Constriction decreases blood circulation, causing a series of changes in skin color. During reduced circulation, the skin will be white, blanched, or pale. As the affected area loses oxygen from decreased blood flow, the color turns blue. When blood flow returns and the area warms up, the skin becomes flushed or red. After the attack subsides and normal circulation returns, the usual skin color is restored. Tingling, numb, and cold sensations may accompany the “blue” and “white” stages. Warmth, burning, or throbbing may accompany the “red” stage. Some patients find Raynaud’s Phenomenon painful.
Minimizing exposure to cold (ex. outdoor weather, air conditioning, reaching into a refrigerator or freezer) and keeping your extremities/body warm is extremely important. Patients should wear mittens or gloves, especially when touching refrigerated or frozen items. In addition, many warming devices are available to protect the hands. Thick socks, hates, ear muffs, and warm clothing made from silk, cotton, wool, or down can help people maintain their body temperature. At home, electric heaters, comforter, and electric blankets can also be used. Keeping the entire body warm helps prevent Raynaud’s Phenomenon attacks. Although avoidance of emotional upset and stress isn’t always possible, various relaxation techniques (self-taught or learned in training courses) have proven effective for some. One example, biofeedback, has been used to increase finger temperature; however, its benefit is still unclear.
People with scleroderma should avoid smoking. Concurrent carpal tunnel, estrogen, and stimulants may also aggravate the condition. To make sure that you are not taking something that may worsen Raynaud’s Phenomenon, your physician should review all of your current medications.
When a Raynaud’s Phenomenon attack does occur, carefully waving the arms in an underhand, circular motion (similar to a softball pitch) may restore blood circulation. Rubbing or massaging the affected area may also work. Other possible options for relieving an attack include a warm bath/shower, heating pads, or hot water bottle on the back.
In a capillaroscopy, the physician assesses severity by examining the patient’s nailfolds under a microscope. The presence of Raynaud’s Phenomenon should not be proved by provocation testing (ex. placing the hands in ice water).
Most of these medications work by dilating (opening up) the blood vessels. Common drugs include vasodilators such as prazosin and calcium channel blockers such as aldipine and nifedipine. Drugs that decrease the stickiness of platelets as well as mild blood thinners such as aspirin may ask improve circulation. In addition, medication for pulmonary arterial hypertension (PAH) may also help prevent and treat Raynaud’s Phenomenon attacks.
This condition also appears in people with lupus, rheumatoid arthritis, and other connective tissue diseases. Many otherwise healthy people experience Raynaud’s Phenomenon without any other illness; this is called Primary Raynaud’s Disease.
Sjögren’s is pronounced “SHOW-grins.” This disease causes the immune system to attack the tear and saliva producing glands, as well as the mucous-secreting glands of the vagina. The unusual dryness of the eyes resulting from Sjögren’s Syndrome may lead to severe irritation and inflammation.
Frequent use of artificial tears and ophthalmic ointments may help lubricate dry eyes. Sipping fluids during the day (ex. a squirt bottle filled with water), chewing sugar-free gum, or sucking sugar-free sour candy may help keep the mouth lubricated. The latter two options stimulate salivary activity. Artificial saliva is also a possibility.
Patients with Sjögren’s Syndrome should make regular visits to an ophthalmologist.
The esophagus connects the mouth and throat to the stomach. The lower esophageal sphincter acts as a gate, opening to allow food to enter the stomach and closing to prevent food from coming back up. The “gate” of systemic scleroderma patients does not close properly, resulting in a backwash of acid. As the food and acid return into the esophagus, the patient feels a burning sensation called heartburn. The acid may cause scarring and a narrowing (stricture) of the esophagus by injuring the lining of the lower portion of the esophagus.
Being overweight and wearing girdles or other tight-fitting clothing can also aggravate this problem. Antacids, especially those in liquid form, can also help neutralize acids. These methods can be used to treat problems of acid reflux and heartburn. However, some antacids can cause constipation or diarrhea. Before choosing over-the-counter products, consult your physician or pharmacist. Your physician may also prescribe antacid medications (ex. proton pump inhibitors or H-2 blockers) to decrease stomach acid production or drugs (ex. metoclopramide) to promote muscular activity and improve esophagus function.
Thus, staying in an upright position after eating is helpful. Other common-sense measures to prevent acid reflux include eating smaller and more frequent meals, avoiding eating for several hours before bedtime, and elevating the head of the bed with wooden blocks (6-8 inches).
It is important to eat slowly and chew thoroughly. Eating softer foods (ex. preparing food in a blender) and avoiding foods that tend to stick in the throat may make swallowing and digesting easier. The physician may periodically dilate the esophagus if it has narrowed significantly.
One possible consequence is the overgrowth of bacteria, leading to diarrhea. If the intestine is stretched, patients may experience a bloated, distended feeling and pain. Malabsorption, in which nutrients remain in the intestine rather than being absorbed into the body, may lead to stool abnormalities and weight loss. The physician may suggest reducing the amount of fatty foods in the diet and increasing carbohydrate intake, or he/she may prescribe antibiotics, supplementary fat-soluble vitamins, and/or iron.
This results in constipation or other colon abnormalities. High fiber intake and at least six to eight glasses of fluids daily, especially water, will help prevent constipation. Fresh fruits and vegetables act as natural laxatives, and exercise can help maintain regular bowel movements. The physician may also recommend stool softeners or bulking agents such as Metamucil.
Collagen build-up thickens lung tissue and causes fibrosis or scarring, and thus oxygen transport into the bloodstream becomes more difficult. This is known as Interstitial Lung Disease (ILD). Symptoms include shortness of breath, decreased tolerance for exercise, and a persistent cough. To detect or confirm pulmonary involvement, the physician may order a chest X-ray, a CAT scan of the lungs, an echocardiogram (ultrasound of the heart), or special breathing tests (pulmonary function tests).
The physician may give medication to decrease inflammation, which is thought to lead to the scarring.
Therefore, scleroderma patients must take every possible measure to avoid further lung damage. In addition to avoiding smoking, a major cause of lung disease, patients should avoid exposure to air pollutants, which may worsen breathing problems. Your physician may suggest deep breathing exercises, a graduated aerobic exercise program, or medications to make breathing easier.
Pulmonary arterial hypertension (PAH) is pulmonary hypertension caused by the narrowing of pulmonary arteries. When the pulmonary vessels have high blood pressure, the right side of the heart pumps harder to move blood into the lungs and pick up oxygen; this can lead to heart failure on the right side of the heart.
People with scleroderma have an increased likelihood for developing PH from several mechanisms, and they often have multiple causes of their PH.