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June 17, 2020

Living and Thriving with Acute Myeloid Leukemia (AML)

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Featuring Dr. Harry P. Erba, MD, PhD of Duke University Medical Center and his patient Debbie Beavers, in remission.[/vc_column_text][/vc_column][vc_column width=”3/4″][vc_column_text]

Thriving with Acute Myeloid Leukemia

Speaker Dr. Harry Erba, Survivor Debbie Beavers

The median age for AML is 68-70 and an allogeneic transplant is the standard transplant.  However, transplants among those older than 70 years old with AML in the USA precludes possibility of bone marrow transplants due to pre-existing conditions; heart, blood pressure, kidney failure etc.

Our own bone marrow has an amazing responsibility to produce blood which is finite.  Red cells have a life of 3-4 months, platelets 1-week, white cells 1,000 per day before going into tissue to fight infection.

If bone marrow does not continually make blood, the bone marrow failed causing AML.  AML syndrome is common in older people as they live longer allowing mutation to cumulate. Some genetic changes occur with no consequences.  In others there is a silent mutation, yet in others such changes cause the cells to die leading to the advantage for changes to remain in the marrow and not go away.

Different types of changes and classes of enzymes and proteins are responsible for the change.

By aging we all collect mutation in the bone marrow.  A study shows blood donors that do not have cancer including leukemia, 10% over 65 who donated find genetic changes commonly that are commonly found in AML.  These people have 10-fold higher chance of developing cancer.  To complete an analysis there needs to be a complete menu of different genetic changes, not one common factor.  Some of the genetic changes cause the cells to start growing one after another getting out of control filling bone marrow with cancer cells.

FLT3  is when cells replicate too fast.

IDH1, IDH2 gene mutation will lead to blocking of normal development.

BCL-2 mutation involves a family of proteins responsible for cell death that is not working properly so cells are not dying appropriately.

We have made great advances based on those 3 based on genetic changes.

The patient, Deb 69 diagnosed with AML July, 2019.  Genetic analogy critically important, 2 genetic changes IDH1, FLT3 are the more common genetic changes.  Do not occur in isolation usually four in a complicated mixture.  Standard chemo used over the past 40 years, no remission still there 20-25% of the time.  Chemo with drug Midostaurin turns off FLT3.  Midostaurin with chemo previously used on anyone untreated AML with FLT3 genetic changes lead to a better chance of survival. FDA approved Midostaurin in 2018.  Unfortunately, her disease was still there.  Standard chemo with a higher dose of Midostaurin still there. Given the advances, 10 years ago there would be fewer options available.

Debbie was then given Ivosidenib, a drug that turns off IDH1, with mild chemo and went into remission.  She then went through conditioning chemo before her allogeneic transplant.  She was then put back on Ivosidenib.  Genetic analysis is necessary for proper treatment of disease.

The challenges after a transplant remains relapse.

Years ago, 69 there would be no consideration of bone marrow transplant.  This is due to the intensity of chemo and ability to tolerate.  Newer conditioning regimes, with chemo and drugs that are better, transplants are being done on people in late 70s.

65-70 average age 68 were not able to tolerate the intensity of chemo.  In US 2/3 over 65, no treatment for AML two-month life expectancy with an average of six months.

Developments in agents, standard treatment with a drug for BCL2 over expressed proteins show oral drug Venetoclax with low chemo given to patient showed a 75% survivor rate.  Average survival was 15 mo.- Now showing  40-50% still alive beyond this average.

Headway in treatment of older people 75% not 100% average survival 15 months.  Durable responses needed, investigating drugs that target these genetic changes and over expressed proteins that lead to AML, new agents that are not chemotherapy, but targeted w/AML.

Had carpal tunnel surgery.  Was holding my arm up to prevent swelling, was doing a lot of snoring.  Throat was red and swollen so I went to my doctor.  A blood sample was taken, doctor called and wanted another sample.  More samples were taken and was told I would be contacted by the end of the week.  Next day my doctor called and told me I was extremely sick, AML…do not look it up on the internet I was told.  Met with several doctors, including Dr. Erba.

Once we reviewed what was needed, I went for the treatment.  I was truly fortunate as I only had a blocked savory gland, ate lots of lemon drops.  Very little side effects. All who visited had to wear PPP so no infections would arise.  Rigors and tremors more of an inconvenience. Walked each day, no loss of appetite.  Dr. Erba said, use Plan A, if it does not work, Plan B, if that does work, Plan C. After transplant no issues, went well, chemo again after the transplant.  Restrictions after with food and water. As for the medications, not entirely sure what I was on, too many different ones mentioned.

Q and A

Nexavor, after transplant I am taking 400 mg Nexavor after 4 ½ years 200 mg.  Is it a fit as to post transplant surgery?

Common relapse after stem cell transplant. Relapse without increasing side affects that a person may already have been going through transplant.  Nexavor inhibits FLT3 mutation how long for maintaining therapy unknown.  Investigation is still ongoing.

If disease returns, do not really know then to stop.  Recommend with no toxicity to the drug, stay on Nexavor.  Inhibitors to decrease relapse.  Study no transplant, had chemo use PC436, oral pill Azacitidine prolongs survival without increasing relapse with maintenance therapy.  Tolerable previous relapse.

Sixty-four male had AML in 1990, relapse in ’92, bone marrow transplant ’94.  Research looked at relapse, no restriction whether on long term transplant patient.  Possibly relapse down the road.

Risk of relapse after 30 years is unlikely.  Your bone marrow with the bone marrow donator relapse chances are incredibly low.  Maintenance will have side effects and risk of relapse.  No recommendation due to test at this time.  Side effects, endocrine, hypothyroidism, etc., develop different cancers.  Continue with, PSA, monitoring skin for melanoma.

After care transplant, PCP education?

Important long-term communications with PCP regarding stem cell.  There is a higher risk for infection, fever, more serious.  Carry info concerning transplant; endocrinology, osteoporosis, early menopause, increase risk of other cancers, graft versus host disease, etc.  Instruction are usually sent to the PCP after transplant with recommendations for monitoring.

Survivor clinics each center works differently.  Remember how sick you were, physicians and PA deal on a daily basis with persons that are that sick.  Transplant centers are more apt to take concern and will spend the least amount of time with the patient after they are in a safer area of their care.

As positive as you are, what about the tough moments.  You had to be surprised when you were told of AML.

Positive, faith and never felt death was an option.  Husband was a great support.  Caregivers are an essential part for physical and emotional support.  Positive attitude, great support and faith are important.  Get up, get dressed and encourage yourself to move forward.

We acknowledge and thank the following Link Partners for their ongoing support.

• The Leukemia & Lymphoma Society
• Incyte Corporation
• Pharmacyclics & Janssen