A MUST READ article Caroline wrote!!
RRP Patient Case History
Focus Feature on RRP Patient, Caroline Lang
By Caroline Lang
Many of you reading this newsletter will probably remember me from the RRPF listserve, on which, from time to time, I’ve been quite active. My name is Caroline Lang and like you or your loved one, I have RRP. I am now 34 years old and had my first surgery, of what would be many more to follow, at six years old. I believe I’ve had about 136 surgeries on my larynx and trachea since then, but as you know, it sometimes gets difficult to keep track. That is not counting other surgeries or procedures I’ve had that are also related to the RRP either, which I think would put me much closer to about 150. Although my RRP, did effect my life (and my family’s) from the time I had my very first symptoms, I always tried my hardest not to let it stand in my way from living my life to the fullest I could. I managed to finish nursing school and really enjoyed taking care of my patients. It really fulfilled me being able to reach out to help others in this way. I think because of my own challenges with dealing with RRP, it enabled me to relate to them in a way I never would have been able to otherwise. I understood that when a person’s health and wellness was disrupted, (no matter what the cause), that a person isn’t just effected physically, but emotionally, socially, and spiritually, as well. It was my endeavor to try and assist patients in a holistic manner that attended to all their unique, individual needs. For many years, I worked taking care of persons in the community, from babies to older adults, (though mostly children), who had chronic multiple disabilities. Working with these special individuals really gave me a lot of insight. It helped me to understand a lot better what really was important in life. After my husband and I started dating, we knew very quickly that we wanted to marry and start a family. My husband was already a single father then to a five-year-old son, his ex-wife having passed away just before his son turned three. Since then, we have added two more boys to our family. The boys are now 13, 7, and 3 and they are a handful, but very, very, loved. Our family has dealt with the same challenges that most deal with, but a few more were added to our list over the years.
In October 2006 we became aware of several changes occurring within my lungs. Although a secondary infection of methicillin-resistant staphylococcus aureus was identified soon after and treated, it wasn’t until March 2008 (and after a pregnancy that was very complicated by very aggressive RRP necessitating an early delivery) that it was established that I had pulmonary papillomatosis. I had begun some preliminary research (and didn’t find very much back then) into pulmonary papillomatosis and wasn’t at all encouraged by my findings. It seemed that basically it was a death sentence despite whatever interventions I might take. I came across one report of a woman treated with IV cidofovir that seemed to stabilize her disease. Since that was all that I had to go on, (and also the only thing any of my doctors came up with), that was what I started. I had done the injections to the vocal cords for several years, but the IV cidofovir was much more difficult to tolerate given the toxicities involved. I managed with that for eight months, after I had a mediport placed in my chest for the treatment in April 2008. I was literally coughing up pieces of tumor at times (as much as an inch long), and blood at times, but the growth on my vocal cords had completely stopped while I was on it. I had begun to get suspicious though. As I did a little more research, I found that there were quite a few cases where people with pulmonary papillomatosis developed lung cancer and I was worried about it. I finally got them to test one of the tumor pieces I’d been coughing up and the results came back inconclusive on whether it was cancer or not. I ended up getting pneumonia again (I’d had several episodes since my initial lung problems had turned up that were MRSA related) and my CT showed a lot of new growth in my right lower lobe. I went in for another bronchoscopy in March 2009 and the results were highly suspicious for cancer. When I started coughing up gross amounts of blood the next week, the pulmonologist told me bluntly that in his opinion, I was going to need the right lower lobe removed and that I may have several lymph nodes involved as well that will need to be removed. I had an emergent lobectomy done the next day in Detroit. I wont go into the details of that recovery in this newsletter, other than to say it wasn’t easy, and was quite complicated. I ended up needing another surgery to save my life about 12 days later. It was a very scary time.
The pathology from my lobectomy confirmed a combination of papilloma and squamous cell cancer. In the beginning, I don’t think I really took in the full ramifications of what this meant to me. I knew lung cancer was bad, but I just didn’t know how bad. After getting out of the hospital, I threw myself into research – research on lung cancer, research on pulmonary papilloma, and research on cancer linked to HPV. I gathered dozens and dozens of articles, from medical journals all over the world. I clung to anything that looked remotely helpful, since the general prognosis that I was finding on both conditions was so incredibly poor and discouraging. It’s been a whirlwind of trying different treatments to see what – if anything, would help. I’ve tried (in order- more or less) artemisinin derivatives, more IV cidofovir, subcutaneous interferon, high frequency radiowave ablation therapy (RFA), embolization of my bronchial artery in the left lower lobe, artemisinin derivatives, chemotherapy (carboplatin and taxol), IV Avastin, more RFA, IV Avastin, RFA again, more chemotherapy (Nexavar), and most recently another embolization procedure this time on the bronchial artery in what is now the right lower lobe (used to be the medial lobe before the lobectomy). Of these interventions, I feel that the RFA has been quite successful (so far) in killing the cancerous tumors that have been treated with it. I also feel the IV Avastin really slowed down the growth of the papilloma and most likely the cancer. I went from having surgeries on my vocal cords at an average of 4 or more a year to 15 months between surgeries on the vocal cords and 13 months between surgeries on the trachea. Unfortunately, I still had the continued growth in the lungs, necessitating the repeated RFA procedures, those areas all confirmed cancerous. Of late, (I’ve been of the IV Avastin since the fall) I’ve needed much more frequent surgical interventions on my airways. The latest pathology results show that my right main stem bronchus, trachea, and vocal cords all are now showing high-grade dysplasia – just a step down from cancer.
All of this treatment has taken a toll on me, as have the symptoms of my disease itself. I’m not sure if it was from growths on my trachea on bronchi, or from pneumonia, or both, but I’ve had to deal with horrible coughing fits all last fall, winter, and going into the spring that were occurring several times a day. I would cough and cough, triggering my gag reflex and then I would be throwing up. My sides would ache from all the coughing and the only thing that would settle the episodes down were taking narcotics that would sedate me. I also had been dealing with chronic pain from my arthritis. I had come to believe that one of the things that had contributed to the progression of my RRP disease was the use of immunosuppressive steroids to control my RA (and my pregnancies). While the steroid dose I was on was a low one, I was completely dependent on it. I tried multiple times to go off of it following my lobectomy, and each time, was overwhelmed with severe joint pain to a point where I was completely crippled and so miserable that life wouldn’t have even been worth living in that shape. I was forced to make a horrible decision: take a drug that I know will ease my pain and suffering and make life livable again, but also one that will likely make my RRP and cancer worsen, ultimately shortening my life and disabling the fight I was trying to make for my life, or not take it and have more of a fighting chance to “beat” my cancer, but be so overwhelmed with pain that I had no quality of life at all. The choice was Quantity vs. Quality. I chose quality. I don’t regret it. I still have had to deal with joint pain and am limited with my treatment options, but it hasn’t gotten to the severity it was without the steroids, so I’m grateful for that much.
One thing that has kept me going through all of this is counting my blessings – what I’m grateful for, each and everyday. There are so many things we take for granted that we can’t even begin to realize – that is one thing I learned from working with my special needs patients. I’m grateful for each and everyday and count it as a blessing. I know that I have already defied the odds. I’ve had 5 separate cancerous tumors in 4 different lobes over the last 2 years and I’m still here. I know I’m blessed. I count my children as one of my greatest blessings – they definitely give me the drive to keep on fighting. I want to be here for them as long as I possibly can be. I used to have a lot of fears regarding how much time I had left, wondering if my kids would remember me, and how they would fare without me around. Through my faith, (I’m a Christian), I’ve been able to let go of so many of those fears and find peace in the midst of crises after crises. Sometimes, even I’m amazed by it. I pray a lot. I know there are a lot of other people praying for me too. I trust that God has a plan and purpose for me, and that whatever His plan is, I know that it is better than any I could’ve come up with, and that it will ultimately prevail, and because I can trust that I’m able to calm down and find the serenity. I’d be completely lost without my faith. I don’t know how I’d hang on through all of this otherwise; thankfully, I don’t have to go through this alone. I have God on my side, my family, my friends, and also this community of others fighting RRP disease. I know that my story is different from yours. All of our stories are different, but they are the same in at least on way – we’re all fighting RRP and we’re all here for each other through support groups like the RRPF and the RRP-ISA. I only found these networks just before my lobectomy, just over two years ago. I would especially like to thank the RRPF for their generous donation to help me financially with meeting the high cost of my COBRA payment. I’ve been unable to work since February 2009 and my husband has to help out at home with our young children, since that also is sometimes too much for me to do alone. Without the donations made by the RRPF and other individuals and charities, I simply wouldn’t have any health insurance right now, when I have needed it more than ever. I’m exceedingly grateful. When the RRPF asked me to help out with the Newsletter, as a Coordinator, in return, I was thrilled at the opportunity to do so. I can only hope that I will be able to do justice to what needs to be done.
I’m so glad to have had the contact with other patients, especially ones going through similar experiences to mine. Malena Hamilton was the first person I started corresponding with through the listserve. Like me, she was dealing with lung cancer caused by RRP. She had a young son, just as I had young children. We were the same age. We both worked in human service related fields. I related with her on so many different levels. Her death last year was disheartening, except that I know she is no longer suffering, but has found the peace she so greatly deserves and that gives me comfort. I miss her dearly. As for me, I’m still fighting. Recently, I had a molecular analysis done on my newest cancerous tumor and from that they were able to come up with a list of targeted treatments that they believe will be the most helpful in treating my cancer (and hopefully my RRP too, though time will tell on that). I started the number one recommendation, Nexavar, for a few days and ran into complications with bleeding around my RFA site that may or may not have been related to the drug. My latest embolization will, hopefully, prevent future episodes of bleeding in that lobe. I will be starting up again next week with the Nexavar and am hoping for the very best. I know that is the hope of each and everyone of you reading this also, and that gives me so much strength, so thank you and I promise I will keep you posted.
You can visit my carepage for updates at www.carepages.com/CarolineLang. I’ve been writing there for over two years, since just after my lobectomy. I’m hoping to use the material there in addition to much more to put together a book. I want this book to serve multi purposes and audiences. First off, I think it should be known that it would be a reflection of my faith, because as I’ve said before, without that, I wouldn’t be here at all, anyway. Secondly, I would like for it to be a resource and guide for patients with RRP and their families. Thirdly, I would like to advocate for the prevention and treatment of all HPV related diseases, especially concerning getting as much of the appropriate population as is possible vaccinated with Gardasil. Finally, I would hope that it might be inspirational to anyone facing adversity in his or her life. I have a lot of work, yet to do on it. The most challenging aspect will be the research and technical components, but I think that such material (even if it ends up getting published as a separate work) would be of tremendous value to patients and even doctors. The title of my main work will be: While I Breathe, I Hope. I will continue to keep busy as a mother to three young boys. For those of you who are parents, you know that is enough of a job right there. It is my hope that at some point I will be able to return to (paid) work. If my book is successful, (and my health would cooperate) I would love to visit schools to educate and promote vaccination of Gardasil to young persons, especially. Right now, I take things one day at a time and am always grateful for each day. Whatever it may bring, good or bad, it is still another day, and for that alone, I will be grateful.