Tiffany, my angel

I try to live every day of my life journey in gratitude and love. I am especially grateful for special people in my life who are wise beyond their years and have sometimes unexpectedly taught me valuable life lessons. I am grateful for those who have made my life worth living, and those who make my life easier and more joyful. I like to think of these special people as the angels in my life.

One of my angels is Tiffany. She is the cousin of my daughter-in-law Elizabeth. This wise and witty young woman had a liver transplant when she was 18 and then less than a decade later her liver started failing her. She underwent numerous bouts of unsuccessful chemotherapy and medical treatments for several years.

At our family Thanksgiving holiday in Corpus Christy, Texas, she announced that she “had enough of being picked at and prodded and infused with chemicals.” She said she had done all that she could do and had resigned herself to THE reality. In her witty manner Tiffany explained that she had directed her doctor to give her plenty of morphine when the time was right to take her to “Marguarita-Land.” She also announced that she was in the middle of planning a “bad-ass” celebration party of life and all things good that was taking place at the beginning of January. We were all going to eat and dance ourselves silly. We all started chanting, “Party! Party! Party!” Tiffany burst out laughing! To most families this might sound inappropriate, but we loved her dearly and we knew that each of us surrounding her at the table would also not be leaving this earth alive. We also knew that being inappropriate was sometimes appropriate.

I had just been diagnosed with leukemia three months before and was given a poor prognosis…

About 3 a.m., hours after everyone had gone to sleep, Tiffany and I tiptoed into the kitchen to sneak a piece of pie and surprised each other. We talked well into the morning. I was 57 when I was diagnosed. I had lived a great life. I was married to a man who was not only my husband since I was 19, but also my best friend and someone I was still crazy about. I had children, grandchildren, and several careers. I was working my way through the reality of accepting was is and accepting what is not.

Tiffany was only 28. My heart ached for her.

She said that she had lost some friends along her journey with cancer and some friends unexpectedly surfaced. She said that she now understands that when her friends stress about petty nonsense, it wasn’t petty nonsense to them. It was their life and it was okay. Tiffany came to a place where she realized the rest of the world did not revolve around her illness. She was not afraid, but she was thankful for modern medicine and morphine to eliminate the suffering. She helped me immensely that night – my wise and courageous little angel.

Tiffany passed before Christmas that year. She was surrounded by most of her family. Everyone was crying at her bedside, when she suddenly woke up. Apparently, she had not been given enough morphine. She saw everyone crying and proclaimed, “Why are you crying? I ain’t dead yet!” The room filled with laughter. And then quietly she passed smiling. Having the last word was always her thing.

We had the party in January, as she instructed, and ate, danced, hugged each other and laughed. We celebrated Tiffany. It was good.

She has been with me my entire journey. I carry her in my heart. Thank you MaMa BeBe for your daughter. She gives me great comfort.

Patient Advocacy

Well, it has been a busy patient advocacy time for me lately. I was on a panel with two researchers/physicians for the private screening of the PBS documentary "The Emperor of All Maladies" sponsored by the Leukemia & Lymphoma Society.

Adrienne Briggs, MD, Arizona Oncology; Raoul Tibes, MD, PhD, Mayo Clinic; 

La Verne Abe Harris, PhD, patient advocate

I also participated in a panel discussion on living well at the Tempe (AZ) Town Hall Meeting for CLL/SLL patients and caregivers. Here is my interview (10 minutes) with Andrew Schorr of Patients Power.
http://www.patientpower.info/video/dr-la-verne-abe-harris-i-live-in-the-here-and-now/?autoplay=1&utm_source=health-topic-alert&utm_medium=email&utm_campaign=CLL-2015-0014-02&utm_content=efc19eaf01

I recently returned from a trip to the National Institutes of Health. Good news! I am still in clinical complete remission. My white blood count is now a little over 7,000, which is in the normal range. I am enjoying this beautiful moment.

Living Life

I have been busy living life. That’s a good thing. I don’t know how much time I have in remission, so I am taking advantage of every minute and living my life in gratitude.

“Man.
Because he sacrifices his health in order to make money.
Then he sacrifices money to recuperate his health.
And then he is so anxious about the future that he does not enjoy the present;
the result being that he does not live in the present or the future;
he lives as if he is never going to die, and then dies having never really lived.”

-- Daliai Lama

Here is my gift to you. It is an on-going list of 50 things to live your life to the fullest:

1)    Be mindful.

2)    Be kind.

3)    Do something each day that inspires you or makes you smile.

4)    Do what you love.

5)    Check out the sunset at least once a week.

6)    Check out the sunrise at least once a week.

7)    Look around you and make a note of the beauty in this world.

8)    Surround yourself with people who love you just as you are.

9)    Spend some quiet time with yourself in meditation or prayer.

10) Contact someone you have not been in touch with for a long time.

11) Try something you have not tried before.

12) Listen to music.

13) Go for a walk.

14) Laugh.

15) Dance.

16) Grow a garden.

17)Stop taking yourself so seriously.

18) Stop taking life so seriously.

19) Forgive others.

20) Be compassionate.

21) Organize your closet and your living space.

22) Eat slower.

23) Have a glass of red wine.

24) Simplify your life.

25) Keep your promises.

26) Sleep well.

27) Slow down and stop driving like you are in a race.

28) Learn to say “No.”

29) Eat one meal a day with someone.

30) Continue to learn and be curious.

31) Be a child.

32) Stop wasting time on non-essential tasks.

33) Mistakes get made. Learn from them and move on.

34) Challenge yourself.

35) Go outside and enjoy nature.

36) Listen to others.

37) Stop complaining. Do something about it or ignore it.

38) Celebrate the success of others.

39) Don’t be afraid to fail.

40) Don’t gossip.

41) Take care of your body.

42) Take care of your mind.

43) Improve your posture.

44) Cherish your parents.

45) Stop trying to please everyone.

46) Work on improving yourself.

47) Accept constructive criticism.

48) Stop blaming others.

49) Make someone’s life easier.

50) Live in gratitude.

A Visit with the UCSD CLL/SLL Experts

My husband Carl and I met with Dr. Michael Choi, a researcher in The Thomas Kipps laboratory at the Moores Cancer Center at the University of California – San Diego in La Jolla. We discussed some of the clinical trials and options for CLL/SLL cancer patients who have relapsed. We also discussed some of the unknowns.

RECENT RESEARCH:

Recent worldwide cancer therapy research has focused on

• targeted therapies (i.e. ibrutinib, idelalisib):  

Ibrutinib was FDA-approved July 2014 as a frontline treatment for 17p deleted CLL/SLL patients. Infinity Pi3K oral inhibitor drug (idelalisib) was FDA approved July 2014 for relapsed CLL, follicular lymphoma and small lymphocytic lymphoma (SLL). Inhibitor oral cancer drugs are successfully tolerated for the most part; however, these targeted therapies developed to attack only cancer cells still have an effect on other body tissues, often leaving the body subject to infections and low immunoglobulin levels.

• monoclonal antibodies (i.e. rituximab, obinutuzumab, ofatumumab):

According to Dr. Choi, Gazyva (obinutuzumab) is superior to rituximab and is a new agent. It was FDA approved November 2013. Arzerra (ofatumumab) received FDA approval to treat CLL April 2014. These monoclonal antibodies also have an effect on other body tissues, often causing infections and low immunoglobulin levels.

• targeted chimeric antigen receptor T-cell (CART) treatments:

This cancer treatment, which is in clinical trial, has cured a number of leukemia patients, but it has left them with no healthy B-cells, since the treatment wipes out all B-cells. This means the patients must have monthly blood infusions. Researchers are continuing to figure out a way to target only leukemic B-cells.

UNKNOWNS:

One of the unknowns in the CLL research community includes what to do when ibrutinib stops working. Does the patient move to another kinase inhibitor (i.e. ABT-199, IPI-145)? Are monoclonal antibodies combined with the kinase inhibitors? What are the long-term side effects of continuing to use a drug after the patient has reached clinical complete remission? Can a patient safely stop or does the cancer return full force?

UCSD CANCER LAB TRIALS:

I was particularly interested in the ABT-199 and ROR-1 clinical trials at UCSD. There are a number of ABT-199 clinical trials in Phases 1b to 3 alone and in combination with monoclonal antibodies at UCSD. Lots of possibilities.

The ROR-1 clinical trial at UCSD is in the early stage of research. The researchers at the Moores Cancer Center have been working on addressing the goal of specific leukemic cell targeting without harming other body tissues.  The researchers discovered that a protein called ROR-1 is found on leukemic cells, but not found on normal B-cells or other normal body tissues. ROR-1 is also expressed in other cancers. A monoclonal antibody named Cirmtuzumab (a.k.a. UC-961) was found to bind to ROR-1 in preclinical tests with laboratory mice without any apparent toxicity. June 2014 the FDA gave the go-ahead to experiment on relapsed or refractory CLL patients in a Phase 1 clinical trial to determine safety, optimal dose, and whether it is tolerable. Patients will be infused intravenously every two weeks for two months. The experiment will continue based on safety and effectiveness.

Even though this clinical trial is in the early stages of blood cancer treatment, it could have a profound effect on all cancers. Blood cancer research is leading the way for the cure of all cancers. It is a good day.

Clinical Complete Remission at 30 Months on Ibrutinib

I just returned from my quarterly visit to the National Institutes of Health (NIH) in Bethesda, Maryland, where I am in a clinical trial with the drug ibrutinib. I flew to NIH and arrived with the outside temperatures at 10 degrees. Brrrrrr. It was 70 degrees in Arizona. I brought along sinus and lung congestion that I have been fighting for a while.

I will get right to the very good news: If my bone marrow biopsy results on July 2015 are the same as the results of July 2014 (which Dr. Adrian Wiestner is optimistic about), then he declares that I am in clinical complete remission at 30 months of ibrutinib treatment. This is the best news I have had since I was diagnosed with CLL/SLL (17p deletion plus p53 mutation, a poor prognosis) August 2009. Hurrah! Hurrah!

This, however, does not mean I am cured and this is why:

A cure by definition means the end of a medical condition. A disease is incurable if there is a chance for relapse. I still have 59% damaged 17p deleted cells lurking about in my peripheral blood, according to my FISH test from last summer. When I began the clinical trial, I had 97%. So I have improved. The remarkable feature of ibrutinib is that it is not dependent upon a 17p diagnosis as being a poor prognosis. If I were undergoing chemotherapy intravenously, then the 17p-deleted cells would matter.

I never expected a clinical complete remission. I knew that 17p deleted patients were having partial remission (PR) on ibrutinib.

A remission by definition means the temporary end to the symptoms of an incurable disease or the absence of disease activity. A complete remission (CR) is the complete disappearance of all manifestations of the disease. A partial remission (PR) is the 50% or greater reduction of cancer cells.

I personally only knew of one 17p patient who had a clinical complete remission early on with ibrutinib and that was my blood brother Dr. Matthew Hils. He told me he had messy cytogenetic and 17p was not the only problem in his blood. He relapsed and passed away before he could be treated with ABT-199, another oral kinase inhibitor in a clinical trial. Matt was such a good person. All of us at NIH miss him.

In spite of the fact that I arrived at NIH with sinus and lung congestion, my blood work still managed to be normal. That was exciting news.

As a proactive patient, my next concern was a Plan B. What do I do if I relapse on ibrutinib, since the 17p deleted patients have been relapsing in about three years? Dr. Wiestner said that there would be many more options available for relapsed patients in the future. If I relapsed TODAY, Dr. Wiestner said that the options would be:

•   idelalisib: An FDA approved (July 14, 2014) PI3K inhibitor effective with p53 mutated patients, but still too costly for me, since the U.S. Congress has not passed the cancer drug parity bill. The drug company reports that the monthly cost for idelalisib is $7,200, which they report is lower that the comparable price for ibrutinib, which sells for $8,200 a month.

•   ABT-199: Currently in clinical trial

•   Half-match stem cell transplant: Remember that since I am bi-racial, I have less than a 1% chance of finding a match. He said that my brothers may be half matches and that I am still young enough for that option until I am 70. I turn 63 this month. Siblings have a 50% chance of being a half-match for each other a 25% match of not matching at all, and a 25% chance of being a perfect match.

I asked about the ROR-1 studies. He said they are still in the early phases.

This month I am driving to San Diego to have a consultation with Dr. Michael Choi, who works with Dr. Thomas Kipps at the UCSD medical research center. They are doing a clinical trial using ABT-199. My name is in the system. I just want an option available just in case.

Then Dr. Wiestner smiled and said that because I used ibrutinib as a front-line treatment, I am one of the 17p-deleted patients least likely to relapse. I paused, took a deep breath, and had the unbelievable urge to call my husband and tell him that I may be around a little longer.