SERENDIPITY

The big news is that as of October 4, 2012 the clinical trial at National Institutes of Health in Bethesda, Maryland (NCT01500733) has been suspended and is currently under review. This research area of the federal government is one of the most valuable resources for people like me who are left with little options. This means that the additional 17p-deleted leukemia patients, who thought they were new participants in the clinical trial, are temporarily on hold and are not allowed to be on the study until further notice. This does not include me, because I am not a new patient.

Professor Chaya Venkat, my patient advocate, met with the research director of Johnson & Johnson to try to get compassionate use of ibrutinib, since these patients will not live long enough for the drug to be FDA approved. She said she would pay her way to California to meet with the drug company stakeholders. She was denied.

It is also unclear whether untreated 17p-deleted patients (me) will be included in the initial FDA approval. The key is to get enough participants in the trial to have a decent enough sample size to warrant inclusion. Suspension of the trial is a big kick in the stomach. We are 5% of the CLL/SLL cancer population.

NIH will continue to follow patients in this trial and continue to provide the drug as long as the drug company provides the drug to NIH. NIH may continue to monitor patients after the trial.

I have written a letter to President Obama letting him know about the importance of the people who provide funding for the research. What is done here at NIH is of incredible value to patients. This is how medicine is suppose to be.

This posting is about my health update.

BLOOD WORK

The good news is that my white blood count (WBC) has dropped another 25,100, which is heading in the right direction. These are normal: electrolytes, kidney, liver, sodium, potassium, red blood count, hemoglobin (which means I am not anemic), and lungs.

These counts I need to keep an eye on:

Lactate dehydrogenase: slightly higher than normal

Uric acid: slightly higher

Platelet function: slightly lower

I have no eosinophils or basophils in my immune system.

Neutriphils are 9.5%, when they should be 34.0% to 71.1%.

BONE MARROW BIOPSY

According to Dr. Adrian Wiestner in the Hemotology Branch of the National Heart, Lung, and Blood Institute (NHLBI) of NIH, I have a very good reduction of malignant B-cells in my body and the experiment is going as expected. My bone marrow biopsy indicates lymphoid infiltration involving 20-30%, as opposed to 40-50% eight weeks earlier. Before the clinical trial, researchers knew that the drug would diminish the size of lymph nodes and eradicate the malignant cells in a patients’ blood through apoptosis (cell death). They were not sure it would help the bone marrow, in which much of the malignant cells hide out. Well, now we have a new scientific finding! It is working on the bone marrow too!

CD3 shows scattered positive T-cells. CD3 is required for T-cell activation.

CD34 protein has slightly increased to 3-5% positive. This is an important adhesion molecule and is required for T-cells to enter the lymph nodes. It facilitates cell migration.

I also have positive megakaryocytes, which are large bone marrow cells from which mature blood platelets originate.

CT SCAN

According to the CT scan, there is a decrease in adenopathy (swelling or abnormal enlargement of the lymph nodes) since June 20, 2012. In the September 7, 2012 report, there is evidence of lymphadenopathy, but the number and size of the lymph nodes on the neck have “dramatically decreased” when compared to several months prior.

The CT scan indicated degenerative disease in my neck of which I was aware. Prior to beginning the clinical trial, I was going to physical therapy at The Barrow Institute in Scottsdale. The attending physician had looked at my scans and thought he had pulled the wrong medical records when I entered his office. He said that if he had just looked at the medical scans, he would have said that I was a patient who had to immediately go in for spinal surgery. He said he was surprised at how good I looked. Surprise! But this is another story for another day…

MORE GOOD NEWS

In spite of the fact that I have had some minor side effects, such as slight bruising, a little dizziness, and occasional tenderness in my mouth, I have no breathing problems, no high blood pressure, no appetite issues, no mouth ulcers. I have had a little more fatigue the past cycle (even though my blood work does not indicate a reason for the fatigue). I think I was so excited that I was feeling better that I overdid it a little.

FINAL THOUGHTS

The researchers at NIH are pleased with my progress on this experimental drug. It seems to be doing what it is supposed to do. I am about as happy as an “egg-suckin’ dog in the middle of a chicken coop” (my husband Carl’s words).

One thing I understand is that even when my WBC is normalized (between 4,000 to 10,000). I will still be immune compromised since the drug does not cure me. It simply manages the cancer. Medicines that interferes negatively with Ibrutinib are the following: anti-fungal systemic pills, anti-depression medicine, acid reflux medicine, and blood thinners (a BIG no-no).

Being immune compromised also means that I cannot ever have inoculations with live viruses, such as a live shingle shot. I also cannot be around people who have had the live virus shot for about a week to 10 days. That also includes live virus inoculations given to my grandchildren, my dog, and people at the Walgreens Pharmacy.

When you are first diagnosed, you should have a pneumonia shot, which should last about five years. A flu shot should be given every year, even though it may not help a patient who has had leukemia for a while, but it might help a little.

Since the number one cause of death for patients like me is pneumonia, the herd mentality is in place here. If you have been inoculated and protected from whatever diseases and you are in good health, flock around me, because you will protect me from the germy people. LOL.

I personally will be eternally grateful for the opportunity I have been given to participate in the NIH clinical trial with Ibrutinib (PCI-32765), regardless of whether this experimental drug helps me in the long haul or regardless of whether I continue to have access to it. I know I am helping others in the future, who can benefit from the findings of this scientific research.

RANDOM THOUGHTS

Well, it is getting close to my next visit to the National Institutes of Health on the East Coast – more tests, more drugs, and then an informative visit with Dr. Chaya Venkat, the most wonderful patient advocate for leukemia. I will be meeting with her and several others in her living room in Colombia, Maryland. This blog posting is about my random thoughts as I am getting ready for the trip.

CRAP IN MY LIFE

Cancer has a way of making me stop and reflect upon my life journey. I realize that I have gratitude for all my blessings – and I have many. I have also come to terms with the fact that I am oddly respectful of all the negative and painful events in my life that have taught me my life lessons. It wasn’t pretty, but it made me who I am today.

When something that I perceive as negative or painful happens in my life, I ask myself:

“What did that teach me? What am I supposed to learn from this in my life journey? How can I make this event transform me into a better human being? Who do I NOT want to be like?”

Do you remember the children’s song “Sticks and stones will break my bones, but words will never hurt me”? Oddly enough, it is usually the words (or lack of them) that are more painful in our adult life. As we get older, it is not the details of the event that we remember, it is how the person made us feel that lingers in our heart and often still causes pain after all these years.

As I reflect back on my life, there are people I hope will forgive me for my real or perceived bad behavior. There are others that I need to forgive – especially for their angry and cruel words directed at my family or me.

WORDS OF MY MOTHER

My mother was one of the most positive people I have ever known. She did, however, hate hospitals and hated to be alone. So one of the nights I spent with her shortly before she died, we were up all night giggling and laughing about the stories of all the guys she dated before she met my Dad. The oncology nurse barged in the room and sternly pronounced, “If I hear any more noise from you two, I will kick you out of the hospital!” My mother, answered back, “You promise?!?” The nurse then realized what she had just said to a terminally-ill cancer patient.

Sorry folks. Having a positive attitude does not cure cancer, but it certainly makes life a lot more bearable for the patient and his or her family and friends. I am not a Pollyanna, but I certainly know that a negative attitude can make you sicker, and it can make life miserable for everyone who loves you. It is such a waste of limited energy. I have learned to avoid toxic people like the plague, and my life has improved substantially.

I have been thinking lately why I am one of the few people that I know who is not on “happy pills.” One of the reasons I believe is because I have such a demented sense of humor and I can find humor even in the most dire situations. I learned that from my Mom. It is definitely a survival tool and a gift she gave me. I thank her every day for teaching me that.

WORDS OF MY FATHER

When I was a child, I was one of those who learned by observing others. My Dad said that was the Japanese part of me. It saved me a lot of pain.

In my life I witnessed the wrenching pain caused by people being cruel to each other, and I learned the importance of mercy and kindness.

In my life I witnessed people stressed out with anxiety, and I learned that I would rather choose inner peace and meditation.

In my life I witnessed the emotional damage angry people have on others and themselves, and I have learned about the importance of gentleness and self-control.

It is interesting that these are also the words of my father. I thank him everyday for teaching me kindness, inner peace, and gentleness. These were teachings from a man who fought in two wars, was wounded, and had his best Army buddy blown up next to him in the foxhole.

THE ULTIMATE REALITY

You do understand that none of us are leaving this world alive? Whether I am blessed with many more years or I die sooner than I like, that is just how it is. As I have said many times before, I am in a win-win situation. I win if I stay alive for years to enjoy my friends, children, and soon-to-be 11 grandchildren. I know where I am going if I die, so I win when that happens. So I don’t care to waste my time wallowing in the quagmire. I like to get on with my life and be grateful for every second.

I am still a work in progress. There is much to be said about a little imperfection. Some times I need to remind myself that I have many blessings in my life. Some times I need to remind myself that there are people in this world much worse off than me.

A SELF-INDULGING MOMENT

Most of the time my blog updates are very clinical. I become the “pigmy nerd mama” that my son used to tease me about when he was in junior high school. This is because that is how I am best able to cope with the diagnosis of leukemia. My friend Nancy understands this well. I take the scientific approach and report the facts, and that has a way of removing me personally from the reality of the stupid cancer. I become the university researcher – a role I have been quite comfortable with for many years, since I left my life as a creative and art director.

The truth is that several women I personally know who have cancer have died way before their time. And moments when this happens cause me to pause and doubt my mortality… But only for a moment, because I only give myself a moment for the luxury to be self-indulgent, and have my self-pity party. And then I must move on, because life moves on.

One beautiful woman with the sweetest disposition died because she forgot to value her life more than the value of her job. She spent hours working overtime to be loyal to her company, and did not seek out cancer specialists who could have saved her, until it was too late. Her family was and probably still is very angry about this.

Another beautiful woman tried every treatment possible, until her body could take no more. She made the brave decision to stop the chemo treatments and die gracefully. I remember giving her a foot massage when she was in the hospital and we laughed about how hospital rooms should be made into spas where sick people could be pampered and powdered. I still think it is a great idea.

And then there was my beautiful friend, who was dismayed because the steroids she had to take made her gain weight and gave her a chipmunk face. “Like to shop?” I asked her. We spent the afternoon trying on all the stretchy and layered clothing that hide imperfections, and smelling different oils from India. It was a girls’ day out and we had a blast. She died the first day of my cancer treatment when I was at National Institutes of Health back East. I couldn’t figure out why she didn’t return my emails and voicemails. In all the chaos, the women in my cancer support group (including me) were not notified until a month after she died.

My friends and my family have been instrumental in supporting my journey. One of the most delightful comments I got was from my friend Laura who told me that she forgets that I have cancer, because I don’t look or act like I do. She asked me if that was a bad thing. I said “absolutely not.” Because you see for that moment in time when we talk and laugh, I am the La Verne she has always known. I am not “La Verne, who has cancer.”

But there are moments when I need the personal confirmation from friends and family that they understand the severity of the invisible face of cancer. I truly value those people in my life who share their time with me, for time is the most valuable gift anyone can give me.

P.S.: I hope this message was not too much of a downer. Sometimes when I write, it gets it all out on the table, and I can move on. The glass of red wine (which I cannot have with this clinical trial) would have really helped. I did buy a bottle of red Zinfandel to send to Dr. Keating; however. He can enjoy it for me…

P.S.S.: This is an anonymous quote about time I would like to share with you. It is a little corny, but hey, what’s new?

Have you been to the bank?

Imagine there is a bank that credits your account each morning with 86,400. It carries over no balance from day to day. Every evening it deletes whatever part of the balance you failed to use during the day. What would you do? Draw out every cent, of course!
Each of us has such a bank. Its name is TIME.
Every morning, it credits you with 86,400 seconds.
Every night it writes off, as lost, whatever of this you have failed to invest to good purpose.
It carries over no balance. It allows no overdraft.
Each day it opens a new account for you.
Each night it burns the remains of the day.
If you fail to use the day’s deposits, the loss is yours.
There is no going back.
There is no drawing against the “tomorrow”.
You must live in the present on today’s deposits.
Invest it so as to get from it the utmost in health, happiness, and success!
The clock is running.
Make the most of today.
To realize the value of ONE YEAR, ask a student who failed a grade.
To realize the value of ONE MONTH, ask a mother who gave birth to a pre-mature baby.
To realize the value of ONE WEEK, ask the editor of a weekly newspaper.
To realize the value of ONE HOUR, ask the lovers who are waiting to meet.
To realize the value of ONE MINUTE, ask a person who missed the train.
To realize the value of ONE SECOND, ask a person who just avoided an accident.
To realize the value of ONE MILLISECOND, ask the person who won a silver medal in the Olympics.
Treasure every moment that you have! And treasure it more because you shared it with someone special, special enough to spend your time.
And remember that time waits for no one.
Yesterday is history
Tomorrow is mystery
Today is a gift
That’s why it’s called the present!!

Cycle 3, Day 1

UPDATE

Just returned from National Institutes of Health (NIH) in Bethesda, Maryland. I am now beginning my Cycle 3 of my cancer treatment with the experimental drug Ibrutinib (PCI-32765).

LYMPH NODES

According to the CT scan, the largest lymph nodes under my arm pits (there were 15-20 of them) have been reduced to eight enlarged lymph nodes. I had lymph nodes in my abdomen, which are smaller now. The lymph nodes in my neck and clavicle area are also smaller. So this is moving in the right direction. I will receive the full CT scan report in about two weeks.

WHITE BLOOD COUNT (WBC)

In the past month my white blood count has decreased from 135,000 to 110,000. This is good news. Dr. Farooqui told me that the participant who has been in this study the longest has been taking the drug for 8-9 months and he still has not normalized, so don’t be disappointed if I am not. As you recall, normalization of the white blood count is between 3, 500 to 10,000.

Absolute lymphocyte count (ALC) is 104.05 K (normal is 1.18-3.74 K/uL).

BONE MARROW BIOPSY

I will receive the results of the bone marrow biopsy in about two weeks. I will post the results when I get it.

OTHER FINDINGS

My spleen is the same size.

Neutrophils are low at 5.3% (normal = 34-71.1%). Neutrophils are produced in bone marrow and are the body's primary defense against bacterial infection and physiologic stress.

Lymphocytes are 93.8% (normal = 19.3-51.7%).

Monocytes have gotten lower in the past two weeks from 1% to  0.4% (normal = 4.7-12.5%). Time for my B12 shot.

Eosinophils are low at 0.4% (normal = 0.7-5.8%).

These are very helpful in defending the body against parasites.

Eosinophil Absolute is high at 0.44 K/uL (normal = 0.04-0.36 K/uL). Eosinophils become active when you have certain allergic diseases, infections, and other medical conditions, but I do not understand why the Eosinophiils are low and the Eosinophil Absolute count is high. This is a question for my hemotologist/oncologist I see next week.

Basophils are 0 (normal = 0.1-1.2%). Basophils protect the body, killing bacteria and parasites, including external parasites such as ticks. Basophil Absolute is low at 0 (normal = 0.01-0.08 K/uL).

Beta-2-Microglobin is normal at 1.5 mg/L (normal = 0.9-1.7 mg/L).

EXPANDING OUR CLINICAL TRIAL

The last I heard from Dr. Farooqui was that he had met with the drug company on numerous occasions and the stakeholders were NOT going to expand the study. Well the good news is that the drug company changed their mind!

Originally the NIH clinical study had slots for a total of 64 participants (two cohorts -- Cohort A with 32 participants who were over the age of 65, and Cohort B with 32 participants who had 17p deletion, which trumps everything since it is a poor prognosis). The drug company agreed to add six more slots for untreated patients with 17p deletion.

Dr. Farooqui also mentioned that when the drug will get approval (probably in about two years), it will be for relapsed CLL/SLL patients. MD Anderson in Houston and NIH are the sites that are doing studies on untreated 17p deleted patients. More research will have to be done on untreated 17p deleted patients before the drug company approves the drug for them.

Cycle 2, Day 14

PERSONAL MESSAGE

It has taken me three years to finally cry. Believe me, I don’t cry easily. I was afraid to cry before, because I was worried that I would never be able to stop. Now finally something is being done to help me and I am so relieved. I am not good at doing nothing and sitting idly by. These tears are tears of hope.

My friend George asked me what it means when I wrote that I am a person of faith. I told him that I try every day to live my life so that my actions are loving and they speak louder than my words. It doesn’t always work out that way, but I keep trying. Perhaps this quote sums it up better than I can:

“Love the Lord your God with all your heart and with all your soul and with all your mind. This is the first and greatest commandment. And the second is like it: Love your neighbor as yourself.” (Matthew 22:37-39).

And what does this have to do with leukemia? Everything. My faith is my foundation. It is what keeps me grounded in this crazy world with this stupid cancer diagnosis. My faith also makes me accountable. I am reminded of the words of Mahatma Gandhi:

“I like your Christ, I do not like your Christians. Your Christians are so unlike your Christ.”

There is truth to that. So this is something I need to work on every day.

08-22-2012 BLOODWORK (Cycle 2, Day 14)

My bloodwork from August 24^th – two weeks after the 135,000 white blood cell count – shows a decrease to 114,300. This is about two weeks earlier than the average time in the study. Usually it isn’t until Cycle 3 that this occurs. This means that if all goes well, I am on the downhill slide of getting rid of the malignant B-cells that have been targeted by the Ibrutinib. In two weeks my body has gotten rid of about 20,000 B-cells. When you consider that a normal person only has 3,000 to 10,000 B-cells total in their blood, that is pretty amazing.

My lymphocyte count is still high. Lymphocytes, as you recall, are a type of white blood cell in the immune system.

My I-Bili (Indirect Bilirubin) is slightly elevated at 0.7, when it should be 0-0.6 mg/dL. This has to do with liver function.

My monocyte count is 1% and it should be between 2-8%. The low monocyte count is a side effect of illnesses of the bone marrow. This often also indicates deficiencies in vitamins such as folates and B-12. I am still getting my B-12 shot every month and taking those pink vitamins every day. Maybe that is why I at least have 1%.

My segmented Neuts are low. They are 5% when they should be 45-75%. Neutrophils are produced in bone marrow and are the body's primary defense against bacterial infection and physiologic stress.

My red blood count is normal.

My blood glucose level is normal. That is the amount of sugar present in my blood.

My platelet count is 156 (normal range 130-400). When the number of platelets is too low, that means that you will bleed easily. If it is too high, blood clots resulting in a stroke can occur.

The only side effect I have been having is a little dizziness when I wake up in the morning, look up, or move my head suddenly. I close my eyes, hang on to something, and breathe slowly. It goes away quickly, unlike my vertigo episodes. It is very minor in comparison.

ON A GOOD NOTE

Dr. Farooqui and Dr. Wiestner are having me work on an infographic illustrating how this magical drug works. This was my suggestion, mainly because I want to understand this process better, and many participants and their families also want to be better informed. It is a work in progress, because the experts are just now finding out the details of how Ibrutinib works.

Finishing Cycle 1 and on to Cycle 2

I just completed Cycle 1 of my experimental treatment at NIH and “donating” 12 tubes of blood. My white blood count has increased from 69K on Day 1 to 135K on Day 28. One more cycle of climbing – dumping those bad boys from my lymph nodes into my blood – and then I will brace myself for the decent to a normal range, which is 4,500-10,000 white blood cells per microliter (mcL).

Hemoglobin has remained steady at 13. The average for adult women is 12 to 16 gm/dL. Low hemoglobin level means a low red blood count or anemia.

Unlike normal cells, platelets do not have nucleuses. Their lifespan is only twelve days at most. The main purpose of these cells is to form blood clots. Platelets have gone from 150K on Day 1 and continued to rise to 191K. The normal range is 150K to 400K platelets per microliter (mcL), so I am headed in the right direction.

The NIH computer had issues, so I will get my full blood work numbers mailed to me by Susan Soto, the NIH nurse.

I had my first real symptom on Day 27 – a massive leg cramp in my left Tibialis anterior. I had never had a leg cramp there. I was suddenly woken in the middle of the night and did not know which way to pull or push my foot. I finally figured out I needed to push it like I was pointing my toes in ballet. I did my Lamaze breathing and massaged the muscle until the cramp went away. It seemed like it took forever. The doctor did not know if it was caused by the drug or my overdoing it at the gym on the cycle. One teaspoon of mustard is supposed to help, along with eating my bananas for potassium and replenishing my electrolytes. Mercy!

One of my Ibrutinib colleagues suggested a book for me to read called The Emperor of All Maladies: A Biography of Cancer, (2010). It was written by a physician named Siddhartha Mukherjee. I believe I will put it on my wish list. Still reading the bio of Steve Jobs, along with a couple more books on my night stand.

Just started Cycle 2…

How a BTK inhibitor works

In the average healthy adult between 50 and 70 billion cells die each day due to apoptosis (cell death). B-cells, which are lymphocytes, are part of the immune system. When a person gets a cold, these white blood cells increase, attack the germs, and when they have done their job, they die. All it takes is a mistake in one cell that leads to the cloning of white blood cells that do not die. It is just bad luck.

There has been a decade of scientific research supporting the importance of the B-cell receptor signaling pathway in rapidly out-of-control growing B-cells (leukemia). Dr. Michael Keating from Houston’s M.D. Anderson was the first physician to tell Carl and I about the new drug PCI-32765, which was later named “Ibrutinib.” I remember the excitement in his voice when he showed us photos of before and after lymph nodes, and exclaimed, “This is what you need!”

The proteins in my leukemic white blood cells prevent cell death, so they continue to proliferate in my blood, my lymph nodes, and my organs. Because, quite frankly, blood runs through every organ of your body. The white blood cells crowd out healthy red blood cells and platelets and enlarge lymph nodes, which often affect blood circulation. With a weakened immune system, there is constant worry about getting infections.

BTK is an essential kinase (a type of enzyme) in the signaling pathway downstream of the B-cell receptor. The pathway in which BTK is involved turns several protein enzymes that prevent cell death either on or off.

Scientists are hoping that Ibrutinib, which is a BTK inhibitor, will target the pathway and not affect other organs or tissues of the body. When Ibrutinib molecules target and irreversibly bind to the kinase, it will do so for as long as 24 hours, which means I have to take the drug every day until it stops working.

When Ibrutinib is ingested in the body, the leukemic white blood cells in the lymph nodes dump into the blood stream. This causes a temporary increase in white blood count (WBC) for about two months. When the drug blocks BTK, it induces cell death in white blood cells that refuse to die and the cells leave the body.

That, my friend, is how it is theoretically supposed to work.

Since I am experiencing the mutation in which the short arm of chromosome 17, where the gene for p53 resides, is deleted, my B-cells are free of the tumor suppressor. This is not good, and is a poor prognosis. This is what some of my doctors have termed “a true death sentence.”

However, I beg to differ… I do not believe I have been given a death sentence. I believe Ibrutinib has just given me a life sentence.