articles

Lymphedema: a condition impacting millions

Moving to Better Health, Carrboro, NC
Dr. Lorna H. Harris

PHD, RN, FAAN

Professor of Nursing, Chairperson, Department of Nursing, North Carolina Central University, Durham, NC

In my hometown, there was a schoolteacher who had one arm that was three or four times larger than the other. She always wore sleeveless outfits, even in the winter. If she wore a jacket, the sleeve for the affected arm was enlarged and looked out of place. I was always curious as to what had happened to cause this problem. When I was older I learned that this lady had an operation for breast cancer and the swelling was a result of the surgery. I remember hearing that this school teacher was lucky to be alive, despite the fact she had been disfigured. All of this tragic had happened to someone who was one of the best dressed ladies in our community. In nursing school, I learned the official name for the condition, lymphedema, but it was not a condition that was widely addressed or emphasized.

In 1998, I was diagnosed with breast cancer myself, and the term lymphedema took on new meaning and greater importance. After my mastectomies I visioned myself as the schoolteacher I was so curious about thirty years before. You can imagine the fear when after a few weeks I noticed the swelling in my fingers, my bracelets were fitting tighter and there was this collection of fluid under my right arm pit and upper back that could not be drained away. I remember being told that lymphedema was a possibility because I had so many lymph nodes removed and if swelling happens, I would need to wear a compression sleeve to keep it under control. That was the extent of my education and preparation, for what would be a life long condition after my mastectomies.

As nurses we have a responsibility to help clients adapt and adjust to the changes that illness, and the consequence of illness, have on their lives. In the area of lymphedema, there is much that nurses need to learn to be the effective caregivers and advocates for clients with this condition. I am fortunate to live in a community that is progressive in most areas of health care and when I sought help in dealing with my lymphedema I was able to find a program in the area that specialized in the treatment of the condition using a process of lymph massage along with compression garments and other strategies. As a result of my treatment, my right hand and arm appear normal and I have just minor swelling in my upper shoulder and back, more importantly my clothes fit.

I shared my story with you so that you could have a sense of the importance of this issue in the lives of many of the clients that we serve. The remainer of this article is written with one of the therapist who has cared for me over the past three years. It is hoped that you will use the material presented here to spark your interest in the topic of lymphedema and become informed so that you can be an effective resource in your community on the condition.

What is Lymphedema?

Lymphedema, which is also spelled lymphoedema, is abnormal swelling of the tissues, due to the presence of excess protein-rich fluid known as lymph. This swelling occurs when the lymphatic system malfunctions or is damaged and lymphatic fluid cannot drain as quickly as it is produced. It affects an estimated 100 million men, women, and children around the world, including at least 3 million Americans. The story that is told in the beginning of this article is about lymphedema of the arm as a result of treatment for breast cancer. Breast cancer is only one of the many causes of lymphedema. Therefore, almost every nurse will come across lymphedema in her practice. Lymphedema most commonly occurs in the extremities (arms or legs); however, it can also affect the trunk, breast, abdomen, neck, head, and or genitals.

The fluid that causes the swelling of lymphedema is protein-rich and this makes the tissues easily susceptible to infections. Each infection damages lymphatic structures and places the area more at risk for developing lymphedema. Once the lymphatic systems malfunctions or lymphatic structures are damaged and lymphedema has developed it can be treated but not cured. Early treatments can usually effectively control the swelling. Without treatment, the lymphedema symptoms become progressively more serious. Left untreated, lymphedema is much more serious than the inconvenience of a swollen limb. It is a progressive condition in which frequent infections cause serious pain and may require hospitalization. Skin changes, like benign papillomas may develop. Eventually, lymphedema can lead to the loss of mobility and total disability. In rare occasions malignant skin cancer develops, like lymph-angiosarcoma (Stewart-Treves-Syndrome.)

The Categories of Lymphedema

Lymphedema is classified as being either primary (genetic) or secondary (acquired.)

Primary Lymphedema
Primary Lymphedema, usually, is caused by the malformation of lymphatic vessels and affects approximately one out of every 6,000 individuals. Typically, the characteristic swelling begins at the foot and progresses upward toward the body. Primary lymphedema is congenital and can have its onset at different moments in life. The phase of life in which primary lymphedema develops is described in the following paragraphs.

Millroy’s Disease is lymphedema that is present at birth or develops within the first two years of life. Lymphedema that is present at this early age is not always called Millroy’s Disease. Lymphedema can also be present at birth as part of the genetic condition Turner’s syndrome.

Lymphedema Praecox, also known as Meige Disease, is lymphedema that appears in puberty. Approximately 75% of all primary lymphedema cases develop during this period. Lymphedema Praecox affects four times as many females as males and the swelling usually occurs in only one leg.

Lymphedema Tardum, which develops around the age of 35, affects both males and females; however, it occurs most frequently in females. Onset of this condition is sudden, often with no apparent cause, and the swelling may affect one or both of the legs.

Secondary Lymphedema
The lymphedema that is referred to in Lorna Harris’ personal story is known as secondary lymphedema. This is the more common form of lymphedema and is an acquired disorder caused by damage to the lymphatic system. As part of the treatment of cancer, lymph nodes in the armpit or groin are removed. With cancer surgery the limb adjacent to the surgery area is most frequently affected; however, lymphedema can develop in more parts of the body, including the head and neck, trunk (chest and abdomen), or the genitalia. In contrast to primary lymphedema, this swelling can begin anywhere in the limb, depending which nodes are removed, how heavy radiation was and how severe scarring had become.