Case Study: Roslyn, a six-year-old, is attending public school for the first time. Her parents brought her to the clinic and stated, “she appears to have pain in her legs and won’t participate in playtime.” The parents noticed recently Roslyn appears pale and acts weak. Roslyn is diagnosed with sickle cell anemia. After her parents and siblings undergo genetic

The initial interventions for this patient will be pain management and fluid replacement. After assessing the intensity of the pain, analgesics are administered to the patient. Non-pharmacological interventions, such as massage, heat and cold applications, and guided imagery, can also be applied. Intravenous fluid replacement, using normal saline, is done to combat dehydration and compensate for the ongoing loss.

Anticipatory Guidance

The nurse should explain the different types of sickle cell crises to Roslyn and her family. Furthermore, they should be taught about the trigger factors, their manifestations, and how to manage a crisis if it occurs (Morse et al., 2022). They should also be taught about maintaining their hydration status and how to watch out for signs of a stroke. When attending school, she should be allowed frequent restroom breaks and adjustments during physical education and recess activities.

Supporting the Child and Family

The nurse can support the child and family by providing them with resources on sickle cell anemia. They can teach them about its pathophysiology, clinical presentation, and management. The patient and family’s awareness and understanding of the disease process are important. It eases the treatment process and improves outcomes (Figueiredo et al., 2018). The occurrence of crisis is prevented, and if they occur, symptoms are identified early and managed appropriately.

Discharge Instructions

Roslyn should take plenty of water to maintain a good hydration status. The water should be taken frequently in small amounts and not a copious amount at once. She should avoid triggers of crisis, such as extreme temperatures or physical activity, stress, smoking, caffeine, or dehydration (Veluswamy et al., 2019). The family should be taught about the signs of stroke and the symptomatic presentation of crisis. They should also be taught about the initial steps of management before going to the hospital.

Activity Restrictions

Roslyn can only participate in moderate exercises such as swimming, cycling, running, and jumping. Moderate exercise improves blood flow in skeletal muscle. It also partially reverses small vessel discrepancies seen in patients with sickle cell disease (Merlet et al., 2020). Strenuous activity is a risk factor for the occurrence of sickle cell crises. Roslyn will also be unable to tolerate activity in extremely cold or warm temperatures. She will also have to avoid extreme activities that cause dehydration.

References

Figueiredo, S. V., Lima, L. A., Silva, D. P. B., Oliveira, R. D. M. C., Santos, M. P. D., & Gomes, I. L. V. (2018). Importance of health guidance for family members of children with sickle cell disease. Revista Brasileira de Enfermagem, 71, 2974-2982. https://doi.org/10.1590/0034-7167-2017-0806.

Merlet, A. N., Féasson, L., Bartolucci, P., Hourdé, C., Schwalm, C., Gellen, B., & EXDRE Collaborative Study Group. (2020). Muscle structural, energetic, and functional benefits of endurance exercise training in sickle cell disease. American journal of hematology, 95(11), 1257-1268. https://doi.org/10.1002/ajh.25936.