Being Part of the Team
Some of the most enjoyable times in my life were the months I spent playing softball in a summer league in Galena Park, Texas. The smell of my worn leather glove, the dirt from a great slide into home, and the team that played together with one goal in mind: to win the trophy at the end of the season. It took each of us to make our team function. Not just one person, but all of us together.
When living with an inhibitor, not only do you need a team to help determine the best treatment possible, but you also need to become a part of the team. Your firsthand knowledge of living with an inhibitor 24/7 is paramount in finding the right treatment, because you are the expert on your own story. When clinicians and families work as a team to determine the best approach to care, attention is given to the uniqueness of the inhibitor, and to understanding the patient and social environment. Then, the best comprehensive plan for treatment can be achieved.
The hemophilia treatment center (HTC) model provides a comprehensive approach to care that handles various aspects of a patient’s overall well-being. About 70% of people with hemophilia in the US receive multidisciplinary, comprehensive care in a network of federally funded HTCs.1 The HTC team consists of nurses, social workers, physical therapists, and other healthcare providers who specialize in treating people with bleeding disorders. This team not only manages the day-to-day care of an individual, but works to prevent and reduce complications.
Sometimes inhibitors challenge even the providers’ expertise and threaten the team approach. My youngest son, Caeleb, has had a tough journey living with an inhibitor. He endured bleeds that seemed never to heal, and the joint damage to his knee and ankle were significant. Treatment with bypassing products proved difficult, and he developed an allergic reaction to infused factor VIII, in addition to his persistent high-titer inhibitor.
At the same time, our HTC was in a transitional stage with staff changes. I felt that we needed a meeting to discuss Caeleb’s treatment. So my husband and I met with the interim lead hematologist and asked if he thought we needed help from an HTC in a neighboring state. The wave of relief that washed over him was obvious, and we began a new chapter in Caeleb’s inhibitor journey. We began a new relationship with the HTC in Colorado. It wasn’t easy at first, because we were inviting a new member to join our team—to join our family.
With regular blood draws and often daily infusions, life with an inhibitor can be demanding. “When inhibitors occur, the families of the hemophilia patient are challenged by uncertainty, unanticipated complications, costs, and difficulties that few people understand,” says Dr. Stuart Winter, professor and vice chair of pediatric research, University of New Mexico Health Sciences Center. Not only was my family dealing with the enormity of the bleeds and pain that persisted for Caeleb, but we also had to plan for regular trips out of state for treatment. At one point, we were referred to a special allergist in Colorado; this required us to stay in Denver for a week, and put a financial and emotional strain on our family. But the result was worth it.
An inhibitor heightens the stress endured by many living with hemophilia without this added complication. Dr. Shirley Abraham, director of the Ted R. Montoya Hemophilia Treatment Center in Albuquerque, New Mexico, states that “inhibitor families deal with several unusual and severe bleeds which require specific management. They deal with emotional issues such as the anxiety of having the inhibitor and not knowing what will work for them and for how long.” My
husband and I had to figure out how to get both of our HTCs to communicate. They agreed to work together, but in the middle of a crisis, reminding one HTC to contact
the other wasn’t always easy. We had to advocate for Caeleb, and this became uncomfortable. The truth is that when your child is suffering, you do whatever you can to improve the situation. Sometimes you must be the squeaky wheel to get what your family needs.
Developing a relationship deeper than simply patient and provider is common with inhibitor patients. “The [inhibitor] families are seen and contacted much more frequently than most with their annual [HTC] visit,” says Claudia Mackaron, RN, nurse coordinator for the Ted R. Montoya HTC. “This makes the inhibitor family more a part of the HTC members’ lives.” When Caeleb started spending less time in the hospital, we missed our HTC team. The constant contact we’d had with our HTC brought the staff into our family life outside of a crisis.
The comfort level varies between a patient and HTC team, and this can be challenging. At times, I disagreed with a clinician. I questioned the care my son was receiving, and despite my comfort level with the team, I had to speak up. I realized that, like family, the team members weren’t easily offended, because we were all working toward the goal of giving Caeleb the best treatment. Frequent communication that is not necessarily for routine checkups often occurs as both patient and medical team work together to handle a crisis. A bleed that seems to never resolve, a product not being as effective as in the past, and issues regarding insurance and reimbursement problems are concerns that plague families with inhibitors.
Keeping an open line of communication with the HTC team is critical for optimal care and when a patient is in distress. “Sometimes anxiety and frustration can become a road block to proper communication,” says Abraham. “The psychosocial support from the HTC will be crucial to help. Social workers and a psychologist or psychiatrist can guide families through different financial and emotional struggles.” Trust can be established when both the HTC and the patient are actively communicating, and the best outcomes have a better chance to evolve.
When patients and providers voice their concerns about treatment, this increases the chance of obtaining the best results. “Good communication is paramount to make sure that care is delivered appropriately,” says Janet Ratte, RNC, specialty nurse pediatric hematology, Ted R. Montoya HTC. When in doubt, I always call my HTC. When the HTC asks personal questions, the patient may feel threatened; but if a good relationship has been established, new findings could help change the course of treatment, often for the patient’s benefit. And when patients question the HTC, staff are pushed to think outside the box to find new approaches and ideas. As long as everyone communicates, the best comprehensive plan may be achieved.
Living with an inhibitor can be devastating. But when the patient, family, and HTC team form a strong unit, moving forward with the patient’s best interests in mind, this eases some of the difficulties. “Feelings of sadness and despair impact the social fabric of the family,” says Winter. To handle the emotional challenges that inhibitors bring, allowing the HTC team to become part of the extended family can bring a huge sense of relief. One doctor finally stood back, assessed Caeleb’s situation, and told us that we needed a “roadmap” in place for his care. Once done, that was the final piece that both of our HTC teams needed, including my husband and myself. We could now see the big picture, communicate more effectively, and implement a team plan of treatment for a better life.
Cazandra Campos-MacDonald is a motivational speaker, educator, and patient advocate for families with bleeding disorders. She writes a blog chronicling the journey of her two sons with severe hemophilia and inhibitors, and has written articles and blog posts for various publications. Cazandra’s older brother, Ronaldo Julian Campos, died of complications from hemophilia as an infant. Cazandra lives with her family, Rev. Joe MacDonald, Julian (21), and Caeleb (11), in Rio Rancho, New Mexico.