Atypical HUS: Perspectives
an aHUS Alliance interview with Cameron James
Cameron James is a Nashville-based singer and songwriter. In 2014 her young daughter Elise was diagnosed with the rare disease atypical HUS, and as Cameron remarked in a recent interview “At first, it was a tidal wave of panic.” (B Schmitt, The Tennessean: for USA Today) Fast forward after becoming informed about aHUS and slowly developing a sense of strength through faith and gratitude, Cameron received another shock – as three years later her husband James Shadinger’s fever led to a hospital trip to the ER and his own aHUS diagnosis. In March Elise turned 8 years old, and their family’s rhythm of life includes eculizumab home infusions for two.
Music: a Healing Outlet
Music tells a story that many can relate to, along with the release and freedom of artistic expression. How has that been helpful to you? Has your songwriting changed, and is there a difference in lyric or tone in the songs before and after aHUS?
Music has always been an emotional outlet for me. I have been singing since I was a little girl, but I started to write songs my sophomore year in college. I loved being able to storytell through melody and lyrics. Naturally, the songs that came out of me reflected the season I am in. You can definitely see a difference in what I’m writing about after my daughter and husband were diagnosed with aHUS. I have been writing a lot about faith and overcoming obstacles. I write about the struggle and the heartbreak, but there is definitely an undercurrent theme about my efforts to hold on to hope. I look at my music as a powerful tool to connect to people and encourage people. I want people to feel something when they listen to my music, to be moved. Music allows you to say something you aren’t always ready to say in conversation. Honesty is what touches people. I want my art to leave people feeling connected to the struggle but also leave them with hope.
I think everyone needs an outlet to process disappointment and heartbreak, and for me it was music. It felt good to be honest about all that was happening while doing something that I loved. My outlook also changed when I shifted the focus off of me and my family’s situation to others who were hurting and suffering too. I went to Washington DC to speak about my support for the American legislative bill “The Access to Marketplace Insurance Act” which would allow charitable contributions for chronically ill patients. Advocating for the aHUS community and other rare disease patients made me feel like there was something positive coming out of something so negative. It gave purpose to the pain, and it felt good to focus on the things I was in control of instead of all the things I couldn’t. I would encourage anyone feeling defeated to start helping and encouraging others, and I promise you will watch your paradigm shift.
The Family that Infuses together, Stays Together
When your husband was diagnosed, how was the experience different for you? Having your child undergo medical care is one thing, but there are probably wide differences in your emotions and actions when your husband was ill.
Elise got sick in November of 2014 when she was three. It was absolutely heartbreaking and I was suddenly thrust into this world of being “ultra rare.” It was terrifying knowing there was only one treatment and that eculizumab was one of the most expensive drugs in the world. As a parent you are already dealing with the trauma of seeing your child suffering, but then you add the pressure of what if the medicine doesn’t work or you can’t afford it? The pressure of the situation was unlike anything I had ever experienced before in my life. There were so many unknowns at the time. I had no idea Elise was going to regain 100% kidney function and that she could still have a normal life even with the inconvenience of biweekly infusions. I had no idea that we weren’t that rare. Even though we technically were the first pediatric aHUS case from Nashville, we would later be connected to other families and a whole community of people who relate to what you are going through. I had no idea about the financial support available to rare disease patients and that there were people who would do everything they could to help you stay on this life saving medicine.
There is a statistic within the aHUS community that says for every million people there are approximately two with aHUS. After Elise got sick, our family did genetic testing. It was discovered that my husband, James, was the genetic carrier. Elise and James have an MCP mutation which is common in about 10% of aHUS patients. In learning about genetics we discovered that just because you have a genetic mutation doesn’t mean the disease will ever manifest. In fact James’s parents underwent the genetic testing and it was revealed that the genetic mutation came from his mother. She has never manifested this disease nor has anyone in her family. The odds of James’s manifesting, according the estimate of two per every million people, were extremely rare.
We feel Elise saved James’s life. Because of her we were equipped and educated, and recognized when James started manifesting aHUS symptoms. James was able to walk into the ER and tell them his genetic mutation and request a blood panel, informing them to specifically look at his kidney numbers. When he arrived at the ER, my husband James was at 20% kidney function. He was crashing much faster than our daughter Elise did. Thankfully, our daughter’s nephrologist coordinated with James’ s hematologist and everything got fast tracked. He was on the same life saving medicine within 24 hours.
I was absolutely terrified when James called me from the ER after the blood panel revealed his aHUS had indeed manifested. I called my family and prayer warriors, and I faced this storm the same way we did with our daughter…with tears and faith. My faith in God is without question the anchor that keeps me from going under. I truly believe that God is sovereign over what happens to us even when He is not the author of disease. I saw God’s faithfulness during our storm with Elise and I knew He would be with me again with the storm that now involved my husband. It was easier with James because I knew the road we were on. It was awful and familiar but I believed more confidently this time in the turnaround and I knew I wasn’t alone.
When Elsie was in the hospital we had our friends and church family bring us meals. They raised money for our hospital bills and many came with me to my daughter’s infusions when my son was born. I was blessed to have to so many people want to help and in turn I received that blessing because I allowed people to help. Relationships can grow and deepen during these times of difficulty. Letting people in when you are in a challenging time is hard, but I can’t say enough how much it helped practically and emotionally.
Information is Empowering
Did you have an informed medical team already in place? Emotions must vary with the different roles filled by your husband vs child. What was easier, what is harder?
Yes, our daughter’s nephrologist, who now has 4 aHUS patients, was able to work with James’s medical team. Information about James’s genetic mutation was already noted in his medical records, which was incredibly helpful,and the same hospital board that approved the medicine for Elise remembered us. Her previous aHUS journey definitely aided the speediness of James’ diagnosis and treatment approval. The more educated you are, the easier the diagnosis and approval of treatment. This potentially can aid with shorter recovery time and prevention of long term organ damage. No reason to fear, instead just be prepared with knowledge and especially in recognizing symptoms should you or a loved one ever start manifesting symptoms.
My husband’s job is a tremendous blessing. We have been very fortunate that his company has good insurance and that his job tenure has benefits that allowed him to miss work without fear of consequences. However, it is always scary when the rock of your family and the main income earner gets sick. I absolutely have dealt with fear of what if he gets fired and lost his insurance. What if he didn’t recover and I now had replace the income and medical insurance my husband provided. I love my family and was absolutely prepared to do whatever I needed to do. I feel very, very blessed that James recovered 100% of his kidney function like Elise did and was able to return to the work he loves. I still have moments where the fragility of our situation catches my breath, but this is again where my faith helps me work through my fear. God promises to never leave or forsake us and to work all things for our good. Whatever happens, I know God will help me and help my family get through.
Family Genetics & aHUS
People often feel conflicted about the pros and cons of having other family members undergo genetic testing to see if they have similar mutations that predispose them for aHUS. Did you decide to do genetic testing, and what considerations weighed in to your decision process?
The more we learned about aHUS and its genetic components, it was a natural decision to do familial genetic testing. We wanted to support the research of this disease and also wanted answers. I also knew I wanted to have another baby. We knew in our hearts our family was not complete. James and I understood there was a 50/50 chance that his genetic mutation could be transferred to our future child. However, we prayed and felt peace. We didn’t want to make a decision based out of fear. Bottom line is Elise got through her trial. There is a wonderful, effective treatment for aHUS patients and we saw how life could truly return to normal outside of routine infusions. We knew that even if the unthinkable happened, and our second child also had aHUS, there was a clear path ahead. That brought us comfort. So after Elise’s health rebounded we got pregnant with Cade. After Cade was born he had genetic tests, which came back negative. We were relieved but already had a peace in our hearts for whatever the results would have been.
How has aHUS changed your lifestyle? Your career plans? Your husband’s plans? Has there been a shift in your goals, priorities, or outlook? What insights would you like to share with others?
Atypical HUS definitely re-centers your priorities. My family is forever my number one priority but I’m so grateful that there is still plenty of room for travel and career goals. It does require planning, but that has not stopped anything. I think aHUS has made me more sensitive to other people’s pain and journey, and I’m honored that my family’s story has helped people believe that things can be ok again. One lesson I have humbly learned from both my husband and daughter’s story is to not judge the future based on the present…to leave room for the turnaround. The medical world is constantly changing and evolving and I’m excited to learn about the changes ahead. Infusion schedules are changing, one day different treatment delivery options, lots of reasons to be hopeful.
I think the number one insight I have for researchers and pharma is the value in easily accessible medicine. One of the biggest setbacks of aHUS is the infusion schedule. I am very grateful my husband and daughter are able to infuse at home and together. It requires a lot of planning and it’s costly, medically speaking. I look forward to getting rid of my daughter’s port. I dream about the day I can simply give her a pill and not worry about a port infection. Elise has already had one port infection and had port replacement surgery. I think the more time I can give back to my daughter the less of a hold this disease has over her life. That’s why new drugs given by a shot or longer times between treatments or infusions will be so exciting in the future. Anything that can be done to make effective medicines for aHUS more accessible, more easily administered, and more affordable – the better for all.
Two years ago Cameron James spoke with various Senators and Congressmen in Washington DC, to lobby against a USA restriction that prohibits certain groups and charities from assisting with medical co-pays and insurance premiums for people with chronic and rare diseases (Gospel Music Assn, 2017 interview).
The aHUS Alliance wishes to thank Cameron for this interview, and join with her in encouraging aHUS patients and their families to launch advocacy efforts in their own nations. Collaborative efforts can make a difference in the lives of others struggling with rare disease issues.
Contact us regarding interest in sharing your aHUS Perspectives at info@aHUSallianceAction.org
More about Cameron James, her music and aHUS Advocacy
2016 aHUS Awareness Day – Cameron James’ video to raise awareness for aHUS: 24 SEPT campaign
All Is Not Lost – Music video by Cameron James, about her daughter and husband’s journey with aHUS. (Song co-written and performed by Cameron James)
Born To Bring The Light – Music video by Cameron James, released on DREAM Records. In a TCB article, this song is about how destiny can be revealed through heartbreak, with lyrics that center on her family’s medical challenges and strength gained through her faith.
2018 Global Genes Summit – Featured Performing Artist
Cameron James www.cameronjamesmusicofficial.com/bio
Her Social Media Includes Instagram cameronjamesmusicofficial