My Desire to Inspire

Systemic sclerosis is a clinically heterogeneous, systemic disorder which affects the connective tissue of the skin, internal organs and the walls of blood vessels. It is characterized by alterations of the microvasculature, disturbances of the immune system and by massive deposition of collagen and other matrix substances in the connective tissue. This review discusses epidemiology and survival, clinical features including subsets and internal organ involvement, pathophysiology and genetics, microvasculature, immunobiology, fibroblasts and connective tissue metabolism and environmental factors. Early diagnosis and individually tailored therapy help to manage this disorder, which is treatable, but not curable. Therapy involves immunomodulation as well as the targeting of blood vessel mechanics and fibrosis. Physical therapy and psychotherapy are also important adjunctive therapies in this multifactorial disease.

CREST is a type of collagen vascular disease. CREST is a type of scleroderma. There are two types -- localized and systemic. Some people with scleroderma also have other collagen vascular disease, or a hybrid of two types (an "overlap" syndrome), so some evaluation is always needed.

The term CREST is an acronym derived from the five most prominent features:

C - Calcinosis - calcium deposits in the subcutaneous layer of the skin.

R - Raynaud's phenomenon - extreme sensitivity to cold or stress causing color changes in the skin and pain.

E - Esophageal dysfunction - swallowing problems caused by scarring, Esophageal Reflux disease.

This is a picture of my right hand with what is called TELANGIECTASIA. This is the "T" part of C.R.E.S.T. Syndrome which is a form of the disease I have.

This is my left foot. The blue swollen toes are caused by Raynaud's Syndrome which is the "R" is C.R.E.S.T.

This is my right foot. Raynaud's syndrome starts with white (no circulation) and turns purple and blue for when circulation starts to return.

This is a full on Raynauds attack of the hand. Notice the circulation cuts off at the knuckles.

Currently I do not show signs of Calcinosis. The "C".

But, I do have sclerodactyly. The "S". This is when the fingers curve and the skin tightens.

Simply put, I have R.E.S.T. which is quite ironic eh?.

 Trigeminal neuralgia(TN) is among the most painful afflictions known. It is characterized by sudden attacks of pain that are typically brief, lasting only seconds to two minutes. These attacks are severe and described as intense, stabbing or electrical shock-like. TN pain occurs on only one side, involving the upper, middle and/or lower portions of the face. Each attack may come on spontaneously (without warning) or be triggered by specific light stimulation (gentle touch or movement), usually in the affected areas of the face. Common triggers include touch, talking, eating, drinking, chewing, tooth brushing, hair combing, water from a shower and kissing. Pinching or pressing these same trigger points will not usually cause TN pain.

     During an attack of TN, the sufferer will almost always remain still and refrain from speech or movement of the face, so as not to trigger further attacks of pain. The face may contort into a painful wince. Early descriptions of TN confused these sudden attacks with seizures, leading to the term tic doloureux or neuralgia epileptiforme. TN attacks rarely occur when the sufferer is asleep, but may be worsened or alleviated by leaning or lying in a specific position. During an attack, TN pain never crosses over to the other side of the face. In rare cases of bilateral TN, (in which pain occurs on both sides) the left and right-sided pains are separate and distinct.

     The disease course of TN is characterized by periods of flare-up (i.e. exacerbations), when painful attacks come on often and may be very difficult to control with medications. Then there are periods of remission, when no pain occurs. However, even during periods of remission, TN sufferers live in fear of their next flare-up. Over time the periods of exacerbation become more frequent and more severe, while the remissions become shorter. Therefore, the need for more aggressive medical or surgical treatments increases as the disease invariably progresses.

 Hypothyroidism means your thyroid is not making enough thyroid hormone. The thyroid is a butterfly-shaped gland in the front of your neck. It makes hormones that control the way your body uses energy.

Having a low level of thyroid hormone affects your whole body. It can make you feel tired and weak. If hypothyroidism is not treated, it can raise your cholesterol levels and make you more likely to have a heart attack or stroke. During pregnancy, untreated hypothyroidism can harm your baby. Luckily, hypothyroidism is easy to treat.

In the United States, the most common cause is Hashimoto's thyroiditis. It causes the body’s immune system to attack thyroid tissue. As a result, the gland can't make enough thyroid hormone.

Polymyalgia rheumatica (PMR) is a disorder of the muscles and joints characterized by pain and stiffness, affecting both sides of the body, and involving the shoulders, arms, neck, and buttock areas. Usually diagnosed in the elderly, there are rare cases (such as myself) of diagnoses before the age of 50. The cause is not known, only associated with autoimmune and pain disorders. There is no cure.