Sickle Cell Anemia

What is Sickle Cell Anemia?

Sickle Cell Anemia is a a blood disease that is usually diagnosed at birth and primarily impact black people. The red blood cells in people with sickle cell have a crescent shape. The disease is thought to be an adaption to the malaria virus. The sickled shaped cells are more resistant to malaria bacteria. Which is good for people that live in or want to visit a country with high chances of catching malaria from mosquitos, but the disease has many drawbacks. The sickled shape causes the red blood cells to be less effective in carrying oxygen and nutrients throughout the body. Unlike normal red blood cell can easily flow through blood vessels, sickled cells tend to get stuck. Whether it is on another sickled cell or on the wall of a blood cell, they can cause a blockage in blood vessels.

Places Where Malaria is Prevalent

Learn more about Malaria

Places Where Sickle Cell is Prevalent

Learn about Sickle Cells Demographic

Types of Sickle Cell

Sickle Cell Anemia (SS): Two hemoglobin-S genes are inherited for , the most severe type.
Sickle Hemoglobin-C (SC): One hemoglobin-S and one hemoglobin-C is inherited. This type is less severe because they do have some normally shaped hemoglobin and therefore have less anemia.
Sickle Hemoglobin-D (SD): One hemoglobin-D and one hemoglobin-S is inherited.
Sickle Hemoglobin-O (SO): One hemoglobin-O and one hemoglobin-S is inherited
Sickle Beta-Plus Thalassemia: Two beta globin genes are inherited, the "plus" identifies that there is a low presence of normal hemoglobin:
Sickle Beta-Zero Thalassemia: Two beta globin genes are inherited and the "zero" indicates that there is no normal hemoglobin produced.

Sickle Cell Crises

A sickle cell crisis is the result of a red blood cell blockage in the blood vessels that carry blood to the bones.

Types of Crises:

Aplastic Crisis: This is a when hemoglobin levels fall more than 20 grams per liter of blood and when there is a reduce or absences of reticulocytes in peripheral blood.
Acute Sequestration Crisis: Along with a drop in the levels of hemoglobin and reticulocytosis. Reticulocytosis is an increase in reticulocytes. It is the result of an increased need for red blood cell production. An acute crisis also is associated with enlargment of the spleen and/or liver. During this type of crisis there can be symptoms of circulatory insufficiency.

Possible Circulatory Insufficiencies:

Tachypnoea: rapid breathing
Tachycardia: fast heart beat
Hypotension: low blood pressure

Hyper-hemolytic Crisis: This type of crises is characterized by a drop in hemoglobin associated with jaundice, reticulocytosis, red blood cells that are abnormally color, and excessive amounts of bilirubin in the blood and urine.

Mortality Rate

An estimate of 94% of people with sickle cell in the United States survive to adulthood.

Complications

There are many complications that result from blockages in blood vessels. Complications can be minor but most can lead to death. All of the complications cause extreme pain because of tissue death as a result of blood flow being blocked and oxygen being low in for too long. Some of these complications are organ failure, pulmonary hypertension, blindness, and a stroke.

Treatment

Learn more about Bone Marrow Transplants
Blood Transfusion: Blood from a person without sickle cell is given.
Bone Marrow Transplant: Bone marrow from a person without sickle cell is implanted. Bone marrow is responsible for the production of red blood cells. The new bone marrow would produce normally shaped red blood cells.
There are medicines to used to prevent cell from sickling or sticking to the walls of blood vessels.
Most of the time if it is not an emergency, patients will have to endure the pain of a crisis and sometimes pain medicine is prescribed.

Nutritional Needs

The body of a person with sickle cell is constantly trying to compensate for lack of normally shaped red blood cells. This causes the body to use more energy than a person without sickle cell. There is not much research on the nutritional needs of people with sickle cell. The research available suggests that people with sickle cell should be consuming more calories than average people and specifically that they have an increased need for L-arginine.

Case study on Nutrition Needs for SCD

L-arginine is considered to be a non-essential amino acid because the body can produce. In people with sickle cell the process of red blood cell destruction and production makes other bodily functions go on the back burner so L-arginine may be essential for them. Other nutrients that are important in a diet for sickle cell patients are zinc, folate, l-arginine, and nitric oxide. Zinc is involved in the process of cellular metabolism, and important for catalytic activity when it comes to enzymes, it is also important in aiding protein and DNA synthesis. Folate acts as a coenzyme during cell division, and is also mostly found in liver. Arginine is the precursor to nitric oxide, ornithine, polyamines, proline, glutamate, creatine, and urea. Nitric oxide is a vasodilator and stimulates muscles to relax. It lowers blood pressure, improves blood flow and endurance level during aerobic exercise. Iron is essential for people for people that don't get frequent blood transfusion. People that have to get chronic blood transfusions can develop iron excess which causes an increase of free radicals in the blood.

The increased need for energy combined with the lack of knowledge makes people with sickle cell experience stunted growth and development in children and teens. In adults, it leads to poor muscle gain and maintenance.

My Connection to Sickle Cell

My personal connection to sickle cell disease is a family member. They were diagnosed at birth and from what I can remember have SC. It is a less severe version of the disease and they haven't had many crises. This person is still active and doesn't let the disease get in their way. I think it is a blessing that they haven't suffered these complication. Since doing research for this project I am going to try and get them to make some diet changes. The person is still growing so I think it would be beneficial to increase their caloric intake and maybe even get them to try supplements.

References:

Amazon.com: Truwild all natural nitric beets nitric oxide vegan ... (n.d.). Retrieved April 30, 2023, from https://www.amazon.com/TruWild-Natural-Circulation-Superfood-Supplement/dp/B08MJJ68GL

Centers for Disease Control and Prevention. (2020, April 9). CDC - malaria - about malaria - where malaria occurs. Centers for Disease Control and Prevention. Retrieved April 30, 2023, from https://www.cdc.gov/malaria/about/distribution.html

Eleise Britt (MSc Nutrition) Eleise is a university qualified Nutritionist from Melbourne. (2019, June 12). L-arginine supplementation: Splitting fact from fiction. Diet vs Disease. Retrieved April 30, 2023, from https://www.dietvsdisease.org/l-arginine-supplementation/

Hyacinth, H. I., Gee, B. E., & Hibbert, J. M. (2010, January 1). The role of nutrition in sickle cell disease. Nutrition and metabolic insights. Retrieved April 2023, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3085005/

Juwah, A. I., Nlemadim, E. U., & Kaine, W. (2004, June 1). Types of anaemic crises in paediatric patients with sickle cell anaemia seen in Enugu, Nigeria. Archives of Disease in Childhood. Retrieved April 30, 2023, from https://adc.bmj.com/content/89/6/572

Mary Kugler, R. N. (n.d.). Living with sickle cell disease. Verywell Health. Retrieved April 30, 2023, from https://www.verywellhealth.com/sickle-cell-anemia-2861015

Mayo Foundation for Medical Education and Research. (2022, March 9). Sickle cell anemia. Mayo Clinic. Retrieved April 30, 2023, from https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876

Naqvi, E. (2021, November 29). Sickle cell disease life expectancy. Rare Disease Advisor. Retrieved April 30, 2023, from https://www.rarediseaseadvisor.com/hcp-resource/sickle-cell-disease-life-expectancy/#:~:text=A%20study%20conducted%2025%20years,(HbSS)%20survived%20to%20adulthood.&text=The%20current%20estimate%20is%20that,in%20London%20survive%20to%20adulthood.

Sickle-cell. Rimed Ltd. (2020, January 27). Retrieved April 30, 2023, from https://rimed.com/clinicalapplications/sickle-cell/

Stem Cell (bone marrow) transplant. St. Jude together. (n.d.). Retrieved April 30, 2023, from https://together.stjude.org/en-us/diagnosis-treatment/treatment/bone-marrow-transplant.html

Types of sickle cell disease. Sickle Cell Association of the National Capital Area, Inc. (n.d.). Retrieved April 30, 2023, from http://scancainc.org/learn/types-of-sickle-cell-disease/#:~:text=There%20are%20several%20types%20of,and%20Sickle%20Beta%2DZero%20Thalassemia.

U.S. Department of Health and Human Services. (n.d.). Treatment. National Heart Lung and Blood Institute. Retrieved April 30, 2023, from https://www.nhlbi.nih.gov/health/sickle-cell-disease/treatment#:~:text=A%20blood%20and%20bone%20marrow,manage%20complications%2C%20including%20chronic%20pain.

Umeakunne, K., & Hibbert, J. M. (2019, May 23). Nutrition in sickle cell disease: Recent insights: NDS. Nutrition and Dietary Supplements. Retrieved April 30, 2023, from https://www.dovepress.com/nutrition-in-sickle-cell-disease-recent-insights-peer-reviewed-fulltext-article-NDS

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Naomi Robertson