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New Treatment Plan Leads to Better Pain Control for Acute Sickle Cell Crisis

May 15, 2017
Patient controlled analgesia or scheduled IV injections recommended over as needed pain control are just 2 recommendations to reduce pain and decrease hospital stays in patients with sickle cell crisis.

Interview with Jennifer Yu, MD, and Paula Groncy, MD

There’s new hope for the 70,000 to 80,000 Americans who are currently living with sickle cell disease (SCD). Thanks to new guidelines for therapy, SCD patients have better pain control during acute pain crisis and reduced hospital stays. The guidelines were presented by Jennifer Yu, MD, a pediatric hematologist/oncologist at Rady Children's Hospital-San Diego and an assistant clinical professor of pediatrics at UC San Diego School of Medicine during the 2017 American College of Physicians (ACP) Internal Medicine Annual Meeting held in San Diego.1

Dr. Yu said these recommendations are based mainly on the National Institutes of Health’s Evidence-Based Management of Sickle Cell Disease, Expert Panel Report 2014.2

Exploring the Link Between Sickle Cell Disease and Pain

“Sickle cell disease is caused by a mutation in hemoglobin, which is the protein within our red blood cells that carries oxygen to all of the cells in our body. When the red blood cells are low on oxygen, the mutation causes an irreversible change in the sickle hemoglobin that makes the usually flexible cells [become] so rigid that they get stuck in the small blood vessels and cause a vaso-occlusion. The vaso-occlusion also involves white blood cells and platelets that cause inflammation,” Dr. Yu explained.

This situation can lead to sudden episodes of acute pain (commonly referred to as an acute pain crisis) and risk for organ damage.3 “Pain is the most common reason that patients with sickle cell disease are admitted to the hospital,” Dr. Yu said in an interview with Practical Pain Management.

Creating a Comprehensive Response to Manage Pain

For SCD patients, the majority of whom are of African descent with a smaller number of Hispanic descent, the treatment options available to date have been quite limited. “The only disease modifying therapies we have for patients with sickle cell disease at this time are red blood cell transfusion and hydroxyurea [Hydrea and Droxia],4 and the only cure currently is bone marrow transplantation,” Dr. Yu said.

Since both options have been associated with complications and side effects, Dr. Yu pointed out that clinicians may find that adopting strategic guidelines to get the most out of these therapies can lead to improved quality of life for the people affected.

Dr. Yu's pain management recommendations:

  • Treating patients suffering from an acute pain crisis by delivering opioids or other pain medications via injection on a scheduled basis—rather than as needed—so the pain can be better controlled
  • Checking with patients regularly to adjust dosing as needed for optimal pain control
  • For those who need frequent doses of IV pain medications, using patient-controlled analgesia (PCA)
  • Using maintenance intravenous (IV) hydration for sickle cell patients in pain in order to effectively reduce the concentration of sickle cells
  • Using an incentive spirometer, which is a device that helps patients take deep breaths, every few hours to keep the lungs open during an acute pain episode
  • Exploring therapy and alternative approaches such as biofeedback and heat packs

To modify the course of the disease, Dr. Yu recommends:

  • Performing a blood transfusion using phenotypically-matched blood in severe cases to replace atypical hemoglobin (or hemoglobin S) with red blood cells containing normal hemoglobin
  • Using hydroxyurea to produce hemoglobin F, which can help oxygenate the tissues and achieve a similar effect as a blood transfusion with fewer complications. (Please note: Medical follow-ups are crucial due to the risk of bone marrow suppression occurring.)

Dr. Yu pointed out that such therapies should be part of a broader, comprehensive strategy to manage the many other acute and chronic complications that can occur with SCD.

Applying the Guidelines to the Pediatric Population

Although the guidelines Dr. Yu discussed at the meeting were directed at clinicians who treat adults with SCD, she pointed out that children can also benefit from adopting these steps as part of their general protocol.

“The Pediatric Sickle Cell Center at Miller Children's Hospital, similar to most pediatric sickle cell centers across the nation, has been treating pain episodes in children with sickle cell disease using either around-the-clock opioids or PCA combined with around the clock nonsteroidal anti-inflammatory drugs [NSAIDs],” said Paula Groncy, MD, board certified pediatric hematologist/oncologist and Medical Director Miller Children’s & Women’s Hospital Long Beach (California) Sickle Cell and Hemophilia Centers.

“We agree that length of stay is decreased with Dr. Yu’s approach [of providing pain medicine on a schedule] rather than using them on an as-needed/as-requested basis. When our older teens/young adults are treated elsewhere with as-needed pain medications, their length of stay is longer,” Dr. Groncy said in an interview with Practical Pain Management.

“Hydroxyurea, which is now recommended for all patients with sickle cell disease starting at 6 to 12 months of age, not only significantly reduces pain episodes and episodes of acute chest syndrome, but also has been demonstrated to reduce organ damage caused by sickling of red blood cells,” she added.

“About 10 of our patients with an HLA [Human Leukocyte Antigen]-identical sibling have undergone successful bone marrow transplants,” Dr. Groncy said. HLAs are proteins that are located on the surface of the white blood cells and on other tissues in the body. When 2 people have a match, their tissues will be immunologically compatible with each other, she added. For patients who don’t have an HLA identical sibling for a donor, bone marrow transplant may worth exploring.

Incorporating Screening, Prevention, and Research Opportunities

For SCD patients of all ages, Dr. Yu stressed that the guidelines go beyond treatments to also recommend following patients regularly to ensure they receive the appropriate screening and preventative measures.

“There are also multiple ongoing trials for therapies that can treat an acute pain crisis and gene therapy trials looking to cure SCD,” Dr. Yu said. “Since patients with SCD have a much shorter life expectancy than the average American [closer to 40 to 50 years old, rather than 70 to 80 years old]," finding more treatment options to change the course of the disease is so critical, she said.

Last updated on: May 15, 2017
Continue Reading:
New Look at Sickle Cell Patients With Chronic Pain

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