Vol. 19 Issue 7
A Helping Hand for Scleroderma
Patients with scleroderma can get relief with physical therapy
By Lauren Fritsky
Imagine how tight and unyielding your hand would feel wearing two pair of heavy hospital scrub gloves, one pair over the other.
The sensation shares similarities with what sufferers of scleroderma, a chronic connective tissue condition that causes skin to harden, experience in their hands and other parts of the body. The effects can prove devastating.
"We need our hands to do everything. When you touch the hands of patients with advancing disease, the skin feels very taut and thick," said Jane Brandenstein, PT, who works at the Centers for Rehab Services in Pittsburgh and presented at a national scleroderma conference last year. "Untreated, the metacarpophalangeal (MP) joints extend, and the proximal interphalangeal (PIP) and distal IP joints (DIP) flex, yielding a claw-like hand."
But scleroderma sufferers can get some relief in all affected areas of the body, including their hands, with physical therapy.
A Passion is Born
Brandenstein became interested in rheumatology while working in the inpatient unit of the University of Pittsburgh Medical Center many years ago. The clinical research unit there at the time tested scleroderma patients and sometimes administered PT and OT.
"I had worked in rheumatology since I graduated from school and took an interest in the patients, thinking that PT and OT interventions could offer hope and actually benefit them," she said. "We would have about 60 minutes to teach all we knew about the disease and how important exercise is. The feeling at the time was the symptoms would get worse, but later get better, and patients needed to remain as mobile as possible. This has proven true in many patients."
Scleroderma is categorized as one of the autoimmune rheumatic diseases. It causes the affected skin to harden, itch, feel tight or lose feeling. The condition can progress and its seriousness depends on what parts of the body get affected.
"Generally we think 'skin,' but the heart, lungs and kidneys are all connective tissue and can be involved," Brandenstein added. "In a nutshell, skin is a combination of collagen and elastic fibers. In scleroderma, the manufacture of collagen overtakes the elastic."
About 300,000 people in the United States currently suffer from the disease, which affects mostly women between ages 25 and 55. Its cause and cure have not been identified, though many experts believe that certain individuals carry a genetic predisposition for the disease triggered by something in the environment such as a virus or a chemical.
There are two main types of scleroderma, localized and systemic. Localized scleroderma affects the skin and may also lead to muscle weakness from disuse, but it does not affect internal organs.
Systemic scleroderma, also known as systemic sclerosis (SSc), progressive systemic sclerosis (PSSc) or familial progressive systemic sclerosis (FPSS), can affect the skin, blood vessels and/or internal organs. It can produce Raynaud's disease, a condition characterized by blood vessel spasms brought on by cold or stress that causes the fingers and/or toes to become white or blue or red on re-warming. When systematic scleroderma hits the internal organs, it may cause disability or even death.
Brandenstein said one of the challenges scleroderma presents involves the fact that patients manifest the disease in different ways.
"Probably the most frustrating thing about scleroderma is we don't know what is going to happen," she said. "For instance, with pneumonia, we know the deal. Also, for patients, it is not common enough for people to know someone with the disease. They crave good information."
Most patients, however, experience problems in their hands that often proceed up the arm. Symptoms of scleroderma can begin with the characteristics of Raynaud's phenomenon and advance into areas of tight, shiny skin, distal finger tip pits, nail-fold capillary lesions and areas of subcutaneous calcification. Less common symptoms can include swelling of the hands and feet, pain and stiffness of the joints, thickening of the skin, digestive difficulty, oral and facial problems, generalized weakness and Sjšgren's syndrome, which produces dry mucous membranes.
Unfortunately, there's scant research showing that exercise stops the worsening of scleroderma. Full rehabilitation is rarely guaranteed, but function can be retained through PT. Movement can help retard the contractures caused by scleroderma and help the patient maintain strength and cardiovascular health.
"We believe if people continue to use their hands and exercise, they do not develop the worst contractures, but this would be a difficult study to perform," Brandenstein said. "Not to have patients exercise when you believe they must would be philosophically difficult. One can only imagine it you do not exercise or use your hands, being unable to grip or grasp things, the hands would become useless."
Stretching to Function
What can therapists do to help a scleroderma patient retain hand function? "Stretch, stretch, stretch!" Brandenstein said. "The goal is to maintain full fist and keep fingers straight. Sometimes using a paraffin bath or warm water to soften the tissue prior to exercise is helpful to allow the taut skin to stretch better. Also, we encourage them to 'use' their hands rather than delegate things to others. This is definitely a 'use it or lose it' deal."
An article Brandenstein wrote for the Summer/Fall 1999 issue of Scleroderma Foundation Newsline outlines hand and arm exercises that PTs can help scleroderma sufferers do:
Lifting the arms or legs against gravity is actually strengthening and may be the beginning of the program. The client can then move on to elastic loops or therapy bands, cuff weights, or machines for resistance. Barbells are not recommended. Strengthening is encouraged on an every-other day schedule to give the muscles a chance to recuperate from activity. It is important to exercise all joints and both upper and lower extremities.
Start with arms at the sides, keep the elbows straight and bend hips and knees. Turn palms toward the body and raise both arms back over the head, leading with the thumbs as far as possible in a slow, wide arc of motion. Slowly return to the starting position and relax for 10 seconds.
Sideward Elevation (Abduction/Adduction)
Start with arms at the sides and keep elbows straight, palms upward and hips and knees bent. Slowly slide arms out to the sides and up over the head as far as possible, leading with the thumb. Slowly return to the starting position and relax 10 seconds before repeating. Be sure to keep the elbows as straight as possible.
Move the elbows away from the body as far as possible, keeping them up to shoulder level. Bend them at 90 degree angles and rest them on a bed with fingers pointing to the ceiling and palms toward the body. Keep hips and knees bent. Rotate the shoulder while attempting to touch the little finger to the mattress at hip level.
Elbow Flexion and Extension
Start with arms straight at your side, with palm(s) resting on a bed and hips and knees bent. Turn the palm up and bend elbow in an attempt to touch the shoulder. Then turn the palm away from the face and attempt to touch the opposite knee while straightening the elbow as much as possible. Repeat with the other arm.
Finger and Wrist Flexion and Extension
Put arms at the sides and point fingers toward the toes with palms facing body. Start by curling or bending fingers and touching the fingertips to the palms. Then bend the wrist as far as possible away from hips. Slowly straighten the fingers as much as possible and bend the wrist toward the hips. Return to the starting position and relax.
Modifications to exercises are possible, as are devices to help scleroderma sufferers with harder to perform hand functions, such as built-up silverware with bigger handles, toothbrushes, dental floss and holders, Brandenstein said.
"I believe in exercise for fitness, no matter what the diagnosis, and just because a person has scleroderma does not mean they could not or should not be doing this," she said. "So, a walking program, whole body stretching and strengthening are also important."
Lauren Fritsky is assistant editor at ADVANCE. She can be reached at firstname.lastname@example.org