Friday, March 21, 2014

Crashing a doctor party

As Greg mentioned in a previous post, I just got back from a whirlwind trip to Denver attending a workshop on stem cell transplantation in CTCL hosted by the United States Cutaneous Lymphoma Consortium, a new organization with a goal of fostering a multidisciplinary approach to research and treatment of cutaneous lymphoma. Basically, it's the Cutaneous Lymphoma Foundation for clinicians. 

The event bought out the nation's top CTCL dermatologists, oncologists and transplanters in an effort to better under the role of transplant in fighting this tricky disease. I was out of my comfort zone in this crowd but felt it would be beneficial to attend since Greg is one of only a couple hundred people in the world who has had a transplant for this disease - in fact, as I learned, he was one of only 12 CTCL patients transplanted nationwide last year. CTCL, by default, has become a passion of mine for obvious reasons and I wanted to hear the latest discoveries. 

While I can't say I came away with much more than I knew before, I found watching the doctors collaborate fascinating. The structure of many sessions was interactive, lively and informal. Ultimately, there were more questions than answers but I think some good recommendations and perhaps even guidelines will come out of it. 

The first part of the morning involved a general overview from Yale's Francine Foss, who outlined the greatest challenges for patients undergoing transplant. This includes finding donors, made even trickier for individuals who aren't white. Foss said about a third of her patients have no sibling match or donor from the national registry. In general, people are getting transplants at older ages and seeing improved outcomes with unrelated donors with identical HLA matching. Major complications include disease relapse as the primary cause of death, followed by GVHD and infections. I learned that full immune system recovery is actually closer to two years even in healthy patients who aren't immunocompromised. 

The day followed with raw data presented from Yale, MD Anderson, Northwestern, UPenn, City of Hope, Stanford and Harvard. There were seven transplant docs who attended and I'd guess around 75 dermatologists and oncologists. As the day progressed, moderator-led discussions focused on questions like criteria for deciding when to send a patient to transplant, whether patients need to be in complete remission pre-transplant and how is that defined, what is the best conditioning regimen, are particular agents given pre-transplant associated with better outcomes, and what should be done in the face of post-transplant relapse. Stanford's Wen-Kai Weng gave a presentation about the balance of fighting the disease while warding off GVHD. The short of it? It's difficult and often involves a lot of adjusting of medications to achieve desired response.  

Dr. Cutler presenting Dana-Farber's data. After the presentation, he told me Greg was in the green post-transplant cure column, which was nice to hear. It was weird to see his info on the screen with fresh memories of living through the hell of that experience. 

Going into transplant, it's important for people to know the risks involved, and the risks are very real. As we've written extensively on this blog, the chronic graft vs. host disease Greg is living with is a nightmare. His skin, fascia and muscle have hardened across 90 percent of his body and all we can do is adapt and hope it will be reversed. He is in excruciating pain that requires high doses of pain medication. He wills himself through the worst of it but it is an exhausting process that has brought great anguish to our lives. No treatment, for both CTCL and GVHD, seems to ever have higher than a 30 percent response rate, which isn't good enough, but it's all we have to go on at the moment. 

The day concluded with two patients speaking on behalf of the Cutaneous Lymphoma Foundation, Joe Carey and Danna Wessels, about their transplant experience. Both are lucky to to have had relatively smooth sailing post-transplant, and neither have had a lick of GVHD. Early in the day Susan from the foundation asked if I would be interested in joining them. I said no because I thought it should be about the patients but really wish I had accepted her offer. There were several questions from the doctors about assessing risk and communicating openly that I would have loved to answer. I think my perspective would've added real value but that's a blog post for a different day, and maybe Greg and I will be invited to speak next year.  

I would have liked to see a bigger focus on managing GVHD post-transplant, but ultimately those questions are for transplant doctors, not CTCL doctors.  

Here are some facts I found interesting: 

- Most transplant centers use reduced-intensity conditioning (often radiation but no or little chemo) to treat their CTCL patients. Dana-Farber is an exception to this rule. The goal at DF is to induce a little GVHD to combat disease. 

- Penn has had zero cases of GVHD in the 13 patients it has treated for CTCL. Stanford's numbers are also low due to lower-intensity conditioning regimens. 

- Very few MF/SS patients achieve complete remission going into transplant. The goal is to knock back the disease enough to let the new cells do their job. That's about a six-month window post-transplant. 

- Two-thirds of patients generally relapse post-transplant but often it is a less aggressive disease that can be managed by tapering immune-suppressants and letting the graft do its job, giving more cells through donor-lymphocyte infusion (DLI), with topical treatments, or targeted therapies. As we have seen first hand, relapse is not the end of the world. Greg is now in remission after relapsing at four months. 

- Stanford and MD Anderson have conducted the most transplants for CTCL. Northwestern, Dana-Farber and City of Hope are close behind. 

- Patients with large cell transformation are more likely to recur after transplant. 

-  There is a very small window of time when you can go to transplant and finding that takes skilled teamwork among derms, oncologists and transplant doctors. 

- Cord blood transplants offer hope to those without HLA matches but doctors have not fully jumped on the bandwagon. DLI is not possible with cord blood so it may not be a good option for CTCL patients.

- Age cutoffs vary depending on the center

- Acute skin GVHD, although it can be bad, is often manageable. Frequently affects the skin, eyes and GI tract.

- Chronic GVHD acts more like an autoimmune disorder that can affect many organs. About 50 percent of patients have some form, mild to severe. 

- Optimal pre-conditioning regimen and GVHD prophylaxis is unknown. Each facility has its own protocol, every doctor has his or her own idea of what is right. That's true across the treatment spectrum with rare diseases. 

- The doctors treating this disease are incredibly humble, down-to-earth and kind. I wanted to hug them all but that probably would have been inappropriate. I met a lot of the next generation of docs coming up in this field and they are enthusiastic to take the torch. I also learned that my husband's former transplant physician's drink of choice is an Old Fashioned and that he is not only a great doctor but a wonderful person. 

If anyone is interested in more info, feel free to get in touch: 


  1. Great job. I wish I could have observed that meeting. It was interesting to find out I was one of the twelve transplants and I know another man at Northwestern that would be in the same group. I don't think anyone understands how time consuming it is for the care giver aftet transplant. I am so thankful for my husband. I have relapsed in my lymph nodes and some on my skin. They did a DLI 8 weeks ago. I have had almost no gvhd so they are starting me on peg interferon. I guess I am just the opposite of Greg. I hope he is able to start moving and reversethe gvhd soon.
    Deb Richards

  2. Thank you for your great coverage of the meeting. I wish I could have been there. It was interesting to read there were only 12 transplants for ctcl . There is Greg, me, and another man at Northwestern that I know. Greg got severe gvhd and no ctcl, I got almost no gvhd but relapse in my lymph nodes of ctcl. I just had a DLI 8 weeks ago and will start peg interferon to help induce gvhd to enhance the graft vs tumor. I really don't know where I would be without my care giving husband. You guys really carry the burden for us. Good luck to Greg, get that gvhd under control.
    Deb Richards

  3. Sorry, I didn't think the first comment went through!

  4. Amy and Greg - thank you for all the beta! Great report. I am still here in NH thinking of you, praying for you, and meditating on how to help my stem cells play nice with my little brother. I admire your collective strength. Love, Amanda

  5. Amy, Great summary of the workshop. It was great meeting you there and speaking with you briefly. You are a blessing to your husband and I admire your obviously tireless support of his ordeal with this. I hope to hear soon that he is getting past the GVHD. All the best to you and your family. Joe