Published in Winter 2009 New York State Association of School Nurses "The Communicator"
Fibromuscular Dysplasia
by Kari Ulrich, RN
A child comes into the nurse’s office complaining that her tummy hurts; she is tired and has a headache. The teacher and her parents have noticed she is not eating that well. As a school nurse you hear these symptoms frequently. In some children, however, these symp- toms are more than the typical stomachache. Such was the case with Jordan, who presented with the above complaints and was found to have a blood pressure of 290/150 mmHg. Jordan has a disease called Fibromuscular Dysplasia, or FMD. Looking back at Jordan’s symptoms, would your nursing assessment have included a blood pressure check along with taking her temperature?
Fibromuscular Dysplasia is a non-atherosclerotic, non-inflam- matory disease that usually affects medium sized arteries. Although many physicians consider FMD to be rare, it is most likely overlooked or misdiagnosed. Fibromuscular dysplasia typically presents with hypertension and on physical examination auscultation may reveal a bruit. FMD typically involves the renal and carotid arteries, although it can be present in most other vascular beds.
Three histological types of FMD are recognized and are character- ized by which layer of the artery is predominately affected:
•Medial fibroplasia: is characterized by its classic “string of beads” appearance which represents the most common type of medial dysplasia
•Intimal fibroplasia: Most common form found in children appears as a concentric band or a long smooth narrowing
•Adventitial fibroplasia: Rare, accounts for less than 1% of cases
Renal and mesenteric involvement may be more common in children than in adults. Taking a few extra minutes to assess a child’s vital signs including a blood pressure check could make an impact on finding this disease. Depending on which arterial bed is involved will depend on what symptoms if any a person will present with. Persons with FMD of the carotid arteries may present with dizziness, visual problems, tinnitus, neck pain, headaches, TIA or stroke. Persons where FMD affects the mesenteric arteries may experience nausea and vomiting that can lead to dehydration, abdominal pain and unintentional weight loss. FMD in the limbs may cause circulatory symptoms such as cold hands and feet, and pain with movement. FMD of the renal arteries may present with hypertension even at a young age. There can be more than one artery involved with FMD.
The etiology of FMD is unknown. Several theories have been suggested as a cause of FMD such as genetic, environmental, hormonal and mechanical. The disease is more commonly seen in young women but it can affect men, children and in rare case infants. There are a few connective tissue disorders that may be associated with FMD, such as Ehlers-Danlos Type (IV) syndrome and Marfan syndrome.
The “Gold-Standard” for diagnosing FMD is by angiography. The appearance of the “string of beads” is most commonly found in young adult women. The “string of beads” appearance is caused by abnormal cell development of the arterial wall in which the cel- lular tissue becomes narrowed and dilated. Other diagnostic tests include ultrasound, MRI, MRA, and 3D CTA. Consideration must be made if using contrast materials in someone who has impaired renal function. If carotid artery FMD is present a MRA is warranted to rule out an aneurysm. When obtaining an ultrasound of the carotid arteries it is important that the technician be familiar with the disease. FMD of the carotids can be missed if the technician does not look high enough toward the base of the skull to view the distal carotid arteries.
Unfortunately there is no cure for FMD. At this time there is no established protocol for treatment of FMD. Treatment depends on which vascular bed is affected with FMD. Good control of blood pressure can usually be achieved by using antihypertensive medica- tions. Some patients will need several medications to keep their blood pressure under control to prevent further damage to the kidney and loss of kidney function. Efforts to improve the blood flow to a severely stenosed artery are made by performing angioplasty otherwise known as PTA (percutaneous transluminal angioplasty). If angioplasty fails other options may include stenting and bypass, although these options are not usually recommended as primary treatment. Preventing an event such as a stroke or dissection from occurring is a key factor in this disease. Patients should be placed on daily aspirin for the anti- platelet effect; also blood thinners such as Coumadin maybe required. All treatment options should be discussed with the patient’s physician. If an aneurysm is present careful monitoring must be established and if needed coiling or clipping of the aneurysm may be done.
It is important for patients to work with their physicians to decrease other risk factors.
Thanks to the efforts of the Fibromuscular Dysplasia Society of America this disease has gained the attention of the medical community. Currently, FMDSA is working toward establishing an International Patient Registry. In June 2008 FMDSA held its first conference where patients and doctors came together to bring aware- ness and education to this disease. Both the American and National Stroke Association recognize FMD as a cause of stroke. FMD is listed on The National Organization of Rare Diseases.
Your assessment skills as a school nurse can play a critical role in the health care of a child with this disease and prevention of many other diseases. As nurses we must not minimize the important role of blood pressure screening as a tool we can easily utilize. Often, elevated blood pressure can be asymptomatic. Blood Pressure screen- ings in school age children can make an impact in disease prevention. FMD can strike young women, men and children and when left undiagnosed or mistreated it may be critical causing strokes, arterial dissections, loss of kidney function and sometimes death. Educating health care professionals is a start in preventing adverse outcomes in both children and adults with FMD. At a time when funding for school nurses is not a priority, and staff to student ratio’s are high, what seems to be a simple task of taking a blood pressure can be a challenge. With the help of School Nurses across the nation together we can make an impact on children’s health.
For more information on FMD and FMDSA please visit our web site at www.FMDSA.org or write to us at FMDSA, 20325 Center Ridge Road, Suite 620 Rocky River, Ohio 44116. Membership to FMDSA is free.
Kari Ulrich is a Registered Nurse with both Pediatric and Adult Emergency Room experience; she is a FMD patient.
Content Reviewed by
Kevin E. Meyers, MD Pediatric Nephologist Assistant Professor of Pediatrics The Children’s Hospital of Philadelphia and University
of Pennsylvania Philadelphia, PA
Bibliography
Begelman, Susan M. “FMDSA Frequently Asked Questions.” FMDSA. 2 Aug. 2004. Staff Physician, the Cleveland Clinic Foundation.
.
Meyers, Kevin E. C., and Neha Sharma Ba Iv. “Fibromuscular Dysplasia in Children and Adolescents.” Cath Lab Digest 15 (2007): 6+.
Olin, Jeffery W. Recognizing and Managing Fibromuscular Dysplasia. Medical Grand Rounds, Apr. 2007, CLEVELAND CLINIC. Cleveland, Ohio: CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 74 • NUMBER 4, 2007.
2007, CLEVELAND CLINIC. Cleveland, Ohio: CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 74 • NUMBER 4, 2007
