A Comprehensive Care Plan for Pediatric Patients With Vaso-Occlusive Crises

Sickle cell disease (SCD) disease is inherited as an autosomal recessive trait that results in several interactive processes: polymerization of hemoglobin S (HbS), hemolysis, nitric oxide depletion, and vaso-occlusion.[3] The most common manifestations of sickle cell disease are vaso-occlusive pain events, specifically dactylitis which typically occurs as the first clinical manifestation.[4] As patients age, the early onset peripheral pain moves to more central regions resulting in increased severity of vaso-occlusive crises.[4] Pain from the blockage of sickeled cells in vessels causes poor blood flow to a localized area. There are few clinical and laboratory findings to indicate the severity of pain associated with sickle cell crisis,[4] making it difficult for a physician to identify the amount of pain the patient actually experiences. Length of stay in the hospital depends largely on physician discretion and patient report.

Sleep is instrumental to many aspects of physical and emotional health during childhood and adolescence. Sleep disruption from pain is a significant concern in patients with sickle cell disease due to the prevalence of their acute and chronic pain.[7] In a study conducted by Daniel et al., sickle cell patients between 4-10 years old, were more likely to exhibit disrupted sleep, specifically night-waking and sleep-disordered breathing, than children who were not diagnosed with sickle cell disease from similar demographic backgrounds. [7] Furthermore, children with more severe forms of sickle cell disease were reported to have more restless sleep than children with less severe forms. [7] Overall, the literature suggests that sleep disruptions may interfere with children's daily functions, along with their ability to cope with SCD and its complications.[7] Daniel et al. recommends discussing ways to improve sleep habits with children suffering from SCD because it may increase efficacy in pain management, priapism, and enuresis.[7] Another study by Valrie etal., suggests that poor quality of sleep is correlated with increased occurrence of sickle cell disease pain the following day.[9] They also discovered that high pain severity was related to poor sleep quality and this relationship was exacerbated during stressful situations.[9] Developing a suitable sleeping environment (e.g. removing the television, or limiting the lighting) will maximize sleep efficacy resulting in better health outcomes and quality of life.[7] There has been minimal research on evaluating the relationship between exercise and sickle cell disease in children and adolescents. Studies conducted on sickle cell disease in adults with respect to the effects of exercise have produced mixed results partly due to patient comorbid illnesses. In 2012, Chirico et al. compared two fitness level group including, subjects who did not partake in regular physical activity for two years prior to the study versus subjects who partook in regular, weekly physical activity for several years leading up to the study. [5] Both groups contained sickle cell trait carriers and subjects with normal hemoglobin. [5] Subjects performed an incremental maximum exercise test while data was collected on heart rate via a chest monitor and on oxidative stress levels via blood samples at various points following completion of the exercise task. [5] Results indicated regular physical activity helped to blunt the increase in oxidative stress and the decrease in nitric oxide metabolism and up regulation of antioxidant enzyme activities in response to exercise in sickle cell trait carriers. [5] These findings suggest an attenuated sickle cell crisis in the subjects with regular exercise when compared with the sedentary group.

Presently, at Phoenix Children's Hospital, no protocol exists for management of sleep and exercise in sickle cell patients. The most relevant policy named "Parenting and Visitation" includes guidelines regarding sleep and visiting hours but these are not well enforced. In our hospital, the sickle cell population is known to have some of the longest length of stays. Between October 2014 and September 2015, there were 89 admissions for a vaso-occlusive crisis with an average length of stay of 6 days and 12 admissions greater than 10 days and 5 admissions greater than 20 days. Prior research suggests that implementation of standardized care (e.g. order sets, protocols, and flowcharts) can improve compliance and patient outcomes [8], reduce overall length of stay, and mitigate costs. Therefore, we have developed a new comprehensive care plan (CPP) that enhances standard of care by including the following care aspects:

1. Patient Controlled Analgesia (PCA). PCA will be initiated and managed at the discretion of the Pain Management team. Naloxone infusion will be available for opioid related side effects. Once tapered, patients will be transitioned to oral opioids to be administered as needed. Patients will be discharged home, when appropriate, with oral opioids and weaning instructions.
2. Other Medications. All medications will be given during daytime hours, if possible. All home medications will be continued while inpatient. Ketorolac will be given intravenously every 6 hours for 12 doses then switched to ibuprofen by mouth every 6 hours until discharge home. Stool softeners will be utilized while on narcotics to prevent constipation. A clonidine patch may be used for adjunct analgesia and anxiolysis. A lidocaine patch may be used for localized pain.
3. Pain Psychology. Consultations will begin on Day 2 and continue as needed. The psychologist will teach the patient sleep hygiene techniques, and non-pharmacologic pain management skills including: distraction, diaphragmatic breathing, progressive muscle relaxation, and guided imagery with medical hypnosis.
4. Physical Therapy. Consultations will begin on Day 2 and continue as needed. Patient is expected to spend time out of bed by Day 2 participating in light activity.
5. Sleep Hygiene. Will be introduced to the experimental group. Patient must sleep between the hours of 10 PM and 5 AM daily. Window blinds will be open by 8 AM. Patient must be awake and ready to participate in therapies by 9 AM. Napping will be at the discretion of the physician. Practitioners should examine care orders critically, such as routine vital signs, and determine if it is safe and/or appropriate to defer these during sleep. Room lights will be turned off to help promote the circadian rhythm. No video games, television, cell phones, or computers will be allowed during this time.
6. Extracurricular. Will participate in child life activities as recommended (e.g. Playroom, or Zone). TV, phone, computer, and video games only between hours of 4-9 PM (computer okay outside of hours only for school work).
7. Other. Patient will eat meals during regular daytime hours (e.g. breakfast consumed before 9 am) and shower daily. No overnight visitors will be allowed.

We propose to evaluate the feasibility of the new CPP in a pilot randomized control trial to determine if pain and length of stay can be reduced in patients with sickle cell disease. We also propose to evaluate a sleep regimen to determine if this can reduce the hospital stay and help with pain. We hypothesize increased physical activity and proper sleep, as implemented in the CPP, are correlated with decreased hospital length of stay and decreased pain. Additionally, we believe that creating a standardized nighttime environment at the hospital will help the children stay in their circadian rhythm thus promoting improved sleep and a more effective inpatient disease management.