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378 O. C. Nin et al.
Postoperative Care
In the postanesthesia care unit (PACU), the patient should be evaluated for anxiety and
pain. If an epidural was placed and the effect of the spinal anesthetic has dissipated
adequately, a test dose with epinephrine containing local anesthetic can be performed
in order to rule out intrathecal and intravascular location of the epidural catheter prior
to initiating the infusion. Further pain management actions can proceed as noted in
previous sections.
Continuation of the patient’s home medication regimen should proceed as toler-
ated and allowable. Psychiatric medication can be very important in the process of
recovery from surgery and should not be taken lightly. Mental health support, phys-
ical therapy,and a positive environment provided by staff are all integral to an optimal
outcome. Also, a private hospital bed is ideal for patient privacy and comfort.
A very important take-home message regarding SRS is that these gender-affirming
surgeries should be considered to be medically-necessary surgeries that can provide
potentially life-saving mental health benefits for our transgender patients [17].
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Sickle Cell Disease (SCD)
and Perioperative Pain Management
Cynthia Iradukunda
Abstract Sickle cell disease is an autosomal recessive disorder that involves the
inheritance of a mutant Beta globin gene. The Beta globin gene is encoded at chro-
mosome 11. The defective hemoglobin S (HbS) results from a substitution mutation
that occurs at the sixth amino acid where Glutamine is substituted for Valine on
chromosome 11 (Kumar et al, Robbins basic pathology, Elsevier, Saint Louis, pp
411–3, 2012 [1]; Lanzkron, Sickle cell anemia straight to the point of care [Internet],
2023 [2]). At least one HbS allele must be present to develop sickle cell disease. In
cases where only one HbS allele exists, co-inheritance of defective Beta hemoglobin
such as occurs in the setting of Beta thalassemia or Hemoglobin C disease may
result in clinical manifestations of sickle cell disease. Inheritance of a hemoglobin
S allele from both parents results in the most severe form of sickle cell disease
also known as sickle cell anemia (Lanzkron, Sickle cell anemia straight to the point
of care [Internet] 2023, [2]). Patients with sickle cell trait have one normal gene
(HbA) and another sickle gene and are commonly asymptomatic or have symptoms
of diminished magnitude.
Keywords Sickle cell disease (SCD)
· Postoperative surgical pain · Acute painful
crisis
· Chronic pain · And chronic opioid therapy
1 Epidemiology
The Global Prevalence of SCD increased by 41.4%. from 5.46 million people in 2000
to 7.74 million in 2021 [3]. The ancestry of patients with sickle cell disease can be
traced back to sub-Saharan Africa, the Caribbean, India, Saudi Arabia, and Mediter-
ranean countries [3, 4]. In the United States of America, approximately 100,000
people suffer from sickle cell disease, and the condition is most prevalent among
African Americans. The incidence of sickle cell disease is 1 for every 365 live births
C. Iradukunda (
B
)
Department of Anesthesiology, School of Medicine and Public Health, University of Wisconsin,
Madison, USA
e-mail: ciradukunda@uwhealth.org
© 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_24
385
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386 C. Iradukunda
in the African-American population and 1 in every 16,300 Hispanic-American births
[5]. Recent advances in medical therapy have made it possible for patients with sickle
cell disease to survive into adulthood [5–7]. However, given the pathogenesis of sickle
cell disease, there is an increased incidence of surgery in this population compared
to the general population [1, 8, 9]. In an earlier cooperative study of surgery and
sickle cell disease, 69% of sickle cell patients underwent a single procedure, 21%
underwent two procedures and 11% had more than 2 procedures over a10 year study
period [9]. Additionally, patients with SCD who underwent surgery were reported to
have more complications, increased pain scores, and increased opioid requirements
[10–12].
2 Pathogenesis of Sickle Cell Disease
Understanding the pathogenesis of sickle cell disease and sickle cell anemia plays
a critical role in optimizing the surgical care of patients with SCD. Normal adult
hemoglobin (HbA) is a tetramer with 2 alpha and 2 beta chains encoded at chromo-
some 16 and chromosome 11 respectively [1]. A substitution mutation of glutamine
for valine at chromosome 11 results in a mutated beta Hemoglobin S (HbS). Sickle
cell disease follows an inheritance of at least one abnormal beta hemoglobin chain
(HbS) from one parent, with varying degrees of severity as dictated by the patient’s
genotype. With hemoglobin electrophoresis, the most severe forms of sickle cell
disease that is HbSS and HbSbeta
0
are found to have more than 80% of the Beta
hemoglobin mutant. [13]. Patients with sickle cell anemia (HbSS) can present with
severe features as early as 6 months and are bound to endure multiple hospital
admissions and blood transfusions [2].
Underlying the unique clinical presentations of sickle cell disease is the process of
polymerization of HbS within the red cells in the presence of hypoxia which results
in morphological changes of the red blood cell from a normal discoid to a sickle
or crescent shape. The rate of HbS polymerization is directly proportional to its
concentration within the cell. Once the cells are sickled, they increasingly adhere to
endothelium promoting thrombosis, decreased blood flow and a shortened life span
of 20 days versus 120 days of life for the normal red cell [1] When sickling occurs in
the microvasculature, partial or complete obstruction of blood flow occurs inducing
an acute or subacute vaso-occlusive painful crisis, in the affected organs. In organs
with increased transit time such as the spleen and bone marrow, the sickled cells
are subjected to increased hemolysis resulting in anemia [1, 14]. Tissue and organ
infarction usually results from multiple vaso-occlusive episodes following different
triggers. Reversing hypoxemia with improved oxygenation has been shown to atten-
uate further sickling and occasionally reverse sickling. In addition to hypoxemia,
dehydration, acidosis, cold temperatures, extreme exertion, inflammation, stress, and
infection are the other commonly identified triggers of red blood cell sickling [2].
The two major outcomes of sickled red blood cells are [1]:
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Sickle Cell Disease (SCD) and Perioperative Pain Management 387
1. Recurrent hemolysis resulting in chronic hemolytic anemia.
2. Microvascular obstructions result in thrombosis, vascular congestion, and infarc-
tion of multiple organs resulting in sudden vaso-occlusive pain crises including
acute chest syndrome, a life-threatening complication.
3 Sickle Cell Disease and Surgery
Beginning in the preoperative period, an Anesthesia professional should work closely
with a multidisciplinary team including the patient’s primary care physician, Hema-
tologist, and the acute pain service [15–17]. Patients with SCD may require surgical
interventions to provide symptomatic relief or to definitively treat the sequalae of
infarction. Surgical encounters in SCD start early on in childhood and are generally
associated with longer hospital stays secondary to poorly controlled postoperative
pain among other factors [8, 18, 19]. Cholecystectomy, splenectomy, and adenoton-
sillectomy are reported as the most common procedures, however, other procedures
may include hip surgery for avascular necrosis of femur, incision and drainage for
abscess, surgery for priapism, cardiac surgery, neurosurgery for stroke, arteriovenous
malformations, and MoyaMoya disease [9, 20–23].
4 Perioperative Pain Management in Patients with Sickle
Cell Disease
Patients with sickle cell disease are most vulnerable in the perioperative period due
to the accentuation of multiple triggers for sickling including dehydration, stress,
surgery, hypoxia, and increased inflammation following surgical manipulation [16].
Prolonged hours of fasting predispose patients to dehydration, which in turn increases
anxiety and stress resulting in a vicious cycle of multiple triggers for polymer-
ization of the defective haemoglobin (HbS) and red cell sickling. Consequentially,
vaso-occlusive acute painful crises often occur perioperatively and complicate the
management of postoperative surgical pain.
Postoperative pain in patients with SCD remains complex and systematic
approaches to analgesia should incorporate the patient and a diverse group of medical
professionals.
5 Hematology Consult
Once the patient is scheduled for surgery, the patient should be seen or reviewed
by an in-house hematologist to establish and ensure continuity of care. A patient’s
hemoglobinopathy should be ascertained since some forms of SCD are more severe
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388 C. Iradukunda
than others [13]. Ascertaining the genotype of patients from endemic areas of the
world [5, 24] is essential in guiding individualized postoperative analgesia plans and
appropriating resources as determined by the disease severity [13, 25, 26] Addi-
tionally, the expertise of the Hematologist is also crucial to guide the timely initia-
tion of sickle disease-modifying therapies such as hydroxyurea, crizanlizumub, L-
glutamine and voxelotor [7].
6 Individualized Pain Plan
Optimizing postoperative pain management in patients with sickle cell disease
requires that the provider be familiar with the complex manifestations of the disease
in situations of increased stress that may be encountered in the perioperative period.
Goal setting is pivotal in managing postoperative pain in SCD patients with a focus
on return to baseline functional status which may mean complete pain resolution for
some versus baseline chronic pain state in others.
Postoperative pain management in SCD patients begins in the preoperative period
with a focused pain history and an assessment of analgesic elements (Table 1) perti-
nent to SCD patients followed by the drafting of an individualized pain management
plan [27, 28]. In general, a multimodal analgesic approach to postoperative pain
is suitable for most surgical patients, however, SCD patients present several chal-
lenges and may require additional resources. Distinctive in patients with SCD are
several factors that impact and compound the presentation of postoperative pain in
the perioperative period as outlined in the following paragraphs.
7 Early Recognition and Management of an Acute Painful
Crisis
A vaso-occlusive painful crisis in the perioperative period is often multifactorial in
etiology [2, 12]. In the postoperative period, an acute painful crisis often presents as
a new severe acute pain and may be localized to a non-surgical site often with precip-
itating factors that should be identified and treated promptly [29, 30]. Maintaining
adequate hydration status by allowing clear fluid intake up to 2 h before surgery and
careful intravenous fluid maintenance for those who are strict NPO status is advised
[31]. Throughout the perioperative period, vigilant monitoring of a patient’s input
and output should be performed and this can provide valuable information about the
patient’s hydration status [29]. To prevent hypoxia, supplemental oxygen may be
required during the intraoperative and postoperative periods to maintain an oxygen
saturation > 95%. Incentive spirometry is encouraged in patients with SCD under-
going surgery to mitigate pulmonary complications and the use of CPAP in patients
with Obstructive Sleep Apnea may be necessary [23, 26].
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