Nursing Care Plan (NCP) for Marfan Syndrome

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Outline

Pathophysiology

Marfan syndrome is a genetic condition in which the proteins that make up the connective tissue that supports the heart, blood vessels and other vital organs is weakened.  Most people with this condition have heart and blood vessel problems, often resulting in aortic aneurysm or valve dysfunction. Patients with Marfan syndrome have tall, thin frames, long legs, fingers and hands and may have problems with vision.

Etiology

Marfan syndrome is caused by a mutation in the gene that controls how the body makes fibrillin-1, which is a protein that helps make up the connective tissue in the body. The mutation of this gene causes the body to produce more of a growth protein than fibrillin-1 creating weaker connective tissue that supports the heart, blood vessels and vital organs. The mutated gene is most commonly passed from a parent to child, but research has found that in about 25% of cases the new mutation occurs spontaneously.

Desired Outcome

Patient will have optimal cardiac output with adequate tissue perfusion. Patient will remain free from injury.

Marfan Syndrome Nursing Care Plan

Subjective Data:

  • Difficulty seeing objects at a distance (nearsightedness)
  • Foot pain
  • Back pain

Objective Data:

  • Disproportionately long arms, legs and fingers
  • Tall and thin body type
  • Curved spine (scoliosis)
  • Flexible joints
  • Flat feet
  • Crowded teeth
  • Stretch marks on skin (not related to weight changes)
  • Heart murmur
  • Retracted or protruding sternum

Nursing Interventions and Rationales

  • Perform complete physical assessment

 

Get a baseline of data, note retractions or protrusion of sternum.

 

  • Assess and monitor vital signs

 

Get a baseline and monitor for elevated BP and heart rate that may indicate aortic injury or damage.

 

  • Auscultate heart and lung sounds

 

Listen for murmurs, leaky valves and abnormal breath sounds to determine progression of condition.

 

  • Obtain 12-lead EKG and echocardiogram as appropriate

 

Monitor for signs of cardiac involvement, disease progression and determine cardiac function.

 

  • Assess vision using Snellen chart

 

Patients often have extreme nearsightedness. Assess for degree of vision impairment and make recommendations for ophthalmologist.

 

  • Monitor x-rays, CT, MRI

 

Monitor for bone and joint deformities or malformations and monitor for spine curvature (scoliosis) to determine if braces, splints or other appliances are necessary.

 

  • Assess ROM and perform ROM exercises (AROM, PROM)

 

Assess and monitor flexibility and range of motion as exercises are important for maintaining strength and mobility.

 

  • Cluster care and provide rest periods

 

Patients may have fatigue or an intolerance to activity due to medications or cardiac involvement of the condition. Provide periods of rest to promote normalized blood pressure and prevent injury.

 

  • Administer medications appropriately and monitor for side effects
    • Beta blockers
    • Calcium channel blockers
    • Angiotensin receptor blocker

 

Medications are given to reduce the blood pressure and relieve the force of pressure on the aorta. The first-line medication is a beta blocker, but calcium channel blockers or angiotensin receptor blockers may be used in patients who are unable to tolerate beta blockers.

Writing a Nursing Care Plan (NCP) for Marfan Syndrome

A Nursing Care Plan (NCP) for Marfan Syndrome starts when at patient admission and documents all activities and changes in the patient’s condition. The goal of an NCP is to create a treatment plan that is specific to the patient. They should be anchored in evidence-based practices and accurately record existing data and identify potential needs or risks.

  • Provide pre- and post- operative care as appropriate

 

Surgery may be required to stabilize or correct aorta or valve issues. Other surgeries may be to repair or treat sternum problems from retraction or protrusion or for eye surgeries to repair retina or lens displacement.

Provide appropriate care and education before and after surgery as required.

 

  • Provide patient and parent education for safety and nutrition

 

  • Help relieve anxiety about the condition and promote safe activities.
  • Patients should avoid high risk activities and contact sports to prevent injury.
  • A balanced low-sodium diet is encouraged to protect the heart and reduce blood pressure.

 

  • Provide patient education and support for self-image issues

 

Especially in the adolescent and teen years, patients are often self-conscious about appearance.

Help them to find beauty in themselves and offer information about resources and peer groups to build confidence and promote self-esteem.

Writing a Nursing Care Plan (NCP) for Marfan Syndrome

A Nursing Care Plan (NCP) for Marfan Syndrome starts when at patient admission and documents all activities and changes in the patient’s condition. The goal of an NCP is to create a treatment plan that is specific to the patient. They should be anchored in evidence-based practices and accurately record existing data and identify potential needs or risks.


References

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Transcript

This is the nursing care plan for Marfan syndrome. The pathophysiology behind Marfan; it’s a genetic condition where the proteins that make up the connective tissue that supports the heart, blood vessels, and other vital organs is weakened. So some nursing considerations that we want to think of: we want to monitor and manage their blood pressure; we want to do EKG monitoring, preferably 12 lead; and we want to assess their vision with the snellen chart. The desired outcome for this patient is that this patient will have optimal cardiac output with adequate tissue perfusion. We also want to make sure that this patient is safe and remains free from injury. So Marfan syndrome, this patient has a few things that they are going to complain about. First, they have difficulties seeing at a distance. So that is near-sightedness. 

They’re going to be very nearsighted. They’re going to also complain of foot pain and back pain. Some things that we are going to observe, some objective data that we can pull from the patient. It’s pretty typical for those with Marfan syndrome, they’re going to have some disproportionately long arms and legs and fingers. They’re going to be tall and their body’s going to be very thin. They’re going to have a curved spine. So that is called scoliosis. They’re also going to have very flexible joints. A lot of those with Marfan syndrome are what we call double-jointed. They’re going to have very flat feet. They can present with crowded teeth, stretch marks on the skin. They oftentimes have a heart murmur, and they’ll have a retracted or protruding sternum. So what are some things that we can do, uh, or intervene? How can we intervene with this? Well, let’s do an assessment. Assessment is always good, so we can gather the information we need. We want to perform a complete physical assessment, as well as auscultate the heart and lung sounds. This will help to get baseline data. We’re going to note any retractions, any protrusions in the sternum. We’re going to listen. We’re also going to check for murmurs in the heart leaky valves or any abnormal breath sounds to determine the progression of this condition. Next we’re going to do a good assessment of their eyes because this is a connective tissue disorder. They oftentimes present with, uh, extreme near-sightedness. So we’re going to assess their vision using the Snellen vision chart. We’re going to assess the degree of vision impairment and make any appropriate recommendations for the Ophthalmologist. 

There are medications that we want to administer, but we also want to make sure that we monitor for these side effects. These are primarily aimed at controlling their blood pressure. So we’re going to administer beta-blockers, calcium channel blockers and angiotensin receptor blockers. We want to make sure that we control their blood pressure and relieve the force of the blood on the aorta. The first-line medication is the beta-blocker, but calcium channel blockers and angiotensin receptor blockers may be used in patients who are able to tolerate beta-blockers. We want to obtain a 12 lead EKG and an echocardiogram as appropriate. This is to monitor for cardiac involvement. We want to look for signs of the disease progression, and we want to determine cardiac function. 

We want to assess range of motion and perform a range of motion exercises. This is to assess and monitor the flexibility and range of motion and exercises. They’re very important for maintaining strength and mobility. The next thing that we could do is we want to provide patient and parent education for safety and nutrition. Patients, specifically those with Marfan syndrome, want to avoid high risk activities and context sports. We want to prevent injury. Finally, we want to monitor any diagnostics. So x-rays and CT scans. The CT scans and x-rays of the mouth and eyes. And the reason why we want to do that is we want to monitor for bone and joint deformities or malformations. Okay. 

So the key points, these are the key points. Remember Marfan syndrome is a disorder where the connective tissue that supports the heart blood vessel and other vital organs is weakened. Some subjective data: What is the patient going to say? The patient is going to say that maybe they can’t see at a far distance. They’re very, near-sighted, they’re going to complain of pain in their foot or back. Some things that we’re going to see are the hallmark signs of Marfan syndrome. We’re going to see a tall, thin body, a curved spine or scoliosis. We’re going to see flexible joints, flat feet. They may have a heart murmur when we listen and we’re going to notice a retracted sternum. Our goal is to minimize the complications. So we’re going to do some good BP management. We’re going to manage this, the blood pressure, and we’re going to treat it. Elevated blood pressure and elevated heart rate may indicate aortic injury or damage we’re going to do frequent assessments. 

The patient is at risk for cardiac issues, such as an aortic aneurysm. So we’re going to assess any new or developing murmurs. I just want to let you all know, uh, leave you with this quote here. So I thought I saw this and I thought that this would be really good for you all in nursing school. The best way to gain self-confidence is to do what you are afraid to do. Not everybody can go through this journey. Nursing school is very difficult, and I know that maybe that last test was not your best test, but just know that as long as you utilize your resources and you just keep looking forward to your end goal, your end goal is to get the NCLEX pass. Don’t feel like a failure. Just embrace this journey on your way to becoming a registered nurse. So as always, we love you guys; go out and be your best self today, and, as always, happy nursing.

 

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