Sickle Cell Anemia Case Study

Posted by: Daisy Jane, RN, MN October 16, 2010 Comments (0) 5

Definition Sickle cell anemia is an inherited disorder on the beta chain of the hemoglobin resulting to abnormally shaped red blood cells. RBCs assume a crescent or C-shape that decreases the cells life span and ability to function thereby, producing various complications. In some cases, these sickled cells may block the flow causing pain and organ damage. Incidence

Equal male-female ratio Increase incidence in African Americans

Etiology

Autosomal recessive disorder

Review of Related Anatomy and Physiology Red blood cells or erythrocytes carry oxygen to the different parts of the body. Different from other cells, RBCs do not contain a nucleus (anucleated). These small cells are circular and flattened with depressed centers on both sides resembling to that of a doughnut when viewed under a microscope. Their size and shape provides a large surface area for carrying oxygen in relation to their volume. The normal RBC count is 4-6 million/mm3. RBCs has the life span of 120 days. Hemoglobin is a form of protein that contains iron which is responsible for transporting oxygen that is carried in blood. Adult hemoglobin contains a beta chain (HBB) while a fetus hemoglobin has a gamma chain. Hemoglobin is comprised of four protein (amino acid) components. It has two beta-globin and two alpha-globin. The subunit beta-globin is located inside the RBCs. These amino acids carry an iron-bearing molecule called heme. Heme molecules, which are only found in mature RBCs, enables the erythrocytes to pick oxygen from the lungs and transport it throughout the body. Once oxygen attaches to hemoglobin it gives the blood its bright red pigment. The more hemoglobin molecules the RBC contain, a higher amount of oxygen will they be able to carry. If the hemoglobin is defective, the erythrocyte will also malfunction. A red blood cell is just a vessel; the one that performs the oxygen transportation is the hemoglobin. Normal hemoglobin is 13-18 grams/100 ml of blood in males and 12-16 grams in females. Pathophysiology Erythrocytes in sickle cell anemia contain abnormal hemoglobin that affects the beta-chain producing hemoglobin S or HbS. In this disorder, the beta-chains (beta-globins) are replaced by Hemoglobin S. Valine (an amino acid) takes the place of the normally appearing glutamic acid in beta-chains. Replacement of glutamic acid with valine causes the polymerization of HbS components to cohere forming long and insoluble particles. These distort the red blood cells, which assumes an inflexible crescent or sickle shape. The abnormally shaped cells become sharp and spiky when the RBCs are discharging oxygen molecules and in cases where the oxygen content in blood is low such as performing vigorous exercise and being in high altitude areas. Typically, a sickled cells lifespan is only 20 days. The deformed erythrocytes also rupture easily and they tend to be trapped in the microcirculation, obstructing blood flow and oxygen transport that might lead to painful episodes of ischemic injury. Sickle cell crisis refers to episodes of acute and severe sickling that blocks the circulation posing a threat of extensive organ damage. Severe pain is noted during these incidents caused by occluded vessels in the bone possibly resulting to bone necrosis. The crisis is triggered by hypoxemia, acidosis, or other conditions such as dehydration, infection, vigorous exercise, pregnancy or cold weather.

A condition called a sickle cell trait is identified by the presence of a single defective gene, instead of two. Individuals with this trait are essentially normal however, they are carriers. Meaning two sickle cell trait carrier parents can contribute a defective gene to a child that will carry the sickle cell disorder. Diagnosis Prenatal:

Chorionic Villi sampling Amniocentesis (blood from the cord)

At birth:

Newborn Screening Hemoglobin electrophoresis

Signs and Symptoms The following manifestations are observed in children with a sickle cell disorder, at about 6 months of age (because fetal hemoglobin contains gamma, not a beta chain):

Fever Anemia Swelling of the hands and feet (hand-foot syndrome) caused by blood stasis and infarction Protruding abdomen due to enlarged spleen and liver secondary to trapping of sickled cells in microcirculation and obstruction of blood flow Icteric sclera caused by bilirubin release during hemolysis (sickle cell destruction) Priaprism (males) due to pooling of abnormally shaped erythrocytes in the blood vessels of the penis. Chest syndrome symptoms same with pneumonia that is the major cause of death in sickle-cell patients

Management Conservative Management 1. Pain relief with Acetaminophen (Tylenol) or IV of morphine to reduce metabolic demand of oxygen thereby, terminating cell sickling. 2. Adequate hydration IV fluids and electrolyte replacement 3. Oxygenation 4. Antibiotics if the cause of sickling is infection. 5. Blood transfusion with packed RBCs 6. Hydroxyurea antineoplastic agent that increases production of fetal hemoglobin in children.

7. Exchange transfusion replacing sickled with normal cells If the patient does not respond to the usual therapies STEM CELL TRANSPLANTATION is done. Nursing Management 1. Monitor vital signs. Assess for pain. 2. Obtain blood and urine culture, chest x-ray and CBC results if infection is the cause of sickling. 3. Monitor childs nutritional intake with hydroxyurea. If taken orally, this drug can cause anorexia. 4. Assess for kidney function by noting if the child has urinated or not. (Kidney infarction may occur) 5. Do not administer potassium if the kidney function is not verified. Potassium if not excreted by the kidney may cause arrhythmia. Possible nursing diagnosis 1. Ineffective tissue perfusion R/T decreased hemoglobin concentration in blood 2. Acute pain R/T impaired blood flow due to obstruction of sickled cells image from elev8.com, daviddarling.info

About Daisy Jane, RN, MN Currently a Nursing Local Board Examination Reviewer. Subjects handled are Pediatric, Obstetric and Psychiatric Nursing. Previous work experiences include: Clinical instructor/lecturer, clinical coordinator (Level II), caregiver instructor/lecturer, NC2 examination reviewer and staff/clinic nurse. Areas of specialization: Emergency room, Orthopedic Ward and Delivery Room. Also an IELTS passer. Related Nursing Articles 1. Types of Sickle Cell CrisisOverview Sickle cell anemia is an inherited disorder on the beta chain of the hemoglobin resulting to abnormally shaped red blood cells. RBCs assume a crescent or C-shape that decreases the cells life span and... 2. Classifications of AnemiaOverview The main function of a red blood cell or erythrocyte is to carry and transport oxygen to the different parts of the body. The normal RBC count is 4-6 million/mm3. Hemoglobin (Hgb), an iron-bearing... 3. ThalassemiasThalassemias Definition Thalassemia is a group of inherited disorders which is associated with hemoglobin defects. The disorder results in excessive destruction of red blood cells leading to anemia. Types of Thalassemia There are two main...

4. ErythropoiesisRed Blood Cells Red Blood Cells (RBCs) also called erythrocytes, are oxygen carrying cells. It is derived from the Greek words erythros meaning red, kytos meaning hollow and cyte translated as cell in modern language.... 5. Nursing Care Plan AnemiaAnemia is the reduction in red blood cells (erythrocytes) thus decreasing the oxygen carrying capacity of the blood due to the following (1) excessive blood loss (2) deficiencies and abnormalities of RBC production (3) Excessive... Filed under Case Study Tagged with autosomal recessive, chest syndrome, hand-foot syndrome, hydroxyurea, sickle cell, sickle cell crisis, sickle cell trait, stem cell transplantation DISCLAIMER: Comments expressed in this website are those of the readers and do not necessarily reflect the position of NursingCrib.com or any of its sister sites. NursingCrib.com does not knowingly publish false information and may not be held liable for the views of readers exercising their right to free expression. All articles in this site was meant for educational purposes only. We dont claim full ownership of the videos, pictures and some articles posted on this site. All other trademarks are the property of their respective owners.