Table of ContentsCase StudyPathophysiologyManifests and DiagnosisPhysical ExaminationLaboratory Work and DiagnosisTreatmentComplicationsEducationConclusionReferencesCase StudyA 19-year-old African American woman, hereafter referred to as “AP,” presents to her treating physician with fatigue , fever and abdominal pain in the right upper quadrant (RUQ) for 3 days. AP has a significant medical history of sickle cell disease (SCA). AP also complains of “crushing” generalized pain rated 10/10 intermittently, with notable swelling of bilateral hands and feet. Sickle cell disease (SCD) affects approximately 70,000 to 100,000 Americans. It is estimated that one to three million Americans carry sickle cell trait. This condition is usually diagnosed in early childhood and associated symptoms can be debilitating. The purpose of this article is to discuss SCA in general and as it relates to the above case study. Say no to plagiarism. Get a tailor-made essay on “Why Violent Video Games Should Not Be Banned”? Get the original essay Pathophysiology Sickle cell disease is an autosomal recessive disorder located on chromosome 11p15.5 affecting the function of red blood cells. Worldwide, sickle cell disease affects millions of lives and is most commonly seen in people whose ancestors originated in Africa, although people from Mediterranean countries, the Arabian Peninsula, South America, Central America and the Caribbean may also be affected. Sickle cell disease is an umbrella term for people who express an abnormal gene that causes the body to produce hemoglobin (Hb) S. The different types of SCD are Hb SβO thalassemia, Hb Sβ+ thalassemia, HbSC , HbSD, HbSE and HbSS ( NHLBI, nd). AP contains HbSS, which is the most common and severe form of SCD. Other types of SCD are not as common. AP inherited an abnormal Hb gene from each parent in order to have SCD (NHLBI, nd). Parents of AP may have had sickle cell disease or sickle cell trait themselves to pass it on to their children. People with sickle cell trait may not know they have it unless they are tested during a newborn screen or their parents know their own status. People with sickle cell trait are rarely symptomatic due to other types of Hb in the cell. Sickle cell disease is characterized by the irregular shape of Hb, causing stiffness of the red blood cells and "C"-shaped or sickle-shaped red blood cells. The sickle shape of red blood cells is because Hb S is less soluble when deoxygenated, causing it to precipitate and polymerize more quickly. Oxygen circulates throughout the body with the help of Hb, a protein found in healthy red blood cells (ASH, 2018). Red blood cell injury causes hemolytic anemia, microvascular occlusions, tissue disruption and damage that can lead to organ failure. Hemoglobin S polymerization, impaired blood flow caused by vascular occlusion, hemolysis-mediated endothelial dysfunction, and activation of sterile inflammation are four major developments involved in a vicious cycle that constitutes the SCD. Individuals with SCD and sickle cell trait exhibit resistance to falciparum. malaria and have a lower number of parasites (Serjeant, 2013). Although there are different theories as to why individuals with sickle cell trait exhibit resistance to malaria, one theory states that when an individual is infected with malaria, the abnormal Hb hastends to sickle with the malaria parasite and is then eliminated by malaria. macrophages. AP has not traveled outside the country recently and therefore malaria is not included in the differential diagnosis. Manifestations and diagnosis AP is referred to the emergency room to be evaluated for sickle cell crisis and possible cholecystitis. Physical Examination AP appears to be in severe distress. AP describes his pain as intermittent generalized pain but worsening in the arms, feet, and RUQ of the abdomen. AP reports that she is currently going through major stress at school. AP states that it feels like "a seizure" but that her abdomen usually doesn't hurt "this much" and that she is now experiencing nausea. AP states that the pain has lasted for 3 days and is worse after eating. AP brought with her a copy of her medical records. She is noted to have the HbSS genotype, also known as SCA. The hands and feet appear swollen, and AP reports that "the pain worsens with joint movement." Additionally, lower extremity ulcerations are noted, which may be a chronic manifestation of sickle cell disease (Linder & McCance, 2019). The initial vital signs of PA are: pulse 115 bpm, respirations 24, blood pressure 120/79, temperature 100.9, pulse 96% on room air. On examination, his abdomen is slightly distended and tender to palpation on the RUQ with a positive Murphy. sign. Since acute chest syndrome (ACS) can be a life-threatening complication seen during a vaso-occlusive crisis, it is important to evaluate the patient and recognize the most common signs and symptoms associated with this condition, such as such as cough, hemoptysis, shortness of breath, chest pain, low oxygen saturation, wheezing or other abnormal lung sounds. Acute chest syndrome is the leading cause of premature death in patients with sickle cell disease. A thorough examination is therefore necessary when patients present with fever, hypoxia or a new infiltrate on chest x-ray. An acute drop in Hg levels in patients with sickle cell disease may indicate worsening disease or the presence of other complications such as ACS or hepatic or spleen sequestrations (Simon et al., 2016). In this case, AP does not appear to have labored breathing, her oxygen saturation is normal, lung sounds are clear bilaterally, and she denies any cough, shortness of breath, or radiating chest pain. AP also denies any history of SCA. However, further testing is needed to rule out ACS. The differential diagnosis of AP is: vaso-occlusive crisis, acute chest syndrome, infection due to impaired splenic function, cholecystitis, acute sickle liver crisis. Laboratory work and diagnosticsA CBC, liver function panel, coagulation studies, typing and screening, chest x. -ray, RUQ ultrasound (US) and continuous oxygen monitoring are indicated. There is leukocytosis with a shift to the left. Hemoglobin is 6.5 g/dL and hematocrit is 25%, consistent with previous laboratory values ​​during a PsA attack, according to his medical record. Liver function, coagulation studies, and chest x-ray are within normal limits. The oxygen saturation of the ambient air continues to be >95%. Ultrasound of the right upper quadrant shows thickening of the gallbladder wall and pericholecystic fluid. Since abdominal pain is common in patients with sickle cell disease, it is important to identify the cause. In this case, the pain is localized to the RUQ, so diagnostic tests are necessaryto differentiate cholecystitis, cholelithiasis or acute sickle liver crisis. The clinical presentation of acute cholecystitis includes RUQ pain, fever, nausea, and vomiting. Laboratory and imaging studies may show elevated white counts, elevated transaminases, thickening of the gallbladder wall, pericholecystic fluid, or dilation of the common bile duct (Simon et al., 2016). . Acute sickle cell liver attack tends to have a presentation similar to that of cholecystitis, however hepatomegaly is seen in acute sickle cell liver crisis. Additionally, it is recommended to obtain prior laboratory testing to establish a baseline of a patient's Hb levels and liver function and thus identify acute processes. In the present case study, AP was diagnosed with vaso-occlusive crisis complicated by cholecystitis. TreatmentAP was subsequently admitted to hospital for treatment of a vaso-occlusive crisis and cholecystitis. Due to the seizure and the high likelihood of impending surgery, the patient's treatment would include a blood transfusion. A blood transfusion would contain normal Hb, which would then survive longer in the circulatory system, reducing the risk of blockage and increasing oxygen delivery to the tissues (NHLBI, nd). Before surgery, patients with sickle cell anemia may receive blood transfusions to avoid further postoperative complications. Medication management would be initiated for PA to prevent further sickling and complications of tissue damage. In cases of severe pain episodes, interventions would include pain management, hydration, and possibly the use of anti-inflammatory agents. Frequent pain assessments and management would be necessary for PsA. A multidisciplinary approach would be appropriate for pain management. Often, out-of-hospital patients treat their pain with over-the-counter ibuprofen or aspirin, but due to the severity of PsA pain, an opioid pain reliever will be necessary. The drug hydroxyurea is an example of a home medication used to prevent seizures in patients with sickle cell disease. Hydroxyurea stimulates fetal Hb production, which prevents the production of sickle cell disease. AP does not take hydroxyurea as a home medication. AP states that she "stopped taking it a year ago because she would forget to take it due to school stress." As AP showed complicated signs of cholecystitis, an operation might be necessary. The most standard procedure would be a laparoscopic cholecystectomy. The use of prophylactic antibiotics for AP would be necessary because patients with sickle cell disease are more susceptible to infection due to abnormal or absent spleen function. Broad-spectrum antibiotics, such as cephalosporins alone or in combination with gentamicin, may be a therapeutic option for AP. During cholecystectomy of the PA, special intraoperative care should be provided to keep the PA warm and avoid subsequent occlusion. A thermal blanket can be used to keep the patient warm. Postoperatively, PA would require oxygen therapy, adequate pain control, and fluids may need to be continued for 1 to 2 days. Frequent assessments and laboratory monitoring would be necessary to evaluate for signs and symptoms of infection and other complications. Complications Sickle cell disease, or sickle cell disease, can increase the risk of complications, including: strokecerebral, pulmonary hypertension, organ damage, blindness, vascular damage, gallstones. , etc. For the purposes of this case study, BP will need to be monitored specifically to detect pulmonary, vascular, and neurological complications. Pulmonary complications include acute chest syndrome and pulmonary hypertension. Acute chest syndrome is the second leading cause of hospitalization for sickle cell patients. Due to sickling of red blood cells, occlusions will occur in the organ systems, including the lungs, and this damage will lead to increased pressure in the lungs. Pulmonary hypertension affects approximately 10% of adults with sickle cell disease, particularly pulmonary hypertension. Vascular complications related to hemolysis in SCD can include leg ulcers and priapism (Dubert et al., 2016). Complications related to low Hb levels in MCS include albuminuria and leg ulcers (Dubert et al., 2016). As previously noted, sickling red blood cells can cause clumping in the vessels and lead to vascular occlusion, leading to further complications. Neurological complications can occur in SCD because sickle cells can damage vessels when they cluster together. The risk of stroke is the most serious of these neurological complications. It has been shown that heart attacks and brain atrophy are seen in patients with sickle cell disease, including children, and this can lead to cognitive decline (Venkataraman & Adams, 2014). Prevention includes screening from the age of 2 and, if the risk is considered high, regular blood transfusions. Prevention in adults is not yet developed. AP escaped any major complications. AP tolerated the operation and blood transfusions well and recovered within 2 days in a Med-Surg unit. His pain has decreased to an overall level of 5/10 and is managed with anti-inflammatory medications. AP remains afebrile and tolerates soft foods well without any complaints of nausea. AP is now ready for discharge planning, which includes discharge instructions and education. EducationEducation for AP will focus on post-cholecystectomy instructions, preventing future infections, and managing stress and health with SCD. The Centers for Disease Control and Prevention website (CDC, 2018a) offers several educational materials for patients with sickle cell disease and could be used as a free online resource. After a cholecystectomy, AP will need to follow a specific diet to decrease the risk of diarrhea or other gastrointestinal (GI) symptoms. To reduce the risk of gastrointestinal symptoms, the PA should be encouraged to follow a low-fat diet for at least one week postoperatively. Patients with a history of sickle cell disease should be encouraged to drink 8 to 10 glasses of water per day. This would also help prevent dehydration if AP experiences diarrhea. To help prevent infection, AP should wash their hands frequently, especially before preparing or eating food and after using the restroom (CDC, 2018c). AP is advised that use of hand sanitizer is acceptable, but hand washing to prevent transmission of pathogens is best practice. AP would be responsible for continuing to take all prescribed antibiotics until they wear off, monitoring surgical incision sites for inflammation or drainage, and immediately reporting any signs of infection to his doctor. If a patient experiences future complications related tosickle cell disease, such as sudden, uncontrolled pain, chest pain, severe headache, or vision problems, he should consult a doctor. To help prevent a future SCD attack, stress and health management should be a priority. As a student, AP previously said she was "undergoing major stress at school." To specifically manage stress, some interventions may include: providing time for rest during daily activities when possible, creating an on-campus support network of people who are aware that AP has sickle cell disease and who can provide support. support if overwhelmed, establish a working relationship with an academic advisor to advocate for PA in an emergency, and join a support group with other students with SCD (CDC, 2018e). AP was already putting into practice some of the recommendations to improve quality of life with sickle cell disease, seeing a doctor when she had symptoms and carrying a copy of her medical records with her. Other considerations that AP would be encouraged to make are to stay active with exercises that will not make AP too hot, too cold or too tired, to eat a healthy diet with lots of fruits and vegetables, to increase her intake water and systematically follow primary care. doctor or hematologist to manage ACS.Keep in mind: This is just a sample.Get a personalized article now from our expert writers.Get a personalized essayConclusionIn summary, AP has been evaluated and treated for a vaso-occlusive crisis and cholecystitis linked to his history of SCA. . She tolerated pain management, IV hydration, and surgical treatment well, and was discharged from the hospital. During follow-up with her hematologist, AP states that she has finished her antibiotics, has had fewer loose stools recently, and denies abdominal pain. AP denies fever or signs of infection and says she tried to drink plenty of water. AP says her pain is controlled at home with anti-inflammatory medications. AP has reorganized part of his school schedule and will successfully complete the semester with better managed stress. AP states that she will consider resuming her prescribed hydroxyurea, which is encouraged by her hematologist. Sickle cell disease can be painful and debilitating, but with proper symptom management, a patient can have the opportunity to live a full life. References Al Talhi, Y., Shirah, B. H., Altowairqi, M., & Yousef, Y. (2017). Laparoscopic cholecystectomy for cholelithiasis in children with sickle cell disease. Clinical Journal of Gastroenterology, 10(4), 320-326. doi:10.1007/s12328-017-0750-3American Society of Hematology [ASH]. (2018). Sickle Cell Disease [Website]. Retrieved from https://www.hematology.org/Patients/Anemia/Sickle-Cell.aspxBender, M.A. (2017). Sickle cell anemia. Retrieved from https://www.ncbi.nlm.nih.gov/sites/books/NBK1377/Centers for Disease Control and Prevention [CDC]. (2017). Complications and treatments for sickle cell disease [Website]. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/treatments.html#PainCenters for Disease Control and Prevention [CDC]. (2018a). Sickle Cell Disease (SCD) [Website]. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/materials/factsheets.htmlCenters for Disease Control and Prevention [CDC]. (2018b). Living well with sickle cell disease [PDF document]. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/documents/tipsheets_living.pdfCenters for Disease Control and Prevention [CDC]. (2018c). 5 Tips to Help Prevent Infections [PDF Document]. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/documents/tipsheets_5.pdfCenters for Disease Control and Prevention [CDC]. (2018d). Emergency Guide: When, 37(4), 171-177..2014.01.008