IndexCase StudyPathophysiologyManifestations and DiagnosisPhysical ExaminationLaboratory Work and DiagnosticsTreatmentComplicationsEducationConclusionReferencesCase StudyA 19-year-old African-American female, hereafter referred to as “AP,” presents to her primary care physician with fatigue, fever and right upper quadrant (RUQ) abdominal pain for 3 days. AP has a significant medical history of sickle cell anemia (SCA). AP also complains of generalized “bone crushing” pain rated 10/10 intermittently, with notable swelling in 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 the sickle cell trait. This disease is usually diagnosed in early childhood and the associated symptoms can be debilitating. The purpose of this article is to discuss SCA in general and with regards to the case study above. Say no to plagiarism. Get a tailor-made essay on "Why Violent Video Games Shouldn't Be Banned"? Get an original essay Pathophysiology Sickle cell anemia is an autosomal recessive disease located on chromosome 11p15.5 that affects the functioning of red blood cells. Worldwide, sickle cell disease affects millions of lives and is most often found in people with ancestry from Africa, although individuals from countries in the Mediterranean, Arabian Peninsula, South America, Central America and the Caribbean may also be affected. Sickle cell anemia is a general term for individuals who express an abnormal gene that causes the body to produce hemoglobin (Hb) S. The different types of sickle cell anemia are Hb SβO thalassemia, Hb Sβ+ thalassemia, HbSC, the HbSD, the HbSE, and the HbSS ( NHLBI, n.d.). AP has 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 to have SCD (NHLBI, n.d.). AP's parents may have had SCD or sickle cell trait to pass it on to their children. Individuals with sickle cell trait may not know they have it unless they are tested through newborn screening or their parents are aware of their status. Individuals with sickle cell trait are rarely symptomatic due to other types of Hb in the cell. Sickle cell anemia is characterized by the irregular shape of Hb, which causes stiffness in red blood cells and "C"-shaped or sickle-shaped red blood cells. The sickle shape of red blood cells is due to the fact that Hb S is less soluble when deoxygenated, which causes it to precipitate and polymerize at a faster rate. Oxygen is moved throughout the body with the help of Hb, a protein found in healthy red blood cells (ASH, 2018). Injury to red blood cells causes hemolytic anemia, microvascular occlusions, disorders, and tissue damage that can lead to organ failure. Polymerization of hemoglobin S, alteration of 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 sickle cell disease. Individuals with sickle cell disease and sickle cell trait have been found to have resistance to falciparum malaria and have fewer parasites (Serjeant, 2013). While there are several theories as to why individuals with sickle cell trait are resistant to malaria, one theory states that when an individual is infected with malaria, the abnormal Hb sickles along with the malaria parasite and is subsequently removed by macrophages. AP has not traveled out of the country recently, and therefore malaria is not included in the differential diagnosis. Manifestations and diagnosis AP comessent to emergency room to be evaluated for sickle cell crisis and possible cholecystitis. Physical exam AP appears to be in severe distress. AP describes her pain as generalized but worsening intermittent pain in the arms, feet, and RUQ of the abdomen. AP reports that he is currently experiencing severe stress at school. AP states that it feels like "a seizure," but her abdomen doesn't usually hurt "that much" and she now suffers from nausea. AP states that the pain lasts for 3 days and gets worse after eating. AP brought with him a copy of his medical records. Note that he has the HbSS genotype, also known as SCA. Hands and feet are noted to be swollen, and AP reports that "pain worsens with joint movement." Additionally, ulcerations of the lower extremities are noted, which may be a chronic manifestation of sickle cell disease (Linder & McCance, 2019). Initial vital signs for AP are: pulse 115 bpm, respirations 24, blood pressure 120/79, temperature 100.9, pulse 96% on room air. On examination, her abdomen is mildly distended and tender to palpation at the RUQ with a positive Murphy sign. Since acute chest syndrome (ACS) can be a life-threatening complication seen in vaso-occlusive crisis, it is important to evaluate the patient and recognize the most common signs and symptoms associated with this condition such as cough, hemoptysis, shortness of breath, pain chest pain, low oxygen saturation, wheezing, or other abnormal lung sounds. Acute chest syndrome is the number one cause of premature death in patients with SCD, so a thorough workup is necessary when patients present with fever, hypoxia, or new-onset infiltrate on chest x-ray. An acute drop in Hg in patients with sickle cell disease may indicate worsening of the disease or the presence of other complications such as ACS, hepatic or splenic seizures (Simon et al., 2016). In this case, AP does not appear to have shortness of breath, his oxygen saturation is normal, lung sounds are clear bilaterally, and he denies any cough, shortness of breath, or pain radiating to the chest. AP also denies any prior history of ACS. However, further testing is needed to rule out ACS. The differential diagnosis for AP is: vaso-occlusive crisis, acute chest syndrome, infection due to impaired splenic function, cholecystitis, acute sickle cell liver crisis.Laboratory work and diagnosticsA Complete blood count, liver function panel, coagulation studies, type and screening , a chest x X-ray, RUQ ultrasound (US), and continuous oxygen monitoring are indicated. Leukocytosis with left shift is noted. Hemoglobin is 6.5 g/dl and hematocrit is 25%, values ​​consistent with previous laboratory values ​​during the AP crisis according to his medical record. Liver function, coagulation tests, and chest x-ray are within normal limits. Oxygen saturation in ambient air continues to be >95%. Ultrasound of the right upper quadrant shows thickening of the gallbladder wall and pericholecystic fluid. Because abdominal pain is common in patients with sickle cell disease, it is important to identify its cause. In this case, the pain is localized to the RUQ, therefore diagnostic tests are needed to distinguish between 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 increased white blood cell count, increased transaminases, thickening of the gallbladder wall, pericholecystic fluid or duct dilation common biliary (Simon et al., 2016) . Acute sickle liver crisis tends to have a similar presentation to cholecystitis, however in acute sickle liver crisishepatomegaly. Furthermore, it is recommended to obtain prior laboratory tests to establish a baseline of the patient's Hb levels and liver function and thus identify acute processes. In the present case study, AP was diagnosed with a vaso-occlusive crisis complicated by cholecystitis. Treatment AP was then admitted to hospital for treatment of vaso-occlusive crisis and cholecystitis. Due to the crisis and the high probability of impending surgery, the patient's treatment included a blood transfusion. A blood transfusion would contain normal Hb, which would then survive longer in the circulatory system decreasing the risk of blockage and increasing oxygen delivery to the tissues (NHLBI, n.d.). Before surgery, sickle cell patients may receive blood transfusions to prevent further postoperative complications. Medication management would be initiated for AP to prevent further sickling and tissue damage complications. With episodes of severe pain, interventions would include pain management, hydration, and possibly the use of anti-inflammatory agents. Frequent pain assessments and management would be required for PsA. A multidisciplinary approach would be appropriate for pain management. Often, patients out of the hospital treat their pain with over-the-counter ibuprofen or aspirin, but due to the severity of PsA pain an opioid pain reliever will be needed. 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 cells. AP does not take hydroxyurea as a home medication. AP says she "stopped taking it a year ago because she would forget to take it due to school stress." Since AP had the complicated signs of cholecystitis, an operation may be necessary. The most standard procedure would be a laparoscopic cholecystectomy. The use of prophylactic antibiotics for AP would be necessary because patients with SCD are more susceptible to infections due to abnormal or absent spleen function. Broad-spectrum antibiotics, such as cephalosporins alone or in combination with gentamicin, may be a treatment option for AP. During AP cholecystectomy, special intraoperative care must be provided to keep the AP warm and prevent further occlusion. A thermal cover can be used to keep the patient warm. Postoperative AP would require oxygen therapy, adequate pain control, and continued fluid administration may be necessary for up to 1-2 days. Frequent evaluations and laboratory monitoring would be necessary to evaluate for signs and symptoms of infection and further complications. Complications Sickle cell disease, or sickle cell disease, can increase the risk of complications including: stroke, pulmonary hypertension, organ damage, blindness, vascular damage, gallstones. , etc. For the purposes of this case study, AP will need to be monitored specifically for pulmonary, vascular, and neurological complications. Pulmonary complications include acute chest syndrome and pulmonary hypertension. Acute chest syndrome is the second most common reason for hospitalization of patients with sickle cell disease. Due to sickling of red blood cells, blockages occur in organ systems, including the lungs, and this damage leads to increased pressure in the lungs. Pulmonary hypertension affects approximately 10% of adults with sickle cell disease, particularly pulmonary arterial hypertension. Vascular complications related to hemolysis in SCD may include leg ulcers andpriapism (Dubert et al., 2016). Complications related to low Hb levels in sudden cardiac death include albuminuria and leg ulcers (Dubert et al., 2016). As stated previously, shearing of red blood cells can cause clumping of vessels and lead to vascular occlusion, leading to further complications. Neurological complications can occur in SCD because sickle cells can damage vessels when they clump 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 MCI patients, including children, and that this can result in cognitive decline (Venkataraman & Adams, 2014). Prevention includes screening by age 2 and, if deemed high risk, the administration of regular blood transfusions. Prevention in adults has not yet been developed. AP escaped any major complications. AP tolerated surgery and blood transfusions well and recovered in 2 days in a Med-Surg unit. His pain has decreased to 5/10 generalized and is managed with anti-inflammatory medications. AP remains afebrile and tolerates soft foods well without complaining of nausea. AP is now ready for discharge planning, which includes discharge education and instructions. Education Education for AP will focus on post-cholecystectomy instructions, future infection prevention, and stress and health management with sudden death. The Centers for Disease Control and Prevention website (CDC, 2018a) contains several educational materials for sudden death patients and could be used as a free online resource. After cholecystectomy, the AP will need to follow a specific diet to reduce the risk of diarrhea or other gastrointestinal (GI) symptoms. To reduce the risk of gastrointestinal symptoms, the AP should be encouraged to follow a low-fat diet for at least one week after surgery. Patients with a history of SCD should be encouraged to drink 8-10 glasses of water per day. This would also help prevent dehydration if AP has diarrhea. To help prevent infection, APs are asked to wash their hands often, especially before preparing or eating food and after using the bathroom (CDC, 2018c). AP was advised that the use of hand sanitizer is acceptable, but that hand washing to prevent transmission of pathogens is best practice. AP would be asked to continue taking all prescribed antibiotics until they wear off, to monitor surgical incision sites for inflammation or drainage, and to immediately report any signs of infection to his doctor. If a patient experiences future complications with sudden cardiac death, such as sudden, uncontrolled pain, chest pain, severe headache, or vision problems, they should see a doctor. To help prevent future MCI flare-ups, stress and health management should be a priority. As a college student, AP previously said she was "going through a lot of stress in school." To specifically manage stress, some interventions may include: scheduling rest times during daily activities when possible, creating an on-campus support network of people who are aware that AP has SCD and who can be supportive if is overwhelmed, establishing a working relationship with an academic advisor to support PA in an emergency and joining a support group with other college students with SCD (CDC, 2018e). AP was already putting into practice some of the recommendations to improve the quality of life with SCD, seekingmedical assistance when symptoms appear and carrying a copy of your medical records. Other considerations AP would be encouraged to make are staying active with exercises that don't make her too hot or cold or fatigued, eating a healthy diet with plenty of fruits and vegetables, increasing her water intake, and consistently following up with her primary care doctor or hematologist to manage the SCA. Please note: this is just a sample. Get a custom paper from our expert writers now. Get a Custom Essay Conclusion In summary, AP was evaluated and treated for vaso-occlusive crisis and cholecystitis related to her history of ACS. She tolerated pain management, intravenous hydration, and surgical treatment well and was discharged from the hospital. Upon checking with his hematologist, AP states that he has finished antibiotics, has had fewer loose stools recently, and denies abdominal pain. AP denies fever or signs of infection and says he tried to drink plenty of water. AP says his pain is controlled at home with anti-inflammatory medications. AP has rearranged some of his school schedule and will finish the semester successfully with his stress better managed. AP says he will consider resuming his prescription hydroxyurea, which his hematologist encourages. Sickle cell disease can be painful and debilitating, but with proper management of symptoms a patient can have the chance to live a full life. BibliographyAl 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 anemia [Website]. Retrieved from https://www.hematology.org/Patients/Anemia/Sickle-Cell.aspxBender, M.A. (2017). Sickle cell anemia. Retrieved fromhttps://www.ncbi.nlm.nih.gov/sites/books/NBK1377/Centers for Disease Control and Prevention [CDC]. (2017). Sickle Cell Disease Complications and Treatments [Website]. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/treatments.html#PainCenters for Disease Control and Prevention [CDC]. (2018a). Sickle cell anemia (sickle cell disease) [Website]. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/materials/factsheets.htmlCenters for Disease Control and Prevention [CDC]. (2018b). Living Well with Sickle Cell Anemia [PDF Document]. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/documents/tipsheets_living.pdfCenters for Disease Control and Prevention [CDC]. (2018c). 5 Tips to Prevent Infection [PDF Document]. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/documents/tipsheets_5.pdfCenters for Disease Control and Prevention [CDC]. (2018d). Emergency guide: when to consult your doctor [PDF Document]. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/documents/tipsheets_guide.pdfCenters for Disease Control and Prevention [CDC]. (2018e). 9 tips for living well with sickle cell disease at university [PDF Document]. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/documents/SCD-factsheet_9steps.pdfDubert, M., Elion, J., Tolo, A., Diallo, D.A., Diop, S., Diagne, I. , … & Ranque, B. (2016). Degree of anemia, indirect markers of hemolysis and vascular complications of sickle cell disease in Africa. American Society of Hematology, 130, 2215-2223. doi:10.1182/blood-2016-12-755777Ghosh, S., Adisa, O., Chappa, P., Tan, F., Jackson, K., Archer, D. R., & Ofori-Acquah, S. F. (2013). Extracellular hemin crisis triggers acute chest syndrome in sickle cell mice. Journal of Clinical Investigation, 123(11), 4809-4820. doi:10.1172/JCI64578Gordeuk, V., Castro, O. & Machado, R. F. (2016). Pathophysiology and treatment of pulmonary hypertension in sickle cell anemia. Blood, 127, 820-828. doi:10.1182/blood-2015-08-618561Howard, J., Hart, N., Roberts-Harewood, M.,, 37(4), 171-177..2014.01.008