Planning for the What ifs – Part Three
Falls, Extreme Immobility and Secretion Management
Today we continue Parkinson’s Disease: Planning for the What Ifs, a special series of posts to address both motor and non-motor issues of people with advanced Parkinson’s disease (PD). We are defining advanced PD as those who are no longer independent in their activities of daily living, and require help for their self-care such as eating, bathing, dressing and toileting. Remember, PD is a very variable condition and many never reach the advanced stages. Additional background and the full introduction to the series is still available if you missed it.
In previous blogs, we addressed the mental health issues of advanced PD which include: cognitive decline/dementia, depression, anxiety, apathy, psychosis, mania and behavioral problems.
Today, Dr. Pravin Khemani, a Movement disorders neurologist at Swedish Medical Center in Seattle, will address three other difficult topics of advanced PD: falls, extreme immobility, and drooling and swallowing difficulties. As you’ll see, a team-based comprehensive approach is imperative in both recognizing and addressing disabling motor and nonmotor symptoms. Such a team typically includes the neurologist, primary care provider, social worker, nurse, rehabilitation specialist, and clinic staff who are familiar with the clinical course of PD.
Falls are one of the major causes of emergency room visits and hospitalizations for PD patients.
Falls can result from:
- Balance problems from PD itself
- Imbalance due to bothersome dyskinesias (which are a side effect of Levodopa treatment)
- Neurogenic orthostatic hypotension (NOH), a common non-motor symptom of PD that results in sudden drops in blood pressure when standing or walking
Understanding which of these factors are contributing to falls is important since each is treated in a different way. The balance problems of PD may be responsive to increases in PD medications. Bothersome dyskinesias can be treated by adjusting PD medications or initiating amantadine. A number of strategies can be used to manage NOH including increased fluids and dietary salt as well as compression stockings. If necessary, medications to increase blood pressure can also be considered.
Once medications are optimized, the next step in the treatment of falls in PD is a thorough rehabilitative assessment by a trained physical therapist to identify the true limits of independent mobility and to implement fall safety measures both in and out of the home. A physical therapy program is essential to maintain independent mobility as much as possible.
‘Fear of falling’ is a state of heightened anxiety which constrains walking – above and beyond the reasons listed above. ‘Fear of falling’ is also potentially treatable with effective physical therapy.
Despite the available treatment options, falls can eventually lead to wheelchair confinement for the safety of the patient, which has the inadvertent consequence of curtailing independent mobility. If wheelchair confinement is inevitable, then a continuous home or outpatient rehabilitation program is recommended to prevent long-term problems that can occur due to immobility such as limb deformities, contractures, pressure ulcers, and loss of muscle mass.
Health insurances may limit the number of physical therapy sessions per year which places the responsibility of a home exercise program on the care partner or other members of the family.
In early and mid-stages of PD, motor symptoms of slowness and stiffness respond well to PD medications and to deep brain stimulation. As PD progresses into advanced stages however, motor symptoms may become less responsive to increasing doses of medication or to adjustments of deep brain stimulation (DBS) parameters. This may mean that the person with advanced PD has severe slowness and stiffness and is no longer able to walk. It could also mean that within the confines of the wheelchair, the person is not able to perform much movement on their own of their arms, legs or torso, and instead remains very still.
If such a stage is reached, it is still reasonable to consider the possibility that increased medication may improve mobility. It is important to fully optimize medications to determine if their full benefit has been achieved. However, the benefits of increasing medication must be weighed against the potential risks which include side effects such as hallucinations or drops in blood pressure. There may in fact be a role for decreasing medications if such side-effects are observed.
If the decision is made to reduce medications, this must be done carefully under the supervision and advisement of a neurologist. It may appear that the person’s immobility is severe and that the medications are not providing any benefit. However, reduction of medication could nevertheless even further reduce mobility, so care should be taken.
Consequences of extreme immobility
Extreme immobility can contribute to difficulties, including limb deformities, contractures, pressure ulcers, and loss of muscle mass. Modified exercise, physical therapy and passive range of motion manipulations are recommended to try to prevent these complications as much as possible.
Limb deformities and contractures
Hand and foot deformities as well as other abnormal body postures such as flexion at the waist or tilting of the torso are common in advanced PD. There is a range of severity of these deformities. As the severity increases, it is more difficult to maneuver the limb into a normal position. The reasons for development of these deformities are not well understood but are likely a combination of rigidity (stiffness), dystonia (muscle contractions that cause abnormal posturing) as well as factors that we don’t fully understand.
A contracture is a permanent tightening or shortening of a muscle and can result from a persistent limb deformity or abnormal body posture. This results in an inability to even passively move or manipulate a body part through the normal range of motion. Once a contracture is present, these deformities do not typically respond to treatment.
Treatments of limb deformities (prior to development of a contracture):
- PD medication optimization – early on, limb deformities may respond to an increase in PD medication
- Regular range of motion exercises
- Physical therapy
- Botulinum toxin injections
Immobility coupled with cognitive decline are the primary risk factors for development of pressure ulcers, breakdown of skin and underlying tissue resulting from prolonged pressure on a particular site on the skin (commonly known as bedsores). The incidence of pressure ulcers is high in the frail elderly who are dependent on caregivers for movement. If not promptly recognized and treated, pressure ulcers can lead to infections of the underlying tissues and even result in sepsis (a life-threatening illness caused by your body’s response to an infection).
Pressure ulcer prevention
Pressure ulcers develop most commonly when bony prominences (such as the heels, wrists, sacrum, elbows, shoulders) are subject to unrelieved pressure from prolonged contact with hard surfaces (wheelchair, chairs, beds), or when skin is damaged by friction caused by dragging against a surface. Pressure ulcers can develop quickly, therefore it is important to take steps to prevent pressure ulcers. These include:
- keeping skin clean and dry
- daily skin inspection
- relief of pressure around susceptible areas by mobilization
- frequent repositioning
- avoiding skin friction through use of appropriate resting surfaces (such as alternating pressure mattresses)
- protecting bony prominences in contact with the resting surfaces
- optimizing fluid and nutrition intake
Unfortunately, pressure ulcers may develop despite best efforts. Keeping a keen eye out for pressure ulcers in the immobile person, coupled with regular skin inspections, facilitates prompt recognition of pressure ulcers.
Identifying pressure ulcers
- Pressure ulcers are usually staged from I to IV in order of increasing severity. At the earliest stage, there is no skin breakdown. Clues that a pressure ulcer is forming are changes in skin color, texture, or temperature compared to the adjacent tissue, or local tenderness to skin palpation.
- In later stages there is progressive loss of skin, leading to damage of underlying tissues and deepening of the wound. The deeper the wound, the longer it will take to heal and the more likely that infection of surrounding areas will occur.
Treatment of pressure ulcers
Treatment of pressure ulcers is most successful in the earliest stages when the skin is intact. In addition to all of the preventative measures outlined above, specialized wound dressings are used to treat pressure ulcers once they have formed. Some evidence suggests that wound dressings applied to high-risk intact skin may prevent development of pressure ulcers. A wound-care professional who is knowledgeable about risk-factor reduction and wound dressing selection should be consulted as soon as possible for treatment – ask your doctor for a recommendation. More severe pressure ulcers may require debridement, (removal of damaged tissue) or surgical repair to expedite healing. Antibiotics are usually necessary as well. When pressure ulcers are in more advanced stages, they warrant interdisciplinary care involving nurses, nutritionists, wound care specialists, surgeons, and infectious disease specialists.
Loss of muscle mass
Loss of muscle mass can be a direct consequence of the combination of immobility and malnutrition. Malnutrition can in turn be caused by swallowing difficulties, poor appetite and motor issues which lead to a decreased ability to buy and prepare food. Loss of muscle mass and malnutrition can subsequently be the cause of increased susceptibility to infection, increased fatigue and increased frailty. The situation can spiral with more fatigue and frailty causing a further decrease in activity and function.
Optimizing nutrition and maintaining weight bearing activities gives the person with advanced PD the best chance of maintaining muscle mass.
Drooling and swallowing difficulties
In a prior blog, we addressed drooling and dysphagia (swallowing difficulties) for the general PD population. These problems can be compounded in the advanced stages of PD. A few differences to note:
Drooling in the latter stages of PD requires more complex treatment as it may be a result of irreversible weakening of swallowing muscles. Drooling can be the very first sign of swallowing difficulties, interfering with adequate food and liquid intake if it persists despite medication optimization. It is important to remember that swallowing difficulties in PD may occur with or without drooling. A stooped posture and neck, very commonly seen in PD, may further complicate drooling as does the presence of dementia.
Oral drugs and skin-patches are available to treat persistent drooling, but they should be used cautiously and administered in small doses as they can inadvertently worsen mental status, blood pressure control and bladder and bowel function. Botulinum toxin periodically injected in the salivary glands is a safe and effective treatment of drooling when administered by an experienced injector.
A speech language pathology (SLP) and swallowing evaluation is essential when drooling or difficulty with swallowing food or water is resistant to treatment with PD medications. Based on the results of the swallowing evaluation, the SLP expert makes recommendations to prevent aspiration while maintaining adequate oral nutritional intake. Thickening of fluids, chin-tuck maneuver during swallowing, and soft foods are some common measures of dysphagia treatment. Multiple medications should be minimized to reduce the burden of swallowing pills. However, despite these measures, in the advanced stages of PD, progressive difficulty swallowing can lead to a high risk of aspiration — when saliva or food enter the trachea and lungs instead of the esophagus. Aspiration can lead to pneumonia.
There may come a time, when aspiration is so prevalent, that there is no food that is safe for a person with advanced PD to eat, or no safe maneuvers that the person can do in order to eat safely. It is also possible, that a person with advanced dementia may not able to perform the maneuvers, such as chin-tuck, to allow for safe eating. In anticipation of this time, an advanced directive, or document that outlines a person’s preference for end-of-life care ahead of time, should be in place. This will guide family members and doctors of the person’s wishes as it pertains to inserting a feeding tube to maintain adequate nutritional intake.
If an advanced directive is not created ahead of time, at the very least a person should appoint a healthcare proxy, a person (family member or friend) chosen to make healthcare decisions on his/her behalf. If the person with PD can no longer participate in medical decisions because of advancing dementia, then the decision of whether to insert a feeding tube is made by the person’s healthcare proxy.
It must be noted, that although a feeding tube can be used to deliver nutrition, PD medications, and pain medicines, it does not prevent aspiration pneumonia. That is because anything inserted into the feeding tube can make its way up the GI tract and down the wrong tube anyway. The feeding tube may also potentially lead to complications associated with inserting and maintaining the tube. Based on observational studies, feeding tubes in advanced neurologic disease, do not improve overall quality of life. Therefore, the decision to insert a feeding tube should be made in an informed manner after the pros and cons are fully considered. Many loving families decide not to insert a feeding tube and allow their loved one to continue to enjoy the pleasures of food at the advanced stages of PD despite the high risk of aspiration.
Navigating the more severe symptoms of PD can be very challenging, both emotionally and physically. That’s why it is important to educate yourself and understand the possibilities that may (or may not) come your way so you have a sense of what to look out for and how to prepare. The issues mentioned above do not necessarily present all at once; one sign may precede the other, therefore, treatment plans need to be in place so patients and care partners can avoid catastrophic emergencies and hospitalizations. Also, these steps take some of the burdensome decision making off of the care partner and ensures that the wishes of the person with PD are carried out.
Tips and Takeaways
- Falls, extreme immobility and swallowing dysfunction are three major causes of decreased quality of life in people with advanced PD
- Falls can result from balance difficulties of PD itself, bothersome dyskinesias and neurogenic orthostatic hypotension
- Extreme immobility can lead to limb deformities and contractures, pressure ulcers and loss of muscle mass
- Swallowing difficulties can lead to malnutrition and aspiration pneumonia
- It is important to discuss advanced PD symptoms before they manifest. This will help you be aware of what to look out for so early interventions can help minimize the issue(s) and also help family members understand the person with PD’s wishes for their care should advanced symptoms appear.
- An advanced directive and palliative care options should be in place before postural instability (impaired balance) starts causing falls, dysphagia becomes an issue, or signs of dementia are identified.