In This Issue...
The Leigh's Center for Children at the University of California in San Diego (UCSD) was officially opened June 15, 1994. The event was marked with a small morning reception at the University of California Medical Center with special guests Mr. and Mrs. Dan Wright and their two children, Kelsey and Austen. Other notable guests included Dr. Richard Haas, Director of the Leigh's Center; Dr. Stanley Mendoza, Chairman Department of Pediatrics; Dr. Michael Ziegler, Director of the General Clinical Research Center (CRC); Dr. Deborah Marsden, staff physician; Cece Echon, nurse manager of the CRC, and Angie Longenecker, nurse coordinator for The Leigh's Center. The opening of the Center was made possible by a generous donation from the Wright family. In his opening remarks Dr. Haas stated, "This Center will make a real difference in the evaluation, care, treatment, and research for children with Leigh's Syndrome and it provides new hope to their families."
The Leigh's Center is designed to provide comprehensive evaluation, treatment, support, and research for children with Leigh's Syndrome from the United States and throughout the world. Children coming to The Leigh's Center will receive inpatient care at UCSD's CRC, and will be followed through The Center's outpatient clinic for follow-up care. The Center will serve as a resource for both health care providers and parents by maintaining an up-to-date library on Leigh's Syndrome, a parental support network, newsletter, and a toll free "800" number for community outreach.
The Center is supported entirely by private donations from individual sponsors. For information on sponsorship contact The Leigh's Center for Children, 200 West Arbor Dr., San Diego, CA 92103-8467, or call (800) 3-LEIGHS. Fax: (619) 294-6191.
One of the most common questions parents ask us is about their child's hospitalization in the general Clinical Research Center (CRC) at the Medical Center. We hope to paint a picture of the visit in this article.
Once it is decided that your child needs to come to the CRC for an evaluation, we need to get some information about your child in order to schedule the visit. We need your child's name, birthdate, home address, telephone number, insurance carrier and policy number. With this information we can schedule the visit. Generally the visits are for four nights and five days and we work with the CRC and the parents to make the visit as pleasant as possible. Your child will most likely not have a private room, but will share the room with another child. Your child will sleep in a crib or a bed. One parent or family member may stay overnight with the child and we can provide a bed for you. There is a metabolic kitchen in the CRC which can provide all your child's special diet needs and many of their "favorite" foods. Our metabolic dietitian will carry out a dietary evaluation as part of the diagnostic work-up. There is a hospital cafeteria always open for adults.
We would like you to arrive about 7am on the day of admission and you will then go to register. When this is completed you will go to the CRC, located on the ninth floor of UCSD Medical Center. The CRC is a warm and friendly environment staffed by caring physicians and nurses. Games, toys, a playroom and even VCRs are available for your child. If your child has a favorite toy or game, please bring them along because there are long stretches of time where it seems like nothing is happening to the children (although timed studies may actually be in progress). Initial laboratory tests will be taken and an IV access will be inserted . This IV is necessary so that your child will not be subjected to many needle sticks, but hopefully just the one, and all subsequent labs can be drawn from the line.
During your stay on the CRC your child will have many tests which provide the physicians with valuable information about your child and his or her body's response to the disease. For most of these procedures you may stay with your child if you choose. The CRC staff are very understanding and if at all possible will accommodate your wishes. Your child will be "fasted" for some studies, but the IV access will provide essential fluids for hydration and all the blood chemistry will be monitored very closely. Your physician may not always be present, but another well trained physician is always only seconds away.
While you are in the hospital, there will be medical students, residents, and attending physicians involved in the care of your child so there is plenty of opportunity to ask questions. Before coming to the hospital the staff of the Leigh's Center will be available to answer any questions or concerns. We will also make any special arrangements needed for your stay in San Diego.
Although summer is upon us, your child's health is still a primary concern as fall and winter approach.
Fall is a good time of year to give some thought to your child's immunization status. Are your child's immunizations up-to-date? Are there new vaccines that they should receive? Unfortunately, children that are frequently ill may miss scheduled immunizations because of an acute problem--and then forget to get the immunization after they are well. It is particularly important for children with metabolic diseases to be protected against dangerous childhood illnesses.
In addition to the usual childhood immunizations, it is now recommended that children receive the Hepatitis B vaccine which is a three shot series. A yearly "flu shot," or influenza vaccine is suggested in the fall months prior to the flu season. The influenza vaccine is given in either a one or two shot series depending on the age of your child. A new vaccine to protect against chicken pox and varicella was made available in late 1994 and is recommended for children who have not had chicken pox. As with all medical care, be sure to check with your child's physician, or public health agency about the optimal immunization schedule for your child. Remember, protection is the best defense against infectious disease.
Treatment for Leigh's syndrome is limited and only partially effective. Our approach following detailed evaluation in the Leigh's Center is to recommend a diet tailored to the individual patient, vitamins and cofactors as indicated, and physical , occupational and speech therapy. Drug treatments may be needed for seizure control, movement disorder, cardiac or renal complications. Recently, we have received FDA approval to use an experimental treatment, dichloroacetate (DCA) for the treatment of children with sustained lactic acidemia. Our trial of DCA is underway.
Some children with Leigh's syndrome do not have elevated blood lactate levels but do have elevated levels in their cerebrospinal fluid allowing them to be treated. In some cases, lactate levels are not high enough to warrant treatment. DCA adds to the list of treatments for the metabolic consequences of mitochondrial disease. It is not a cure. Only gene therapy offers the possibility of a cure and we at the Leigh's Center along with many scientists around the world are working hard to develop gene therapy for mitochondrial diseases. For further information on treatment for Leigh's syndrome contact the Leigh's center.
The new "toll free" number at the Leigh's Center (1 800 3-LEIGHS) was made possible by a special donation from Dr. Madrid Hamilton-Turner of San Francisco in memory of her granddaughter, Andrea Hamilton. This toll free number will help families get the latest information to help their child. We are grateful to a loving grandmother for her donation and thoughtfulness.
The Leigh's center has developed a home page on the internet. We will have information on the center, this newsletter, abstracts of the scientific literature on Leigh's syndrome , a mailbox and other information available. The address is:
Thanks to the kindness of the San Diego Zoo, Wild Animal Park, and Sea World we are able to offer complimentary passes to the children and immediate families of those children being seen for Leigh's Syndrome at the Clinical Research Center. All we need is a 24 hour working day advance notice and these facilities will provide the passes. Please let us know if you are interested.
The Leigh's Center Newsletter is a publication of the Leigh's Center for Children at the University of California, San Diego. Viewpoints and statements in this newsletter do not necessarily reflect the policies and/or the official position of the University of California.
Note: The Leigh's Center for Children has expanded to become the Mitochondrial and Metabolic Disease Center at UCSD.
Return to MMDC@UCSD home page.