Common Traits & Indicators of SCD

People with sickle cell disease (SCD) begin showing signs during the first year of life, usually around 5 months of age. Infants won't show symptoms of SCD at birth because fetal hemoglobin protects the red blood cells from sickling. At around 4-5 months old, the fetal hemoglobin is replaced by sickle hemoglobin and the cells begin to sickle. Symptoms and complications of SCD are different for each individual affected and can range from mild to severe.

As I continue my research into sickle cell disease, I've noticed that there isn't much to do in the way of prevention and treatment other than blood transfusions. This seems to become just a part of normal everyday life for those individuals. These individuals must be watched closely for iron build up in the body, which can have life-threatening damage to the organs.

It is important to remember that children with sickle cell disease are usually smaller in frame, have delayed puberty, or experience jaundice (yellowing of the skin and eyes). Children with SCD may also experience difficulty playing with other children, and may hinder social interpersonal development. These outward signs may make children living with SCD  easy targets for bullying and the psychological damage done can be equally devastating. As educators, keep this in mind and try to include other avenues for social development that all students can benefit from.

Symptoms are unique to each individual and include the following (information directly provided from the Center for Disease Control and Prevention):

• Hand-Foot Syndrome- swelling in the hands and feet, usually the first symptom of SCD. This swelling is often accompanied by fever and is caused by the sickle cells getting stuck in the blood vessels and blocking the flow of blood in and out of the hands and feet.
• The most common treatments for swelling in the hands and the feet are pain medicine and an increase in fluids, such as water.
• Pain "Episode" or "Crisis" - Pain is the most common complication of SCD and the number one reason people effected go to the emergency room or hospital. When sickle cells get stuck in small blood vessels and clog the blood flow, this causes mild to severe pain that can last for any length of time.
• To prevent a pain crisis, people with SCD can: drink plenty of water, try not to become exposed to extreme heat or cold, avoid situations that expose you to high altitudes, or take medications (as seen fit by your doctor!)
• Most patients can be treated with over the counter pain medications such as ibuprofen or aspirin.
• Anemia - with SCD, the red blood cells die early, which means there are not enough healthy red blood cells to carry oxygen throughout the body. The following symptoms may occur when this happens: tiredness, irritability, dizziness/lightheadedness, pale skin color, jaundice, slow growth, delayed puberty.
• Blood transfusions are used in severe cases of anemia.
• Infection - people with SCD, especially infants and children, are more susceptible to infection. Pneumonia is one of the leading causes of death in children with SCD.
• Vaccines can protect against harmful infections along with other overall good hygienic practices (i.e. washing your hands with soap many times a day, safely prepared food, penicillin)
•  Acute Chest Syndrome - this can be life-threatening and should be treated in a hospital. Symptoms and signs are similar to pneumonia and include chest pain, coughing, difficulty breathing, and fever.
• Adults can take a medicine called hydroxyurea to help prevent acute chest syndrome, but need to be watched closely for side effects such as low white blood cell count which increases the risk of infection.
• Splenic Sequestration - this happens when a large number of sickle cells get trapped in the spleen and cuase it to suddenly get large. Symptoms include sudden weakness, pale lips, fast breathing, extreme thirst, abdominal pain on the left side of the body, and fast heartbeat.
• Treatment typically is a blood transfusion.
• Vision Loss - includes blindness and can occur when blood vessels in the eye become blocked with sickle cells and the retina gets damaged. Some patients develop extra blood vessels in the eye from the lack of oxygen. Laser treatment often can prevent further vision loss if the retina is damaged by excessive blood vessel growth.
•  Leg Ulcers - usually occurs on the lower part of the leg. They happen more often in males than females and usually appear from 10 - 50 years of age. A combination of factors cause ulcer formation, including: trauma, infection, inflammation, and interruption of the circulation in the smallest blood vessels of the leg.
• Stroke - a stroke can happen if sickle cells get stuck in a blood vessel and clog blood flow to the brain. About 10% of children with SCD will have a symptomatic stroke. Stroke can cause learning problems and lifelong disabilities.
• A special exam called, transcranial Doppler ultrasound (TCD), can help identify children who are at risk for stroke. Frequent blood transfusions can help to prevent stroke.
• Deep Vein Thrombosis & Pulmonary Embolism - sickling of red blood cells can increase blood coagulations and induce an increased risk of  blood clot in a deep vein (DVT), or in the lung (PE) if the clot moves from the deep vein. DVT and PE can cause serious illness, disability and, in some cases, death.
•  Other possible complications - damage to body organs (i.e. liver, heart, or kidneys), tissues, or bones because not enough blood is flowing to the affected area(s), gallstones, priapism, and renal medullary carcinoma (a very rare form of kidney cancer).

Sources:

Centers for Disease Control and Prevention