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Marfan Syndrome Pathochart (Cheat Sheet)
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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.
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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