Neurological Disease/ Brain / Movement Disorders

Amyotrophic lateral sclerosis, commonly called ALS or Lou Gehrig’s disease, is a progressive disease that affects the nervous system. The disease eventually affects someone’s mobility, independent functioning, communication, swallowing and breathing.

Generally, care for people with ALS includes these considerations:

• the illness itself;
• the impact of the illness on daily functioning;
• planning for care when eating and breathing become difficult;
• planning for issues arising near the end of life.

In early stages of the illness attention is give to approaches that may slow disease progression. A person with ALS is assessed by a neurologist, who is up to date on the latest medications that may help. The person also may be assessed by a specialized team, which can include a speech-language pathologist, physiotherapist, occupational therapist, and dietician or nutritionist. These health care providers work together to develop a plan that helps the person function as well as possible throughout the course of the illness.

Swallowing difficulties can be addressed by speech-language pathologists, dieticians or nutritionists. They can suggest ways to adjust food texture, and calorie intake as well. There may come a time when someone can’t swallow. At that time a feeding tube or intravenous fluids become options to be discussed. The person with ALS may decide to refuse artificial replacements for eating and drinking, and may choose instead to focus on comfort. This is an individual choice, and each person must decide what’s best for her or him.

If breathing becomes weak, breathing machines or ventilators will likely be offered as possible interventions to help a patient breathe. Two machines are commonly used. One is referred to as Continuous Positive Airway Pressure (CPAP), the other as Bi-level Positive Airway Pressure (BiPAP). Each supplies oxygen through a mask that fits over the face. The oxygen is under pressure, which helps the lungs expand. If breathing becomes even weaker, then a machine called a ventilator may be considered. This delivers oxygen directly into the lungs through a tube placed into the trachea. As with food and fluids, some people may choose to refuse artificial breathing assistance. If so, then the focus of caring for this person is on comfort.

Some people with ALS have pain. This can be controlled with opioids such as morphine, which are adjusted as needed. Opioids also help if someone feels short of breath.

Many important issues arise as the end of life nears, which require decisions by the patient and family. The person with ALS and the family need to make decisions about care, or about other matters. Decision making is best addressed before the need arises, while people still have the strength and capacity to take part in discussions.

The progression of cerebral palsy varies a great deal. In general, however, people with cerebral palsy have some common experiences in the final weeks or months:

• Decreasing strength and energy
  This causes decreasing mobility, communication, alertness and ability to breath and cough. Usually it’s not a cause of physical distress. Rather it’s a gradual fading, which eventually results in less time awake and more time asleep.
   
• Decreasing appetite and eating
  As with people in advanced stages of any illness, people with cerebral palsy become less active and so need fewer calories. They also lose the ability to process nutrients, and can become uncomfortable if they’re made to eat. It’s helpful for caregivers to understand this, as part of the final stages of an illness.
   
• Decreasing lung strength and function
  The lungs may no longer be able to expand fully. Scoliosis, a sideways curvature of the spine, may constrict the chest; or a person’s general weakness may prevent normal breathing. This results in poor coughing and clearing of secretions, which in turn brings a risk of infection.

People who experience shortness of breath may find relief with a range of measures:

• Medications
  Opioids seem to reduce the effort of breathing and block distressing signals of air hunger and pain. Oxygen may also help. Anxiety medications may be used to ease distress that can aggravate breathlessness.
   
• Air movement and temperature
  Breathing can be eased by using a fan or opening a window to circulate air, and by keeping the air in the room cool and less humid.
   
• Body position
  Some positions can ease breathing, typically sitting propped up or on one side.

In the final hours or days of life, people almost always are less alert. Often a chest infection develops. Pneumonia is a serious complication and can be the reason that death occurs. With attention to comfort, this stage can be a calm, gradual slipping away. If there is any pain, restlessness or shortness of breath, they can be controlled with medications such as opioids, sedatives or medications for anxiety.

It is useful to discuss possible developments and treatments with the health care team. Such discussions can be very difficult for a family, but it helps to develop a plan for dealing with what may happen before it occurs. If the patient is conscious, communicating, and old enough to understand treatment decisions, then the person may want to be part of these conversations.

Multiple sclerosis (MS) is a progressive disease of the nervous system. It can cause a wide variety of symptoms, which may continue or worsen as the disease progresses. The most common symptoms include fatigue, walking difficulties, bowel and bladder disturbances, vision problems, changes in brain function, changes in sexual function, pain and depression or mood swings.

With diseases such as MS, for which there currently is no cure, palliative care expertise can help in all stages of the illness. A palliative approach offers help with symptoms, and help in anticipating and planning for issues and decisions that are likely to come up as the disease progresses. This ensures that a person’s wishes will be met at any stage of the disease. At the end-stage of MS, people commonly face some particular challenges.

Care setting

The choice of where to care for someone with advanced MS depends on each person's needs and available resources. Options may include home, hospice, personal care or nursing home, palliative care unit, or hospital. Where there are community-based resources, then care at home is often preferred by most patients and families. At times, the support needed may require care in a long-term facility. In such settings the person with MS may be far younger than other residents and have greater mental capacities. This can challenge the facility’s program resources, and can affect a person’s quality of life. A palliative care facility or hospice may be preferable at the end of life, if the person meets the criteria for these resources.

Swallowing and eating

Swallowing problems are uncommon in the early stages of MS, but in late stages, as many as one third of people with MS have trouble swallowing. They also may have eating difficulties as a result of hand tremors and muscle spasms. Some people in the late stages of MS show signs of declining mental processes. As a result, they may be unaware of the act of eating or of the need to swallow when being fed. It’s helpful for caregivers, patients and health care providers to talk about how food and fluids will be provided in the advanced stages of illness.

Communication

Speech difficulties may require assessment by a speech-language pathologist (SLP). An SLP also can suggest ways to handle feeding and swallowing problems.

Pain

People with advanced MS may have pain caused by muscle spasms, neuropathic (nerve) pain, and pain caused by immobility. Neuropathic pain and pain from spasms can be challenging to treat. Medications for neuropathic pain include anticonvulsants and antidepressants. If tricyclic antidepressants are used, be aware that urinary retention can be a side effect. Cannabinoids may help relieve neuropathic pain related to MS. Muscle spasms are often treated with muscle relaxants. Physiotherapy to improve range of motion may help with muscle spasms and spasticity.

Final stages

Someone diagnosed with MS often is stable for long periods, can decline, and then stabilize again. Ultimately, the person dies from complications related to the advancing disease. In many diseases of the nervous system deteriorating respiratory function usually brings on the final decline. This may be related to shallow and inefficient breathing, which can lead to complications such as pneumonia. Shortness of breath and respiratory secretions often need to be controlled. Pain medications and sometimes sedatives may help people who are short of breath. Oxygen may be used if it’s available. Medications may help decrease secretions if this becomes a source of discomfort or distress. Certain symptoms may limit the methods available to administer medications. One option is through a feeding tube, if a tube is already in place. Another option is sublingual administration, if swallowing ability and secretions allow it. Subcutaneous administration of medications can also be considered.