Hopeful and Happy New Year

Hopeful and Happy New Year

Facts about NUT Midline Carcinoma

Click here for Grace’s most recent scans

The past two weeks are the closest Grace and I have come to experiencing ‘normalcy’, and it has been a complete blessing.  We have great hope. Her most recent scans from 12/20/18 show continued improvement without evidence of active malignancy (PET shows no new FDG avid disease, and all known lesions measure 1.8 SUV or less).  She is now done with 6 cycles of chemotherapy, but will continue immunotherapy (pembrolizumab) and possibly undergo radiation.  Unless her health changes, we will continue 4 cycles of immunotherapy followed by repeat scans. We are nowhere near out of the woods, but her response to therapy has been the best we could have hoped for and she is doing incredibly well.

Backtracking a bit, I want to share what has transpired since my last post. At the beginning of December between Grace’s 5th and 6th cycles of chemo, we traveled to Boston for a thoracic surgery consult at Brigham and Women’s Hospital, and we visited with the world experts in NUT Midline Carcinoma at Dana Farber Cancer Institute.  Given her low white blood cell counts and everyone coughing on the plane, we wore hospital masks and sat in the back corner of our flight. No one sat next to us, despite both flights being “full,” so the masks did their jobs.

Starting with the surgical consult, the surgeon we met has operated on 5 NUT patients with virtually all of them having recurrences in the postoperative period. Though he was amenable to a left lower lobectomy with radical lymphadenectomy of the mediastinum, he wanted to leave that decision to medical oncology. We all agreed that now is not the time, and there likely will not be a time, but we wanted to investigate the option.  If anything, we would pursue radiation, and if she remains recurrence-free after radiation, only then would surgery be considered; it would still be very controversial and we would be very hesitant to proceed (The rationale for surgery in this setting would be to remove the previously affected lung lobe with the goal of eliminating any microscopic disease that remains, since the pattern of recurrence for patients with thoracic NUT tends to occur in the mediastinal nodes and near the site of the primary tumor).  

Later that day we went to Dana Farber Cancer Institute for a consult with medical oncology and pathology to meet the doctors who have the most experience with this disease. Since literature tends to lag a few years, I was hoping to hear about a number of success stories that had not yet been published, but they only shared one additional case that I did not know about. To this day there are really only three known long term survivors with this disease.  After a lengthy discussion, I felt comfortable that we have options and a contingency plan in place. If her next scan showed progressive disease, we would enroll in a clinical trial as opposed to switching to an alternative chemo regimen. Historically, patients have not responded well to a second chemo regimen if they fail on the first regimen. They also discussed trial options with us, highlighting two trials opening soon and a third trial opening in about a year. If her next scan showed improvement without any new metastatic lesions, we would hold off on the trial, with the plan to pursue consolidative radiation to the left lower lobe and mediastinum.  We could also continue pembrolizumab, since she had such a great response with combined chemotherapy and pembrolizumab. 

On 12/20/18 Grace’s scans showed further improvement. PET demonstrated previous malignant sites now at background activity and no new sites of malignancy were identified. Thus, we would proceed with the plan to pursue radiation. After multiple radiation oncology consults, and a variety of opinions, it seems the general consensus is to hold off on radiation unless we see a recurrence. (Essentially, since she has no targetable disease on CT right now, radiating would require the radiation oncologists to radiate a large field. This raises multiple concerns: 1) it could put her at risk of severe toxicity such as pneumonitis and cardiac toxicity, 2) it could limit our ability to radiate in the future due to dose concerns, and 3) it would require her to suspend pembrolizumab for the 6 week period of radiation.

When we met with one of our oncologists on 12/21/18, he was happy with Grace’s progress. He considers her in “controlled remission,” which I know is just semantics, but it gives us hope. If she were enrolled in a clinical trial, they would likely consider her a “complete response.” We’re hesitant to tell people the word “remission,” because most people hear the word and think “oh great, so you’re good again,” when it’s a little more complicated than that. Controlled remission means that you are disease free on your scans while you are on treatment, and Grace has been on treatment and will continue to be on treatment for a while. True remission is being disease free off of any kind of treatment. Some would say a “cure” in oncology terms is disease-free scans for 5 years without treatment.

Given her excellent response/controlled remission/whatever you want to call it, our oncologists tentatively want to continue Grace on pembrolizumab for 35 total doses, and she will now be off chemotherapy unless she has a recurrence. Our oncologists think that the immunotherapy is the key ingredient in Grace’s success, the rationale being that the chemo probably lysed the tumor, but the immunotherapy probably being the thing that picks off any remaining cancer cells. If she were to have a recurrence, we would either switch to a clinical trial or re-start chemotherapy since she has not failed chemotherapy.

We asked about the possibility of continuing chemo now, but our oncologists did not support that idea. The carboplatin carries an increased risk of a hypersensitivity reaction now that she has had 6 doses, and the paclitaxel would be extremely hard on her bone marrow. Normally they only do 4 cycles of this regimen, but they extended it out to the soft maximum of 6 cycles in Grace because she was responding so well and her body was young and healthy enough to take the beating. All things considered, that afternoon when our oncologist summarized how well Grace has done was the greatest emotional high I have had in the right direction since our wedding day.

Later that night, we had a unique opportunity to meet with the longest survivor of NUT midline carcinoma, Joe Schwartz, who is now 10 years from initial diagnosis. Having recently moved to Southern California, he lives about an hour away from us and when he met Grace he let her know that she is the only NUT patient he has ever met in person. Hearing his story and spending time with him was reassuring for Grace and myself. It’s the first time we have met someone with her diagnosis and it truly gives us hope. Below is a picture of Grace and Joe after dinner.

I know many of you reading this may not be Christian or ascribe to religious beliefs, but the rest of this post describes part of our spiritual journey.  Regardless of what you believe, I would challenge you to consider your life and what an amazing gift it is.  When the threat of death seems so far off in the future it is easy to not think twice about the subject, but when the threat of death is all too real, how would you process it?

The most difficult part about seeing Grace go through this cancer is the emotional battle and combating the lack of control.  Two things she said that were incredibly difficult for me to hear are, “I don’t want to die bald,” and “I’m scared you will remarry and forget about me”.  Hearing this is so incredibly painful, and it is only magnified by looking at the data and wishing it were literally any other cancer. However, this lack of control strengthens our relationship and faith in God. Though it takes one gloomy thought of “you know other NUTs get recurrences and die, what makes you any different?” to make everything feel dark and impossible again. But the thing we are learning to do more and more reflexively to combat these toxic thoughts, our “shield of faith,” is gripping to the belief that she is healed through the blood of Jesus. The more we pray, the more we find peace.

When you step back, it’s amazing at how far God has delivered Grace from where she started, and we have clung every single day to faith that He will continue to deliver her. The prayers of SO many people are beginning to be answered. God has allowed this healing in Grace to happen despite the odds. Grace had every reason not to respond to chemo and immunotherapy because we were told most other patients stopped responding to chemo after 2 cycles and Grace didn’t have any PDL-receptor markers that would make her an optimal candidate for her immunotherapy. But Grace got through 6 cycles of chemo/immunotherapy, still showing improvement on the PET scans. Grace’s mom is still in awe, as she tells us that she word-for-word prayed that Grace’s scans would be disease-free by our last scan in December, and God answered this prayer. Seriously, how awesome is that? We are so thankful to God.

At this point, we feel like the Israelites when they were in between the Red Sea and Pharaoh’s army. They had just seen all of God’s power in freeing them from Egypt, but here comes the army that could kill them on the banks of the Red Sea before they even get to the promised land. They know that God has made a covenant with them to bring them to Canaan, but in their minds I’m sure they were thinking at that moment: “how can we get to Canaan if Pharaoh’s army kills us first?!” Likewise, there is every reason to be afraid in our own situation: The fear of recurrence can be paralyzing when doctors tell us of other NUT patients who had short-lived responses followed by rapid recurrences quickly ending in death. Recurrence is our Pharaoh’s army. But God is the great “I AM.” He is so great that His miracles surpass anything. We have faith, believe, and pray boldly that God will perform Grace’s version of splitting the Red Sea in that Grace will have no recurrence, and that she will be cured. We’ll never know whether it was the specific chemo combination, the immunotherapy, or Grace’s NUT-variant gene type that explains why Grace is in such a good place right now, but we believe that it is God’s power that ultimately explains this success at this moment.

David says in Psalm 6 “among the dead no one proclaims Your name” but that “the Lord has heard my cry for mercy” and “the Lord accepts my prayer.” This psalm has resonated with us for a long time, and we pray that Grace will not die an early death where she cannot proclaim God’s name, but we hope that she will be a testament of God’s power in overcoming the impossible and outliving this death-sentence for many many many more years. We keep praying for the fruition of this miracle, for no recurrence, for cure.

13 thoughts on “Hopeful and Happy New Year”

  • Amen!!!! Standing in faith with you guys! Grace is healed in Jesus Name. Thank God for how far he has brought her and continuously pray for his grace and guidance as you all make decisions regarding the next steps.
    Love, Ezi

  • I’m so happy to hear about Grace’s continued progress. We pray for you guys every day. I admire both of your steadfast faith in the darkest time of your life and have no doubt you’ve been blessed because of it. Looking forward to the next good update.

  • Lord be near Grace and Ashton and continue to strengthen their faith and marriage. We rejoice in the miracle of healing Hallelujah and we pray healing over Grace!! Rain peace over their minds and hearts. We trust in You Lord. Encourage Grace and Ashton with the goodness of all that you are. In Jesus name Amen

    We love you guys and are praying for miracles!! God bless you both

  • I am so thankful for you both and your endurance in this fight, we continue to pray and are grateful and humbled by the positive news updates, thank you so much for this post, may 2019 be a truly blessed year for you both as you walk this journey with the Lord each day, love you both!

  • God bless you and the family. Your faith will bring you hope and positive
    Progress. Our prayers are with you.

  • I love your proclamation of faith, your testimony of God’s work and how you are giving Him glory! You are in my prayers!

  • So happy to hear the news! I pray that Grace keeps getting good reports. The Lord watches over you and Ashton. If I had it my way I would change places with you! I have lived most of my life in chronic pain. I do wish it was me but God has his plan and for now you are doing great!! I love you both so much. Thank you for making the trip down to see us !! We always enjoy you both! Love Karen Kulp

  • Soooo proud of you, darling Grace! And so proud of our sweet Ashton too! What a wonderful Christmas present to receive…the gift of positive news. We love you both, and continue to love and support you from afar.

  • I was waiting for the update. I am so happy to hear the good news. I am also amazed that your faith is getting stronger, Ashton. I will keep praying that God provides you guys more strength in many ways every day through this journey. God will work through people. He will give extra wisdom to her Drs . and extra healing hands and warmness to the medical team, especially nurses 😉 Sending prayers,,,.

  • Praise God for the wonderful news~! Rejoicing with you guys! And continuing to pray for complete healing and faith in I AM for deliverance, hope, and endurance . <3

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