Lymphoedema is a chronic and debilitating condition, which can have a serious impact on a patient's quality of life. The prevalence and incidence of lymphoedema is difficult to determine, as it is often misdiagnosed or confused with other conditions. However, estimates for its prevalence in the UK range between 80 000 and 124 000 people (Macmillan Cancer Support, 2011).
Dementia is an umbrella term used to describe a range of progressive neurological disorders. It is most often seen in developed countries, where people are more likely to live to an older age and access to health care is more readily available (Dementia UK, 2018).
Dementia UK (2018) reports there are over 850 000 people living with dementia in the UK today. Of these, approximately 42 000 have young onset dementia, which affects people under the age of 65 years. It is estimated that the number of people living with dementia in the UK by 2021 will rise to over 1 million (Dementia UK, 2018).
Currently, there are no figures available for people who have both lymphoedema and dementia.
It is understood that patients who report good communication with their practitioner are more likely to share information, follow advice and adhere to the prescribed treatment. There is an increasing expectation of collaborative decision-making, with practitioners and patients participating as partners to achieve the agreed goals and attain quality of life (Fong and Longnecker, 2010). Compliance, as defined by the Oxford Dictionary (2011) as ‘action in accordance with request, command’, will be affected by many factors. Some of these can be influenced by the practitioner and others are outside the practitioner's control.
Lymphoedema
Primary lymphoedema
In primary lymphoedema, there is an abnormality of the lymphatic system, which has been present from birth. In some people, lymphoedema will be evident at birth, but in others it may not be identified until later in life, possibly following an event which has caused further damage to the system, such as injury, infection or surgical procedure. Some primary lymphoedemas are hereditary (eg Milroy's disease). There are a number of genes associated with syndromes in which lymphoedema features, suggesting potential hope for gene therapy in the future.
Secondary lymphoedema
Secondary lymphoedema arises as a result of damage to a normally functioning lymphatic system. Generally, this falls in to two main categories: cancer- and non-cancer-related lymphoedema. In cancer-related lymphoedema, lymph nodes may have been surgically removed or irradiated. Non-cancer related lymphoedema causes include venous disease, deep vein thrombosis, immobility, chronic inflammation and infection.
Lymphatic system
The lymphatic system has three main functions:
- Maintenance of fluid balance
- Immunity and defence by transporting antigens and immune cells, and generating immune responses to infection and malignant cell antigens
- Fat absorption from the gut and transport back into the circulatory system.
Disruption to the lymphatic system can cause swelling, which may be soft and pitting initially. Over time, and without treatment, the interstitial fluid may be replaced by fibrosis and adipose tissue. Subcutaneous tissues then become thickened and increasingly firm. Disruption may also cause predisposition to cellulitis, which in turn further damages the superficial lymphatic vessels. Furthermore, lymph blisters may develop and areas of lymph leakage or lymphorrhoea may occur.
Treatment of lymphoedema
The aim of treatment for lymphoedema is to reduce swelling in order to increase function, mobility, and tissue health. An important part of lymphoedema management is educating and advising the patient so they can become informed ‘managers’ of their own condition. The management of lymphoedema is divided roughly into two parts; initial treatment and long-term maintenance therapy and selected treatments will depend on the extent, severity and agreed outcome (Box 1).
Box 1.Lymphoedema treatment
- Decongestive Lymphatic Therapy (DLT) - a course of intensive treatment to include compression
- Exercise and physical therapy
- Compression-including intermittent pneumatic compression, bandaging, garments and wraps
- Manual lymphatic drainage (MLD), including teaching simple/self-lymphatic drainage techniques
- Skin care
- Light emission therapy, deep oscillation therapy, negative pressure therapy
- Kinesio taping
- Lifestyle and psychological support
- Surgical options-lymphaticovenular anastomosis, lymphatico-lymphatic bypass, lymph node transfer, liposuction
Dementia
There are more than 200 subtypes of dementia. Regardless of which type is diagnosed and what part of the brain is affected, each person will experience dementia in their own unique way. In order to ascertain particular factors that may influence compliance, it is useful to have a short overview of the most common subtypes of dementia.
Alzheimer's disease
Alzheimer's disease is the most common form of dementia (it accounts for around 60% of diagnoses in the UK), although it is comparatively rare in people who are under 65 years old (Dementia UK, 2018). The exact cause of Alzheimer's disease is unknown, but ‘plaques’ and ‘tangles’ form in the brain due to two proteins – amyloid and tau – creating beta amyloid, which is toxic to the brain cells. In addition, people with Alzheimer's may have reduced levels of acetylcholine affecting transmission of neuromuscular signals.
Symptoms of Alzheimer's disease are mild at first and gradually worsen over time. These symptoms, common to many types of dementia, may include:
- Difficulty remembering recent events (although long-term may be good)
- Poor concentration
- Difficulty recognising people or objects
- Poor organisation skills
- Confusion
- Disorientation
- Slow, muddled or repetitive speech
- Withdrawal from family and friends
- Problems with decision-making, problem solving, planning and sequencing tasks.
Treatment for Alzheimer's disease is generally with cholinesterase inhibitors (National Institute for Health and Care Excellence, 2018).
Vascular dementia
Vascular dementia is the second most common type of dementia and it is also comparatively rare in the under 65s (Dementia UK, 2018). Vascular dementia is caused by disruption to the blood supply of the brain, usually through atherosclerosis or haemorrhage.
Symptoms of vascular dementia depend on which area of the brain has been affected, and may be a sudden onset, followed by a period of relative stability. Memory problems may not be an issue initially, although might occur later on in the disease trajectory.
Mixed dementia
Mixed dementia is more common in older age groups over 75 years, and is most often a combination of Alzheimer's disease and vascular dementia.
Dementia with Lewy bodies
Dementia with Lewy bodies is caused by abnormal clumps of protein (Lewy bodies) gathering inside brain cells, particularly the areas of brain responsible for thought, muscle movement and visual perception. It is less common than Alzheimer's disease and vascular dementia. Although memory is often less affected than with other types of dementia, additional symptoms for this group of patients can include:
- Being prone to falls
- Exhibiting tremors
- Having trouble swallowing
- Having visual and auditory hallucinations due to nerve cell damage
- Experiencing disrupted sleep patterns due to intense dreams.
Frontotemporal dementia
Frontotemporal dementia (Pick's disease) is a relatively rare form of dementia, which commonly affects people between 45 and 64 years of age. Frontal or temporal lobe involvement can develop over several years, and particular symptoms may include:
- Disinhibition and inappropriate social behaviours
- The developing of obsessions or unusual beliefs
- Decline in language abilities; repeating commonly used words
- Changes in food preference, for example over-eating or eating more sweet items.
Young onset dementia
Young onset dementia is defined as dementia diagnosed under the age of 65. There are an estimated 42 000 people with young onset dementia in the UK. Only about 34% of dementias diagnosed in younger people are Alzheimer's types. Frontotemporal lobe dementia accounts for about 12%, and the remaining percentage is made up of rarer familial forms of dementia including genetic mutations. In addition to other symptoms, younger people are likely to exhibit depression and anxiety, often connected with social situations and circumstances, including the risk of losing their paid employment (Dementia UK, 2018).
Addressing compliance
Compliance with treatment is affected by multiple factors, some of which the practitioner can influence. Use of appropriate language by the practitioner will encourage two-way verbal communication; examples of this include clarity of speech, tone, modulation, volume, avoiding confusing medical terminology, using the patient's referred name and asking open questions. Non-verbal communication, such as good posture, allowing personal space, appropriate eye contact and, relaxed facial expression will help to put the patient at ease. Active listening (eg, nodding to show encouragement and understanding) will further enhance interaction between patient and practitioner. Creating a welcoming setting by removing clutter and avoiding interruptions to the patient contact time will help to put the patient at ease. The practitioner can examine their own expectations, ensuring that they set realistic outcomes for each patient, within appropriate time and commodity parameters.
Other factors for the patient will be outside the practitioner's influence, and may have a greater impact on compliance. The patient might lack understanding of their own medical condition. If a patient perceives past experience of medical intervention to be a negative experience, through pain, discomfort or failed treatment, this may affect their level of trust in the practitioner and the patient's willingness to comply. The psychological wellbeing of the patient, including anxiety status, optimism levels and confidence may also impact the patient's adherence to advice; medication may also affect their tiredness or mood levels. The patient's current circumstances can affect their ability to commit to treatment-these could include family, work and social events taking priority over a course of treatment. Furthermore, other co-morbidities affecting general health, for example on-going cancer treatments, cardiac abnormalities, degenerative neuromuscular conditions, may be seen by the patient as taking a higher priority than current lymphoedema treatment.
The addition of dementia in managing compliance may include more specific issues. It may be difficult for the patient to concentrate on what is being said as they may ‘lose the thread’ of a conversation. Furthermore, the person with dementia may struggle to remember the right word to describe a symptom, or ask a question. Dementia-related mood changes such as withdrawal, unexplained sadness, anger or loss of self-confidence can cause disruption of the assessment or treatment caused by mood changes. They may also experience difficulty in adhering to treatment due to planning and sequencing inability.
When a person is unwilling to do something, it might be described as ‘refusal’ or ‘resistance’. However, for the person with dementia, there may be many possible reasons for non-cooperation or poor compliance:
- The person may not understand what they are being asked to do
- The request being made might not fit with the person's standards or preferences
- The person could be trying to maintain a sense of control, instead of being told what to do
- There may be a misinterpretation of the situation or environment, for example seeing an examination couch and thinking they are being asked to go to bed
- The person may not trust the health professional because they are reminded of someone with negative associations (Social Care Institute for Excellence, 2015).
The issue of consent and capacity
Before treatment for lymphoedema can commence, consent by the patient or their representatives must be obtained. Consent is a fundamental principle. It represents an individual's right to autonomy about what is or isn't done to them where they have the capacity to give consent (Association of Independent Healthcare Organisations, 2017).
The Mental Capacity Act 2005 protects patients and their carers/family supporters and states a person has the right:
- To be assumed to have capacity (unless an assessment shows otherwise)
- That decisions made on their behalf will be made in the person's best interests
- That liberty can only be taken away in very specific situations based around keeping a person safe or ensuring correct medication (deprivation of liberty)
- To receive support from an advocate who can speak on their behalf, but who does not have legal authority to make financial or personal decisions for them.
A Capacity Assessment can be made by anyone supporting a person, although situations involving complex or major decisions should involve a GP or specialist psychiatrist or psychologist (Social Care Institute for Excellence, 2017).
The two stages of a capacity test asks the following questions:
Stage 1 – is there an impairment of, or disturbance in the functioning of a person's mind or brain? if so
Stage 2 – is the impairment or disturbance sufficient that the person lacks capacity to make a decision at a time it needs to be made?
The Mental Capacity Act says that a person is unable to make their own decisions if they cannot do one more of the following;
- Understand information given to them
- Retain that information long enough to be able to make the decision
- Weigh up the information available to make the decision
- Communicate their decision – by talking, using sign language or even simple muscle movements such as blinking an eye or squeezing a hand.
Under these circumstances, decisions can be made on behalf of that person as long as those are made in the person's best interests. These decisions can include whether to have:
- Examinations and tests by doctors and health professionals in order to reach a diagnosis
- Treatment from a doctor or dentist, including surgery and resuscitation
- Chiropody, physiotherapy and nursing care.
Routine actions can also be carried out by carers or professionals without the need for legal authority or court permission, including;
- Giving the person routine medication
- Taking the person to hospital for treatment and assessment
- Giving nursing care or emergency first aid.
In the case of the patient with dementia who has been assessed as lacking capacity, the practitioner will be reliant on the carer/supporter to carry out or reinforce instructions for treatment. This may also have a bearing on compliance depending on the carer's understanding and their relationship with the patient.
In order to examine the management of lymphoedema and compliance to treatment, it would be useful to look at the different stages of management that a patient might go through, highlighting some of the particular issues and needs of a patient with dementia.
The patient journey
For a patient to seek treatment or have treatment sought on their behalf, a need for treatment must be acknowledged. In the case of lymphoedema secondary to cancer treatment, it will most likely be the team responsible for the cancer-related review who make the referral to a specialist lymphoedema department.
In the case of non-cancer-related lymphoedema, the patient might present at the GP to ask, for example, for treatment for their ‘swollen legs’ or it may be a relative or carer who first suggests that a condition needs further investigation or treatment.
Inviting patients to attend for assessment along with a family member or other support person will hopefully result in someone who knows the patient well to be in attendance. Assuming that informed consent for assessment has been obtained and documented, including data protection regulation requirements, the lymphoedema assessor must elicit a complex medical history from a patient whose memory for recent events may not be clear. Pre-empting this by requesting a medical history from the referring agent along with a list of current medications will assist greatly.
‘Many of the compliance issues for a patient with dementia will have a better chance of being addressed if a reliable carer or family member attends with the patient.’
Past and current medical history will have a bearing on treatment, particularly in the case of congestive cardiac failure, cellulitis and acute-phase venous thrombosis – the three contraindications to compression therapy (Ogawa, 2012). Other comorbidities may also influence treatment, particularly diabetes, arterial insufficiency, renal disease and rheumatoid arthritis.
Assessment will include noting where and with whom the patient lives. Ascertaining knowledge of social and daily routines is particularly important with this group of patients if compliance is to be gained. Asking a patient to don a compression garment each morning after their shower is ineffective if that patient's routine is to have a weekly bath, and telling patient to ‘walk as much as possible’ will not be welcomed by the carer who already has to keep track of a person who has a tendency to ‘wander’.
Inspecting the skin and tissue condition and taking measurements of limb circumferences may cause concern or distress for the patient. While invading the patient's personal space, the practitioner must be aware of their own safety, in case of sudden involuntary movements from hands or legs. Engaging the patient in distracting conversation can be a useful technique, as can offering a drink and biscuit to focus attention elsewhere.
Many factors can affect the outcome of treatment for this patient, besides their ability and willingness to comply with recommendations, including venous complications, high BMI and chronic skin conditions.
Finding out what the patient perceives as the biggest problem or impact of their lymphoedema on their daily life will help the practitioner to develop a realistic and motivational treatment plan. Exploring what the patient wishes to achieve with treatment and then making a treatment ‘contract’ between patient (and supporters) and practitioner can be a very effective way of reminding a patient whose compliance may be less than ideal. The challenge for the practitioner here is in listening to the patient who may have a very different goal to the one which might be expected of them.
The treatment plan formulated between the practitioner, the patient (and their representatives if present) may include:
- A course of decongestive lymphatic therapy to include bandaging
- Teaching remedial exercises to aid lymphatic drainage
- Use of intermittent pneumatic compression therapy in clinic or machine to loan at home
- Skin care including cleanliness, moisturising, observing for signs of cellulitis
- Manual lymphatic drainage which may include additional techniques, eg use of sound wave technology, negative pressure equipment, low level laser therapy
- Teaching simple lymphatic drainage
- Measuring for compression garments, choosing appropriate garments or wraps and discussing their use and care
- Use of Kinesio Tape for drainage/pain, and other scar management materials
- Appropriate referral to other professionals-physiotherapists, weight management programmes, counselling therapies, district nurses, surgical intervention teams.
Carrying out the arranged treatment will bring it's own set of compliance issues, relevant to all patients, but they may be exacerbated in the patient with dementia:
- Remembering to attend appointments for treatment
- Transport and support arrangements for attending appointments
- Understanding what is being asked of the patient on the day in terms of self care between appointments – skin care, exercise, simple lymphatic drainage, removal of kinesio tape, observing for signs of cellulitis, adequate hydration and nutrition
- Forgetting from one appointment to the next what they have been asked to carry out at home, eg precautionary factors-when to remove bandages etc
- Confusion/distress at the bandages being in place after the visit to the clinic
- Following up the collection of prescription garments
- Ability to don/doff prescribed compression garments
- Remembering to request from GP or collect repeated prescription garments at 6 monthly intervals.
Many of the compliance issues for a patient with dementia will have a better chance of being addressed if a reliable carer or family member attends with the patient. Morning appointments may suit this patient better. A care plan can be written to take away to maintain some continuity of care between appointments, and at the point of discharge. It is important to understand how the patient usually remembers routine actions, such as taking medication, as this can be a good guideline as to how their compliance with lymphoedema treatment can be enhanced. A large print calendar of appointments could be used. It may be that an exercise sheet or a pictorial chart of skin care needs to be laminated and put on display in a prominent place for the patient to see. The provision of a short video on simple lymphatic drainage, or the application of compression garments that the patient can use at home might also be considered.
The choice of compression garments for the patient with dementia, as with any patent who might struggle to comply with donning them, needs to be carefully considered. The challenge for practitioners is to select a compression garment that will have a positive effect on the patient's lymphoedema. The patient must be able to apply and remove this, possibly with assistance, and also tolerate it's daily wear.
Much confusion around the collection of prescription garments can be alleviated by having the GP prescription sent to the practitioner who then posts it along with a measurement chart, if appropriate, to one of the delivery services (eg Daylong, Jobst Delivered) who can return garments directly to the patient's home.
A reminder telephone call or letter for follow up reviews might be used to avoid missed appointments. When discharge of the patient is appropriate, it is even more important to ensure that appropriate services are in place to support the patient with dementia.
The practitioner journey – ‘working with the rabbit in the room’
Understanding a patient's perspective and challenging your own decisions and responses, particularly for a patent with dementia is important to achieving desired outcomes.
For example; while I was treating a wheelchair-bound patient with dementia, it was necessary to remove her lower leg bandages. These were placed in a white clinical dressings bag in a receptacle by her feet. The patient looked down and said; ‘see, a rabbit’. The patient did not ask me if there was a rabbit present, nor did she say ‘what's that?’, she merely stated there was a rabbit.
For a few moments I considered my response-I could have said ‘no, it's just a plastic bag’, giving the message that the patient was incorrect, and potentially foolish. It made no difference to the process whether she thought it was a rabbit, it did not distress her, in fact, it offered a small distraction from having her legs treated. My integrity was challenged, but not in a way which affected the treatment of her lymphoedema. In the end I asked her what we should do with the rabbit, and after some lively discussion, I took it out of the room and left it in a suitable disposal bin.
Conclusions
Health professionals are continuing to treat increasing numbers of patients with dementia and lymphoedema. For each patient who goes through their journey of treatment for lymphoedema, we, as practitioners can learn something new and take that learning forward in our practitioner's ‘toolkit’.
Attempting to understand the patient's perspective, not once, but every time we meet them, and how that can change dynamically, according to many external factors, is crucial to our partnership with them to achieve agreed goals.
Key Points
- The incidence of both lymphoedema and dementia are rising
- Addressing compliance at all stages of treatment depends on multiple factors
- Dementia will affect each patient uniquely and have different effects on their compliance to lymphoedema treatment
CPD reflective questions
- As a practitioner, how can you listen sufficiently to your patients in regard to their expectations and goals?
- Is your and your place of work's expectations of outcomes for an individual reasonable and realistic?
- How do you challenge your own response or do you tend to respond to situations in a standard manner?
- Are you a creative practitioner-what innovative ways do you use to help patients achieve outcomes?