28
The economic and psychosocial impact of HAE is difficult
to quantify based on the variability in patient phenotype.
Presumably, those who have more frequent or more
severe attacks may experience greater detriment to their
quality of life and greater costs in terms of absenteeism,
hospitalizations, and other medical treatment.
5
In a study conducted in the United States, Wilson et al
estimated the total annual per-patient cost at $42,000
for the average HAE patient, ranging from a total cost
at $14,000 for patients with mild attacks to $96,000 for
patients with severe attacks.
63
The humanistic burden was assessed by Lumry et al via a
web-based survey.
64
They reported that pain, discomfort,
disfigurement, and the life-threatening potential of acute
attacks resulted in significant reduction of (health related)
quality of life and increased incidence of depressive
symptoms.
64
As research in the field of HAE progresses, several inter-
national consensus documents and guidelines regarding
management of the disease have been published or
updated recently. The International Consensus Algorithm
for the Diagnosis, Therapy, and Management of HAE was
published in 2010.
11
Evidence-based recommendations for
the therapeutic management of HAE were formulated by
an international working group of experts in 2011.
67
The
Moreover, lack of recognition among clinicians and,
consequently, inaccurate or delayed diagnosis can lead to
undue and/or prolonged suffering for patients, lengthy
or frequent hospitalizations, and unwarranted medical
procedures.
5,7
Understandably, patients may experience
frustration and/or depressionwhen physicians fail to provide
a satisfactory explanation for their symptoms.
Bowie et al reported that patients with HAE who suffered
exclusively from abdominal attacks without cutaneous
angioedema reportedly had unnecessary laproscopies,
appendectomies, and other GI surgeries before receiving
an accurate diagnosis.
65
Zingale et al reported that 27%of patients in the PREHAEAT
registry had 1 or more relatives who died during an
HAE attack.
33
As might be expected, those who have
lost relatives to HAE may experience heightened anxiety
regarding their own fate.
5
By contrast, several researchers
have noted the psychological benefit and added reassurance
of home possession of C1-INH therapy for patients and
their families.
5,17,66
International Home Therapy Consensus Document was
published in 2010.
68
Additionally, consensus documents for
the management of HAE in pediatric patients and during
pregnancy and breast-feeding were published in 2012.
69,70
In 2012, the World Allergy Organization (WAO) HAE
International Alliance presented the first global guideline
for the management of HAE.
71
The WAO guideline results
from a review of earlier consensus documents and thorough
assessment of the quality of evidence.
3.3 Psychosocial and Economic Impact of
C1-INH Deficiency
3.4 International Consensus Guidelines
for the Management of HAE
