Event 2: Meet Tylee as a Child

Tylee's Health History


Sickle Cell Disease (SCD-SS)

Pain History

From 6 months to 5 years of age:

  • 1 hospitalization
  • 3-5 pain crises a year

From 5 years to 1- years of age:

  • 1-2 pain crises a year
  • Episodes are managed at home


  • For low back pain

Medications and Treatments

  • Received IV morphine sulfate via patient-controlled analgesia (PCA) while in hospital
  • Discharged home on oxycodone and oral ibuprofen as needed
  • Physical therapy training while in hospital to help with range of motion (ROM) and adaptive exercise
  • Learned some additional age appropriate coping skills with the help of child life specialist - guided imagery

Tylee's Sickle Cell Disease (SCD) Treatment as an Adolescent


Tylee's mother advocates:

  • For his teacher and school nurse to know about his Sickle Cell Disease (SCD)
  • If he complains of feeling sick the school may need to call his mom
  • If he is sleepy at school because of anemia, the teacher needs to know he may need to rest at the nurse's office. It it important for Tylee to learn self-care management and how his SCD affects him
  • If it is very cold out, he may need to skip going outside for recess

School Care with Sickle Cell Disease (SCD)


  • Joint pain in arms and legs
  • Chest pain
  • Back pain
  • Abdominal pain (rule out other causes of abdominal pain, e.g., gallstones, splenic sequestration
  • Fever
  • Priapism

Tylee's Pain Assessment

Tylee's Sickle Cell Disease (SCD) Related Pain

  • Eyes
    • Glassy eyed, yellow tinged sclera, dark circles under his eyes
  • Body Temperature
    • Low grade temperature 38.4 C
  • Position
    • When not interacting with health care staff, patient observed lying on side with his eye closed
  • Abdomen
    • Abdominal pain (new)
  • Skin
    • Skin dry to touch
  • Back
    • Lower back Sickle Cell Disease (SCD) pain (typical)
  • Legs
    • Thigh Sickle Cell Disease (SCD) pain (typical)

Pain Diary

Keeping a written record of all aspects of pain management and providing this documentation to clinicians at the point of care is valuable.

Home Pain Diary Description

Provides the health care team with a daily, weekly or monthly (depending how often entries are made) account of the patient's status, use of treatments and response to treatments.

Should include:

  • date/time/level of pain
  • behaviors of pain along with descriptors  and effect of the pain on activity
  • interventions used with dates and times
  • medication taken: amount and type
  • fluids and food consumed
  • use of heat
  • use of relaxation or distraction techniques
  • hours of sleep

Tylee's Pain Diary Example (Kept By His Mother)


Woke up in pain this morning (legs - 5, stomach - 7). Out of oxycodone so I gave him 15 ml of ibuprofen at 7am. I called the school nurse, if needed she will provide 15 ml of ibuprofen at 1pm (will let his history teacher know).

Pain got worse on the way to school. Came home and is resting on sofa w/aromatherapy pillow for heat. Lit candles for him. He is calm. Took another 15 ml of ibuprofen at 1pm. (Legs - 4, stomach - 6). Only eating soup and some crackers. Has had 10 glasses of water. 15 ml of ibuprofen at 7pm. (Legs - 4, stomach - 6).

Took hot shower around 8:30pm and went to bed with aromatherapy pillow still on stomach. 15 ml of ibuprofen at 1am (legs - 5, stomach - 8), reheated pillow in microwave.


Woke up in more pain (legs - 5, stomach - 8), 15 ml of ibuprofen, said he wanted to go to the hospital. I told him we can manage and I will stay home today to keep him distracted. And keep his pillow hot. He said distraction is not going to work this time!

11am reporting pain in stomach is a 9! Out of control stabbing in his stomach. His legs are sore and achy (6).

Tylee's Mom Describes His Pain Diary

Tylee's Oucher Scale

Tylee's Oucher Scale is at a 9 out of a maximum 10, with 10 being the most painful.

Since sickle cell patients have frequent pain, they may be used to continuing to function despite what for others might be severe pain. Caregivers may interpret this appearance of functioning as incompatible with the patient's self-assessment of pain. 

It is important to respect the patient's pain self-report.

Tylee's Self-Care Management

Tylee uses self-care management to prevent, eliminate or reduce the pain he experiences from his sickle cell disease (SCD).

Pain Management

  • Focuses on preventing, eliminating, and/or reducing painful sensations through pharmacological and non-pharmacological methods
  • Is accomplished through interprofessional collaboration and team-based care that is patient and family-centered


Tylee says he uses distraction like watching TV, playing video games, reading books and listening to music to help manage his pain.

Play Therapy

Tylee says play therapy helps him act out characters, share experiences, and discuss his fears of pain and his disease. He likes that this is done with someone he can trust because it helps answer some of his questions and change his perception of pain.

Warm Baths

Tylee says warm baths or warm compresses to the specific areas that are causing him pain and soothing touch like massage, being held, and cuddling help decrease his pain.

Asking for Help

Tylee says asking for help with the pain, using positive self talk statement and learning how to cope with his pain has helped him feel more in control.


Tylee says staying hydrated by increasing his fluid intake through water, soups, gelatin, water ice, and sherbet helps.

Guided Imagery

Tylee says guided imagery helps him focus his mind on peaceful images rather than his current pain. This helps work all of his senses and reduces his anxiety around the pain.

Pediatric Patients with SCD Use of Complementary and Alternative Therapies

  • 42% prayer
  • 28% lifestyle/mind-body therapies (including relaxation, exercise, diet and/or visual imagery)
  • 12% biochemical (including herbs, mega vitamins, and folk remedies)
  • 5% massage
  • 54% overall (n=31) used complementary and alternative therapies (57 parents of children with Sickle Cell Disease - SCD - were interviewed by phone reporting their child's use of Complementary and Alternative Therapies)

Sibina EM, Shindell DL, Casella FJ, Duggan AK. Wilson MH., 2006

Tylee's Treatment

  • Analgesics titrate to pain relief
    • NSAID: oral ibuprofen, intravenous ketorlac
    • Opioids
  • Hydration
  • Avoid infection (immunizations)
  • Heat
  • Massage

*Observe for side effect

Treatments for Sickle Cell Disease (SCD) in Children

Analgesic Medications for Mild to Moderate Pain

  • Ibuprofen and acetaminophen
    • Do not exceed daily recommended dose
    • Avoid giving too much acetaminophen, which is used in multiple Over the Counter (OTC) medications such as cold medications
  • Acetaminophen with codeine phosphate
    • Not recommended (FDA black box warning about codeine in children). Learn more here.

FDA restricts use of prescription codeine pain and cough medicines and tramadol pain medicines in children; recommends against use in breastfeeding women. Available at https://www.fda.gov/media/104268/download

Severe Pain Managed in the Hospital

  • Morphine sulfate or hydromorphone
    • Around the clock (ATC) bolus administration with intermittent IV administration
    • Patient-controlled analgesia (PCA) for children age 6 and older
  • Ketorolac - IV (ATC administration)
    • Renal function must be normal; platelet function and number must be normal
    • Limit administration to 3 days

Puri L, Nottage KA, Hankins JS, Anghelescu DL: State of the Art Management of Acute Vao-occlusive Pain in Sickle Cell Disease. Paediatr Drugs 20 1:29-42, 2018

Pediatric Multimodal Analgesia

  • Maximizes pain control while limiting side effects
  • NSAIDs are effective for the treatment of mild or moderate pain in children, and can be opioid sparing
  • The combination of NSAIs and acetaminophen produces better analgesia than either drug alone
  • NSAIDs-bases pain management is beneficial due to the inflammatory nature of sickle cell pain

For additional information on the pharmacological management of both acute and chronic pain in pediatric populations, click here for guidelines.

Multimodal Strategy: Combining Classes of Agents

  • Foundation of multimodal analgesia is the combination of 2 or more classes of analgesics
  • Generally involves adding a second class of drug to an opioid
    • NSAIDs oral: ibuprofen (COX-1 and COX-3 inhibition, endogenous cannabinoid modulation)
    • NSAIDs IV: ketorolac (COX-1 and COX-2 inhibition)

Isakson P, Hubbard R., 2004; Hinz B, Cheremina O Brune K., 2008; Hogestatt ED, et al., 2008

Test Your Knowledge

Question 1

Chose the best pharmacologic approach to effectively treat severe Sickle Cell Disease (SCD) related pain.


Question 2

Which of the following statement is true of non-pharmacologic strategies, such as distraction or massage, for pain relief?


Question 3

Tylee is your patient; what is the first step you would take to relieve Tylee's pain?


Tylee's Discharge

Healthcare providers decided to discharge Tylee home because:

Discharge Home Criteria

  • Pain relief continues for a minimum of 90 minutes following oral analgesia
  • Pain is relieved after 1 or 2 doses of IV analgesia
  • Relief is maintained on oral analgesic therapy
  • Absence of other complications of sickle cell disease

Reasons to Admit

Short Stay Unit Admission Criteria

  • Greater than or equal to 6 months of age
  • Non-toxic (i.e., appears well)
  • Uncomplicated vaso-occulusive pain event requiring multiple IV doses of analgesics

Inpatient Admission Criteria

  • Greater than 6 months of age
  • Focal neurologic findings
  • Acute chest syndrome
  • Splenic sequestration
  • Fever
  • Reticulocyte count less than 1% (unless Hgb greater than 10g/dl)
  • Severe anemia (Hgb less than 5 g/dl)
  • Pain unable to be controlled after repeated doses of IV analgesia

Ellison A, et al., ED Pathway for Evaluation/Treatment of Children with Sickle Cell Disease and Pain. Available at: http://www.chop.edu/pathways/emergency-department/sickle-cell-disease-pain/

Tylee's Discharge Plan

  • A follow-up appointment is arranged with the hematologist in 3-5 days unless symptoms worsen
  • Tylee's mother is asked to fill the oxycodone prescription in the hospital pharmacy due to limited access at her pharmacy

Next: Meet Tylee as a Young Adult

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