Edema Therapy Examination
The patient was referred to the lymphedema service for edema management and was seen on day 3 for examination of bilateral lower extremity swelling. ET was provided in separate sessions from PT. The patient's main complaints were dizziness and bilateral lower extremity swelling. He reported that his swelling began a month earlier during his last hospital admission. The patient presented with 1+ pitting edema throughout his bilateral lower extremities without abdominal or genital edema and without skin breakdown. Baseline lower extremity circumferential measurements were assessed (see Table 4).
Clinical Evaluation and Plan of Care
Prior to this admission, the patient's course of amyloidosis had been characterized by refractory orthostasis due to autonomic dysfunction. Medical management had been optimized, and there were no plans for medication changes. The patient was admitted because of syncope with out-of-bed activity at home. During the PT and ET examinations, the patient demonstrated lower extremity edema, symptomatic OH, and decreased endurance, which limited his ability to safely transfer out of bed, ambulate, participate in daily activities, perform social roles, or access the community, and increased his risk of injury from syncopal episodes. He presented with hypoalbuminemia in the setting of nephrosis and heart failure, and rehydration alone was expected to cause worsening peripheral edema. Applying external compression was intended to minimize peripheral interstitial fluid accumulations and direct fluid intravascularly. We hypothesized that external compression coupled with progressive functional and exercise training could decrease this patient's lower extremity circumferential measurements, increase his tolerance for out-of-bed activity, increase his functional independence, and optimize his quality of life.
Through edema management and rehabilitation, the patient hoped to reduce his leg swelling and to walk without syncope. Given the patient's recent inactivity and the nature of having a chronic condition, both PT and ET aimed to provide educational interventions targeting the importance of prescribed exercise and developing self-management skills to address edema, OH, and current need for assistance with daily activities. Without such knowledge, the patient had increased risk for deconditioning, skin susceptible to breakdown and infection, increased risk for syncope with possible fall and injury, and loss of functional independence. The therapists recommended follow-up PT 4 times per week and follow-up ET 3 times per week, with home PT services, followed by outpatient edema services, after hospital discharge. See Table 5 for detailed plan of care.
Physical Therapy Intervention
The patient received 8 follow-up PT sessions throughout this admission while wearing either compression bandaging or knee-high antiembolism stockings (see Table 4 for detailed PT interventions). Therapeutic exercise interventions focused on establishing a tolerable bed-level and seated home exercise program (HEP) that the patient could safely perform without risk of syncope. Functional training interventions focused on safe and efficient transitions out of bed, from bed to chair, and from bed to commode. The physical therapist educated the patient on optimal sequencing and hand placement during transfers with a rollator. Verbal cueing and instruction were gradually withdrawn as the patient demonstrated increasing independence with techniques. The patient was educated to rest after each transitional movement and monitor for symptoms of orthostasis. Gait training focused on cardiovascular endurance training while ambulating with a rollator and stair training to allow safe access to the patient's home environment. Caregiver training emphasized patient-guarding technique and reinforcing strategies for managing OH.
The patient demonstrated hypotension after standing transfers and ambulation throughout this admission (see Table 4). He began to consistently identify the sensation of “fatigue” or “weakness” in bilateral lower extremities that limited his gait tolerance and appeared to correlate with positional hypotension. During most PT sessions, the patient's blood pressure was in 70s/40s mm Hg when he required seated rest due to these leg symptoms. When sitting to rest and asymptomatic, his blood pressure was usually 80s/50s mm Hg or higher. The patient's blood pressure symptoms consistently resolved with seated rests incorporating leg exercises to reduce venous pooling. Vitals were assessed immediately upon sitting after walking and after approximately 2 minutes of rest. The physical therapist educated the patient to continue resting if still symptomatic, though 2 minutes was typically sufficient.
On day 10, the patient initiated stair training, ascending and descending 4 stairs nonreciprocally with 1 handrail and minimal assistance provided by 2 therapists for postural control and safety. Once sitting, the patient reported increased “fatigue” in his legs as compared with ambulating on level surfaces and demonstrated blood pressure of 76/48 mm Hg. Since the patient had 8 stairs to enter his home and 11 stairs to access his bedroom, stair training became the focus of PT to prepare the patient for discharge home. As a result, less time was spent on endurance training on days 11 and 13 and the patient ambulated less total distance with PT. However, the patient negotiated more stairs each day. On day 13, the patient was able to safely ascend 8 steps, ascend and descend 11 steps, and descend 8 steps with 2-minute seated rests between each bout of activity. The patient negotiated stairs with 2 hands on 1 railing in a sidestepping pattern. He received minimal assistance and cueing to safely place both feet on each step. The patient and his wife were educated on safe stair negotiation strategies including avoiding prolonged static standing, positioning chairs on each landing at home to allow seated rests, and monitoring for onset of fatigue, weakness, light-headedness, or dizziness to avoid syncope. After day 10, the patient was asymptomatic negotiating bouts of 8 or 11 steps, followed by seated rest with 1 exception. On day 17, a physical therapist assistant saw the patient earlier in the day than usual before the patient's second dose of midodrine. The patient reported dizziness with out-of-bed activity, required more frequent seated rests, and demonstrated blood pressure lower than any other PT session.
Upon discharge, the patient was issued a transport wheelchair to ensure safe mobility at home and to attend medical appointments. The patient and his wife were educated regarding proper use and safety with the transport wheelchair and the rollator.
Edema Therapy Intervention
The patient received 7 ET sessions during this hospital admission. Beginning on day 4, multilayer compression bandaging was donned bilaterally including toe wraps, stockinette, cotton rolls, and short-stretch bandages. Bandaging was applied with a spiral technique from the metatarsophalangeal joints to the fibular head and secured with paper tape. The patient reported that the bandaging felt comfortable and supportive. He was instructed to keep the bandages on for 12 to 24 hours and to remove the bandages if he experienced pain, tightness, itching, discomfort, shortness of breath, coldness, or discoloration of his lower extremities or toes. In addition, he was instructed to perform diaphragmatic breathing exercises and ankle pumps as part of his HEP. The primary nurse was also made aware of the compression wearing schedule and contraindications.
During each session, the therapist doffed the compression bandages, performed skin checks, washed, dried, and moisturized the skin, and reapplied the multilayer bandages. Previous instructions and HEP were reinforced. On day 12, the patient was received wearing knee-high antiembolism stockings provided by his medical team. The patient reported increased neuropathic pain throughout both feet, leading him to doff the compression bandages. The therapist recommended trialing the multilayer bandaging without toe wraps in an effort to decrease the neuropathic pain, and the patient was agreeable. On day 14, the patient was again received wearing antiembolism stockings. He reported limited tolerance for the modified bandaging due to exacerbated neuropathic pain. At this point, the therapist shifted to using knee-high compression stockings in follow-up sessions because of decreased bulk, increased ease with donning footwear, and patient preference. The edema therapist recommended gradient compression garments for long-term management to provide graded pressure and reduce peripheral blood pooling in the lower extremities.
The physical therapist reevaluated the patient on day 14. Impairment-level examination revealed improved muscle strength within normal limits in all extremities and an otherwise unchanged presentation. While wearing antiembolism stockings, the patient continued to report leg “fatigue” with ambulation and demonstrated OH after walking. He was able to transfer out of bed and ambulate using a rollator without hands-on assistance and demonstrated improved 6MWT performance from 50 to 325 ft (see Tables 2 and 4 for details). The improvement of 275 ft (83.82 m) is well above the 6MWT minimally clinically important difference of 54 and 50 m established among individuals with chronic obstructive pulmonary disease and older adults with stroke, respectively.16,17
Upon admission, the patient was unable to safely ambulate at home due to syncope. At reassessment, he could ambulate 1560 ft with intermittent seated rests and had developed awareness of when he required seated rests without cueing by identifying a sensation of “fatigue” in his lower extremities. He avoided episodes of syncope during PT sessions. He continued with decreased endurance and increased risk of syncope due to persistent OH, requiring supervision for safety with transfers and ambulation, particularly in the morning. Otherwise, all PT goals were met. The therapist recommended home PT services at discharge to progress ambulation tolerance, to provide stair training, and to increase the patient's independence with accessing his home environment safely.
On day 17, the ET specialist reassessed lower extremity circumferential measurements, and a decrease in leg swelling was observed bilaterally without abdominal or genital edema (see Table 5). The patient denied light-headedness or dizziness with standing transfers since the initiation of lower extremity compression (see Table 3). The patient and his wife verbalized understanding of long-term skin care and agreed to obtain fitted, graded compression garments upon discharge. All ET goals were met. The therapist recommended outpatient ET to assess patient tolerance to the fitted garments.
As a result of therapy, the patient and his wife demonstrated improved understanding of the management strategies necessary to minimize risk of syncopal episodes, falls, and accidental injuries. He became increasingly self-directed and was able to educate caregivers about modifying home environments or activities in ways that afforded him more independence but did not compromise his safety. The patient had increased tolerance for ambulation and had no syncopal episodes this admission. He was independent with an HEP for strengthening and edema management. The patient began using multilayer compression bandaging and transitioned to compression stockings. This allowed increased ease and comfort with donning his shoes. Overall, edema interventions were tolerated and successful despite exacerbation of peripheral neuropathy.
A positive effect was observed in multiple daily laboratory values (see Table 6). Blood urea nitrogen, estimated glomerular filtration rate, and creatinine values all improved, suggesting increased renal perfusion. Creatinine remained higher than normal limits; however, this measure is known to be elevated in AL amyloidosis.9 The BNP level greatly decreased from 2690 pg/mL on day 1 to 1520 pg/mL on day 11, suggesting alleviation in heart failure symptoms and possible correlation with decreasing leg edema.6 The albumin level and weight remained stable, supporting the idea that nonpharmacologic interventions alone were successful in decreasing edema and directing peripheral fluid intravascularly without the use of diuretics or supplemental serum protein, and without creating abdominal or genital edema. Finally, the patient's daily urine output was greater than 1500 mL, indicating adequate fluid intake and lessening the likelihood of dehydration causing his OH.9
The patient continued with OH throughout this admission; however, this is a common sequela of AL amyloidosis with neurologic involvement.1 Typically, the goal in managing autonomic OH is to reduce and control symptoms of hypotension but not necessarily to restore normotension.9 Nonpharmacologic interventions can substantially improve quality of life. Common recommendations include moving from supine to standing positions with gradual postural changes to allow autonomic adaptation, avoiding prolonged recumbency to minimize deconditioning, performing submaximal exercises that avoid straining and maintain venous return to the heart, and ensuring adequate hydration.9 Custom-fitted elastic stockings and abdominal binders may also be recommended to provide compression up to the waist to reduce peripheral blood pooling in the lower extremities and splanchnic circulation.9
With improved self-management skills including compression therapy, progressive exercise training, and caregiver education, the patient demonstrated a reduced risk for syncope and increased independence with meaningful activities of daily living. The patient was discharged home with family support and follow-up home PT services. It is unknown how the patient progressed after discharge, as he was not seen for therapy follow-up at this institution.
Further research is needed to evaluate the efficacy of pharmacologic versus nonpharmacologic interventions for amyloidosis-related orthostasis. One advantage of this report is that all interventions were nonpharmacologic. It would be interesting to observe whether this patient had improved symptoms or activity tolerance throughout the day, as PT sessions were typically in the afternoon (after daily fludrocortisone and 2 doses of midodrine). Patients who are educated in self-monitoring for OH and fall risk-reduction strategies may avoid hospital admissions for OH and syncope. If compression therapy is also introduced when patients are initially diagnosed with amyloidosis, patients may maintain greater functional independence through the natural course of amyloidosis.
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Keywords:Copyright 2019 © Academy of Oncology Physical Therapy, APTA
amyloidosis; compression; edema; lymphedema; physical therapy