Andrea's smile
by Susan Craig
"Hamburguesa, perro calientes!" Luis Olivo hollered on a busy street in Barquisimeto, Venezuela. The street vendor parked his pushcart at a main intersection, trying desperately to sell his hamburgers and hot dogs to make more money. It was midnight and he was tired. He would have to get up the next day for work at 7:30 a.m.
A hard living? Maybe so, but Luis was not only a street vendor. He was also a dentist with his own practice. He spent his days cleaning, filling and repairing teeth, and at night joined his wife Mary in selling food into the wee hours of the morning. What was behind the Olivos' tireless motivation? The answer is Andrea's smile.
Andrea is the Olivos' 11-year-old daughter who has a large, bluish lesion composed of abnormally formed, dilated veins on her face, neck and palate. The lesion is a venous malformation (VM), a painful condition that, because it is close to Andrea's airway and brain, could be life threatening. The malformation has progressively worsened since Andrea was born, and makes even smiling difficult for her.
Andrea's smile, though it looks different and sometimes causes her great discomfort, is the reason her parents have looked far and wide for help. Their little girl's resilience to the hardships of having such a visible facial abnormality inspired the Olivos to work literally night and day to get her help.
The gift that goes a long way
Ten-year-old Campbell Abbott rides his scooter in Barcelona, skis in the Alps and wrestles his dog, all despite an arterio-venous malformation (AVM) in his right thigh that causes extreme swelling and pain.
An AVM is a congenital abnormality in which the capillary bed, a network of tiny blood vessels that normally connects arteries and veins, is missing.
Before treatment, Campbell's leg swelled to three times its normal size, requiring him to use a wheelchair. Campbell's mother Lisa literally searched the world to find help for her son. Finally, she was led to the Vascular Anomalies Center at Children's Hospital Boston, which is recognized as the world leader in diagnosing and treating vascular anomalies. Each year, the center's 20-member interdisciplinary team treats hundreds of children from the United States and abroad.
The VAC team first treated Campbell with several embolizations, procedures in which the blood vessel is manipulated from within. He had more than 15 hospital admissions, including several extended stays for surgical procedures to remove excess tissue from his leg. These challenges took both a physical and psychological toll on Campbell and his family, but the difficulties have paid off.
"He is as pain-free, active and happy as he was before we started down this long road," says Lisa.
The Abbotts recently showed their support for the Vascular Anomalies Center with a philanthropic gift of $1.5 million. The gift will enable the center to research the processes that drive the development of AVMs and other vascular anomalies.
The Abbotts' generous gift supports the following research projects:
- Fat often associated with AVMs has an abnormal arrangement of blood vessels, which researchers hypothesize may be part of the disease process. Using gene therapy, they have found that manipulating the stability of the vessels that supply blood to the tissue can also regulate the growth of the abnormal vessels. They first plan to treat these fatty AVMs with drugs to destabilize the vessels, and then possibly use angiogenesis inhibitors to stop blood vessel growth.
- Implanting the blood vessels from a human cancer tumor in mice. This tumor stimulates the growth of arteries and veins, and researchers believe growth factors in the tumor can alter the host's blood vessel pattern. They hope to find novel factors in the tumor that stimulate growth of large arteries and veins typically found in AVMs.
- Examining the effectiveness of using water-soluble polymers or chemical compounds to deliver angiogenesis inhibitors (such as TNP-470) to the AVM site. They believe TNP-470 may inhibit blood vessel growth in AVMs in much the same way it does in cancerous tumors. Using polymers allows for sustained release of the drug and may inhibit the recurrence of an AVM.
- Characterizing the genetic patterns of vascular malformations and normal arterial and venous endothelial cells in order to identify the molecular changes responsible for the origin and progression of vascular anomalies. This data will be used to classify vascular malformations at the molecular level as opposed to the current method of trying to name tissue examined under a microscope.
"We are encouraged that, with continued research, we will be able to better treat patients with Campbell's type of anomaly, as well as patients with other lesions," says Patricia Burrows, MD, chief of the Division of Interventional Radiology. "Great progress has been made possible through the Abbotts' generosity."
If you are interested in supporting additional vascular anomalies research, please contact Julie Considine in the Children's Hospital Trust at (617) 355-6193.
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The family consulted several doctors in their country, but soon discovered that no hospitals in Venezuela have the technology or expertise to treat such malformations. So Mary, who had never used a computer, searched the Internet with a friend's help and found the Web site for the Vascular Anomalies Center (VAC) at Children's Hospital Boston.
Filled with hope after e-mails from the center indicated there was treatment that could benefit Andrea, the Olivos set out to raise money for a trip to Boston. They sold hot dogs, staged rodeos and small fairs, worked with the Venezuelan press to publicize their cause and approached their government for financial help.
By October 2000, they had enough money to make their first trip to Boston. Their first appointment was with Patricia Burrows, MD, chief of the Division of Interventional Radiology. She and the VAC team treat more than 450 patients each year with all types of vascular anomalies. Thirteen percent of these patients come from outside the United States.
Burrows and her staff used advanced radiological techniques to assess the number of veins making up the growth and the blood flow through Andrea's anomaly. In addition to the VM throughout the left side of her face, Andrea had a sinus pericranii on her right side. This meant that the malformation connected with irregular veins from the brain, a situation that further complicated treatment of the malformation and could be life threatening if the VM continued to expand.
The most common type of vascular anomaly, venous malformations are usually apparent on the skin, but can also be on a mucous membrane or in any organ. Although the precise cause of VMs is unknown, doctors believe that a deficiency of smooth muscle cells in the walls of veins causes them to stretch, similar to a weak area in a tire. The stretched veins then gradually enlarge the affected tissues.
Burrows and her colleagues from different disciplines around Children's meet weekly to review the charts and films of the most challenging vascular anomaly cases from all over the world. Together, the specialists decide either to have the patient come to Children's for treatment or provide advice to the patient's own physician.
After careful review, the group decided Andrea was a candidate for sclerotherapy, a process that involves injecting an irritating solution, such as alcohol, into the lesion to clot and shrink the abnormal veins.
Sclerotherapy is frequently done prior to partial or total surgical removal of a lesion, because once the lesion's mass has been reduced, a surgeon can better remove it. Treatment often involves several sclerotherapy sessions, gradually decreasing the size of an anomaly and stunting its growth. The process relies on internal scarring of the mass of veins, so patients usually require several months between sclerotherapy procedures.
After Andrea's first session, the Olivos returned to Venezuela, where they raised money for subsequent treatments. In May 2001, they returned to Boston to complete Andrea's treatment. They found a place to stay with the help of the Hospitality Program, a local organization that arranges lodging for patients and families in the homes of area residents. To make ends meet, Andrea's parents have worked various odd jobs, often in exchange for food or services, while Flavia, Andrea's 15-year-old sister, attends school in Brookline.
Though it has been a struggle, Mary says, "We are very blessed. When our spirits get down, we are lucky to be surrounded by people who lift them. The staff at the hospital, the doctors and nurses, always ask us how we are getting by."
After her second procedure, Andrea returned to Children's for sclerotherapy treatments in July, September and November 2001. The September procedure focused on reducing the size of her engorged tongue, which had been especially bothersome and made it difficult for Andrea to swallow her saliva.
In November, Burrows placed microcoils, which are platinum threads with fibers, into Andrea's neck to help close the abnormal veins that flowed from her head to her heart. The coils begin the clotting process, which then makes sclerotherapy more effective because the sclerosant can't seep out.
On Jan. 3, 2002, just after celebrating Christmas and New Year's with her family, complete with hallacas (a traditional Venezuelan Christmas dish), Andrea was admitted to Children's for surgery to remove part of the VM. John Mulliken, MD, senior associate in Surgery and a plastic surgeon, removed much of the scarred VM in Andrea's cheek, approaching the tissue from inside her mouth.
"I always prefer to resect a VM after sclerotherapy has shrunk the vessels as much as possible," Mulliken explains. "The surgical goals are to reduce the size of her VM, restore function and relieve pain. All children, and especially those as vivacious as Andrea, deserve to giggle without pain."
By all measures, Andrea's surgery was a success. The blue hue of her cheek has faded and her tongue is smaller and moves better. And although Andrea will continue to receive sclerotherapy and will need corrective lip surgery, the malformation is smaller and the swelling has gone down. The Olivos are hoping to return to Venezuela this summer, but Mary says, "Everything depends on Andrea's treatment. We will stay as long as we have to."
Presently, Andrea enjoys being a fifth-grader at the Brookline Runkle School, where she started last fall. Entering school in a new country, especially when you don't speak the language, is a challenge. It was even harder for Andrea because she looked different, but she has adapted very well.
"Sometimes I don't want anyone to ask me about my face and sometimes I really don't care," says Andrea. Although you'd never know it, she does let her guard down to her mom now and then, saying, "I don't want to be rejected anymore. I want to be like other girls."
Victoria Karian, RN, pediatric nurse practitioner in the Vascular Anomalies Center, talks to patients about being different. "I tell them their malformation is what makes them special, and that being able to cope with this birth defect makes it easier to deal with other things in life. They become strong enough to make it through anything," says Karian. "Andrea has a very bright personality. That's what gets her through."
Mary helps her daughter deal with people's reactions to her malformation. Recently, when Mary picked up Andrea from a park, Andrea asked, "Mama, did you hear that kid called his mother over to look at the monster? Do I look like a monster?" Mary, without missing a beat, said to her daughter, "Well, he must not know what a monster looks like because you are not a monster." Only later, when Andrea was asleep, did Mary cry about how cruel kids, and sometimes parents, can be.
Mary is encouraged, however, by how Andrea's treatment at Children's has already improved Andrea's quality of life. Her malformation is not curable, but Andrea's face will be more symmetrical, and she will have less swelling than before treatment. Still, she faces risks. Her parents will need to watch for swelling in her tongue and other areas in her mouth and throat, as these could block her airway and interfere with breathing. Enlargement of the veins behind her eye could also damage her eyesight. And while most patients tolerate the sclerotherapy well, it can cause scarring, nerve damage and cardiovascular problems. Because of these potential side effects, the Vascular Anomalies Center at Children's is actively looking for ways to improve the treatment of vascular malformations.
"They want Andrea to have a better way to live, a better way to eat, to talk, to look," says Mary. As a mother, Mary recognizes Andrea's anomaly is something her daughter will deal with for the rest of her life, but the family takes one day at a time. Together, they will decide Andrea's course of treatment as she grows.
For now, through her good grades, newfound command of English, and uncanny ability to persevere, Andrea continues to do what most kids do so well. She makes her parents smile just by being herself.
