Page 2265 - Hematology_ Basic Principles and Practice ( PDFDrive )

2012 Part XII Hemostasis and Thrombosis

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associated with the birthing process, particularly with a traumatic and behavioral and emotional problems in school-aged children.
vaginal delivery. ICH is also seen in young children with severe There may be multifactorial causes for this, including potentially
hemophilia, particularly in those who are not on prophylaxis or have silent ICH. These children may grow up with resentment toward
an inhibitor. In older adults, ICH again becomes more common, their peers and nonhemophilic siblings and they may harbor sup-
particularly in patients with coexisting HIV or hepatitis C. In such pressed anger about having the condition. Hemophilia can also
patients, mild to moderate thrombocytopenia is frequently seen impact vocational choices and opportunities for adults with hemo-
either as a result of portal hypertension–induced splenomegaly or philia with established arthropathy. In addition to all of the psycho-
from HIV-associated immune thrombocytopenia. The combination logic sequelae of hemophilia, unfortunate men with hemophilia who
of thrombocytopenia together with hemophilia increases the risk of acquired HIV and hepatitis C also have to deal with the myriad
central nervous system bleeding. ICH is rare in patients with mild or emotional and social ramifications of these devastating infectious
moderate hemophilia. illnesses.
ICH may be subdural, epidural, subarachnoid, or intracerebral. The child’s mother may experience guilt knowing that as a carrier
Intraspinal (usually epidural) bleeding is rare, but when it occurs, can she passed the hemophilic mutation to her son. This can contribute
cause spinal cord compression. Most cases of ICH occur after trauma, to marriage breakdown, although it has not been ascertained whether
but the trauma may be quite trivial, such that the ICH appears to separation occurs more frequently in marriages of parents of children
occur spontaneously. Patients with ICH usually present with neuro- with hemophilia than in parents of children without the condition.
logic symptoms soon after trauma. Typical neurologic symptoms Clearly, persons with hemophilia and their families require significant
include drowsiness, loss of consciousness, seizures, headache, and education, counseling, and support throughout the individual’s
vomiting. Any of these symptoms should raise the suspicion of ICH. lifetime.
Thus, any person with hemophilia who has neurologic symptoms or For society in general, hemophilia with or without HIV or hepa-
signs should immediately be given a bolus of an appropriate factor titis C has a substantial impact, mainly because of the high cost of
concentrate to raise the factor level to about 100%. Replacement care.
should always be given before any diagnostic procedure (e.g., com- The key aspects of hemophilia management are preventive therapy,
puted tomography or MRI) is performed to confirm the diagnosis of treatment of bleeds, and care of the complications of hemophilic
ICH. If an ICH is present, in addition to appropriate factor replace- bleeding.
ment, patients should be hospitalized for 10 to 14 days during which
time they should be maintained on factor concentrate (by continuous
infusion or frequent bolus infusions) to maintain a factor level over Preventive Therapy
100% at all times.
Lumbar punctures and neurosurgical interventions can safely be Preventing trauma does much to reduce bleeding frequency in
performed in persons with hemophilia provided that 100% factor persons with hemophilia. One of the strategies involved in bleed
correction is achieved. prevention is restricting the child from high-risk sports (e.g., ice
hockey, American-style football, rugby, and martial arts). A number
of other sports (e.g., soccer, basketball, baseball, and tennis) also
Surgery and Bleeding entail a certain amount of risk but for the child’s psychologic well-
being are usually permitted. Other sports considered to be relatively
With the exception of circumcision in neonates, excessive bleeding is safe include road cycling (with a protective helmet), running, and
almost inevitable in patients with severe or moderate hemophilia who swimming. Ideally, persons with hemophilia should be encouraged
undergo surgery without adequate replacement therapy with appro- to keep active throughout their lives because osteoporosis and obesity
priate hemostatic agents. The bleeding can be acute or can be delayed are growing problems in this population as a result of inactivity. All
for several hours or days. In addition to bleeding, surgery in such patients with severe and moderately severe hemophilia should be told
patients is characterized by poor wound healing as a result of poor to wear helmets for bicycle riding, rollerblading, skateboarding, and
clot formation along with prolonged bleeding and subsequent infec- downhill skiing. There is controversy regarding the use of helmets
tion at the wound site. For surgical management of hemophilia see (outside of sports) in very young children as they are learning to walk
later section in this chapter. and are unsteady on their feet and prone to falling. Some clinics
advocate that patients between the ages of 1 and 2.5 years with severe
forms of hemophilia wear a helmet at all times, with the exception
CLINICAL MANAGEMENT OF HEMOPHILIA of when they are sleeping. Whether this policy reduces the incidence
of ICH is not known. Advising families on the importance of good
As a lifelong, expensive, and disabling illness, the management of dental hygiene is important because good oral hygiene may prevent
patients with hemophilia is complex and ideally best undertaken or reduce the need for dental procedures (extractions, root canals),
through comprehensive care in hemophilia treatment centers with which entail a risk of bleeding. Unfortunately, dental care, including
multidisciplinary teams of health professionals. These teams should regular cleaning by a dentist, is often not subsidized by society and
include a physician (usually a hematologist) with specialist training as such may be an expensive undertaking for families.
in hemophilia, a dedicated nurse, a physical therapist, and a social
worker. Additional members that should be available for consultation
include an orthopedic surgeon, a dentist, a genetic counselor, an Vaccinations
obstetrician/gynecologist, a psychologist, a rheumatologist, a urolo-
gist, and (increasingly, for the management of elderly persons with Children with hemophilia have normal immune systems and should
hemophilia) a cardiologist. Fortunately, the role of the orthopedic receive all routine vaccines. In addition, they should receive hepatitis
surgeon in the management of hemophilia has been reduced in B vaccinations at a young age. Recombinant factor concentrates and
countries where prophylactic therapy can be provided. currently available plasma-derived factor concentrates do not transmit
Over the course of a lifetime, a patient and his family will experi- hepatitis B. However, patients with hemophilia are at a higher risk
ence numerous issues related to hemophilia. Significant psychologic of requiring blood transfusions because of trauma-associated blood
sequelae ensue for both affected children and their families. These loss, and there is still a small risk of acquiring hepatitis B from blood
children may be overprotected, and this may result in their experienc- transfusions. It is not clear whether patients with hemophilia should
ing difficulties in adjusting to the school environment and to society receive the hepatitis A vaccination. However, the US National
in general as they age. Hemophilia impacts children’s school experi- Hemophilia Foundation’s Medical and Scientific Advisory Council
ence and what sports and recreational activities they are allowed to has recommended that all persons with hemophilia who are seronega-
undertake. Some studies have shown lower academic performance tive for hepatitis A should be immunized against hepatitis A.