COPING STRATEGIES AND PERCEIVED EFFECTS OF SICKLE CELL DISEASE ON QUALITY OF LIFE AMONG PATIENTS ATTENDING UNIVERSITY OF MAIDUGURI TEACHING HOSPITAL

ABSTRACT

Sickle Cell Disease (SCD) is a potentially overwhelming condition that is caused by an autosomal recessive inherited hemoglobinopathy. Sickle Cell Disease affects all areas of the patient’s life and results in different events or experiences (occurrences which leaves an impression on someone). This study aimed at understanding the perceptions of adults living with SCD on triggering, aggravating and alleviating factors for pain, describing the perceptions on pain management, and understanding perspective experiences of social implication on living with SCD. Methods: The method used in this study was phenomenological study that used descriptive qualitative method. Study population was of adults living with Sickle Cell Disease in Dar-es -Salaam Region, aged 18 years and above. Sample size was of 15 adults living with SCD. Sampling procedure used to select study participants was Purposive/judgmental. Adults aged 18 years and above screened at University of Maiduguri Teaching Hospital SCD Cohort and confirmed to have SCD, who consented to be enrolled in the study were included. Adults with SCD who were not enrolled in the cohort were excluded. Data were collected at University of Maiduguri Teaching Hospital from March 2017 to May 2017. Fifteen in-depth interviews with adults living with SCD aged eighteen years and above were conducted to collect primary data. Data were analyzed by using content analysis approach. Findings: Adults living with SCD mentioned different factors that perceived to trigger, aggravate and alleviate pain. Most participants reported to manage pain by home remedy together with self-medication, however all participants seek hospital care whenever the pain continues. Sickle Cell Disease was also found to have led to poor school attendance and hence poor academic performances, also it has been found to have caused difficulties in getting life partner and establishing family as well as failing to work and become independent eeconomically. Recommendations: Living with a sickle cell disease is a challenge. According to experiences reported by participants, health outcomes of adults living with SCD depend on good physical, social and psychological management. These can be influenced by the health care system, health personnel and individuals. The government through the Ministry of Health, Community Development, Gender, Elderly and Children, should support on prevention of the disease, (cut off the chain of inheritance), and management of the disease.

 

CHAPTER ONE

1.0 INTRODUCTION

1.1 BACKGROUND INFORMATION

Sickle Cell Disease is a potentially overwhelming condition that is caused by an autosomal recessive inherited gene for hemoglobinopathy. The genetic abnormality is due to a substitution of thiamine acid valine for glutamic acid at the sixth position on the beta globin chain. This was first described over one hundred years ago. Hemoglobin S (HbS) is produced as a result of this defect. It is a hemoglobin tetramer which is insoluble and polymerizes when deoxygenated. Patients with combination of two hemoglobinopathies in general, have the most severe forms of SCD (Neville& Panepinto, 2011).

 

SCD is an umbrella term representing several hemoglobin (major protein imparting the color to RBCs) disorders. Sickle hemoglobin (HbS) is a modification of normal hemoglobin (HbA), which arose many thousands of years ago. HbS is made differently from HbA, when oxygen is removed, the molecule tend to stick together forming long fibres which may distort the RBCs into the sickle shaped. It happens more rapidly in circulation and block flow in the vessels, poorly oxygenated blood supply to tissues, anemia, pain, and multi-system failure. The commonest type of SCD occurs when the HbS gene is inherited from both parents causing homozygous SCD or SS disease. Other types inherited which cause SCD are HbC (common in West Africa), beta thalassemia minor and beta thalassemia major. The HbSS and beta thalassemia major variants manifest as a more severe form of the disease, while HbSC and beta thalassemia minor as a less severe form. If both parents have the sickle-cell trait (AS), or one has sickle-cell trait and the other has another abnormal interacting hemoglobin trait, there is a 25% chance at each pregnancy that the child will have a form of Sickle Cell Disease. If one parent has AS, and the other has SS (or any form of SCD) the risk is 50% at each pregnancy (Adegbola et al. 2012).See figure 1: Inheritance sketch.

 

Figure 1: Inheritance sketch

 

 

EPIDEMIOLOGY

SCD disease is prevalent in Africa, the United States, Caribbean, Central America, South America, Saudi Arabia, India and the Mediterranean (Center for Disease Control and Prevention, 2011a). Approximately 90,000 to100,000 Americans are affected by SCD, making it the most common genetic disorder (Matthie, 2013). It is estimated that 16% of the population in Africa has a sickle hemoglobinopathy which is the highest proportion worldwide.

CLINICAL MANIFESTATION

 

Acute sickle-cell pain is typically sharp and/or throbbing in nature, and can beof a sudden or gradual onset. It may last from minutes to weeks in duration depending on how pain is managed. Acute sickle-cell pain is believed to be secondary to vasoocclusion by sticky sickled red blood cells (RBCs) that adhere to vascular endothelium. Vascular occlusion causes ischemia, consequently damaging tissues of any organ supplied by the occluded vessel. Tissue damage, in turn, causes a state of inflammation with the release of several inflammatory mediators. The chemical energy caused by inflammation is changed into an electrical signal of pain transmitted along peripheral nerves, spinal cord and region of Central Nervous System (CNS).The impulse ascends along the contralateral spinothalamic tract to the thalamus which then interconnects reversibly with the limbic system and other regions of the central nervous system (CNS).When the CNS tries to inhibit the transmission of the painful stimulus with endogenous serotonin and epinephrine. This modified electrochemical pain impulse is eventually sent to the cerebral cortex where it is perceived as pain (Samir K Ballas, 2011).

SCD is different from other diseases associated with chronic pain such as osteoarthritis, and rheumatoid arthritis, as sickle cell is acute pain manifests itself in infancy and recurs throughout the life span of the affected individual. With age, acute pain retains its unpredictable relapses and may result in chronic pain. Chronic pain may evolve into neuropathic pain. Acute pain, mostly, dominates the clinical picture and requires urgent treatment with parenteral opioids in the Emergency Room (ER) and/or hospital (Ballas,S.K,2011). The clinical presentation of SCD pain is complex. In the beginning, it may be largely episodic; however, tissue necrosis and the changes in the central nervous system and other factors, may be due to the cumulative impact of pain itself inducing an increasingly chronic component of SCD pain later in life (Hollins et al. 2012).

MORBIDITY AND MORTALITY

In the United States Platt et al, reported that many adults with Sickle Cell Disease died during acute sickle cell-related complications such as pain, acute chest syndrome, and stroke. Most common causes of death reported are pulmonary hypertension, the sudden death of unknown etiology, renal failure, and infections.

During their life time, patients with Sickle Cell Disease are susceptible to various complications such as bone pain (dactylitis, avascular necrosis of flat/long bone), priapism, anemia (acute splenic sequestration, chronic hypersplenism), abdominal pain (abdominal pain crisis, cholecystitis), fever (septicemia, urinary tract infections, viral), deep jaundice (chronic hepatic congestion, viral hepatitis), leg ulcer, nervous system (ocular involvement, stroke) and acute chest syndrome. However, pain crisis is the most common symptoms reported by patients, and is considered the most common reason of referral to the emergency department and hospitals (Ahmadi et al. 2014).

People living with SCD face many types of morbidity and early mortality. Various characteristics causing morbidity and mortality are; increased susceptibility to infections, chronic hemolytic anemia, end organ damage, and intermittent episodes of vascular occlusion which result in acute and chronic pain (Wilkie et al. 2010).

 

In some African countries, nearly all babies born with SCD die in early childhood. In the United States (U.S.), newborn screening, prophylactic penicillin treatment in children and other aggressive treatments for pain and disease complications have increased the life expectancy for the 100,000 people with SCD to age 42 years for men and 48 for women. Recent advances in the treatment of SCD, such as hydroxyurea, prolong and improve the quality of life for many people, some living into their eighties. Unfortunately, people with SCD live with many threats to the quality of life. Palliative care offers the hope of reducing these threats (Wilkie et al . 2010).

 

CONSEQUENCES

Most often, chronic pain, affects all areas of the patient’s life. Chronic pain is challenging for healthcare providers to manage, and can negatively change the patient’s life into pathologies that include depression and coping difficulties (Adegbola, Maxine, 2015). In order to cope with the disease, SCD patients must adapt themselves to a set of behaviors that promote self-management and prevent complications associated with the disease (Ahmadi et al. 2014).

 

A comprehensive, dedicated SCD program that provides, follow-up care, family and patient education and counseling, and prevention and treatment of complications could have a significant impact in reducing morbidity and mortality and result in longer more productive lives(Makani et al. 2015).

 

 

1.2 PROBLEM STATEMENT

 

Living with the SCD has been a challenge to many adults with the disease. The effects of SCD are multi-dimensional, ranging from causing high morbidity, and reducing the quality of life, to imposing a high socio-economic burden on individuals, and families (Tluway & Makani, 2017).

Pain is one of the major problems of Sickle Cell Disease (SCD). SCD pain typically emerges in the second half of the first year of life, as red blood cells with the fetal form of hemoglobin wane, replaced by cells that are capable of sickling

(deforming),preventing blood flow, and thus producing ischemia, hypoxia, and possible tissue damage. Pain is gradually in Sickle Cell Disease, affecting all aspects of life (Hollins et al. 2012;Adegbola et al. 2012;Akingbola et al. 2011;Ballas, 2011;Jenerete &Lauderdale,2008;Wilkie et al. 2010).

Management of SCD pain has to be holistic (take into account all aspects of patient’s life without forgetting mental and social factors and not just the physical symptoms of the disease), but this has not been the case among health care providers in hospitals.

Poor painful crises management can lead to increase in frequencies of crises, and later to chronic pain that may result in recurrent hospital admissions, frustration, loss of precious time for adult’s daily activities, and hence have direct consequences on economy and implicate social life.

Little is known about adults living with Sickle Cell Disease in Nigeria that show their experience or how the disease implicates normal life, and how they manage the pain.

Therefore, this study is intended to fill this missing gap of information.

 

 

 

1.3 SIGNIFICANCE OF THE STUDY

 

The findings of this study will help understand what adults living with SCD know about self-care management and help fills in the gaps of knowledge; will help recognize nursing gaps in caring, guiding nursing action, and educating. Will also help to improve care in hospital to the adult patients with SCD. Understand these will help develop intervention plan like preparing education sessions on self-care management, prevention of/or reduction of crisis occurrences.

The finding will also help understand social implication on living with SCD. By understanding these, nurses will be able to know what to prepare in education, and also what are the problems needs counseling. Management of the adult living with SCD will then be improved.

These can be achieved by advocating them to right to places example in hospitals, and Ministry of Health, Community and Development, Gender, Elderly and Children.

 1.4 DEFINITION OF TERMS

PAIN is an unpleasant feeling and emotional experience associated with actual or potential tissue damage. It is a subjective experience with both psychological and sensory components, and tissue damage does not need to be present for pain to be experienced (Nicholson, 2006).

ACUTE SPLENIC SEQUESTRATION-Is a sudden increase in spleen size associated with trapping of red blood cell (RBCs) (Rezende et al.2009).

CHRONIC HYPERSPLENISM-Is a chronic red cells sequestration (removing from circulation) associated with marked splenomegaly  (Elmakki, 2012).

PRIAPISM

Stuttering priapism is a recurrent short-lived attack (usually 3-6 hours) of painful erection of the penis unassociated with sexual desire, while major priapism attack usually lasts two days. The patient complains of an extremely tender and painful erection, and the pain is referred to the suprapubic and perineal region  (Crane, & Bennett, 2010).

 

SELF-CARE MANAGEMENT (INDEPENDENT-DOING YOUR SELF)

This refers to all actions and coping strategies, carried out at home, and needed to take part in therapeutic behaviors targeted at preventing health complications, improving psychosocial conditions, and maintaining health (Jenerette & Murdaugh, 2008).

 

SOCIAL SUPPORT

This refers to all available helping, encouraging, whether positive words, financial/other aids from family or friends (Jenerette & Murdaugh, 2008).

 

1.5 OPERATIONAL DEFINITION

 

HYDRATION

In this study hydration means to supply plenty of fluid to a person to maintain a balance of fluid in the body, at least three to four liters of water/fluid per day (at least twelve to sixteen glasses) the water can be in the form of water, juice, tea, soup and other fluids. Alcoholic drinks should be avoided because alcohol exacerbates dehydration and can lead to other complications.

HEALTHY DIET

In this study a healthy diet means the diet that gives the body the nutrients it needs to function correctly. The food should be estimated in proportions e.g. One third of plate should be vegetable and fruits, another one third should be protein and the remaining one third should be carbohydrate. To get food rich in iron and calcium such as fish/sardine (small fish), liver, dark green leaves vegetables, beans and red meat are important. Also foods that have folic acid have to be included, such as fruits and Vegetables e.g. spinach, broccoli, avocado, peas, citrus fruits. They should also eat beans, cereals, rice, nuts, and fresh juice. People with Sickle Cell Disease have a greater risk of getting infections; it is important that food be handled with particular care, such as, maintaining strict hand hygiene, and proper food storage.

 

 

 

 

 

 

 

Figure 2: Healthy diet Plate and drinking water

 

 

 

 

 

 

 

1.6 OBJECTIVES

1.6.1 BROAD OBJECTIVE

To explore the experiences of living with sickle cell disease among adults.

1.6.2 SPECIFIC OBJECTIVES

 

• To understand the perceptions of adults living with SCD on triggering, aggravating and alleviating factors for pain.
• To describe the perception of adults living with SCD on pain management.
• To understand participants’ perspective experiences of social implication on living with SCD.

1.7 RESEARCH QUESTIONS

 

• What are the perceptions of adult living with SCD on triggering, aggravating and alleviating factors for pain?
• What are the perceptions of adult living with SCD on pain management?
• What are the participants’ perspective experiences of social implication on living with SCD?

 

1.8 CONCEPTUAL FRAMEWORK

 

HEALTH OUTCOMES

In this study, health outcomes of adults living with Sickle Cell Disease are physical (number of painful crises, number of complications), social (availability of different supports (e.g. Family), availability of health care services, coping behavior) and psychological (such as frustration, low self-esteem, and depression). For populations with chronic disease, measurement of productive life gives a meaningful way to decide the impact of health care when cure is not possible. Physical, social and psychological outcomes are the results of interrelated factors which are; health personnel, health care system factors and individual factors that may have either positive and/or negative influence. In this study the bolded physical and social outcomes were addressed.

 

HEALTH PERSONNEL FACTORS

In this study, health personnel are nurses and doctors. Health personnel factors can influence the proper management (right treatment and management, right dosage, family and patient education, treatment of complications and counseling) of patients. Health personnel factors include knowledge, competence and attitudes. Health education to patients and family includes a lot of things such as adherence to treatment, self-care management, early recognition of problems, how to reduce number of crises, encouragement on follow-up clinics and others.

HEALTH SYSTEM FACTORS

In this study, health system factors are factors within the health system (hospital, Ministry of Health, Community Development, Gender, Elderly, and Children-MoHCDGEC) that can influence nurses or doctors in the management of patients. Health system factors, in this case, are availability of early screening clinics, follow-up care system, treatment, diagnostic equipment availability, human resource, training, and preventive measures.

All these have an influence in the proper management of patients.

INDIVIDUAL FACTORS

Individual factors in this study (education, social support, employment, cultural and religious/Prayers, self-care management, and action) can have either positive and/or negative influences on self-care management, as self-care management influences a more productive life.

Despite having many influencing factors, according to the limitation of time, this study focuses on education, social support, employment, cultural and religious/prayers, self care management, physical outcome, and social outcome) that influences adults to have different life experiences. Other factors are beyond the scope of this study.

 

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