê P, Heijmans C, Huybrechts S, Devalck C, et al. Is pica
under-reported in children with sickle cell disease
? A pilot study in a Belgian cohort. Hematology
2014; 20 (7):429–432.
6. Khan Y, Tisman G. Pica
in iron deficiency: a case series. J Med Case Rep
2010; 4 (1):1–3.
7. Call N, Simmons C, Mevers J, Alvarez J. Clinical outcomes of behavioral treatments for pica
in children with developmental disabilities. J Autism Dev Disord
2015; 45 (7):2105–2114.
8. Bay A, Dogan M, Bulan K, Kaba S, Demir N, Öner A. A study on the effects of pica
and iron-deficiency anemia on oxidative stress, antioxidant capacity and trace elements. Hum Exp Toxicol
2013; 32 (9):895–903.
9. Lemanek K, Brown R, Armstrong F, Hood C, Pegelow C, Woods G. Dysfunctional eating patterns and symptoms of pica
in children and adolescents with sickle cell disease
. Clin Pediatr
2002; 41 (7):493–500.
10. O’Callaghan E, Gold J. Pica
in children with sickle cell disease
: two case reports. J Pediatr Nurs
2012; 27 (6):e65–e70.
11. Rabel A, Leitman S, Miller J. Ask about ice, then consider iron. J Am Assoc Nurse Pract
2016; 28 (2):116–120.
12. Rao K. Iron deficiency and sickle cell anemia. Arch Intern Med
1983; 143 (5):1030.
13. Rodrigues N, Shih S, Cohen LL. Pica
in pediatric sickle cell disease
. J Clin Psychol Med Settings. 2019:10.1007/s10880-019-09671-x.
14. Peters MDJ, Godfrey C, McInerney P, Baldini Soares C, Khalil H, Parker D. Chapter 11: scoping reviews. In: Aromataris E, Munn Z (Editors). JBI Reviewer's Manual [Internet]. Adelaide: JBI; 2017 [cited 2020 Feb 26]. Available from: https://reviewersmanual.joannabriggs.org/
15. Moher D, Liberati A, Tetzlaff J, Altman DG. the PRISMA Group. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. Ann Intern Med
2018; 169 (7):467–473.