Cancer Rehabilitation.
Cancer as a disease process reminds us often unexpectedly of our mortality.
It frequently compromises our patient's vitality.
Breast cancer.
Breast cancer.
Handicap may be defined as a physical condition that interferes with a patient's ability to engage in social, educational, recreational, and vocational pursuits.
In essence, handicap compromises patient's full integration into personal relationships and family and societal roles
Cancer is caused by both external factors (eg, chemicals, radiation, viruses) and internal factors (eg, hormones, immune conditions, inherited mutations).
Causal factors may act together or in sequence to initiate or promote carcinogenesis.
Ten or more years may pass between carcinogenic exposure or inheritance of a mutation and detectable cancer.
Today, cancer is treated with surgery, radiation, chemotherapy, hormones, and/or immunotherapy
Each year, the American Cancer Society estimates the number of new cancer cases and deaths expected in the United States in the current year and compiles the most recent data on cancer incidence, mortality, and survival using data from the National Cancer Institute (NCI) and using mortality data from the National Center for Health Statistics .
Incidence and death rates are age standardized to the 2000 standard million population in the United States.
When deaths are aggregated by age, cancer has surpassed heart disease as the leading cause of death for persons younger than 85 since 1999
When adjusted to delayed reporting, incidences of cancer stabilized in men from 1995 through 2001 but continued to increase by 0.3% per year from 1987 through 2001 in women.
Cancer sites for which survival has not improved substantially over the last 25 years include the uterine corpus, uterine cervix, larynx, liver, lung, pancreas, stomach, and esophagus
. Neoplastic disease can develop in virtually any organ system.
This unregulated growth injures and compromises organ systems that are functioning normally.
Cancer-related diseases are often treated with therapeutic modalities that, in themselves, compromise normally functioning organ systems.
As a consequence, PM&R practitioners must dynamically respond both to disease progression and to effects of various treatments that may contribute to impairment, disability, and handicap.
. The rehabilitation approach to the treatment of cancer originated with the National Cancer Act of 1971.
This legislation declared cancer rehabilitation as an objective and directed funds to the development of training programs and research projects.
In 1972, the NCI sponsored the National Cancer Rehabilitation Planning Conference.
This conference identified 4 objectives in rehabilitation of patients with cancer.
. Psychosocial support.
Optimization of physical functioning.
Vocational counseling.
Optimization of social functioning.
In the 1970s, a number of models for cancer rehabilitation were initiated and supported through the NCI cancer-control program
Cancer rehabilitation can be defined as a process that assists the cancer patient to obtain maximal physical, social, psychological, and vocational functioning within the limits created by the disease and its resulting treatment
For excellent patient education resources, visit eMedicine's Cancer and Tumors Center and Women's Health Center .
Also, see eMedicine's patient education articles, Bladder Cancer , Brain Cancer , Breast Cancer , Mastectomy , and Ovarian Cancer |
. Multidisciplinary approach to rehabilitation.
. Rehabilitation specialists have proposed several general principles regarding rehabilitation interventions for patients with cancer.
Rehabilitation requires an interdisciplinary team approach because of the variety of potential problems patients may face during the course of illness.
The availability of professionals from major disciplines is essential to offering comprehensive care.
The patient's needs determine the team members involved.
Over the last 3 decades, collaboration between PM&R and the specialty of cancer medicine (ie, oncology) has been growing.
. The healthcare team must develop rehabilitation goals within the limitations of the patient's illness, environment, and social support.
Goals must be objective, realistic, and attainable in a reasonable time to demonstrate gains from active participation in therapy and thereby maintain the patient's motivation.
Patients, family members, and significant others must be active participants in the rehabilitation process.
Patient and family involvement assists in goal setting.
The physiatrist serves as liaison among team members, providing a considerable degree of coordination, especially when rehabilitation and clinical management of the disease are simultaneous
In some settings, social workers lead support groups and actively assist in discharge-planning activities, such as for arranging home-care services and for transfer to other healthcare settings
Patients and their families often have a number of psychological and adjustment issues related to the illness, its treatment, and its resulting disabilities.
The psychologist assesses and treats patients to help them manage their cancer-related psychological distress.
As a member of the rehabilitation team, the psychologist assists other team members when psychological issues, either in patients or their family members, complicate efforts to provide effective therapy.
The goal of consulting the psychologist is to maximize the benefit the patient derives from rehabilitation.
A Danish study determined that compared with the general population, a greater percentage of individuals who have been diagnosed with cancer are hospitalized for depression.
4 According to the report, which investigated depression-related hospitalizations occurring between 1973 and 2003, the relative risk for depression in the first year after an individual had been diagnosed with cancer ranged from 1.16 (in women with colorectal cancer) to 3.08 (in men who had been diagnosed with brain cancer).
The authors concluded that depression must be recognized early and treated effectively in persons who have been diagnosed with cancer in order to avoid the need to hospitalize these individuals for depression.
. Occupational therapist .
. Occupational therapists evaluate patients' ability to carry out tasks related to self-care, including activities of daily living (ADLs), such as dressing, bathing, meal preparation, and homemaking.
These professionals also assist patients to increase ability to perform ADLs, including the use of compensatory techniques and adaptive equipment.
In addition, occupational therapists evaluate home environments for potential modification, and they provide instruction in driving with adaptive devices.
Furthermore, they implement interventions to promote upper-extremity ROM, strength, endurance, and coordination.
. Dietitian .
. Diet and nutrition are important factors in cancer rehabilitation.
A healthy diet and adequate nutrition substantially influence the patient's ability to actively participate in an applied therapy program and are essential for radiation therapy and chemotherapy.
The role of the dietitian is to evaluate the patient's current nutritional status and to provide recommendations regarding his or her specific dietary needs.
Patients with cancer often require dietary supplements and alternative foods.
Dietitians also assist in teaching patients and family members about the importance of appropriate diet in successful rehabilitation.
Speech therapist .
The speech therapist evaluates and treats communication deficits, dysphagia, and cognitive dysfunction in patients with cancer.
The treatment of patients with oral defects or aphasia also falls within the purview of the speech therapist.
This therapist also treats swallowing deficits that result from illness or treatment
Vocational counselors assist patients in adapting to the effect of cancer and its treatment on their employment.
After initial screening, representatives from other disciplines conduct clinical assessments based on the patient's present needs and/or those the care coordinator identifies.
Dietz identified 4 categories of cancer rehabilitation that address the scope and course of the illness.
5 A variety of approaches to rehabilitation of the patient with cancer are described below
Characteristics of patients needing rehabilitation .
Lehman et al in 1978 were among the first authors to investigate the frequency of problems that cancer patients encounter in rehabilitation programs.
6 They screened 805 patients with cancer, as well as psychological and physical problems.
A variety of cancers, including leukemia and cancers of the head and neck, breast, respiratory, nervous system, bladder, and bone, had been diagnosed.
More than 50% of patients had problems associated with physical medicine, with a substantial portion having problems similar to those of other patients undergoing rehabilitation.
. Much of the population had evidence of psychological problems.
Psychological problems were more prevalent in patients with physical problems than in those without physical involvement.
More than 50% of patients with physical involvement had psychological problems, and approximately 29% of patients without physical involvement had psychological difficulties.
In patients with cancer of the nervous system, the incidence of psychological problems was higher than that in individuals with cancer at other sites
The investigators concluded that many patients with cancer have coexisting physical-medicine and psychological problems and that many of these patients may benefit from rehabilitation interventions because their problems are similar to those identified in many other patient populations undergoing rehabilitation
Ganz surveyed 500 patients with colorectal, lung, and/or prostate cancer and found that the typical patient had been living with the disease for more than 3 years.
More than 80% of the sample reported problems with ambulation and, for more than 50%, the problems were severe.
Psychosocial problems varied widely among patients who survive longer than 1 year after their cancer was diagnosed
Needs of patients needing rehabilitation .
Movsas et al confirmed the findings described above.
7 They examined the rehabilitation needs of patients in a different manner in an acute medical setting.
Many patients with cancer had easily remediable but unrecognized rehabilitation problems, such as deconditioning, which indicated the importance of interdisciplinary efforts to preserve patient function.
VanHarten et al devised a questionnaire to address patients' need to receive professional care related to health problems.
8 Although 258 patients with cancer were invited to participate, only 147 completed the study.
The sample consisted of patients with nonmetastatic breast and colon cancer who were living in the community.
Performance of expected roles and mobility were notable problems in 26% of patients.
Relaxation exercises.
Patient education, especially on disease-related matters.
Instruction and counseling of patients and relatives on coping strategies, especially dealing with crisis and fear.
Social and cultural therapy designed to help formulate new and realistic goals in life.
Dietary advice.
In a prospective observational study, Van Weert et al examined 34 patients with cancer-related physical and psychosocial problems.
9 Their 6-week, intensive, multifocal rehabilitation program consisted of 4 components: individual exercise, sports, psychoeducation, and information.
Measurements were performed before and after 6 weeks of rehabilitation to assess symptom-limited bicycle ergometry performance, muscle force, and QOL (on the RAND-36 instrument, Rotterdam Symptom Checklist [RSCL], and Multidimensional Fatigue Inventory [MFI]).
Statistically significant improvements were found in symptom-limited bicycle ergometry performance, muscle force, and several domains of the QOL instruments (RAND-36, RSCL, and MFI).
The rehabilitation program had immediate benefits on physiological variables, QOL, and fatigue
Oncologists were the first practitioners to assess and survey QOL in patients with cancer after the advent of chemotherapy.
Since then, a number of programs intended to ensure QOL have been developed, modified, and used
Satisfaction with treatment, including financial concerns.
Occupational functioning.
QOL instruments clinicians currently in cancer treatment in rehabilitation include the following
Functional Living Index for Cancer (FLIC).
Eastern Cooperative Oncology Group (ECOG) scale.
European Organization for Research and Treatment of Cancer (EORTC) QOL questionnaire.
QOL index.
Cancer rehabilitation evaluation system.
Functional assessment of cancer therapy.
Global adjustment-to-illness scale.
Purpose and emphasis of rehabilitation .
The purpose of rehabilitation for patients with cancer is similar to that for patients with other diseases.
Breast cancer can occur in any adult.
Incidences have been increasing over the last decades for both premenopausal and postmenopausal women.
Although the incidence of breast cancer increases during postmenopausal years, it is the leading cause of cancer death in women younger than 50 years.
Age is not a predictor of complications, but it may affect the patient's outcome, ability to cope, and extent of psychological distress.
Surgery and/or radiation therapy is used for local control and often successful in early-stage breast cancer.
If they are smaller than 5 cm and limited to the breast and axillary nodes, most such cancers may be treated surgically with modified radical mastectomy or breast-conserving surgery.
In both cases, the axilla is usually dissected.
Disease-free survival rates are equal in patients undergoing mastectomy and breast-conservation surgery.
Locally advanced breast cancers are treated with modified radical mastectomy, preceded or followed by chemotherapy.
. At present, the available aromatase inhibitors belong to 1 of 2 classes.
Class I inhibitors irreversibly bind aromatase and have a steroidal structure (eg, exemestane).
Class II agents reversibly bind aromatase and are nonsteroidal (eg, anastrozole and letrozole).
Because of the specificity of its mode of action, this class of compound is well tolerated and thus lends itself to the management of both early- and advanced-stage disease
In metastatic breast cancer, radiation therapy is often successful in palliating symptoms from painful bony sites, brain metastases, or other metastatic sites causing symptoms or obstruction.
Metastatic breast cancer rarely is curable; however, studies are underway investigating efficacy of high-dose chemotherapy followed by peripheral stem-cell rescue of bone marrow to eradicate metastatic cancer
Current issues in breast-cancer management .
Current issues in breast-cancer management include the following
Necessity for axillary-node dissection and/or breast irradiation after wide excision of breast cancer in patients with a good prognosis (eg, those with small tubular, colloid, or mucinous tumors).
Necessity for whole-breast treatment for intraductal carcinoma.
Timing and type of chemotherapy with surgery and radiation.
Utility of high-dose chemotherapy with stem-cell rescue in poor-prognosis breast cancer.
Treatment of young and old women with breast cancer.
Role of estrogen replacement in breast cancer.
Surgery and its acute and chronic morbidity.
. Breast-conserving surgery is increasingly used for many breast cancers because disease-free survival rates are equal for women undergoing either this procedure or non&8211;breast-conserving surgery.
Breast-conserving surgery is associated with improved body image and, perhaps, hastened psychological recovery
Breast-conserving surgery refers to removal of the cancer along with a margin of normal breast tissue and axillary dissection.
In breast-preservation surgery, wide excision implies the removal of a 1- 2-cm margin of normal tissue, whereas in segmental mastectomy, even more normal breast tissue than this is removed
. A relatively uncommon surgical procedure is quadrantectomy.
This is a procedure to remove the quadrant of the breast that contains the tumor plus the underlying pectoral fascia.
No known treatment exists for this adverse effect.
Injury of the medial pectoral nerve results in atrophy of the lateral portion of the pectoralis major muscle.
Injury to the intercostobrachial nerve results in reduced sensation along the medial aspect of the arm, and, in some patients, subsequent disabling neuralgia develops.
Intuition suggests that breast reconstruction offers a woman the opportunity to retain a positive self image, mitigating concern about breast cancer treatment significantly and perhaps even encouraging women to seek earlier diagnosis of breast cancer.
However, the psychosocial benefit of reconstruction is only slight when patients who have undergone surgical reconstruction are compared with patients treated with mastectomy alone.
Breast-preserving surgery affects body image less than mastectomy and breast reconstructive procedures do.
Studies show lower scores for body image in women who have undergone breast reconstruction than in patients who have undergone breast-preserving surgery.
This phenomenon may be related to the complicated nature of reconstructive surgery
Reconstruction of the breast can be accomplished in several ways at any time after surgery.
Breast cancer.
The TRAM flap has become the flap of choice because of the volume of tissue that can be moved.
However, cigarette smoking, diabetes mellitus, and obesity are relative contraindications because of decreased microcirculation.
When the irradiated chest wall is reconstructed, the TRAM flap is preferred because of its vascularization.
. The pedicle TRAM flap procedure requires the entire rectus abdominis muscle for construction of a new breast.
The surgeon rotates the muscle, pulls it up through a previously constructed tunnel in the chest, pockets it out, and molds it into a breast.
Blood supply from the superior epigastric artery and vein remain intact at their source, and they are pulled up with the muscle.
. The free TRAM flap procedure requires only a portion of the rectus abdominis muscle.
The surgeon fully removes a portion of the muscle from the donor site, with blood supply intact from the deep inferior epigastric vein and artery, and reattaches it to the chest wall to reconstruct the breast.
The surgeon then connects the tiny vessels to recipient vessels, most often the thoracodorsal artery and vein in the axilla near the new breast, in a separate microvascular procedure
Some patients may desire an abdominal binder in addition to the supportive bra
At 3 or 15 months after surgery, approximately 80% of patients continue to report at least 1 problem.
Exercises, such as wall climbing, and use of pulley or wand, should be added.
After all sutures are removed, exercises more aggressive than these can be incorporated
Physical modalities may be helpful.
A home exercise program should be implemented, and follow-up PT assessment should be included.
Massaging of scars is usually incorporated into this program around 1 month after surgery.
With radiation treatment, ongoing ROM exercises are particularly important to prevent contracture formation
. Discuss lymphedema precautions with the patient before surgery, and review her condition within several days of surgery.
. Use of radiation therapy after breast-preserving surgery is common to reduce the probability of recurrence in the breast and after mastectomy, when the risk of recurrence in the chest wall is high.
The breast is treated with tangential techniques that also include irradiation of the underlying muscle, rib, and anterior surface of the lung.
After mastectomy, the chest wall is treated with similar techniques, but radiation is delivered after subcutaneous tissue is damaged by production of skin flaps.
The supraclavicular, axillary, and sometimes internal mammary nodes are irradiated when the risk of nodal recurrence is high.
Direct anterior fields are used to treat increased volumes of rib and lung tissue.
The brachial plexus is often in the node fields, but damage is uncommon with standard doses.
Irradiation of the axillary nodes is associated with an increased risk of lymphedema; avoid it unless the risk of recurrence in the axillary nodes is clinically significant
Irradiation exaggerates the effects of surgery.
Fibrosis secondary to radiation in the treatment field may cause the following effects
. Increased obstruction of arm lymphatics (if in the radiation field).
Increased tightness of the chest wall and pectoralis decreasing shoulder mobility (most prevalent in patients undergoing mastectomy).
Pain in subcutaneous tissues, intercostal muscles, or ribs.
Always compare chest radiographs with radiation portal images to confirm the etiology of the disease process
Most patients have subclinical effects of the lung.
In most patients, the diffusing capacity of carbon monoxide decreases but returns to normal levels by 24 months.
However, patients who smoke cigarettes have greater deficit and less recovery than those who do not smoke.
Cigarette smoking affects the tolerance of the lung to radiation; therefore, encourage patients to stop smoking.
Permanent injury to the lung because of interstitial fibrosis is localized to only the radiation field and can be identified on lung radiographs.
Long-term effects of lung fibrosis are related to the volume of irradiated lung and to the patient's pulmonary status before irradiation
. Radiation-induced brachial plexopathy is characterized by shoulder discomfort and progressive paresthesias and weakness in the arm and hand.
No treatment, other than symptomatic management, is known.
Examine patients annually because of a possible risk of endometrial carcinoma secondary to tamoxifen.
In the adjunct setting, chemotherapy is usually administered in 4-6 cycles of 3-4 weeks.
The clinician must anticipate these concerns, particularly nausea, hair loss, and lifestyle changes, when introducing the topic of chemotherapy.
Immediate effects of chemotherapy include general fatigue, as well as nausea and vomiting, which are effectively countered with medication, including prochlorperazine, lorazepam, ondansetron, and granisetron.
Patients often gain weight because food may relieve nausea, and their basic metabolic rate may decrease.
Fatigue can be overwhelming and affect exercise and activity levels.
Work and family issues may be important during chemotherapy because treatment can last for many months.
During therapy, many women have a diminished immune status, which puts them at risk for infection.
These periods are short, but some women require increased intervals between chemotherapy cycles or use of growth factors, which are associated with their own adverse effects.
Further results from this trial are awaited with interest, particularly because preliminary results from other studies have not yet confirmed these findings.
. Encourage women to be active and to seek support.
Evidence suggests that participating in support groups or having a confidant increase probability of survival.
Continuation of regular activities during chemotherapy is beneficial.
In 1 study, 41% of women found that treatment was easier than expected.
The first stage is where pitting is associated with edema and temporarily reduced with elevation of the arm.
In the second stage, the edema does not reverse spontaneously.
Protein-rich edema persists and can lead to proliferation of connective tissue.
With such changes, fibrosis occurs and brawny edema is seen on clinical evolution.
In the last stage, lymphostatic elephantiasis, the patient has enormous volume with cartilage-like hardening of dermal tissue along with papillomatous outgrowths
Injury to organ systems.
. Cancer syndromes, either as a consequence of tumor-induced organ-system injury or of toxic therapeutic interventions, can produce inactivity in the patient.
If long-term catheter use is required, consider a condom catheter in the male patient or intermittent catheterization in the female patient.
Provide a bedside commode for patients with intact spontaneous voiding to allow them to void in a relatively upright position when they can be transferred.
. Head and neck cancer: overview.
. The American Cancer Society estimated that 9880 new cases of laryngeal cancer (7920 in men and 1960 in women) and 3770 related deaths (of 2960 men and 810 women) occurred in the United States in 2005.
An estimated 2500 cases of hypopharyngeal cancer are diagnosed each year.
About 60% of larynx cancers start in the glottis.
Another 35% develop in the supraglottic region, and the remaining 5% occur in the subglottis.
18 , 19 , 20 , 21 , 22 , 23 Swallowing and mastication .
. Swallowing and mastication are the most salient deficits that arise as a result of the treatment of cancer of the oropharynx.
. Swallowing occurs in 3 stages.
The first is voluntary, and the other 2 are reflexive.
Swallowing starts with voluntary contraction of the mylohyoid muscles, which throw the bolus back onto the posterior pharyngeal wall.
The rich sensory innervation, provided by glossopharyngeal nerves, then triggers complex, coordinated movements of the involuntary phases of swallowing.
These movements involve the base of the tongue, the soft palate, the larynx, the posterior pillars of the fauces, and the pharynx
Mastication is a complex process resulting from fine and coordinated movements of the mandible at the temporomandibular joint carried out by 4 main muscles originating from the base of the skull, the temporal arch, and the temporal fossa (outer pterygoid, inner pterygoid, masseter, and temporal), and 3 secondary ones on the floor of the mouth (digastric, geniohyoid, and mylohyoid).
Therefore, act of chewing is permitted because of the anatomic and functional integrity of active structures (eg, muscles) and passive structures (eg, mandibular lever, teeth, mucosal lining, salivary glands)
Treatment modalities .
. As with other cancers, the treatment modalities for head and neck cancers depend on the site, size, and histopathology of the tumor and on evidence of metastasis.
The treatment is often defined on a consensus-based system of grading and staging.
. The combination of radiation therapy with concurrent chemotherapy, primarily platinum based, has curative potential in many patients with advanced squamous cell carcinoma (SCC) of the oropharynx, hypopharynx, and larynx.
These treatment regimens are particularly attractive for patients in whom the alternative treatment involves surgical resection of a large portion of the tongue base, oropharynx, hypopharynx, or larynx
The oncologic efficacy of chemoradiation therapy in this population in comparison with radiation therapy alone has been well documented.
However, little detailed information is available on the long-term health-related QOL (HRQOL) outcomes after chemoradiation therapy to treat advanced head and neck cancer or on randomized trials comparing chemoradiation therapy with surgery plus radiation therapy.
Despite this lack, the presumption persists that posttreatment QOL is generally better with chemoradiation therapy than with surgery plus radiation therapy
Circumstances that determine how a person perceives or is affected by performance status (QOL) in a specific area of functioning vary substantially between individuals.
These are subject to adaptation over time, and exert a variable influence on overall QOL for different individuals.
. In addition, the general assumption that nonoperative intervention uniformly leads to superior QOL also fails to account for the potential for a nonfunctioning but anatomically preserved organ.
Some health states in which an organ is preserved (eg, chondronecrosis, chronic aspiration) may be less desirable than not having the organ.
Furthermore, advances in ablative surgical techniques, surgical reconstruction, and rehabilitation after surgery may help preserve and restore function
Patients treated for head and neck cancer can present with some of the most significant posttreatment morbidity of any group of patients with cancer.
Functional deficits can affect nutrition, swallowing, communication, dental health, and the musculoskeletal system.
The usual treatment involves surgery and/or radiation, though chemotherapy is most frequently used as a neoadjunct agent.
Underlying comorbid illnesses or problems, such as alcohol abuse, poor nutritional status, and cardiopulmonary diseases, are more common in these patients than in others
Extensive surgical treatment can lead to visible deficits and may interfere with socialization and employment.
Therefore, functional deficits associated with treatments should be considered with diagnosis of head and neck cancers.
In general, treatment selection is the first step in that process because each treatment at each disease site has specific effects on function.
Success of the palatal bulb prosthesis depends on the capacity of the patient's lateral pharyngeal walls to move inward to meet the prosthesis and achieve velopharyngeal closure during speech and swallowing.
Sufficient space between the prosthesis and the walls of the pharynx is important to enable comfortable nasal breathing, but enough motion of the pharyngeal wall is needed to contact the prosthesis and close off the passageway to the nose at critical times during speech production and swallowing.
Design of this prosthesis can be difficult, particularly in patients who have had radiotherapy to the pharynx because radiotherapy can reduce motion of the pharyngeal wall
Some patients who undergo removal of the soft palate can never wear prostheses successfully enough to provide obturation of the velopharyngeal space, because they have inadequate pharyngeal-wall activity.
In these patients, the prosthesis may need to be large enough that it completely blocks the passage to the nose; therefore, it is uncomfortable.
If the prosthesis is too small, air can pass through the nose, leaving the patient with nasality during speech and leakage of food up the nose during swallowing.
Sometimes, optimal results are not achieved despite participation of the most had prosthodontist and speech or language therapist in the design of a palatal bulb prosthesis.
. Surgical procedures involving the tongue .
In general, the percentage of the oral tongue and tongue base that is resected and the nature of the surgical reconstruction govern the extent of the patient's speech and swallowing problems after surgery.
This generalization is true whether patients' disease is at an anterior or posterior site.
Good oral hygiene.
Bone of adequate quality and volume and in a suitable arch relationship.
Adequate oral function (particularly of the tongue and for swallowing).
No medical contraindications to further surgery.
Some surgeons and radiation oncologists advise the use of hyperbaric oxygen therapy when implants or reconstruction surgery involves viable tissue material, especially if radiation therapy is used at the high dose ranges
Nature of oral reconstruction and its effects .
The nature of reconstruction in the oral cavity after resection of a tumor may substantially facilitate or impair the patient's speech and swallowing abilities.
In general, the best reconstruction is primary closure, in which no foreign tissue from another part of the body is introduced into the oral cavity.
. The voice prosthesis somewhat stabilizes the tracheoesophageal wall.
. The flanges of the voice prosthesis give optimal protection against leakage of saliva and gastric reflux.
. The prosthesis reduces irritation of the stoma and the fistula tract (possibly because of less migration) compared with a feeding tube taped to the skin around the stomal area.
. Because the indwelling prosthesis is positioned flush against the posterior tracheal wall, it does not interfere with a cannula or a heat and moisture exchanger (HEM) after surgery.
Patients can become familiar with maintenance of the voice prosthesis soon after their operation, with the help of the nurses.
. It eliminates early postoperative prosthesis fitting, when the stoma is incompletely healed, when it may still be sore, and when the patient's mental and physical status is not yet optimal.
. Rehabilitation for patients with head and neck cancer begins with treatment planning in which all the previously cited rehabilitation professionals are represented.
At this time, integrate rehabilitation and treatment plans for the patient and provide appropriate counseling.
Arrange for each of the rehabilitation professionals to meet with the patient before treatment begins to define patient's goals.
. Rehabilitation is not a passive process.
The patient must be an active participant.
Allow the dentist and/or maxillofacial prosthodontist and the speech or language therapist time to perform a detailed pretreatment assessment.
The social worker frequently conducts in-depth psychosocial interviews.
Pretreatment assessments become difficult as third-party payment officials authorize shorter and shorter hospital stays for patients undergoing treatment for head and neck cancer.
Patients often enter the hospital the day of surgery.
When possible, hold a pretreatment conference at least 1 week in advance of treatment to notify the rehabilitation professionals of the patient's potential needs and to allow them time to schedule appointments with the patient and relevant others.
. Overall (global) QOL in patients surviving head and neck cancer tends to improve over time and may be better than that of healthy controls.
Speech and swallowing problems (as the patient assesses them) and pain are probably the most important factors determining the patient's general well-being after 12 and 24 months.
However, the relationship is complex, as these items represent only part of 1 of the several domains contributing to the QOL construct.
Therefore, their effect on social, sexual, occupational, and family functioning varies according to circumstances and the individual's coping skills.
Although pain, dysphagia, and psychological distress are important QOL correlates, predictors of QOL may also exist to help identify patients who are likely to have difficulty late in their recovery.
De Graeff et al reported that a high level of depressive symptoms, low performance status, and combined modality treatment were significant predictors of physical and psychological morbidity after treatment.
Hammerlid et al found that depression and physical function at diagnosis were independent predictors of global QOL at 3 years.
These factors should be actively sought.
. Goals of rehabilitation include relief of pain and improved ambulation and function.
The literature about the effectiveness of traditional inpatient measures of patients with a malignancy involving bone is limited.
These individuals often have clinically significant loss of mobility and have much to gain empirically from treatment.
. Bunting et al examined 58 patients with 62 pathologic fractures at various bony sites.
31 The average length of rehabilitation stay, 37 days, was only slightly higher than that for general patients with fractures.
Functional results were mixed.
Twenty-six patients achieving independent transfers; 23, independent ambulation; and 27, improved scores for ADLs.
A total of 34 patients were discharged home, and 7 to other facilities.
The mortality rate was high; 17 patients died.
Hypercalcemia and the need for parenteral narcotics were risk factors for death or a poor result from rehabilitation.
. In a separate study, Bunting et al found that the risk of fracture during PT among patients in an oncology unit was low, involving only 1 of 54 patients.
32 However, 12 patients did have fractures during hospitalization. (The circumstances were not described.).
Allan et al reported results of periacetabular reconstruction in 25 patients with metastatic disease.
33 Only 50% were living 6 months after surgery.
At a mean of 14 months after surgery, all surviving patients had progressed from wheelchair or non&8211;weight-bearing status to restricted weight-bearing ambulation.
They found an overall increase in anxiety and depression in women with tumors in the left hemisphere.
This level decreased postoperatively.
. Few investigators have adequately addressed the cognitive and psychosocial function of adults with brain tumor.
Investigators have generally evaluated only a small number of subjects, using a retrospective designs and/or study-specific questionnaires.
. Hahn et al prospectively examined 68 patients.
35 They intentionally enrolled patients early in the course of treatment, before definitive radiation therapy, to avoid having treatment-associated neurotoxicity confound the results.
An extensive battery of neuropsychologic testing was performed and correlated with patient and tumor factors to identify patients for whom medical and psychological interventions might improve function and QOL.
Patients with left-sided tumors have significantly increased memory loss and decreased verbal fluency and verbal learning.
Neuropsychologic testing did not show deterioration in function with the bilateral cranial insult of total-body irradiation.
However, this result might have been due to laterality and also due to the degree of insult of the tumor versus low-dose radiotherapy.
They also noted that depression was most frequent with left-sided tumors.
. Patients with GBM performed worse than patients with other histologic features.
Why these data show poor performance in patients with high-grade tumors is uncertain.
It may be because of the rapidity with which aggressive tumors progress.
Just as patients with stroke generally have more deficit than other patients with tumors, the rapid progression of high-grade tumors likely cause insult in a timeframe that makes recovery more difficult than it is with a slower process.
. Oligodendrogliomas .
. These tumors start in brain cells called oligodendrocytes.
They spread or infiltrate in a manner similar to that of astrocytomas, and, in most cases, they cannot be completely removed with surgery.
. Oligodendrogliomas sometimes spread along the CSF pathways but rarely spread outside the brain or spinal cord.
Only about 4% of brain tumors are oligodendrogliomas
. Ependymomas .
About 2% of brain tumors are ependymomas.
These tumors arise from the ependymal cells, which line the ventricles.
Ependymomas may block the exit of CSF from the ventricles, causing the ventricles to become large, a condition called hydrocephalus.
Unlike astrocytomas and oligodendrogliomas, ependymomas characteristically do not spread or infiltrate normal brain tissue.
As a result, some but not all ependymomas can be completely removed and cured with surgery.
Spinal cord ependymomas have the greatest likelihood of surgical cure.
Ependymomas may spread along the CSF pathways but do not spread outside the brain or spinal cord
Gliomas .
This is not a specific type of cancer.
Glioma is a general category that includes astrocytomas, oligodendrogliomas, and ependymomas.
About 42% of all brain tumors, including benign ones, are gliomas.
Counting only malignant tumors, 77% are gliomas.
. Epidemiology .
. Metastatic brain tumors are the most common intracranial neoplasms in adults and are a notable cause of morbidity and mortality.
The prevalence of primary brain tumors is 6.6 cases per 100,000 population, and estimated incidences of metastatic brain tumors have varied from 8.3-11 cases per 100,000 population.
The frequency of metastatic brain tumors is thought to be rising because of prolonged survival after primary cancer is diagnosed; this change is a direct result of improvements in early detection and effective treatments.
Individuals with primary lung, breast, melanoma, renal, and colorectal cancers account for most of those with diagnosed brain metastases.
Although most brain metastases come from the lung, melanoma has the highest propensity of all malignant tumors to metastasize to the brain
. In a study by Barnholtz-Sloan et al, the incidence proportions of brain metastases for African Americans was significantly higher than those for white patients in terms of lung, melanoma, and breast cancers and was significantly lower for renal cancer.
. The best diagnostic test for brain metastases is contrast-enhanced MRI.
In the patient who presents with history of systemic cancer and multiple brain lesions, usually little doubt exists about the diagnosis; however, metastases must be distinguished from primary brain tumors (benign or malignant), abscesses, and cerebral infarcts and hemorrhages.
Identify patients with single metastases because their subsequent care may differ from that of patients with multiple metastases.
When contrast MRI does not substantially help in distinguishing brain lesions, perform stereotactic biopsy
Experience with MRI has shown that incidence of multiple metastases is higher than previously was believed.
About 67-75% of patients have multiple brain metastases at diagnosis.
With widespread use of MRI and new improvements in MRI contrast agents and resolution, the proportion of known multiple brain metastases is likely to be even higher in the future.
Diagnosis .
Because many commonly used anticonvulsants have adverse effects and because only a minority of patients with brain metastases develops seizures, withholding anticonvulsants is a reasonable practice unless (or until) the patient has a seizure.
. Corticosteroids serve an important role in the management of acute neurologic symptoms and signs in patients with intracranial neoplasms, as well as in patients with epidural metastasis and peripheral-nerve metastasis.
The mechanism of action of corticosteroids is not completely understood, though edema surrounding the metastatic tumors is frequently reduced.
Dexamethasone is the preferred corticosteroid because it has minimal mineralocorticoid effect and a relatively low tendency to induce psychosis.
More than 70% of patients have symptomatic improvement after starting steroid therapy.
Symptoms from generalized neurologic dysfunction or brain edema respond more consistently to steroids than do focal symptoms, such as hemiparesis
. Clinical effects of steroids are noticeable within 6-24 hours after the first dose and reach maximum effect in 3-7 days.
Despite relatively unimpressive increase in median survival, radiotherapy is effective at achieving local control of disease in many patients.
Data from large retrospective studies show that more than 50% of patients treated with WBRT ultimately die from progressive systemic cancer and not directly from brain metastases.
Best results are achieved in patients with KPS of 70% or greater, patients with an absent or controlled primary tumor, patient younger than 60 years, and patients with metastatic spread limited to the brain (true solitary metastasis)
Current typical radiation treatment schedules for brain metastases consist of short 7-15 day courses) of WBRT with relatively high doses per fraction (150-400 cGy per day) with total doses of 3000-5000 cGy.
These schedules minimize the duration of treatment, while still delivering adequate amounts of radiation to the tumor
. Radiotherapy has its complications, some of which include the following.
Almost all patients have temporary loss of hair, though their hair usually returns 6-12 months after therapy.
. In the short term, patients may have transient worsening of neurologic symptoms while receiving therapy.
Many physicians believe that maintaining patients on steroids during radiotherapy minimizes complications of radiotherapy, though no conclusive proof exists.
. During initial days of treatment, mild symptoms (eg, nausea, vomiting, headache, fever) are common.
This acute reaction may be related to distorted cerebrovascular autoregulation or increased capillary permeability.
Rarely, radiation-induced parotitis and loss of taste occur with cranial irradiation.
. 12 mo) develop symptoms, such as dementia, ataxia, and urinary incontinence.
Many reports of uncontrolled studies of highly preselected patients have been published.
Combined results of several reports suggest that radiosurgery prevents or controls local recurrence in 80-90% of treated metastases with about 5-10% risk of radiation necrosis or new neurologic deficits.
Prospective clinical trials currently underway are expected to help in determining the role of radiosurgery, both in the primary treatment of patients with single metastases and in the management of recurrent brain metastases.
. Use of interstitial brachytherapy, a technique involving placement of radioactive implants in the area of the tumor, has been advocated in selected patients.
Implants allow the delivery of high-dose focal radiation to the tumor while minimizing risk of clinically significant radiation exposure of surrounding normal brain tissue because of rapid fall off in radiation intensity at margins of the precalculated target area.
The procedure is limited to relatively small metastases in surgically accessible regions of the brain.
. Preliminary data suggest that brachytherapy may be effective in selected patients with brain metastases.
The major complication of brachytherapy is radiation necrosis, which may appear as an expanding mass months after treatment.
Biopsy is often required to differentiate tumor necrosis from recurrence; steroids, and surgical resection occasionally helps to reverse neurologic symptoms secondary to radiation necrosis.
The frequency of this complication varies with the amount of radiation administered.
. Along with radiosurgery, brachytherapy may be an additional treatment option for patients with unresectable metastases or who previously received maximal doses of WBRT.
However, the role of brachytherapy in management of brain metastases has yet to be determined.
. Chemotherapy has been used in the treatment of brain metastases from a variety of primary tumors.
However, results have generally been unimpressive.
Although small uncontrolled studies of patients with certain highly chemosensitive tumors (eg, breast cancer, small-cell lung cancer, germ-cell tumors) have been reported, chemotherapy is not usually the primary therapy for most patients, and it is seldom the only therapy.
Given the present data, chemotherapy to small, asymptomatic brain metastases that are known to be chemosensitive seems reasonable.
If progression occurs with the administration of chemotherapy alone, definitive treatment with surgery or radiation may be indicated.
Accumulating evidence suggests that chemotherapy may have a role in treatment of carefully selected patients with brain metastases.
However, the efficacy of chemotherapy in managing brain metastases has not been demonstrated conclusively.
Most systemically administered chemotherapeutic agents that have been proven effective against systemic cancer are ineffective against cerebral metastases from the same cell population.
Delivery of waferlike formulations of chemotherapeutic agents has been studied mostly in primary brain tumors, such as glioblastomas and astrocytomas.
However, they may show promise in the treatment of metastatic tumors
All patients need careful follow-up care, no matter what type of initial treatment they receive for brain metastases.
No standard has been set for the frequency of follow-up neuroimaging studies after treatment.
MRI or CT is clearly indicated any time after therapy if patients develop new neurologic symptoms
For patients treated with surgery, contrast-enhanced MRI should be performed within 5 days of surgery to detect residual disease.
This test is important, especially if the option to forego postoperative radiation therapy is being considered.
If residual disease is present, patients should obviously be given WBRT or IMRT.
For all patients treated with WBRT, follow-up images should be obtained at regular intervals after treatment.
In general, it takes about 6 weeks after WBRT for definite change to appear on images; therefore, patients usually do not need imaging immediately after they complete radiotherapy.
. The most important diagnostic tests are lumbar puncture and contrast-enhanced MRIs of the brain and/or spine.
In selected patients, it may be prudent to rule out a mass lesion with an MRI before lumbar puncture is performed.
If the MRI is unequivocally positive for leptomeningeal metastases, lumbar puncture may be unnecessary.
CSF cytologic studies are positive in 45-50% of patients after 1 lumbar puncture and in more than 90% of patients after 3 lumbar punctures.
. Contrast-enhanced MRI of the brain and/or spine demonstrates meningeal tumor in 30-50% of patients.
The disrupted blood-CSF barrier leads to enhancement of neoplastic meningeal vessels, which appear as a thin rind or as multifocal nodules over the brain or spinal cord.
Enhancement in a pattern consistent with the patient's clinical findings is often considered sufficient evidence of leptomeningeal metastases to justify initiation of treatment, even if results of CSF cytologic studies are negative.
Communicating hydrocephalus can also be a sign of leptomeningeal metastases because meningeal tumor often impairs CSF pathways.
. Leptomeningeal metastases are treated with a combination of radiation therapy and intrathecal chemotherapy.
Radiation therapy can be directed at the entire neuraxis, at symptomatic sites, or at areas of enhancing bulky disease.
Focal radiation therapy to the brain or spine is generally recommended for patients with symptomatic disease.
Intrathecal chemotherapy is given by lumbar puncture or with a ventricular access device such as an Ommaya reservoir.
Use of an Ommaya reservoir may help provide uniform drug concentrations throughout the neuraxis
The survival rates of patients with leptomeningeal disease with and without treatment, because such a poor prognosis, has implications on rehabilitation
The functional deficits, which result from leptomeningeal disease, are variable.
The disease can rapidly progress, with functional decline.
Most patients with systemic cancer develop skeletal metastases, and the spine is most commonly involved.
Spinal metastases are present in 40% of patients who die of cancer.
Autopsy studies have shown that distribution of spinal metastases parallels the bulk of the vertebrae; therefore, the lumbar spine is most often affected, followed by the thoracic and cervical segments.
However, in clinical practice, symptomatic spinal metastases most often involve the thoracic spine (70%), followed by the lumbar segments (20%) and the cervical segments (10%)
Secondary spinal tumors most often originate from primary tumors of the breast, lungs, and prostate, reflecting both the prevalence of these cancers and their propensity to metastasize to bone.
About 10% of patients with symptomatic spinal metastases present with no known primary lesion.
Symptomatic spinal metastases produce a characteristic clinical syndrome beginning with pain, followed by weakness, sensory loss, and sphincter dysfunction.
Local back or neck pain is the earliest and most prominent feature in 90% of patients.
Palpation or percussion over the posterior spine at the affected level usually elicits local tenderness.
Associated radicular pain distribution indicates irritation of segmental roots.
When movement aggravates local back or neck pain, which is then relieved by immobility, suspect spinal instability.
Rates at which spinal cord compression develops vary; however, once established, weakness, sensory loss, and sphincter dysfunction progress to complete and irreversible paraplegia unless timely treatment is undertaken.
. MRI is the imaging modality of choice for spinal tumors, including spinal metastases.
The spine may be evaluated in various planes, and the entire spinal column can be visualized in sagittal cross-sections.
Patterns of extradural metastases can be identified, including an isolated level of focal disease, multiple levels of contiguous involvement, or multiple levels of disparate tumor foci.
MRI with gadolinium enhancement permits identification of typical intradural extramedullary drop metastases typically found along the cauda equina nerve roots and also reveals any intramedullary metastases.
Coronal, sagittal, and transverse reconstructions from MRI provide important information concerning location and geometry of secondary spinal tumors and demonstrate integrity of adjacent vertebral bony elements, all of which are essential for planning optimal treatment
Treatment .
Surgical intervention is often used for radioresistant tumors, such as melanoma, and for stabilization of the spine.
. Parameters of response involve pain and functional status.
These parameters may influence and/or predict survival.
One study reported that pretreatment functional status is maintained in more than 90% of surviving patients for average of 3 years after radiation therapy.
More than 40% of patients who could ambulate before and after radiation therapy for spinal cord compression survived for 1 year, and 20% of this group survived for 3 years after treatment.
In contrast, only 30% of patients who were nonambulatory at presentation, and 7% of nonambulatory patients were alive at 1 year after treatment.
. Techniques treat spinal cord compression with radiation account for factors of radiation dose and treatment volume.
Although a variety of schedules are used, the most common is 3000 cGy administered in 10 treatments (300 cGy per treatment) to the area of the spinal cord compression.
In radiobiologic terms, this regimen is approximately equivalent to administering 3600 cGy by using 200 cGy per treatment in a conventional radiation schedule
Use of an abbreviated course of radiation is considered advantageous in patients who have severe pain because they often have other intervening medical problems.
If tumor involves long segments of the spine or if radiation can exit through viscera (eg, stomach), 3500 cGy is administered in 14 treatments to improve patient tolerance.
This radiation schedule delivers a dose to the spinal cord that is radiobiologically equivalent to 3850 cGy if administered at 200 cGy per treatment.
This combination of different areas involved by the same process has led to use of the inclusive term paraneoplastic encephalomyeloradiculoneuritis.
This syndrome is seen most often in patients with small-cell carcinoma of the lung, but it also may be seen with those with Hodgkin disease or other neoplasms.
The mechanism responsible for this paraneoplastic phenomenon is not understood.
Chronic sensorimotor polyneuropathy is most often observed in association with lung cancer.
According to electrophysiologic criteria, as many as 50% of patients with lung cancer have evidence of peripheral nerve dysfunction.
Most patients are asymptomatic.
About 5% of patients with cancer have clinically significant neuropathies, most of which fall into this category.
The taxoids paclitaxel (Taxol) and docetaxel (Taxotere) have been introduced for cancer chemotherapy.
Approximately 25% of patients require ventilatory assistance.
CSF protein content is elevated in some cases.
Some patients recover completely 1-2 months after the drug is discontinued.
Plasma exchange has been tried in an uncontrolled fashion, with mixed results
The neuropathy gradually improves after discontinuation of the drug.
. Cytarabine .
Cytarabine is used mainly in the treatment of hematologic cancers, often in combination with other agents.
Usual therapeutic doses do not typically cause PNS toxicity.
Although each of these agents has shown some benefit in the prevention or treatment of CIN, none are currently approved by the US Food and Drug Administration (FDA) for this indication.
. Patients infrequently have dysesthetic pain late in the course of peripheral neuropathy or even during recovery.
Severe sensory disturbances result in sensory ataxia and can be debilitating.
Signs and symptoms of peripheral neuropathy commonly worsen for as long as 6 months after treatment and substantially improve at 12 months with continued gradual improvement occurring for as long as 48 months.
Theoretical concern that cisplatin neurotoxicity may be irreversible has not been borne out for most patients
Metastatic involvement of the brachial plexus is documented in 1-5% of patients with cancer referred for neurologic consultation, but the exact incidence has not been defined clearly.
About 70% of these patients have breast or lung cancers.
Pain is the typical presenting symptom, antedating other signs by weeks or sometimes months.
The pain, often severe, and it may involve the shoulder diffusely.
Most characteristically, it extends along the inner aspect of the arm and ulnar side of the forearm and hand.
Weakness and paresthesias are found in more than 70% of patients in a distribution corresponding to the portion of the plexus (eg, C8-T1 root levels).
In the remainder, the entire plexus is involved.
Idiopathic, postinfectious, and familial brachial plexopathies predominantly involve the upper portions of the plexus (eg, C5-7 root levels).
Radiation injury often involves the entire plexus
Surgical exploration is required for diagnosis in a few patients.
Antineoplastic therapy, commonly focal radiation, may be beneficial for pain control, but recovery of neurologic function is uncommon.
Because of this and because of the high frequency of coexisting spinal epidural involvement, early diagnosis is essential to limiting neurologic morbidity from brachial-plexus metastases.
Lumbosacral plexopathy from neoplasia is most commonly related to colorectal cancer, but sarcoma, breast cancer, lymphoma, cervical carcinoma, and a variety of less common pelvic and retroperitoneal tumors may involve the plexus.
Direct extension of tumor from adjacent soft tissue or bone is the mechanism of involvement in 75% of cases.
Like brachial plexus invasion, pain is by far the most common initial symptom, occurring in 70% of patients
Patients often describe a combination of local pelvic and/or sacral discomfort and pain radiating into the leg.
Pain is often present for weeks or months before other neurologic signs and symptoms become apparent.
Sensory disturbance, weakness, and reflex loss involve the lower (ie, sacral root levels) and upper (ie, lumbar root levels) portions of the plexus with approximately equal frequency.
Involvement of the entire plexus occurs in about 20% of patients.
In less than 10%, involvement is bilateral.
In bilateral cases, the lowest sacral roots from each side are involved as they exit the sacral foramen in close proximity.
Low sacral metastases may cause incontinence and impotence without epidural extension
. Myelography demonstrates epidural extension in almost 50% of patients with lumbosacral plexopathy.
Weakness typically is symmetric and most apparent in proximal muscle groups (ie, shoulders, pelvic girdle).
Difficulty working with the arms above the head, as in combing the hair, and difficulty rising from a seated position or climbing stairs are common symptoms.
In addition to distribution of weakness, features that distinguish these disorders from peripheral neuropathies and other causes of weakness are preservation of reflexes and absence of sensory symptoms or signs.
The other major symptom of neuromuscular junction and muscle disease is fatigue.
Excessive fatigue and exertional intolerance are extremely common complaints in patients undergoing treatment for cancer.
The agent of choice is 3,4-diaminopyridine.
Plasmapheresis and immunosuppressive therapies are also effective though the results are modest with regard to symptomatic improvement and electrodiagnostic testing.
. Myasthenia gravis .
The prevalence of myasthenia gravis is 5 cases per 100,000 population.
In 95% of patients, autoantibodies directed against postsynaptic acetylcholine receptors are present in serum.
The disease is characterized by fluctuating weakness and excessive fatigue
Myasthenia gravis is associated with thymoma in about 10% of patients.
. Toxic and metabolic myopathies probably are underrecognized in patients receiving treatment for cancer.
Glucocorticoids are implicated most frequently.
Mild-to-moderate symmetric shoulder and pelvic-girdle weakness with preserved reflexes is considered typical.
Sensory deficits and pain are not found.
The disorder may be related to dose and duration of treatment, but neither of these parameters is well defined.
Synthetic fluorocorticosteroids (eg, dexamethasone) are incriminated more often than nonfluorinated drugs such as prednisone
Laboratory investigations are of limited value in diagnosing this condition.
Muscle-enzyme results (ie, for creatine kinase [CK], aldolase) are normal.
Results of EMG and muscle biopsy are either normal or show nonspecific abnormalities.
Therefore, diagnosis of steroid myopathy is clinical, based on exclusion of other causes of weakness.
Some patients complain of myalgias.
The serum CK level is typically elevated 8-100 times normal.
Unlike in other paraneoplastic syndromes, the underlying malignancy is typically diagnosed simultaneously.
Many of the reported cases were associated with clinical and biopsy evidence of a concurrent fasciitis and synovitis, with the latter resulting in joint contracture.
Donor B lymphocytes seem to be responsible for the antibody production, possibly triggered by antigenic differences in acetylcholine receptors between donor and recipient.
Patients respond to treatment with pyridostigmine, prednisone, and azathioprine
Acute and chronic demyelinating polyneuropathies have been reported in BMT patients.
Many patients with severe demyelinating neuropathies have had GVHD.
Some cases of GBS have been attributed to chemotherapy or infection and have improved with plasma exchange or intravenous immunoglobulin (IVIG).
In other patients, the neuropathy responds to increased immunosuppressive therapy and resolution of the GVHD
Dermatomyositis and polymyositis in adults are associated with increased incidence of cancer.
Incidence of cancer in these patients is 10-15%.
The relative risk of cancer is increased 1.7-3.4 times as determined in a population-based cohort study.
The highest risk is found in women with dermatomyositis.
In this subgroup, risk of ovarian cancer is increased 17-fold; however, unlike many other paraneoplastic neurologic syndromes, myopathies may be associated with a variety of malignancies.
Most common associations are with breast, lung, ovarian, colorectal, gastric, and pancreatic neoplasms.
Algorithm for cancer-related
fatigue.
Fatigue may be caused by the malignancy itself or by cancer treatment and treatment-related anemia.
Physiologic factors known to contribute to CRF are cachexia, deconditioning, and high levels of certain cytokines (eg, interleukin-1, interleukin-6, tumor necrosis factor-alpha).
Psychosocial factors contributing to fatigue include anxiety, depression, and insomnia.
Fatigue is also associated with high levels of other symptoms, especially pain
Several studies indicated that cancer of the lung, GI, urogenital, and hematologic systems provoked the highest levels of fatigue.
Patients with leukemia, non-Hodgkin lymphoma and testicular cancer had the most intense fatigue levels before cancer treatment as compared with individuals with breast, GI, prostate cancer or melanoma.
Patients who had non-Hodgkin lymphoma described a fatigue that was more incapacitating than that of patients with breast cancer, and more distressful and depressing than those with prostate cancer
Patterns of fatigue during the course of cancer treatments vary according to the type of treatment.
Fatigue typically rises sharply after intravenous cytotoxic chemotherapy to a peak 48-72 hours later and drops to near-normal levels 3 weeks later, with a smaller peak occurring on days 10-14 with some regimens.
Studies have not shown substantial increases in fatigue during successive infusions
. During radiation therapy for breast cancer, fatigue levels typically increase linearly over time to a maximum intensity during the fourth week of treatment and then plateau.
Levels of fatigue after radiation therapy return to normal in most patients within 3 weeks to 3 months but are most likely to persist at high levels after chemotherapy.
The NCCN has developed guidelines for the evaluation and treatment of CRF on the basis of available research findings and clinical experience (see the Web site of the NCCN for the most recent guidelines).
This multidisciplinary panel of experts in CRF developed an algorithm in which patients are screened regularly for fatigue by means of a brief screening instrument and are treated according to their level of fatigue and clinical status.
The algorithm includes phases of screening, primary evaluation, intervention according to 3 levels of clinical status, and reevaluation.
. The guidelines recommend that screening for the presence and severity of fatigue occur at the patient's initial contact with an oncology care provider, at appropriate intervals (including the follow-up period after treatment ends), and as clinically indicated.
If the patient reports the presence of fatigue during screening, the fatigue should be quantified for future comparison.
Although a variety of valid and reliable research instruments are available to measure the multiple dimensions of fatigue, many are lengthy and burdensome for patients with CRF.
The guidelines recommend measuring the intensity of fatigue by using a brief clinical instrument such as the 0-10 rating scale commonly used to measure pain.
On the scale, 1-3 is generally considered to be a mild level of fatigue; 4-6, moderate; and 7-10, severe.
An essential component of the focused history is an assessment of treatable factors that are known to commonly contribute to fatigue.
. Rehabilitation of patients with CNS and PNS tumors.
Investigation with regard to the efficacy of rehabilitation for patients with primary brain tumors has been limited.
44 , 45 , 46 , 47 , 48 , 49 , 50 Sherer et al provided preliminary support for the use of treatment approaches originally developed for patients with traumatic brain injury with patients with primary malignant tumors of the brain.
51 Compared with the typical outcome of such patients, their patients had favorable outcomes in terms of community independence and employment.
Gains made during treatment were generally maintained during follow-up studies performed an average of 8 months after discharge.
This finding is relevant because previous studies have shown that similar patients often have a decline in functioning over time.
Furthermore, these improvements in QOL were made with a relatively brief intervention and at relatively low cost.
The average cost and length of treatment was notably less than that for survivors of traumatic brain injury who were treated in the same program
Follow-up revealed that the patients maintained functional gains at 3 months after discharge.
. Of interest, Mukand et al found no significant difference in gains in FIM score between patients with metastasis (ie, 18.6) and those patients with primary brain tumors (ie, 19.8).
52 .. In their 1993 survey of 30 caregivers of patients with brain tumors, Meyers and Boake reported a clear pattern of concerns that appears to differ from concerns of other medically ill populations.
53 The most salient problems patients with brain tumors faced were lack of energy; inability to perform usual activities around the home (ie, paying bills, making repairs); social isolation; lack of sexual activity; general slowing of behavior; and problems with reasoning, memory, and concentration. In contrast to other medically ill populations, caregivers of patients did not endorse certain problems with brain tumors as being worthy of concern.
These nonproblems included depression, ability to perform basic ADLs (eg, dressing, eating), ambulation, and speaking and being understood.
Therefore, it appears that neurobehavioral problems have the largest effect on QOL of patients with tumors and their families
Two studies enrolled a heterogeneous group of cancer syndromes with subanalysis of brain tumor patients.
Marciniak et al reported significant gains across all domains of the FIM.
54 They included patients who presented with metastatic disease, as well as patients receiving radiation therapy.
Medical complication rates, and transfer rates back to acute care hospitals, were higher than those of inpatients without cancer syndromes.
. Cole et al retrospectively examined 200 patients with cancer diagnoses who had been admitted to an inpatient rehabilitation facility.
55 FIM data were analyzed according to motor and cognitive subscore categories.
All groups of cancer syndromes (ie, hematologic, lung, GI, genitourinary, intracranial, breast, gynecologic, miscellaneous) demonstrated significant gains in FIM motor scores.
All groups except patients with intracranial neoplasms and advanced terminal disease had gains in cognitive scores of the FIM
In 3 studies that addressed functional outcomes in patients with primary and secondary brain cancers, investigators did not separate intrinsic brain cancers from metastatic brain cancers.
Primary brain tumors may be classified according to histologic grade.
Unlike metastatic lesions, they may originate at sites other than the cerebral hemispheres, cerebellum, and brainstem.
Patients with low-grade gliomas tended to have relatively long survival, namely, 5-8 years.
Patients with high-grade gliomas tend to have a short survival time, averaging 9-18 months with treatment
. Huang et al reported a comparative analysis of patients with brain tumors versus patients with stroke (ie, cerebrovascular disease).
56 They scrutinized admission, discharge, FIM D-A, and FIM efficiency data.
They separated motor function into mobility and ADLs and documented FIM cognitive scores.
No statistically significant differences were encountered in most of the subjects of the 2 groups
Two studies specifically addressed patients with brain tumors compared with patients with traumatic brain injury.
The study conducted by O'Dell et al (1998) was limited by a small sample.
Nonetheless, results were consistent with those from the earlier study by Huang et al
In a later study by Huang et al, patients with brain tumor underwent comparative analysis with patients with traumatic brain injury.
Careful matching and data collection confirmed results of O'Dell et al. 57 FIM subscores were similar for the 2 groups on admission, except for those on the cognitive subscale.
Patients with traumatic brain injury had low scores.
No significant differences were noted in the discharge FIM scores.
In a study by Catz et al, the overall recovery rate was impressive.
Data from all studies seem to agree that ambulatory function at time of diagnosis is the most important criterion for the outcome of ambulatory function
. They used Frankel grades A, B, and C, with total or partial paralysis and nonfunctional movements that cannot be used for daily activities.
About 57% of subjects who had nonfunctional movements (Frankel grade C) and full or substantial neurologic recovery, with their conditions upgraded to grades D or E.
However, recovery varied according to the severity and etiology of spinal cord lesion (SCL) and to the patient's age at rehabilitation.
As in other studies of traumatic and nontraumatic SCL, recovery was inversely related to the severity of the original neurologic deficit as measured with Frankel grades.
The initial Frankel grade, which was the principal predictor of recovery in previous research, was also a major factor that was predictive of recovery in this study.
Young age at rehabilitation positively affected recovery, but this effect was insufficient to promote recovery from a nonfunctional to a functional state.
This implies that recovery depends on the injury that damaged the spinal cord more than on any of the other potentially affecting factors that were studied.
. Neurologic recovery rate was generally higher than that achieved by previously examined patients with traumatic spinal cord lesions (TSCLs) during rehabilitation.
The NTSCL advantage was most prominent for admission grades A and B, less so for admission grade C.
This and the fact that NTSCL recovery from initial grade D was not significantly different from that of any recovery from initial grade A are probably due to a ceiling effect in patients with NTSCL
In a study by Hirabayashi et al, factors that affected survival after surgery for metastatic spinal tumor included pretreatment neurologic status and tumoral histology.
59 The most significant factor influencing survival was the primary tumor site, as determined from the overall clinical history.
Bone marrow [myeloma], thyroid, or prostate findings were favorable and indicated improved neurologic outcome and survival.
Unfavorable histologic origins were the lung, GI tract, or unknown primary sites.
The type of histology determines radiosensitivity, aggressiveness, and response to cytotoxic chemotherapy and dexamethasone
Postoperative ambulatory function was also a significant factor in the survival of their patients.
Influence from this factor had not been considered in previous reports.
The process by which postoperative neurologic status affects survival is not entirely known.
However, increased susceptibility to intercurrent infections, immobility-related problems such as development of decubitus, and overall deterioration in paraplegic patients with cancer all undoubtedly contribute to a shorter survival time.
Previous studies have shown that poor survival is correlated with these posttreatment clinical factors.
Data from other studies have confirmed that patients with spinal cord injury have reduced life expectancy with both the level and severity of neurologic lesion being important determinations
In their study, Hirabayashi et al focused on postoperative ambulation time as a parameter concerning QOL.
Survival time of patients who could walk after surgery was longer than that of patients who could not walk after surgery.
No difference in survival or ambulation time was observed between patients who were and were not ambulatory before surgery.
Survival of patients who were ambulatory postoperatively was significantly longer than that of nonambulatory patients in general.
For those with metastases from the prostate, liver, and lung in particular, ambulation time after surgery was correlated with survival
However, most studies reveal that pretreatment ambulatory function is the main determinant for posttreatment gait function.
Therefore, symptoms of even minor signs of spinal cord compression should lead to supplementary radiological investigations.
Survival time is short, especially in nonambulatory patients, and it can be improved only by restoring gait function in nonambulatory patients by immediate treatment.
When spinal cord compression is diagnosed, the patient by definition has disseminated disease, which alone may indicate a shorter survival.
The prognostic variables in the inpatient acute rehabilitation setting may be more challenging to identify and reproduce.
Guo et al did not find a prognostic factor that helped identify a subgroup who survived longer than the others and who would therefore benefit from longer rehabilitation.
60 Five studies have been performed to examine rehabilitation outcomes for patients with metastatic spinal cord injury compared with patients with traumatic spinal cord injury
McKinley et al found a pattern of less severe neurologic impairment in subjects with nontraumatic spinal cord injury than in those with traumatic spinal cord injury.
61 50 y) and included more women than the other group
Despite the older age of patients of nontraumatic spinal cord injury, individuals were able to achieve significant gains in FIM during inpatient rehabilitation.
In addition, 90% of patients from both groups were discharged home.
After completion of statistical analysis by using 2 approaches, the FIM score at admission proved to be the most reliable predictive variable.
The FIM score reflects the patient's general clinical situation.
Therefore, the indication for rehabilitative efforts should be based on the patient's clinical status at presentation.
Tumor type and level of lesion should also be considered, but they were less potent than the FIM score as indicators of survival.
The distribution of tumor types (less aggressive in women than in men) may explain the prolonged survival observed in women
63 Patients stayed in the spinal cord unit for an average of 104 days (6&8211;336 d).
One of the most important findings was that some patients with epidural metastatic SCC survived for a long time.
One year after discharge from the spinal cord unit, 52% of the patients were still alive.
Reachers allow patients to retrieve objects that would otherwise be inaccessible.
Dressing and bathing aids minimize the amount of force and coordination required for these self-care activities.
Therapeutic exercise is generally integrated with instruction in compensatory strategies and proper use of assistive devices to develop a composite intervention geared toward maximal autonomy
Orthotics can be helpful in enhancing stability and safety for patients with motor deficits.
Bracing strategies can be applied to protect and stabilize joints controlled by weak muscles, to maintain joints in positions of function, and to compensate for lost motor function.
Truncal orthotics can be cumbersome and are generally reserved for cases of symptomatic vertebral metastasis.
Upper-extremity orthotics can help patients with paresis or plegia involving the distal extremity to grasp and manipulate objects.
AFOs allow patients with anterior tibialis weakness to clear their feet during the swing phase of gait, as with a drop foot.
Knee orthoses prevent buckling in patients with motor deficits affecting the quadriceps.
Knee-ankle-foot orthoses extend distally to encompass the ankle if there is associated weakness of the ankle dorsiflexors.
Orthotic options should be explored for any patient with clinically significant motor deficits irrespective of their prognosis, because function can be substantially improved
Equipment for functional restoration can be prescribed to alter patients' environments and compensate for lost motor capacities.
Ramps can be purchased, allowing wheelchair-dependent patients independent access to their homes.
Ramps, lifts, and railings may be added to homes, when feasible, to greatly enhance patient safety and independence.
Motor and sensory deficits may coexist secondary to plexopathy or peripheral neuropathy.
Therefore, optimal therapy integrates motor and sensory reeducation techniques appropriate to each patients' unique disability.
Physical therapists work on static posture as well as on transfer and gait training, encouraging patients to rely on visual rather than sensory or proprioceptive cues.
Patients are taught to use assistive devices, usually canes or walkers, during ambulation and stair climbing to compensate for reduced sensory input.
Assistive devices may also be required to broaden patients' base of support and enhance their stability
Tactile input received by means of an assistive device held in the upper extremity can supplement for diminished lower-extremity proprioceptive acuity.
The development of safety awareness to protect insensate extremities is a prominent dimension of therapy.
If proprioceptive deficits are severe, orthoses can maintain affected joints in functional alignment for ambulation or grasp.
Many modified utensils for performing ADLs are available with ergonomic alterations that neutralize sensory deficits.
Providing patients with such devices is perhaps the most beneficial intervention in restoring autonomy with home and self-care.
Cutlery, cups, can openers, combs, buttonholers, and toothbrushes are but a few examples of the many items available.
Neoplastic polymyositis/dermatomyositis (PM/DM) have been associated involvement of other organs (heart, lung, and joints) and complications of therapy (infections, osteonecrosis, and osteoporotic compression fractures) that affect disability status aside from the proximal weakness that characterizes the syndrome.
Evaluation before designing the rehabilitation program involves determination of disease stage, impairments, and functional limitations
. Muscle weakness usually begins proximal in hips, then affects the shoulders and anterior neck muscles.
The clinical course of PM/DM is varied; some patients experience an acute fulminant illness lasting a few years, others a remitting and relapsing course or a chronic stable course.
Adults with PM or DM can recover completely or have residual mild-to-moderate weakness and fatigue that is amenable to rehabilitation therapy.
Responsiveness to therapy in PM and DM cannot be predicted at the outset.
. In reviews by Forrest and Krivickas, early phase rehabilitation therapy aims to preserve muscle function, prevent disuse atrophy, preserve ROM, and prevent contractures with passive stretching exercises.
Severity of the clinical problems in the acute stage is related to the severity and distribution of muscle weakness.
If weakness is confined to the shoulder and hip-girdle muscles and if 50% of normal strength is preserved, patients require minimal assistance with ambulation and ADLs.
. Rehabilitation therapy should focus on work amplification, pacing physical activities, gait training, use of assistive devices to reduce fall risk, and instruction for active and passive daily stretching exercise routine to preserve ROM and prevent contractures.
With more widespread involvement of a muscle group and with profound weakness, patients lose functional independence and require substantial assistance with eating, grooming, transfers, dressing, and bathing, and they are nonambulatory.
Weakness of the neck muscles limits bed mobility, transfers, and sitting endurance and is easily assisted with a soft collar.
Neck-muscle weakness predicts swallowing dysfunction due to weakness of the oropharyngeal muscles that may cause aspiration and pneumonia.
Dysphagia occurs in 30-60% of patients with DM.
Patients with such weakness should undergo swallowing evaluation by a speech pathologist to determine the need for implementation of aspiration precautions.
Techniques that may reduce the risk of aspiration pneumonia include upright seating with slight neck flexion and chin tuck with double swallowing.
. Weakness of the respiratory muscles can be monitored by measuring tidal volume with a bedside spirometer each day.
Intercostal muscle weakness reduces thoracic expansion during inspiration, leaving the patient dependent on diaphragmatic excursion to produce adequate ventilatory volumes.
The seated position provides the maximal diaphragmatic respiration.
If the diaphragm muscle becomes weak, ventilatory assistance will be necessary, initially with negative pressure ventilation (cuirass, iron lung, rocking bed) and with bilevel positive airway pressure (BiPAP) or tracheostomy if it progresses
Assistive devices and adaptive strategies are recommended to compensate for muscle weakness, fall risk, ADLs dependency, and reduced endurance.
Patients with hip-girdle weakness and difficulty arising from a chair are helped with an elevated seat cushion, lever-controlled booster seat, raised toilet seat, shower chairs, and hand held shower extensions.
Upper- and lower-extremity orthotics help stabilize joints in position for optimal function.
A wrist splint with a volar shank supports the wrist and augments grip strength.
With quadriceps weakness, a short leg brace with 5&176; of plantar flexion produces an extension moment at the knee, increasing knee stabilization.
However, a brace with dorsiflexion to support a weak ankle will create a flexion moment at the knee and increase fall risk if the quadriceps is weak.
Canes are useful primarily for balance.
Walkers may maintain ambulation if upper extremity strength is sufficient.
Motorized wheelchairs or scooters are important for maintaining community ambulation.
Patient and family education regarding the variability in clinical course, the elements of chronicity with unpredictable remissions and exacerbations, and the importance of compliance with medication and rehabilitation programs are needed for a realistic understanding of the disease process
Prognostication is difficult because of the variability in disease severity and responsiveness to treatments.
Referrals for vocational rehabilitation should be made when it becomes clear that certain functional deficits are likely permanent.
Referral to rehabilitation medicine, occupational therapy, and PT should be made early to design the long-term multimodal therapies that will have to be continuously revised according to the patient's condition.
Fatigue is a distressing component of PM/DM that requires thorough patient education regarding strategies for energy conservation and work simplification techniques to maximize functional level.
Case reports and series reveal promising results without deleterious effects in controlled stable disease
. Burnham and Wilcox indicated that a low- to moderate intensity aerobic-exercise regimen can be a safe and effective means to improve aerobic capacity, body composition, flexibility, QOL, and other measures (energy, fatigue, anxiety) in patients who survive cancer.
65 Furthermore, the exercise regimen was well tolerated, and exercise adherence was excellent.
These findings make a strong case for incorporating low- or moderate-intensity exercise in the rehabilitation of survivors of cancer
With regarding to fatigue, the management may be categorized by clinical strategic approach.
Factors include the following
Energy-conservation strategies: Use sleep-promoting, cognitive-behavioral strategies if patients have insomnia.
Information-seeking strategies Create information packets and teaching materials that address the specific needs of people living with cancer-related fatigue.
Supply written instructions, advice, and information to patients to address cancer-related cognitive impairment due to fatigue.
. Bruera et al showed that patient-controlled methylphenidate improved fatigue, a number of other symptoms, and overall QOL; patients also chose to continue treatment for an additional 4 weeks.
66 Their findings suggest that patients had consistent improvement in their fatigue level throughout the day.
However, because it appears to affect many symptoms, establishing the relationship between fatigue and overall QOL improvement was not possible given the small sample.
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