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Sickle Cell Disease
WHAT YOU NEED TO KNOW:
Sickle cell disease (SCD) causes your RBCs to be sickle (crescent) shaped. The sickle shape is caused by abnormal hemoglobin attached to the RBC. Hemoglobin carries oxygen to all tissues in your body. Sickle-shaped RBCs can get stuck to the walls of blood vessels. This can stop or slow blood flow, and prevent oxygen from getting to tissues. When this happens, it is called a sickle cell crisis. SCD may also cause low red blood cell (RBC) levels (anemia).
Call 911 or have someone else call for any of the following:
- You have any of the following signs of a stroke:
- Numbness or drooping on one side of your face
- Weakness in an arm or leg
- Confusion or difficulty speaking
- Dizziness, a severe headache, or vision loss
- You have any of the following signs of a heart attack:
- Squeezing, pressure, or pain in your chest that lasts longer than 5 minutes or returns
- Discomfort or pain in your back, neck, jaw, stomach, or arm
- Trouble breathing
- Nausea or vomiting
- Lightheadedness or a sudden cold sweat, especially with chest pain or trouble breathing
- You have a seizure.
- You lose vision in one or both eyes.
- You lose consciousness or cannot be woken.
Seek care immediately if:
- You feel lightheaded, short of breath, and have chest pain.
- You cough up blood.
- You have a fever of 100.4°F (38°C) or higher.
- You have a severe headache.
- Your pain does not get better after you take pain medicine.
- Your arm or leg is painful, red, and larger than usual.
- You feel like you can no longer cope with your pain, or feel like harming yourself.
- You have abdominal pain, nausea, and vomiting.
- You are a man and have an erection that is painful and does not go away after 4 hours.
- You cannot think clearly, or you feel like you are going to faint.
- Your urine is dark, or you are urinating less than usual or not at all.
- You see blood in your urine.
- Your eyes or skin are yellow.
- You have a cold or the flu.
- You have a cough or are wheezing.
Contact your healthcare provider if:
- You are more tired than usual during the day.
- You are constipated or have diarrhea.
- Your vision has changed in one or both eyes.
- You feel anxious or depressed.
- You have new or worse swelling in your joints.
- You have an open sore on your skin that will not heal.
- You are pregnant or think you are pregnant.
- You have questions or concerns about your condition or care.
You may need any of the following:
- Antibiotics may be given to prevent a bacterial infection.
- Hydroxyurea helps your body make red blood cells that are less likely to sickle. This may help decrease your pain and prevent sickle cell crisis.
- Folic acid helps your body may healthy red blood cells.
- Prescription pain medicine may be given. Ask your healthcare provider how to take this medicine safely. Some prescription pain medicines contain acetaminophen. Do not take other medicines that contain acetaminophen without talking to your healthcare provider. Too much acetaminophen may cause liver damage. Prescription pain medicine may cause constipation. Ask your healthcare provider how to prevent or treat constipation.
- NSAIDs , such as ibuprofen, help decrease swelling, pain, and fever. This medicine is available with or without a doctor's order. NSAIDs can cause stomach bleeding or kidney problems in certain people. If you take blood thinner medicine, always ask your healthcare provider if NSAIDs are safe for you. Always read the medicine label and follow directions.
- Acetaminophen decreases pain and fever. It is available without a doctor's order. Ask how much to take and how often to take it. Follow directions. Read the labels of all other medicines you are using to see if they also contain acetaminophen, or ask your doctor or pharmacist. Acetaminophen can cause liver damage if not taken correctly. Do not use more than 4 grams (4,000 milligrams) total of acetaminophen in one day.
- Take your medicine as directed. Contact your healthcare provider if you think your medicine is not helping or if you have side effects. Tell him or her if you are allergic to any medicine. Keep a list of the medicines, vitamins, and herbs you take. Include the amounts, and when and why you take them. Bring the list or the pill bottles to follow-up visits. Carry your medicine list with you in case of an emergency.
- Apply heat to areas of pain. Use a heating pad or take a warm bath. Do this for 20 to 30 minutes every 2 hours for as many days as directed.
- Gently massage areas where you feel pain. A professional massage may also help with pain.
- Balance rest and exercise. Rest during a sickle cell crisis. Over time, increase your activity. Exercise may help decrease pain. Take breaks during exercise and drink plenty of water. Ask your healthcare provider which activities are safe for you.
- Get acupuncture treatment. Acupuncture may help decrease pain and help you relax. Ask your healthcare provider for more information about acupuncture.
- Eat healthy foods. Healthy foods will improve your overall health and make it easier to manage SCD. Examples of healthy foods include fruits, vegetables, whole-grain breads, low-fat dairy products, beans, lean meats, and fish. Ask if you need to be on a special diet.
Prevent a sickle cell crisis:
A sickle cell crisis may be caused by illness, changes in temperature, stress, dehydration, or being at high altitudes. Do the following to help prevent a sickle cell crisis:
- Drink liquids as directed. Dehydration can increase your risk for a sickle cell crisis. Ask how much liquid to drink each day and which liquids are best for you.
- Avoid quick changes in temperature. Do not go quickly from a warm place to a cold place. Get in a pool slowly instead of jumping in. Dress in light clothing in the summer and warm clothing in the winter.
- Ask about vaccinations. Vaccinations can help prevent a viral infection. Get a flu shot every year as directed. You may also need a pneumonia vaccine.
- Wash your hands frequently. Handwashing can help prevent illness. Wash your hands before you prepare or eat food, and after you use the bathroom.
- Do not smoke. Nicotine and other chemicals in cigarettes and cigars can cause lung damage and sickle cell crisis. Ask your healthcare provider for information if you currently smoke and need help to quit. E-cigarettes or smokeless tobacco still contain nicotine. Talk to your healthcare provider before you use these products.
- Limit or do not drink alcohol. Alcohol can cause dehydration and increase your risk for sickle cell crisis. If you drink alcohol, also drink plenty of water.
- Manage your stress. Your healthcare provider may recommend relaxation techniques and deep breathing exercises to help decrease your stress. Your healthcare provider may recommend you talk to someone about your stress or anxiety, such as a counselor or a trusted friend.
- Do not travel in an unpressurized plane or travel to high altitudes. These environments are low in oxygen and may cause a sickle cell crisis.
Wear medical alert identification:
Wear medical alert jewelry or carry a card that says you have sickle cell anemia. Ask your healthcare provider where to get these items.
What you need to know about family planning and pregnancy:
- If you do not want to become pregnant, your healthcare provider may recommend birth control pills that contain only progestin. The pills will prevent pregnancy and make your periods lighter. Lighter periods may help treat low RBC levels.
- Talk to your healthcare provider before you get pregnant. SCD increases a woman's risk for problems, such as a miscarriage and having a baby that weighs less than normal. You will need close monitoring during pregnancy. You may need to take certain vitamins and have 1 or more blood transfusions during pregnancy.
- You will pass a gene for sickle cell disease to your child. Your partner should be tested for the sickle cell gene. This information can help predict your child's risk for sickle cell disease.
Follow up with your healthcare provider as directed:
You may need ongoing screening for conditions that can develop because of sickle cell disease. Examples include kidney disease, hypertension (high blood pressure), retinopathy (eye problems), and problems with your lungs. Write down your questions so you remember to ask them during your visits.
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The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.