Overview of Sickle Cell Anemia

Introduction

The Center for Disease Control (CDC, 2019) estimates that about 1 in 13 African-American babies are born with sickle cell trait. They also believe that sickle cell disease occurs among 1 out of every 365 African-American births (CDC, 2019). While sickle cell anemia and crises are a major cause for hospitalization, there is often not much discussion about the disease in nursing school. This paper will further explore this disease.

Pathophysiology

 Sickle cell anemia is a mutation of red blood cells due to both parents being carriers of the mutated gene. These cells are “sickled” which means instead of them being a normal circular shape, they are shaped like the moon and are not whole. These sickled red blood cells are not efficient in carrying oxygen and nutrients to the body. In narrow blood vessels these sickled cells can get stuck and block blood flow (CDC, 2019). When oxygen and nutrients can not be delivered adequately, there is almost always pain involved and lasting health effects that will be discussed below. There are a number of forms of sickle cell, including sickle beta thalassemia and hemoglobin sickle cell diseases. For the purpose of this paper, only sickle cell anemia (homozygous sickle mutation) will be discussed.

Signs and Symptoms

 There are a slew of symptoms associated with sickle cell anemia which can be divided into acute and chronic. Acute symptoms include infections, acute chest syndrome, stroke, myocardial infarction and priapism, among many more. Infections occur commonly due to a lack of adequate nutrients and oxygen being carried to a site in need of healing. Acute chest syndrome is a vaso-occlusive event which involves fever, chest pain and trouble breathing (CDC, 2019). So why does this happen to people with sickle cell disease? Inflammation in the lungs (either from smoking or a prior infection) leads to a loss of oxygen in the lungs which in turn leads to further sickling of red blood cells. Stroke and heart attacks are due to sickled blood cells blocking the flow of blood to the affected area. Priapism (long, painful erections) can lead to erectile dysfunction in men, according to Mayo Clinic (2019).

 Chronic symptoms can include pain, anemia, leg ulcers and delayed puberty and growth (CDC, 2019). Pain is a result of lack of oxygen being delivered throughout the body. This can be referred to as a crisis and the pain can occur anywhere in the body. It can last for hours, days or longer. Anemia is of course due to compromised red blood cells. Chronic leg ulcers and delayed growth is also due to a lack of nutrients being delivered throughout the body. It is not so obvious at first why sickle cell anemia is such a major disease for African Americans to deal with, until people learn that red blood cells are vital in carrying oxygen and nutrients throughout the body to assist in healing and growth.

Medical Treatment

 Unfortunately there are not very many options for medical treatment. Hydroxyurea is the most common drug prescribed for patients with sickle cell anemia. Hydroxyurea is classified as an antimetabolite and used for both cancer and sickle cell. For patients with sickle cell anemia this drug helps prevent the formation of sickled red blood cells, according to medlineplus.gov (2020).

Blood transfusions are usually given to treat and prevent complications. This is because the body is being infused with healthy red blood cells that help deliver nutrients needed throughout the body. A newer “curative” treatment for sickle cell anemia is hematopoietic cell transplant (HCT). This is a sort of stem cell transplant where the patient receives healthy bone marrow from a donor (CDC, 2019). Of course this treatment is not extremely common due to the risk of surgery, the advanced medical practice and the need for a healthy donor.

Nursing Interventions and Care

 Nursing interventions include monitoring for signs of stroke (slurred speech, facial drooping) and myocardial infarction (chest pain that can radiate). If a patient is admitted to the unit for a crisis then adequate pain control should be managed well. Depending on the patient’s age, use either the FLACC, faces or number scale to rate pain in the pediatric patient. When administering blood transfusions it is crucial to look for signs of a reaction: hives, shortness of breath, bloody urine and severe headache (Gersten, 2019). Sickle cell is lifelong with no simple cure; nursing care should include looking at the psychosocial aspect of the patient and adhering to emotional needs related to suffering from this disease.

Discharge Teaching

 Information about adequate nutrition is important to compliment other medical treatments. Most patients will take 1mg of folic acid daily, vitamin d if deficient and a daily multivitamin without iron (excess iron can contribute to iron depletion) (CDC, 2019).

It’s also important to teach the parents and child that this is a lifelong disease that they will have to learn to handle. It is important that the parents (and child, if appropriate age) know the signs of myocardial infarction, stroke and other possible complications. Patients should wear a medical alert bracelet and parents should be fully informed on any medications, their side effects, dosage and times to be taken.

Conclusion

 African-American couples planning to conceive should be encouraged to consider genetic testing to determine if they are both carriers for the sickle cell gene and should be informed of what this disease entails. New parents may be overwhelmed at the amount of change this requires for their child, and children may be scared of the possible complications and pain from sickle cell. It is important to address these concerns and stress that there is no safe cure as of now for this disease. Nurses should ensure that the patient is receiving adequate pain control as this is a major component of this process.

References

• Data & Statistics on Sickle Cell Disease. (2019, October 21). Retrieved from http://www.cdc.gov/ncbddd/sicklecell/data.html
• Gersten, T. (2019, January 29). Hemolytic transfusion reaction: MedlinePlus Medical Encyclopedia. Retrieved February 16, 2020, from https://medlineplus.gov/ency/article/001303.htm
• Hydroxyurea: MedlinePlus Drug Information. (n.d.). Retrieved from https://medlineplus.gov/druginfo/meds/a682004.html
• Priapism. (2019, June 15). Retrieved from http://www.mayoclinic.org/diseases-conditions/priapism/symptoms-causes/syc-20352005