Alexandra “Lexi” Martinez, of Burlington County, NJ, is an active, outgoing 7-yearold. Doctors diagnosed Lexi with fibromuscular dysplasia (FMD) at the age of 5. She was in kindergarten. In people with FMD, the dysplasia, or abnormal cellular development or growth, involves the walls of one or more arteries in the body. FMD is usually found in the arteries that supply the kidneys with blood, or renal arteries, although it can also be found in arteries that supply the liver, spleen and intestines as well as arteries in the extremities. Usually in FMD, the arteries appear as a “string of beads.”

Kevin Meyers, MD, a nephrologist at the Children’s Hospital of Philadelphia, and a member of the Medical Advisory Board of the Fibromuscular Dysplasia Society of America, says, “FMD in children affects the renal vessels far more commonly than the head and neck vessels. And this is a different pattern than what is seen in adults.” FMD in the renal arteries may cause high blood pressure. Progression of the disease can also lead to the death of kidney tissue over time, or ischemic renal atrophy. This can eventually lead to kidney failure.

During a routine wellness check at school, the nurse discovered Lexi’s blood pressure to be unusually high for her age. The nurse called Lexi’s mother, Jennifer

Martinez , and discussed the results. Jennifer is also a nurse, and immediately came to the school to check Lexi’s blood pressure herself. Jennifer then took Lexi straight to the pediatrician, and Lexi was then admitted to the emergency room. Within a week, the pediatrician and the nephrology team had their diagnosis: FMD.

“To make matters worse, at the time of Lexi’s hospitalization in May of 2005, my husband was four months into his 12 month deployment in Iraq,” said Jennifer. “I think if someone had taken my blood pressure, it would have been high, too! As they say, when it rains it pours!”

Jennifer credits her supportive and loving family with helping her get through that difficult time. “Our families stepped up to the plate when he was away. My parents and sister were at the hospital within two hours from the time I arrived at the emergency room with Lexi.” Lexi understood she had high blood pressure, though because of her young age, did not necessarily understand that it was due to a problem in her kidneys. There is no standard procedure for treating FMD, and no cure, so Lexi remained a strong patient while enduring a battery of tests and procedures, including many IVs, various ultrasounds, lots of blood work, many pills and an angiogram.

Jennifer reports that Lexi was a remarkable patient for being so young. “The nurse couldn’t get over the fact that she was 5 years old. Lexi just went with the flow and even said she was having a good time and couldn’t wait to pick her next meal and have it served on a tray. At the hospital, she walked around in her gown and Hello Kitty slippers as if she were at the Hilton.”

Friends and family members admired Lexi for her strength and courage, noting she never once appeared to be afraid. Now that Lexi is a little older, she understands FMD and kidney disease and what it means for her. She has been on medication to regulate her blood pressure since her hospitalization in 2005.

Lexi sees her nephrologist every three to four months. She takes an ACE inhibitor and a diuretic twice daily. Jennifer monitors her blood pressure at home every two to three days. Lexi’s condition has been stable on these medications. Also, her kidney enzymes have remained stable. She has had two angiograms, the first during her initial hospitalization in 2005 and another in January of 2006. The second angiogram showed no significant change. A blood test to check her kidney function is done with every visit to the nephrologist.

Lexi says one of the negatives of having FMD is “being thirsty and drinking lots of water.” She is very active, and thinks she could be a better runner if she didn’t have FMD. Lexi maintains a positive attitude, saying, “I feel like a regular person!”

Lexi and her sister, 6-year-old Abrielle, attend Southampton Township School Number One. Lexi is now a second grader and Abrielle is a kindergartner. Lexi loves to take dance lessons. This year she is studying Jazz. She plays soccer in the fall, basketball in the winter, softball in the spring and swims in the pool all summer long. She even plans to be on a swim team this year. Her swim teacher says she is a natural swimmer. When asked if FMD has ever held her back, Lexi said, “No!”

The Martinez family understands Lexi’s FMD and how they must all cope with it. Says Jennifer, “We all know what she has and even her little sister somewhat understands but never has it interfered with anything we do or say as a family. Abrielle can tell you what medications Lexi is taking. Abrielle even has me check her own blood pressure to make sure it is normal. I don’t think Abrielle and Lexi see it as a bad thing, just as a small issue that we all deal with as a family.”

As a nurse, Jennifer understands what FMD is and the potential of the disease. She is aware of what kidney disease is and knows the different types of kidney diseases. Jennifer has seen both chronic kidney disease (CKD) patients and end-stage renal disease (ESRD) patients in hospitals, but she had never heard of FMD until Lexi was diagnosed.

Jennifer says, “I find myself always educating people about this rare and unknown disease. When Lexi was first diagnosed, I did research online and came across the Fibromuscular Dysplasia Society of America (FMDSA) and other online articles, but there were few. We were once given pens and pads by FMDSA Chairperson Pam Mace to distribute to get people asking about the disease. Lexi proudly took them to school and handed them to her teachers and still gives them to all her other doctors.”

Very little is known about FMD. Says Dr. Meyers, “Although there are many theories about the cause of FMD, the actual cause or causes of FMD are unknown. I predict that FMD is not going to be a single disease entity and that a number of possibly overlapping or intersecting causes will eventually be discovered.”

Although not enough is known about FMD, Jennifer and her family stay hopeful. “It’s a wait and see kind of disease,” she says. Jennifer, Lexi and the Martinez family insist on approaching FMD with a positive outlook. Lexi says that she is doing fine, and her attitude toward her family is “very loving.” And the Martinez family will continue to count their blessings – they are expecting another baby in June.

To learn more about FMD, visit the FMDSA Web site: http://www.fmdsa.org , or call (330) 653-8416.

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