According to cdc.gov, a Sickle cell is a group of inherited red blood disorders. It affects the shape of the red blood cells. The red blood cells’ shape is round for the healthy ones. When affected by sickle cell disorder, the red blood cells change their shape to become C-shaped and look like the sickle farm tool. The red blood cells are also sticky and die early with the disease (Jucker 900). This leads to a shortage of red blood cells in the blood. The sickle-shaped red blood cells cause clogs in the blood flow after moving in small blood vessels because they usually get stuck. Chest pains, stroke, and infections are some of the challenges of sickle cell (Bhosale p80). People who inherit one gene of the sickle cell from one parent and another normal gene from the other parent have the sickle cell trait. People with the sickle cell trait do not show the symptoms of the disease but can transmit the disease to their offspring. Those with the sickle cell trait can have health problems usually associated with the disease (cdc.gov).
Types of sickle cell disease
HbSS. This is the type of sickle cell where the offspring has inherited two sickle cell genes from the parents, with either parent contributing one gene. This is the most severe form of the disease, usually known as sickle-cell anemia (cdc.gov).
HbSC. This is inherited from one parent. “C” is the name of the abnormal hemoglobin inherited from the other parent. This form of sickle cell disease is mild.
HbS beta-thalassemia. People with this type of disease have one gene of thalassemia inherited from one parent, and the other gene for the sickle cell is inherited from the other parent. This type of sickle cell disease exists in two forms, the “0” and the +. Those with the HbS beta+ thalassemia have a mild form of the disease compared to those with the “0” state of the disease (cdc.gov).
Diagnosis
According to mayoclinic.org, the newborns usually get routine newborn screening to check the form of hemoglobin that underlies the sickle cell. Adults and older children’s tests are also done in hospitals. In young children and infants, the blood for screening is usually taken from the heel, while in adults, the blood for the tests is taken from a vein in the arm. The blood sample is then taken to the lab to screen the type of sickle cell in the hemoglobin. When children are diagnosed with sickle cell, the doctor is likely to refer them to a genetic counselor for other tests to check the compilations of the disease. A particular ultrasound machine is also used to reveal that children with a higher risk of being affected by stroke in the future. It uses sound waves to measure the children’s blood flow in the brain. Regularly getting blood transfusions can reduce the child’s risk of getting affected by stroke. The presence of sickle cell can also be determined before the birth of a child. The diagnosis can be made by testing some of the fluid surrounding the baby while in the mother’s womb. If one of the parents has the sickle cell trait, the screening is essential because knowing about the disease on time is essential to the child for better treatment. The disease treatment can be complex when recognized at later stages.
Treatment
Pain is severe if one suffers from the disease. The disease needs management to help the patient be relieved of the pain (mayoclinic.org). Relieving symptoms early can also help avoid complications resulting from the condition. Treatments of the disease may include blood transfusions and other medications. Stem cell transplant might also cure the disease in some children and teenagers. Some helpful medications may consist of:
Hydroxyurea-administering to the children and adults suffering from the daily disease hydroxyurea can help reduce the painful crisis and reduce the need for blood transfusions. This is also likely to increase the risk of infections resulting from the disease. Pregnant people should not take the drug.
L-glutamine oral powder. This drug was recently approved for the treatment of sickle cell anemia. This drug, like hydroxyurea, helps reduce the patient’s pain crisis.
Crizanlizumab (adakveo). This drug is given by injection. It is administered to children older than 16 years and adults. This disease also reduces the frequency of pain crises for the patient. The drug also has side effects that children younger than 16 cannot tolerate (mayoclinic.org). They may include; fever, back pain, and nausea.
Vexolotor (Oxbryta). This drug is used to treat adults and children older than 12 years. When the drug is orally administered, it may improve blood flow and lower the risk of anemia. The side effects of the drug include; diarrhea, rash, fatigue, fever, nausea, and headache.
Pain-relieving medications. The doctor is likely to prescribe narcotics that may help to relieve pain during the sickle cell pain crisis.
It prevents infections
Children with the disease should be administered penicillin between 2 months to 5 years. Using the drug can help reduce the risks of infections such as pneumonia which are very severe for children and can lead to death. Adults suffering from sickle cell need to take penicillin over their lifetime if they have had pneumonia before or undergone surgery to remove the spleen.
According to mayoclinic.org, vaccinations are essential for preventing disease in children. When children are vaccinated early, getting infections can be minimized. Illness in children who suffer from sickle cell anemia can be very severe. This makes it necessary always to give them the vaccinations. Vaccines can also be required in adults who suffer from sickle cell anemia. During an outbreak of a contagious viral disease like the covid-19, the children and adults with sickle cell anemia took extra precautions.
Blood transfusions. These are important in preventing and treating complications like a stroke in those suffering from the disease. In the red blood cells transfusion, the cells are removed from the supplied donated blood and then are administered to the patient through the veins. This helps to increase the number of normal blood cells. This reduces the symptoms of the disease. The risks involved with blood transfusions include the immune response of the blood donor. This makes it hard to get future blood donors. Excess iron in red blood cells can result from constant blood transfusions that can damage the heart and the liver (Funkhouser p559). One might also need another treatment to reduce the iron if one has had continuous blood transfusions.
The stem cell transplant. It is also called the transplant of the bone marrow. It involves removing the affected person’s bone marrow and replacing it with healthy bone marrow. The stem transplant procedure consists of getting the matching donor of the bone marrow (Hicks & David 978). The siblings of the sickle cell anemia patient who do not have the disease may match to become the donor. Many risks are involved with the transplant; thus, it is only recommended only for people who have significant symptoms of the presence of sickle cell. This method is the only proven cure for the disease that can be recommended to many people (mayoclinic.org).
The recommended lifestyle and home remedies
Choosing a healthy diet for sickle cell patients and taking folic acid supplements is helpful for people suffering from the disease. Making new blood cells in the bone marrow has folic acid and other vitamins as one of the essential requirements. Inquiring with the doctor may help know the foods necessary for gaining the vitamins (Schweiger 232). Eating whole grains and taking a variety of colored fruits can help the patients increase their immunity and reduce the risks involved with sickle cell anemia.
Avoiding the temperature extremes can reduce the risks associated with the disease because exposure to high temperatures can increase the risk of being affected by the sickle cell crisis that increases the pains. Involving oneself in constant exercise regularly without overdoing it also helps the patient avoid the problem of pain. Talking to the doctor to get advice on the necessary practices can help the patient cope with the disease.
Drinking plenty of water is essential for the patients. Getting dehydrated usually increases the risk of sickle cell crisis for the patient. Aiming at about eight glasses of water every day can help reduce the patient’s dehydration. When the body is hydrated, the patient is likely to be at peace because he will not be affected by the excessive pain associated with the disease (Richardson et al., 14).
Avoid smoking. The patient is advised to avoid tobacco because it increases the pain crisis. Living far away from active smokers is also essential because the patient will not be exposed to the nicotine smokers usually emit. The patients are also advised to avoid using drugs that the doctors do not prescribe (Jorns 180). Medications like naproxen sodium and Advil used to reduce the pain can affect the kidneys. Before using any medicine, the necessary consultation with the doctors must be done.
Coping and support
Joining support groups can be necessary for the patient. Talking with other patients who face similar challenges can help manage their status and reduce stress. This is important because one also gets someone to speak with about the challenges faced in coping with the disease. In the support group, making the necessary arrangements to talk to a mental health professional can help the patients deal with their worries. A social worker, a psychologist, or a counselor can be necessary for the patients. Getting more knowledge about the disease from these professionals helps the patient know the dos and don’ts of dealing with the disease.
References
“Sickle Cell Anemia – Diagnosis and Treatment – Mayo Clinic.” Mayo Clinic – Mayo Clinic, 30 Jan. 2020, www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/diagnosis-treatment/drc-20355882
“What is Sickle Cell Disease?” Centers for Disease Control and Prevention, 14 Dec. 2020, www.cdc.gov/ncbddd/sicklecell/facts.html.
Bhosale, Mrinalini, et al. “DOMINANCE OF HERBAL MEDICINES IN TREATING SICKEL CELL ANEMIA.” International Journal of Ayurveda and Pharma Research (2021): 79-83.
Bisceglia, Michele, et al. “Littoral cell angioma of the spleen: an additional report of four cases with emphasis on the association with visceral organ cancers.” Tumori Journal 84.5 (1998): 595-599.
Funkhouser, Ann W., et al. “CD30-positive anaplastic large cell lymphoma (ALCL) of T-cell lineage in a 14-month-old infant with perinatally acquired HIV-1 infection.” Journal of pediatric hematology/oncology 20.6 (1998): 556-559.
Hicks, David G., et al. “Primary lymphoma of bone. Correlation of magnetic resonance imaging features with cytokine production by tumor cells.” Cancer 75.4 (1995): 973-980.
Jörns, A., et al. “Loss of GLUT2 glucose transporter expression in pancreatic beta cells from diabetic Chinese hamsters.” Virchows Archiv 428.3 (1996): 177-185.
Jücker, M., et al. “Expression of a mutated form of the p85α regulatory subunit of phosphatidylinositol 3-kinase in a Hodgkin’s lymphoma-derived cell line (CO).” Leukemia 16.5 (2002): 894-901.
Richardson, Tina, Steve Lochan, and Patrick Roberts. “Sickel cell disease.” (1990): 14-15.
Schweiger, D. J. “Red cell distribution width in sickle cell anemia.” The American journal of medical technology 47.4 (1981): 231-233.
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