Sickle Cell Pain

What Is Sickle Cell Disease?

Sickle cell disease is the name given to a group of illnesses that are a result of red blood cells that become misshapen. The most common type of sickle cell disease is sickle cell anemia.

In most people, red blood cells are round and smooth. This round and smooth shape allows the cells to move easily through the blood vessels. People with sickle cell disease have abnormal hemoglobin, which is a protein found in the red blood cells. The sickle cell hemoglobin can cause the red blood cells to change shapes, from the normal round shape into a crescent (or sickle) shape. The sickle shaped red blood cells are hard and sticky and do not pass well through blood vessels. As a result, blood vessels can sometimes become clogged, preventing blood from flowing through them. A clogged blood vessel is called an "occlusion." Occlusions in blood vessels can lead to a painful sickle cell crisis.

Sickle Cell Crisis
Not all people with sickle cell disease have crises. About 30% rarely or never have the pain caused by a crisis. About 50% have only a few crises throughout their lives. And about 20% have frequent, severe sickle cell crises.

People who get sickle cell crises find that there are several factors that may cause a crisis to start. These include:

Having an infection, such as a cold or the flu
Cold weather
Being overly tired or fatigued
Exercising too hard
Being dehydrated (not drinking enough water and other fluids)
Not having enough oxygen in the blood

Sickle cell crises often become so painful that people have to go to the emergency room to get treated immediately with pain medications.

Who Gets Sickle Cell Disease?

In the United States, sickle cell disease is most common among African-Americans. One in 500 African-American children in this country is born with sickle cell disease. About 72,000 people in the US currently have sickle cell disease, and it affects people all over the world.

Sickle cell disease is not contagious, meaning that it cannot be spread from person to person through the air or through contact. Rather, sickle cell disease is inherited. This means that the disease is passed through families, from parents to their children. Each time a baby is born, he or she has two genes that determine the make-up of the hemoglobin -- one that comes from the mother and one that comes from the father. In families with a history of sickle cell disease, there are three possibilities:

Family members may have two genes for healthy hemoglobin. These family members will have no sign of sickle cell disease.
Family members may have one gene for healthy hemoglobin and one gene for sickle hemoglobin. This is called having the "sickle cell trait." People with sickle cell trait are not sick and often do not know they have the sickle cell gene, unless they are tested.
Family members may have two genes for sickle hemoglobin. These family members will have sickle cell disease.

People with any family history of sickle cell disease, or who have the disease, may want to speak with a doctor before having children to find out how likely a child is to be born with the disease.

What Are the Symptoms of Sickle Cell Disease?

Children may start to have symptoms of sickle cell disease as young as 6 months of age. Babies who are suffering from sickle cell symptoms may be irritable or cranky and cry even when their parents do everything they can to comfort them. Also, children with sickle cell disease may develop more slowly and have difficulties in school because of the pain from the disease.

The most common symptom of sickle cell disease is pain. During a painful crisis, pain may seem to come from the bones, usually in the arms, hands, legs, feet, or back. Also, there may be pain in the stomach or abdomen, or in the chest.

Other than pain, there are additional health problems that those with sickle cell disease may experience.

People with sickle cell disease are more likely to get infections.
Sickle cell disease can cause anemia (or a low blood count), which can make people to feel tired.
Organs (such as the liver and kidneys) can be damaged if too many large blood vessels in the area are blocked and the organs do not get enough oxygen.
People with sickle cell disease may suffer from "acute chest syndrome," which is difficulty breathing and severe chest pain.
People with sickle cell disease may have problems with their eyes as a result of blockages in small blood vessels.
Skin ulcers, or sores on the skin that do not heal well, may develop, especially on the lower legs.
People with sickle cell may become jaundiced, meaning that the whites of the eyes or the skin turn a yellowish color.

Family members of people with sickle cell disease are also affected by the illness. It may be stressful to deal with a child or loved one who has special health needs and often requires trips to the hospital. Parents and other family members may feel frustrated with the health care system and the schools for their lack of understanding about the illness.

Treatment for Sickle Cell Disease

Unfortunately, there is no cure for sickle cell disease. The good news is that there are treatments and precautions that can help treat the disease, prevent painful sickle cell crises, and relieve pain during sickle cell crises.

Some sickle cell crises can be prevented by avoiding the factors that bring on the crises. This includes avoiding exposure to cold weather, getting plenty of sleep and rest, being careful not to exercise too hard, and drinking plenty of fluids.

General Medical Treatments
Doctors may prescribe a medication to help with anemia, such as erythropoietin (a medication given as a shot to improve the blood count). Blood transfusions are sometimes used to improve anemia, as well.

Some patients may be prescribed hydroxyurea, a medication that might reduce the chance that the red blood cells will change into the sickle shape.

Also, doctors recommend that patients with sickle cell take extra folic acid, a vitamin.

Treatment for Pain
The terrible pain of a sickle cell crisis often requires a trip to the emergency room. Some sickle cell patients have had bad experiences in emergency rooms and hospitals when health care professionals questioned their pain and why they wanted pain medications. Some may even have been accused of being addicted to the pain medications. But patients and health care professionals should know that wanting medications to help the severe pain of sickle cell crisis does not make a patient a drug addict, and does not mean that they are trying to "get high" from the medications. These bad experiences may lead sickle cell patients to distrust health care professionals. It is important that patients are able to find a doctor who they trust to believe them and take good care of them.

Still, patients and their family members may worry about addiction to pain medications, particularly opioids (also called narcotics). However, for many patients opioid medications can be extremely helpful, particularly during a painful crisis. Patients who have many crises can sometimes benefit from taking opioid medications daily, along with additional pain medication during crises. The daily opioids can help reduce the number of crises and make the pain less severe.

Other medications that may be prescribed by a doctor for relief from sickle cell pain are:

Hydroxyurea
Anti-inflammatory medications, such as ibuprofen
Steroids
Certain antidepressant medications or anticonvulsant medications that also help relieve pain

Nondrug Treatments
A doctor may recommend that people with sickle cell disease see a physical therapist. Physical therapists can use gentle exercises and heat and cold treatments to help with sickle cell disease pain.

Also, it may be beneficial for people with sickle cell disease to see a psychologist, social worker or psychiatrist to get help coping with the illness or learn techniques that may be helpful in controlling pain. It can also be helpful for family members of patients with sickle cell disease to speak with a mental health professional about the stress and frustration they may feel. For more information on sickle cell pain and treatments, visit our Pain Medicine section.

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