ROLE OF THE NURSE NAVIGATOR IN AMYLOIDOSIS CARE (~25 minutes to complete)

Role of the Nurse Navigator in Amyloidosis Care

Speaker: Elizabeth Brandes

Clinical Pearls:

• Multidisciplinary holistic team approach to Amyloidosis is essential to patient care
• The Multidisciplinary team includes nurses, physicians, and advanced practice providers across a wide range of specialites

INTRODUCTION TO DISEASE STATE: AMYLOIDOSIS (~40 minutes to complete)

Lessons in this section:

Basic Concepts, Amyloid Light Chain, Amyloid Transthyretin: 

Speaker: Chris Bell ACNP

TTR Amyloidosis 

Speaker: Cindy Bither RN, MSN, ANP-C, ACNP-C, CHFN

Clinical Pearls

• Amyloid = starching substance, protein found in the body
• Three subtypes of cardiac amyloidosis: AL, ATTR hereditary, and ATTR Wild Type
• Red Flag Symptoms of cardiac amyloidosis: Left Ventricular Hypertrophy seen in echo, EKG with voltage disproportionate to LVH, refractory arrhythmia, Low flow AS, Bilateral carpal tunnel release and/or spinal stenosis, motor/sensory polyneuropathy
• AL – blood dyscrasia of plasma cells, closely related to, but distinct from multiple myeloma
• Untreated, medical survival from the onset of heart failure symptoms is less than 6 months in AL
• TTR is involved in the transport of Thyroxine and Vitamin A and it is produced in the liver
• TTR tetramer breaks apart into monometers that misfold and aggregate together
• TTR Wild Type – develops with age – acquired, where normal TTR destabilize
• Hereditary TTR- due to a genetic mutation in the TTR gene that leads to abnormal TTR protein
• It is important to do SPECT with PYP scan to rule out blood pooling of the dye – mistakenly diagnosing ATTR
• Genetic Counseling is Absolutely critical for the diagnosis of ATTR hereditary – it has consequences for generations

• ATTR hereditary is genetic -Inheritance pattern of mutation: Autosomal Dominant (one of the 2 alleles of the TTR gene carries a mutation)
  All first-degree relatives carry a 50% chance of carrying the mutation and develop the condition
• Homozygous or compound heterozygous – mutation on both alleles
• Certain presentations (phenotypes) are correlated to certain mutations (genotypes) – some could have cardiac presentation, neuro presentation, or both
• Reduced penetrance – not everyone that carries the mutation will develop the disease
• Genetic testing should be done after TTR has been diagnosed
• Genetic testing is recommended even when Mass Spectrometry done on a tissue (biopsy) and it has identified a mutation or not as mass spec can miss mutations and cannot differentiate between heterozygous and homozygous
• Implications of a positive result – subtyping of ATTR, Eligibility for medications or clinical trial participation
• When to start testing is based on age and specific mutation as some present earlier vs later in life
• A positive genetic test does not mean that the patient will go on and develop the disease
• Role of genetic counselor: Explaining test results, discussing psychosocial implications, discussed results with and providing resources
• Pre-symptomatic genetic testing – survival’s guilt, anxiety if positive result, etc
• GINA – Genetic information non-discrimination act – protects against health insurance companies or employers based on a genetic test result but does NOT include life insurance or long- term disability insurance policies – discuss these before genetic testing
• Patient tools – patient handouts
• Mutations are like “misspelling” words in our DNA
• Amyloidosis Support Groups Association and Amyloidosis Research Consortium are important resources for patients and families with Amyloidosis

DIAGNOSIS & MANAGEMENT OF CARDIAC AMYLOIDOSIS (~45 minutes to complete)

Diagnostic Process & Pitfalls

Role of the Nurse Nav Intro & Take-Home Points

Diagnostic Testing for AL

Diagnostic Testing for ATTR

Disease Modifying Therapies

Therapies for AL

Diagnostic Process & Pitfalls Infiltrative Cardiomyopathy: AL Cardiac Amyloidosis 

Speaker: Dr. Ajay Kadakkal, MD FACC

Clinical Pearls
-Amyloidosis should be considered in all patients with increased LV wall thickness on echocardiogram
-Pattern recognition: this helps to identify “red flag” symptoms of amyloidosis
-Echocardiogram, EKG, and clinical history can provide diagnostic clues
-When amyloidosis is suspected, diagnosis starts with serum and urine immunofixation plus serum free light chains to look for a monoclonal gammopathy
-Cardiac MRI provides both diagnostic and prognostic information, based on the presence/absence of late gadolinium enhancement
-When a monoclonal gammopathy is identified, collaboration with hematology is key and patients will need a biopsy of the affected organ to make a diagnosis
-When there is no monoclonal gammopathy, technetium pyrophosphate (PYP) scan can be used for diagnosis if performed correctly
-TTR amyloidosis has an insidious onset, while AL patients tend to “fall off a cliff”
-Genetic counseling and testing are needed for accurate diagnosis of TTR subtype even in patients with a biopsy confirming the diagnosis
-Significant advances in treatment make early diagnosis and treatment essential

SYMPTOM MANAGEMENT & ADVANCED THERAPIES IN CARDIAC AMYLOIDOSIS (~55 minutes to complete)

Speaker: Cynthia Bither CRNP

Clinical Pearls:
-Amyloidosis is a restrictive cardiomyopathy, meaning patients have a narrow window of euvolemia
-Volume management is challenging; daily weights might not present the full picture and edema can be multifactorial
-Traditional GDMT is not used in patients with amyloidosis and beta blockers should be avoided
-Home management is helpful: home blood pressures, visiting nurses, and even home IV diuretics may prevent hospitalizations
-Orthostatic hypotension is common and can be severe
-Medical and nonmedical treatments for orthostatic hypotension can be used: midodrine, support hose, recumbent exercise, small frequent meals
-High risk of stroke in patients with amyloid: consider routine ambulatory monitoring, AC regardless of CHADS-VASC
-Atrial arrhythmias with RVR are poorly tolerated and there is a high risk of recurrence even after ablation
-High risk of sudden cardiac death, esp in patients with documented NSVT; may benefit from ICDs outside of typical guideline recommendations
-Due to advances in treatment, advanced therapies including heart transplant may be an option for patients with cardiac amyloidosis
-Non-cardiac organ involvement and/or frailty may limit use of advanced therapies; multi-disciplinary evaluation is required
-Mechanical circulatory support, including VAD, are rarely successful in patients with cardiac amyloidosis. Inotropes may be beneficial even for patients with advanced disease.
-Palliative care referral is appropriate early in treatment course given the need for symptom management and goals of care

NEUROLOGIC CHANGES WITH AMYLOIDOSIS AND NAVIGATION OF THE MEDICATION COSTS (~30 minutes to complete)

Neuro Amyloidosis Involvement
Speaker: Hannia Ramos

Pharmacologic Therapies
Speaker: Jocelyn McAdoo

Clinical Pearls

• There is only one medication available for cardiac amyloidosis- Tafamidis 
• Patients must have polyneuropathy to qualify for the more aggressive Amyloid medications costs are high.
• Nurse Navigator must be available with these and will be the primary person to coordinate this in the clinic.
• Symptoms and onset of hATTR can vary depending on the specific gene mutation that the patient has been diagnosed with.
• The Nurse Navigator may be the person to whom they are discussing their pain and neuropathy issues with and should be familiar with the symptoms that are most common so they can refer to a neurologist.

Estimated time to complete: 5 minutes

Please complete this overall evaluation to receive your CE credit and Completion Certificate.

Accreditation Statement

The American Association of Heart Failure Nurses (AAHFN) is accredited as a provider of nursing continuing professional development by the American Nurses Credentialing Center's (ANCC) Commission on Accreditation.

Learning Outcome(s) They must be written with observable and measurable outcomes)

<85% of all participants will self-report that by participating in this activity, they are better able to review diagnosis and manage complex cardiac disease-cardiac amyloidosis >

<85% of all participants will self-report that by participating in this activity, they have increased confidence in providing care for the cardiac amyloidosis patient population and in their ability to work with a multidisciplinary team in the management of these patients.>

Content Integrity Expectations

This educational activity must adhere to the following requirements:

- Educational materials that are part of accredited education (such as slides, abstracts, handouts, evaluation mechanisms, or disclosure information) must not contain any marketing produced by or for an ineligible company, including corporate or product logos, trade names, or product group messages.

- All recommendations for patient care in accredited continuing education must be based on current science, evidence, and clinical reasoning, while giving a fair and balanced view of diagnostic and therapeutic options.

- All scientific research referred to, reported, or used in accredited education in support or justification of a patient care recommendation must conform to the generally accepted standards of experimental design, data collection, analysis, and interpretation.

- Evolving topics are presented without advocating for, or promoting, practices that are not, or not yet, adequately based on current science, evidence, and clinical reasoning. Sessions that cover these topics must be clearly identified in the program or agenda.

- Accredited education cannot include unscientific approaches to diagnosis or therapy, or education that promotes recommendations, treatment, or manners of practicing healthcare that are determined to have risks or dangers that outweigh the benefits or are known to be ineffective in the treatment of patients.

- Faculty (speakers or authors) must not actively promote or sell products or services that serve their professional or financial interests during accredited education.

- Only relevant relationships are disclosed to learners prior to the activity.

Contact Hours

Attendees may earn (up to) 3.00 contact hours by attending this activity and completing the evaluation. 

Claiming Contact Hours

Attendees can earn one nursing contact hour for every hour of educational time. Learners must complete an evaluation to receive contact hours. The evaluation is available after the activity.

Disclosure of Relevant Financial Relationships

The individuals in control of content for this activity did not have any relevant relationships with ANCC- defined ineligible companies to disclose, unless listed below. All relevant relationships were mitigated prior to the start of the activity according to The Standards for Integrity and Independence in Accredited Continuing Education.

Jen Fox, CRNP
Alnylam-speaker board
Johnson and Johnson speaker board
Bristol Myers Scribb - speaker board
Merck-Speaker board

Rachel Barish, ANP-BC, AACC, CCK
Merck, CVS, United Health Group, and Johnson & Johnson: stock ownership
Astra Zeneca: honorarium for speaking

Chris Bell, NP
Alnylam -- Speakers' Bureau - honoraria
NovoNordisk --Ad Board - honoraria
BMS -- Ad Board - honoraria
ScPharma-- Ad Board - honoraria

Disclosure of Commercial Support

AAHFN did not receive commercial support for this program.