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Sickle Cell Disease (SCD) and Perioperative Pain Management 389
Table 1 Elements for postoperative pain assessmentin SCD
Element Questions
Triggers for acute pain crisis Do you often experience painful crises? What
triggers your painful crisis? (hypoxia, exercise,
infection, dehydration) How often do they
occur?
Frequency of ED visits and hospital admission How many times have you visited the ED due
to increased pain, uncontrolled pain or other
reasons?
Number of times admitted to the hospital in the
last (month, 6 months, 1 year)
Location of the chronic pain
Location of the any pain including the new pain
1. Where is the pain? Is it localized or
generalized
2. Is this pain new or different from the pain
you usually experience at home?
Intensity How severe is this pain? (use a validated pain
intensity)
Quality of pain Is the pain sharp or dull? Is it constant or
intermittent?
Aggravating and relieving factors What makes the pain better or worse?
Current pain regimen What do you take regularly to remain
comfortable? What do you take for
breakthrough pain?
a
Modified from Chou et al. [28]
Avoiding hypothermia throughout the perioperative period can be achieved with
the use of Bair huggers, warm blankets, warm intravenous fluids, and prewarming the
room for most of the general cases in conjunction with temperature monitoring [29].
In particular, the use of cold ice packs and other cold therapies should be avoided in
SCD patients.
Perioperative transfusion of red blood cells increases the proportion of HbA and
may alleviate the presentation of a painful crisis. However, this does not represent
routine clinical practice and implementation should be considered on a case-by-case
basis [12, 23, 29, 32]. Ballas et al. further demonstrated that the use of hydroxyurea
among adults experiencing an acute painful crisis resulted in lesser opioid require-
ments and shorter hospital stays [12]. This modality can be explored with critical
input from the Hematologist.
Parenteral opioids are commonly utilized in the severe forms of acute painful
crises, and in the setting of acute postoperative pain, this would translate into higher
opioid dosing requirements for extended periods. The clinician(s) involved in the
postoperative care of SCD patients should be acutely aware of these deviations
from the general population to avoid suboptimal pain relief from suboptimal opioid
prescription and administration.
Acute chest syndrome is a life-threatening complication [12, 33] and is reported
as the most common postoperative cause of morbidity and mortality in sickle cell
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390 C. Iradukunda
patients requiring ICU admission. It is defined as a new pulmonary infiltrate on
imaging and presents in children with fever, shortness of breath, chest pain, and
tachypnea [33]. Risk factors include sickle cell disease genotype, h/o pulmonary
disease, and recent surgery [33, 34]. Acute chest syndrome can be triggered by the
combination of surgery and other known sickling triggers as noted above. [33, 34].
In cases of acute chest syndrome, multiple interventions including early recogni-
tion, prompt hematology consultation coupled with timely institution of mechanical
ventilation and transfusion protocols are crucial in preventing fatal outcomes.
8 Management of Persistent Chronic Pain and Chronic
Opioid Use
Chronic pain in SCD is considered when painful symptoms are present for most
days in the previous 6 months or more [35]. With the high prevalence of chronic
pain in SCD adolescents and adults, of more than 50%, most SCD patients will
be opioid-tolerant and will require higher than anticipated opioid doses to control
postoperativepain [10, 11, 36]. American Pain Society guidelines on the management
of postoperative pain strongly recommend early consultation with pain specialists
for patients who are at high risk of sub-therapeutic management of postoperative
pain such as those on chronic opioid therapy [28, 37]. A multidisciplinary team with
a holistic approach to SCD pain presentations encompassing the different aspects of
chronic SCD pain compounded by new post-surgical pain is highly recommended
[35, 36].
9 Patient-Centered Treatment Plan
Opioids remain the mainstay of management for moderate to severe postoperative
pain. Patients with SCD generally require elevated postoperative opioid dosing and
it is crucial that frequent patient assessments are performed to establish responses to
therapy and monitor for opioid related adverse events [38]. Several guidelines can be
referenced to guide pain management in patients on chronic pain therapy. In partic-
ular, the American Society of Hematology 2020(ASH 2020) guidelines for sickle
cell disease [39] provide recommendations on acute and chronic pain management
in SCD patients. It is critical to bear in mind that following surgery, SCD patients
will have, in addition to their baseline pain(s), post-surgical pain depending on the
extent of the surgical procedure which may also be complicated by an acute painful
crisis.
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Sickle Cell Disease (SCD) and Perioperative Pain Management 391
10 Multimodal Analgesia Approach
The American Pain Society and the ASH 2020 guidelines on the management of
postoperative pain recommend a multimodal analgesic approach to provide optimal
pain relief. While opioids have been routinely prescribed in the immediate postsur-
gical period, the opioid epidemic has introduced opioid restriction measures that
may affect SCD patients negatively [12, 34]. It is therefore i mportant that healthcare
professionals find a way to utilize different pain relief modalities bearing in mind
the unique pain syndromes and patterns experienced by patients with SCD. [12,
37]. To maximize the efficacy of multimodal analgesic approaches, pain assessment
using age-appropriate pain-validated tools is recommended [38, 40]. In adults, visual
analog and numerical rating scales are commonly used [40, 41] (Table 2).
Ketamine
SCD patients maintained on chronic opioid therapy may experience refractory
pain with minimal responses to escalated opioid doses. Etiologies to blunted anal-
gesic responses following opioid administration may include central sensitization
resulting in allodynia, opioid-induced hyperalgesia, and neuropathic pain. Subanes-
thetic ketamine infusions have demonstrated an ability in patients with SCD to
decrease opioid utilization and improve pain control [42].
Table 2 Multimodal analgesia
a
Reported pain intensity Recommendedanalgesicmodalities
Mild pain Acetaminophenand NSAIDs- ibuprofen, ketorolac,
naproxen, diclofenac
Moderate Enteralopioids: codeine, oxycodone, morphine
sulphate
Severe pain and acute post-surgical pain Parenteralopioids
IV hydromorphone PCA, IVketamine infusion[42],
IV lidocaine [43],fentanyl(nasal, transdermal patches,
IV), regional and neuraxialanalgesia[44, 45]
Acute painful crisis Parenteralopioidsas above
Consider transfusion of red blood cells
Consider sickle cell modifying therapies such as
hydroxyurea [12]
Optimal volume status, oxygenation, and temperature
regulation (normothermia) [2, 29]
a
Tabl e 2 constructed from multiple data sources [2, 6, 12, 28, 29, 39, 42–45]
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392 C. Iradukunda
Regional and Peripheral Nerve Blocks
Regional blocks can be performed for anesthesia or analgesia in SCD and may
result in less opioid consumption in the immediate postoperative period [44, 45].
In male patients, priapism can develop and if unrecognized, complications of
penile infarction, and impotence. Mandatory monitoring in this patient population is
highly recommended to facilitate timely surgical decompression and other relevant
interventions.
Non-pharmacological Therapies
The American Society of Hematology for Sickle Cell Disease provides no recom-
mendation for or against the use of non-pharmacological therapies for acute pain
management in sickle cell disease and these include physical therapy, behavioral
therapies, and psychological therapies [39]
11 Compassionate Care
Too frequently, optimal pain management in patients with SCD may be hampered
by the prejudices of healthcare professionals responsible for their care [27]. These
patients may be labeled as difficult, addicted or drug seeking and may therefore suffer
needlessly as their description of pain symptomology is minimized [46]. To combat
these potential inherent biases, the development and implementation of specific clin-
ical guidelines may help to ensure that patients receive the essential components of
a tailored analgesic regimen. In addition, patients should be empowered as active
participants in their healthcare and their descriptions of pain symptomology should
be addressed with at least the same attention as any other patient. Finally,staff training
in mechanisms to collaborate care and analgesic pathways in patients with SCD may
effectively minimize challenges in access to appropriate analgesic care [47].
12 Conclusion
Perioperative pain management in patients with SCD remains a challenging endeavor
for healthcare professionals. To optimize pain relief in SCD, a systematic approach
that begins in the preoperative period with a focused pain history capturing the
baseline chronicity of sickle cell pain, frequency of acute painful crises, and current
pattern of opioid consumption together with a multidisciplinary team should be
adopted.
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Sickle Cell Disease (SCD) and Perioperative Pain Management 393
Healthcare professionals involved in the perioperative care of patients with SCD
should consider the pathogenesis of SCD as this is critical in early diagnosis, differ-
entiation, and management of postoperative surgical pain compounded by acute
painful crises, life-threatening acute chest syndrome, and chronic pain. In these
circumstances, we recommend frequent patient assessment to quantify the need for
escalating opioid dosing for extended periods to achieve the goal of returning to
baseline individual functional status.
Key Takeaway Points
1. Starting in the preoperative period, the Anesthesia professional should work
closely with other healthcare professionals and draft an individualized pain
management plan.
2. Recognize the several facets of pain in patients with Sickle cell disease including
the different pain patterns of chronic pain [6, 36], and the challenges associ-
ated with chronic or long-term opioid use to avoid the pitfall of subtherapeutic
perioperative pain management.
3. Actively prevent and treat the triggers for sickling coupled with timely diagnosis
and management of vaso-occlusive painful crises.
4. Return to baseline functional status is the expectant goal of perioperative pain
management in patients with SCD and this should be clearly communicated to
the patient and the involved healthcare professionals.
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Peripartum Pain Management
Jarna Shah, Kristin Swope, Sudheer Potru, and Sejal Tamakuwala
Abstract In 2021, the US had over 3.6 million deliveries, of which 32% were via
Cesarean delivery. Improved monitoring and anesthetic techniques have improved
the safety of peripartum care, enabling patients with more complex cardiopulmonary
conditions to become pregnant. Each of these factors contribute to an increasing
complexity of patients presenting to the labor and delivery ward each day. The accom-
panying fetal changes of pregnancy complicating these patients’ comorbidities makes
labor pain management inherently challenging. In this chapter, we present strategies
for managing peripartum pain in complex situations.
Keywords Pregnancy
· Opioid use disorder · Pain · Obstetric analgesia ·
Postpartum pain
J. Shah
Department of Anesthesiology, University of Arkansas for Medical Sciences, Little Rock, AR,
USA
e-mail: jshah2@uams.edu
K. Swope · S. Tamakuwala (
B
)
Department of Gynecology and Obstetrics, Emory University School of Medicine, Atlanta, GA,
USA
e-mail: sejal.tamakuwala@emory.edu
K. Swope
e-mail: kristin.i.swope@emory.edu
S. Potru
Complex Pain Clinic, Atlanta VA Healthcare System, East Point, GA, USA
Department of Anesthesiology, Emory University School of Medicine, Atlanta, GA, USA
S. Potru
e-mail: sudheer.potru@emory.edu
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2024
A. Abd-Elsayed and K. Schroeder (eds.), Perioperative Pain Management,
https://doi.org/10.1007/978-3-031-67648-2_25
397
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398 J. Shah et al.
1 Introduction
In 2021, the US had over 3.6 million deliveries, of which 32% were via Cesarean
delivery [1]. In concert, advancing reproductive technologies are allowing for preg-
nancy at more advanced ages while unplanned pregnancies and obesity rates in
pregnancy are rising rapidly. Improved monitoring and anesthetic techniques have
improved the safety of peripartum care, enabling patients with more complex
cardiopulmonary conditions to become pregnant. Each of these factors contribute
to an increasing complexity of patients presenting to the labor and delivery ward
each day. The presence of a fetus and accompanying fetal changes of pregnancy
complicating these patients’ comorbidities makes labor pain management inherently
challenging. In these situations, an anesthesiologist must be well-versed in avail-
able analgesic modalities to optimize pain control and prevent maternal morbidity
and mortality, the latter of which is at an all-time high in the United States [2].
In this chapter, we present strategies for managing peripartum pain in both routine
and complex situations.
At baseline, there is a significant female predominance in both episodic and
chronic pain syndromes among women of reproductive age, including chronic
abdominal and pelvic pain, migraines, fibromyalgia, and several other painful condi-
tions [3]. A study of 1.1 million women found that one in five pregnant patients
(21.6%) enrolled in Medicaid filled at least one opioid prescription during preg-
nancy, although there were pronounced regional and racial variations in dispensa-
tion patterns; the majority of these prescriptions were for hydrocodone or codeine
[4]. Common indications included abdominal pain, back pain, headaches, or joint
pain [4]. Several confounding pain factors during pregnancy and delivery include
premature labor pain, contractions, changes in ligamentous laxity, anxiety, stress,
depression, and physiologic and hormonal changes. Medication options during preg-
nancy are few, due to concern for teratogenic effects and limited safety data. Fluo-
roscopic injections for chronic pain are also contraindicated. These challenges all
make treating pain during pregnancy more complex and challenging. Implications
of poor acute pain management during labor include the concerns for opioid use and
opioid use disorder, development of chronic pain syndromes, and trauma associated
with the birthing experience.
2 Intrapartum Pain Management
During labor and delivery, pregnant patients experience significant pain, and it is
important to understand the various mechanisms through which pain is caused. Labor
is divided in three stages. The first stage consists of uterine cervical dilation caused
by rhythmic contractions which can be further categorized into passive (0 to 5 cm
dilation) and active stage (6 to 10 cm dilation). Contractions are generally irregular/
less frequent during the passive stage and regular/more frequent during the active
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Peripartum Pain Management 399
stage. The second and third stages of labor consist of expulsion of the fetus and
placenta respectively. Pain caused during the first stage of labor is visceral and a
result of uterine contractions compressing the myometrial blood vessels and causing
localized hypoxia. The C-fibers in the myometrial tissue transmit the nociceptive
stimuli to the ipsilateral dorsal roots (T10 to L1) during contractions. While in the
second stage of labor, pain results from somatic stimulus, caused by stretching of
pelvic, perineal, and vaginal tissue, and transmitted to the spinal roots f rom S2 to S4
[5, 6]. Pain is a subjective experience, and several physical and psychological factors
impact its severity and duration. Maternal age, parity, size of the fetus/position of
the fetus in relation to the maternal pelvis, maternal medical conditions are some
physical factors [6]. Fear, anxiety, apprehension, family member/birthing companion
presence are some of the psychological factors that influence pain during childbirth
[6].
Non-pharmacologic pain management options most frequently used include child-
birth education, emotional support, massage, aroma therapy, and audio analgesia.
Non-pharmacologic interventions requiring specialized training or equipment which
are not rigorously studied scientifically are acupuncture/acupressure, hypnosis,
TENS, biofeedback, hydrotherapy, and intradermal water injections [6].
Childbirth education is practiced worldwide and, over the years, several studies
have been conducted to understand its role in use of analgesia, labor duration, and
mode of delivery. The results of such studies are inconsistent and are of poor quality
[6]. One systematic review and meta-analysis noted that childbirth education ante-
natally can reduce maternal stress, improve self-efficacy, lower the cesarean birth
rate, and decrease the use of epidural anesthesia, but it is unclear what impact these
educational efforts have on maternal/fetal outcomes. Education resources are widely
different and these materials require standardization before conclusive statements
can be made about actual mental and physical effects [7].
Treating pain in obstetric patients is crucial for optimizing patient comfort and
satisfaction during labor. Pain management is generally achieved through the admin-
istration of neuraxial or non-neuraxial options including parenteral opioids and
nitrous oxide. Parenteral opioids that are commonly used include fentanyl, morphine,
nalbuphine, butorphanol, and remifentanil. There is no great evidence demonstrating
a substantial benefit of one opioid versus others with regard to side effect profiles such
as nausea, vomiting and drowsiness. However, a Cochrane review did conclude that
there was generally poor pain relief during labor, which could represent a potential
impetus for the delivery of some opioid analgesic [8]. Opioids cross the placenta and
drug elimination takes longer in newborns than adults. Patients should be counseled
that parenteral opioids are not often used near the time of the delivery due to these
effects.
Neuraxial anesthesia includes epidural and spinal techniques. The goal of
neuraxial techniques is to provide appropriate analgesia with minimal motor block
[9]. Epidural analgesia includes a mixture of medications, which typically includes
at least a local anesthetic and often an opioid as well. This medication is adminis-
tered in either a continuous or repeated fashion through a catheter which is placed
into the epidural space. Commonly used local anesthetics include bupivacaine and
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