On Rare Disease Day

The anatomy of grace

I had my first appointment with the medical oncologist yesterday, on Rare Disease Day. Leiomyosarcoma (LMS) is a rare disease — and the retroperitoneal type (RPS) which I have makes up 0.2% of all cancers. Rare diseases are tough because there’s such a small market that there’s no economic incentive for companies to invest in research. So to some extent solutions are reliant upon the goodness of others, and ideas more abstract than return on investment.

I’ve been thinking a lot about that, because experiencing the goodness of others been so much of my life lately. Honestly, I think I may have cried more from all the kind gestures that you’ve shown me and my family than I have about the cancer itself, because I did nothing to deserve it. All I did was get cancer! Which is arguably a stupid thing to do.

When you face a rare disease with no clear solutions, you also think about God a lot. And all this love that you’ve shown me has reminded me of the gospel of grace — that what we receive is not always because of anything we’ve done to earn it, but sometimes it is given purely out of love.

The appointment

When we met with the medical oncologist, we talked through some treatment options. I got an echocardiogram yesterday too.

Chemo first

She recommended we start with chemotherapy before surgery, because:

  1. This allows us a chance to shrink or stop the growth of the tumor, and any other early stage tumors not yet detected, which may also make surgery easier.
  2. This also enables us to find out if the LMS responds to the chemo — we can see once we surgically remove the tumor what the impact was. Otherwise, if do the chemo post-surgery, we won’t know whether or not it works.

What is not clear in LMS is the benefit of pairing chemo with surgery, but it is generally done since directionally studies suggest better outcomes when both are done than with surgery alone.

Possible regimens

Recommended regimen

The regimen she recommended is called AIM (doxorubicin / ifosfamide / mesna). This is a very hard and aggressive regimen, and is given in-patient. I’d go in for 6 cycles of 4-5 days in the hospital, where it’s given to me via IV, 16 days at home, and repeat. It also has some long term risks such as congestive heart failure (1%) and risk of blood cancers like leukemia (1 in 1,000). I hear it is also hard on the kidneys — and remember, I’ll only have one after surgery.

People on this regimen cannot do much of anything — and generally don’t work during this time.

Other regimens

There is another she suggested called gem / docataxel, which is as more data behind it for uterine sarcomas than RPS. But easier to take.

From a patient board, I talked to someone with RPS who had consults at Memorial Sloan Kettering (MMSK), who recommended doxorubicin and olaratumab as the first line of therapy, because there seemed to be better survival data around it and it was less harsh on the kidneys. It is technically only approved for non-resectable (non-removable) tumors so there may be an insurance barrier. She then had two rounds of chemo after surgery, with zinecard to protect the heart (the doxorubicin is what increases your chances of heart failure).

She also went to MD Anderson who recommended doxorubicin and decarbazine, again to spare her kidneys.
Another guy had radiation only. Both patients are cancer-free a year later.


I asked my oncologist to poll a few doctors whose names I got from MMSK and Dana Farber to get ideas. But at the same time, I’m working to see if I can get a quick referral to get a consult myself for a second opinion.

For someone who is data driven, this is hard, because there really isn’t good data around the relative performance of these drugs. As my cousin Gloria shared, there are no head-to-head comparisons of the regimens. Because the number of patients in the studies is very small, even differences in disease-free survival and overall survival are hard to tease out as the expected survival between the study populations could have been very different before treatment.

So, as a person qualified only to play a doctor on TV, I will have to make a call as to the treatment to pursue.

The bright side

Can I just grouse for one second about this sarcoma before we get to this part? I don’t even get new boobs out of this #@!$@ cancer!

  • The past few weeks has been like a walking yearbook — it’s been so nice to reconnect and see friends from so many different chapters of my life and from all over. Thank you.
  • The kindness of strangers has been overwhelming: I’ve received gifts and encouragement from strangers! From a student of a dear friend; from the sister of another. Thank you.
  • I think my kids think it’s Christmas. They’ve gotten so much love and attention. Thank you.
  • So many surprises. I can’t name them all. The time you’ve taken to think of us, share things with us, feed us. Thank you.
  • More soft clothes. You know who you are. Thank you.
  • Hindsight: this week there was a local boy in the news with bacterial meningitis. Some of you will recall the one-in-a-million type of bacterial meningitis Audrey had when she was two, and what a scary time that was — and hard it was afterward (and those of you who know her will know now she is a force to be reckoned with!). But that experience gave me the ability to reach out to a friend of the family’s as a resource, and to be grateful for that experience, hard as it was, in many ways. That’s probably for another post.

18 thoughts on “On Rare Disease Day”

  1. Thank you Sandi for keeping everyone that cares about you informed. I see that your sense of humor is still intact and you make me smile, grousing about no new boobs. I laugh again just writing this.

    You’re such a smart young woman; if anyone deserves to find their way through this mess it’s you.

    My thoughts and heart are with you Sandi

    Liked by 1 person

  2. Love that you’re able to see and feel God’s powerfully deep, amazing grace! It’s been so humbling for me too. Your family is so very special and have gone through so much – your cancer, Audrey’s condition, Jonah, etc – God has been there EVERY STEP! He promises to never leave you nor forsake you! (Hebrews 13:5). He has plans to prosper you, to give you a HOPE and a future! (Jeremiah 29:11). xoxo.

    Liked by 1 person

  3. Hi again,

    Thanks for keeping us all up-to-date. I’m always thinking of you.

    Today I went to the hair salon (yet again, I’m writing to you about hair!) and saw how great my long-time hairdresser looks and feels. She’s a breast cancer survivor, and she is now literally back on her feet all day in the salon. She has daughters who were about the same age as yours when she started her cancer treatment. And, she also has a positive outlook to match yours. (However, I think your sense of humor is beyond compare!) I just wanted to send yet another story with a happy ending.

    Liked by 1 person

  4. Hang in there! You are tackling this like a real pro. Let us know if we can help connect you to anyone at MSK. Sending energy and love from NYC.

    Liked by 1 person

  5. Sandi, My thoughts and prayers are with you. Your positive outlook along with your sense of humor will help you get through this..Linda L.

    Liked by 1 person

  6. Keep the faith, with God we can do everything. I pray daily that this will all come to an end in favor of you, I pray that God will give all your family the strength to get through this terrible illness. May God keep you on his Loving arms and guide you through this. Love Jane

    Liked by 1 person

  7. Thanks for the update, Sandi! I’m sure you’ll pick the best path forward and my thoughts are with you at all times. Big hug from a not-very-into-hugs person.

    Liked by 1 person

  8. Praying that God will give you wisdom and faith as you consider your treatment options. Thankful that you are finding comfort in God’s amazing love for you! May you and your family continue to hold onto that! Xoxo

    Liked by 1 person

  9. Thank you so much for sharing your journey, Sandi. I admire and praise God for your sense of humor, intelligence and strength that I could tell from your reflection although we haven’t met in person yet. I will continue to pray that God will pour his special love to you – love that is so special and overwhelming that only people walking through the darkest valley can experience (Psalm 23: 4) and that God would give new songs to you – the songs that are so previous to his years as you sing them in the night (Jobs 35:10). Lots of love,

    Liked by 1 person

  10. Wow, not sure I really followed it all but boy, you take this stuff seriously! No one, and I mean no one, is gonna pull the wool over your eyes! You are a force to be reckoned with Sandi and I’m not sure this cancer has worked that out yet. Keep your faith solid and your friends close. We are all here with boxing gloves ready to fight the fight with you. Big love xx (ps I’m still sorry about all that bloody sugar but hey, enjoy)

    Liked by 1 person

  11. Thanks Sandi…didn’t get any update notif. from WordPress….Thanks for today’s FB update…been praying for you….glad to read that the rolled oats no longer taste like paste…did he omit to put honey and milk (Almond..really yum!) & apple and berries..in there?? and Pat is almost a vegan ever since he is diagnosed (he is allowed a bit of chicken)…so nowadays he always try to confuse steak with chicken and I had to call him out several times…’do not even try’…yes, the whole family should follow the same diet but it is just hard…….but he is certainly looking healthier than me!….Be strong and enjoy all the love from God and all your wonderful family and friends…..BTW, are you familiar with this:https://news.usc.edu/103972/fasting-like-diet-turns-the-immune-system-against-cancer/? There is a also a great documentary on amazon prime on Dr. Longo’s studies….

    Liked by 1 person

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