Marfan Syndrome
Included In This Lesson
Study Tools For Marfan Syndrome
Outline
Overview
- Autosomal Dominant Genetic disorder
- Causes abnormalities in connective tissue, including musculoskeletal, cardiovascular, and integumentary system.
Nursing Points
General
- Defect in body’s production of fibrillin, a protein found in connective tissue
Assessment
- MSK
- Tall, thin body
- Disproportionately long arms, legs, fingers, and toes
- Chest deformities
- Joint hypermobility
- Scoliosis
- Positive wrist sign
- Cardiovascular system
- Mitral Valve Prolapse
- Aortic Enlargement
- Aortic aneurysm
- Vision problems
- Myopia
- Glaucoma
- Stretch marks (shoulders, hips, lower back)
Therapeutic Management
- No cure
- Monitor cardiovascular status closely
- May need surgery
- Valve replacements
- Prophylactic antibiotics should be taken prior to dental work or invasive procedures
- Medications
- Beta Blockers
- Reduce cardiac afterload to to reduce stress on aorta
- Beta Blockers
- May need surgery
- Monitor scoliosis
- Surgical intervention likely
Nursing Concepts
- Mobility
- Human Development
Patient Education
- Avoid sports that put a lot of stress on the heart and joints
- Long-distance running, football, scuba diving
- Preventative measures for endocarditis
- Physical or occupational therapy may be required
- Educational topics for scoliosis
[lesson-linker lesson=”221520″ background=”white”]
ADPIE Related Lessons
Related Nursing Process (ADPIE) Lessons for Marfan Syndrome
Transcript
Hey guys, In this lesson we are going to go over the basics about Marfan Syndrome.
Marfan Syndrome is a genetic disorder that causes abnormalities in connective tissue. Essentially there is a mutation on something called the fibrillin gene. This gene plays a really important role in forming connective tissue. Without it patients have problems with their musculoskeletal system, their cardiac system the eyes, and skin.
I made this table so you can quickly look at the various symptoms. Probably the most noticeable symptom is a tall, thin body with very long arms, legs and fingers. A positive wrist sign, which you can see in the photo here demonstrates how long their limbs and fingers are. Chest deformities are common and their joints are hypermobile. They are also very prone to having severe scoliosis.
In the cardiac system the major issues are the valves and in the aorta. Mitral valve prolapse, aortic enlargement and aortic aneurysm. The problems with the aorta increase their risk for having an aortic dissection, which is a tear in the aorta that is often fatal.
Stretch marks are common on the skin and in the eyes the lens can be dislocated. Nearsightedness is common and so is glaucoma.
The top priority for management is to keep a very close eye on the child’s cardiac status. They may need a valve replacement and they need to be aware of signs of an aortic dissection (the most distinct symptom is sudden severe chest pain that feels like tearing or ripping).
To reduce the risk of damage to the aorta these patients are often prescribed a beta blocker. This helps by reducing cardiac after load so there is less pressure on the aorta.
Another important part of their management is to treat the scoliosis. Again, they usually have severe scoliosis so surgery is usually needed.
Most people with Marfan Syndrome can life a full life with a normal life expectancy so long as their cardiac function is very carefully monitored and efforts are put in place to minimize stress on the aorta. One big part of this prevention is making sure that these kids avoid activities that will strain their heart and joints.
Your priority nursing concepts for a patient with Marfan Syndrome are mobility and human development.
Okay, lets to over your key points for this lesson. Marfan syndrome is a genetic disorder that affects connective tissue. It primarily causes problems with the musculoskeletal system, cardiac system, eyes and skin.
For the cardiac system it can cause mitral valve prolapse and there’s an increased risk for aortic aneurysm and aortic dissection. Beta blockers are commonly prescribed to reduce stress on the aorta.
They will likely need surgical treatment for scoliosis.
Patient education is super important so they know to avoid activities that will put stress on the heart and joints.
That’s it for our lesson on Marfan Syndrome. Make sure you check out all the resources attached to this lesson. Now, go out and be your best self today. Happy Nursing!
NCLEX
Concepts Covered:
- Circulatory System
- Emergency Care of the Cardiac Patient
- Cardiac Disorders
- Cardiovascular
- Shock
- Shock
- Disorders of the Posterior Pituitary Gland
- Endocrine
- Disorders of Pancreas
- Disorders of the Thyroid & Parathyroid Glands
- Hematology
- Gastrointestinal
- Upper GI Disorders
- Liver & Gallbladder Disorders
- Newborn Complications
- Lower GI Disorders
- Multisystem
- Neurological
- Nervous System
- Central Nervous System Disorders – Brain
- Renal
- Respiratory
- Urinary System
- Respiratory System
- Noninfectious Respiratory Disorder
- Test Taking Strategies
- Note Taking
- Basics of NCLEX
- Prefixes
- Suffixes
- Medication Administration
- Gastrointestinal Disorders
- Respiratory Disorders
- Pregnancy Risks
- Labor Complications
- Hematologic Disorders
- Fundamentals of Emergency Nursing
- Factors Influencing Community Health
- Delegation
- Perioperative Nursing Roles
- EENT Disorders
- Basics of Chemistry
- Adult
- Emergency Care of the Neurological Patient
- Acute & Chronic Renal Disorders
- Emergency Care of the Respiratory Patient
- Respiratory Emergencies
- Studying
- Substance Abuse Disorders
- Disorders of the Adrenal Gland
- Behavior
- Documentation and Communication
- Preoperative Nursing
- Endocrine System
- Legal and Ethical Issues
- Communication
- Understanding Society
- Immunological Disorders
- Infectious Disease Disorders
- Oncology Disorders
- Female Reproductive Disorders
- Fetal Development
- Terminology
- Anxiety Disorders
- Cognitive Disorders
- Musculoskeletal Trauma
- Intraoperative Nursing
- Tissues and Glands
- Vascular Disorders
- Renal Disorders
- Eating Disorders
- Prenatal Concepts
- Microbiology
- Male Reproductive Disorders
- Sexually Transmitted Infections
- Infectious Respiratory Disorder
- Depressive Disorders
- Personality Disorders
- Psychotic Disorders
- Trauma-Stress Disorders
- Peripheral Nervous System Disorders
- Integumentary Disorders
- Neurologic and Cognitive Disorders
- Integumentary Disorders
- Newborn Care
- Basics of Mathematics
- Statistics
- Labor and Delivery
- Proteins
- Emergency Care of the Trauma Patient
- Hematologic System
- Hematologic Disorders
- Developmental Considerations
- Skeletal System
- Digestive System
- Urinary Disorders
- Postpartum Care
- Basic
- Musculoskeletal Disorders
- Bipolar Disorders
- Metabolism
- Cardiovascular Disorders
- Concepts of Population Health
- Musculoskeletal Disorders
- EENT Disorders
- Postpartum Complications
- Basics of Human Biology
- Postoperative Nursing
- Neurological Emergencies
- Prioritization
- Disorders of Thermoregulation
- Writing
- Community Health Overview
- Dosage Calculations
- Neurological Trauma
- Concepts of Mental Health
- Health & Stress
- Endocrine and Metabolic Disorders
- Childhood Growth and Development
- Prenatal and Neonatal Growth and Development
- Concepts of Pharmacology
- Integumentary Important Points
- Emotions and Motivation
- Renal and Urinary Disorders
- Developmental Theories
- Reproductive System
- Adulthood Growth and Development
- Psychological Emergencies
- Growth & Development
- Basics of Sociology
- Somatoform Disorders
- Reading
- Intelligence and Language
- Oncologic Disorders
- Med Term Basic
- Med Term Whole
- Central Nervous System Disorders – Spinal Cord
- Muscular System
- Neonatal
- Learning Pharmacology
- Pediatric
- Psychological Disorders
- State of Consciousness
- Sensory System