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Behind The Online Pictures, There's The Trauma Of Hemophilia

I signed up for a Facebook account in 2008. Social media was foreign to me then, but I tried it and was excited to find old friends from high school and college. Catching up on one another's lives was enjoyable, and seeing my friends' children resembling their parents was fascinating.

Yet I had no idea how this platform would become a significant part of my journey to understanding the challenges of living with a bleeding disorder.

I searched through my family pictures to find the best ones to post. I've always found it easy to put myself out to the world, and in the early days of Facebook, sharing what I called the "bright and shiny" images of family was the norm. These depicted moments of joy, laughter, and celebration and were a way to show the blessings of our family and friends. These choices of images made life seem perfect.

However, there's something to understand as we show off the best of ourselves: Exotic trips, families in coordinating colors, and announcements of college acceptance and graduation don't necessarily reveal the whole truth. Grief and sadness are also in my "bright and shiny" pictures.

Six of the author's Facebook images, depicting her and her family (with dogs Hildie and Laggie). (Courtesy of Cazandra Campos-MacDonald)

I remember the dreams I had for my sons. Their rare genetic bleeding disorder was never part of the plan. Behind our smiles lie uncomfortable facets of life; trauma and grief are woven into the fabric of my family's experience.

My youngest son, Caeleb, has endured trauma at the hands of hemophilia. Joint bleeds, an allergy to factor VIII, loss of mobility, numerous ports, and needle phobia are a few parts of his story. I remember his weeks of hospitalizations, nights of screaming in pain, and missed days of school. He also had days of feeling "less than" because he couldn't participate in sports; his knee and ankle were too damaged. Climbing stairs and walking to class took a toll on Caeleb. That trauma remains with him at 18.

Changes over time

I've found over time that when I share a more recent family photo, I see the contrasts from when my sons were little. Many viewers don't see how Caeleb's art is more than a hobby or skill, for example, but I can see that it's his way of expressing the depth of his experience and is a testament to his resilience.

Julian's path with hemophilia may have been smoother, but his support of his brother and pursuit of his dreams show a different kind of strength. His passion for performing is what feeds his soul, and hemophilia is something he lives with; it doesn't dictate his life. My sons' resilience is a source of inspiration and hope for us all.

Alongside the trauma and grief, I feel an overwhelming sense of pride. My sons are now adults. Their experiences have made them into the men they are today. All the bleeds, hospitalizations, and medical challenges have only served to strengthen them. Their resilience and determination command my deepest admiration and respect.

The polished images shared online can never capture the whole truth. But as I continue to post, I find myself more comfortable unearthing the layers of our reality.

It's not just about the highlights anymore; it's about honoring the messy, painful, and beautiful journey we're on. It's about showing that life can still be profoundly meaningful and deeply blessed even when it doesn't look like the picture-perfect postcard. We've learned to embrace the pain and beauty of our journey — not just Caeleb and Julian, but our entire family.

Note: Hemophilia News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or another qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Hemophilia News Today or its parent company, Bionews, and are intended to spark discussion about issues pertaining to hemophilia.

Hemophilia Patients More Likely To Be Infected With SENV: Study

People with hemophilia are more likely to be infected with the SEN virus (SENV), which is associated with blood transfusion hepatitis, or liver inflammation, than healthy people, a study in Iran suggests.

The virus was detected in more than half of patients with hemophilia A and most commonly in those with more severe disease.

"Our findings highlight the importance of continuous monitoring and follow-up of high-risk groups in relation to blood-borne pathogens, providing reassurance about the ongoing efforts in the field," the researchers wrote.

The study, "Investigation of SEN virus prevalence in hemophilia patients," was published in New Microbes and New Infections.

Hemophilia is mainly caused by mutations in genes that provide instructions for producing proteins essential for blood clotting, called blood clotting factors, that prevent excessive bleeding. People with hemophilia produce either a faulty version of a clotting factor, low levels of it, or none at all.

The main treatments for hemophilia are factor replacement therapies, which involve supplying the patient a version of the missing clotting factor. Because these factors are commonly obtained from donor blood, there's a risk of contamination with blood-borne pathogens, including SENV.

Analyzing prevalence of SENV in hemophilia

Here, a team led by researchers at the Birjand University of Medical Sciences in Iran analyzed the prevalence of SENV and related complications in people with hemophilia. The study included 135 participants, 80 with hemophilia and 55 healthy people. The hemophilia patients were predominantly male (93.7%) and had a mean age of 27.4. The healthy participants were mainly female (63.6%) and had a mean age of 41.6. Most patients (86.3%) had hemophilia A, followed by hemophilia B (6.3%).

The SENV virus was detected in 58 participants (43%), with a significantly higher prevalence among those with hemophilia than healthy people, 58.8% versus 20%. These results underscore a "potentially heightened susceptibility to SENV infection among hemophilic individuals," the researchers wrote.

The virus wasn't detected in any participant with hemophilia B, but 65.2% of hemophilia A patients were positive for it.

Those with severe hemophilia A, meaning levels of clotting factor VIII below 1% of normal, had a higher prevalence of SENV (69%), followed by patients with moderate disease (65.8%; patients with FVIII levels between 1%-5% of normal). The virus wasn't detected in patients with mild hemophilia A (FVIII levels between 5%-10% of normal).

SENV-positive participants were generally younger and had significantly higher levels of the liver enzyme aspartate transaminase (AST), a marker of possible liver damage.

SENV-H and SENV-D are the most common types of the virus. In the study, type D was the most frequently detected (60.3%), followed by SENV-H (39.7%). Coinfection with both SENV-H and SENV-D was seen in 19% of participants.

Clarifying the "immune signature associated with SENV infection" is important for a better understanding of the virus's "pathogenesis and potential clinical implications," wrote the researchers, who noted doing so may shed light on possible preventive and therapeutic approaches for "managing infections in high-risk and general populations." They said future research should include a larger sample size to "enhance the statistical power and generalizability of the results."

Marstacimab Gets FDA Nod For Hemophilia A Or B Without Inhibitors

The FDA approved marstacimab (Hympavzi) for routine prophylaxis to prevent or reduce bleeding episodes in adults and adolescents ages 12 years and older with hemophilia A without factor VIII inhibitors or hemophilia B without factor IX inhibitors, the agency said Friday.

Marstacimab targets the Kunitz 2 domain of tissue factor pathway inhibitor (TFPI), a natural anticoagulation protein that functions to prevent the formation of blood clots and restore hemostasis. It is the first anti-TFPI approved in the U.S. For the treatment of hemophilia A or B.

"Today's approval of Hympavzi provides patients with hemophilia a new treatment option that is the first of its kind to work by targeting a protein in the blood clotting process," said Ann Farrell, MD, director of the Division of Non-Malignant Hematology in the FDA's Center for Drug Evaluation and Research. "This new type of treatment underscores the FDA's commitment to advance the development of innovative, safe, and effective therapies."

The FDA's decision was based on the phase III BASIS trial of 116 adult and pediatric male patients with either severe hemophilia A or severe hemophilia B, both without inhibitors, who had received usual care with replacement factor either on-demand (33 patients) or as prophylaxis (83 patients) during an observational period. These patients then received marstacimab prophylaxis for 12 months.

Of those who had received treatment on-demand, the annualized bleeding rate (ABR) for treated bleeds fell from a mean of 38 during the observational period to 3.2 after treatment with marstacimab, representing a 91.6% reduction in mean ABR during the 12-month active treatment period.

Among those who had received routine prophylaxis as usual care, the ABR fell from a mean of 7.85 during the observational period to 5.08 after treatment with marstacimab, translating into a 35.2% reduction in mean ABR.

"The approval of Hympavzi is a meaningful advancement for people living with hemophilia A or B without inhibitors for bleed prevention, with a generally manageable safety profile and a straightforward once-weekly subcutaneous administration," said Suchitra Acharya, MD, director of Northwell Health Hemostasis and Thrombosis Center, Cohen Children's Medical Center, in New Hyde Park, New York, in a news release from Pfizer, the drug's developer.

"Hympavzi aims to reduce the current treatment burden by meeting an important need for these patients, including many who have required frequent, time-consuming intravenous treatment infusion regimens," added Acharya, who was a co-investigator in the BASIS trial.

Marstacimab comes with warnings and precautions about thromboembolic events, hypersensitivity and embryofetal toxicity. Common side effects include injection site reactions, headache, and itching.

Pfizer said the drug is the first once-weekly subcutaneous prophylactic treatment approved for eligible people living with hemophilia B and the first to be administered via a pre-filled pen or syringe for eligible people living with hemophilia A or B in the U.S.

Mike Bassett is a staff writer focusing on oncology and hematology. He is based in Massachusetts.

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