A Secondary Diagnosis

People who develop CRPS are at risk of developing secondary condition and diseases. Unfortunately, this has happened to me. I have been diagnosed with secondary *LYMPHEDEMA* and *SJOGRENS SYNDROME* which are two conditions that most commonly develop in persons with full body crps in stages three/four. Tonight I will only be talking about lymphedema which I have in both legs and both arms.

Lymphedema  (LE) is defined as

“… an accumulation of lymphatic fluid in the interstitial tissue that causes swelling, most often in the arm(s) and/or leg(s), and occasionally in other parts of the body. 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).

Lymphedema should not be confused with edema resulting from venous insufficiency, which is not lymph-edema. However, untreated venous insufficiency can progress into a combined venous/lymphatic disorder which is treated in the same way as lymphedema.”

Lymphedema can develop in any part of the body.  The signs and symptoms include:

·         a full sensation in the limb(s)

·         skin feeling tight

·         decreased flexibility in the hand, wrist or ankle

·         difficulty fitting into clothing in one specific area, or ring/wristwatch/bracelet tightness. 

It is important that you seek immediate medical advice if you notice persistent swelling as early diagnosis and treatment improves both the prognosis and the condition.  There are three stages to lymphedema:

·         Stage 1 (spontaneously reversible):

Tissue is still at the "pitting" stage, which means that when pressed by fingertips, the area indents and holds the indentation. Usually, upon waking in the morning, the limb(s) or affected area is normal or almost normal size.

·         Stage 2 (spontaneously irreversible):

The tissue now has a spongy consistency and is "non-pitting," meaning that when pressed by fingertips, the tissue bounces back without any indentation forming). Fibrosis found in Stage 2 lymphedema marks the beginning of the hardening of the limbs and increasing size.

·         Stage 3 (lymphostatic elephantiasis):

At this stage the swelling is irreversible and usually the limb(s) is/are very large. The tissue is hard (fibrotic) and unresponsive; some patients consider undergoing reconstructive surgery called "debulking" at this stage.

When lymphedema remains untreated it may result in several severe consequences, including fibrosis, joint immobility, amputation and life-threatening infections that may require repeated hospitalizations for intensive intravenous antibiotic therapy. Failure to obtain proper treatment, including compression garments, devices and bandages, predisposes the patient to these serious consequences.  protein-rich fluid continues to accumulate, leading to an increase of swelling and a hardening or fibrosis of the tissue. In this state, the swollen limb(s) becomes a perfect culture medium for bacteria and subsequent recurrent lymphangitis (infections). Moreover, untreated lymphedema can lead into a decrease or loss of functioning of the limb(s), skin breakdown, chronic infections and, sometimes, irreversible complications. In the most severe cases, untreated lymphedema can develop into a rare form of lymphatic cancer called Lymphangiosarcoma (most often in secondary lymphedema).

Depending on the severity of the lymphedema, the recommended treatment plan should be determined using an approach based on the Complex Decongestive Therapy (CDT) methods which consist of:

1.      manual massage

2.      bandaging

3.      proper skin care & diet

4.      compression garments (sleeves, stockings, devices such as Reid Sleeve, CircAid, Tribute, as well as other alternative approaches)

5.      remedial exercises

6.      self-manual lymphatic drainage & bandaging, if instruction is available 

I currently attend The Lakeshore Outpatient Clinic twice a week where they perform manual massage and then the therapists put the affected limbs in a sleeve that pumps air around the limb forcing the fluid upwards towards the kidneys.  I was being wrapped using eight different types of bandages from toe to knee; and was taught how to do it so the wraps could be removed for baths.  Today I now wear the Tribute garments; which look like oven mitts and are custom made.  This garment is quite expensive but I believe that it works much better than the bandages and the Tribute is easier to put on and take off.  Prior to being sent to the Lakeshore Clinic, I experienced several onsets of lymphangitis/cellulitis, a severe infection resulting from my LE disease, requiring multiple hospitalizations.

Lymphedema is no small matter.  It is a serious disease and requires a lifelong commitment to keep it under control.  If you have CRPS and suffer from constant edema; please see your doctor and discuss with him the possibility that you may have acquired LE.

Revised © 2005 National Lymphedema Netwo=rk.  Permission to print and duplicate this page in its entirety for educational purposes only, not for sale.  All other rights reserved. 

The information above is adapted from Lymphedema: An Information Booklet, a classic NLN publication now in its 8th edition. This 16 page booklet addresses the lymphatic system, causes of lymphedema, symptoms, lymphangitis (infection), lymphedema risk reduction, treatments, contraindications, diagnostic studies, special warnings and reimbursement issues