Tips on scleroderma self management by Alan Tyndall, Professor and Head of Department of Rheumatology, University of Basel, Switzerland – with thanks to my patients, who taught me many of the tips.
The most important first tip is to find a competent specialist in the field and have no hesitation to contact them any time. Scleroderma may have many subtle manifestations which need to be detected EARLY by relatively simple special techniques e.g. echocardiogram for diagnosing high blood pressure in the lungs and for which early treatment is available and effective. The first signs may be just feeling a bit more “puffed” climbing stairs. Many doctors are rather nihilistic about scleroderma treatment, so don’t be fobbed-off with facile explanations.
Staying warm: Core body temperature is as important as extremity temperature, so thermal underwear, hats and socks, plus high quality gloves are critical. Thermal protection must be used BEFORE exposure to cold otherwise a vicious cycle of blood vessel spasm and tissue damage is established. There are gloves with built- in heating battery operated elements, and re-usable chemically triggered heating units for pockets are useful. Be careful that numb hands and feet are not burned by too hot devices! In addition, wet extremities with a wind chill factor will induce blood vessel spasm, even if the ambient temp is not low e.g. swimming areas in summer. Examination-rooms and especially operating theatres should be extra heated for scleroderma patients.
Staying mobile: Scleroderma patients may have an inflammation of the muscles called myositis, and this is often missed, since it is often not painful. There are blood tests (CK level) and MRI examinations which confirm the diagnosis. However, even without this infrequent muscle inflammation, fatigue, stiffness and weakness are common complaints in scleroderma. Stretching, weight training, and supervised light toning exercises with blood oxygen monitoring are anti-stress and very useful for both maintaining fitness and self -esteem. Also, various hand exercise devices keep fingers mobile.
Staying moist: Dryness, especially skin and mucus membranes may be troublesome. Constant moisturising creams and artificial tears plus vaginal lubricants and bronchial mucus softening agents (biosolvon) may greatly reduce the suffering. Obviously smoking, both passive and active are to be strictly avoided and careful dental hygiene is essential. Rapidly progressive dental caries may be the first sign of such a problem, and in some countries a letter from the specialist may enable medical insurance to also cover some dental bills.
Staying infection free: Minor injuries to the extremities may result in poorly healing ulcers which do not respond to antibiotics quickly. Both problems are due to the reduced blood supply to the skin. Therefore protective gloves and shoes in the first instance, plus early local and systemic treatment once established are critical to avoid larger tissue loss. Also a persistent cough and malaise may herald a lung infection called pneumonia, which may be difficult to detect on a simple chest x-ray if some lung scarring is already present.
Staying nourished: Reflux of stomach contents into the food pipe and “lazy bowels” may lead to indigestion and poor food absorption. Small frequent meals, elevation of the head of the bed by one brick’s height plus antacids reduce reflux. Low fat diets and when indicated, bowel motility stimulants and antibiotics may reduce “bloating” and improve bowel function.
Staying human and dignified: The humiliation of fecal incontinence, erectile failure, or a painful tight dry vagina may be difficult subjects to raise with a doctor or even a partner, but should not be ignored in the face of more “dangerous” but easy to talk about problems such as blood pressure and lung scarring. Unless addressed, they may become more destructive to life quality than other issues. There are approaches to ease these problems (carrying extra pads and underpants, rotating antibiotic schedules, penile erectile implantable devices which are not excluded in scleroderma, vagina widening operations etc, and although beyond the scope of this article, are familiar to specialist clinics. Final decisions are very personal but should only be made after the appropriate information and counselling is made available to the patient plus/ minus partner.
Staying “yourself”: Changing physical appearances, especially one’s face, may lead to a feeling of dissociation from self and identity loss. Selective plastic surgery e.g. removal of deposits of calcium or unsightly enlarged vessels on the skin (telangiectasias) may do a lot to improve self -esteem, but don’t forget that love and attraction is not based solely on appearances, despite popularistic clichés to the contrary. Think how you would react if somebody you loved was in a similar situation.
World Scleroderma Day
Having a disease that nobody has heard of is a lonely business. When even the doctors cannot recognise it, or tell you what is going to happen, it is lonelier still. So World Scleroderma Day was created to tell people, including the medical community, what it means to have this disabling disease. June 29 is a day to recognise the bravery of those who live with scleroderma, and to demand equal treatment and equal care for people with scleroderma across Europe.
Every year, on June 29, we campaign for a world in which equal rights, treatments, and care are offered to people with scleroderma, and in which such rare diseases are not forgotten, but afforded the consideration and attention of other more common diseases.