Suephy Chen, MD, MS, Principal Investigator
Emory University, Atlanta, GA

There is a significant economic burden of cutaneous disease felt by ichthyosis patients and families. This economic burden negatively impacts the quality of life (QOL) experienced by this population. This study aims to evaluate the economic impact of cutaneous disease in ichthyosis by performing a rigorous cost analysis from both societal and individual perspectives, assessing how individual cost of ichthyosis care relates to individual QOL outcome measurements, and comparing the overall economic impact and QOL of this population to other patient populations. Little is known about the overall economic impact of cutaneous disease to ichthyosis patients and families. This study proposes that the lifelong hyperkeratosis, primarily manifested as pruritus, xerosis, and scaly skin, that affects patients with ichthyosis, carries a significant economic burden.

Despite the advances over the last 40 years in therapies for ichthyosis, many of these current remedies are not only costly but also remain extremely time intensive, as patients and their caretakers spend a significant amount of time per day caring for their skin. Accordingly, these medication costs and time spent translate to a significant economic burden which potentially negatively impacts the QOL experienced by this population. Information about the economic impact of disease is important to clarify as the allocation of both research and medical care resources may be influenced by such data.

UPDATE APRIL 2009
To date, an interim analysis of data from a nationwide online survey of CI patients has led to significant findings.
There were 230 subjects with CI at the time of this analysis, of which:
64% were adults (e18 years old),
40% were male
81% were Caucasian
62% were married
43% had a family history of ichthyosis.
 
Classification by CI type was:
 12% x-linked
 29% lamellar
12% epidermolytic hyperkeratosis
  4% Netherton syndrome
43% other.  

Data analyses were done to determine which factors most affected the amount of time patients spent treating their symptoms of ichthyosis. Factors evaluated as possible predictors of time spent on treatment included type of ichthyosis, degree of skin redness, degree of skin scale, whether the patient was a child or adult, gender, race, income level and patient quality of life. Of these factors, the only ones found to significantly affect time spent on treatment were degree of skin redness and quality of life. All factors were then analyzed to determine which ones predicted the number of dermatology visits that subjects went to each year. Having a lower quality of life and being an adult were predictive of an increased number of dermatology visits per year. Increased severity of skin scale and redness as well as ichthyosis type, non-white status, being a child, and having to spend more time treating symptoms of ichthyosis daily were factors which negatively impacted patient quality of life. Our preliminary findings suggest that more effective treatments for the congenital ichthyoses would help minimize disease burden on quality of life and help reduce disease-associated costs. These findings have been accepted for presentation at the Society for Investigative Dermatology's annual meeting in May 2009. Nationwide survey collection has now been completed and final data analyses have begun. In this analysis, we will revisit factors that determine patient quality of life, time spent on treatment and number of dermatology visits per year. We will also perform an in-depth cost analysis to determine cost of treatment to patients and to society for the care of CI. Generating awareness for the significant economic and quality of life burden associated with the CI will hopefully serve to increase funding for research aimed at finding more effective treatments for this disease. 

UPDATE OCTOBER 2010
Disease severity was determined using the previously validated severity scale developed by Chen et al. The severity scale examines a patient’s level of redness, scaling, and hair loss. Resource utilization was determined by the amount of time subjects spent daily treating symptoms of CI (e.g. applying emollients & other topical treatments) as well as the number of ichthyosis-related dermatology visits patients attended each year.

Of 235 study subjects:
60% were female
84% were Caucasian
42% had a family history of CI
 the mean age was 29 years.
 
Analysis showed that increased scaling, redness, age and time spent daily treating symptoms of CI were predictive of worsened quality of life. Younger patients with increased scaling and hair loss attended more annual dermatology visits, as did those patients with no family history of ichthyosis.  Younger patients with increased redness who reported worsened quality of life spent more time daily treating their symptoms.

Taken together, these findings suggest that increased disease severity has a significant negative impact upon quality of life in patients with CI. Furthermore, increased disease severity leads to greater resource utilization, whereas increased age and a positive family history of CI lead to decreased resource utilization. These findings imply that better therapies and greater patient education may lead to improved quality of life and decrease resource utilization among patients with ichthyosis.

Highlights from the Report of the Working Group on Core Measures of the Burden of Skin Diseases

Journal of Investigative Dermatology (2007) 127, 2701–2706. d