Pain Crisis (Vaso-Occlusive Crisis) Under certain conditions—such as exposure to extreme temperatures, fever, dehydration, infection, or stress—red blood cells can become sickle shaped in people who have sickle cell disease. This may block the blood vessels, preventing oxygenated blood from getting to the body’s tissues. This lack of oxygen can be very painful. Damage to the blood vessels and swelling in the area of the blockage can increase
pain. Dactylitis, or hand and foot syndrome, often is the first type of sickle-cell related pain experienced by babies and toddlers with sickle cell disease. Dactylitis is swelling and extreme tenderness in the hands, feet, or both. Pain not attributed to an injury (for example, a sprain, broken bone, or constipation) should be evaluated and treated as a pain crisis. Older children and adolescents will mainly experience pain in their long bones (arms and legs), chest, and back. Your child’s healthcare provider will prescribe pain medications and give instructions for their administration. However, there will be times when home treatment is not enough to relieve the pain. In these cases, admission to the hospital or a trip to the emergency room or clinic may be necessary. This is discussed more in Pain Medication section. Acute Chest Syndrome Acute chest syndrome is a very serious complication of sickle cell disease and is the second most common reason those with sickle cell disease are hospitalized. Acute chest syndrome is the result of sickle-shaped red blood cells blocking the tiny vessels of the lungs. It is characterized by chest pain, difficulty breathing, fever, and changes to the lungs as seen on an X ray. Acute chest syndrome may be caused by pneumonia. A pain crisis may lead to acute chest syndrome, because pain can make it more difficult to expand the lungs and take deep breaths. Pain medications can make a child drowsy, which also interferes with taking deep breaths. For this reason, you and your child will be taught deep-breathing exercises, called incentive spirometry , to be used during a pain crisis to help prevent or treat acute chest syndrome. Often, individuals experiencing acute chest syndrome will need a red blood cell transfusion. Given early, a transfusion may prevent transfer to an intensive care unit or the need to use a ventilator. Aplastic Crisis
If your child develops signs of worsening anemia, such as fatigue (extreme tiredness), severe headache, or extremely pale lips and nail beds, your hematologist may suspect an aplastic crisis (a decrease in red blood cell production). An aplastic crisis has various causes, including parvovirus. Parvovirus B19 infection, known as “fifth disease,” is a very common childhood infection that causes mild fever, cold symptoms, and a rash on the cheeks and other body parts. Parvovirus B19 infection can shut down the production of red blood cells in the bone marrow for up to 10 days. For most children,
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