What is sickle cell disease?

Sickle Cell Disease
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Sickle cell disease: Overview

Sickle cell disease turns normal, round red blood cells into misshapen cells that look like sickles or crescent moons. The sickle-shaped cells can get stuck in blood vessels, blocking blood flow and causing severe pain. The sickle-shaped cells also can harm organs, muscles, and bones. It is a lifelong condition. Sickle cell disease is passed down in families. You can talk to your doctor about whether to have genetic tests to find out the chance of having a child with the disease. Your doctor also may recommend that your family members get tested for sickle cell disease.

Your doctor may treat you with medicines. Some people get blood transfusions or a bone marrow transplant. Managing pain is an important part of your treatment.

Sickle cell disease

Sickle cell disease is a blood disorder in which the body produces an abnormal type of the oxygen-carrying substance hemoglobin in the red blood cells. Normal hemoglobin is called hemoglobin A, but people with sickle cell disease have only hemoglobin S, which turns normal, round red blood cells into abnormally curved (sickle) shapes.

Sickle-shaped blood cells are destroyed by the body faster than normal blood cells, which can result in an inadequate oxygen supply to the body (anemia). Most people with sickle cell disease have at least mild symptoms of chronic anemia, including:

  • Weakness and fatigue.
  • A pale appearance.
  • Yellowing of the skin and the whites of the eyes (jaundice).
  • Shortness of breath.

Sickle-shaped blood cells are also more likely to get stuck in and block small blood vessels throughout the body. Reduced blood flow caused by blocked blood vessels can damage certain organs, muscles, and bones. This may cause repeated episodes of pain (called sickle cell crises) that may last from hours to days. The pain most often occurs in the bones of the spine, arms and legs, the chest, and the abdomen.

People who have sickle cell disease need special medical care throughout their lives to treat the variety of problems that can be caused by the illness.

What happens when you have sickle cell disease?

When a child is born with sickle cell disease, it's impossible to predict which problems will develop, when they will start, or how bad they will be. During the first 6 months of life, infants have a high level of fetal hemoglobin (HbF) in their blood, which protects them from red blood cell sickling. But dangerous complications of sickle cell disease may quickly develop between ages 6 months and 5 years, after levels of fetal hemoglobin decrease.

Older children and adults with sickle cell disease may have few problems. Or they may have a pattern of ongoing complications that shortens their lives. The most common and serious problems caused by sickle cell disease are anemia, pain, and organ failure.

What are the symptoms of sickle cell disease?

Painful events (sickle cell crises) in the hands or feet, belly, back, or chest are the most common symptom of sickle cell disease. This pain may last from hours to days. Most people with sickle cell disease are anemic, which can make them feel weak and tired.

How is sickle cell disease treated?

Treatment involves getting routine tests to monitor health, managing pain events (sickle cell crises) with medicine, and treating related health problems as they arise. Some people need regular blood transfusions to lower the risk of stroke and to treat anemia and other problems.

How is pain from sickle cell disease treated?

In many cases, you can treat pain at home. You can use a combination of pain medicines, plenty of fluids, and comfort measures such as heating pads or hot water bottles. Do not use heating pads with children.

A painful event (crisis) can become severe and may last for days to weeks. If home treatment doesn't help enough, you'll need strong pain medicine that must be monitored in a hospital or treatment center.

Treating pain at home

Pain can be treated at home with:

  • Pain management skills. These skills can help you and/or your child focus away from the pain. They can help even when you also take pain medicines. A pain specialist can teach you pain management skills. These skills include:
    • Guided imagery.
    • Relaxation.
    • Distraction.
    • Deep breathing.
    • Positive, encouraging self-talk.
  • Medicine. Use the pain management plan you developed with your doctor to help decide what type of pain medicine to use. You are probably the best judge of when it's necessary to use a stronger prescription medicine.
    • For mild pain, you might use over-the-counter ibuprofen (such as Advil) and/or acetaminophen (such as Tylenol). Children and teens younger than 20 should not be given aspirin, which can cause Reye syndrome, a serious illness. Be safe with medicines. Read and follow all instructions on the label.
    • For moderate or severe pain, you might take pain medicine prescribed by your doctor. These are usually opioid medicines. You and your doctor will talk about which medicines to use, how much to take, and how often to take them. The instructions may change depending on how bad your pain is.

Treating pain at the hospital

If you go to the hospital, you will be treated with a strong pain medicine. This is usually an opioid medicine, such as morphine.

  • The medicine is given directly into a vein (intravenously, or I.V.). You may be given a button to push to release a dose when needed.
  • When you feel less pain, I.V. opioid medicine is slowly reduced. It's replaced with a less powerful medicine you take by mouth. These actions can prevent your body from going through sudden drug withdrawal symptoms.

How is sickle cell disease diagnosed?

Sickle cell disease is diagnosed when a simple blood test shows abnormal hemoglobin. A sickle cell test looks for sickle cell trait and sickle cell disease.

How can you care for your child who has sickle cell disease?

  • Have your child take medicines exactly as prescribed. Call your doctor if you think your child is having a problem with a medicine.
  • Give pain medicines exactly as directed.
    • If the doctor gave your child a prescription medicine for pain, give it as prescribed.
    • If your child is not taking a prescription pain medicine, ask your doctor if your child can take an over-the-counter medicine.
  • Try to help ease pain by distracting your child. Have your child learn to use guided imagery, deep breathing, and relaxation exercises. A pain specialist can teach you and your child pain management skills.
  • Dress your child warmly in cold weather. The cold and windy weather can lead to severe pain.
  • Give your child lots of fluids.
  • Keep your child away from smoke. Do not smoke or let anyone else smoke around your child or in your house.
  • Make sure your child gets plenty of sleep.
  • Make sure your child gets regular eye exams. Sickle cell disease can cause vision problems.
  • Have your child wear medical alert jewelry that says that your child has sickle cell disease. You can buy this at most drugstores.
  • Help your child avoid infections such as COVID-19, colds, and the flu. Remind your child to wash their hands often. Get your child a flu shot every year. Stay up to date on your child's COVID-19 vaccinations.
  • Make sure your child gets a pneumococcal vaccine shot. This is a standard vaccine given to children starting at 2 months of age for a total of 4 shots. Your doctor can tell you if your child needs another shot.
  • Make sure that your child takes antibiotics regularly until age 5. This is done to prevent infections.

Sickle cell disease: Preventing problems

Here are some things you can do to help prevent problems when you or your child has sickle cell disease.

  • Make and follow a plan for how to treat pain.

    Work with your doctor to make a plan that includes instructions on how to treat pain at home. Your plan should also tell you when you need to go to the hospital if your pain gets worse.

  • Avoid infections.

    Make sure that you and your child get all the recommended vaccines on schedule.

    To help your child with sickle cell disease, make sure that your child takes antibiotics until age 5. And help your child avoid contact with anyone who might have fifth disease. A virus that causes fifth disease can suddenly stop the body from producing red blood cells. This is called an aplastic crisis.

  • Get routine eye exams.

    Eye exams can find problems early.

  • Drink water and other fluids to prevent dehydration.
    • Drink extra fluids before, during, and after exertion and when you're in the heat.
    • Drink plenty of fluid if you have a fever or infection.
    • Limit alcohol.
    • Children should keep a water bottle with them during school, play, and outings.
  • Eat healthy foods.

    Healthy eating helps keep your body's immune system strong. Talk to your doctor about what foods can help you stay healthy and any dietary supplements you may need. These are often a necessary part of the diet for people with sickle cell disease. They're even more important if you aren't eating enough folate-rich leafy vegetables (such as spinach).

  • Don't smoke.

    Smoking and secondhand smoke reduce the amount of oxygen in your bloodstream.

  • Exercise safely.
    • Rest when you feel tired.
    • Drink plenty of fluids when you exercise. Strenuous exercise can cause dehydration and reduced oxygen levels in your blood. This may cause red blood cells to sickle.
    • Children can exercise and play normally if they:
      • Stay hydrated.
      • Take rest breaks.
      • Stay warm. Being exposed to cold air, wind, and water can trigger a sickle cell crisis. Dress children in warm layers of clothing for cold-weather activities. Avoid swimming and playing in cold water.
  • Stay safe at high altitudes and during air travel.

    Low oxygen levels caused by high altitudes and plane flights can cause problems. To avoid problems, drink plenty of water and fluids during air travel or when you're at altitudes higher than 5000 ft (1524 m) , such as in the mountains.

  • Recognize serious symptoms.

    Talk to your doctor about what symptoms to watch for. And know when to call your doctor and when you need emergency help.

Sickled red blood cells

Inside view of red blood cells in blood vessel, with close-up of normal and sickled red blood cells

People with sickle cell disease, an inherited condition, have red blood cells that can change form. The red blood cells can change from round into a "sickle" or crescent shape.

What causes sickle cell disease?

Sickle cell disease is inherited, which means it is passed from parent to child. To get sickle cell disease, a child has to inherit two sickle cell genes—one from each parent. These sickle cell genes are defective hemoglobin S genes.

What other health problems can happen when you have sickle cell disease?

Sickle cell disease may cause problems such as:

  • Anemia, pain, and organ failure. These are the most common and serious problems.
  • Sickle cell crisis. This is a common and painful problem that happens when blood vessels are blocked.
  • Splenic sequestration. Large, sickled blood cells get trapped in the spleen. It can cause sudden and life-threatening anemia.
  • Acute chest syndrome. Symptoms include coughing and chest pain.
  • Severe infections.
  • Aplastic crisis. The bone marrow stops producing red blood cells. This causes sudden, severe anemia.
  • Stroke.

Other problems include:

  • Growth slowdown. Children who have sickle cell disease often grow more slowly than normal and go through puberty later.
  • Open sores on the legs and feet. These sores can be very painful and heal slowly.
  • Eye damage. Long-term vision problems, including blindness, may result from blocked blood flow in the inner lining of eye.
  • Pulmonary hypertension. This means blood pressure is very high in the lungs.

Sickle Cell Disease

Sickled cells blocking a blood vessel

Normal red blood cells move easily through blood vessels, taking oxygen to every part of your body.

People who have sickle cell disease have mostly sickled red blood cells. These cells can get stuck and block blood flow through a blood vessel. That means red blood cells and oxygen can't flow to some parts of the body.

Treating pain from sickle cell disease at home

If you have sickle cell disease, you can take steps to treat pain at home.

  • Use a hot water bottle or a heating pad set on low.

    Do not use a heating pad when you are in bed. You may fall asleep and burn yourself.

  • Learn pain-management skills.

    These can help take the focus off of the pain, or they can help the pain medicine work better. You can work with a pain specialist to learn about different techniques, such as:

    • Guided imagery. This process uses your senses to guide your imagination into a more relaxed state.
    • Progressive muscle relaxation. This process helps you relax your muscles, which can help lower tension and anxiety.
    • Distraction.
    • Deep breathing.
    • Positive, encouraging self talk.
  • Use pain medicines.

    You and your doctor can make a pain management plan. You're probably the best judge of when you need to use a stronger medicine.

    • For mild pain, use over-the-counter ibuprofen (such as Advil), acetaminophen (such as Tylenol), or both.
    • For moderate or severe pain, you might take pain medicine prescribed by your doctor. Prescription pain medicine includes oral medicines such as codeine and ibuprofen, or codeine and acetaminophen. Some people might take oral controlled-release morphine at home.

When should you call for help?

Call 911 anytime you think you may need emergency care. For example, call if:

  • You have symptoms of a severe problem from sickle cell.
  • You have symptoms of a stroke. These may include:
    • Sudden numbness, tingling, weakness, or loss of movement in your face, arm, or leg, especially on only one side of your body.
    • Sudden vision changes.
    • Sudden trouble speaking.
    • Sudden confusion or trouble understanding simple statements.
    • Sudden problems with walking or balance.
    • A sudden, severe headache that is different from past headaches.
  • You are in severe pain.
  • You have symptoms of a heart attack. These may include:
    • Chest pain or pressure, or a strange feeling in the chest.
    • Sweating.
    • Shortness of breath.
    • Nausea or vomiting.
    • Pain, pressure, or a strange feeling in the back, neck, jaw, or upper belly or in one or both shoulders or arms.
    • Lightheadedness or sudden weakness.
    • A fast or irregular heartbeat.
    After you call 911, the operator may tell you to chew 1 adult-strength or 2 to 4 low-dose aspirin. Wait for an ambulance. Do not try to drive yourself.

Call your doctor now or seek immediate medical care if:

  • You have a fever.

Watch closely for changes in your health, and be sure to contact your doctor if you have any problems.

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The content above contains general health information provided by Healthwise, Incorporated, and reviewed by its medical experts. This content should not replace the advice of your healthcare provider. Not all treatments or services described are offered as services by us. For recommended treatments, please consult your healthcare provider.