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Caring for a loved one suffering from prion disease is challenging.

Here are ideas that may help.

Families who receive a prion disease diagnosis are often shocked, confused and overwhelmed. They must rapidly accept the news that their loved one – who probably appeared quite healthy just weeks ago – is suffering from a terminal disease that has no treatment.

And they must quickly determine who will care for the patient, and where. 

Develop a Care Plan

Families who receive a prion disease diagnosis should contact hospice as early as possible. While hospice care may not be immediately needed in the early stages, the patient’s needs are likely to evolve rapidly. Choosing a hospice provider and setting up care in advance will free the family to focus more on the patient later. Hospice providers bring knowledge and resources that are invaluable to a family that is confronting rapidly evolving care needs.

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Listen to this recorded teleconference with Brian Appleby, MD, Medical Director, CJD Foundation, and Director, National Prion Disease Pathology Surveillance Center, and Lauren Somple, MSW, LISW-S, Clinical Social Worker, University Hospitals Foley Elder Health Center of Cleveland, to hear more about:

  • Understanding the CJD diagnosis
  • Developing a care plan
  • What hospice can provide to support the patient and family
  • How to apply for SSDI
  • What else to consider after receiving a diagnosis

When receiving a diagnosis of prion disease, a family will need to quickly consider several issues:

Expect Frequent Changes

A patient can experience many or just a few of the common symptoms associated with prion disease. One consistent factor, though, is frequent change. For a caregiver, it is important to stay attuned to the shifting needs of the patient. This becomes challenging as the patient loses the ability to communicate. It would be helpful for caregivers (family members and professionals) to communicate with each other regularly, or possibly keep a notebook, with any changes in the patient’s symptoms, ideas on what seems to soothe them, and concerns. 

Create a Soothing Environment

Heightened sensitivity is common for prion disease patients. Reactions occur more commonly to unexpected touch, loud or sudden noises and music. This may produce immediate signs of distress and agitation.

Caregivers should limit stimuli to make the patient comfortable, and stay attuned to factors that distress them. If reflections seem to cause visual disturbance, the family can cover reflective surfaces such as mirrors, windows, or glass tables or shiny appliances. A flickering television can be disturbing or could even induce hallucinations. Soft lighting is ideal, as is quiet music if the patient tolerates it well. It may be necessary to avoid loud sounds and large groups of people if they appear disruptive to the patient.

Offer Support for Activities of Daily Living (ADLs)

Over time the patient is likely to need increasing help with eating, bathing, dressing, toileting, and transferring. Hospice and health aides can provide invaluable assistance in planning and problem solving. They have knowledge and access to countless approaches, tools, and resources that can aid in patient comfort and care. They may suggest a caregiving aid (such as a shower chair or hospital bed) before the family realizes the patient needs it.

Communicate with Your Physician

As the disease progresses, you will want to contact the physician, hospice, or healthcare provider about any concerns, such as:

Be bold in reaching out to the healthcare professional if you are not hearing a response.  With prion diseases, the patient’s condition may change more quickly than the doctor is accustomed to.

Sometimes families are overwhelmed when they see their doctor and forget to mention important items that they intended to discuss and/or they do not want to mention important changes in front of their loved one.

Download and fill out this worksheet to give to the doctor at the beginning of the visit.

Manage Visitors

After a prion disease diagnosis, your family may feel compelled to invite large groups to your home to visit the patient or say goodbye to them. Before inviting a large group, assess whether the patient might adjust well to the group. If visits make the patient restless or agitated, it is best to ask visitors to come in pairs or small groups, keep the visit short, and have low expectations for the patient’s level of involvement in the discussion.

Although the patient may be confused or even unable to communicate, you can try to include them in conversations, and speak to them kindly about your shared history, your love for them, pride in their accomplishments, and happy memories together.

Take Care of the Caregiver

Relieving your exhaustion and stress is crucial for both you and the patient. You may have little time to care for yourself while caring for the patient, but self-care is vital. Exercising, getting adequate sleep, and eating healthfully will benefit you and enable you to continue the grueling tasks of caring for your loved one.

You may also need to ask for help from professional caregivers or family and friends.

If friends offer to help, you may want to ask them to provide respite care, set shifts for sitting with the patient, or provide a break or support for you in some way. Friends could organize meal trains or offer to do errands and help with maintaining the home. As the caregiver, you can also benefit from stress reduction through therapy, meditation, massage, and exercise.

For more information on Caregiving for a prion disease patient, contact the CJD Foundation Helpline to ask questions or request a Caregiving Guide: 800-659-1991

Caregiver Tips

Incontinence can make the patient agitated, restless, and distressed. It can give them a feeling of losing dignity and independence, and in later stages, the movement required to change the patient can be jarring. Working with the hospice or a medical care provider, the family can assess options for addressing incontinence and learn how to minimize movement when changing bedding and clothing.

As prion disease progresses, the patient may have difficulty with appetite, chewing, focusing on a meal, and ultimately with swallowing. At early stages, ergonomic utensils may be helpful to enable self-feeding. As dysphagia progresses, pureed foods and thickener in fluids may become necessary.

The caregiver should balance nourishment with enticing foods.  If the patient prefers less healthy foods such as milkshakes, or begins focusing on a very limited set of preferred foods, it is fine to move in that direction as long as the patient is obtaining adequate nourishment and is not encountering digestive difficulties. The patient’s desire for foods may fade. As long as they are hydrated, caregiver should follow the patient’s lead where possible, and should not force foods. Mouth care is important to keep the patient comfortable. Options include a washcloth, a child’s toothbrush, and swabs.

While the patient’s temperament and personality may change radically, it is important for the family to remember that it is often the disease talking, and not the patient. Remaining calm and patient, and not arguing when the patient expresses delusions and hallucinations, is ideal. As long as the patient is not a hazard to themselves or others, reassuring them and working through episodes with patience is best. If symptoms worsen, the caregiver should communicate frequently with the physician to ensure medications and dosages are as effective as possible in managing symptoms.

If the patient develops myoclonus, caregivers can help by minimizing touching and movement in caring for the patient. At times, gentle massage may help relax the muscles, and the family may ask the physician about medications to help reduce spasticity and tremors. While myoclonus spasms are not painful for the patient, they are certainly disruptive, so reducing their effects can enhance the patient’s comfort.

Additional Resources

This pamphlet provides suggestions for preventing falls, home modifications to make, ways to stay active and maintain mobility, assistive devices, and transfer tips.

Speech-Language Pathologists (SLPs) are experts of swallowing, communication, and the cognitive-linguistic processes related to memory. This pamphlet details how to approach each.

Watch this panel discussion to learn from clinicians specializing in Prion Disease, followed by a Q&A period: Brian Appleby, MD, Director of National Prion Disease Pathology Surveillance Center; Gregory Day, MD, MSc, Dept. of Neurology, Mayo Clinic; Michael Geschwind, MD, PhD, Memory and Aging Center, University of California, San Francisco (UCSF); Moderator: Prof. Richard Knight, Emeritus Professor of Clinical Neurology, CCBS, University of Edinburgh.

Best Practices for Diagnosing and Caregiving with Richard Knight, BA, BM BCh, FRCP(E); Michael D. Geschwind, MD, PhD; Brian Appleby, MD; and Neil Cashman, MD