Overweight and obesity are associated with adverse health outcomes such as heart disease, diabetes, some cancers, hypertension, stroke, liver disease, gallbladder disease, sleep apnea, respiratory problems, and osteoarthritis [1]. Mental health outcomes may include stigmatization, discrimination, and lowered self-esteem. Overweight is identified by a body mass index (BMI) between 25 and <30 kg/m² for adults, and ≥ 85^th to < 95^th age- and sex-specific percentiles for children (aged 2–19 years); obesity is identified by a BMI of ≥30 for adults and ≥95^th percentile for children [1, 2] In 2009–2010, 35.7% of U.S. adults were obese; 16.9% of U.S. children and adolescents were obese [3]. Recent estimates of the annual medical costs of obesity are as high as $147 billion [4]. On average, persons who are obese have annual medical costs that are $1,429 more than for those with a normal weight [4].

In contrast to current understanding about obesity-related health risks for the general population, the study of overweight and obesity-related health risks faced by individuals with bleeding disorders is in its infancy. Persons with hemophilia are living longer and therefore are at risk for developing chronic conditions that may be further complicated by overweight/obesity [5, 6, 7, 8]. Emerging evidence makes the importance of obesity prevention clear. Obesity has become a key public health problem for the hemophilia population [7].

Analysis of data from CDC’s Universal Data Collection System (UDC) collected between May 1998 and May 2002 revealed that the prevalence of overweight and obesity among children and youth with hemophilia in the US was similar to that among the general population [9]. Interestingly, in a 2011 Canadian study of boys under 18 years of age with hemophilia, the prevalence of obesity was increased for those with severe hemophilia compared to those with mild or moderate hemophilia [8]. Furthermore, when compared to Canadian population data, hemophilic boys who were 2–5 years old tended to have a higher prevalence of overweight and obesity compared to the age-matched Canadian population. In contrast, the prevalence of overweight/obesity was similar among older hemophilic boys and the age-matched general population.

Soucie, et al. [9], studied the relationship between joint range of motion and age for a national sample of U.S. children and adolescents with hemophilia, aged 2–19 years old using multivariate analysis of UDC data. Higher body mass index (BMI) was associated with decreased joint range-of motion (ROM) regardless of the severity of hemophilia [9]. Additionally, joint ROM became more limited as age and BMI increased. In a subsequent study, Monahan, et al.[7], carried out multivariate analysis of UDC data to identify predictors of physical functioning in boys with hemophilia, aged ≤ 18 years. Obesity was associated with increased risk for inactivity and limited mobility as indicated by use of mobility aids including crutches/walkers and wheel chairs.

Financial costs related to factor replacement can be great. Factor products have been shown to account for the majority of annual costs for hemophilia care. Johnson et al. summarized studies from 1998–2007 and showed that clotting factor costs account for 45%–93% of the total health care cost for hemophilia depending on severity and treatment regimen [10]. Guh et al. showed that the annual mean expenditures for health care during 2008 for Medicaid-enrolled children with hemophilia were $113,867 and $142,987 for adults with hemophilia [11]. Further complicating the cost is the fact that, product dosage is currently based on body weight as opposed to ideal body weight. As a result, factor usage and cost are increased by overweight and obesity. For instance, analysis of a large U.S. health care medical claims database from 2001 to 2007 revealed that the median hemophilia-related cost per kg over 4 years for non-inhibitor patients, ranged from $837 to $1043 [12].

Although overweight and obesity are health problems faced by many people with hemophilia, little has been documented about the knowledge of providers or current practices of hemophilia treatment programs related to weight management and obesity prevention. To address this gap, the CDC Healthy Weight Working Group conducted a survey of federally funded Hemophilia Treatment Centers (HTC) to identify routine clinical practices of BMI assessment, perceptions about the issues of overweight and obesity, and the role of HTCs in assisting patients with bleeding disorders to attain and maintain a healthy weight.

Ninety of the130 HTCs contacted responded to this survey and provided feedback about staff involvement, weight monitoring practices and level of concern and intervention for patient overweight and obesity. For most centers, survey responses were provided by a nurse coordinator, nurse practitioner or research nurse (n=75). At other centers, responses were provided by a clinical, program, or research coordinator (n=9), dietitian (n=4), or by a social worker (n=1) or health educator (n=1). Eighty seven (n=78) percent of centers reported that patients served by their HTC included children, and 83% (n=75) reported that patients served included adults. Ninety-nine percent of HTCs indicated that they routinely documented measured weights and heights in patient charts. Sixty seven percent of centers reported that they calculated BMI for patients at clinic visits. BMI was most often calculated automatically by an electronic medical record (37%) or using a web-based computer program (33%). Almost half of the centers (48%) indicated that they reported BMI results to the patient. Among centers that provided care to adults, 36% used the standard cutoff values for obesity and for overweight; 44% reported using other or subjective definitions, and 19% did not know the definitions used by their center. For children, 32% used the standard cutoff values for defining both obesity and overweight, while 41%, reported using subjective definitions to classify obesity for children, and 26% did not know the definitions used by their center.

Most centers identified obesity as a “big concern” (38%) or “moderate concern” (49%); few centers viewed obesity as a “small concern” (11%) or as “not a concern” (1%). Almost all respondents indicated that helping patients maintain a healthy weight was a shared responsibility that included the patient (99%), family (99%), HTCs (98%), primary care provider (97%) and schools (85%). Within the HTCs, all team members (hematologist, nurse coordinator, physical therapist, nutritionist and social worker) were responsible for talking with patients about healthy weight. Fifty-five percent of centers reported spending 0–5 minutes talking with patients of normal weight about the importance of maintaining a healthy weight; two thirds reported spending 5–15 minutes talking with patients who were overweight or obese about the importance of achieving and maintaining healthy weight.

While the majority of centers (64%) regularly provided consultation to patients about healthy weight maintenance at each comprehensive visit, 16% did so sporadically and 18% did not address weight at each comprehensive visit. When educational messages were provided to overweight patients, topics included health risks of being overweight or obese (33%), advice on how to increase physical activity (34%) and follow a healthier diet (21%), make lifestyle changes (15%), use portion control (10%), and decrease TV and computer screen time (3%). Seven percent of centers reported that no educational message was given. For patients who were overweight or obese, 51% of centers reported making referrals to nutritionists external to the HTC for services. In other centers patients were counseled by HTC staff, without other referral (36%). About one quarter of centers (26%) referred patients to a weight management program, 11% referred to a physical therapist, and 11% to the primary care provider for weight management. Only 2% of centers reported using all of these referral strategies, and 10% reported taking none of these actions. Only six centers (7%) had a written protocol or guidelines to address excess weight in their clinic population. Of centers without a written protocol, 53% indicated that a protocol was needed at their center while 13% indicated a protocol was not needed.

Open-ended questions were asked about barriers or benefits encountered in helping patients maintain a healthy weight. The most common topics reported as barriers to helping patients maintain a healthy weight included patient defensiveness, cultural differences, family misconceptions about obesity, distance to the HTCs, and financial barriers. The most common topics reported as benefits of maintaining a healthy weight included fewer joint bleeds, less pain, enjoyment from participating in sports, and increased confidence and self-esteem.

This is the first evaluation to assess how HTCs address weight in the hemophilia population. We found that obesity is reported to be a moderate to a major concern for many centers, consistent with concerns raised nationally. Unexpectedly, we found that while most HTCs routinely calculated patient BMI, they did not routinely use the available guideline [1] recommended to identify people that were overweight and obese. This suggests that clinicians may not only need updated information about current weight guidelines, but they also need training in order to better identify these problems. Furthermore, when BMI was calculated, results were not routinely reported to patients. Interestingly, evidence from a national sample of adults indicates that among overweight or obese patients, being told by a physician that they were overweight was associated with more realistic perceptions of their weight, desire to lose weight, and report of recent attempts to lose weight [16]. HTC providers have an important opportunity to educate hemophilia patients about their weight at HTC visits and apply evidence-based approaches to promote behavior changes needed to achieve healthy weight goals.

Few centers reported having a clinic protocol or guidelines to address overweight although most centers reported that they would like to have one. The need to maintain a healthy weight was viewed as a responsibility that should be shared among families, healthcare providers, including HTCs, and schools. Although not investigated by this survey, linkage of HTCs with additional community partners and resources may offer expanded education opportunities for HTC patients and staff to learn about healthy weight maintenance and evidence-based strategies for behavioral change. HTCs can also engage in promotion of policy and environmental changes that will help make dietary improvements and increasing activity feasible and rewarding [17]. The development of guidelines for HTCs to address overweight offers an opportunity to help clinicians systematically and appropriately evaluate patients’ BMIs and to take action to support patients’ achievement of a healthy weight.

Many HTCs reported patient/family defensiveness about discussion of healthy weight issues as an obstacle in addressing this topic. Guidelines and provider education materials that include practical communication tools would facilitate effective collaboration with patients towards lifestyle behavior changes and improved weight outcomes.

There were a few limitations to our investigation. Most notably, we were not able to interview staff from all the HTCs. We were able to contact only 90 (70%) of the 130 HTCs so findings may not be representative of the full range of treatment center practices in the U.S. However, our sample is geographically diverse, as it includes HTCs from each MCHB region across the nation which constitutes a reasonable representation. Next, since not all people with hemophilia seek care at HTCs there is some concern as to whether these findings could be generalized to the hemophilia population outside of the HTCs. Lastly, since the survey was administered by personnel from the CDC, there is a chance that some of the participants may have given answers deemed more favorable.

HTCs recognize that obesity is a risky and costly clinical and public health problem in hemophilia. Patients receiving care at HTCs are more likely to benefit if current NIH guidelines for assessment of BMI are applied consistently and if tools for healthy weight intervention strategies are available to HTC staff. The knowledge gained from this evaluation will be useful in provider training modules; these modules would help improve weight management practices and thus reduce the prevalence of overweight and obesity in people with hemophilia.