Counseling of Patients in Various Connective Tissue Disorders Presenting to Dermatology Outpatient Department

Counseling is a learning-oriented process that empowers individuals, families, and groups to accomplish mental health and wellness.[1] It is of utmost importance to ensure that the patients understand the importance of the instructions provided to them. Connective tissue disorders (CTDs) are immune-mediated multisystem inflammatory diseases that can have overlapping clinical presentations. The patients should be aware of these clinical features to recognize them and present them to the clinician as soon as possible for treatment and to avoid factors causing their exacerbation. These diseases may not be completely cured, but there are treatments to ease suffering and keep the disease under control. Counseling of patients with systemic sclerosis Systemic sclerosis (SSc), an immune-mediated disease, is characterized by skin and internal organs fibrosis and vasculopathy. Raynaud Phenomenon (RP) The principle in managing RP is to maintain the core body temperature, not only to keep the extremities warm. Avoid precipitating factors such as cold exposure, smoking, alcohol, caffeine, vibratory tools, emotional stress, and vasoconstrictive drugs.[2] Yoga and breathing exercises help with stress management.[2] A few simple measures, such as putting hands in warm water or a warm location, such as the axilla, installing a geyser in the home, using warm water for household work, and wearing a woolen sweater, gloves, and socks, can prevent ischemic attacks.[3,4] The windmill maneuver, a measure to manage acute RP, harnesses centrifugal force to increase blood flow through spastic arteries. By rotating their arms backward in a softball pitching motion, patients can achieve symptomatic improvement and decrease the duration of RP attacks. A ”frisbee maneuver” may be used as a substitute to abort RP episodes in patients with difficulty performing the traditional “windmill” maneuver. The frisbee maneuver is easy to use, affordable, and widely accessible. Figures 1 and 2 show the diagrammatic representation of the windmill maneuver. Figure 3 shows the diagrammatic representation of the frisbee maneuver.Figure 1: Diagrammatic representation of windmill maneuverFigure 2: Diagrammatic representation of windmill maneuverFigure 3: Diagrammatic representation of frisbee maneuverGastrointestinal involvement The gastrointestinal tract (GIT) is commonly involved in SSc patients. The management aims for nutritional intervention, improving GIT symptoms, preventing malnutrition-related morbidity, and improving quality of life. The aim of dietary recommendations is to prevent acid reflux and regurgitation of food and liquids. The following measures can be taken to achieve the above goals: intake of several small frequent meals (5–6 times/day), avoid food or liquid intake at least two hours before bedtime and avoid eating while lying down, sit upright during meals and avoid recumbence for at least four hours following a meal to facilitate bolus transmission, take plenty of oral fluids and eat somewhat cold meals to ease swallowing.[5] Other measures include eating soft consistency or pureed foods, taking sips of water or other liquids in between bites to help with swallowing, and taking food as per tolerance. In addition, patients can avoid food and beverages, such as alcohol, carbonated beverages, chocolate and derivatives, coffee, tea, wine, vinegar, spices (pepper, mustard, cloves, oregano), citrus fruits, fermented cheese, whole grains bread, brown rice, whole wheat pasta, whole grains, bran, fat, garlic, and onion.[5] Conseling of patients with dermatomyositis The desired goal in the counseling of patients with myositis is to prevent further muscle damage during the active myositis state and to promote recovery by controlled activity. Patients are instructed to avoid exercising during an active inflammatory flare as it can lead to further muscle damage and worsen myositis. The patient should be advised to perform necessary everyday tasks during the disease’s remission stage without overusing the muscles. Exercise should start as soon as symptoms are under control to maintain muscle strength. They should be designed to strengthen muscles that are not affected by the disease process while protecting the muscles that are affected, with the guidance of a therapist. However, if the patient finds that he has to work much harder to perform everyday activities, the exercise may likely cause undue stress to the involved muscles. Therefore, he should seek advice from a physical therapist to see how best to change the program. Counseling of patients with systemic lupus erythematosus (SLE) SLE is a chronic, multifaceted autoimmune inflammatory disease affecting multiple organs. Photoprotection Ultraviolet (UV) light is well known to exacerbate connective tissue diseases, such as SLE and dermatomyositis.[6] UV radiation exposure may aggravate preexisting skin disease, often resulting in new cutaneous lesions.[6,7] SLE flares may also occur and are typically reported as weakness, fatigue, fevers, or joint pain.[8,9] It is better to avoid sun exposure when the Sun’s rays have high intensity, between 10:00 AM and 4:00 PM.[7] The household chores or any outdoor works should be scheduled at other times to avoid undue sun exposure. Using an umbrella, scarf, or broad-brim hat with >7.5 cm brim whenever going outside provides an add-on protection with sunscreen. Avoiding traveling to sandy areas, beaches, and hill stations is advised. An appropriate clothing pattern that offers better sun protection should be used. Clothes with better sun protection are tightly woven and made of wool and synthetic materials, such as polyester and denim. In addition, old and dark-colored clothes with more distance between the fabric and skin can be used. Ultraviolet protection factor (UPF) increases after the first wash and does not change significantly with subsequent washes. Prolonged wear and tear beyond the “standard” lifetime of a garment may eventually cause thinning of the individual fibers and thus alter the UPF.[10] Glass with a dark tint offers significant protection against UVA and visible light.[7] A broad-spectrum sunscreen around 2mg/cm2 with a minimum sun protection factor (SPF) of 30 should be generously applied to all exposed skin 20 minutes before sun exposure. It must be reapplied every two to three hours, or after swimming or sweating. Daily use of sunscreen is more protective against UV-induced skin changes than intermittent use. Even on cloudy days and while swimming, sunscreen application is important. Sunglasses with 100 percent UV-ray protection reduce the risk of cataracts, and wrap-around glasses provide complete protection.[11] Some medicines can increase the skin’s sensitivity to the sun. Therefore, it is important to discuss such medications with a doctor. Table 1 enlists the various drugs known to cause drug-induced lupus erythematosus.[12,13]Table 1: Enlists the various medications causing drug-induced lupus erythematosusCounseling of patients with Sjögren syndrome (SS) SS patients presenting with ocular dryness and/or abnormal ocular tests should use artificial tear drops at least twice daily, with the frequency increased to as often as hourly, as indicated by symptoms and/or objective signs.[14] Patients should avoid constantly having beverages throughout the day to relieve symptoms of dry mouth. Even sipping on water washes away saliva produced and diminishes its protective effect. Drinks with high sugar and/or acidity should be avoided. Therefore, to treat the symptoms of dry mouth, xylitol gums and salivary stimulants should be used. Patients with poor oral health in the form of missing or severely diseased teeth prefer to choose softer and more carbohydrate-rich foods that are easier to chew. Because symptoms, such as dry mouth, can cause people to select a smaller range of acceptable meals, it is critical to concentrate on eating a nutritious diet. Patients should avoid spicy, acidic, salty, and/or dry foods and carbonated drinks because they might be painful for those with dry mouth or interfere with digestion in SS. Consume water frequently (6–8 glasses daily) to maintain proper body functions, saliva production as well as saliva quality. Increased intake of antioxidants can positively affect salivary output, dry eye, and inflammation. Therefore, patients are advised to consume foods rich in omega-3 fatty acids. Counseling for vaccination Various CTDs, along with the use of immunosuppressive drugs, increase the risk of severe pulmonary infections, considering the altered immune status of the patient. Tables 2 and 3 summarize the various vaccinations and their schedule in patients with CTDs.[15,16]Table 2: Recommendations for vaccination in various rheumatic disease patientsTable 3: Schedule of vaccinations in various rheumatic disease patientsCounselling for pregnancy SLE in pregnancy is well tolerated by mothers in remission for at least three months before conception, except those with nephropathy or cardiomyopathy. If conception occurs during SLE’s active stage, around 50% of patients worsen during pregnancy, and a few die or experience permanent renal damage. Patients whose SLE first occurs during pregnancy have a high frequency of severe manifestations. Neonatal lupus may occur in the baby of a mother with lupus. Patients with dermatomyositis and polymyositis may experience deterioration during pregnancy.[17] Table 4 summarizes the recommendations for various immunosuppressive therapies in pregnancy and lactation.[18-20]Table 4: Summarizes the recommendations for various immunosuppressive therapies in pregnancy and lactationRituximab treatment in utero has been shown to diminish neonatal B-cells.[18,21] Live vaccinations should not be administered to infants who have been exposed to in-utero biological immunosuppressants until six months of age. The mother should be aware of this fact and should inform her child’s pediatrician. However, evidence is now available that shows no higher prevalence of congenital abnormalities with rituximab. Rituximab may also be linked to spontaneous abortion and premature birth. Therefore, it is advisable not to conceive till one year after rituximab therapy.[18] Counselling for lactation Avoid direct application of topical formulations to the nipple or areola before breastfeeding, and avoid breastfeeding 4 hours after intake of oral corticosteroids. Rituximab is acceptable during lactation, but waiting for at least 2 weeks to resume therapy may decrease the transfer to the infant.[21,22] It is recommended to wait 4-6 hours after the pre-injection antihistamine dose before resuming breastfeeding.[21,22] Role of psychological support Dermatological conditions are associated with high morbidity and dermatology patients report a wide range of biopsychosocial challenges that, go beyond the illness-related distress. Such challenges include psychological distress, anxiety and depression, social challenges such as work, housing and financial issues, as well as relational issues. Timely and appropriate psychosocial assessment is paramount to ensure the quality of care and person-centered practice. It is also conducive to a biopsychosocial approach in dermatology practice. Studies in other parts of the world have shown that improvement of social support through psychotherapeutic interventions may benefit patients with chronic skin diseases for better social adjustment.[23,24] Conclusion Counseling is a learning-oriented process that empowers individuals, families, and groups to accomplish mental health and wellness. Effective management of any dermatoses depends not only prescribing medicines to a patient but also on providing adequate education and counseling. It improves patient compliance as well as satisfaction with any treatment. It also enhances the doctor–patient relationship. This article has discussed counseling for various CTDs commonly encountered by dermatologists. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Use of artificial intelligence (AI) The preparation of this manuscript was carried out entirely by the author without the use of artificial intelligence technologies.