Pediatric Pain SIG Newsletter
Fall 2021
IN THIS NEWSLETTER
2. Research corner : "Sleep and Pain in Pediatric Sickle Cell Disease" by: Dr. Cecelia Valrie, PhD
3. Clinical Spotlight: Patient Stories
UPDATES FROM THE PAIN SIG CO-CHAIRS
Updates from the Pain SIG Co-Chairs!
We are pleased to share the Fall 2021 edition of Division 54's Pain SIG Newsletter! We hope to highlight important topics in pediatric pain, including research, clinical, and education activities, as well as update readers on what we've been up to in the Pain SIG. We also gathered lived experiences from a diversity of youth with pain across the country who were courageous in sharing their “pain journey”!
First, September is Pain and Sickle Cell Disease Awareness Month. The newsletter will highlight research from Dr. Cecelia Valrie discussing the importance of sleep and pain management in youth with sickle cell disease.
Second, we are co-hosting a webinar with the Hem/Onc/BMT SIG in honor of Pain, Childhood Cancer, and Sickle Cell Disease Awareness Month. Dr. OJ Sahler will be speaking about non-pharmacologic management of pain on September 27th, 2021 at 12pm EST. To Register: https://forms.gle/FYHHKcDfnLN4X37t9 Questions: hemoncbmt@gmail.com.
Third, the Hem/Onc/BMT and Pain Special Interest Groups (SIGs) are collaborating to coordinate a Scientific Symposium submission for consideration at the upcoming 2022 Society of Pediatric Psychology Annual Conference (April 8-10 in Phoenix, Arizona). We are soliciting individual abstracts to be included in this symposium submission. Specifically, we seek abstracts consistent with the broad conference theme of Advancing Advocacy and Leadership through Science and Practice in Pediatric Psychology. Abstracts should be focused on pain and/or its management in the context of hematology, oncology, and/or bone marrow transplant populations. Abstracts (300 words max) should be sent by October 8, 2021 to Victoria Cosgrove, PhD (veileen@stanford.edu) and Aimee Hildenbrand, PhD (aimee.hildenbrand@nemours.org) for consideration. Abstracts should adhere to the specific guidelines outlined in the Society of Pediatric Psychology’s Call for Proposals (pedpsych.org/sppac/call-for-proposals/).
Finally, if you have an article that you would like to contribute to a future edition, please contact our Student Representative, Angela Pascale (pascaleac@vcu.edu)! A special thanks to those who contributed to the current newsletter! Please enjoy the read!
Your SIG co-chairs,
Katie Salamon, PhD
Emily Wakefield, PsyD
RESEARCH CORNER
SLEEP AND PAIN IN PEDIATRIC SICKLE CELL DISEASE
Written by: Dr. Cecelia Valrie, PhD
Associate Professor, Department of Psychology
Adjunct Associate Professor, Department of Internal Medicine
Interim Director, Health Psych PhD Program
Director, Pediatric Health and Development Lab
Chair, VCU iCubed Culture, Race, and Health Core
Virginia Commonwealth University, Richmond VA
Email: cvalrie@vcu.edu
Sickle cell disease (SCD) is the most common red blood cell genetic disorder in the world, affecting people of primarily African descent. [1] Pain is the most recognized complication associated with the disease, and often presents as recurrent, acute pain episodes during childhood transitioning to chronic pain during late adolescence/adulthood. [2-4]Sleep problems in individuals with SCD are also common and tend to be comorbid with SCD pain. [5, 6] In this article, I will be describing key findings from my team’s work focused on understanding the sleep and pain relationship in youth with SCD, and using it to highlight important implications and future directions needed to advance the field.
An important question to address in understanding the sleep-SCD pain relationship is whether sleep should be treated as a consequence of pain experiences or a factor that drives or helps us predict pain. Thus, we evaluated the temporal relationship between sleep features and pain in 88 youth (aged 8 to 17 years) with SCD using mHealth technology (i.e., ecological momentary assessments, sleep actigraphy, and overnight pulse oximetry). [7] Results indicated a bi-directional relationship between poor self-reported sleep quality during the night and high daily pain severity. Sleep duration was not related to pain severity. These findings were consistent with a study of 30 youth with SCD that found a cyclic relationship between poor sleep efficiency and pain; and no relation between sleep duration and pain. [5] They highlight the need to examine what specific aspects of sleep are related to pediatric SCD pain features, as this knowledge is key for determining the most effective targets for clinical interventions.
We also found that older youth with SCD experienced more sleep problems, and that as age increased, the strength of the relationship between poor sleep and high pain increased. These findings raise the question of whether adolescents are particularly sensitive to the effects of poor sleep on their pain, or if the cumulative effects of poor sleep might lead to increasing pain problems as youth age. Both issues are important for informing intervention development. [7]
A question we are currently exploring is what are some of the primary mechanisms linking sleep and SCD pain. Babiloni and colleagues (2019) review of pain-sleep relationships highlights multiple possible mechanisms linking the two variables, including affective factors. [8] Thus, we investigated positive and negative affect as possible mediators of the sleep-pain relationship in youth with SCD using our previous mHealth data. [9] Preliminary findings indicated that low positive affect mediates the bi-directional relationship between poor sleep at night and high pain during the day, while negative affect did not. These findings support a focus on interventions that target positive affect when trying to disrupt the bi-directional sleep-pain relationship in youth with SCD. More research is needed to validate these findings and identify other modifiable mechanisms that can be targeted via psychologically-based interventions.
While our previous findings are mostly consistent with other pain populations, which indicate bi-directional sleep-pain relationships and highlight affect as an important factor in understanding this relationship, youth with SCD face unique challenges that need to be considered as we advance. [10] These challenges come from both the unique pathophysiology of SCD and the socio-cultural challenges commonly faced by a largely Black population. For example, individuals with SCD are at increased risk a range of sleep problems, including insomnia, sleep apnea, and periodic limb movement disorder. [11] Cognitive behavioral therapy for insomnia (CBT-I) is the recommended treatment for insomnia symptoms [12], while recommended treatments for sleep apnea and periodic limb movement disorder are more medically based – CPAP and medication. [13, 14] Work is needed to tailor CBT-I interventions for pediatric SCD populations that consider socio-cultural factors that may both act as barriers or may strengthen the effectiveness of the intervention for the population. Patients with SCD face many barriers to access high quality interventions; thus, work is needed to tailor our interventions and our implementation of these interventions to optimize their access. For example, Palermo and colleagues evaluated the feasibility and acceptability of web and smartphone-based applications to deliver CBT for chronic pain to youth with SCD to address transportation and scheduling issues commonly experienced by the population. [15]
Lastly, youth with SCD experience more sleep disturbances than demographically similar controls, [16, 17] but this does not eliminate the need to identify socio-cultural factors that may contribute to sleep problems, and subsequent pain, for this population. For example, there is a rising body of literature indicating the poor sleep is a key mechanism linking racial discrimination and poor health outcomes. [18, 19] Individuals with SCD tend to experience racial bias and discrimination both inside and outside of health care settings. [20-22] Future research needs to explore whether these experiences and other socio-cultural factors are risk factors for sleep problems, and subsequently high pain, in youth with SCD. If so, how do we address and mitigate their effects. Overall, important strides have been taken in understanding the relationship between sleep and pediatric SCD pain, but there is still plenty of work to be done to fully understand the relationship and use that information to improve the lives of youth with SCD.
References
1. Rees DC, Williams T and Gladwin M. Sickle-cell disease. Lancet 2010;376:2018-2031.
2. Ballas SK. Current Issues in Sickle Cell Pain and Its Management. Hematology 2007;2007:97-105.
3. Dampier C, Palermo TM, Darbari DS, et al. AAPT Diagnostic Criteria for Chronic Sickle Cell Disease Pain. Journal of Pain 2017;18:490-498.
4. Shapiro SB, Dinges FD, Orne CE, et al. Home management of sickle cell-related pain in children and adolescents: natural history and impact on school attendance. Pain 1995;61:139-144.
5. Fisher K, Laikin AM, Sharp KMH, et al. Temporal relationship between daily pain and actigraphy sleep patterns in pediatric sickle cell disease. J Behav Med 2018;41:416-422.
6. Valrie CR, Gil KM, Redding-Lallinger R, et al. The Influence of Pain and Stress on Sleep in Children With Sickle Cell Disease. Children's Health Care 2007;36:335-353.
7. Valrie CR, Kilpatrick RL, Alston K, et al. Investigating the Sleep–Pain Relationship in Youth with Sickle Cell Utilizing mHealth Technology. J Pediatr Psychol 2019;44:323-332.
8. Babiloni AH, Frimerman L, Verner M, et al. Sleep quality and prescription opioid misuse: the potential mediating role of pain intensity, negative affect, and opioid craving. Sleep medicine 2019;64:S152-S153.
9. Valrie CR The role of affect in the daily pain-sleep relationship in youth with sickle cell disease. In: Anemia. PMAtSC ed. Symposium conducted at the 2019 American Psychosomatic Society Annual Conference in Vancouver, British Colombia , 2019.
10. de la Vega R, Dudeney J and Palermo TM. Sleep and pain in children and adolescents. Oxford Textbook of Pediatric Pain 2021:146.
11. Hankins JS, Verevkina NI, Smeltzer MP, et al. Assessment of Sleep-Related Disorders in Children With Sickle Cell Disease. Hemoglobin 2014;38:244-251.
12. Tikotzky L and Sadeh A. The role of cognitive–behavioral therapy in behavioral childhood insomnia. Sleep Medicine 2010;11:686-691.
13. Kuhle S, Urschitz MS, Eitner S, et al. Interventions for obstructive sleep apnea in children: A systematic review. Sleep Medicine Reviews 2009;13:123-131.
14. Pigeon WR and Yurcheshen M. Behavioral sleep medicine interventions for restless legs syndrome and periodic limb movement disorder. Sleep medicine clinics 2009;4:487-494.
15. Palermo TM, Dudeney J, Santanelli JP, et al. Feasibility and Acceptability of Internet-delivered Cognitive Behavioral Therapy for Chronic Pain in Adolescents With Sickle Cell Disease and Their Parents. Journal of Pediatric Hematology/Oncology 2018;40:122-127.
16. Daniel LC, Grant M, Kothare SV, et al. Sleep patterns in pediatric sickle cell disease. Pediatr Blood Cancer2010;55:501-7.
17. Valrie CR, Trout KL, Bond KE, et al. Sleep Problem Risk for Adolescents with Sickle Cell Disease: Socio-demographic, Physical, and Disease-Related Correlates. Journal of pediatric hematology/oncology 2018;40:116-121.
18. Yang T-C and Park K. To what extent do sleep quality and duration mediate the effect of perceived discrimination on health? Evidence from Philadelphia. Journal of Urban Health 2015;92:1024-1037.
19. Hisler GC and Brenner RE. Does sleep partially mediate the effect of everyday discrimination on future mental and physical health? Social Science & Medicine 2019;221:115-123.
20. Wakefield EO, Pantaleao A, Popp JM, et al. Describing Perceived Racial Bias Among Youth With Sickle Cell Disease. J Pediatr Psychol 2018;43:779-788.
21. Wakefield EO, Popp JM, Dale LP, et al. Perceived Racial Bias and Health-Related Stigma Among Youth with Sickle Cell Disease. J Dev Behav Pediatr 2017;38:129-134.
22. Hood AM, Crosby LE, Hanson E, et al. The influence of perceived racial bias and health-related stigma on quality of life among children with sickle cell disease. Ethn Health 2020:114.
CLINICAL SPOTLIGHT
We are pleased to share these stories from youth diagnosed with chronic pain conditions. We asked youth to document their "pain journey" in what ever way they wanted and the three entries below capture the depth of the pain experience.
Submitted by a 19 year old Black female diagnosed with Familial Mediterranean Fever and AMPS
From the second I was born, doctors were unsure if I was going to live. I was born almost
2 months earlier than expected with multiple birth complications. My newborn body was so
small that I could fit into my mother’s hand. Growing up, mom always reminds me of the precise moment she knew I was going to be okay, which was when I held her finger through the heated incubator. On the face of it, I was a fighter and made a faster recovery than the hospital expected. As I grew older, I was hospitalized for numerous viruses and bacterial diseases because of my underdeveloped immune system. Those memories sometimes play in my mind like I am watching a 20-second recap of a sitcom. However, I was not living inside the chaotic episodes until the age of 10. This was when my transition from a mere viewer, to the character I grew up seeing, took place.
Throughout the first 10 years of my life, I began to get sicker than what I would typically
experience. I remember feeling such excruciating pain and discomfort, to the point where I
would refuse to be touched. Anything that brushed against my body would ignite the already
burning fireworks in my joints. Due to the pain, I couldn’t get out of bed and would develop a
rigid neck. Nobody knew what was going on inside me. Every emergency room visit resulted in a misdiagnosis of a flu virus. That was until one, out of the seemingly dozens of visits, the ER Supervisor decided that something else was happening within my body besides the virus. She consequently put together a team of specialists to perform tests, compile diagnoses, and find a treatment plan. After 9 months of an extremely tedious process of endless appointments and tests, I was finally given an answer to what was happening within me. I found out I had a chronic illness called Familial Mediterranean Fever Disease (FMF).
Of course, being an overdramatic pre-teen, I was purely convinced that I was dying as
soon as I got word of my diagnosis. A multitude of thoughts were ambushing my mind about
how this disease is going to overtake my body, as there is no cure or actual treatment for it. The medication prescribed, I was told, is originally used to treat gout patients. Everyone seemed completely perplexed and lost. To learn more about the disease, my mom joined an FMF support group on Yahoo to talk with others around the world about their journey, the triggers, and treatments for it. In the meantime, I had to completely readjust my life and way of living. I fully eradicated gluten, lactose, and garlic from my diet for my flares to be less extreme, and less frequent. Whilst getting used to my new presence in the world, I went for a followup doctor appointment, and it was discovered I had severe scoliosis. I felt as if I was falling into a void with no end. I did not know if I should claw at the sides, or peacefully hope that soft pillows awaited me in the never-ending abyss.
Looking back, I realize just how overwhelming this was for a 12-year-old to endure.
During that time, I would beat myself black & blue, as I felt I should be stronger in handling my
health news better. It was not only a hard time for me, but for the people around me. There was so much going through my mind: What if I can’t handle this? What if I will never be able to take care of myself? Is it worth thinking about my future? I felt defeated and lost.
To add to my stress, in 2016, the middle of my 8th-grade year, I headed back to the ER
doubled over with abdominal pain. After an immediate x-ray, it turns out I needed to have an
emergency appendectomy. The following year, I continued experiencing extreme body pain that was inexplicable. I was then diagnosed with Amplified Musculoskeletal Pain Syndrome
(AMPS). Then in 2018, my scoliosis continued to worsen. It was to the point where the back
brace was not an option. So, five days after my sixteenth birthday, I had a 12-hour spinal fusion surgery to correct my scoliosis. All of these events impacted me physically and emotionally, and my mental health worsened each year throughout my life. Shortly after, I was diagnosed and put on medication for Anxiety and Depression.
My emotions were running rampant. As an adolescent, I thought that because of my pain,
I was not capable of existing on my own. These thoughts cycled in my mind constantly. I had
experienced one too many emergency room visits where the first thing I would hear someone say is, “I’m glad you brought her in when you did.”. My health frustrated and overwhelmed me. I knew that there was a possibility that I would never have a “normal” life. I was petrified.
Everything was beginning to pile up, and I was stuck watching my family and friends live their
lives through a window. Trapped inside a personal horror movie, where all of my fears
swallowed me whole.
As I write this today, in 2021, I am sitting in my college dorm during my Freshman year.
I am at a place now that I never thought I would live to see. It is clear to me now that I am
stronger than I ever gave myself credit for. I wish I could go back and tell my younger self that it is going to be okay. That the waves of the deathless ocean are only momentary. That you clawed tooth and nail against the sides of the seemingly eternal abyss. That I love you and I know it’s rough, but it will get better. One day, you will be a high school graduate and be friends with some of the most incredible people. You have so many wonderful mentors and an even more amazing support system. You’ll get accepted into 8 colleges and pick the one that fits you perfectly. Now, you have dreams and aspirations for your future. Now, you are not completely pain free, but soon you will know that you are tough enough to handle it. I am more than proud of you and what you have accomplished so far. Please always be kind to yourself, and know that you will grow to be an exceptional person.
This is a piece of what has fashioned me into who I am. I hope that this can help lessen
someone's pain, even if it only decreases from a 10 to a 9.5. Baby steps are still steps
nonetheless.
CLINICAL SPOTLIGHT (cont.)
Submitted by an 11 year old White female diagnosed with Ehlers Danlos Syndrome and a mitochondrial disorder
Meeting with the therapist has helped me in so many ways. I found ways to be happier by finding what I enjoy. She told me to be less on technology, except for school work of course and digital drawing (see one of my favorite drawings). Then the therapist had me make a list of things that I like to do. My list of things that I enjoy kept growing and growing. Finding things that I like to do made me very happy. Then, when I have sad times and something upsets me or I have pain, I look at my list of things that I enjoy, such as drawing, reading, painting, playing with my dogs or talking to a friend and that helps me cope with the challenges that I am dealing with.
CLINICAL SPOTLIGHT (cont.)
Submitted by an 18 year old Black female diagnosed with AMPS
My pain journey started in the 2nd grade. I noticed when I sat down for too long the back of my knees would start to hurt. I brought this to my mom that I was feeling this pain. I then had to go to the rheumatologist to find out what was wrong. The doctor told me that I had AMPS (Amplified Musculoskeletal Pain Syndrome). I was told to do physical therapy and to see a psychologist. That is when everything changed for me. People started treating me differently because of my pain. They would always talk about how I never went to school. Peers thought I was lucky for not being there. I was bullied by my peers, which made me not want to go to school which is sad because I loved school up until that point. I went to group therapy with a bunch of older girls, they didn’t really understand me or where I was coming from being in elementary school, so I just said whatever to fit in. Physical therapy didn’t work for me nor did group therapy, so I started to get really depressed. Being sad because I was being bullied and always in pain or sick, I started to lose a lot of myself. I had no friends and my mom was struggling too. It was hard being in pain all the time.
Around 5th grade, I started going to therapy. I didn’t know how to express myself, so it was hard in the beginning. At first, I only talked about stuff that wasn’t important or relevant. I used to think I said too much, that my therapist didn’t like me, or thought I was annoying, but she still worked with me. I was held back in 6th grade. Seeing everyone my age moving forward was hard for me. Middle school became harder. I did eventually make friends and even became an honor student. I was still very lonely and just got out of a toxic household. I missed many days in middle school and got all the same comments about pain from teachers and peers. I was going to therapy once a week and only started to go to school to make my therapist proud. I had many breakdowns because I just couldn’t understand why I had to feel like this and the fact that I couldn’t do anything about it was heartbreaking. I just wanted to stop feeling the pain.
Fast forward to the day before my 15th birthday, I was so scared and so tired. I was turning into a nervous wreck, I had no idea how I would handle more stress and more anxiety. As I got into high school, it was hard because I wanted to break down the walls that I put up through middle school, but I didn’t know how. I made many friends and got two best friends in the mix. In my freshmen year, I started on a good note, work was in on time, and I was paying attention. However, my mother got really sick, she was in and out of hospital a lot. My mom was not only my ride to school, but she is my rock. I could get to school, and I was so concerned about my mom and her heart.
On my 16th birthday, my mom was in the hospital and needed to have surgery. She still made sure that I had a good birthday even though she couldn’t be there. That summer I had to do summer school, that was very upsetting to me, but I had decided that I was going to work hard and not worry about pain. In 10th grade, I tried ignoring pain and depression, which only made me very anger when I would mess up and by mid year my mom told me that I had to go to court for truancy. The judge said that if I get good grades and good attendance, she will drop the case. I started to become really mean toward my friends because I was so hurt. My grades were the best they ever were that year, the case was dropped, and I finished the year with a 3.5 GPA. In 11th grade, I had to do virtual schooling because of COVID-19, it was terrible. I was very confused on what I was supposed to do. I had a hard time communicating with my teachers. My grades dropped, but most of all I lost myself again. The pain and anxiety started to come back worse than before because I was stressed and thought I was going crazy. I feared failing and I feared growing up. I felt isolated and lonely. I was trying to force myself into a breakthrough and all that would happen was a breakdown. I realized that I just had to go through with it, have my breakdowns, be upset, and it was okay.
Over the summer I started believing in myself again. Finding that being 18 was a lot of fun and the freedom is nice. Now in 12th grade, everything that I have learned about myself and pain helps me get through tough times. My therapist said “sometimes you have to pursue happiness” and I try to do that every day.
CLINICAL SPOTLIGHT (cont.)
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