Page 1299 - Hall et al (2015) Principles of Critical Care-McGraw-Hill

906 PART 7: Hematologic and Oncologic Disorders

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TABLE 96-4 Cardiac Catheterization Data for Adults With Sickle Cell Disease a VASO-OCCLUSIVE PAIN CRISIS (VOC)
Parameter Without Pulmonary HTN With Pulmonary HTN VOC is perhaps the most characteristic manifestation of sickle cell disease.
These severe painful attacks, most frequently affecting the extremities
Pulmonary artery systolic (mm Hg) 30.3 ± 5.77 54.3 ± 12.26 and back, result from acutely impaired microvascular blood flow. Most
Pulmonary artery diastolic (mm Hg) 11.7 ± 4.86 25.2 ± 7.72 commonly affected are bones, due primarily to ischemia and infarction
Pulmonary artery mean (mm Hg) 17.8 ± 4.86 36.0 ± 7.78 of the bone marrow and inflammation of the periosteum, with intense
bone pain due to the resulting edema and tissue swelling in the inelas-
Cardiac output (L/min) 8.62 ± 3.03 8.60 ± 1.76 tic compartment of the bone marrow medulla. Most often, pain affects
Pulmonary capillary wedge 10.6 ± 3.82 16.0 ± 5.87 the lumbosacral vertebrae, iliac and the long bones, although any bone
pressure (mm Hg) may be affected. Joint effusions of the elbows and knees are common. At
Pulmonary vascular resistance 57 162 times, especially in children, there is difficulty in distinguishing painful
(dynes·s/cm ) 5 crisis from osteomyelitis, another complication of sickle cell disease. The
mainstays of treatment for pain crisis are analgesics, in particular nonste-
a Ranges are mean ± standard deviation.
roidal anti-inflammatory drugs and opioids. Correction of dehydration
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Data from Castro et al and Anthi et al. 58 or hypoxemia is important, as is careful monitoring for development of
acute chest syndrome (ACS; see below). Clinicians frequently undertreat
the severe pain of sickle cell crisis. Opioid tolerance due to recurrent or
chronic opioid treatment may dramatically increase the dosage required
in individual patients. There is little, if any, risk for developing opioid
dependency with short courses of large-dose opioids for VOC.
■ STROKE

Ischemic stroke is a frequent complication of sickle cell disease, occur-
ring at any age, but mainly in children. This topic is discussed in detail
below in the section on special problems in the ICU.
■ EYE DISEASE

A proliferative retinopathy develops in 10% of patients with SS disease
and at least twice as often in patients with SC disease. This chronic
complication may be treated with laser photocoagulation. Two acute
complications present ophthalmologic emergencies. The first is vision
loss due to acute occlusion of the central retinal artery. This may resolve
with emergency exchange transfusion before retinal infarction occurs.
The second emergency is traumatic hyphema in a patient with sickle
cell disease or sickle trait. The environment of the anterior chamber of
the eye promotes sickling, and this can readily cause acute glaucoma.
Evacuation of the blood should be undertaken for hyphema without
delay to avoid vision loss. 14
■ LUNG DISEASE

Sickle cell disease is associated with a high incidence of acute and
chronic lung complications. The ACS is an acute lung injury syndrome
and, in cases of extensive lung involvement, is similar to ARDS. It
is defined by a new pulmonary infiltrate secondary to alveolar con-
solidation, not atelectasis, in a patient with sickle cell disease often
accompanied by chest pain, fever, tachypnea, wheezing, or cough. It is
caused frequently by pulmonary fat embolism syndrome from infarcted,
necrotic bone marrow or by an exuberant pulmonary inflammatory
response to common respiratory pathogens. Its pathogenesis and treat-
ment are discussed in detail in the section on special ICU problems.
FIGURE 96-3. Sickle cell disease injury to soft tissues. A. Lung cavitation resulting from Chronic complications can include the development of restrictive lung
previous acute chest syndrome, causing infarction and necrosis of a subsegmental region of disease, pulmonary fibrosis, or pulmonary hypertension; with the latter
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the left middle lobe. The cavitary lesion (arrow) has an inflammatory wall that subsequently occurring in approximately 10% of adults with sickle cell disease.
resolved after antimicrobial therapy. B. The calcified, atrophic spleen (arrow) is visible on an Pulmonary hypertension appears to be the most serious risk factor for
abdominal scan from the same patient. This is typical in adults with homozygous sickle cell early mortality in adult patients with sickle cell disease and, in the past,
disease. The stomach is visualized by oral contrast material. may have been underrecognized by clinicians. Reactive airway disease
or asthma occurs with increased frequency in children with sickle cell
disease. Both pulmonary hypertension with right heart failure and
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COMMON CLINICAL PROBLEMS reactive airways disease commonly complicate the acute chest syndrome
and lead to a more severe clinical course. 16
Common and significant clinical problems in patients with sickle cell
disease are summarized in Figure 96-1. Virtually every organ system ■
is subject to acute ischemia-reperfusion injury and infarction during HEART DISEASE
vaso-occlusive events. Many organ systems are subject to chronic dys- Although vaso-occlusion of the coronary microcirculation can pro-
function, particularly in the adult aging sickle cell disease population. duce myocardial infarction, acute wall motion abnormalities, and

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