Monthly Archives: June 2010

Why brain donation and why Mayo Jax?

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Someone emailed me this question today about where a loved one’s brain should be donated:

“Robin – I know you favor the research facility in Florida. How have you reached the conclusion that Mayo Jax is the best?”

Here’s my answer.

There are two key reasons to donate a loved one’s brain — to confirm the diagnosis and to enable research. Brain bank evaluation needs to consider both of these objectives.

With the goal of confirming a diagnosis, these items are important:

* the speed of getting a neuropathology report to families. (Mayo Jax does in 6-8 weeks what it takes 8-16 months for other brain banks to do.)

* the thoroughness of the neuropathology report. (Ask for an idea of how many pages the report will be. Or ask for a sample report for someone diagnosed with the disorder you are interested in. Incredibly, I’ve seen two-sentence neuropath reports!)

* the standing of the neuropathologist, and how many cases he/she has seen of the disorder you are interested in. (I have seen several neuropath reports where the neuropathologist says “this looks like PSP but it also looks like CBD.” In those cases, I’ve suggested that the family order brain tissue be sent to Mayo Jax, and the neuropathologist there comes up with a conclusive diagnosis quite easily. You want someone who has seen lots of cases before such that diagnosis is very straightforward.)

* the financial cost to the family in working with that brain bank. (The disadvantage of Mayo Jax is that the family may pay up to $1500 to accomplish brain donation.)

* the “hassle” to the family in working with that brain bank. This includes how extensive the required paperwork is, the family’s involvement in ordering medical records, and the requirement to find a person to do the brain procurement.

With the goal of utilizing brain tissue for research, these items are important to consider when evaluating a brain bank:

* the quantity of PSP brains they have. If you have a lot of tissue, you can consider brain research. If you have a few dozen brains, it’s probably not enough for a major research article. How do you find this out? Ask the brain bank coordinator how many PSP brains (or whatever the disorder is) they have in their brain bank. Look at the Schellenberg PSP/CBD genetics update to see how many brains were contributed by the various institutions around the world. (See: http://forum.psp.org/viewtopic.php?t=8223)

* the quantity of medical journal articles being published by that brain bank utilizing brain tissue for the disorder of interest. How do you find this out? Ask the brain bank coordinator for a list of all the published journal articles by the neuropathologist on a specific disorder. Or, do a PubMed search (pubmed.gov) with the name of the disorder and the name of the institution (or neuropathologist).

* how long will this brain bank likely be around? Sadly, there is a brain bank located in the LA area that is on the verge of shutting down its freezers, and no longer accepts PD or atypical parkinsonism brain donations! Many major US brain banks are severely cutting back due to the financial situation in the US. (Both UPenn and UCSD no longer accept MSA brain donations though the families/patients were told they could donate brain tissue her upon death. One Southern California woman’s dying wish to donate her brain could not be met by the family because of this problem.)

In the case of PSP/CBD brain donation, Mayo Jax is absolutely the best choice. If you donate a brain here, CurePSP will reimburse part of your brain donation costs. (Lately, the grants have been about $700.)

I mentioned that there are two key reasons to donate a loved one’s brain — to confirm the diagnosis and to enable research. Other reasons our family had or reasons I’ve heard from others include:

* since the diagnostic accuracy of the disorder your loved one was diagnosed with may be less than 55% (this is the case for PSP-parkinsonism, CBD, MSA without early autonomic symptoms, and DLB), the *only* way to in fact discover the diagnosis is upon brain donation. An excellent MD’s best guess is insufficient for most disorders.

* have something hugely positive come out of a bad situation.

* in case something inherited or genetic is ever discovered about PSP or CBD, blood relatives can have certainty in the diagnosis of their loved one.

* many people have said to me that their loved one had a scientific mind and would want to participate in a scientific endeavor.

* many people have said to me that their loved one was a very generous person and would want to make this most generous of donations. Some point to the fact that their loved ones are organ donors.

* one woman said to me that donating her loved one’s brain was the last step in caring for him. She felt that brain donation was a natural extension of caregiving.

* many people report peace of mind in knowing the final diagnosis.

What’s your opinion about these things?

Robin

Study of depression, anxiety, etc in PSP and MSA

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This study by UK researchers looks at the psychological well-being and health status of 188 PSP patients and 286 MSA patients.

Researchers concluded that:

1. PSP patients had significantly higher depression scores. PSP patients “reported significantly more symptoms compatible with anhedonia and concentration/apathy.” (anhedonia = inability to experience pleasure)

To “establish whether depressive symptoms are a secondary, later phenomenon or an early feature of PSP, longitudinal follow-up shortly after diagnosis is necessary to map how the disease process, depression, anxiety, and health status evolve in the course of PSP or MSA.”

2. MSA patients had significantly greater pain/discomfort. “Pain is an important aspect of PD and MSA in which multiple mechanisms, including hypoperfusion, rheumatic, sensory, dystonic and off-period related aspects have been postulated. In a previous study, MSA with predominant parkinsonism was associated with more pain than MSA with predominant cerebellar ataxia.”

“Difference between pain in the MSA and PSP groups is likely to reflect the different clinical presentation of PSP with relatively less typical limb parkinsonism and more prominent axial symptoms, less common response to levodopa and fewer autonomic symptoms. However, we did not explore the type and location of pain experienced in MSA.”

3. Anxiety was the same in both groups.

“As expected, disease severity showed significant negative correlations with the EQ-5D summary index for both groups. In contrast to a previous finding in PSP, disease duration did not correlate significantly with health status for either the patients with PSP or patients with MSA. This lack of association between disease duration and health status could be explained by the variable rate of progression of these disorders.”

Researchers say that this is important information for clinicians and patients because these symptoms should be “important targets for clinical management. … The results of this study emphasize again the importance of depressive symptoms as important for health status for both patient groups, but also highlight anxiety as being associated with health status. In addition, pain is a particularly important aspect of MSA for patients, and should be targeted in their clinical management.”

I’ve copied the abstract below.

Robin

Movement Disorders. 2010 Apr 13;25(eight):1077-1081. [Epub ahead of print]

A comparison of depression, anxiety, and health status in patients with progressive supranuclear palsy and multiple system atrophy.

Schrag A, Sheikh S, Quinn NP, Lees AJ, Selai C, Mathias C, Litvan I, Lang AE, Bower JH, Burn DJ, Low P, Jahanshahi M.
Institute of Neurology, University College London, London.

Abstract
The objective of this study was to compare subjective health status and its correlates in progressive supranuclear palsy (PSP) and multiple system atrophy (MSA). One hundred eighty-eight patients with PSP and 286 patients with MSA completed EQ-5D and Hospital Depression and Anxiety Scale.

The impact on mobility, usual activities, and self-care was similarly high in both groups after similar duration.

Fifty-six percent of PSP and 43% of MSA had probable depression, and 37% of both groups had probable anxiety. Patients with PSP had significantly higher depression scores, but groups did not differ in anxiety scores.

Patients with MSA had significantly greater pain/discomfort than patients with PSP.

The most important association with subjective health status was with depressive symptoms, which accounted for 38% and 29% of EQ-5D variance in patients with PSP and MSA, followed by disease severity and anxiety scores. We conclude that depressive symptoms were common in both disorders, but more severe in PSP.

Anxiety symptoms affected 37% of patients in both groups and contributed to impaired subjective health status.

Pain was more problematic in MSA than PSP. (c) 2010 Movement Disorder Society.

PubMed ID#: 20535826 (see pubmed.gov for this abstract only)

“Care for the Family Caregiver”

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The National Alliance for Caregiving updated its “Care for the Family Caregiver” brochure in 2010 and it has a new location on the web.  See:

www.caregiving.org/data/Emblem_CfC10_Final2.pdf

Care for the Family Caregiver: A Place to Start
March 2010 edition
Prepared by EmblemHealth and National Alliance for Caregiving

These are (still) the best sections:

“Helpful Tips for Family Caregivers: A Place to Start,” page 18  (page 22 of PDF)

“Caregiver Training,” page 20  (page 24 of PDF)

“Caregiver Health: Taking Care of Yourself,” page 27  (page 31 of PDF)

“Caregiver Resource Guide: Where to turn for help,” page 37  (page 41 of PDF)

I’ve copied below the section on “Helpful Tips for Family Caregivers: A Place to Start.”

Robin

Excerpt from
Care for the Family Caregiver
Helpful Tips for Family Caregivers: A Place to Start
National Alliance for Caregiving
March 2010

Caregiving can require an enormous physical and emotional commitment, as well as some basic skills. The pages that follow provide tips and information on where to start.

* Create a safe environment at home.

Conduct a home safety inspection of your loved one’s home or your own if you are caring for someone there. For example, check for adequate lighting, install grab bars in the bathroom and hook up a cordless phone for emergencies.

Home safety checklists are available on the Internet and from the AARP. (Go to www.aarp.org, and search Home Safety Caregiving Checklist.)

* Get caregiver training.

Seek out educational resources in caregiving. (See the Caregiver Training section that follows.) For example, learn the correct way to transfer a loved one from a bed to a wheelchair. This can help you avoid serious injury to yourself and the person for whom you are caring.

In addition, learn how to properly bathe someone with mobility problems. This can reduce the risk of hospitalization for chronic sores and infections.

* Maintain medical records.

Keep a current, complete list of all medications and physicians, along with notes on medical history. Be sure to take this if you accompany your loved one to doctors’ visits.

Most care recipients (93%) take at least one prescription drug. It’s important to keep a list of all medications the care recipient is currently taking. Be sure to also record the dosage or strength, such as 10 mg; for what condition the drug is taken; and how often it is taken, such as twice a day. A drug regimen may change often, so be sure to make regular updates. Pharmacists in particular are valuable resources for medication information.

If your loved one has access to a personal health record (PHR), use it to record symptoms, doctor visits, medications and other important health information.

* Learn about the disease.

Find out all you can about the disease the care recipient has, its treatments and the prognosis. Armed with this information, you and your family will have a better idea what to expect in the future and how you can help. This information can help you with planning.

* Learn how to communicate with healthcare professionals.

In order to be a better advocate for your loved one, understand and use the terminology that doctors, nurses, discharge planners, therapists and other health care professionals use in discussing the case. Be calm but firm in advocating for being a part of the health care and support service decision-making team.

* Minimize stress, especially during holidays.

Holidays can be especially stressful for both caregivers and care recipients. Try to reduce stress, simplify activities, relax, slow the pace and ensure that there is plenty of quiet time to reminisce.

* Get the extended family involved in caregiving.

Organize and hold a family meeting involving all decision makers. Identify and discuss the issues of providing care for the family member in need.

* Ask for help with household activities.

Seek help with yard work and other household tasks. Consider asking a friend or neighbor for help. Hire someone to mow the lawn. Look into delivery services for groceries or drugstore items.

* Delegate to friends and family.

Remember, be specific when asking for help from family and friends: “Can Jill come for a couple of hours on Saturdays to stay with Grandma while I do the shopping?” or “Can George mow the lawn every other week now that Dad can’t do it any longer?”

* Manage your time.

Keep an appointment book or calendar to schedule your daily activities, including doctors’ visits. Some computer programs or personal devices can help you schedule and manage your time. Consider using an online calendar you can share with other family members on the Internet, such as Google Calendar.

* Seek help that meets your situation.

Each caregiving situation is unique. For example, if you care for someone who is not living with you and lives a long distance away, you may face special logistical, financial and emotional challenges. Seek out resources that meet your special long distance needs; for example, consider using a geriatric care manager.

“Alzheimer’s Stalks a Colombian Family” (NYT)

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This very large family in the country of Colombia has become very important to Alzheimer’s researchers. Here’s a good New York Times article on the family in yesterday’s paper:

www.nytimes.com/2010/06/02/health/02alzheimers.html

Alzheimer’’s Stalks a Colombian Family
By Pam Belluck
New York Times
June 1, 2010

Here are two quotations from two of the neurologists involved in researching this Colombian family:

* “This is the only place in the world where we can find a family like this, where it’s possible to do prevention therapy more easily,”” said Dr. Francisco Lopera, a Medellín neurologist who identified the family’’s illness 28 years ago and helped discover its cause, an altered protein on the presenilin 1 gene on chromosome 14: the Paisa mutation.

* Scientists consider Colombians not only valuable subjects but also deserving ones. “”We’’d be giving people at the highest imminent risk of Alzheimer’’s access to treatment they wouldn’’t otherwise have,”” said a project leader, Dr. Eric M. Reiman, director of Banner Alzheimer’s Institute in Phoenix.