From the staff of the Georgia Comprehensive

Sickle Cell Center at Grady Health System

Pain is one of the most common and distressing symptoms that sickle cell patients have. This booklet is a review of pain prevention, home treatment, emergency treatment and inpatient treatment in the hospital. The more one knows about the causes, prevention and treatment of pain, the better the chances of an early recovery. There are different types and different causes of pain requiring a clinician’s help and advice about treatment. Pain assessment, treatment and education are better measured because of new requirements by the Joint Commission on Accreditation of Healthcare Organizations, (JCAHO), the agency that inspects and certifies all US hospitals. These new pain management standards should improve the pain care sickle cell patients receive.

For general pain prevention remember FARMS –
F - for Fluids and Fever, drink plenty of water and Fever management. If you get a fever see your health care provider right away.
A –is for Air and making sure you do not get into problems with not enough oxygen like an unpressurized airplane.
R- is for Rest, so get plenty of sleep, do not over do it and take plenty of breaks when your body feels tired.
M - is for prevention medications, like daily penicillin for children under six or hydrea for pain prevention. The vitamin Folate is needed to make new red blood cells
S - is for Situations to avoid, like getting too hot or cold, avoiding smoking, alcohol or illegal drugs.

Exercise and work out doors with the proper clothing for the season, drinking plenty of water, and take frequent rest and water breaks. Carry a water bottle with you to keep drinking. Avoid swimming pools that are too cold or hot tubs that are too hot.

Avoid emotional stress by pacing projects and work. Avoid situations that you know are upsetting. Join a support group, church or faith based group that offer spiritual and emotional support.

There are five types of pain, each with different treatments.

1. Acute sickle cell pain episode – this is pain that can last for several minutes to several days caused by blocked blood flow from the sickle red blood cells. This pain is the same as past pain episodes, usually deep in the bones and muscles of the arms, legs, and back. Pain in the head, chest, or belly should be evaluated by your health care provider. Pain with fever should also be seen immediately by a health care provider

2. Acute pain from another cause – pain that comes on suddenly that feels different than your usual pain episode should be seen by your health care provider. Sickle cell patients may have other causes of pain such as stomach ulcers, appendicitis, a slipped disk, menstrual cramps and so forth.

3. Chronic pain from sickle cell bone damage – This is pain that lasts longer than a few weeks and may be present daily. This occurs when bones are damaged by the blocked blood flow.

4. Chronic pain from other causes – This is daily pain that lasts more that 6 weeks from other causes such as a slipped disk, rheumatoid arthritis, old injuries and so forth.

5. Chronic nerve pain – This is caused by damage to the nerves from injury, sickle cell blockage, or other conditions like diabetes. The nerve damages causes a burning, tingling, numbing type of discomfort on a daily basis.

It helps your health care providers and yourself to record the following information about your pain in a daily diary. Remember the acronym LOCATES to locate the pain

L – Location - note the exact location of the pain and describe if it travels anywhere.

O – Other Symptoms - record any other symptoms like fever, nausea, cough… that came with the pain.

C – Character – describe the pain. Is it deep, burning, throbbing…

A – Aggravating and Alleviating Things – What makes the pain better and what makes the pain worse.

T – Timing – When did the pain start and has it been there all the time or does it come and go.

E – Environment and Effect – Where were you and what were you doing when the pain started. How does the pain affect your daily routine.

S – Severity – Rate how much pain you are having on a zero to ten scale. Zero is no pain at all and ten is the worst pain you have ever had in your life. Put down a number that best describes your level of pain from 0 to 10.

Pain scales should be available in the ER and the hospital. A scale that you should be familiar with is the Visual Analog Scale or VAS. It is an 10 centimeter line with numbers from 0 to 10. 0 is no pain and 10 is the worst pain you have ever had in your life. Mark on the line where you would rate your level of pain.

 0______________________5______________________10

No Pain                                                                            Worst ever pain

A printable pain diary is available by Clicking Here

1. Acute sickle cell pain episode – If this is typical pain episode type pain then start by drinking more water, laying down and resting. Take a warm bath and use distractions, such as music or relaxation techniques. If your health care provider has given you pain medication to use start taking it as prescribed. Pain medications that are available without prescription are –

Acetaminophen (one trade name is Tylenol) Acetaminophen will block fever, so do not use it until a health care provider has evaluated the fever and given the go ahead. Fever could mean a serious life threatening infection is present. Acetaminophen is often combined with mild opiates like codeine (Tylenol #3) or hydrocodone (Vicoden). Be aware these combinations will also block fever.

Ibuprofen (Motrin, Advil). Ibuprofen blocks pain in the muscles and bones. It does not affect alertness. It can block fever – so it must not be used until the fever is evaluated. Ibuprofen can cause stomach upset and ulcers. It is best taken after a meal or snack. It can block platelets from their ability to stop bleeding from cuts. This should not be used if there are kidney problems, stomach ulcers, bleeding problems or asthma. This medication is good for menstrual cramps.

Aspirin It has the same cautions as Ibuprofen. One caution to note is that aspirin has been associated with Reyes Syndrome and should not be given to children with fever or cold symptoms.

Codeine and hydrocodone are milder opiate medications that block pain in the brain. These are usually given in combination with Acetaminophen or Ibuprofen. They can be given by themselves to block pain when a fever is being monitored. These will not block fever, platelets, or cause stomach ulcers. These medications may cause drowsiness, nausea and itching. These are prescription medications.

2. Acute pain from another cause – pain that comes on suddenly that feels different than your usual pain episode should be seen by your health care provider. Pain in the head, chest, or abdomen and pain with fever should be evaluated immediately. The pain should be carefully evaluated then the pain can be safely managed.

3., Chronic pain from sickle cell bone damage – This is pain that lasts longer than a few weeks and may be present daily. This occurs when bones are damaged by the blocked blood flow. The best treatment for this type of pain is long acting. Physical therapy, weight loss, decrease weight bearing, Transcutaneous Nerve Stimulators (TNS), mild heat. The long acting arthritis medications are very helpful for daily pain control. New pain agents are being developed with fewer side effects. The best medications for long pain fighting effect with the least side effects are the arthritis medications called non steroidal anti-inflammatory drugs or NSAIDs for short:

Salsalate (Disalcid) is recommended for adults only at a dose of 1000mg twice a day. Caution is to be used in those with kidney problems or stomach ulcers.

Celecoxib (Celebrex) Adult dose 100mg - 200mg twice a day

 Celecoxib, may be safer than other arthritis medications for long term use in causing less stomach ulcers. Check with your doctor and let them know if you have kidney or stomach ulcer problems.

Opiate medications may be used with the NSAIDs if the pain is not controlled or if the NSAIDs can not be used. The long acting opiates are morphine (MS Contin), oxycodone (Oxycontin) or methadone. All of these agents block pain in the brain and cause drowsiness, constipation, tolerance, physical dependence and physical withdrawal if they are suddenly stopped. Antidepressant medications work to help along with the pain medication to help fight chronic pain. A stool softener should be used when opiates are being taken daily to prevent constipation.

4. Chronic pain from other causes – This is daily pain that lasts more that 6 weeks from other causes such as a slipped disk, rheumatoid arthritis, old injuries and so forth. All of the same therapies listed above are used as well as, nerve blocks, and disease specific medications

5. Chronic nerve pain or neuropathic pain – This is caused by damage to the nerves from injury, sickle cell blockage, or other conditions like diabetes. Treatment with anti- seizure medication has been helpful in pain control. One such medication is gabapentin (Neurontin).

The emergency room (ER) is the next stop if home treatment fails or a danger sign such as fever, weakness, atypical pain, headache, chest pain, or abdominal pain is present. There are special emergency rooms for sickle cell patients in Atlanta and New York City, and we hope for more to come., Most sickle cell patients must seek out the emergency room with the best possible care. Some hospitals may not have staff well trained in the care of sickle cell patients. The best defense is a good offense, come prepared with knowledge.

First your vital signs should be taken including your temperature (normal 37.8 or below) breathing rate (normal 15 – 20 per minute), your heart rate – pulse (Normal 80 – 100 per minute) , blood pressure (Normal 129/80) pulse oximetry – a measure of the oxygen in your red blood cells (Normal 95% - 100%) and your pain intensity score – how much pain you are having from 0 to 10.

A nurse should find out your main problem and assess how quickly you will be seen. In all ERs the most life threatening conditions must be seen first. A pain episode, although distressful is not life threatening. There are symptoms that could be serious such as fever, chills, headache, chest pain, abdominal pain, weakness, and abdominal swelling that need to be reported immediately.

A doctor, physician assistant or nurse practitioner should examine you for signs of infection or complications. They should look in your ears, eyes, mouth , nose, listen to your heart, chest and abdomen. They should also feel your abdomen for tenderness or swelling of the liver or spleen. They should check all of the areas that are hurting for swelling, heat or tenderness.

Blood tests including a complete blood count (CBC), reticulocyte count and chemistry values may be done. A urine sample may be checked for blood, protein and infection.

Your doctor may have developed an individual treatment plan for you. Generally, an IV or intravenous line should be started with D5W or 5% sugar (dextrose) in water to rehydrate the sickle red blood cells. Generally, normal saline should not be used. Your legs are the last resort for an IV because a leg ulcer could develop.

Pain medication should be given in the IV if possible and should be given on a fixed time schedule based on the medication. Medication given on a fixed schedule assures a good pain fighting blood level can be reached and kept. If pain medication is given as needed (p.r.n.) or as requested, the pain relief is like a roller coaster up and down. Pain medication given by Patient Controlled Analgesia or PCA pump allows a continuous amount to go in all the time. You can use the pump button to give extra doses at a safe rate for extra pain control

Your pain should be reassessed to see if the medication is working.

Try to continue distraction therapy by, reading a book, watching TV, listening to music or playing a game. Relaxation techniques can be used.

Many good pain medications are available for pain episodes and many work better in combinations.

Morphine – Morphine is opiate medication that blocks pain in the brain. It can be given by mouth, IV, in a patient controlled analgesia pump (PCA), or by shot in the muscle. Morphine takes effect in 15 minutes and lasts up to 3 hours. It can cause itching, nausea, vomiting and constipation. The body can become physically dependent on morphine after use for several days. The dose should be tapered gradually and not stopped suddenly. It slows breathing down as the dose goes up.

Nalbuphine (Nubain) is an injectable pain medication that is safe and effective in doses of 10 to 20 mg IV, every 3 hours. It does not slow respirations as morphine does. It has a "ceiling effect" that is if it does not control pain at 20 mg every 3 hours, you must switch to another medication. You can not increase the dose beyond this maximum. This medication has fewer side effects with less itching, nausea, and drowsiness than morphine. This medication may cause withdrawal symptoms in-patients taking daily opiates (MS Contin, Oxycontin, Methadone…) and it should not be used in those patients.

Ketorolac (Toradol) – This is like an IV ibuprofen that blocks pain in the bone and muscle tissue where the blocked blood flow from sickled red cells has caused damage. All of the cautions are the same as ibuprofen. The usual dose in an adult is 30 mg IV every 6 hours. This medication can only be used for a maximum of five days continuously.

Meperidine (Demerol) – This opiate pain medication is good for acute pain, but not for pain lasting more than a few days. It breaks down in the body to a substance called normeperidine that can cause seizures in high doses. This medication has all of the same cautions as morphine. Morphine is a better first choice opiate and Nalbuphine is a reasonable choice for those with moderate pain who have not been exposed to opiates on a regular basis.

Hydroxyzine (Vistaril) in doses of 25mg every 6 hours can prevent nausea, and itching caused by opiates. It can also help calm fear.

Admission to the hospital for pain management, or treatment is required if the pain does not go down to a manageable level after 8 –12 hours of treatment in the ER or if a complication is present. Returning for further pain therapy within 48 hours of previous inpatient or outpatient treatment of a pain episode is one complicaton. Other complications are.

- Infection – temperature over 38 C, pneumonia, kidney infection, blood infection (sepsis)

- Low blood oxygen or too much acid in the blood

- Stroke

- Pregnancy.

- Heart problems or failure

- Priapism that will not go away.

- Blood clots in the lung or acute chest syndrome

- Decrease in blood counts

- Liver inflammation, gall stones or gall bladder inflammation

After you are admitted in the hospital, treatment started in the ER should continue. There are several things you can do to insure adequate pain management. Pain assessment is important and should be done to record your pain level. You should let the staff know your pain intensity level as a number from 0 to 10. Also tell the nurses if you are having side effects like nausea, vomiting, constipation, itching or feeling too drowsy. Your mood is also important and should be reported to the nursing staff. Feelings of depression, fear, anger, and sadness can all hinder your pain treatment. Counsel from chaplains, social workers, nurses, as well as medication can help in many cases.

Medication by Patient Controlled Analgesia (PCA ) pump and the start of long acting pills is one of the best options for pain control in the hospital. PCA pumps give you the patient some control over the amount of medication you get. It allows you to self medicate safely and prevents lapses in time between medication doses. Report to your nurse any change in symptoms, or sites of pain.

Pain can be helped by thinking about something else. Dwelling on the pain can make it feel worse. Being tense tightens muscles, reduces blood flow and increases pain. Relaxation techniques allow you to help relax muscles and get you mind off of the pain.

Start by getting in a quiet room, with some soothing music if it is available. Get in a comfortable position. Make a fist and then release you fingers and concentrate on letting them go limp. Tense your arm muscles and then let them go limp. Tense your shoulders, then let them go limp. Tense your neck then let it go limp. Squench your face muscles and let them go limp. Tense your toes and feet, then let them go limp. Tens your leg muscles, and let them go limp. Tense your stomach and let it go limp.

All of your muscles should be relaxed. If any feel tense, repeat tightening them and letting them go until they are relaxed. Think of a calm place that you have visited like a beach or park. Meditate on your favorite scriptures, song or event.

Biofeedback can help train you to relax.

All opiate medications can cause changes in the response to continuous use for a week or two. This is called physical dependence. The body then requires higher doses of the opiate to get adequate pain control. This is called tolerance. If any of the opiate medications are used for several days the body will have withdrawal symptoms such as cramps, sweating, abdominal pain and shakes. Your role is very important to prevent these symptoms. Withdrawal can be avoided by slowly decreasing the amount of the medication used over several days to allow the body to adjust. As your pain level decreases, ask your providers to decrease the amount of medication. Many healthcare providers do not understand these effects of opiates and it is all mislabeled as addiction. Opiate addiction occurs when the person seeks and uses the drug despite harm to them and their loved ones. Their life revolves around use of the drug at the expense of family, and health. True drug addiction occurs in only 5 – 10% of patients on daily opiates. After following over 2400 sickle cell patients in the Georgia Comprehensive Sickle Cell Center, only 5% met the criteria for drug addiction.

Pseudo-addiction – patients under treated with opiates may experience uncontrolled pain and withdrawal symptoms. Many health care workers have mislabeled these symptoms as addiction and drug seeking behavior. Patients must keep returning to the ER with pain seeking help. An adequate understanding of how opiates work and the proper doses of long acting medications can stop this vicious circle.

Conclusion

You are an important partner in your care. Discuss your pain management plan with your health care provider when you are well and not in pain. Make a plan that is right for you. Keep this book with you and fill in the important information to share with each health care provider you see. There are free problem oriented guidelines for sickle syndromes available for health care providers on the internet 24 hours a day at www.SCInfo.org

Ask your nurses, doctors, and healthcare providers questions about your care and health. Empower yourself with knowledge. It is the greatest weapon you have in the battle against pain.

James R. Eckman, MD, Professor and Chief of Hematology/Oncology, Winship Cancer Institute, Adjunct Professor of Pediatrics, Division of Medical Genetics Emory University School of Medicine, Medical Director, The Georgia Comprehensive Sickle Cell Center at Grady Health System

Lewis Hsu, MD, PhD, Assistant Professor of Pediatrics, Division of Pediatric Hematology-Oncology Bone Marrow Transplant, Attending Physician at the Georgia Comprehensive Sickle Cell Center at Grady Health System

Melanie Copper-Effa, MD,   Assistant Professor of Hematology/Oncology, Winship Cancer Institute, Emory University School of Medicine, Attending Physician at the Georgia Comprehensive Sickle Cell Center at Grady Health System

Beatrice Gee, MD, Assistant Professor of Pediatric Hematology Oncology Morehouse School of Medicine, Attending Physician at the Georgia Comprehensive Sickle Cell Center at Grady Health System

Allan Platt, PA-C, Clinical Instructor, Physician Assistant Program  Emory University,  Program Coordinator, The Georgia Comprehensive Sickle Cell Center. Website coordinator.

Marietta Collins, PhD, Pediatric Psychologist at the Georgia Comprehensive Sickle Cell Center at Grady Health System

JoAnn Beasley, RN, Clinical Manager, Newborn Screening Coordinator the Georgia Comprehensive Sickle Cell Center at Grady Health System

Ingrid Whittaker-Ware, Esq, Patient Representative

Artwork by Donna Dent, Research Coordinator, The Georgia Comprehensive Sickle Cell Center and Emory University School of Medicine

Send mail to aplatt@emory.edu with questions or comments about this web site.
Copyright © 1997 Sickle Cell Information Center
Last modified: January 12, 2007