EXPLORING THE ISSUES SURROUNDING SICKLE CELL DISEASE IN NIGERIA

Introduction

Sickle cell disease (SCD) is a chronic condition of general public health significance. SCD is a common hereditary illness which has significant effects on individual’s health, it is a condition that affect the function or structure of haemoglobin in the body (Nnaji, et al., 2013; Ola, et al., 2016). However, children inherit the disease from their biological parents, this happened when both parents have an abnormal haemoglobin SS genes (HbSS) unlike a normal haemoglobin AA (HbAA). The child will become anaemic also known as sickle cell anaemia (SCA) (Nnaji, et al., 2013). Also, a child can be a carrier (sickle cell trait) of the disease when one of his parents is a sufferer (HbSS) and the other has an HbAA gene.

However, HbSS gene is the commonest pathological haemoglobin abnormality globally, and majority of teenagers born with the disease dies before the age of five (Nnaji, et al., 2013). SCD is a life threatening blood disorder with a wide range of potentially severe symptoms; comprises of anaemia, vaso-occlusive pain crises, organ damage, severe fatigue, leg ulcers and psychosocial stressors (Burnes, 2008). Sickle cell treatment involves constant therapeutic consultation from health care professionals to prevent sickle cell crises and other life-threatening infections; avoidance of crises triggered situations were advised by health professionals. Sickle cell symptoms begin to manifest at early childhood and the mortality rate start to arise at childhood (Raji, 2016; Burnes, 2008). Therefore, sickness management requires a serious interference in the lives of the sufferers and their families (Burnes, 2008; Nnaji, et al., 2013; Raji, 2016).  Hence, pre-marital screening for SCD is an excellent strategy for the control of the disease.

Globally, more than 330,000 infants are born with SCD every year, and these figures were estimated to increase to 400,000 by 2050 (Ola, et al., 2016). The world health organisation (WHO) has recognised SCD as a public health priority in Africa (Ola, et al., 2016; Nnaji, et al., 2013). However, two-thirds of the world’s annual sickle cell cases (330,000 new cases) are found in Africa with Nigeria taking the least half of the cases. In Nigeria, over 150,000 infants are born with SCD each year and the country is said to be among the countries with the highest SCD prevalence (Raji, 2016; Ola, et al., 2016).

Even though SCD is mostly common among individuals of African, Mediterranean, Arabian and Asian background, the disease has become a universal health issues due to migration (Raji, 2016). Nonetheless, the prevalence of SCD in Nigeria is 20 per 1000 live births. According to Nnaji et al (2013), high school students are said to have poor knowledge of SCD compared to those with higher qualifications. Furthermore, official professional genetic counselling is not common particularly for people without affected pro-band. In Turkey the occurrence rate of HbAS is 4.6% (in one spouse with SCT) and 2.6% for both spouses with HbAS. In the same country, SCT rate was 0.05% while in Saudi Arabia the prevalence rate of SCT was 4.27% and 0.26% in a single and both spouses respectively. Likewise, in Bahram the figures were 4.2% and 0.26% correspondingly. In western Nigeria, standard haemoglobin HbAA was found in 73% of the subjects and HbSS in about 2.4% (in one spouse with HbSS) (Nnaji, et al., 2013).

Generally, the prevalence of SCT ranges between 10 and 40 percent among the people of equatorial Africa and less than 1 percent in southern part of Africa and declined to between 1 and 2 percent in northern part of Africa (Anie, 2010). In Nigeria and Ghana, the rate of SCT are 30% and 15%, whereas Tanzania and Uganda have shown extensive differences of up to 45% in some regions.  The distribution has reflect on the fact that historic and current exposure to plasmodium malaria infection has no effect on people with SCT; carriers appears to be protected from any associated death due to malaria and therefore has improved the survival and continued to decline the transmission of HbSS gene (Anie, 2010).

Nevertheless, there are many studies that have documented the high co-occurrence of chronic medical conditions and psychiatric morbidity associated with SCD. Even so, majority of these studies are exploring the psychiatric complications of the disease, mostly focusing on the adult caregivers and the paediatric age groups (Raji, 2016). However, only few reports explored the psychiatric co-morbidity of SCD and depression between individuals with SCD, with prevalence rate ranging between 2% and 57%. Some studies have reported alcohol abuse, psychosis, sleep disturbance, anxiety disorder, and suicidal ideation to be common among individuals with SCD (Ola, et al., 2016; Raji, 2016; Nnaji, et al., 2013). Frequent hospital visitations, poor quality of life and pain crises, are said to be the major cause of psychiatric co-morbidity among people with SCD. In developed countries, teenagers with SCD live longer than those in developing countries due to effective therapeutic interventions, like counselling and monthly check-ups (Raji, 2016).

As there is no cure for SCD and only few means of pain management and limited treatment options. However, advance knowledge of the disease has improved medical treatment over the years. Antibiotic prophylaxis is used to preclude infection particularly among teenagers (Anie, 2010).

Additionally, rehabilitations aimed to reduce painful episodes and symptoms of the disease. Sickle cell episodes are coped mainly by staying hydrated and through the administration of analgesia in clinics or hospitals. Analgesic pain management is still in its progressive phase and involves a range of prescriptions going from morphine for severe pain and paracetamol for mild pain management (Anie, 2010). People with SCD are prone to other health complications like stroke; nonetheless, hydroxyurea has been found to be effective in reducing the ‘’sickling’’ process and subsequently reducing frequent hospitalization and pain experiences by individuals with SCD (Anie, 2010). Also, bone marrow transplant has been found to be a cure for the disease, however the process required a matching donor among other criteria; regrettably these treatment is still on a trial basis and not achievable to all children affected by the disease (Raji, 2016; Anie, 2010).

Families and individuals with SCD suffers from psychosocial issues mainly as a result of symptoms and painful crises on a daily basis, and due to negative attitude, they perceived in the society (Anie, 2010). In Africa, cultural factors are important to these issues due to traditions and beliefs practices.

In Nigeria, cultures and religions has greater influence on people’s health behaviour and beliefs, for instance coping strategy (Anie, 2010; Ola, et al., 2016). In Nigeria for example, people with SCD are believed to be as a results of reincarnation (Ogbanje). Other studies (Ola, et al., 2016; Anie, 2010) have shown that religious beliefs are seen as a positive coping mechanism including faith in God, prayers, and doctors; a seemingly positive tactic to health problems in Nigeria. Anie, K., (2010), study have shown that individuals with SCD in Nigeria normally used prayers and hope as a survival tactic, which appears to be encouraged by other factors like superstition, faith and stigmatisation; unlike in developed countries like the United Kingdom (UK).

Stigma has far reaching behavioural health consequence on people. These consist of depression, psychological stress, fear, poorer treatment progression and delay in diagnosis (Leger, et al., 2018). Health behaviours and treatment outcomes can be affected by stigma, in order to reduce stigma, increase pain management and health behaviours there is need for implementation of an effective intervention.

Conceptual framework

There are many programs that aim to change the behaviour of people with SCD and promote their health as well as assist them in managing their pain crises. These programs can be in form of interventions that uses health models to evaluate and understand the best course of action to promote health; some of these models are the trans-theoretical stage of change model and the bio-ecological systems theory (Treadwell, 2011).

The trans-theoretical stages of change model aimed at assessing people’s willingness to act on new behaviour that will support their health improvement. The stages of change consist of pre-contemplation, at this stage people have no plan to change their behaviour in the near future; contemplation, at this point people are thinking of changing their behaviour; preparation, at this stage people are willing and planning to change their behaviour in the foreseen future; action, at this stage a specific lifestyle modification are being made, and maintained (Leger, et al., 2018). The model has shown a step-by-step procedures to guide persons through the stages of change, the model also consist of key construct of self-efficacy as shown in figure 1 below (Treadwell, 2011; Leger, et al., 2018).

Figure 1: Trans-theoretical stages of change

Sources: Adapted from Treadwell et al (2010).

Another model is the bio-ecological systems theory which aims at improving the environmental factors of individuals in a given population group. This theory identified five environmental systems comprising of microsystem (the immediate environment comprising schools, families, peer groups and the neighbourhoods, as well as person’s ecology, that is the identification of SCD (Figure 2) (Treadwell, 2011). The mesosystem discusses the association between microsystems or contexts as in relative to family experiences, institutional experiences and professional understanding in association to healthcare services for instance. Also, external environmental settings have an indirect effect on development, which is considered as exo-system (Treadwell, 2011; Leger, et al., 2018). The macro-system defines the principles at which individuals live. The cultural context described how industrialised a country is, as well as its socioeconomic and ethnicity status.

Lastly, the chronosystem refers to the modelling and development of the environmental events along with socio-historical situations throughout lifetime. It is important to consider the relationship between social, educational, and employment entities while viewing the change in SCD from an environmental standpoint (Treadwell, 2011).

Medical providers should consider the role of community-based authorities in relation to the lives of individuals and families with SCD and put these model into the health plan particularly in the developing countries like Nigeria. These models were used to create the proposed intervention of the last section of this assignment.

Figure 2: Bio-social ecological model of health

Source: Treadwell et al (2010).

A review of existing interventions

As there is no particular cure for SCD, various interventions have been put into place to reduce the prevalence of the disease and manage painful episodes (crises) (Ola, et al., 2016). Some of these interventions involves ways to decrease stigma towards young adults with SCD, while another intervention focused on improving clinicians’ attitudes toward people with SCD. The commonest things these interventions have are educating the public about SCD and how painful crises can be managed. At this time, two essential interventions will be evaluated. The interventions are picked based on the critical issues surrounding SCD; like painful crises, perceive increase of stigma toward individuals with SCD by health care providers and the inexcusable negative attitudes toward SCD patients from clinicians.

Intervention to improve clinicians’ attitude toward patient with SCD

In 2002, a seminar conducted by the institute of medicines has explored the contribution of clinicians’ attitudes toward the development of ethical and racial health care disparities, the ‘unequal treatment’ was developed to remove these disparities as part of the national health priority (Haywood, et al., 2011). There are several evidences that suggested the attitude of clinician toward patients to be based upon the basis of patients’ appearances (e.g., ethnicity/race), these variations can have negative impact on the health care quality they receive and these attitudes can be intricate and hard to change (Gallo, et al., 2014; Haywood, et al., 2011).

However, individuals with SCD required a specific treatment to help them maintain and manage their illness, and clinician’s attitudes can have particular imprint on patients’ health quality (Haywood, et al., 2011). Severe painful crises is the most recognized problem of SCD. According to Jenerette et al (2014) and Haywood et al (2010) studies have highlighted the clinicians’ attitudes toward individuals with SCD to be an issue that prevents them from getting an excellence pain management. Some of the negative attitudes has been described by patients and clinicians as: mistrust of patient’s report of pain, lack of compassion to patients by clinicians, stigmatization of the patients as being addict to opioid and perceived unwillingness to include patients in the course of their own treatment by the clinicians (Haywood, et al., 2011). Haywood et al (2010) conducted a video intervention that features three adults with SCD and a hematologist (Lanzkron, S.) discussing about the challenges individuals with SCD goes through when seeking pain treatment at hospitals and clinics. 276 clinicians were enrolled (response rate =98%), and about half of the participants (53%) were randomized into intervention group (figure 3) (Haywood, et al., 2011). An estimate of 68% of the participants report treating patients with SCD 3 month earlier. The racial composition of clinicians were 53.9% white, 11.7% Asian, African-American were 27.1, and 7.3% were others (Haywood, et al., 2011).

Figure 3: Controls vs. Video intervention

Source: Haywood et al., 2010.

They developed their outcome based on existing literature that assess the beliefs and attitudes of sickle cell patients who possess certain negative or positive characteristics; the point at which clinicians retain positive attitudes in association to patients with SCD, and the points at which certain behaviors were presented by patient to indicate that the patient is drug-seeking (Haywood, et al., 2011). The concept of their outcome was validated using previous validated ‘’Medical Condition Regard Scale (MCRS)’’ which evaluates the point at which patients with certain medical condition were considered as worthy of medical resources.

Attitudinal Scales

Haywood et al (2010) retained 17 of the 31 items of the factor analysis, which were differentiated into 4 sub-scales with respectable psychometric features (figure 4) (Haywood, et al., 2011).

  • Positive attitudes scales: the positive attitude scale were shown as: mean=45.7; standard deviation=24.3; latent range=0 – 100. Additionally, higher scores on this scale were shown to confirm the positive opinion about SCD by the clinicians
  • Negative attitudes scale: the negative attitudes scale were shown as mean=36.5; standard deviation =21.9; in addition to latent range =0 – 100. Higher score on this scale showed a confirmation of more negative opinions about SCD patients.
  • Concern raising behaviour scale: this scale shows the behaviours as mean=53.7; standard deviation=22.0; potential range=0 – 100. The highest scores shows more confirmation of the belief that the behaviour of individuals with SCD increases the fear of clinicians as it has indicate that these individuals maybe drug-seeking.
  • Red-flag behaviour scale: the red-flag behaviour scale are means mean=58.7, Standard deviation=22.7, potential range=0 – 100. There is a distinctive difference between the red-flag and concern raising behaviour scale. However, comparable to the ‘’concern raising behaviour scale’’, higher score on this scale shows greater confirmation of belief that some patients behaviour increases clinicians fear about patient’s drug-seeking (Haywood, et al., 2011). The factors leading to clinicians’ attitudes are shown as a scale in the table 2 below.

Figure 4: clinician attitude scales

Source: Haywood et al., 2010.

Haywood et al (2010) intervention shows significant reduction of clinicians understanding of behavior as concern raising (Mean difference = -7.8, 95%CI [13.1, -2.5] Cohen’s d=0.36). The intervention has also decreases negative attitude of the clinician toward SCD patients (Mean difference = -8. 9, 95%CI [-14.2, -3.6]; Cohen’s d=0.14) (figure 5). The intervention group has highlighted that fewer patients exaggerate their pain and ignore the medical advice by the doctors and abuse drugs or alcohol, clinicians understand the patients motives as drug-seekers when the visited the hospital, and they (patients) ignore their knowledge on crises prevention and management. There is a likelihood of the intervention group not viewing patient’s request for a specific dose or narcotic drug or patient’s appearance of being calm while complaining of pain as a sign of drug-seeking (Haywood, et al., 2011).

Figure 5: Control vs Video intervention group (Clinician attitude scores)

Source: Haywood et al., 2010.

Furthermore, both the SCD patients and the clinicians mentioned that negative attitude from clinicians have great effect on the excellence of SCD care patients received (Haywood, et al., 2011). Their video intervention has shown some evidence of attitude improvement from the clinicians toward their patients. Heywood et al (2010) intervention has demonstrated a significant reduction of negative attitudes toward SCD patients by the clinicians (i.e. patients are not seen as drug-seekers). The intervention has also illustrated a sign of reduction of negative behaviors showed by SCD patients toward clinicians.

Intervention: Reducing the stigma associated with adults with SCD

According to jenerette et al (2014) individuals with SCD visited the emergency room for acute pain crises are been left unattended for about an hour before they were given an analgesia for the pain. Although the inheritance of SCD is well-known, the delay can be due to the fact that sickle cell crises and painful crises for individuals with the disease are poorly understood, and it is hard to accurately evaluate pain crises. Another barrier to adequate pain management consist of the fact that most people with SCD in the United State and United Kingdom are African-American and majority of which are from poor socio-economic background or status (Jenerette, et al., 2014).

There are three key reason for the delay of care-seeking and Jenerette et al (2014) has linked it to stigmatization.

  1. Individuals with SCD are stereotypically labeled as drug-seekers, substance abusers, ‘’frequent flyers’’ (people that visit emergency departments for medication for non-urgent health concerns) and ‘’clock watchers’’ (people that asked for pain medication before the administration time).
  2. Due to poor understanding of the disease, adults with SCD often cannot clearly explain their pain or show no evidence of pain, hence these led to health workers to stigmatize and stereotype individuals with SCD as addicts.
  3. Adolescents with SCD try to maintain their pain crises at home to evade the emergency room and avoid been admitted into the hospital because of perceived stigma during care-seeking for severe pain (Jenerette, et al., 2014).

Jenerette et al (2014) intervention was a prospective longitudinal approach. The participants were selected and randomized to either the attention control or the Care-Seeking Intervention (CSI) group. The CSI group comprised of 2 section that was delivered individually to participants by 2 trained nurses. Jenerette et al (2014) uses Haywood et al (2010) intervention as session one of the CSI and developed their session two from Haywood’s intervention. The second session of the CSI group focused on self-confident communication assistances with a video named ‘’Speak Up to the Care You Need’’. It is a brief video written and produced by jenerette C., it portrayed potential patients-provider communications (Jenerette, et al., 2014). The video also featured a positive outcomes where it shows the advantage of seeking treatment early and the use of ‘’Situation, Background, Assessment, Recommendation (SBAR)’’ communication method, to evaluate the results of their intervention (Jenerette, et al., 2014).

The intervention involved adolescents with SCD among which 61% females are, 36% were employed; 16% in a relationship and 63% report having SCD. Based on the results of the intervention there were no significant differences between control and the CSI group in regard to disease and demographic characteristics, except in terms of employment where 25% of the control group reported employment (p= .0253), compared to the 47.6% of the CSI group who have not mentioned anything about employment (Jenerette, et al., 2014).

Theoretical framework

Jenerette et al (2010) uses theoretical framework as a guide to analyze their intervention. Theory of self-care management for SCD was used (figure 6), which focused on self-management and vulnerability factors of SCD. Jenerette et al (2014) use vulnerability factors (number of acute pain episodes annually, number of complications, lack of sickle cell crises cue identification and overprotection), undesirably influence self-management resources (social support, coping behaviors, self-care ability, self-care action, self-efficacy and confidence communication abilities) and health outcomes (depression symptoms, self-esteem, pain management experience, and perceived health-related stigma) (Jenerette, et al., 2014).

Figure 6: Theory of Self-Care Management for SCD

Source: Adapted from Jenerette et al., 2014.

However, it has been hypothesized that people with SCD in the CSI group received training for the benefit of recognition in communication skills and sickle cell crises; these hypotheses was made through the use of theory of self-care management for SCD. Although, the general health-related stigma was a key outcome, the CSI group were the target of communication between the health provider (most often a doctor) and adolescents with SCD. The findings of the outcomes were the same in Generalized Linear Model (GML) examination as well as analysis of covariance: there was a high level of health-related stigma among the CSI group than the attention control group (Jenerette, et al., 2014). This is contrary to the aim of the study, which focused on reducing health-related stigma in the CSI group. It will be best if health-related stigma awareness has been implemented first and only then there will be decline of health-related stigma towards people with SCD. Fortunately, the attention control group have benefited from the life review interview as they reported considerably less stigma over-time unlike the CSI group (Jenerette, et al., 2014). A life review interview is an interview that allow people to answer questions about their childhood adolescents, adulthood, and family as well as overall life. The first session focused on childhood memories to high school, while session two of the interview looked at the participants live as an adult with SCD (Jenerette, et al., 2014).

However, based on GML model of analysis, the health-related stigma standard total score and the intervention group were important predictors of intervention outcome (Jenerette, et al., 2014). The GLM has a standard value (P <.0001), intervention group (p =.002), and the care-seeking pain level (P =.0353); all these are important predictors of health-related stigma by the doctors. After separating the attention control group from the CSI group, the health-related stigma total score was detected to be standard, it become evident that with time the change between the 2 groups expanded (figure 7 and 8) (Jenerette, et al., 2014). Similar pattern was observed in the mean change over time for health-related stigma through doctors’ subscales.

Figure 7: Study outcomes mean by time

Source: Jenerette et al., 2014.

Figure 8: Outcome mean comparison by time

Source: Jenerette et al 2014.

Key themes of the interventions

The main objective of Haywood et al (2010) intervention is to test the impact of visual (video) intervention, which was design to promote positive attitude of clinicians toward adults with SCD. Haywood et al (2010) intervention has reduced the negative attitudes of the intervention group (the clinicians), at the same time reduced the authorization of certain patient behaviors (negative behavior) as ‘concern raising’. Their intervention have suggested that the attitudes of clinicians can be improved through a relatively easy and short intervention that can be made easily.

However, Jenerette et al (2014) intervention continues from where Haywood et al (2010) stopped but turn their focus to reducing stigma among young adults with SCD. Their findings were promising as they have learned from previous study (Jenerette, et al., 2014), which was also a video intervention that aimed at changing the negative attitude patients perceived from the clinicians while seeking pain management treatment (Haywood, et al., 2011). Jenerette et al (2014) intervention aim at reducing stigma toward adolescents with SCD and their findings has shown significant reduction of stigma among the attention control group, the life review interviews has provided them with resources to help them manage their crises. At the same time provided self-management resources to help with coping mechanism and social support for people with SCD (Jenerette, et al., 2014).

Limitations of the interventions

Haywood et al (2010) intervention was subjected to some limitations like in the interpretation of results. The study has highlighted statistical variances between the control and the intervention groups on three of the four outcomes measures, however the clinical important of these variances were not clear. However, most of studies that looked at the attitudes of clinician towards individuals with SCD were purely qualitative. For this reason, it is not clear on how quantitative evaluation of variances between the attitudes of clinicians toward individuals with SCD, interpret into observed differences of the quality of care delivered to individuals with SCD (Haywood, et al., 2011). Haywood et al (2010) has not mentioned how long the effect of the intervention lasted after the study group completed the survey, directly after the visual presentation of the intervention. It would be helpful and possible to endure the intervention for a long time, through the implementation of periodic ‘’refreshers’’. The level to which Haywood et al., (2010) findings can be use by clinicians employed in other treatment setting is unclear.

Jenerette et al., (2014) intervention has some limitations, one of which was the assumption normally used in the examination of ‘’longitudinal repeated-methods data’’, the study results were similar for both the non-computer and computer; if the non-computers remained in the research. When the assumption of the results was false, there is a high chance of the conclusion of the study to be biased. Furthermore, there is no way of confirming whether the assumption in the study was met. It is important to point out that Jenerette et al (2014) intervention has shown no statistical significant associated to the two baseline factors or any of the two study outcomes.

Proposed intervention – Education

Every disease has it weaknesses, this make it possible to be attacked from there to prevent, control or manage it spread. As there is no definite cure of SCD the primary aims of healthcare and public health professionals is to reduce its spread and manage its episode (Oludare & Ogili, 2013; Piel, 2013). Most people with SCD lack the knowledge of the disease, these led to the high mortality rate and the spread of the disease. To reduce the widespread of SCD and manage its pain crises in developing countries like Nigeria. There is need for educating the public and training of healthcare workers on how they can manage patient’s crisis more effectively (Marsh, 2011; Gallo, et al., 2014). According to Asnani et al (2016), intervention is refers to caregiver or patient-centered education program which is based on a particular manual, protocols or skills, for example early recognition of sickle cell complications and improving the understanding and knowledge of the disease (Oludare & Ogili, 2013). The useful effects of SCD knowledge has been shown in few observational research. Some of these studies (Marsh, 2011; Gallo, et al., 2014; Burnes, 2008) have shown the benefits of maternal, community and institutional education. Educating individuals with and without SCD will increase the understanding and knowledge of the disease, which in turn will encourage greater psychosocial skills (e.g. boldness and confidence when pursuing treatment) and help people gain better control of their disease.

Educating the public and health providers is a significant means of sickle cell management as there is no standard cure for the disease; thus, the reason for the proposed intervention in this patch work. There are many of practices and habits that help reduce, hindered, prevent and manage sickle cell crises within sufferers of the disease and/or caregivers (e.g. parents, relatives and guardians etc.). Those with better Sickle cell knowledge tend to have positive health beliefs, for instance they have high possibility of undergoing sickle cell testing before starting a family. Improving parents’ knowledge on SCD increases family relationship and positivity (Gallo, et al., 2014). Education can be implemented at various stages.

The intervention aims at educating children and adolescent about sickle cell disease and illustrate how mild crises can be manage at home, the intervention will profit the community by decreasing the prevalence of the disease and reduce hospital visitation associated to SCD. The intervention will emphasis on developing countries where the prevalence of SCD is higher than everywhere else. This intervention is designed in a way that it will ultimately benefits both the children, adults and parents. Educating children and adolescents about SCD will reduce the spread of the disease and help in the management of the crises, at the same time reduce the prevalence of the illness. The key benefit and advantage of this intervention is that it can be implemented in schools, universities, health centers and therapy unit in hospitals and clinics.

Approach and rationale

As mentioned previously, the best method to reduce sickle cell spreading and manage the disease is through educating the general public particularly children, adolescents, parents and guardians on the complication and challenges surrounding the disease. This involve changing individuals’ habits and beliefs about the disease. In some southern part of Nigeria as mentioned, majority of people in those communities believe SCD to be a curse or reincarnation; majority of people with the disease face stigma, isolation, discrimination and some regions even disown those with the disease from the community (Anie, 2010; Ola, et al., 2016). However, these issues can be handled by educating the public, hence the goal of this intervention. Subsequently, the intended intervention involves people with and without SCD.

PowerPoint presentations

As Jenerette et al (2014) and Haywood et al (2010) mentioned in their interventions that individuals with SCD do not like visiting hospital at the time of their painful crises due to the negative attitude, stigma and discrimination they perceived by the clinicians, doctors, nurses and other health workers. Sometimes, self-care management for painful crises are not advisable, particularly for people with SCD as their crises has unpredictable outcomes. However, this intervention aimed at those that do not like to be admitted into a hospital for minor pain. The intervention will educate people with SCD on how they can maintain mild pain crises like joint pains, using elbow, knee and wrist pain relief supports, and pain relief creams. It will be a two-part PowerPoint presentation which will be carried out in schools, institutions and sickle cell clinics. A copy of the presented slides will be given to students and staff to carry home, to share with family and relatives. Knee, wrist and elbow pain supports are very effective in terms of pain relief; they reduce pain rapidly and with the application of pain relief creams. Also, individuals will start to have relief, moments after the application of the cream and knee, elbow or wrist support; this is based on personal experience.

As mentioned in the introduction, two health models will be use as a guide to carry out this intervention; the models are: trans-theoretical stage of change model and the bio-ecological systems theory (figure 1 and 2). Using the first model (trans-theoretical stage of change), individuals that are thinking about changing their behavior in terms of pain management will be helped. The slides will show them how they can use the knee, ankle, and wrist supports during their mild crises; a visual presentation will be given to them on how the use the supports. The intervention will help the parents prepared to manage their children crises at the time of emergency like at night (as the hospital in Nigeria are not 24hrs and those that are 24hrs have no doctors on site after midnight) (Mustapha, 2014). Based on personal experience, only few parents and family members know what to do, when their child is having a sickle cell crises. The intervention will help them to act and maintained the child crises at home during mild sickle cell episodes. This approach will help adolescents, adults and families manage sickle cell crises at home, in turn improved their health and lifestyle (as shown in the presentation sample below).

Slide 1: Cover page

Slide 2: Method of sickle cell management

Slide 3: Method of sickle cell management

Slide 4: Knee joint supports

Slide 5: Ankle joint supports

Slide 6: Wrist joint supports

Slide 7: Pain relief creams

Source: Author’s Own

The trans-theoretical stage of change model is suitable for this section of the intervention as it helps those that are thinking of changing the ways of self-care management into something more effective. The presented slides above assist people to that are prepared to manage their mild crises at home to avoid been stigmatize. At the time of the presentation a sample of the support pads will be provided, and demonstration will be made on how individuals with SCD can use it at the time of their need; as well as how the can apply the pain relief creams (slide 4, 5, 6, and 7). At the same time, show them how to maintain their crises as shown in slide 2 and 3 above.

The second model (the bio-ecological systems theory) will be used to educate individuals about sickle cell disease and help in the reduction of stigma and discrimination toward those with SCD. In Nigeria, there is lack of knowledge about SCD and some people live with the disease or are carriers of it but have no clue about it. The second part of the intervention will educate people about the disease and encourage them to attend sickle cell screening. The slides will show a detailed sickle cell gene tree that compared different sickle cell genes and normal genes. Slide 3 to 6 shows probability results of what will happened if individuals with SCD, SCT and normal gene, if individuals with and without the disease are to start a family. It will also show the psychological effect of discrimination and stigmatization on the health of those with the disease, as well as illustrate the effect it will have on the society (as shown in the presentation sample below).

Slide 1: Cover page

Slide 2: Sickle cell definition

Slide 3: Sickle cell tree HbSS genes result

Slide 4: Sickle cell tree HbSS vs HbAS genes result

Slide 5: Sickle cell tree HbAS vs HbAS gene result

Slide 6: Sickle cell tree HbAA vs HbSS gene result.

Source: Author’s Own

Evaluation strategy

To understand whether the objective and outcome of the intervention has been met. An evaluation strategy will be developed to evaluate and analyze the adequacy, efficacy and acceptability of the intervention by the society and individual with SCD. It is significant to analyze the cost-effectiveness of the policy for the intervention to continue. A periodic epidemiology survey will be used to determine whether the intervention is effective, through the evaluation of the prevalence, hospital visitations, incidence and morbidity rate and see if any significant changes has been made in the life of those with and without SCD. The core element of an evaluation strategy should analyze the capability, application, acceptability, availability and effectiveness of the program on health status of the people. However, more public sensitization is required to be carried out using all available resources for example social media, media, schools, seminars and other outreach. This will improve the practice of the adolescents on SCD premarital counselling, as 85% of young adults consider premarital counselling to be helpful in the reduction of sickle cell prevalence (Oludare & Ogili, 2013). This shows that educating the public on SCD will also encourage them to participate on premarital counselling and screening as well as pain crises management.

Mechanism of Effectiveness

Educational qualifications have significant influence on the attitude practice and knowledge of individuals with and without SCD; age is significantly related to practice and attitude of people in the community about SCD (Oludare & Ogili, 2013). Several researchers have highlighted the importance of education and public awareness toward adolescence and those of lower age group in informing them about SCD and premarital screening and counselling before starting a family (Marsh, 2011; Gallo, et al., 2014; Burnes, 2008; Oludare & Ogili, 2013).  Additionally, interventions that support networks and utilize family members have the possible advantage of being more cost-effective in the long run compared to other kind of intervention. Improving family relationship and social support will help people with SCD to manage their mild crises at home without been admitted into the hospital.

The intervention has many advantages, its implementation will help reduce the rate of hospital admission for mild crises, reduce stigma and devaluation of people with SCD in the community; at the same time educate the public and change their negative attitude toward SCD. The key advantages are: it is easy to understand and practice; it is cost-effective compared to other interventions; it is cheap to practice and does not require high-end materials; it is not invasive or uncomfortable technique; it is self-manageable and affordable.

Barriers to success

Regardless of the benefits this intervention will have on Nigerian people with SCD, it also may have some possible limitations. As mentioned by many researchers, SCD has many complication and its crises has no warning signs or occurrence time, slight thing can triggered an episode (Marsh, 2011; Gallo, et al., 2014; Burnes, 2008). The supports pads are effective on relieving mild and moderate pain, however it is not effective during severe episodes of SCD, and individuals with SCD in Nigeria may ignore the ‘’see a doctor when the pain is severe’’ part. This may lead to other health complication or a long-term hospital admission. Based on personal experience, when the pain is severe; the support pads and the pain relief creams have little effect on it. As mentioned by Ola et al (2016), people with SCD will do anything to avoid hospitals especially in Nigeria, where their healthcare system is an out-of-pocket. As the method of self-management is easy and affordable individuals would rather use the pad to manage the severe pain than to be admitted into the hospital and spend a large amount of their income on their love one.

Conclusion

Self-management of mild sickle cell crises and prevalence reduction is the aimed of the intervention. Regardless of the limitations, the suggested intervention has encouraging outcomes. Most of developing countries like Nigeria have already put policy in place to manage SCD but few countries have yet to comprehend the consequences of chronic conditions like SCD. It is important to put this intervention into practice to assist individuals with SCD to self-manage their condition at home. Nigeria and other developing countries have higher incidence rate of chronic illnesses and should consider this intervention as it is easy to carry out and required slight efforts and resources.

References

Anie, k., 2010. Psychosocial impact of sickle cell disorder. In: 1st, ed. Perspectives from a Nigerian setting. London: BioMed Central Ltd, p. 2.

Burnes, D., 2008. Mothers Raising Children with Sickle Cell Disease at the Intersection of Race, Gender, and Illness Stigma. Health & social work, 33(3), pp. 211-221.

Asnani, M., Quimby, K., Bennett, N. and Francis, D., 2016. Interventions for patients and caregivers to improve knowledge of sickle cell disease and recognition of its related complications. Cochrane Database of Systematic Reviews. [Online]
Available at: https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD011175.pub2/epdf/full
[Accessed 3 November 2018].

Gallo, A.M., Wilkie, D.J., Wang, E., Labotka, R.J., Molokie, R.E., Stahl, C., Hershberger, P.E., Zhao, Z., Suarez, M.L., Johnson, B., Pullum, C., Angulo, R. and Thompson, A., 2014. Evaluation of the SCKnowIQ Tool aReproductive CHOICES Intervention Among Young Adults With Sickle Cell Disease or Sickle Cell Trait. Clinical nursing research, 23(4), pp. 421-441.

Haywood, C., Lanzkron, S., Hughes, M., Brown, R., Massa, M., Ratanawongsa, N. and Beach, M., 2011. A Video-Intervention to Improve Clinician Attitudes Toward Patients with Sickle Cell Disease: The Results of a Randomized Experiment. Journal of General Internal Medicine, 26(3), pp. 518-523.

Jenerette, C.M., Brewer, C.A., Edwards, L.J., Mishel, M.H. and Gil, K.M., 2014. An Intervention to Decrease Stigma in Young Adults With Sickle Cell Disease. Western journal of nursing research, 36(5), pp. 599-619.

Leger, R., Wagner, L. and Odesina, V., 2018. Stigma in adults with sickle cell disease and family members: Scale development and pilot study in the USA and Nigeria. International Journal of Africa Nursing Sciences, 9(1), pp. 23-29.

Marsh, V., 2011. ‘All her children are born that way’: gendered experiences of stigma in families affected by sickle cell disorder in rural Kenya. Ethnicity & health, 16(4), pp. 343-360.

Mustapha, Y., 2014. Lack of doctors in kano state hospitals after midnight. [Interview] (22 April 2014).

Nnaji, G., Ezeagwuna, D., Nnaji, I., Osakwe, J., Nwigwe, A. and Onwurah, O., 2013. Prevalence and pattern of sickle cell disease in premarital couples in Southeastern Nigeria. Nigerian Journal of Clinical Practice, 16(3), pp. 309-314.

Ola, B., Yates, S. and Dyson, S., 2016. Living with sickle cell disease and depression in Lagos, Nigeria: A mixed methods study. Social science & medicine, 161(1), pp. 27-36.

Oludare, G. and Ogili, M., 2013. Knowledge, Attitude and Practice of Premarital Counseling for Sickle Cell Disease Among Youth in Yaba, Nigeria. African Journal of Reproductive Health, 17(4), pp. 175-182.

Piel, f., 2013. Global burden of sickle cell anaemia in children under five, 2010–2050: modelling based on demographics, excess mortality, and interventions. Global Network for Sickle Cell Disease, 10(7), p. 148.

Raji, S., 2016. Prevalence and correlates of major depression among Nigerian adults with sickle cell disease. The International Journal of Psychiatry in Medicine, 51(5), pp. 456-467.

Treadwell, M., 2011. Transition from pediatric to adult care in sickle cell disease: Establishing evidence‐based practice and directions for research. American Journal of Hamatology, 86(1), p. 116.

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