One of the nursing home negligence cases my office is currently investigating involves the death of a man related to complications related to amyloidosis. Turns out, the man was suffering from an advanced bed sore on his coccyx (also called pressure ulcers, pressure sores or decubitus ulcers ) that had progressed so far that the bone in the area had become infected (osteomyelitis).
Elderly nursing home residents, especially those suffering from other diseases such as bed sores, have a higher risk of developing amyloidosis, a disease which can damage various tissues and organs. This can cause dangerous complications in residents who are already weak from advanced age or underlying disease.
Amyloidosis is a group of diseases caused by abnormal deposits of amyloid protein (usually produced by cells in bone marrow) in the body’s tissues and organs. The disease frequently affects the heart, kidneys, liver, spleen, nervous system, and gastrointestinal tract. The amyloid protein can deposit in a localized area (localized amyloidosis) or affect tissues throughout the body (systemic amyloidosis). Amyloidosis is diagnosed for testing for the amyloid protein in a biopsy of involved tissue.
Systemic amyloidosis is classified into three major types:
- Primary (AL) amyloidosis
- Secondary (AA) amyloidosis
- Hereditary or Familial (ATTR) amyloidosis
Primary amyloidosis, the most common form of amyloidosis, occurs when a plasma cell in the bone marrow spontaneously overproduces a particular protein portion of an antibody. AL can affect many areas (heart, kidneys, liver, spleen, nerves, intestines, skin, tongue, blood vessels) and can occur with bone marrow cancer (bone marrow cancer), but is a disease entity of its own.
Secondary amyloidosis occurs as a result of another illness (multiple myeloma, chronic infections including tuberculosis and osteomyelitis, or chronic inflammatory diseases including rheumatoid arthritis and ankylosing spondylitis). It usually affects the kidneys, spleen, liver, and lymph nodes. Treatment for AA is usually treating the underlying illness.
Familial amyloidosis is a rare form of inherited amyloidosis that is an inherited autosomal dominant disease (meaning that the offspring of a person with the condition has a 50% chance of inheriting it). ATTR usually affects the liver, nerves, heart, and kidneys.
The signs and symptoms of amyloidosis depend on the tissues or organs affected. The symptoms result from abnormal functioning of the organs and tissues involved. They may include:
- Swelling of ankles and legs
- Numbness or tingling in hands or feet
- Severe fatigue
- Significant weight loss
- Loss of appetite
- Shortness of breath
- Irregular heartbeat
- Diarrhea or constipation
- Protein in urine
- Feeling full quickly
- Enlarged tongue
- Difficulty swallowing
- Skin changes (thickening or easy bruising)
- Purplish patches around the eyes
Risk factors for amyloidosis including:
- Age – older than 65
- Other diseases – having chronic infections or inflammatory disease or osteomyelitis
- Family history – history of amyloidosis
- Kidney dialysis – large, abnormal proteins can build up in the blood
The severity of the disease depends on which organs and tissues are affected. The disease can result in dangerous complications including kidney damage, heart damage, and nervous system damage. Kidney damage can result when amyloidosis affects the kidneys. Kidney problems frequently translate to damage with the bodied blood filtering system, allowing protein to leak from the blood into the urine. This may result in kidney failure.
Heart damage can also occur when amyloidosis affects the heart. This reduces the heart’s ability to fill with blood between heartbeats, meaning less blood is pumped with each beat. Nervous system damage can also occur when amyloid protein deposits affect the nerves. This can result in numbness, tingling, or difficulty controlling blood pressure.
There is no cure for amyloidosis, but treatment may help manage the signs and symptoms of the disease, limit further production of amyloid protein, and treat any underlying disease. Several treatments of primary amyloidosis are being studied including medicine and peripheral blood stem cell transplantation.
Nursing home residents suffering from painful pressure sores are at an increased risk for amyloidosis. Should a pressure sore patient develop amyloidosis, they run the risk of organ tissue damage and increased risk of amyloid protein deposits. Also, paraplegic patients suffering from sepsis as a result of pressure sores also face complications stemming from amyloidosis.
Mayo Clinic: Amyloidosis
Boston University: Amyloid Treatment and Research Program
Pressure sores: aetiology, treatment and prevention (Colin Torrance)