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Amyloidosis Nursing Guide

Content created by: Emily R. van den Berg, MSN, APRN, ACNPC-AG, CCRN

Overview: Amyloidosis

This course is intended as a Quick Reference for Amyloidosis and will cover an overview and nursing considerations utilizing the nursing process.

Etiology and Epidemiology

Proteins are naturally occurring within the body and have many different functions. When proteins are present in extreme numbers or have become misfolded and are able to alter or interrupt tissue function, the condition is known as amyloidosis.

Misfolding, a process stemming from both natural variations and inflammation, is the more likely contributor, and credited with causing most of the cases. The causative proteins can be either localized, where they originate from the same site where they are deposited, or systemic, where proteins produced at one site are deposited throughout the body. Additionally, there are multiple ways in which the generation of these amyloids is caused, including mutations, errors in DNA coding processes, infection, and others still being investigated. To provide individuals with amyloidosis with the best possible care and prognosis, it is important to plan interventions based on the type of disorder (localized or systemic) as well as the fibril protein involved (Holmes, 2021).

Types of amyloidosis can be distinguished based on where the misfolded proteins accumulate in the body and whether these proteins are systemic, hereditary, or localized. Each of the following subgroups and protein types causes different clinical signs and symptoms, which can surface in the individual, or throughout a family unit (Holmes, 2021):

  • Systemic amyloidosis includes types affecting:
    • Immunoglobulin light chains
    • Transthyretin
    • A amyloidosis
    • Beta2-microglobulin
    • Immunoglobulin heavy chains
  • Hereditary amyloidosis includes:
    • Fibrinogen alpha chains
    • Apolipoprotein AI
    • Lysozymes
  • Central Nervous System amyloidosis includes:
    • Beta protein precursors
    • Prion proteins
    • Cystatin C
    • ABri precursor protein
    • ADan precursor protein
  • Ocular amyloidosis includes the following proteins:
    • Gelsolin
    • Lactoferrin
    • Keratoepithelin
  • Localized amyloidosis includes:
    • Calcitonin
    • Amylin
    • Atrial natriuretic factor
    • Prolactin
    • Keratin
    • Medin

The most common systemic amyloidosis is A amyloidosis (also known as “AA”), which can occur within inflammatory disorders, infections, and sometimes within certain cancers. It can be associated with the following, more well-known disorders:

  • Rheumatoid arthritis (RA)
  • Alzheimer disease
  • Multiple myeloma
  • Psoriatic arthritis
  • Familial Mediterranean fever
  • Crohn’s disease
  • Osteomyelitis
  • Tuberculosis
  • Hodgkin and non-Hodgkin lymphoma

Despite being systemic, it most often affects only a few organs, namely the spleen, liver, and kidneys (Holmes, 2021).

Since amyloidosis can be both hereditary and vector-borne, it is important to differentiate risk factors from routes of infection. Risk factors are traits throughout a community which may or may not be altered and can make one particularly susceptible to disease. Routes of infection are ways in which the vector carrying a virus or bacteria can enter the system and transmit the infection. In the case of amyloidosis, risk factors which include hereditary traits may not be altered as they affect specific proteins. For example, in hereditary renal amyloidosis, proteins which make up the kidneys are predominantly affected, but may also occur in other areas, causing additional issues (Holmes, 2021).

Risk factors for amyloidosis (Holmes, 2021):

  • Familial history of Alzheimer disease, dementia, or prion-based disorders
  • Exposure to infectious sources of prion-based disorders
    • (e.g., consuming meat sourced from cows infected with prions)
  • History of consumption of high quantities of high-cholesterol foods
  • History of chronic inflammation

Diagnosis

To correctly diagnose an amyloidosis, both the clinical signs and symptoms as well as the specific protein, the protein’s precursor, and the gene’s location on the individual’s DNA must be considered. Additionally, non-hereditary mutation and conglomeration of proteins, for example in the case of atherosclerosis, can also impact an already-existing amyloidosis (Holmes, 2021).

Management

Depending on the type of protein involved and the extent of the disorder, medication management is possible. For example, two medications have been developed to assist individuals with symptoms based on symptoms such as cardiomyopathy and neuropathy. These medications, tafamidis (Vyndamax®) and patisiran (Onpattro®) are based on targeting proteins within the DNA that cause hereditary-type amyloidosis (Holmes, 2021).

Additional medication therapies are available for amyloidosis with symptoms mimicking RA. In these cases, disease-modifying anti-rheumatic drugs (DMARDs) may also be effective in lessening the symptoms of amyloidosis (Holmes, 2021).

For individuals suffering from amyloidosis, successful management is based on improvements in organ involvement over time rather than full removal of the offending proteins (Holmes, 2021).

Nursing Considerations

Use the nursing process to develop a plan of care for individuals. The nursing assessment (with common findings listed), diagnosis, interventions, expected outcomes, and education for individuals with amyloidosis are listed below.

Assessment

  • Signs and Symptoms (Alnylam Pharmaceuticals, Inc., 2020):
    • Dizziness upon standing
    • Severe constipation
    • Sexual dysfunction
    • Sweating
    • Unintended weight loss
    • Fatigue
    • Numbness and tingling in extremities
    • Weakness
    • Shortness of breath
    • Lower extremity edema
    • Glaucoma
    • Vision problems, including blurred vision or floaters
    • Dementia
  • Past Medical History
    • Symptoms of nerve damage
    • Symptoms of heart failure
    • Symptoms of dementia
    • Vision issues
    • Gastrointestinal issues
  • Laboratory Values (to identify subtypes) (Holmes, 2021)
    • Immunocytochemical studies
    • Biopsy of subcutaneous or endocardial tissue
  • Imaging Studies (to detect the extent of damage and prognosis) (Holmes, 2021)
    • Magnetic Resonance Imaging (MRI) for detection of cardiac amyloidosis
    • Scintigraphy
    • Positron emission tomography (PET) scan
    • Radiography of involved joints

Nursing Diagnosis/Risk For

  • Decreased cardiac output related to:
    • Alterations in cardiac rhythm as evidenced by presence of palpitations or symptoms of cardiogenic shock
    • Alterations in vasculature as evidenced by presence of lower extremity edema
  • Risk of infection related to:
    • Increased incidence of urinary tract infections (UTIs) as evidenced by bacteria in urine and symptoms such as burning and frequency of urination
    • Decreased physical activity level as evidenced by fatigue and neuropathy in extremities
    • Decreased LOC as evidenced by increase in dementia symptoms or stroke-like symptoms
  • Constipation related to:
    • Nerve damage as evidenced by loss of sensation of bowel movement
    • Impaired thirst or hunger sensations as evidenced by decreased level of consciousness or increased dementia symptoms
    • Altered mobility as evidenced by decreased use of lower extremities

Interventions

  • Monitor heart rate and rhythm (Holmes, 2021).
    • Individuals with amyloidosis may have symptoms of heart failure or acute coronary events.
    • Determine necessity of diuretic intervention to assist systemic perfusion.
    • Determine necessity of medical intervention for rhythm changes.
  • Perform reorientation as needed (Holmes, 2021).
    • Due to potential cognitive impairments, individuals may need reorientation in acute phases of this disorder or if they begin to experience stroke-like symptoms.
  • Perform imaging studies to direct medical interventions (Holmes, 2021).
    • Radiography
    • MRI
    • Scintography
    • CT
  • Perform laboratory testing, as needed, to direct medical interventions (Holmes, 2021).
    • Immunocytochemical studies
    • Biopsy of subcutaneous fat
    • Biopsy of endocardial tissue
  • Monitor the following (Holmes, 2021):
    • Level of consciousness
    • Heart rate and rhythm
    • Vital signs
    • Intake and output
    • Safety
    • Medication administration
    • Mobility
    • Vision
    • Neurologic signs and symptoms

Expected Outcomes

  • Maintain or increase level of mobility
  • Maintain neurologic function
  • Maintain stable cardiac rate, rhythm, and output
  • Prevent infection
  • Prevent neuropathic compromise

Individual/Caregiver Education

  • Importance of recording sudden change in cardiac or neurological symptoms
  • Exercises that can assist with neuropathic symptoms
  • Importance of maintaining mobility
  • Methods to assist in preventing UTIs and other infections
  • Weight control
  • Diet modifications to lessen fat and cholesterol intake
  • Importance of reporting sudden changes in weight
  • Medication adherence
  • Acute coronary syndrome interventions (e.g., defibrillation, AED use, etc.)
  • When to call emergency services (e.g., in event of stroke-like symptoms or cardiac symptoms)

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Additional Information

Content Release Date 

4/1/2022

Content Expiration

12/31/2024

Course Contributor

The content for this course was created by Emily R. van den Berg, MSN, ACNPC-AG, CCRN.

She has been a nurse for 5 years and was educated and trained in both Florida and Alabama. She earned her Bachelor’s of Science degree in Nursing and began critical care training after completion, earning her CCRN and then continuing to complete her Master’s of Science in Nursing with a concentration in Adult-Gerontology Acute Care Nurse Practitioner Studies. Her clinical expertise is in surgical patients, and she most recently worked as a Registered Nurse in a Surgical Intensive Care Unit prior to starting her career as a Nurse Practitioner.

Resources

References

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