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Sickle Cell Pain
People with sickle cell disease have red blood cells that are crescent-shaped. These blood cells are hard and sticky and can clog blood vessels, preventing blood flow. Clogged blood vessels can lead to a painful sickle cell crisis.

Wanting medication to relieve the severe pain does not make a patient a drug addict or mean that he or she is trying to "get high."

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What Is Sickle Cell Disease?
Who Gets Sickle Cell Disease?
What Are the Symptoms of Sickle Cell Disease?
Treatment for Sickle Cell Disease
Treatment for Sickle Cell Pain
Bibliography
Sickle Cell Links
Battling Pain...Sickle Cell Anemia Streaming Video Program
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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. While there is no cure for sickle cell disease, there are precautions for preventing painful sickle cell crises and treatments to provide relief during pain crises. 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 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. Folic acid is reduced in sickle cell patients due to the nature of this disease. The increased requirement for folic acid is common to all patients who have this acquired hematologic disorder. The usual requirement is 1 mg per day orally for the rest of the patient’s life. Folic acid prevents severe anemia in sickle cell disease.

Other studies have suggested that the more severe and painful the shingles rash is, the greater the chance of long-lasting PHN pain. Also, some studies have concluded that people who do not cope well with stress and pain may have worse PHN than others who cope better.



Treatment for sickle cell 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. 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 (e.g., Hydrea and Droxia)
Anti-inflammatory medications, such as ibuprofen or aspirin
Steroids, such as prednisone
Tricyclic antidepressants, such as amitriptyline
Anticonvulsants, such as Gabapentin

Non drug 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.

Transcutaneous electrical nerve stimulation (TENS) – a low intensity electrical current can sometimes be effective for pain relief.
Physical therapy – gentle exercises and heat and cold treatments can help with sickle cell disease pain.
Relaxation techniques – learning how to relax can help patients cope better with the illness and pain.
Massage – especially at the first signs of a crisis, massage can decrease muscle stiffness and potentially reduce pain.
Acupuncture and acupressure – can be useful in helping to relieve stress and manage pain.
Distraction – engaging activities (such as hobbies, video games and movies) that change the patient's focus can help relieve stress and pain.
Psychotherapy – speaking with a mental health professional about the stress and frustration of sickle cell disease and/or learning cognitive/behavioral techniques for coping with the disease can be beneficial.

How you can obtain treatment for pain

Find medical practitioners who understand sickle-cell-related pain.
Ensure regular visits to sickle cell pain specialists.
Ask questions of doctors and discuss your concerns.
Take pain medication as prescribed.
Massage sore or painful areas if the physician approves.
Encourage the use of relaxation techniques to relieve stress and decrease pain. Here is more information on relaxation techniques.
Find ways to avoid and manage stress in the household.
Engage in activities you enjoy.
Watch for signs of pain or other discomfort.
Rate and record pain in a daily pain journal.
Contact your medical practitioner if pain is not relieved by medication.




Sickle Cell Links

Organizations

American Sickle Cell Anemia Association (ASCAA)
10300 Carnegie Avenue
Cleveland Clinic / East Office Building (EEb18)
Cleveland, OH 44106
(216) 229-8600
Provides a nationwide directory of support groups that includes groups for both patients and parents. Other services performed by ASCAA include testing, education, counseling, support services, newborn testing, lead screening, genetic counseling, and an online bulletin board where you can discuss issues related to sickle cell with peers.

National Institutes of Health National Heart, Lung, and Blood Institute
2 Rockledge Centre
6701 Rockledge Drive
MSC 7950, Room 100420
Bethesda, MD 20892-7950
(301) 435-0055
This is the primary National Institutes of Health organization for research on sickle cell anemia.

Sickle Cell Disease Association of America
4221 Wilshire Boulevard
Los Angeles, CA 90010
(800) 421-8453
This website provides answers to frequently asked questions about sickle cell disease and offers an online chat room where patients and caregivers can communicate with peers.

Websites

Emedicinehealth: Sickle Cell Crisis
This website provides comprehensive information on prevention and management of a sickle cell crisis.

Health Choices: Battling Pain...Sickle Cell Anemia Video
This video stream features issues commonly faced by sickle cell patients.
Runs approx. 60 minutes.
      To view the videostream, you will need Real Player. Download the latest version free from RealNetworks, Inc.

MedicineNet: Sickle Cell Disease
This site provides medical information (diagnosis, treatment, etc.) as well as patient discussions about sickle cell disease.

MEDLINEplus: Sickle Cell Anemia
MEDLINE is a service of the National Library of Medicine that provides excellent links to websites for a variety of topics related to sickle cell disease.

Nemours Foundation
This site, which is geared toward the parents of sickle cell patients, provides medical information on the disease.

Sickle Cell Information Center
Georgia Comprehensive Sickle Cell Center at Grady Health System
This is one of the most comprehensive sickle cell sites and has information provided and reviewed by an advisory board composed of physicians and other health care professionals. The types of information provided include medical, newborn screening, research, and educational materials for health care professionals.

Espaņol/Spanish Information

March of Dimes: Anemia Falciforme (Biblioteca de Salud)
MEDLINE is a service of the National Library of Medicine that provides excellent links to websites for a variety of topics related to sickle cell disease.




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