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Journal of the Dermatology Nurses' Association:
doi: 10.1097/JDN.0b013e318211ff2e
Feature Articles

Cutaneous T-Cell Lymphoma: Mycosis Fungoides/Sézary Syndrome, Part 2

Booher, Susan MS, RN; McCann, Sue Ann MSN, RN, DNC; Tawa, Marianne C. RN, MSN, ANP

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Reprinted with permission by the Oncology Nursing Society from Site Specific Cancer Series: Skin Cancer. Muehlbauer, P & McGowan, C(Eds). Chap 4: pp 81-93. Copyright 2009, Oncology Nursing Society. Part 1 appeared in the January/February 2011 issue of the Journal of the Dermatology Nurses' Association.

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PATIENT MANAGEMENT AND EVALUATION

Collaborative Management

A treatment plan established jointly among the CTCL specialist, the patient, and the family is critical to successful management of patients living with this chronic illness. In patients with CTCL, a pivotal discussion point in choosing therapeutic options should hinge upon the treatment goals as discussed with the physician but determined and stated by the patient. Fortunately, a number of therapeutic options exist, as previously described, from which the patient and physician can choose to treat, manage, or potentially induce the remission of CTCL. Multiple factors influencing treatment decisions, compliance, and successful management include prior therapies, general health concerns, projected chronicity, stage and current status of the disease, insurance coverage, access to specialized treatment centers, and social/lifestyle concerns (Vonderheid, 2003). In most cases, CTCL treatment choices are guided by a philosophy of choosing long-term, immune-sparing, and/or immune-enhancing therapeutic options.

Patients with CTCL are offered a number of single- or multiple-agent therapy choices that can require an extensive time commitment, demand lifestyle and work-related changes, and drain financial and social resources on an ongoing basis. All of these issues affect patient compliance with the prescribed regimen and must be carefully considered and openly discussed at each patient encounter.

For patients with more extensive disease or an advanced stage, collaboration among dermatology, radiation oncology, and hematology/oncology disciplines is important. This practice effectively establishes a group of providers available to the patient who may require multiple treatment pathways throughout the illness continuum (Vonderheid, 2003). Because CTCL is a complex dermatologic and oncologic disorder, nursing management requires a multidisciplinary approach that may include collaboration and coordination with the patient's CTCL specialist, primary care physician, social service agent, health insurer, pharmacist, nutritionist, homecare professional, or long-term care facility. The nurse becomes a creative facilitator and advocate when assisting the patient to maneuver through the maze of prescribed treatment regimens and insurance regulations and restrictions. Because CTCL is uncommon, it often is necessary to educate the public, insurers, and other healthcare providers about the disease.

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Symptom Management

The management of the disease symptoms is a critical component in treatment and quality-of-life issues and outcomes. Alterations in skin integrity and comfort are among the most problematic symptoms for patients with CTCL. For some patients, symptoms have a pronounced effect on all aspects of daily living, interfering with sleep, self-care functions, relationships, home and work patterns, and socialization. In a landmark 2005 survey of 930 CTCL-affected members of the Mycosis Fungoides Foundation (now called the Cutaneous Lymphoma Foundation), a 68% total response rate revealed the effect CTCL has on patients' lives. More than half of all respondents in this survey indicated that skin redness (64%), skin scaling (60%), and itching (54%) were bothersome symptoms (Demierre, Gan, Jones, & Miller, 2006). The skin's many functions are impaired or lost, resulting in myriad symptoms (Nicol, 2003) (see Table 1). In addition to the available treatment options, strategies and therapies to concomitantly palliate disease symptoms and restore skin function are keys to successful CTCL management.

Table 1
Table 1
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Frequent and judicious moisturizing regimens are helpful in protecting the skin and enhancing the skin barrier function. Ointments are the most effective at providing and maintaining skin moisture but are not always the most aesthetically pleasing to patients because of the "greasy" residue left on skin and clothing. A cream-based product is the next best alternative; however, some patients find that a self-prepared 1:1 mix of both an ointment and a cream produces the most efficient and aesthetic balance with the desired outcome. Nonmedicated ointments, creams, or lotions should be applied as frequently as needed throughout the day. To enhance the absorption and therapeutic benefit of topical regimens, the product should be applied to slightly damp skin, which is easily accomplished just after a bath or shower by gently patting the skin rather than thoroughly or vigorously drying it.

The anti-inflammatory properties of topical steroids are an important factor in the symptomatic management of both redness and dryness. Steroid foams, gels, sprays, and solutions allow comfortable and efficient treatment of the scalp or intertriginous areas. Typical application regimens are twice daily to affected areas with built-in interval rest periods (e.g., five days on and two days off). For exfoliative erythroderma, a technique known as steroid wet-wraps may be helpful in soothing extensively inflamed and symptomatic skin. Variations of this technique are available, and patients may adapt it to best fit their own resources, needs, and schedule. Steroid wet-wraps are intended for short-term use (two to three weeks) with built-in breaks from steroid use (M. Geskin, personal communication, February 7, 2006). See Table 2 for suggested method and steps.

Table 2
Table 2
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Antipruritic measures are essential to controlling itch and improving quality of life. Unfortunately, for some patients, itching does not diminish regardless of what measures are tried. Multiple antipruritic medications are available that may be used as monotherapy or in combinations. The sedating effects of some antipruritics require caution during daily activities, especially those involving driving or operating mechanical equipment. Alternating a nonsedating agent during waking and work hours with a more sedating agent at bedtime may help some patients to obtain relief and to continue to function normally (see Table 3). Other measures to palliate itch include moisturization and topical applications of steroids as previously described. Additional over-the-counter topical treatments that are helpful in breaking the scratch-itch cycle include preparations containing menthol (a cooling agent), oatmeal (a soothing agent), and/or topical anesthetics such as 1% pramoxine hydrochloride. Lukewarm or cool showers/ soaks, oatmeal baths, or ice pack applications to limited areas followed by moisturization sometimes are necessary to obtain relief. Distraction from the itch sensation is another important weapon in the anti-itch armamentarium. Patients usually report less itching when busy with home or work activities but increased itching at night when trying to slow down for the day (typically during bedtime hours). Home humidification also may help to reduce skin dryness and related pruritus. At times, patients require the addition of sleep aids or antianxiety medications to help to combat unresponsive pruritus. A self-reported pruritus scale of 0-10 (similar to the commonly used pain scale) helps to quantify the intensity of itch and the effectives of any antipruritic measures taken.

Table 3
Table 3
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The sensations of skin tenderness, burning, and pain are less common than itch but are still problematic for some patients. Inflamed skin is sensitive and may be tender to touch or have a constant "sunburned" sensation. Edematous, cracked, thickened, and fissured skin makes range of motion and walking painful. Ulcerated or necrotic plaques and tumors can be painful, especially if infection is present. In very advanced patient tumor burden, the skin may desquamate, creating a burn-like scenario and very painful sequelae. Symptom-reducing measures range from adequate pain medication regimens to more aggressive treatment of contributing factors (e.g., CTCL, infection). Additional nursing measures include position changes, application of topical ointments or creams, cold or warm compresses, and dressings to restore barrier function and combat infection. The use of nonirritating clothing and linens, bed cradles, and pressure-reducing beds may be indicated. Patients with extensive desquamation need specialized care in burn units.

Infection or colonization with Staphylococcus aureus (S. aureus) may be a chronic, recurring problem in CTCL patients. Staphylococcus enterotoxin, a product of S. aureus, may flare the skin in erythrodermic MF or SS via interaction between the malignant and nonmalignant T cells (Woetman et al., 2007). An infectious etiology is important to consider in patients who are unable to clear Staphylococcus enterotoxin from the skin, and these patients should be treated with oral or IV antibiotics as indicated. Prophylactic antibacterial measures also may reduce the number of infectious flare-ups (Jackow et al., 1997). Anti-infective approaches to decrease bacterial counts can include the application of mupirocin ointment to the nares of patient and family member carriers and one of the following bath options (M. Duvic, personal communication, June 22, 2007).

* Add ¼ cup of household bleach to bathtub.

* Add 1 cup of white vinegar to bathtub.

* Add Dakin solution (premixed at pharmacy) to bathtub.

* Use hexachlorophene showers periodically.

CTCL-associated signs and symptoms may include fatigue, anemia, hypoalbuminemia, electrolyte imbalance, weight changes, and peripheral edema. Individual management of these issues involves improvement in skin barrier function, nutritional support, protein and iron supplements, electrolyte repletion, compression therapy for edema related to peripheral vascular disease, and lifestyle modifications to lessen the effects of fatigue on activities of daily living. Creative solutions to balancing work and home activities with frequent rest periods can offset decreased energy and fatigue. Physical therapy and assistive and adaptive devices, such as walkers, canes, or wheelchairs, can help to improve function, safety, and mobility. Visiting nurse and in-home assistance help to improve compliance with prescribed regimens and decrease the care burden on the patient and family.

Complex psychosocial issues require ongoing intervention and evaluation. The Demierre et al. (2006) report on quality of life for patients with CTCL revealed a significant psychosocial effect involving depression, frustration, anger, and embarrassment. Respondents indicated they worried about the seriousness of their disease (94%) and about dying (80%). The survey also revealed body image concerns with feelings of shame (39%) and unattractiveness (62%). Corresponding with these feelings were reports of the illness interfering with sexual activities (47%), social activities (48%), family activities (42%), work activities (45%), and ability to be close to loved ones (47%).

The nurse has a pivotal role in helping patients to address these issues. Creating and maintaining an ongoing, honest dialogue with patients will help to create a positive relationship and build confidence in the healthcare team. Involving patients in decision making, allowing adequate time to discuss concerns and problems, ensuring access to care or answers to questions whenever needed, and providing education and resources related to CTCL help to empower and to provide hope and control for patients and family members. Referrals to other disciplines, such as psychiatry, sex therapy, pastoral care, social and disability services, or in-home care may alleviate illness-related burdens. Assistance with body-image issues includes the use of cosmetic camouflage, clothing choices, and wigs or hairpieces for hair loss. Thorough review, reinforcement, and evaluation of whether patients understand all information, instructions, and procedures discussed during patient encounters are critical components to helping them to cope with CTCL.

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Evaluation

Routine follow-up visits are essential in evaluating treatment outcomes and current disease status. In CTCL, unlike most other cancers, the skin provides a transparent window to disease status and is a reflective mirror of therapeutic response. Although survival curves demonstrate a worsening prognosis for a higher disease stage at time of diagnosis, the burden of disease in the skin remains the most commonly relied upon factor for determining prognosis (Demierre, Kim, & Zachheim, 2003). These factors influence the episodic but repeated use of surveillance monitoring. Patients with stage IA or IB disease generally require less vigilant monitoring than more advanced-stage patients. Also, the type of therapy and disease stage and status will determine the frequency and type of evaluations performed. Many medications and treatments (e.g., vorinostat, bexarotene, denileukin diftitox, alemtuzumab) require routine safety cardiac or laboratory assessments at specified intervals. Repeat flow cytometry and molecular studies, skin biopsies, and periodic CT or PET-CT scans are performed to monitor response and stage. At each evaluation visit, the patient's willingness and ability to comply with the treatment plan should be addressed. Potential limiting factors should be reviewed, such as cultural beliefs, lack of transportation, funds, insurance coverage, or the patient's physical ability to perform the required treatments. Nursing assessments at each patient visit include a thorough skin check to determine the extent of disease burden, evidence of fissuring or open/ ulcerated lesions, signs of infection (e.g., crusting, oozing), and pruritus (excoriations). Psychosocial circumstances, access to care, and treatment compliance issues should be discussed and addressed, as well. Nursing interventions include ongoing emotional support, advocacy, and patient education relative to the disease process, skin care, coping strategies, and quality-of-life issues.

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Patient Education

One of the most important and rewarding aspects of nursing management of patients with CTCL is the opportunity to provide education to the patient and family. The information that nurses provide and reinforce includes topics such as disease process, treatment-specific issues, skin care, coping strategies, and quality-of-life issues.

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Disease Process and Treatment-Specific Issues

Because CTCL is a rare disease, the general public and health insurers know little about it. The problem even extends to healthcare professionals, such as primary care physicians, other medical specialists, nurses, and pharmacists, who do not specialize in the disorder. This situation often leaves patients frustrated about where and how to get consistent help and information on the disease. The nurse who has a specialty practice involving CTCL is an ideal resource for the patient and can help him or her to learn about all aspects of CTCL, including specifics on etiology, prognosis, staging, treatments, side effects, follow-up care, and advocacy issues. General principles of adult education apply and must include a respect for autonomy, sensitivity to the patient's situation and needs, and delivery of information in a private and time-sensitive manner. The nurse also should function as a guide and source for educational resources available to patients through local and national organizations and via the Internet (see Survivor Issues). Patients need reassurance regarding etiology and prognosis because they often feel they may have done something to cause their disease and are worried about how the diagnosis will affect their lifespan. As with most patients who must incorporate a cancer diagnosis into their psyche, time is required to assimilate and accept information. Repetition of information with additional detail and depth as the situation dictates is an ongoing patient education need.

The multiplicity, variability, and recipe of treatment options for CTCL require constant attention to therapeutic instructions, potential side effects, routine monitoring, diet modifications, and relevant follow-up instructions. Encourage patients to report potential side effects promptly to avoid more serious consequences. Reliable drug-specific information now is available via the Internet through the pharmaceutical industry, resulting in readily accessible patient handouts applicable to the prescribed or considered treatment plan. The nurse is instrumental in helping patients to absorb new, ever-changing, multifaceted treatment information. In addition, information handouts on local, regional, or national CTCL clinical trials should be available for patient review and discussion.

Patients benefit from receiving information about their diagnostic test results and monitoring reports. Some time is required to initially educate patients on the relevance of specific findings; however, this up-front effort not only increases patient involvement in their care but also is more likely to promote compliance with long-term therapy requirements.

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Skin Care

A good educational foundation of skin-care principles is essential for patients with a disease defined primarily by its skin manifestations. Rules to live by include hydration, protection from trauma or irritation, prevention of infection, and proactive surveillance. The basic information patients need to know include the following.

* Moisturizing agents can make a difference in skin hydration. Ointments work better than creams, and creams work better than lotions. Experimenting with different products may help patients to determine the best combination for their lifestyle and moisturizing needs. Patients should be instructed to use caution with products used in the shower/bath or in the bathroom itself because some products may make the floor very slippery, and fall precautions are necessary.

* Products that are applied to the skin will absorb better if the skin is slightly damp. After shower or bath, topical applications on damp-dried skin offer the best opportunity to capture and retain moisture on the skin.

* Warm baths or showers (no more than 10-20 minutes in duration) are less likely to dry out and irritate skin than hot water bathing.

* Vigilant moisturizing with nonmedicated products should be done as often as needed to maintain hydrated skin.

* At least eight glasses of oral hydration are necessary to promote optimal skin hydration.

Preventing trauma to the skin often is easier said than done. Because CTCL is an itchy condition, scratching is a common, sometimes uncontrollable, behavior. The skin also may be more fragile because of chronic topical steroid use. As such, it is more prone to bruising and skin tears. To prevent as much damage as possible, patients may take several precautions. The nails should be cut short, and the use of scratch tools, such as back scratchers, forks, or doorways, should be avoided. Nonirritating clothing (i.e., without wool or other stiff fabrics) and additive-free laundry soaps and rinses should be used. Caution is required for the removal of any tape or bandages because the skin is easily torn. Elevating the legs of patients with erythroderma or SS may help to alleviate edema, thereby decreasing the amount of associated inflammation and skin breakdown that may follow. Sun-exposure precautions cannot be generalized because some patients are extremely sensitive and any amount of sun exposure will cause a disease flare. Other patients find a therapeutic benefit from the sun and purposefully plan for sun exposure as part of their disease-control regimen. Sun exposure issues are best discussed on a case-by-case basis to determine the best plan for each patient.

Because of the increased risk of bacterial or fungal infections in patients with erythrodermic SS, reviewing infectious signs and symptoms is important so that they may be detected and reported early. Most patients recognize high fever as a sign of infection; however, a low-grade fever and/or shaking chills (rigors) may indicate a more serious infection such as septicemia and must be evaluated with blood cultures. Skin changes, such as oozing, crusting, malodor, tenderness, and open wounds or multiple excoriations, are other indications of potential secondary skin infections. Instruct patients on prophylactic antibacterial and antifungal measures that may be helpful in decreasing bacterial or fungal overgrowth. For patients taking immune- compromising therapies, standard precautions should be used to avoid sick contacts.

Maintaining homeostasis is a challenge for patients who are losing body heat, fluids, and nutrients via the skin. Patients complain of feeling chilled despite hot weather, warm clothing, and a hard-working furnace. Keeping the environment free of drafts and layering clothing may help to increase comfort and preserve body heat. Vitamin, iron, and nutritional supplements to increase calories and protein help to offset metabolic and nutritional losses caused by the impaired barrier function of the skin (McCann, 2007).

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SURVIVOR ISSUES

Because CTCL is a chronic illness, survivor issues revolve around living with a rare disease that has persistent, variable, life-altering symptoms and challenging treatment regimens. The disease affects quality of life in many ways, with significant findings from Demierre's (2006) patient survey (as described previously) indicating that the disease causes 50% of those surveyed to feel discontent with their overall quality of life. Further examples from this survey demonstrate the major effect CTCL has on a patient's life and include

* Activities of daily living and role function (e.g., tiredness [66%], sleep disturbance [59%], missed work [55%], limits in daily activities [50%], limits in family activities [41%], dissatisfaction with coping style [85%])

* Psychosocial concerns (e.g., worry about dying [80%], depression [73%], frustration with the disease [87%], anger about disease [63%])

* Satisfaction with treatment (e.g., not satisfied or not sure how effective treatment was [25%], financially burdened [61%], satisfied with treatment [82%], satisfied with physician explanations of disease [84%]).

Living with CTCL with the goal of improving quality of life involves learning how to find and maximize support, minimize disease symptoms, mitigate treatment effects, embrace advocacy, take advantage of physician experts, use CTCL Centers of Excellence whenever possible, and ensure access to care for the recommended treatments and medications. The rarity of this illness causes many patients to feel alone; therefore, patients should be urged to find and use a network of support that connects patients to each other, current and accurate information, and disease management experts (e.g., physicians, nurses) who are familiar and comfortable with treating CTCL. The caregiver and family involved with the patient with CTCL also require assistance and ongoing support.

Assisting and directing patients who are living with CTCL to develop cancer survivor skills is an important role for nurses. The National Coalition for Cancer Survivorship (n.d.) developed audio-learning modules to help patients to live with and to meet the challenges they face on a daily basis (see Table 4).

Table 4
Table 4
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For each patient with CTCL and family, it is critical for the physician and nurse to address these survivor issues. Open and honest communication about quality-of-life concerns is essential to meeting the holistic needs of the patient. The nurse and physician can address these needs with compassion and provide the patient with

* Sources of disease and treatment information

* Support, coping, and advocacy mechanisms and strategies

* Access to available financial aid.

See Table 5 for a selected list of available survivor resources most valuable to patients with CTCL.

Table 5
Table 5
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SUMMARY

MF and SS are rare and complex lymphomas with many presentations, treatments, and challenges. Nursing care is vital for effective and successful disease control, as well as for helping patients to achieve a healthier quality of life. Nurses play a fundamental role to organize and unite the interdisciplinary team to provide comprehensive and effective care to patients with MF or SS. The many facets of the role of the oncology and dermatology nurse include identifying and understanding the best clinical practice for skin care, pain, pruritus, treatment regimens, related psychosocial issues, and patient advocacy. When nurses begin to understand the plight of patients with CTCL, they begin to understand how to best care for them.

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Editor's Note: Many thanks to the Oncology Nursing Society (ONS) for allowing us to reprint, in 2 parts, this chapter from the book, Site-Specific Cancer Series: Skin Cancer, published by ONS. To order, call the Oncology Nursing Society toll-free at 866-257-4ONS or visit ONS online at http://www.ons.org. ONS Member Price: $40, Nonmember price: $60. Part 1 appeared in the January/February issue of the Journal of the Dermatology Nurses' Association.

© 2011 Lippincott Williams & Wilkins, Inc.

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