Question

Map a nursing care plan/clinical pathway for a patient with a specific genetic disorder (e.g., sickle cell disease, hemophilia, cystic fibrosis, Huntington disease). Prepare to discuss your care plan and rationales following a minimum of three nursing diagnosis for the disease specified.

Answer 1

SICKLE CELL DISEASE:

Definition: Sickle cell disease is a disorder of blood caused by an inherited abnormal hemoglobin which distorts the red blood cells.

Care Plan for Sickle Cell Disease:

S.NO	Nursing diagnosis	Nursing interventions	Rationale
1	Acute pain	Assess for pain Use alternative pain relief measures. Maintain hydration. Apply warm and moist compress oer the painful site. Administer pain medications as indicated.	Sickling of cells potentiates cellular hypoxia that causes infaraction of tissues causing pain. This will reduce the dependency on pharmacological methods to control pain. Dehydration precipitates crisis. Promotes vasodialtion and increases circulation to hypoxic areas. Reduces pain and promotes rest.
2	Impaired gaseous exchange	Check for respiratory rate, depth, use of accessory muscles. Schedule rest periods and encourage to alternate rest and activity. Administer supplemental humidified oxygen. Assist with chest physiotherapy. Administer blood products as per the report of CBC	They are thge indicators for adequecy of respiration. Reducing tghe metabolic demands of the body will reduce the oxygen demand. Supplemental oxygen maximizes the transport of oxygen to tissues. Mobilizes secretions and increases aeration of lung fields. Increases the number of oxygen carrying cells and dilutes the percentage of HbS to prevent sickling.
3	Risk for deficit fluid volume	Maintain adequate I & O and daily weight. Monitor skin turgor and mucus membrane Monitor serum electrolytes. Administer IV fluids as indicated.	Will help to manage dehydration resulting from excessive diarrhea and vomiting on time. This indicates dehydration and signs of vaso occlusive crisis. IV fluid replaces the loss and fills the deficit.