1. Sickle cell disease is characterized by red blood cells that are abnormal and shaped as a rigid sickle. The disease is a genetic disorder. The formation of sickle cells is increased by low oxygen partial pressure. A mutation in the HBB gene causes the disease as it results in a defective hemoglobin molecule. The sickle cell disease is inherited autosomal recessive condition as both contributing parents must have the sickle hemoglobin gene in order for the disorder to be passed to the offspring.
2. As both parents must pass down the gene in order for an infant to inherit sickle cell, it is considered to be autosomal recessive pattern. In order to be a carrier, an individual must have a good copy of the gene as well as a mutated copy (Koch, 2012). The carriers usually have normal hemoglobin and one gene for sickle hemoglobin from their parents. This is because only one parent passed down the gene to the carrier. Because they have one normal hemoglobin gene, the carrier seldom develops symptoms of the disease but are capable of passing the abnormal gene to their offspring.
3. The abnormal sickle shaped red blood cells can block the blood vessels and result in a sickle cell crisis by preventing the flow of blood and oxygen to the tissues and causing extreme pain. The tissues and organs such as the liver and kidney, can be damaged or fail which makes a sickle cell crisis a life threatening condition. A crisis can be triggered by various factors such as a cold, the flue, dehydration, low oxygen levels, dramatic changes in altitude or temperature, and many medical procedures or psychological fluctuations. Notably, changes in altitude and temperature can be caused by travel and patients should take care to monitor these changes when flying or experience a change of season (Vera, 2012).
4.The assessment for a patient with a diagnosis of sickle cell disease will include a complete medical history and physical exam. Additionally, the practitioner will exam for reversible conditions that could be increasing the pain such as dehydration or an infection. The practitioner should inquire about medications that the patient is taking and any side effects. Using the visual analog scale, the practitioner can determine the pain level with 0 representing no pain and 10 representing the worst pain that the patient has ever experienced. While other scales are available, the most commonly used for measuring pain intensity is the vertical line range discussed. After approximately 4-6 hours, if the pain persists, then the practitioner should order narcotics for pain relief.
5.6. Priority nursing intervention for a patient with sickle cell crisis include increasing intravascular fluid volume to promote better blood flow. This can be achieved through hydration as well as the reversal of viscous blood flow in order to avoid tissue necrosis. Promoting the blood flow is essential. Additionally, hydration will serve to help alleviate the patient’s pain. Another intervention will be initiating a blood transfusion. When necessary, a blood transfusion may be order as the packed red blood cells will serve to restore the oxygen carrying capacity of blood volume.
7. Patient information should focus on teaching the patient to stay hydrated by drinking 4-6 liters of fluid per day and avoiding drastic temperature changes by wearing weather-appropriate clothing. Additionally, eating healthy and taking prescribed medication such as folic acid to help stimulate red blood cells
8.The most common medication use to treat sickle cell disease is opioid analgesics (morphine, dilaudid or fentanyl by IV injection or PCA pump) but morphine is the drug of choice. Also, anti-inflammatory drugs are use with opioids. Anti-inflammatory drugs work in peripheral tissue. They are effective in managing mild to moderate pain. Oral doses are indicated for milder pain.
9. Of the possible complications of sickle cell disease, the most commonly documented is related to acute severe pain which is called an acute vaso-occlusive crisis. This is caused from tissue ischemia and is usually in the bones and marrow (Krimm, 2014). Other common acute complications are fevers which are caused by infection, acute anemia or renal failure, splenic sequestration, and acute chest syndrome or strokes.
10. Discharge plans for a patient with sickle cell should include giving information about warning signs that would need to be immediately addressed by medical personnel. I would explain to the patient that sores, or ulcers, on the leg are a sign that the sickle cell anemia is not well controlled and therefore should be monitored carefully. Other signs to watch for would include swelling, pain, or redness in the feet, hands, or abdomen, signs of an infection to include a fever or shortness of breath, and changes in color in the eyes or skin that appear yellowish. Finally, men should be warned to watch for an erection that does not go away. The patient should be instructed to seek medical attention with any of these signs.
11. Choosing to become a parent comes with a lot of responsibility and people should take caution to gather as much information as possible about genetic disorders. However, individuals with sickle cell disease have specific issues and should seek the aid of genetic counselors, coordinate testing, and discuss the probability of passing on the genetic disorder. This probability will be evidence based according to the genotypes of both parents allowing the potential parents to make a true, informed decision prior to reproducing.
- Adams, R. J. (2014). Prevention of Stroke in Sickle Cell Anemia. Journal Of Law, Medicine & Ethics, 135-138. http://dx.doi.org/http://dx.doi.org/10.1111/jlme.12128
- Koch, A. (2012). Sickle Hemoglobin (Hbs) Allele and Sickle Cell Disease. Retrieved from http://ghr.nlm.nih.gov/condition/sickle-cell-disease
- Krimm, J. R. (2014). Sickle Cell Disease. Retrieved from http://www.emedicinehealth.com/sickle_cell_crisis/article_em.htm
- Vera, M. (2012). Sickle Cell Anemia Nursing Management & Interventions. Retrieved from http://nurseslabs.com/sickle-cell-anemia-nursing-management/
