aging in marfan and related conditions syndrome and aging.pdf · •research is providing insights...
TRANSCRIPT
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Aging in Marfan and Related Conditions
Juan Bowen, M.D.
Mayo Clinic
Rochester, MN
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59 Year Old Woman With Marfan Syndrome
• Diagnosis in young adulthood
• Age 53
• Family history aortic dissection
• Aortic root 44 mm
• Mitral valve prolapse with mild mitral regurgitation
• Valve-sparing aortic root repair done
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New Problem: Unsteady Gait and Imbalance
• Problems identified
• Lumbar dural ectasia
• Degenerative hip arthritis
• Peripheral neuropathy
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The Future for Ms. H.
• Health issues related to aging.
• Health issues related to Marfan syndrome.
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4-5 chronic
conditions
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0
20
40
60
80
100
0 20 40 60 80 100
Age
Su
rviv
ing
(%
)
Life Expectancy in U.S.
2003
1990
1950
1900
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Marfan Syndrome Life Expectancy
• 1972 – median survival 48 years
• 1993 – median survival 72 years
•Why?
• Basic and clinical research leading to better
diagnosis and management.
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Major Causes of Death in U.S.
• Cardiovascular disease
• Stroke
• Heart attack
• Cancer
• Other
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Health Behaviors Which Reduce Life Expectancy
• Tobacco use
• Alcohol use
• Obesity
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Normal Aging
• Normal aging
• Decreased reserve
• Characteristic changes in body systems
• Marfan syndrome/related disorder
• Specific issues
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Quality of Life Issues
• Mobility
• Special senses
• Pain
• Fatigue
• Depression
• Anxiety
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Body Systems
• Cardiovascular
• Respiratory
• Kidney
• Musculoskeletal
• Ocular
• Gastrointestinal
• Endocrine
• Neurologic
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Aging of the Cardiovascular System
• Hypertension
• Heart valves
• Coronary arteries
• Conduction system
• Heart muscle
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MFS/RD Cardiovascular System
• Aorta
• Heart valves
• Heart muscle
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Preventive aortic repair
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Aging of the Kidneys
• Expected decline in kidney function
• Hypertension
• Diabetes
• Toxicity from medications
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MFS/RD Kidneys
• Benign kidney cysts
• Kidney damage
• Hypertension
• Diabetes
• Vascular disease
• Medications
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Aging of the Eye
• Cataract
• Glaucoma
• Macular degeneration
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MFS/RD Eye
• Myopia
• Retinal tears
• Lens dislocation
• Glaucoma
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Iridodonesis
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Aging of the Musculoskeletal System
• Degenerative arthritis
• Spine
• Hips and knees
• Decreased muscle mass
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MFS/RD Musculoskeletal
• Spine
• Scoliosis
• Chest wall
• Dural ectasia
• Joints
• Accelerated degenerative arthritis
• Muscles
• Myopathy
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Aging of the Respiratory System
• Chest wall and lung function
• Asthma and emphysema
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MFS/RD Respiratory
• Chest wall effects on lung function
• Sleep apnea
• Pneumothorax
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Aging of the Nervous System
• Imbalance
• Parkinson's disease
• Peripheral nerves
• Cognitive function
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Cognitive Function
• Attention, processing speed, and episodic memory decline in normal aging
• An active and engaged lifestyle early in life is protective
• Continued physical activity is protective
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MFS/RD Nervous System
• Central nervous system
• No known changes
• Peripheral nervous system
• Effects from dural ectasia
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Aging of the Endocrine System
• Type 2 diabetes
• Thyroid
• Osteoporosis
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MFS/RD Endocrine
• Central obesity and risk of diabetes
• Osteoporosis
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Variations in Fat Distribution
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Bone Density Measurement
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Aging of the Gastroinestinal System
• Acid reflux
• Motility disorders
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MFS/RD Gastrointestinal System
• Acid reflux
• Motility disorders
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Gastrointestinal
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Aging of the Hematologic System
• Cell lines
• Red blood cells
• White blood cells
• Platelets
• Most changes in blood counts are reactive and not primary bone marrow diseases
• Bone marrow malignancies
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MFS/RD Hematologic System
• Eosinophilic disorders in LDS
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Aging and Mental Health
• Depression
• Anxiety
• Chronic pain
• Loneliness
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Chronic Pain
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MFS/RD Mental Health
• Depression
• Anxiety
• Chronic pain
• Disability
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Medical Care
• Self care
• Primary care
• Specialty care
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www.marfan.org
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Medications
• Older people take 2 – 9 medications
• Issues
• Altered drug processing
• Drug-drug interactions
• Drug-disease interactions
• Supplements
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Decisions in Medical Care
• Evidence based
• Practice guidelines
• Clinical judgment
• Collaborative
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Primary
Care
Specialty
Care
Psycho-
social
Pain -
Rehab
Spiritual
Team Care
Personal
Behaviors
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Function
Quality of
Life
Access
Resources
Palliative
Care
Spiritual
Geriatric Care
Social
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©2012
MFMER |
3209410-60
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Take Home Points
• Research is providing insights into aging
• Leading causes of death CV disease and cancer
• Life expectancy in Marfan syndrome is now near normal.
• Everyone needs team-based primary care appropriate for the stage of life
• There are special concerns with Marfan syndrome and aging