This is a name which is used for a group of inherited conditions that infect the red blood cells. Sickle cell anemia is the most serious type of sickle cell disease. This disease is usually affecting people of Asian, Eastern Mediterranean, Middle Eastern, Caribbean and African origin. In the United Kingdom, it is particularly common in people with Caribbean or African background [1]. Those people who suffer from sickle cell disease produce unusually shaped red blood cells which can cause problems because they do not live long as healthy blood cells and they can become stuck in the blood vessels. This is a serious and lifelong condition but the long – term treatment can help to manage many of the problems that are associated with sickle cell disease.
Symptoms of Sickle cell disease
Symptoms: This disease can cause many different symptoms. The symptoms of sickle cell disease can start from a few months of age but many children have few or no symptoms if the natural treatment is started early. Here are some of the most common symptoms:
- Painful episodes: Sickle cell crises or episodes of pain are one of the most common and distressing symptoms of sickle cell disease [2]. They happen when the blood vessels to some part of the body become blocked. This pain can be severe and it can last for up to 7 days on average. The sickle cell crisis can affect a particular part of the body, such as
- Legs and arms
- Tummy
- Pelvis
- Spine
- Ribs and breastbone
- Hands or feet, particularly in young children
It varies from person to person how often someone with sickle cell disease gets episodes of pain. Some people can have less than one a year while others can have one every few weeks. The average number is one episode per year. It is not always know what the trigger for bad pain is, but in some cases it can be caused by strenuous activity, dehydration or weather.
- Infections: Those people who have sickle cell disease are having more chances to get infections, particularly when they are young. This is possible because having this condition may means that your body is not very good at dealing with viruses and bacteria. Infections can range. They can be mild, such as cold. They can be much more serious. They can be potentially life – threatening, such as meningitis. Daily doses of antibiotics and vaccinations can reduce the risk of many infections.
- Anemia: It is known fact that nearly all people who have sickle cell disease have anemia. In this condition, the hemoglobin in the blood is low. Hemoglobin is a substance which is found in the red blood cells and it is used to transport oxygen around the body. Usually, it does not cause many symptoms, but in some cases can get worse if you become infected with the virus which is causing slapped cheek syndrome. This virus is known as parvovirus. This condition can lead to a sudden drop in the number of red blood cells and it cause additional symptoms, such as fainting, dizziness, a rapid heartbeat and headaches. This condition is usually treated with a blood transfusion. The swelling of spleen in young children can cause sudden anemia which requires a blood transfusion.
- Other problems: There are some cases when sickle cell disease can cause many different problems, such as
- Kidney or urinary problems, including bedwetting and blood in the urine
- Delayed growth during childhood and delayed puberty
- Pulmonary hypertension (this is high blood pressure in the blood vessels which carry blood from the heart to the lungs) [3]
- Gallstones (these are small stones in the gallbladder) which can cause yellow skin and eyes (jaundice) and abdominal pain
- Vision problems [4], such as blurred or patchy vision, reduced night vision, floaters and occasionally sudden vision loss
- Bone and joint pain
- Leg ulcers (these are open and painful sores on the lower legs)
Causes of Sickle cell disease
Causes: The sickle cell disease is caused by faulty genes which child inherits from the parents [5]. Sickle cell disease is not caused during the pregnancy or anything that parents did before and you cannot catch this condition from someone who has it.
References:
[1] Elander J, Beach MC, Haywood C. Respect, trust, and the management of sickle cell disease pain in hospital: comparative analysis of concern-raising behaviors, preliminary model, and agenda for international collaborative research to inform practice. Ethnicity & Health. 2011;16(4-5):405–21.
[2] Smith WR, Bovbjerg VE, Penberthy LT, et al. Understanding pain and improving management of sickle cell disease: the PiSCES study. Journal of the National Medical Association. 2005;97(2):183–93.
[3] National Heart, Lung, and Blood Institute. Sickle cell disease research & care. 2012. Retrieved from www.nhlbi.nih.gov/news/2012/sickle-cell-disease-research-care.
[4] Al-Naim AF, Al-Mulla AH, Al-Shikmubarak IA, et al. Vision quality assessment in patients with sickle cell disease versus normal population in Al-Ahsa, Saudi Arabia. International Journal of Scientific Study. 2017;5(8):155-8.
[5] NIH/National Heart, Lung, And Blood Institute. Scientists use gene therapy to correct sickle cell disease in mice. Science Daily. 2001. Retrieved from www.sciencedaily.com/releases/2001/12/011214081241.htm.
