Saturday, November 8, 2008

All you ever wanted to know about Lymphedema but were afraid to ask...

Lymphedema comes in two basic forms- Primary, or what we now understand to be genetic, and Secondary, as a result of injury to the lymphatic system usually because of surgery or cancer treatments.

I have Primary Lymphedema. This means that I have a genetic disorder that I have passed down in an active form to one daughter and perhaps a dormant form to the other. Yea, me!

Lymphedema "is a condition of localized fluid retention caused by a compromised lymphatic system. The lymphatic system (often referred to as the body's "second" circulatory system) collects and filters the interstitial fluid of the body". (H/T to Wikipedia.) That point about the lymphatic system being a second circulatory system is an important one. It's a passive system, there is no heart to force the circulation. Lymph fluid moves through the actions of your body and has to fight gravity. Let's think that one through, shall we? In LE (lymphedema), as the affected limb begins to swell, it becomes harder to use. If you've ever had a twisted ankle, you know how this feels. As your ankles swells, it becomes less flexible. It hurts to move it anyway, so you stop. Unless you elevate your foot, the swelling increases and makes it worse. Why do we elevate? To fight gravity of course. So, if the lymphatic fluid moves as you move and you stop moving, the only force working on it is gravity. Thus, the only way to reduce the swelling is to elevate the affected limb. Not a very practical way to live.

According to the National Lymphedema Network , " Lymphedema can develop when lymphatic vessels are missing or impaired (primary), or when lymph vessels are damaged or lymph nodes removed (secondary). When the impairment becomes so great that the lymphatic fluid exceeds the lymphatic transport capacity, an abnormal amount of protein-rich fluid collects in the tissues of the affected area. Left untreated, this stagnant, protein-rich fluid not only causes tissue channels to increase in size and number, but also reduces oxygen availability in the transport system, interferes with wound healing, and provides a culture medium for bacteria that can result in lymphangitis (infection)."

And this lovely description tells us this is a progressive condition. As the fluid collects in the tissues of the affected area, it creates a series of changes that not only causes the swelling to worsen but works on the circulatory and muscular systems as well. Deprived of oxygen and literally squeezed out of space, muscles can atrophy, making it that much harder to move fluid. It's a viscous cycle that once started is the very devil to break unless caught early.

So, we have missing vessels or nodes that create a back-flow in the system. With no where to go, the fluid stagnates. How then is LE treated? Currently, there are only ways to manage this condition, there is no cure. Complete Decongestive Therapy (CDT) is a multi-phased approach used to reduce the swelling and fibrosis (hardening of tissue), improve the skin and increase mobility. First, patients undergo Manual Lymph Drainage (MLD). A therapist physically moves the fluid through the body with a fairly simple form of massage that the patient learns as well. After every session of MLD, patients have the affected limb bound in short-stretch compression bandaging. This provides support and resistance for the limb. Exercises will be used once bandaged to help move remaining fluid. Once the limb has been reduced, the patient will be fitted with a compression garment and encouraged to continue daily MLD on their own.

Let me reiterate: THERE IS NO CURE. Various surgeries have been used over the years, almost always for extreme cases that cause complete disability. They generally do not work and often make things worse. A few years ago, Suzanne Sommers made headlines when she underwent treatment for breast cancer. She also developed LE in her affected arm. She underwent debulking/liposuction surgery. She's an idiot. Here we had a celebrity that could be the face for LE and she defies conventional wisdom and treats her LE as a cosmetic issue. Not a big help.

There is, however, research finally being done. I encourage you to visit the Lymphatic Research Foundation website. There are a whole host of diseases besides LE that are related to the lymphatic system. This organization is doing ground breaking work and pushing forward the boundaries of our knowledge on the lymphatic system. LRF was founded by the mother of a LE patient. She has done more in the past few years to increase research in this vital body system than just about anyone. If you're looking for an organization to donate to, these folks are doing good work.

Just what other conditions are considered part of LRF's mandate? Metastatic cancer is now seen as a lymphatic issue. In fact, without a lymphatic system, there would be no metastasis. It is through the lymphatic system that cancer cells are moved around the body. Autoimmune disorders are being increasingly tied to the lymphatic system. Since the system is the transport for many aspects of the immune system and aids in the removal of cell debris (yes, the lymphatic system is the sewer of the body), it is increasingly seen as tied to a host of autoimmune diseases such as Lupus.

These are just a few. Until recently, the lymphatic system received very little emphasis in medical education. Just a "it's there" and let's move on to those areas we can actually affect change. This is, happily, beginning to change. And it is in no small part thanks to the LRF.

Remember the treatment we discussed, CDT? This has been used in Europe and Australia for almost 4 decades. Until just the last ten years or so, it was considered experimental in the US and was very difficult to find treatment or insurance coverage for. This has changed in part because of the Breast Cancer Patient Protection Act of 2007. This legislation dealt primarily with the idea of drive-though mastectomies, but also addressed post-surgery complications. Unfortunately, since Medicare got involved it mandated a entire set of rules and regulations that actually set back treatment in some cases. MLD therapists are now required to be licensed Physical Therapists. Because MLD was a massage based therapy it was primarily delivered by certified massage therapists. Now those folks either have to go back to school to get a masters in PT or find other work. Way to go Medicare. Also, since Medicare is involved in setting pricing for the treatment and supplies, we'll begin to see fewer providers. Just recently, when I was ordering a new garment, I discovered the company I had been using has stopped providing custom fitted garments. I now have one location I can go to that is covered by my insurance. I can't really give you a good explanation of why Medicare low balls their fee structure but I saw it first hand when I was a Dialysis Tech. Dialysis is one of the few treatment modalities that is completely covered by medicare. Unfortunately, what they pay the centers is insufficient to cover all staffing and treatment costs. If it weren't for those few patients with private health insurance, dialysis centers would find it hard to stay open. This is one of the reasons medical care is so expensive. The idea of Universal Health Coverage is all well and good but the system as it currently stands will not be able to handle the lack of funding that would be inherent in a government-run system. Much will have to change. But that's a whole other ball of wax.

LE is, in short, a progressive condition that must be treated on a daily basis. What this means practically for me as an LE patient is that I must wear some type of compression every day. Right now as I sit here writing this, I am wearing my Reid Sleeves. These are large, bulky boots that reach from toe to hip that are filled with an egg crate foam and adjusted with velcro straps to provide compression. Most days, I wear a compression garment that looks like a pair of panty hose. These garments have come along way in their appearance, comfort and quality over the years. I do self-MLD regularly, though I should be doing it more frequently. I have to take care of my skin almost as if I was diabetic. Any injury, no matter how small, must be dealt with immediately and treated with antibiotic creams. I could be taking a prophylactic dose of oral antibiotics like I was after my last bout with cellulitis but there are risks involved in doing so. Namely, developing antibiotic resistance. Not something I want to contemplate since the last time I had cellulitis was so bad and difficult to treat.

Most days, I go about my business without giving it too much thought. Occasionally, I have pain associated with fibrotic tissue in my legs and the poor circulation resulting. It has become a part of who I am and a limiting factor in some ways. However, I have come to believe that how I approach this mentally is every bit as important as what I do physically. I am probably more handicapped by my weight than I am by my lymphedema. So, I chose to deal with this as someone with any chronic disease must. One day at a time. And an eye to the future.