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Editorial

Scleroderma and Person/Patient-Centered Concerns

With the first issue of 2000, our journal’s title was changed to and then suffering abdominal pain from intestinal motility include the word skin and reflect the increasing recognition dysfunction. How does a person with scleroderma deal with that our readers care for people with both skin and wound painful calcium deposits on his/her fingertips? Fingers that are care needs.1 Some diseases, including scleroderma, have a bent and will not straighten and flexed knees that inhibit normal significant impact on the skin. The disease process includes gait have a profound social impact. Unfortunately, most current skin fibrosis, vascular abnormalities, and autoantibody treatment does not improve skin flexibility, leaving only the production.2 Scleroderma often presents with a ‘‘woody’’ option of frequently applying emollient moisturizers to hydrate skin that loses its flexibility; can you imagine your skin being the skin surface and prevent skin breakdown. hard like a tree trunk, not moving, even with pushing and Providers can still help relieve distal digit pain that may be pulling? associated with skin ulcers on the tips of the fingers and toes. Limited scleroderma (a slower developing, more benign form Severe cases of distal digit ischemia may require intravenous of the disease) is usually distal to the neck, elbows, and knees prostaglandin inhibitors and hospitalization. Patients with less with diffuse involvement having proximal lesions as well.2 The severe Raynaud phenomenon often respond to several options Scleroderma Foundation lists other problems encountered by including an angiotensin inhibitor blocker (losartan), calcium- persons with limited scleroderma.3 Known as the CREST syn- channel blocker (nifedipine), or phosphodiesterase type 5 antag- drome, they are calcinosis or calcium deposits in the skin, Raynaud onists (sildenafil, tadalafil). Warm gloves or socks along with phenomenon (discoloration of digits with temperature change), nitroglycerine patches (12 hours on and 12 hours off but never with esophageal dysfunction, sclerodactyly (woody hard digits), and phosphodiesterase type 5 antagonists orally) can also improve telangiectasia (small linear arteriolar vessels on the skin surface). activities of daily living for persons living with scleroderma. Remember, there are many types of scleroderma, and not all people Nurse theorist Martha E. Rogers4 wrote about her human- who have scleroderma will have all of these symptomsVeach istic science for the whole human being: ‘‘Man is a unified whole person may have different combinations. Scleroderma often first possessing his own integrity and manifesting characteristics that presents with skin involvement or Raynaud phenomenon, but are more than and different from the sum of his parts.’’ internal organs can also become involved. Your editors-in-chief have adapted those words and do believe This month’s CE/CME highlights a literature review of that the person with a ‘‘hole’’ (ie, a wound, ulcer, injury, or skin scleroderma with earlier diagnosis and newer treatment modal- changes) is more than the sum of his/her parts. As skin and wound itiestoaddressitssymptoms.Thisisespeciallyimportantgiven healers, we must always be aware of, assess, and address person/ that there is no cure and patients are living longer with the disease. patient-centered concerns as part of the individual plan of care. However, we should focus not only on the skin changes (‘‘holes’’ ElizabethA.Ayello,PhD,RN,CWON,ETN,MAPWCA,FAAN or wounds, digital ulcers, and tight skin lesions) that a person may have, but also the ‘‘whole’’ person. Scleroderma is largely a rheumatologic disease that affects predominantly young fe- Y males (30s 50s). We encourage you to think about how this R. Gary Sibbald, BSc, MD, DSc (Hons), MEd, FRCPC (Med disease impacts quality of life and the lifestyle changes that may Derm), FAAD, MAPWCA, JM be needed. This is especially important during a time of life when young persons are active, focused on their appearance, and the emphasis is on ‘‘doing’’ with their social peers. The restrictions on upper extremity movement that accom- pany scleroderma such as limited mobility and impaired joint References 1. Doan-Johnson S. Welcome to the start of something new. Adv Skin Wound Care 2000;13(1):6. motion are life altering. How does a younger person adjust and 2. Goodfield MJD, Coulson IH. Scleroderma. In Lebwohl M, Heymann W, Berth-Jones J, Coulson live his/her life with frequent, severe pain in the distal fingers IH, eds. Treatment of Skin Disease. 5th ed. Philadephia, PA: Elsevier; 2017. and toes, or the risk for secondary infection, disfigurement, and 3. Scleroderma Foundation. What is scleroderma? www.scleroderma.org/site/PageNavigator/ patients_whatis.html. Last accessed August 20, 2018. even finger amputation from ischemia? Imagine not being able 4. Rogers ME. An Introduction to the Theoretical Basis of Nursing. Philadelphia, PA: FA Davis to open your mouth wide enough to eat solid foods comfortably, Company; 1971.

Elizabeth A. Ayello, PhD, RN, CWON, ETN, MAPWCA, FAAN is the CoYEditor-in-Chief of Advances in Skin & Wound Care; Faculty, Excelsior College School of Nursing, Albany, New York; and President, World Council of Enterostomal Therapists, President, Ayello Harris & Associates, Inc, Copake, New York, President (1999) National Pressure Ulcer Advisory Panel. R. Gary Sibbald, BSc, MD, DSc (Hons), MEd, FRCPC (Med Derm), FAAD, MAPWCA, JM, is the CoYEditor-in-Chief of Advances in Skin & Wound Care; Professor, Medicine and Public Health, University of Toronto, Ontario, Canada; Director, International Interprofessional Wound Care Course & Masters of Science in Community Health (Prevention & Wound Care), Dalla Lana Faculty of Public Health, University of Toronto; and President (2008Y2012), World Union of Wound Healing Societies.