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!
Tiona RN
Concepts Covered:
- Studying
- Medication Administration
- Adult
- Emergency Care of the Cardiac Patient
- Intraoperative Nursing
- Microbiology
- Cardiac Disorders
- Vascular Disorders
- Nervous System
- Upper GI Disorders
- Central Nervous System Disorders – Brain
- Immunological Disorders
- Fundamentals of Emergency Nursing
- Dosage Calculations
- Understanding Society
- Circulatory System
- Concepts of Pharmacology
- Hematologic Disorders
- Newborn Care
- Adulthood Growth and Development
- Disorders of Pancreas
- Postoperative Nursing
- Pregnancy Risks
- Neurological
- Postpartum Complications
- Noninfectious Respiratory Disorder
- Peripheral Nervous System Disorders
- Learning Pharmacology
- Prenatal Concepts
- Tissues and Glands
- Developmental Considerations
- Factors Influencing Community Health
- Childhood Growth and Development
- Prenatal and Neonatal Growth and Development
- Developmental Theories
- Basic
- Neonatal
- Pediatric
- Gastrointestinal
- Newborn Complications
- Labor Complications
- Fetal Development
- Terminology
- Labor and Delivery
- Postpartum Care
- Communication
- Basics of Mathematics
- Statistics
- Basics of Sociology
- Cardiovascular
- Shock
- Shock
- Disorders of the Posterior Pituitary Gland
- Endocrine
- Disorders of the Thyroid & Parathyroid Glands
- Liver & Gallbladder Disorders
- Lower GI Disorders
- Respiratory
- Delegation
- Perioperative Nursing Roles
- Acute & Chronic Renal Disorders
- Respiratory Emergencies
- Disorders of the Adrenal Gland
- Documentation and Communication
- Preoperative Nursing
- Legal and Ethical Issues
- Oncology Disorders
- Female Reproductive Disorders
- Musculoskeletal Trauma
- Renal Disorders
- Male Reproductive Disorders
- Sexually Transmitted Infections
- Infectious Respiratory Disorder
- Integumentary Disorders
- Emergency Care of the Trauma Patient
- Urinary Disorders
- Musculoskeletal Disorders
- EENT Disorders
- Neurological Emergencies
- Disorders of Thermoregulation
- Neurological Trauma
- Basics of NCLEX
- Integumentary Important Points
- Multisystem
- Test Taking Strategies
- Urinary System
- Emergency Care of the Neurological Patient
- Central Nervous System Disorders – Spinal Cord
- Respiratory System
- Emergency Care of the Respiratory Patient
- Cognitive Disorders
- Anxiety Disorders
- Depressive Disorders
- Trauma-Stress Disorders
- Substance Abuse Disorders
- Bipolar Disorders
- Psychotic Disorders
- Concepts of Mental Health
- Eating Disorders
- Personality Disorders
- Health & Stress
- Psychological Emergencies
- Somatoform Disorders
- Prioritization
- Community Health Overview
- Gastrointestinal Disorders
- Integumentary Disorders
- Respiratory Disorders
- Neurologic and Cognitive Disorders
- Renal and Urinary Disorders
- Infectious Disease Disorders
- EENT Disorders
- Hematologic Disorders
- Cardiovascular Disorders
- Musculoskeletal Disorders
- Endocrine and Metabolic Disorders
- Oncologic Disorders
- Behavior
- Emotions and Motivation
- Growth & Development
- Intelligence and Language
- Psychological Disorders
- State of Consciousness
- Note Taking
- Concepts of Population Health
- Basics of Human Biology