Care and Management of Sickle Cell Disease in Accident and Emergency

Care and Management of Sickle Cell Disease in Accident and Emergency

Sickle Cell Disease (SCD) is a blood condition resulting from abnormal haemoglobin, known as S haemoglobin (Kavanagh et al., 2022). Due to the production of haemoglobin, red blood cells become rigid, changing to a sickle shape, which leads to blood vessel blocking, resulting in pain, organ damage and predisposing SCD patients to more infections. SCD is relatively common in people of African, Mediterranean, Middle Eastern and Asian origin and follows an autosomal recessive mode of inheritance (Williams, 2024). Sickle Cell Disease is caused by an inherited defect in genes that control the normal production and function of haemoglobin, which is responsible for sustaining oxygen and needed nutrient distribution all over the body, especially the major organs of the body (Pecker, 2021). The defective gene results in abnormal haemoglobin, known as haemoglobin S. Sickle Cell Disease is one of the most serious types of autosomal recessive single-based mutation in haemoglobin (Adekile, 2021). The event causes polymerise when oxygen is deoxygenated, reducing the flexibility of the cell and leading to a sickling appearance. Erythrocytes can then form clusters that block blood vessels, resulting in painful crisis, infection and Anemia (Sabir, 2024). It has been estimated that 17,500 people in the United Kingdom have sickle cell disease (Sickle Cell Society, 2024). Thus, this essay explores the care and management of sickle cell disease in accident and emergency settings and strategies that underpin effective patient care. It also explores the evidence-based practices of UK healthcare practitioners in serving patients with SCD.

To this day, there is no real cure for Sickle Cell Disease (SCD), and as more patients are attending the emergency department personally, it should be dealt with by management in line with evidence-based practice. It is not a common health condition known in the United Kingdom; thus, making healthcare staff aware of this disease condition will provide an understanding of the needs and clinical management of the patient (El-Haddad et al., 2022).

Reportedly, Sickle Cell Disease results in chronic Anemia with signs such as fatigue and pallor or acute vaso-occlusive crises with blockages in blood vessels (Barrett, 2022). Further, SCD makes its victims prone to infections resulting from the spleen anarchy and can cause harm to several organs in the body, including the lungs, liver, and kidneys, with the progression over time (Kharel et al., 2022). The symptoms and the problems associated with this form can be best managed if diagnosed and treated early. For such reasons, health caregivers in the United Kingdom incorporate bio-psycho-social research and a holistic approach to managing patients diagnosed with SCD (Paszek, 2020). Another component of management is pain counselling, monitoring and prevention of complications through intercession, such as hydroxyurea therapy, since this therapy has been recommended to decrease the frequency of pain episodes and acute chest syndrome (Klings et al., 2022). The most common patient precautions and teachings are about lifestyle changes, especially in the intake of fluids, activities, and infections, since they are at a higher risk of getting infections (Mattioli et al., 2020). Further, they provide general treatment support to patients, such as psychological and social support, to enhance their quality of life. The attendance of haematologists, pain specialists, and other healthcare workers provides person-centred care and meets each patient's medical, social, and psychological needs.

Here, the role of Emergency department (ED) nurse is pivotal in providing primary care and attention to the SCD patients. ED nurses are vital practitioners as most sickle cell patients present at the hospital in agony from an acute pain crisis (Sherman, 2023). These nurses identify the pain and its potential in setting the physiological status of the patient (Ellison, 2017). They prescribe analgesics and other ways to provide comfort and minimising risks such as opioid use in patient care (Phillips et al. 2017). ED nurses also provide patient education on the prevention of pain from trauma, involving the patient’s families and encouraging teamwork to enhance patient treatment. They also participate in the development of a good pain management plan that is helpful in avoiding the catastrophes and promoting positive results as asserted by Caudill, (2016). Prenatal and Neonatal SCD screening is an early prevention intervention essential to reducing the number of deaths and the most cost-effective method of the screening program and can be used in future conception (Dexter, 2022). More diagnostic procedures are available worldwide in all healthcare settings, such as density-based separation, which can detect sickle cell characteristics within 12 minutes (Kumar et al., 2014). Genotyping application and serological phenotyping in sickle cell help decrease risk-stratifying for alloimmunisation risk. Alloimmunisation risk refers to the risk of developing an immune response to foreign antigens, which in the case of sickle cell disease, can lead to red blood cell alloimmunisation. Patients with sickle cell disease have a high rate of red blood cell alloimmunisation, which increases morbidity and mortality. It helps evaluate the benefits of red blood molecular antigen-typing for both patients and their donors and describes how results from these critical tests could enhance patient safety (Karafin, 2022). In addition, a study by Almosfer (2022) stated that Hemoglobin Electrophoresis helps evaluate hemoglobin abnormalities using methods such as Gel electrophoresis, capillary electrophoresis and HPLC (high-performance liquid chromatography). HPLC is the most used to detect more types of haemoglobin pathology (Sridar, 2021). Another study by Kayongo et al. (2022) stated that point-of-care testing for SCD is the simplest and most economical way of testing for SCD.

Pain is the most common hallmark of sickle cell disease when it lacks therapy; thus, understanding the pathology of the disease process and prevention is important. It can start from infancy, leading to hospitalisation, reduced survival and poor quality of life. Acute painful episodes are the most common reason for emergency department visits among sickle cell disease patients. Early and aggressive pain management is a priority (Lovett et al., 2014). Reportedly, Multifocal aetiology is investigated for nontraumatic vascular necrosis of the femoral head, which accounts for 30-70% of the majority having SCD is still unknown; however, several risk factors play a part in the disease condition (Al-Otaibi, 2022). The fundamental pathophysiology of osteonecrosis is due to vascular occlusion, thus resulting in oxygen and nutrition deprivation (Erhabor, 2022). Considering the patient's condition, such as age, comorbidities, extent of necrosis and amount of pain, this condition could be treated through conservative or invasive methods. SCD is known to be a systemic inherited hemoglobinopathy that causes cerebral complications in all age groups, which can be shared at a young age. Intravascular functions over time decrease as vaso-occlusion happens, and the elasticity ability of the vessels is depleting, thus resulting in poor perfusion in the cerebral tissue (Bernaudin et al., 2022). Vaso-occlusion decreases blood circulation that results in vessel wall damage combined with decreased elasticity after some time. The normal function of the vessels is disrupted and they are not capable of expanding and contracting to regulate blood flow patterns, intravascular functions. When the elasticity is reduced, it becomes very hard to perfuse the tissues because the vessels also become rigid, thereby causing poor blood circulation. Poor perfusion to a organ system can then lead to poor perfusion to a particular tissue and this may lead to poor oxygen and nutrient supply to the organ tissue causing organ injury and compromise of its neurologic function. One of the organs most affected by SCD is the kidney, which progressively damages the kidney tissue. Microalbuminuria is evident in childhood; as time passes, it damages the glomerular filtration rate (GFR) in early adulthood and continuously becomes prevalent (Ghosh, 2022). Good hydration orally or intravenously can be advised to patients in such conditions of the patient. As a patient survives through adulthood, progressive changes to the body happen. It is not fully known, but several factors have been highlighted, such as endothelial dysfunction from ischemia. Cardiac muscles compensate for the decreased ability of the body to produce needed oxygen requirements, thus resulting in tissue changes. An example of this is the cardiac muscles when a patient suffers pulmonary hypertension (PH), elevated tricuspid regurgitation jet velocity (TRV) and diastolic function, which is the most common contributory, identified correlated early mortality in adults. Diseased conditions associated in the pulmonary artery resistance raise the right ventricular pressure and hypertrophy (Inusa, 2019). Thus, as RV strain elevates diastolic function declines due to affection of the relaxation and filling processes. Increased TRV reveals high RV systolic pressure, worse PH and RV loads (Galiè et al., 2015). Diastolic dysfunction also plays in the development of RV failure and the escalating progression of PH (Manes et al., 2018). These factors are related to early mortality in patients with PH because they cause a reduction in the heart’s efficiency and heart failure [Galiè et al., 2015]. Chest painful crisis can be corrected in an emergency with potent opioids, according to clinician advice. Alongside adjuvant pain management such as NSAIDs (non-steroidal anti-inflammatory drugs) within 15 minutes of the patient's arrival in the emergency department, re-assessment should be done accordingly (RCEM 2024). Management starts with the preliminary assessment of pain using approved tools such as VAS or NRS as pointed by Thong et al. (2018). Pain relief, especially in severe cases, can be done through the use of opioids such as morphine or hydromorphone, and exist with the initial administration). Heat application and other related procedures including relaxation exercises are other significant methods that may help in preventing or alleviating the painful sensations as the patient takes medication. Thus, continuous monitoring will reveal the state of pain control and possible complications or the necessity of additional actions (Chou et al. 2016). Nurses also have to assess and alleviate patients’ psychological needs related to pain, including being able to impart knowledge to the patients about their condition.

A study by Klings (2022) stated that Asthma is a multifactorial contributing disease in SCD as it could be a hereditary or allergen-driven reaction. If possible, a routine chest x-ray can be done to see if a chest infection is present, as they are susceptible to infection. Providing oxygen therapy will help promote oxygen deprivation during a crisis like Minor episodes of nocturnal enuresis or priapism. Some patients might be too sensitive to divulge information (NICE guideline for Sickle Cell Disease, 2021). In the past, the expendability of oxygen constricting has been an essential treatment that disciplined and enhanced patient status in emergencies (Maynard, 2022). Disease progression of SCD should be closely monitored to have a basis on how to manage the signs and symptoms. Appropriate antimicrobials should be administered as suggested in the clinician's antibiotics prescribing guidelines. Patients with low haemoglobin levels will benefit from a blood transfusion, as a haematologist advises. Independent nursing interventions include keeping the patient warm, encouraging deep breathing, avoiding pain, and dressing in affected limbs for leg ulcers. Management does not stop upon discharge of the patient. Continuing support should be established. Another study by Lloyd (2018), in contrast, stated that Anemia is depicted as an important condition that requires transfusion and the use of blood transfusion is echoed as being practical, especially when done under the supervision of a haematologist. The patients also need complementary nursing interventions that offer warmth, adequate deep breathing, and pain control to improve the comfort and treatment of leg ulcers (Holloway et al., 2024). Moreover, as stated in the previous sections, the care should not be limited to their discharge from the hospital environment but rather continue further to cater for their needs until recovery and to avoid any further deterioration. Annual or regular GP consultations should be maintained to monitor signs and symptoms that are newly occurring so as to detect and update management. Medication Maintenance of hydroxyurea and other supplements, such as folic acid, should be considered. Keep immunisations up to date to avoid infections when travelling. Health education should be given to the patient to help them understand the dos and don'ts in their activities of daily living.

In conclusion, a holistic approach to management should be implemented as it does not only affect the physical aspect of the body or its bodily function, but it also affects one's mental state. It is a disfiguring and debilitating disease that profoundly affects a person. They might not know when or where the crisis occurs, making them more susceptible to emotional breakdown. Prevention is better than cure; thus, properly disseminating information will help increase awareness of SCD. Strengthen support groups, research funding and information on the importance of blood donation. 

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