Marfan Syndrome In Children

WHAT YOU SHOULD KNOW:

• Marfan syndrome is a disorder that weakens your child's connective tissue and cause problems in his eyes, heart, and bones. The connective tissue gives strength and support to tendons, ligaments, blood vessels, and many other structures in the body. With Marfan syndrome, many of these structures are not as strong as they should be. Your child’s blood vessels may get damaged easily and other body parts may have defects (problems). Marfan syndrome is caused by an abnormal gene that causes connective tissue to become weak. Genes are little pieces of information that tell the body what to do or what to make. Your child may look tall and thin, with long arms and legs, a long face, and a small chin. He may have trouble seeing and breathing, an abnormal heartbeat, and abnormal bones in his body.
  
  
• Blood tests, computerized tomography (CT), magnetic resonance imaging (MRI), electrocardiograph (ECG), and x-rays may help diagnose Marfan syndrome. Treatment is given to prevent further damage to body areas affected by Marfan syndrome. Treatment may also correct problems caused by Marfan syndrome. He may be given medicines to help his heart beat correctly. Your child's caregiver may do surgery to repair damaged areas of his eyes, bones, and heart. Your child may also need to avoid activities that may strain his heart. Diagnosing and treating Marfan syndrome as soon as possible may relieve symptoms such as loss of movement and pain. Treating Marfan syndrome may also help your child live longer.

CARE AGREEMENT:

You have the right to help plan your child's care. Learn about your child's health condition and how it may be treated. Discuss treatment options with your child's caregivers to decide what care you want for your child.

RISKS:

• Treatment for Marfan syndrome may cause unwanted side effects. Medicines may make your child dizzy, vomit (throw up), and have trouble breathing. Medicines may make your child sluggish and sleepy, which may affect his performance in school. Your child may bleed too much or get an infection during surgery.
  
  
• If left untreated, your child's signs and symptoms may worsen and new ones may appear. Eye problems may cause pain, swelling, trouble seeing, and even blindness. His heart may not beat correctly and may even stop completely. A torn aorta (big blood vessel from his heart) may make him bleed inside his body and may cause death. The abnormal shape of his chest may cause trouble breathing. He may have trouble moving his arms and legs, and may have a hard time walking. Call your child's caregiver if you have questions about your child's condition, medicines, or care.

WHILE YOU ARE HERE:

Informed consent:

A consent form is a legal document that explains the tests, treatments, or procedures that your child may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your child's medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done to your child. Make sure all of your questions are answered.

IV:

An IV is a small tube placed in your child's vein. Caregivers use the IV to give your child medicine or liquids.

Medicines:

• Beta blockers These medicines help your child's heart beat softer and slower. Having a softer and slower heartbeat may prevent damage to his heart and aorta.
  
  

Tests:

• Blood tests: Your child may need blood tests to give caregivers information about how his body is working. The blood may be taken from your child's arm, hand, finger, foot, heel, or IV.
  
  
• CT angiography: A special x-ray machine uses a computer to take pictures of your child's blood vessels. Before taking the pictures, he may be given dye through an IV in his vein. The dye helps the blood vessels show up better in the pictures. People who are allergic to iodine or shellfish (lobster, crab, or shrimp) may be allergic to this dye. Tell your child's caregiver if your child is allergic to any of these.
  
  
• Echocardiogram: This test is also called an echo. Sound waves are used to show pictures of the size and shape of your child's heart. The echo can also show how well the heart is pumping and how well blood flows through it. Your child will lie down during the test. Caregivers will squirt clear gel onto your child's chest to help the echo probe move easily. The echo pictures are shown on a TV-like screen. The whooshing noise that you may hear is the sound of blood flowing through the heart. Caregivers may ask you to stay in the room with your child during this test.
  
  
• Eye exam: This test uses special instruments to look inside your child's eyes. Special devices and lenses may be used to look for problems such as glaucoma, cataracts, or retinal detachment
  
  
• Heart monitor: This is also called an ECG or EKG. Sticky pads placed on your child's skin record the electrical activity of his heart.
  
  
• Magnetic resonance imaging: This test, also called an MRI, allows caregivers to see inside your child's body. This may allow your child's caregiver to check for any problems in the bones, lungs, heart, eyes, and brain. Your child will need to lay still during a MRI. Never let him enter the MRI room with an oxygen tank, watch, or any other metal objects. This can cause serious injury.
  
  
• X-ray: These are pictures taken of parts inside your child's chest, abdomen (belly), and arms and legs. These pictures may show problems caused by Marfan syndrome.

Vital signs:

Caregivers will check your blood pressure, heart rate, breathing rate, and temperature. They will also ask about your pain. These vital signs give caregivers information about your current health.

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The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

Learn more about Marfan Syndrome In Children (Inpatient Care)