eNews Online - January 1999 Edition

eNews Online
January 1999 Edition

This Lymphedema eNews is being generated through your request from our website.

Welcome to our January 1999 edition of Lymphedema eNews. We continue to have a growing subscription with positive feedback in response to Lymphedema eNews. Thank you for your positive feedback and we hope the contents continue to be interesting and informative.

Our Monday evening live lymphedema online support group is slowly growing. This is a great way to be in touch with other lymphedema patients and health care providers who treat lymphedema. For patients that do not have access to a local support group, or simply want the benefits of interacting with others online, chat services provide an excellent avenue. For those of you who would like to participate, go to our main page and sign up. For those of you who have signed up and the day and/or time is not condu cive to your schedule, write us with suggestions of other times or days and we will do our best to accommodate as many requests as possible. Since Peninsula Medical staff hosts the chat, keep in mind we are here from 7am until 5pm pacific standard time Monday through Friday. The chat day and time would need to be during those times.

Primary Lymphedema

There have been several questions about the cause and inheritance of primary lymphedema recently and whether the ReidSleeveŽ is an effective treatment for primary lymphedema. Let me answer the easiest part of the questions first. Many patients with primary lymphedema have had significant benefit from use of the ReidSleeveŽ. Infants, young children, teenagers and adults with primary lymphedema have benefited from using the ReidSleeveŽ.

So what is primary lymphedema? Primary lymphedema is an inherited form of lymphedema. Primary lymphedema may present without a specific cause such as trauma, infection or surgery It may occur at any phase of life but it most commonly appears at puberty. In contrast, secondary lymphedema is seen much more often and is due to a specific cause such as surgery, radiation or trauma. In the US, one of the most common causes is axillary dissection as part of the treatment for breast cancer.

There was a report published just a few weeks ago that sheds some light on the cause of primary or hereditary lymphedema (see Ferrell et. al.). The authors of this study evaluated several families where lymphedema presented in an inherited form. From their studies they were able to conclude that hereditary lymphedema presented in an autosomal dominant pattern. This means that the children of parents with lymphedema are at high risk of developing lymphedema if they inherit the abnormal gene. For example, if the mother of a child has lymphedema and the father is normal, the child has essentially a 50% chance of inheriting the defective gene. However, even if the child inherits this gene, it does not mean they will get lymphedema. Even if a child inherits an abnormal gene, this gene may not be fully expressed or symptoms develop only after a certain age or after trauma or another inciting event.

In genetic terms we describe the strength of a gene as penetrance. If all children who inherit the abnormal gene develop the trait, then there is strong penetrance. If only a fraction of children who inherit the abnormal gene actually develop the trait, then the penetrance is reduced or inconsistent or variable. This is the case with the lymphedema gene. While we cannot predict who will get lymphedema, it is clear that not all children who inherit the gene will show evidence of lymphedema. In the case of p rimary lymphedema, the penetrance appears to be variable. In addition, the age of presentation can also be quite variable.

The genetics of this disorder are just becoming understood and there is some evidence to suggest that the problem may be related to abnormal expression of endothelial growth factor (for a discussion of vascular endothelial growth factor see previous eNews). However, further experimentation and study is required to fully understand this problem and it is likely that additional genes will be identified. Fortunately, serious scientific studies are underway and we will keep you up to date on new developments i n the field.

Tony Reid MD Ph.D

Hereditary lymphedema: evidence for linkage and genetic heterogeneity. Ferrell RE, Levinson KL, Esman JH, Kimak MA, Lawrence EC, Barmada MM, Finegold DN
Hum Mol Genet 1998 Dec;7(13):2073-8

Lymphedema: classification, diagnosis and therapy.
Szuba A, Rockson SG
Vasc Med 1998;3(2):145-56

Case of the Month

Sandra was diagnosed with lymphedema in 1993, approximately 2 months post mastectomy. She had little knowledge of any available treatment for lymphedema beyond her elastic daytime sleeve. Her arm continued to get bigger.

In 1997, Sandra learned about the ReidSleeveŽ through a magazine article and sought a prescription for the ReidSleeveŽ for treatment. Sandra received her ReidSleeveŽ in December of 1997 and joined the patient support service program. Her progress was followed and monthly reports generated from our edema calculator were sent to her doctor and to date has shown 82.74% volume reduction.

Sandra stated to me, "I think without the ReidSleeve my arm would have only gotten worse. I couldn't wear my clothes and couldn't find clothes to fit." Sandra is now pleased that she can wear all her "pre-lymphedema" clothing again!

When Sandra ordered her ReidSleeveŽ she had sent in a photograph of her affected arm. She realized that she didn't have any photographs of her arm when it was enlarged, and asked if we still had the picture. Her picture is currently in transit back to her for her to be able to see what her arm looked like prior to her treatment. In turn, she is sending back a current picture of her affected arm which we hope to have posted on the website shortly.