Lymphedema sufferers must cope for a lifetime
BY BORDEN BLACK --
Special to the Ledger-Enquirer (Columbus, Georgia)
Tuesday Oct. 30, 2007
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Lymphedema is one of those little-known conditions that doesn't spark telethons or poster children. It is life-altering, however, and can dictate lifestyle. I should know. I've had lymphedema since the lymph nodes in my legs were removed following cancer surgery.
About lymphedema
The lymph system has been described as the sewage system of the body. Waste products, bacteria, dead cells and large protein molecules are transported in lymph from tissue to the nodes where they are broken down and eliminated. When the system is broken, abnormal accumulation of protein-rich fluid can accumulate, usually in an arm or leg. This results in swelling in the limb and thickening of the skin known as lymphedema. The condition can be painful and disfiguring and can result in infections, depression and decreased mobility.
While primary lymphedema occurs at birth, secondary lymphedema is the result of damage to the lymphatic system. It is the most common form of lymphedema, and frequently occurs in cancer patients. Dr. Kathleen Francis, Medical Director of the Barnabas Lymphedema Treatment Center in Livingston, N.J., points out that if the lymph nodes in the armpit area are removed or radiated as a result of breast cancer treatment, it can lead to lymphedema.
She was among the doctors, therapists and patients who gathered in Atlanta recently for a conference sponsored by the Lighthouse Lymphedema Network. The nonprofit support organization was formed by Joan White, who developed the condition as the result of appendicitis. The idea for the organization grew from a discussion around her kitchen table in 1994. She points out that quality of life is a huge issue for those with the condition, many of whom have survived cancer only to find that they must continue to spend a great deal of time and energy dealing with the resulting lymphedema. The need for support, advice and awareness led to formation of the network.
The problems
Dr. Paula Steward, Medical Director of Lakeshore HealthSouth Rehabilitation Hospital in Birmingham, Ala., explained the problem faced by those suffering from lymphedema by quoting Dr. Robert Lerner: "In the past physicians played down the importance of lymphedema and pointed out that it is rare, that there is no effective treatment and that the patient must learn to live with it... . They have failed to instruct patients on how to avoid lymphedema... and continue to grossly understate the incidence of this serious and lifelong illness." She points out that correct diagnosis is essential in providing effective treatment.
Several other conditions, including inflammatory arthritis and post-phlebitic syndrome, have similar symptoms so accurate tests are needed for proper diagnosis. Currently, ultrasound, magnetic resonance imaging and computed tomography (CT) are used, but a diagnostic tool being employed in Europe is seen as the best option. Dr. Waldemar Olszewski, a world renowned expert in lymphedema, came from the Polish Academy of Science Hospital in Warsaw to discuss with conference attendees isotopic lymphoscintigraphy. The procedure has yet to be approved for use in America, but according to Olszewski: "It is necessary for the establishment of rational therapy."
Once correctly diagnosed, the lymphedema patient faces a lifetime of treatment because there is no cure. Swelling can be managed through a specialized massage technique known as manual lymphatic drainage, by using compression garments and bandages daily, by engaging in regular exercise and maintaining meticulous skin and nail care.
White points out that in many cases physicians are not acquainted with treatment for the condition and patients get most of their information from massage therapists. In fact she says often patients with swelling are diagnosed by therapists. Another difficulty according to White is the failure of insurance companies to pay for therapy or compression garments.
Solutions
Although there is no cure, Dr. David Feingold told those attending the Lighthouse conference that genetics may be the answer. "How do you generate new lymphatics?" That is the question being examined by the genetic researcher. Three genes have been identified, and experiments are under way.
Until an answer is found, awareness and education are the focus of the Lighthouse Lymphedema Network. A bill has been introduced by Georgia state Rep. Debbie Buckner. The house bill provides for a pilot program to test and evaluate a system of coverage for costs associated with the prevention and treatment of lymphedema and to provide for data collection.
The Atlanta organization has also set aside Oct. 22 as Lymphedema Awareness Day in Georgia.
Although awareness is growing, White points out that it is still "an image thing" and many of those with the condition are reluctant to go public and as a result there is still a lack of emphasis.
Ledger Inquirer
Tuesday, October 30, 2007
Saturday, October 27, 2007
Identification of a novel VEGFR-3 missense mutation in a Chinese family with hereditary lymphedema type I.
Identification of a novel VEGFR-3 missense mutation in a Chinese family with hereditary lymphedema type I.
J Genet Genomics. 2007 Oct
Yu Z, Wang J, Peng S, Dong B, Li Y.
Department of General and Vascular Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100730 China.
Keywords: hereditary lymphedema; vascular endothelial growth factor receptor gene (VEGFR-3); missense mutation; autosomal dominant
A novel mutation of vascular endothelial growth factor receptor gene (VEGFR-3), was identified in a four-generation Chinese family with hereditary lymphedema type I (HL-I). Genetic linkage analysis was performed on the known genetic locus for HL-I with a panel of polymorphic markers, and then mutations were screened out by direct sequencing. By genotyping, the family showed the linkage to HL-I locus on 5q35.3. Mutation screening analysis of the exons encoding the intracellular kinase domains of VEGFR-3, revealed a novel missense mutation D1055V. This mutation cosegregated with the disease phenotype in the family and was not found in 100 normal controls.
This finding has expanded the spectrum of the VEGFR-3 gene mutations causing HL-I, and will be useful for further genetic consultation and genetic diagnosis.
Science Direct
J Genet Genomics. 2007 Oct
Yu Z, Wang J, Peng S, Dong B, Li Y.
Department of General and Vascular Surgery, Beijing Tongren Hospital, Capital Medical University, Beijing 100730 China.
Keywords: hereditary lymphedema; vascular endothelial growth factor receptor gene (VEGFR-3); missense mutation; autosomal dominant
A novel mutation of vascular endothelial growth factor receptor gene (VEGFR-3), was identified in a four-generation Chinese family with hereditary lymphedema type I (HL-I). Genetic linkage analysis was performed on the known genetic locus for HL-I with a panel of polymorphic markers, and then mutations were screened out by direct sequencing. By genotyping, the family showed the linkage to HL-I locus on 5q35.3. Mutation screening analysis of the exons encoding the intracellular kinase domains of VEGFR-3, revealed a novel missense mutation D1055V. This mutation cosegregated with the disease phenotype in the family and was not found in 100 normal controls.
This finding has expanded the spectrum of the VEGFR-3 gene mutations causing HL-I, and will be useful for further genetic consultation and genetic diagnosis.
Science Direct
Monday, October 08, 2007
Lymphedema: A Comprehensive Review
Lymphedema: A Comprehensive Review
Ann Plast Surg. 2007 Oct
Warren AG, Brorson H, Borud LJ, Slavin SA.
From the *Harvard Medical School, Boston, MA; †Harvard Medical School, Division of Plastic Surgery, Beth Israel Deaconess Medical Center, Boston, MA; and the ‡Department of Plastic and Reconstructive Surgery, Lund University, Malmö University Hospital, Malmö, Sweden.
BACKGROUND:
Lymphedema is a chronic, debilitating condition that has traditionally been seen as refractory or incurable. Recent years have brought new advances in the study of lymphedema pathophysiology, as well as diagnostic and therapeutic tools that are changing this perspective.
OBJECTIVE:
To provide a systematic approach to evaluating and managing patients with lymphedema.
METHODS:
We performed MEDLINE searches of the English-language literature (1966 to March 2006) using the terms lymphedema, breast cancer-associated lymphedema, lymphatic complications, lymphatic imaging, decongestive therapy, and surgical treatment of lymphedema. Relevant bibliographies and International Society of Lymphology guidelines were also reviewed.
RESULTS:
In the United States, the populations primarily affected by lymphedema are patients undergoing treatment of malignancy, particularly women treated for breast cancer. A thorough evaluation of patients presenting with extremity swelling should include identification of prior surgical or radiation therapy for malignancy, as well as documentation of other risk factors for lymphedema, such as prior trauma to or infection of the affected limb. Physical examination should focus on differentiating signs of lymphedema from other causes of systemic or localized swelling. Lymphatic dysfunction can be visualized through lymphoscintigraphy; the diagnosis of lymphedema can also be confirmed through other imaging modalities, including CT or MRI. The mainstay of therapy in diagnosed cases of lymphedema involves compression garment use, as well as intensive bandaging and lymphatic massage. For patients who are unresponsive to conservative therapy, several surgical options with varied proven efficacies have been used in appropriate candidates, including excisional approaches, microsurgical lymphatic anastomoses, and circumferential suction-assisted lipectomy, an approach that has shown promise for long-term relief of symptoms.
CONCLUSIONS:
The diagnosis of lymphedema requires careful attention to patient risk factors and specific findings on physical examination. Noninvasive diagnostic tools and lymphatic imaging can be helpful to confirm the diagnosis of lymphedema or to address a challenging clinical presentation. Initial treatment with decongestive lymphatic therapy can provide significant improvement in patient symptoms and volume reduction of edematous extremities. Selected patients who are unresponsive to conservative therapy can achieve similar outcomes with surgical intervention, most promisingly suction-assisted lipectomy.
PMID: 17901744 [PubMed - as supplied by publisher]
Ann Plast Surg. 2007 Oct
Warren AG, Brorson H, Borud LJ, Slavin SA.
From the *Harvard Medical School, Boston, MA; †Harvard Medical School, Division of Plastic Surgery, Beth Israel Deaconess Medical Center, Boston, MA; and the ‡Department of Plastic and Reconstructive Surgery, Lund University, Malmö University Hospital, Malmö, Sweden.
BACKGROUND:
Lymphedema is a chronic, debilitating condition that has traditionally been seen as refractory or incurable. Recent years have brought new advances in the study of lymphedema pathophysiology, as well as diagnostic and therapeutic tools that are changing this perspective.
OBJECTIVE:
To provide a systematic approach to evaluating and managing patients with lymphedema.
METHODS:
We performed MEDLINE searches of the English-language literature (1966 to March 2006) using the terms lymphedema, breast cancer-associated lymphedema, lymphatic complications, lymphatic imaging, decongestive therapy, and surgical treatment of lymphedema. Relevant bibliographies and International Society of Lymphology guidelines were also reviewed.
RESULTS:
In the United States, the populations primarily affected by lymphedema are patients undergoing treatment of malignancy, particularly women treated for breast cancer. A thorough evaluation of patients presenting with extremity swelling should include identification of prior surgical or radiation therapy for malignancy, as well as documentation of other risk factors for lymphedema, such as prior trauma to or infection of the affected limb. Physical examination should focus on differentiating signs of lymphedema from other causes of systemic or localized swelling. Lymphatic dysfunction can be visualized through lymphoscintigraphy; the diagnosis of lymphedema can also be confirmed through other imaging modalities, including CT or MRI. The mainstay of therapy in diagnosed cases of lymphedema involves compression garment use, as well as intensive bandaging and lymphatic massage. For patients who are unresponsive to conservative therapy, several surgical options with varied proven efficacies have been used in appropriate candidates, including excisional approaches, microsurgical lymphatic anastomoses, and circumferential suction-assisted lipectomy, an approach that has shown promise for long-term relief of symptoms.
CONCLUSIONS:
The diagnosis of lymphedema requires careful attention to patient risk factors and specific findings on physical examination. Noninvasive diagnostic tools and lymphatic imaging can be helpful to confirm the diagnosis of lymphedema or to address a challenging clinical presentation. Initial treatment with decongestive lymphatic therapy can provide significant improvement in patient symptoms and volume reduction of edematous extremities. Selected patients who are unresponsive to conservative therapy can achieve similar outcomes with surgical intervention, most promisingly suction-assisted lipectomy.
PMID: 17901744 [PubMed - as supplied by publisher]
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