Nidhi Bhatt, MD
Hemophilia is often associated with psychological and psychiatric comorbidities. According to findings from the HERO study, almost half of patients have been affected by these conditions, but only 22% received treatment for them. In addition, many parents of young patients report that they would have liked to receive psychological support but were never offered services. To learn more, Healio spoke with Nidhi Bhatt, MD, pediatric hematology instructor at St. Jude’s Children’s Research Hospital, about the psychological effects of hemophilia and how health care providers can alleviate the burden for young patients and their parents.
When parents first learn that their child has hemophilia, there is an emotional shock and a fear of having a child with a bleeding disorder. Many hemophilia cases are hereditary, so parents might have seen the hardships that family members with hemophilia have gone through. This might actually increase their anxiety level. On the other end of the spectrum, for children with de novo mutations who have no family history of hemophilia, their diagnosis is an even bigger shock for parents. There is a fear of the unknown.
For children, this is a congenital disease that they grow up with. Parents go through progressions. When their baby or toddler with hemophilia has a minor fall or injury, it is a bigger deal for them compared with parents of a healthy child. Then there are concerns when their children start school and want to participate in sports, and when they transition into teenagers.
There are parents in our practice who say they do not see hemophilia as a burden. Their children receive infusions two or three times a week and then go about their life. In contrast, there are patients who say hemophilia is a significant burden. A lot of this depends on what resources are available to them. Treatments are expensive, so parents worry about the financial burden and insurance. For those who live in a developed country, such as the United States, resources are largely available, except for in very rural areas. However, in developing countries, these resources are less accessible.
Up until now, we were treating patients with factors that were given intravenously. There are issues with getting intravenous access or having a port; however, I am an optimist, and I think the paradigm shift in hemophilia care will change the outlook of the disease, especially for severe hemophilia. If we can give emerging treatments — such as those with extended half-lives, monoclonal antibodies and inhibitors — subcutaneously once or twice a week, that will significantly reduce the burden for caregivers.
Children are almost always diagnosed at birth or soon after birth, so this is something they grow up with. By the time children are aware of their diagnosis, the parents are already quite involved in the hemophilia community.
When we do have to break the news to children, the earlier they know about their disease, the earlier they will accept it and try to gain as much knowledge as possible, which will help them manage their disease. Obviously, we want to make sure that we are using familiar terms with our younger patients as well as visual aids and support from child life specialists to help break the diagnosis. We should use all the resources that are available to us and encourage our patients to ask questions to make sure they understand their disease, what they can expect, and what the treatment entails. Children are very intuitive and should be involved in all of the conversations. You would think that they would be sitting in the corner playing, but they are listening.
We do a pretty good job of educating our patients and parents in the hemophilia community. In the past, there was a misconception that hemophilia can cause HIV or AIDs. That is just not true. We have not had an HIV infection since the 1980s. At that time, patients were at risk for being treated with contaminated blood products or transfusions, which may have caused the infection. But that risk is long gone at this point.
There are some smaller misconceptions among parents. They are afraid to let their kids be kids. Sometimes, parents will restrict their child’s exercise or physical activity, even flossing. But in reality, gum health is very important, and exercise is imperative because being obese or overweight can actually precipitate joint problems.
There is a lot of ongoing research focused on transiting pediatric patients with chronic diseases into adult care. Hemophilia has its own variants and problems. A big issue is assessing the readiness of the adolescent. Do they know how to manage their disease? Are they capable of making their own appointments and ordering prescriptions? Can they advocate for themselves? Many patients have had the same pediatric hematology provider their whole life, and now we are telling them they have to switch to an adult hemophilia provider. The unfamiliarity alone may prevent patients from having a smooth transition.
Another issue is, are we the providers ready to let go of our patients? We should not wait until patients are in their adolescent ages to start talking about transition. It is such a vulnerable age. They are trying to juggle so much at that time like going to college and having relationships outside of the family and friends they have had in their neighborhood or school. They are trying to establish who they are and then we add even more stress by having them transition to an adult doctor or an adult hemophilia treatment center (HTC). Pediatric providers and adult providers need to be cognizant of that and there has to be close communication between the HTCs.
The HERO study is a very important study. I have to admit that I think we should do a better job of assessing patients who may need psychotherapy or counseling to help cope with their disease. There are support services available. Local HTCs are usually equipped with a social worker or psychologist who spends a good amount of time with patients during their comprehensive clinic visits. They may also coordinate with patients’ schools to make sure teachers understand their disease and that the school environment is safe for these patients.
There are a lot of other resources that are available online, like hemaware.org. The website has a section called HemAware Junior, which has videos on various topics, including how to break the hemophilia diagnosis to siblings. The National Hemophilia Foundation holds an annual meeting as well as a smaller subgroup where parents and their patients can meet other patients and parents. There are also hemophilia walks, which have a good community feel. A lot of HTCs will coordinate hemophilia camps at these once a year. This allows patients of different ages to meet with their peers. They can learn from each other and know that there are other people out there who are going through the same thing. Several pharmaceutical companies are also very active in the hemophilia community. So, I think there is quite a bit of social support available for hemophilia patients, which is very much needed.
Forsyth AL, et al. Haemophilia. 2013;doi:doi.org/10.1111/hae.12239.
Disclosures: Bhatt reports no relevant financial disclosures.
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