The Most common Complication Among Children With SCD
The Most common Complication Among Children With SCD
Management Of Pediatric And Adolescent Populations Wk 5 – Clinical Case SOAP Note 1. Identify a patient from your pediatric clinicals who provided you with a learning experience. 2. Write a complete SOAP note for the patient encounter (see scenario below). 3. Include the following: • A brief case scenario, including pertinent aspects of the history and physical exam • Your assessment and plan • The patient’s vaccine status, BMI percentiles, nutrient status, and development status 4. Use SOAP note TEMPLATE attached with 7th edition APA format. 5. References need listed in 7th edition APA format at end of SOAP note. 6. Minimum of 2 scholarly peer reviewed references are required. 7. References must be from within the last five years. 8. All treatment regimens, diagnosis, medications, diagnostic testing, etc… need to have proper reference citations for each item. 9. Textbook for NRP/543: Burns, C. E., Dunn, A. M., Brady, M. A., Barber Starr, N., Blosser, C. G., & Garzon, D. L. (2017). Pediatric primary care (6th ed.). Elsevier. Grading Criteria Weeks 5: Clinical Case SOAP Note Content: 20 points possible Points Possible Points Earned Provided patient history and physical exam 5 Provided assessment and plan 5 Included the following components in the SOAP note;
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• patient vaccine status • BMI percentile assessment • Nutrition intake and activity pattern •The Most common Complication Among Children With SCD developmental assessment 10 Office Visit Scenario • A 10-year-old child presents to the clinic for annual well child visit and • with the complaint of right thigh pain. This child is one of your well-known sickle cell disease (SCD) patients, having been followed by your practice since birth. His previous history includes two previous hospitalizations, once at 6 months for fever and another at 12 months for a swollen, painful left wrist. On examination, he is afebrile, but his heart rate is 130 beats/min. You notice tenderness to palpation over the right femur with an otherwise benign physical examination. • Summary: An otherwise healthy 10-year-old boy with known SCD is found to have pain localized to his right thigh and tachycardia. • Questions – What is the most likely diagnosis? Sickle Cell Disease with Vaso-Occlusive Crisis – What is the next step in the care of this patient? Discuss with parents the need to have patient hospitalized and send patient to local hospital to be admitted to administer intravenous fluids (IVF) and to provide pain management, possibly with narcotics. Call and request a bed and hand off given to acute care NP or MD. – What long-term strategies might be employed to prevent recurrence? Discuss with parents administration of hydroxyurea will increase the concentration of fetal hemoglobin, thus reducing the frequency of sickle cell crisis episodes. Objectives – Learn the common complications and treatment strategies for a child with SCD. – Become familiar with the goals of routine well-child (or health supervision) session for a patient with SCD. Considerations – Vaso-occlusive crisis is the most commonly experienced complication for children with SCD. Risk factors for development of this condition include increasing age and high baseline hemoglobin level. Episodes can be triggered by infection, stress, cold temperatures, or high altitude, but often no trigger can be identified for an episode. The site of pain may vary from child to child, but the most common sites are the extremities or back. For many children experiencing recurrent vaso-occlusive crises, the pain tends to occur in similar locations with repeat episodes. The severity of these episodes varies; some successfully treated outpatient. When oral medications are insufficient to control the pain, hospital admission for IVF and intravenous (IV) narcotic medication are required. Definitions – SICKLE CELL DISEASE (SCD): A group of disorders affecting at least one of the ?-globulin genes, resulting in some degree of sickling of the red blood cells (RBCs).The Most common Complication Among Children With SCD – VASO-OCCLUSIVE CRISIS: An episode of severe pain caused by increased sickling of RBCs, which leads to bone marrow ischemia and infarction. – DACTYLITIS: A form of vaso-occlusive crisis that involves painful swelling of hands and/or feet. – ACUTE CHEST SYNDROME (ACS): A new pulmonary infiltrate on chest x-ray (CXR) in addition to one of the following signs: fever, chest pain, shortness of breath, tachypnea, or low oxygen saturations. – APLASTIC CRISIS: Infection with parvovirus B19 (most commonly), which leads to temporary cessation of RBC formation. Reduced lifespan of RBCs in SCD coupled with reduced production may result in profound anemia. Clinical Approach – Vaso-occlusive crises are the most common complication among children with SCD. Pain is the most common presenting symptom, but patients may also have erythema, edema, or joint effusions near the site of the pain. No specific laboratory work is diagnostic of the crisis, but affected patients may have decreased hemoglobin or increased white blood cells (WBCs). Children whose pain is inadequately controlled with home medication regimens must be evaluated. Additional pain medications, such as morphine or hydromorphone, along with hydration, may be attempted in the outpatient setting. If more than one or two doses of these additional pain medications are required, inpatient hospitalization is required. Additional inpatient strategies include IVF (often given at higher than maintenance rates) and IV narcotics with doses and frequencies titrated to control the patient’s pain. RBC transfused typically are not used for simple vaso-occlusive crises, because they have not been found to be effective. Prevention of recurrent episodes of vaso-occlusive crises is attempted by avoidance of known triggers, as well as administration of hydroxyurea. This medication increases the concentration of fetal hemoglobin, thus decreasing sickling. – A patient with SCD with fever (with or without vaso-occlusive crisis) can have a medical emergency. Any SCD child presenting with a fever greater than 38.5°C is evaluated emergently, because SCD causes functional asplenia and predisposes patients to invasive encapsulated organisms (typically pneumococcal disease). In such situations complete blood count (CBC) and blood cultures are warranted, and if the screening results are concerning for infection without obvious source, empiric antibiotics are typically initiated (usually with ceftriaxone). The Most common Complication Among Children With SCD – A variety of other complications occur with SCD. Children with SCD who have significant respiratory symptoms, such as severe cough, shortness of breath, or chest pain may be exhibiting symptoms of acute chest syndrome. Criteria for diagnosis include new pulmonary infiltrate on chest radiograph in addition to one of the following: fever, dyspnea, tachypnea, chest pain, or decreased oxygen saturations. Because of potentially fatal complications of acute chest syndrome, immediate treatment is warranted and includes empiric antibiotics, supplemental oxygen, pain medications, and IVF. Close inpatient observation for respiratory failure is warranted. – Parents of the child with SCD are taught to palpate the abdomen of their younger children to observe for splenic enlargement. A child who has abdominal pain, distension, or acute enlargement of the spleen likely has acute splenic sequestration and requires hospitalization, possibly in the intensive care unit, to observe for cardiovascular collapse. Blood transfusions, perhaps even emergently, may be required and could potentially be lifesaving. As the child ages, the spleen usually auto-infarcts. Although lack of a functional spleen eliminates the complication of splenic sequestration, it also increases the odds of an infection with an encapsulated organism. – About 10% of children with SCD have acute strokes, with peak incident between 4 and 8 years of age. Symptoms might include paresis, aphasia, seizures, cranial nerve palsy, headache, or coma; all such children are admitted to the hospital. Emergency neuroimaging is warranted, repeated neurologic examinations are conducted, and partial or simple transfusions are performed to reduce the percentage of sickled cells. Physical therapy and rehabilitation are provided as the patient recovers. Chronic transfusions are instituted to reduce the risk of recurrence. As part of the routine well-child care of a patient with SCD, transcranial Doppler (TCD) ultrasonography is often recommended to identify those with increased flow velocity in the large cerebral blood vessels, and thus are at high-risk for developing a first stroke. Routine chronic transfusion among these high-risk children has resulted in reduced risk of first stroke. – A child with SCD who presents with a significant increase in pallor, fatigue, or lethargy may be exhibiting signs of aplastic crisis, most often caused by infection with parvovirus B19. These children will have a hemoglobin level below their normal baseline and a low reticulocyte count. They require hospitalization to observe for evidence of cardiovascular collapse, and blood transfusions may be required. – A boy with SCD who has a priapism episode persisting for more than 3 to 4 hours must be evaluated by a urologist. Intravenous fluid hydration and pain control are provided; ice is not to be used. The urologist may be required to aspirate and irrigate the corpora cavernosa to achieve detumescence. Failure of three or four aspirations in the outpatient setting requires more extensive inpatient management, including exchange blood transfusions, further pain control, and additional surgical intervention. – Children with SCD require multiple evaluations by both primary care providers and hematologists per year, especially when young, and should receive screening for the various known complications of the disease. Goals of a health supervision visit for all children, including those with sickle cell and other diseases, incorporate evaluating a child’s physical, developmental, psychosocial, and educational status to identify problems early. Prompt intervention can then be instituted. Anticipatory guidance aims to foster good health habits, prevent illness, and assist in family communication. For the child with a diagnosis such as SCD, another important part improving the overall good health of the patient is to ensure he or she is linked to a comprehensive SCD program. – Routine care for children with SCD includes initiation of prophylactic penicillin as early as possible, the pneumococcal PCV13 series at 2, 4, 6, and 12 to 15 months, and the pneumococcal and meningococcal vaccines starting at 2 years of age. Frequent CBCs are performed, and renal, liver, and lung function are monitored annually beginning at 1 year of age. Parents must also take an active role in the child’s health care supervision, and his or her education is an important part of the well-child checks. Routine spleen palpation should be performed at home and enlargement should prompt an urgent evaluation. Parents should also check temperatures with any illness and bring the child to the primary care physician (PCP) or emergency department (ED) with a temperature of 38.5°C or higher. Clinical Pearls • Children with SCD who have fever (risk of sepsis), pallor (aplastic crisis), abdominal pain or distension (splenic sequestration), pain crisis, evidence of lower respiratory disease (acute chest syndrome) priapism, new neurologic findings (stroke), or dehydration must be evaluated urgently. • Additions to routine care required for all children include initiation of penicillin and folate therapies, as well as administration of meningococcal and polysaccharide vaccines at earlier than typical ages. • A variety of screening tests, such as routine CBC and reticulocyte measurements, begin at 2 months of age or at diagnosis The Most common Complication Among Children With SCD.
