Pages

Saturday, April 13, 2019

'It's not life or death, I just shake a bit': Young and living with Parkinson's

By Emma Siossian  April 12. 2019


thinking Parkinson's is a disease that affects only older people.

Key points:

  • Parkinson's disease is a progressive, chronic neurological condition that affects movement
  • Early onset Parkinson's can affect people in their 30s and 40s
  • Symptoms include muscle rigidity, tremors, postural instability, gait disturbances and bradykinesia
But it can affect people in their 30s and 40s.
Kim Dahler from Port Macquarie on the New South Wales mid-north coast was diagnosed with early-onset Parkinson's in 2013. She was in her mid 40s.
"There's a lot of people in the same position and because the symptoms are gradual you don't seem to notice as much."
She said it took her quite a while to get diagnosed, but during that time she did a lot of research and had a fair idea she had Parkinson's.
"It took a journey of about 12 months to get the diagnosis and it took me a little while to come to terms with it, I'm not sure why, the stigma, the embarrassment, I'm not sure," Ms Dahler said.
"I put my head in the sand a bit I think, and just worked."

Living positively with Parkinson's

Ms Dahler said since her initial diagnosis she had turned her attitude around and was focussing on the positives, still working full-time in a demanding job and travelling.
She is also trying to increase the community's understanding about Parkinson's by telling her story and offering support to others.
"I'm out the other end now and I want to speak up and say it's not the end of the world," she said.
"I work full-time, I have to be a little bit careful with my movements, but life goes on.
"I get a lot of support from my employer, so I am very fortunate.
"It's a balancing act as well, if I do a big drive I will stay in a branch for a day just to balance my health, I have to be sensible and keep it manageable."
Seeing the lighter side can also help. 
Ms Dahler has also designed a light-hearted t-shirt featuring the slogan, 'I haven't got the DTs [delerium tremens caused by withdrawal from alcohol], it's just Parkinson's'.

Don't be afraid to ask for support

Ms Dahler said when she was first diagnosed she did not ask for help, but probably should have, and is now involved in a support group.
Port Macquarie Parkinson's Support group president, Gregg Faulkner, said it was important people reached out for help.
"We have monthly meetings and the most important part is the morning tea," he said.
"It's a disease which has a fairly simply cause — there are a few cells in the brain that don't produce enough dopamine.
"But the end result of that is physical, psychological, emotional, all manner of changes and it's a complex web of symptoms.
"I was diagnosed eight years ago, I'm doing pretty well, my voice sometimes lapses, but I'm still president of the local support group, which is growing like crazy, which is a mixed blessing.

What is Parkinson's?

According to national peak body, Parkinson's Australia, there are more than 80,000 Australians living with Parkinson's and around 20 per cent are of working age, with many diagnosed in their 30s and 40s. 

Parkinson's is a progressive, chronic neurological condition which affects the nerve cells in the brain that produce dopamine, which is particularly important in controlling movement.

Symptoms, which can include muscle rigidity, tremors, and changes in speech and gait, generally develop slowly over years and vary from one person to another.
After diagnosis, treatments can help relieve symptoms, but there is no cure.


PHOTO: Kim Dahler has created a t-shirt to raise awareness and find some humour. On the back it says, 'I just have Parkinson's'. (Supplied: Kim Dahler)

Broken mitochondria use 'eat me' proteins to summon their executioners

Date:
April 11, 2019
Source:
Cell Press
Summary:
When mitochondria become damaged, they avoid causing further problems by signaling cellular proteins to degrade them. In a new study, scientists report that they have discovered how the cells trigger this process, which is called mitophagy. In cells with broken mitochondria, two proteins -- NIPSNAP 1 and NIPSNAP 2 -- accumulate on the mitochondrial surface, functioning as 'eat me' signals, recruiting the cellular machinery that will destroy them.
This image shows localization of NIPSNAP1 both inside (red) and on the surface (green) of mitochondria.
Credit: Anne Simonsen
When mitochondria become damaged, they avoid causing further problems by signaling cellular proteins to degrade them. In a paper publishing April 11, 2019, in the journal Developmental Cell, scientists in Norway report that they have discovered how the cells trigger this process, which is called mitophagy. In cells with broken mitochondria, two proteins -- NIPSNAP 1 and NIPSNAP 2 -- accumulate on the mitochondrial surface, functioning as "eat me" signals, recruiting the cellular machinery that will destroy them.
NIPSNAP 1 and 2 are normally found inside healthy mitochondria, although their function inside the cell is unknown. "When a cell's respiration chain is disrupted, and the mitochondria are damaged, import of these proteins into the matrix and inner membrane space of the mitochondria is interrupted," says senior author Anne Simonsen, a professor at the Department of Molecular Medicine at the Institute of Basic Medical Sciences of the University of Oslo. "In that case, the import system does not function and they remain bound to the surface of the damaged mitochondria signaling for mitophagy."
In this study, the researchers studied human HeLa cells where both NIPSNAP1 and NIPSNAP 2 function were eliminated. "When we do that, these cells cannot clear the mitochondria after damage," says Simonsen. However, in cells with functional NIPSNAP proteins, when mitophagy was induced through the addition of a chemical disruptor, they observed that the NIPSNAP proteins act in concert with the PINK and PARKIN proteins, proteins already known to have a role in triggering autophagy and to have a role in Parkinson's Disease.
PARKIN labels cells with ubiquitin, a small protein that directs the cells towards degradation. "Ubiquitin is the classical signal to recruit autophagy," says co-author Terje Johansen (@TerjeJohansen17), of the University of Tromsø -- The Arctic University of Norway. "What we saw is that in addition to ubiquitin, NIPSNAP proteins are required to recruit autophagy proteins; they are not targeted to the mitochondria unless these NIPSNAP proteins are found on the surface."
The team showed this finding has important physiological implications in vivo by investigating the NIPSNAP/PINK/PARKIN mechanism in a zebrafish animal model. They compared wild-type zebrafish and a fish line with reduced NIPSNAP1 protein abundance.
"We see that the mutant fish lacking adequate functional NIPSNAP1 are not able to move as the wild-type fish," says Simonsen. They have a Parkinsonian-like phenotype with reduced numbers of dopaminergic neurons. However, they could rescue this locomotion defect by adding L-dopa, the same compound used to treat human Parkinson's Disease, to the water.
Even more dramatically, animals entirely lacking NIPSNAP1 protein died within five days. "Clearly, clearance of mitochondria is important for the health of these dopaminergic neurons. That is particularly important since neurons generally cannot divide," says Johansen.
As evolutionarily conserved proteins, NIPSNAP proteins are found throughout the animal kingdom, including humans.
This work was partly funded by the Research Council of Norway, the Norwegian Cancer Society, and the Parkinson's Disease Foundation.
Story Source:
Materials provided by Cell PressNote: Content may be edited for style and length.

Journal Reference:
  1. Yakubu Princely Abudu, Serhiy Pankiv, Benan John Mathai, Alf HÃ¥kon Lystad, Christian Bindesbøll, Hanne Britt Brenne, Matthew Yoke Wui Ng, Bernd Thiede, Ai Yamamoto, Thaddaeus Mutugi Nthiga, Trond Lamark, Camila V. Esguerra, Terje Johansen, Anne Simonsen. NIPSNAP1 and NIPSNAP2 Act as 'Eat Me' Signals for MitophagyDevelopmental Cell, 2019; DOI: 10.1016/j.devcel.2019.03.013

https://www.sciencedaily.com/releases/2019/04/190411145152.htm

FoxFeed Blog: Michael J. Fox and Tracy Pollan Visit 'The Tonight Show' on World Parkinson's Day

Posted by  Allison Boiles, April 12, 2019



What better way to cap off World Parkinson's Day than with a visit to The Tonight Show? Yesterday, Michael J. Fox and Tracy Pollan stopped by the studio to chat with host Jimmy Fallon about their secret to a long marriage, the latest progress of The Michael J. Fox Foundation (MJFF) and Tracy's upcoming Mostly Plantscookbook.
After being blown away by the more than $800 million the Foundation has funded in research, Jimmy asked Michael about what's next in Parkinson's disease (PD) research:
"Our ultimate goal is to find a cure and go out of business. We went in to business to go out of business...We're zoning in now on what are the precursors to Parkinson's, who's going to get it, before they get it. And then hopefully we can treat them with some way to stall it and stop symptoms from ever developing."


This Parkinson's Awareness Month, join a community of patients and families uniting in support of a cure for PD. Give to MJFF and support the best scientific ideas so that we can end Parkinson's and close our doors.

https://www.michaeljfox.org/foundation/news-detail.php?michael-fox-and-tracy-pollan-visit-the-tonight-show-on-world-parkinson-day

United for Parkinsons Campaign video

April 11, 2019

https://youtu.be/8tjDP_wdSfo


https://www.youtube.com/watch?t=34s&v=8tjDP_wdSfo&fbclid=IwAR2S5WbJ6xQA0kcUrKrL24gm_10ji8Rz1M2NGg5UuH5DwPlWl96lRMhyHDI&app=desktop

My Partner Also Has a Chronic Disease. The CHRONDI Creed Helps Us.

APRIL 12, 2019  BY DR. C 




There are quite a few articles that talk about caregivers supporting a person with a chronic illness, and that caregiver is usually the spouse. But what is it like when both people in the relationship have a chronic disease? There is a lot less information out there about this predicament and what people can do under these circumstances to maintain a high quality of life. My partner and I have found that the CHRONDI Creed helps us.
When one partner has a chronic illness, 75 percent of marriages end up in divorce. These couples also suffer high levels of stress and burnout — mental health issues. This has a serious impact on the health of the family.
Writers have offered helpful suggestions on how to limit the negative relationship effects of chronic disease, such as working through frustration and addressing irritability. There are also good sources that provide a literature review of self-care, a review of chronic illness as it overlaps with getting older, and a list of reading materials on caregiving.
This information can be applied to the dueling chronic disease dilemma, but none of these articles accurately articulate the chaos that happens.
When both people in the relationship must deal with their own chronic illness and then be there to help their mate with their issues, chaos ensues.
There is just no way around it – couples argue and fight. It’s made worse when both have a chronic disease, with the accompanying issues and needs that must be met. It’s a swirling storm, and you either decide to travel through it together or you decide to go your separate ways.
We have decided to commit to doing whatever necessary to make the journey easier and healthier for both of us. This is where the CHRONDI Creed comes into play.
Compassion (C-CHRONDI) is the foundation for success: taking the time to hear each other’s needs and respond with a gentle heart. Many of these needs fit into the care demands of disease management (R-CHRONDI), but relationship health must address more than the dueling illnesses.
Couples need to experience times of shared happiness (H-CHRONDI), take walks together embracing nature (N-CHRONDI), and support each other with the development of identities separate from the illness (I-CHRONDI).
There is a plus side to both partners having a chronic illness, which is that both are experiencing loss of function (D-CHRONDI) and both benefit from the support of others beyond the relationship (O-CHRONDI).
We both have agreed to put the CHRONDI Creed in place as an umbrella to help us weather the storms we encounter. It is a shared journey, with each of us taking turns holding the umbrella against the fierce winds.
Our relationship has its rough times — fortunately not that often — but it is filled with mutual interests, love, and a spiritual/soul connection. When the warranty on the body runs out and you can’t get new parts anymore, it is these nonmaterial qualities that bring strength to the relationship. We have always been each other’s best friends, and even in the darkest of times, one of us shows up to hold the umbrella for the other one.
Do you and your partner both have chronic illnesses? Use the comments section below to talk about your experience and your coping strategies.
***
Note: Parkinson’s News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or another qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Parkinson’s News Today or its parent company, BioNews Services, and are intended to spark discussion about issues pertaining to Parkinson’s disease.
https://parkinsonsnewstoday.com/2019/04/12/marriage-chronic-disease-chrondi-creed/

Shake it Off

April 13, 2019

Parkinson’s no longer affects the geriatric population only. People in their forties and fifties can also get afflicted with it


Dr AK Sahni
The diagnosis of an early onset of Parkinson’s disease for a dear one can be upsetting for the whole family. Parkinson’s disease (PD) disturbs the mental and the physical harmony of a person. PD’s early onset, especially when an affected person is between 21 and 50 years, is quiet disturbing. According to recent global trends, Parkinson’s is no more a disease that affects the geriatric population: About 10 to 20 per cent of people diagnosed with Parkinson’s in the USA are under 50. Nearly half of the patients are diagnosed before they reach 40 years.
The disease often goes unnoticed, hence remains undiagnosed and untreated for a long period of time. Though the disease is predominantly diagnosed in people in their sixties, its progress varies depending on a person’s health, regardless of his/her age. 
According to the Centres for Disease Control and Prevention (CDC), Parkinson’s is the 14th top reason of mortality in the world. It affects the dopamine-releasing neurons of the human brain and restricts the balanced functioning of the nervous system. The depletion of neurons in the brain impairs the ability of a person to concentrate, balance and work and satisfactorily and normally. 
People, who suffer from Parkinson’s disease, experience tremors in legs, jaw, face, hands and arms, faulty gait due to motor deficits, limb rigidity and problems in balancing their bodies. 
Causes behind early onset
The reasons remain a mystery though many believe genetic and environmental factors to be the triggers that affect dopamine production.Dopamine acts as a neurotransmitter and sends the signals to the brain for controlling the body movements. However, researchers are still struggling to figure out the correlation between genetics and exposure to external environment in causing the disease. According to the National Parkinson’s Foundation, 32 per cent those between 20 and 30 years are affected because of genetic mutations. Environmental reasons such as exposure to insecticides, fungicides and chemical herbicides could raise the risk of  Parkinson’s.  
In rare conditions, the disease is detected in the teenagers and children, which is termed as Juvenile Parkinsonism.   
Delaying measures
Researchers are still trying to solve the mystery to prevent the Parkinson’s completely. There are no definitive prevention measures yet. However, some of these measures may help delay the onset. 
Intake of caffeine: Consuming caffeine-induced products like coffee, tea and cola may protect one from the early onset of the disease, according to a study published by Journal of Alzheimer’s Disease. 
Vitamin D: Increased proportion and consumption of vitamin D in the body is believed to control and reduce the risk of developing Parkinson’s. 

Exercise: Following a regular workout regime and keeping your muscles active can reduce the muscle stiffness and strengthens the body’s ability to reduce the risk factors irrespective of the gender and age. 
Common indicators
Depending on the severity of the disease, symptoms differ and can vary with age, gender and health conditions. Some of the common symptoms are: 
Tremors: People who develop Parkinson’s suffer from tremors in leg, jaw, face, hands and arms, even when your body is at rest. 
Stiffness and rigidity: Stiffness in muscles hinders the proper body movement, which is quite painful when you try to make movements in any direction. 
Inability to perform movements: Walking, smiling, blinking and other minor muscles spasms become a challenge for someone affected by Parkinson’s disease. 
Speech problems: With the level of severity, one can go from speaking softly to blurry, and sometime one also hesitates before speaking anything. 
Risk factors

Parkinson’s has been associated with several conditions that may increase the chances of its early onset. 
Environment: Exposure to chemicals such as manganese, lead and trichloroethylene (TCE) may promote early onset of Parkinson’s. TCEis used primarily to make refrigerants and other hydrofluorocarbons. It is also used in some household products, such as cleaning wipes, aerosol cleaning products, tool cleaners, paint removers, spray adhesives and carpet cleaners and spot removers. 
Besides exposure to these, head injuries and working in the atmosphere where you are exposed to harmful solvents increase the chances of being affected with Parkinson’s disease prematurely.  
Age: With increasing age, the probability of developing Parkinson’s increases, generally starting from the middle age till the age of 60 and more. 
Gender: In comparison to women, men are more prone to developing this disease.
—The writer is head, neurology department, Indian Spinal Injuries Centre, New Delhi
https://www.tribuneindia.com/news/health/shake-it-off/757403.html

Support call for Shropshire Parkinson’s patients left seeking treatment out of county

April13, 2019

A charity has claimed that patients living with Parkinson’s disease in Shropshire have been left without local support due to service closures and inadequate levels of specialist nurses in the area.




Shropshire's neurology service had to temporarily close to new referrals in 2017, with health bosses saying they were unable to manage demand due to staffing difficulties. 
Shropshire patients who were referred to see a neurology specialist were told they would have to travel out of the county. 
Parkinson’s UK says these people often then have no access to ongoing support via a Parkinson’s nurse, at one of the most critical times in their care. 
Previously there were two nurses to cover Shropshire, but this was reduced to just one when a nurse left Shrewsbury and Telford Hospital NHS Trust (SaTH) in June 2018. 
SaTH says future funding for the second post would need to be agreed, but nothing can be done while the service remains closed to new referrals. 

Unacceptable 

Parkinson’s UK is calling for it to reopen as soon as possible and for the vacant post to be filled. 
Benali Hamdache, Parkinson’s UK campaigns manager, said: “It is unacceptable that the lack of support in Shropshire is placing extra strain on patients by forcing them to travel further to get help. 
"There are more than 40 symptoms of Parkinson’s, it’s an incredibly complex and fluctuating condition, which inevitably makes travelling to appointments more difficult. 
“Parkinson’s nurses provide a lifeline to local people by delivering specialist support and can help to bridge the gap between seeing a neurologist." 
Parkinson’s is a progressive neurological condition with no cure, and the charity says Parkinson’s nurses are crucial to supporting people affected. 

Need information? 

Elaine Payne, 62, from Oswestry, was diagnosed with Parkinson’s in 2016. 
She has been unable to access a Parkinson’s nurse since her diagnosis and only sees her consultant every six to eight months. 
She said: “When you’re diagnosed with Parkinson’s it can feel like you’re left on your own, so people with Parkinson’s need someone with that specialist knowledge and care who is totally dedicated to people living with the condition. 
“I really struggle with stiffness and my mobility, and I have trouble putting words together. 
"The more nervous I get the worse my symptoms become, and I’m not the person I used to be. 
"I would love to have access to a Parkinson’s nurse to discuss my symptoms and medication, and find out if what I’m experiencing is normal or not. 
“There are so many people in Shropshire with Parkinson’s, so it seems crazy that there is only one Parkinson’s nurse, and it means lots of us are missing out on that vital specialist support.” 

Funding 

Since the service closed to new referrals, SaTH has been involved in talks with other organisations including the Walton Centre in Liverpool, in the hopes it can offer an outreach service in the county. 
But it is not yet known when Shropshire's neurology service will be back on track. 
Carol McInnes, assistant chief operating officer for unscheduled care at SaTH, said: “Our neurology service is currently suspended to new referrals and has been since March 28, 2017. 
“During that time one of our two specialist nurse posts, which was funded by charitable funds, ended. 
"Future funding for this post needs to be agreed and a business case has been developed to support this, however as the neurology service remains closed, the service has not seen any new activity since 2017. 
“The existing specialist nurse continues to support those patients living with Parkinson’s, who were referred to SaTH prior to suspension of the neurology service. 
"Patients have been offered a choice of other providers commissioned by the clinical commissioning group.” 
Neurology services treat conditions affecting the nervous system, including the brain, spinal cord, nerve, muscles and their connections.
https://www.shropshirestar.com/news/health/2019/04/13/support-call-for-shropshire-parkinsons-patients-left-seeking-treatment-out-of-county/

New Treatment May Have the Potential to Slow, Stop, or Reverse Parkinson Disease

April 12, 2019  Wallace Stephens

Results from a recent study suggest that a revolutionary treatment may have the potential to slow, stop, or even reverse the progression of Parkinson disease.

Results from a February study of a revolutionary treatment suggest that it may be possible to slow, stop, or even reverse the progression of Parkinson disease, according to findings in the Journal of Parkinson’s Disease.

The 3-part, experimental study investigated whether using a novel delivery system to increase levels of glial cell line-derived neurotrophic factor (GDNF) can regenerate dying dopamine brain cells in patients with Parkinson disease and even reverse their condition. GDNF is a naturally occurring protein that promotes the survival of many types of neurons.

“I believe that this approach could be the first neuro-restorative treatment for people living with Parkinson's, which is, of course, an extremely exciting prospect,”  Steven Gill, MB, MS, FRCS, who designed the infusion device used in the study, said in a statement.

Researchers used robot-assisted neurosurgery to implant a specially designed delivery system, termed Convection Enhanced Delivery (CED), which allowed high flow rate infusion to be administered to patients every 4 weeks. Patients underwent a procedure to have 4 tubes implanted within their brains. GDNF was directly infused to targeted locations via a skull-mounted transcutaneous port located behind the ear. Following implantation, the trial team administered a total of more than 1000 brain infusions throughout the study. Patients demonstrated a high compliance rate of 99.1% and confirmed clinical feasibility and tolerability toward the administration process for repeated brain infusions.

Initially, 6 patients enrolled in a pilot study which evaluated the safety of the treatment approach. After the pilot study, 35 additional individuals participated in a subsequent 9-month double-blind trial. Half of the participants were randomly assigned to receive monthly infusions of GDNF while the other half received placebos.

Positron emission tomography (PET) brain scans from the group who received GDNF showed an improvement of 100% in the targeted area of the brain affected by Parkinson disease, leading researchers to believe that the treatment may have the potential to reawaken or restore damaged brain cells. There was no change in PET brain scans among the group that received placebo. After the trial period, no improvements in symptoms were observed in either group.

"The spatial and relative magnitude of the improvement in the brain scans is beyond anything seen previously in trials of surgically delivered growth-factor treatments for Parkinson's," the study's principal investigator Alan L. Whone, PhD, FRCP, Translational Health Sciences, Bristol Medical School, University of Bristol, said in a statement. "This represents some of the most compelling evidence yet that we may have a means to possibly reawaken and restore the dopamine brain cells that are gradually destroyed in Parkinson's."

Following the 9-month treatment period, a third, open-label extension trial was performed to investigate the effects and safety of continued exposure to GDNF for an additional period of 40 weeks. The group of patients who were already receiving GDNF continued to be exposed to the growth factor. The group that was receiving placebo for the first 40 weeks was then given GDNF.

The study's primary endpoint was the percentage change in the OFF state Unified Parkinson’s Disease Rating Scale (UPDRS) motor score from baseline to the conclusion of the 80-week period between the group that consistently received GDNF and the group that received placebo and then GDNF.

Although symptoms in both groups were found to either moderately or significantly improve after 18 months, once all participants had received GDNF, no significant differences between the groups in the primary or supplementary endpoints were observed throughout the study. However GDNF treatment was attributed to a significant increase in dopamine uptake.

Safety was assessed according to occurrences of adverse events (AEs), presence of anti-GDNF antibodies, and routine laboratory testing . While all patients experienced at least 1 AE between week 40 to week 80, none led to the discontinuance of receiving study medication. Serious AEs were reported for a total of 8 patients but were determined to be unrelated to study medication. GDNF was determined to by safe when administered over an extended time period.

"This trial has shown that we can safely and repeatedly infuse drugs directly into patients’ brains over months or years. This is a significant breakthrough in our ability to treat neurological conditions, such as Parkinson’s, because most drugs that might work cannot cross from the blood stream into the brain due to a natural protective barrier,” said researchers in a statement.

Researchers believe that further studies are required to determine whether GDNF can reverse, slow, or stop the progression of Parkinson disease. They recommend implementation of a larger scale study that would administer higher doses of GDNF.

The study’s lead author emphasized, "It's essential to continue research exploring this treatment further. GDNF continues to hold potential to improve the lives of people with Parkinson's.”

Reference 

Whone AL, Boca M, Luz M, et al. Extended treatment with glial cell line-derived neurotrophic factor in Parkinson’s disease. J Parkinsons Dis. 2019;9(2). doi: 10.3233/JPD-191576

https://www.ajmc.com/newsroom/new-treatment-may-have-the-potential-to-slow-stop-or-reverse-parkinson-disease