Complications and Treatments
Pain is the most common complication of Sickle Cell Disease and the top reason that people with sickle cell disease go to the emergency room or hospital. When sickle cells travel through small blood vessels, they can get stuck and clog the blood flow. This causes pain that can start suddenly, be mild to severe, and can last for any length of time.
There are simple steps that people with sickle cell disease can take to help prevent and reduce the number of pain crises:
Drink plenty of water.
Try not to get too hot or too cold.
Try to avoid places or situations that expose you to high altitudes (for example, flying, mountain climbing, or cities with a high altitude).
Try to avoid places or situations that expose you to low oxygen levels (for example, mountain climbing or exercising extremely hard, such as in military boot camp or when training for an athletic competition).
Adults with severe Sickle Cell Disease can take a medicine called hydroxyurea to help reduce the number of pain crises.
People taking hydroxyurea must be checked often by a doctor because the medicine can cause serious side effects, including an increased risk of dangerous infections.
Most pain related to sickle cell disease can be treated with over the counter pain medications such as ibuprofen and aspirin. Some people who have severe crises are given opioid (i.e. morphine) medications daily, along with additional pain medication.
Anemia is a very common complication of sickle cell disease. With sickle cell disease, the red blood cells die early. This means there are not enough healthy red blood cells to carry oxygen throughout the body. When this happens, a person might have:
Complications and Symptoms
Dizziness and light heartedness
A fast heart rate
Pale skin color Jaundice (yellow color of the skin and whites of the eyes)
Blood transfusions are used to treat severe anemia. A sudden worsening of anemia resulting from infection or enlargement of the spleen is a common reason for a transfusion. Multiple blood transfusions, however, might cause health problems because of the iron content of blood. Iron overload, called hemosiderosis, can damage liver, heart, pancreas and other organs, leading to diseases such as diabetes mellitus. Iron chelation therapy should be started in patients with Sickle cell disease receiving regular blood transfusions to reduce excess iron levels.
People with sickle cell disease, especially infants and children, are more at risk for harmful infections. Pneumonia is a leading cause of death in infants and young children with Sickle cell disease. Prevention Vaccines can protect against harmful infections.
Babies and children with sickle cell disease should have all of the regular childhood vaccines, plus a few extra.
Flu vaccine (influenza vaccine) every year after 6 months of age.
A special pneumococcal vaccine (called 23-valent pneumococcal vaccine) at 2 and 5 years of age.
Meningococcal vaccine, if recommended by a doctor.
Don’t forget to add the extra vaccines listed previously for children with sickle cell disease.
Vaccines are a great way to prevent many serious infections. Children with sickle cell disease should get all the regular childhood vaccines, plus a few extra.
Click here for the regular childhood and teen vaccination schedule. Don’t forget to add the extra vaccines listed previously for children with sickle cell disease. Adults should have the flu vaccine every year, as well as the pneumococcal vaccine and any others recommended by a doctor.
Take penicillin (or other antibiotic prescribed by a doctor) every day until at least 5 years of age.
Treatment Infections are treated with antibiotic medicines and sometimes blood transfusions. At the first sign of an infection, such as a fever, it is important for people with sickle cell disease to see a doctor. Early treatment of an infection can help prevent problems.
ACUTE CHEST SYNDROME
This can be life-threatening and should be treated in a hospital. It is similar to pneumonia and symptoms include chest pain, coughing, difficulty breathing, and fever.
Prevention: Adults with severe sickle cell disease can take a medicine called hydroxyurea to help prevent acute chest syndrome. People taking hydroxyurea must be watched closely because the medicine can cause serious side effects, including an increased risk of dangerous infections.
A person who is on bed rest or has recently had surgery can use an incentive spirometer, also called “blow bottle,” to help prevent acute chest syndrome.
Treatment: Depending on the cause, treatment might include oxygen, medicine to treat an infection, medicine to open up blood vessels to improve blood flow, and blood transfusions.
This can be life-threatening and should be treated in a hospital. It happens when a large number of sickle cells get trapped in the spleen and cause it to suddenly get large.
Symptoms: Include sudden weakness, pale lips, fast breathing, extreme thirst, abdominal (belly) pain on the left side of the body, and fast heartbeat. Parents of a child with sickle cell disease should learn how to feel and measure the size of their child’s spleen.
Prevention: Patients who have had a very severe, life-threatening episode of splenic sequestration or who have had many episodes in the past, it might be necessary to have regular blood transfusions or the spleen can be removed ( is called splenectomy) to stop it from happening again.
Treatment: Treatment typically is a blood transfusion.
HAND AND FOOT SYNDROME
Swelling in the hands and feet usually is the first symptom of sickle cell disease. This swelling, often along with a fever, 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.
Treatment: The most common treatments for swelling in the hands and the feet are pain medicine and an increase in fluids, such as water.
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 Sickle cell disease will have a stroke. Stroke can cause lifelong disabilities and learning problems.
Prevention: Doctors can sometimes identify children who are at risk for stroke using a special type of exam called, “transcranial Doppler ultrasound.” In some cases, a doctor might recommend frequent blood transfusions to help prevent a stroke. People who have frequent blood transfusions must be watched closely because there are serious side effects. For example, too much iron can build up in the body, causing life-threatening damage to the organs.
Other Possible Complications: Damage to body organs, tissues, or bones because not enough blood is flowing to the affected area(s). Gallstones. Painful erection of the penis called priapism, which can last less than 2 hours or more than 4 hours. If it lasts more than 4 hours, the person should get urgent medical help. It can lead to impotence.
Vision loss, including blindness, can occur when blood vessels in the eye become blocked with sickle cells and the retina (the thin layer of tissue inside the back of the eye) gets damaged.
Prevention: People with sickle cell disease should have their eyes checked every year to look for damage to the retina. If possible, this should be done by an eye doctor who specializes in diseases of the retina.
Treatment: If the retina is damaged, laser treatment often can prevent further vision loss.
This usually occurs on the lower part of the leg. They happen more often in males than in females and usually appear from 10 through 50 years of age. The cause of leg ulcers is unclear.
Treatment: Leg ulcers can be treated with medicated creams and ointments. Leg ulcers can be painful, and patients can be given strong pain medicine. Bed rest and keeping the leg (or legs) raised to reduce swelling is helpful, although not always possible.
Sickle cell disease has no available cure. However, certain medications such as hydroxyurea, antibiotics and blood transfusions (for some) can relieve symptoms of this disease.