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768 Part VI: The Erythrocyte Chapter 49: Disorders of Hemoglobin Structure: Sickle Cell Anemia and Related Abnormalities 769

Changes during the progression of
the painful crisis
Prodromal Initial Established Resolving
phase phase phase phase

Numbness Problems with Joint effusion Problems with
parasthesia er personnel signs of hospital personnel
aches anxiety, fear inflammation depression
RBC DI
Dense RBC Temperature Reticulo- Fibrinogen
ISC WBC count cytes Orosomucoid
10 RDW CRP LDH ESR
Categorical pain scale 8 (Steady state values) SAA CPK viscosity
Platelets
HDW
Plasma
6
RDW

RBC
4 Platelets HOW Dense Arbitrary values relativfe to steady state
RBC Hb ISC
DI
2
I II III IV
0 –2 –1 1 2 3 4 5 6 7 8 9 10
Crisis day Ballas 1995, 1992
Akinola et al, 1992
Beyer et al, 1999
Jacob et al, 2005

Figure 49–7. A typical profile of the events that develop during the evolution of a severe sickle cell painful crisis in an adult in the absence of overt
infection or other complications. Such events are usually treated in the hospital with an average stay of 9 to 11 days. Pain becomes most severe
by day 3 of the crisis and starts decreasing by day 6 or 7. The Roman numerals refer to the phase of the crisis: I indicates prodromal phase; II, initial
phase; III, established phase; and IV, resolving phase. Dots on the x-axis indicate the time when changes became apparent; and dots on the y-axis,
the relative value of change compared with the steady state indicated by the horizontal dashed line. Arrows indicate the time when certain clinical
signs and symptoms may become apparent. Values shown are those reported at least twice by different investigators; values that were anecdotal,
unconfirmed, or that were not reported to occur on a specific day of the crisis are not shown. CPK, creatinine phosphokinase; CRP, C-reactive protein;
ESR, erythrocyte sedimentation rate; HDW, hemoglobin distribution width; ISC, irreversibly sickled cells; LDH, lactate dehydrogenase; RBC DI, red cell
deformability index; RDW, red cell distribution width; SAA, serum amyloid A. (Reproduced with permission from SK Ballas, K Gupta, P Adams-Graves: Sickle
cell pain: A critical reappraisal. Blood 120(18):3647–3656, 2012.)

side effects have been raised. Prior use of opioid therapy should be but not atelectasis, chest pain, fever, tachypnea, wheezing, or cough,
125
151
taken into consideration when deciding initial opioid doses as patients and hypoxia (Fig. 49–8). However, respiratory findings on clinical
may be tolerant and require higher doses. Caution should be exercised examination in the absence of radiographic findings should trigger high
with nonsteroidal antiinflammatory drugs and acetaminophen if there suspicion for ACS and warrants close monitoring. ACS is the leading
is renal or hepatic dysfunction. Patients with acute pain are better man- cause of mortality in patients with SCD. Etiology varies depending
121
aged in a setting dedicated to sickle cell patients. A multidisciplinary on age, with viral and bacterial infections dominating in the pediatric
145
approach is needed for pain management, especially if chronic pain is age group and fat embolization resulting from marrow necrosis during
present. 146,147 Opioid side effects should be anticipated and managed. VOE dominating in adults. 152,153 Important pathogens include Chlamy-
Antidepressants, anticonvulsants, and clonidine can be used for neu- dia pneumoniae, Mycoplasma pneumoniae, Streptococcus pneumoniae,
ropathic pain. Occasionally, severe, unrelenting pain may require red Staphylococcus aureus, parvovirus B19, respiratory syncytial virus, and
cell transfusion to decrease sickle Hb below 30 percent in the blood. 148 influenza. Regardless of the triggering factor, the pathogenesis of ACS
There is a paucity of data regarding optimal management of pain involves increased intrapulmonary sickling, intrapulmonary inflamma-
in SCD. A randomized trial of optimizing patient controlled analgesia tion with increased microvascular permeability, and alveolar consolida-
149
strategy was closed because of poor accrual. A trial looking at NO tion. ACS can rapidly evolve with bilateral infiltrates and consolidation
inhalation for treatment of VOE did not show improvement in pain. 150 leading to acute respiratory failure requiring intubation and ventilatory
assistance.
Pulmonary Manifestations Independent risk factors for respiratory failure are age older than
9
Acute Chest Syndrome The acute chest syndrome (ACS) is a con- 20 years, platelet count less than 20 × 10 /L, multilobar lung involve-
152
stellation of signs and symptoms in patients with SCD that includes ment, and a history of cardiac disease. Thrombocytopenia is an inde-
a new infiltrate on chest radiograph defined by alveolar consolidation pendent predictor of neurologic complications during hospitalization

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