Coping with Change While Changing Beliefs

One of my common themes is “Coping with Change.” Kennedy’s Disease forces you to cope with the unplanned changes in your life. Until you accept the changes, it will be difficult to move forward again. Having a strong belief system is as important as coping.

Spencer Johnson has a way with words. He has written over twenty books and sold tens of millions of copies of Who Moved My Cheese? and The One Minute Manager. Most people have heard of The One Minute Manager, but his second book is not as well known, yet is better for those of us dealing with unexpected change in our lives. You can read the book in an hour or so, but you might find yourself talking about it and reading it again.

Who Moved My Cheese?

“It is an extended allegory based on the experiences of four mice who live in a maze that has cheese located at a particular location. Then one day, the cheese is moved to a new location, and their differing reactions determine their success in the new state of affairs.

In this artful way, Spencer Johnson introduces the reader to his fable on how to cope positively with change. Here is four short quotes from the book.

· “What would you do if you weren't afraid?” ...

· “What you are afraid of is never as bad as what you imagine. ...

· “When you stop being afraid you feel good” ...

· “The quicker you let go of old cheese, the sooner you find new cheese.””

Mr. Johnson passed away in 2017 and left behind the unpublished book, Out of the Maze - This is a story about the power of belief. In this month’s CostCo Connection, Adrian Zackheim writes about “The Power of Story.” It is the long awaited sequel. Adrian Zackheim has taken the drafts and published the book.

Out of the Maze

“Who Moved My Cheese? offered millions of readers relief for an evergreen problem: unanticipated and unwelcome change. Now its long-awaited sequel digs deeper, to show how readers can adapt their beliefs and achieve better results. Johnson's theme is that all of our accomplishments are due to our beliefs: whether we're confident or insecure, cynical or positive, open-minded or inflexible. But it's difficult to change your beliefs - and with them, your outcomes. Find out how Hem, Haw, and the other characters from Who Moved My Cheese? deal with this challenge.”

Here are six key principles from the book:

· Don’t believe everything you think.

· Old beliefs do not lead you to new beginnings.

· You can change your mind and choose a new belief.

· You are not your belief. You are the person who chooses your beliefs.

· Let go of whatever isn’t working.

· Look outsize the maze. Consider the unlikely, and explore the impossible.

You can find Who Moved My Cheese? at most public libraries or at Amazon. I can’t wait to read the sequel.

Study: Systemic Delivery of MicroRNA

István Reinhardt emailed me a link to a recent Kennedy’s Disease (SBMA) study. The video is also interesting. Clink on the link below to go to the site or download a PDF copy of the study with this link:  https://www.jove.com/pdf/55724

Systemic Delivery of MicroRNA Using Recombinant Adeno-associated Virus Serotype 9 toTreat Neuromuscular Diseases in Rodents

ABSTRACT

RNA interference via the endogenous miRNA pathway regulates gene expression by controlling protein synthesis through post-transcriptional gene silencing. In recent years, miRNA-mediated gene regulation has shown potential for treatment of neurological disorders caused by a toxic gain of function mechanism. However, efficient delivery to target tissues has limited its application. Here we used a transgenic mouse model for spinal and bulbar muscular atrophy (SBMA), a neuromuscular disease caused by polyglutamine expansion in the androgen receptor (AR), to test gene silencing by a newly identified AR-targeting miRNA, miR-298. We overexpressed miR-298 using a recombinant adeno-associated virus (rAAV) serotype 9 vector to facilitate transduction of non-dividing cells. A single tail-vein injection in SBMA mice induced sustained and widespread overexpression of miR-298 in skeletal muscle and motor neurons and resulted in amelioration of the neuromuscular phenotype in the mice.

In a 2011 post, Ed Meyerthoen explained the defective AR gene. You can read his post by following this link:  https://kennedysdisease.blogspot.com/2011/05/what-is-ar-and-why-is-it-important.html

New Funding for SBMA Research

The MDA announced the funding of $9.9 million dollars in research grants. The grant below is for the study of Kennedy's Disease.

Carlo Rinaldi, Ph.D., associate professor and clinician scientist at the University of Oxford in England, was awarded an MDA Development Grant to study how variants in the gene of the androgen receptor (AR) protein cause spinal-bulbar muscular atrophy (SBMA) and investigate whether a novel antisense oligonucleotide therapy approach can target these toxic variants. The results of the study will provide a better understanding of SBMA disease mechanisms, as well as develop a new therapy potentially capable of treating the disease.

This work has potential implications for other diseases of the motor unit as well, including spinal muscular atrophy (SMA) and ALS. This grant is co-funded by the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM).

Additional information was provided on another page:

“Better understanding of the underlying disease mechanisms, coupled with improvement of gene vector design, therapeutic gene selection, and methods of delivery, have made gene therapy a realistic option for neuromuscular conditions — and neurological diseases in general — for which no treatment option was available until few years ago. Many challenges still lie ahead, but we have good reasons to be very optimistic for the future.”

Carlo Rinaldi, associate professor and clinician scientist at the University of Oxford in England, was awarded an MDA Development Grant totaling $120,000 over 3 years to study the role of androgen receptor isoforms in SBMA pathogenesis and the potential as therapeutic targets. This grant is co-funded by the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM).

Mutations in the gene encoding the androgen receptor (AR) protein cause spinal and bulbar muscular atrophy (SBMA). SBMA is an adult-onset neuromuscular condition affecting males with unmet clinical need. It is not undestood how mutations in AR lead to primary degeneration of motor neurons and muscle in patients. The activity of AR and other hormone receptors can be modulated in human cells by isoforms and/or splice variants, which may block or enhance their functions.

Dr. Rinaldi and colleagues plan to investigate the role of AR alternative isoforms in mediating SBMA toxicity. By revealing how these isoforms regulate AR activity in health and disease, researchers expect to better understand the mechanisms of disease in SBMA and provide a novel rational therapeutic target. If successful, the work could pinpoint tissue-specific targets for therapy development, with implications not only for SBMA but for other diseases of the motor unit as well, including spinal muscular atrophy and amyotrophoic lateral sclerosis.

UCL KD Research Newsletter

Below is the first newsletter from the UCL Kennedy's Disease Research Center. Many thanks to all who provide support as well as search for a treatment and a cure.

FYI - Kennedy’s Disease Clinic

This clinic is linked to the National Register for Kennedy’s Disease and is aimed at providing a central referring point for all patients in the UK. Kennedy’s disease (also known as Spinal Bulbar Muscular Atrophy) is a rare disorder and the Clinic will offer the multi-disciplinary approach available for MND and also provide screening for a number of non-neurological conditions that may associate with Kennedy’s Disease.

Coordinator for this clinic is Jan Clarke (jan.clarke1@nhs.net - Telephone: 020 3448 3517); general enquiries Marcia Forde (marcia.forde@nhs.net - Telephone: 020 3448 8251 - Fax: 020 3448 3633).

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UCL KD Research Newsletter

Hi and welcome to the inaugural research newsletter from the !

We always enjoy letting you know about exciting developments in KD research and care, and a few members of the KD community have asked to know more about the research happening at UCL, Oxford and further afield – so here we are!

We’re planning to update you with research news four times a year. We would really love to hear your feedback on what you like and what doesn’t work so well, so that we can improve what we are sending you. We really want it to be the most useful and interesting for you that it can be. Please do let us know at sbma@ucl.ac.uk.

KD Clinic

It has been wonderful to see so many faces coming through the KD clinic.

From the medical side we have a new consultant, Dr Carlo Rinaldi, who has many years’ experience in KD. Carlo runs a research group at Oxford University studying ways to develop new treatments for KD. Dr Helen Devine, a registrar, has returned from maternity leave and is joining the clinic alongside her PhD using stem cells to study KD.

KD research

There is plenty of ongoing research linked to the clinic, both in UCL and in Oxford. In some cases people with KD have been directly involved, for example: undergoing muscle MRI scans, or donating blood or skin samples that are currently being analysed in the lab. There are also numerous ongoing studies that use disease models to better understand KD and to find ways of changing its course.

We will include updates of these projects in upcoming newsletters.

Results from Japan using testosterone-lowering drug

Many of you have asked us about the results from a clinical trial recently published by colleagues in Japan.

Background: Our bodies naturally produce testosterone. In people with KD, this testosterone binds to faulty androgen receptors – and that causes damage to the nerves and muscles. Gen Sobue’s KD lab in Japan wanted to see if less testosterone would mean less activity of the androgen receptor – and therefore less damage. There is already a drug called Leuroprelin that makes men produce less testosterone. A team lead by the scientist Atsushi Hashizume ran a long-term trial giving Leuroprelin to some people with KD in order to reduce the levels of testosterone in their bodies, to see if they stayed healthier than others who were not given the drug. The scientists chose people who were similar in terms of age, length of disease and CAG repeat length. The first results were published in 2009 and actually showed that there was no clear benefit to people with KD after 18m of treatment.

Results from this study: Treatment was continued with Leuroprelin after the original study end and now the effect of the drug after up to 11.5 years in the longest-term patients has been published. Encouragingly, the researchers found that, over the observed time period, the people who were given Leuroprelin were less likely to develop pneumonia requiring hospitalisation, and they had a slower progression of disease.

Our view: The positive finding of this study is that reducing the action of testosterone can impact on the disease course of KD. On the cautionary side, however, the benefits are modest, and the drug, when taken chronically, has some side-effects.

In summary, although Leuprorelin may not prove to be clearly beneficial for people with KD, these results show that modifying testosterone can have an impact on disease, which brings optimism for future drug therapies.

Symptom management

We thought it would be useful to hear how you deal with KD’s most common and troublesome symptoms to create a resource for all of us to share, review and access.

So our first question is: what are the best strategies you have found to manage laryngospasm?  If you wish to contribute please e-mail Luca at: luca.zampedri@nhs.net

Best wishes,
Pietro, Carlo, Mike, Linda, Helen, Jan and Luca.

Airway Obstruction after Robot-Assisted Laparoscopic Prostatectomy

This PubMed article was forwarded to me from another man with Kennedy's Disease. Read the entire article by following the title link.

It once again reiterates the need for extra caution when preparing for surgery. Discussions with the surgeon and the anesthesiologist are warranted.


Previously Undiagnosed Spinal and Bulbar Muscular Atrophy as a Cause of Airway Obstruction after Robot-Assisted Laparoscopic Prostatectomy

"... CONCLUSIONS: Vocal cord paralysis combined with postoperative laryngeal edema, the cause of which was presumed to be SBMA, likely caused airway obstruction after RALP. As neuromuscular symptoms progress gradually in patients with SBMA, muscle relaxants should be used carefully, even if patients with SBMA present no immobility of their extremities."

CRISPR - Gene Editing Explained

A man living with Kennedy's Disease posted this on Facebook. The short YouTube video (link below) does a good job of explaining what CRISPR is, how it works, and its potential. Those of us living with KD are very interested in the potential tools like CRISPR offer for future generations.

Biologist Explains One Concept in 5 Levels of Difficulty - CRISPR

Video published May 24, 2017

CRISPR is a new area of biomedical science that enables gene editing and could be the key to eventually curing diseases like autism or cancer. WIRED has challenged biologist Neville Sanjana to explain this concept to 5 different people; a 7 year-old, a 14 year-old, a college student, a grad student and a CRISPR expert.

MiR-298 Counteracts Mutant Androgen Receptor Toxicity in Kennedy's Disease

Below is a link to research on Kennedy's Disease that was published in January in Molecular Therapy.

MiR-298 Counteracts Mutant Androgen Receptor Toxicity in Spinal and Bulbar Muscular Atrophy

 Naemeh Pourshafie, Philip R Lee, Ke-lian Chen, George G Harmison, Laura C Bott, Masahisa Katsuno, Gen Sobue, Barrington G Burnett, Kenneth H Fischbec¹ and Carlo Rinaldi

Abstract

Spinal and bulbar muscular atrophy (SBMA) is a currently untreatable adult-onset neuromuscular disease caused by expansion of a polyglutamine repeat in the androgen receptor (AR). In SBMA, as in other polyglutamine diseases, a toxic gain of function in the mutant protein is an important factor in the disease mechanism; therefore, reducing the mutant protein holds promise as an effective treatment strategy. In this work, we evaluated a microRNA (miRNA) to reduce AR expression. From a list of predicted miRNAs that target human AR, we selected microRNA-298 (miR-298) for its ability to downregulate AR mRNA and protein levels when transfected in cells overexpressing wild-type and mutant AR and in SBMA patient-derived fibroblasts. We showed that miR-298 directly binds to the 3’-untranslated region of the human AR transcript, and counteracts AR toxicity in vitro. Intravenous delivery of miR-298 with adeno-associated virus serotype 9 vector resulted in efficient transduction of muscle and spinal cord and amelioration of the disease phenotype in SBMA mice. Our findings support the development of miRNAs as a therapeutic strategy for SBMA and other neurodegenerative disorders caused by toxic proteins.

 Follow the above link for the entire article.

Non-neural phenotype of spinal and bulbar muscular atrophy

Below is an abstract of a research paper published last year. This study was focused on non-neurological characteristics in 73 patients with Kennedy’s Disease. 

“Non-neurological clinical features have not been extensively investigated in previous reports. The aim of the present study was a deep characterisation of the involvement of the main androgen-responsive tissues in a large collection of patients with SBMA. The findings highlight novel non-neural dysfunctions and a wide phenotypic spectrum, suggesting the need for a comprehensive, multidisciplinary approach to SBMA.

Abstract

Objective To carry out a deep characterisation of the main androgen-responsive tissues involved in spinal and bulbar muscular atrophy (SBMA).

Methods 73 consecutive Italian patients underwent a full clinical protocol including biochemical and hormonal analyses, genitourinary examination, bone metabolism and densitometry, cardiological evaluation and muscle pathology.

Results Creatine kinase levels were slightly to markedly elevated in almost all cases (68 of the 73; 94%). 30 (41%) patients had fasting glucose above the reference limit, and many patients had total cholesterol (40; 54.7%), low-density lipoproteins cholesterol (29; 39.7%) and triglyceride (35; 48%) levels above the recommended values. Although testosterone, luteinising hormone and follicle-stimulating hormone values were generally normal, in one-third of cases we calculated an increased Androgen Sensitivity Index reflecting the presence of androgen resistance in these patients. According to the International Prostate Symptom Score (IPSS), 7/70 (10%) patients reported severe lower urinal tract symptoms (IPSS score >19), and 21/73 (30%) patients were moderately symptomatic (IPSS score from 8 to 19). In addition, 3 patients were carriers of an indwelling bladder catheter. Videourodynamic evaluation indicated that 4 of the 7 patients reporting severe urinary symptoms had an overt prostate-unrelated bladder outlet obstruction. Dual-energy X-ray absorptiometry scan data were consistent with low bone mass in 25/61 (41%) patients. Low bone mass was more frequent at the femoral than at the lumbar level. Skeletal muscle biopsy was carried out in 20 patients and myogenic changes in addition to the neurogenic atrophy were mostly observed.

Conclusions Our study provides evidence of a wide non-neural clinical phenotype in SBMA, suggesting the need for comprehensive multidisciplinary protocols for these patients.”

The full article can be found here:  http://jnnp.bmj.com/content/early/2015/11/05/jnnp-2015-311305.full?sid=d584bac7-cae4-4d14-9047-84fd5e49d918

Regaining Control of your Life

If you are a follower of my blog, you know that I use positive sayings to influence how I live. If I see that I need an attitude correction, for example, I’ll start digging through my notes and books I read until something strikes home.

Living with Kennedy’s Disease, or any progressive neuromuscular disorder, requires regular attitude adjustments or you can find yourself wallowing in the gutter. Thoughts are powerful and can change your perceptions immediately.

Today’s topic is the beginning of my "Words of Power" series. These words are important to me. It helps keep me going, especially when I am a little down.

“My life is controlled by what I accept and believe”

This is a powerful statement. One that forces me to place any blame for a negative attitude on myself. My wife mentioned recently that I never complain. My response was, “Why should I? No one ever listens.” 

Unfortunately, my comment isn’t entirely true. I LISTEN! My subconscious listens to what I think. I don’t even need to verbalize the thought for it to do damage.

For that reason,

“I choose to entertain only positive and nourishing thoughts”

That doesn’t mean something negative or destructive doesn’t try to wedge its way in occasionally. But, when I realize what is happening, I pull out my gratitude journal and just read a few pages of my random thoughts. 

B.K. Clive said it best. ““Today, just take time to smell the roses, enjoy those little things about your life, your family, spouse, friends, and job. Forget about the thorns -the pains and problems they cause you - and enjoy life” 

 

Mitochondrial Dysfunction in Kennedy’s Disease

I came across the following on the Kennedy’sDisease – Raising Awareness Facebook page:

I have been taking Acetyl L-Carnitine since September and have seen a dramatic improvement in the reduction of muscle fatigue, resulting in greater activity and better health overall. I started with L-Carnitine at 500 mg to see if there were any side effects, then after one month I switched to Acetyl L-Carnitine (which crosses the blood/brain barrier), again 500mg to start then 1000. Its hard to quantify what 'better' is in our lives, but.... so far this month, I have built 12' of cabinets in the dining room, built a 15x16ft glass greenhouse at the end of the drive and moved two pallets of brick pavers and two yards of soil. All jobs that have been waiting for several years.

There was also a link to the study and I have posted that below.

First, I must qualify myself. I am not a doctor or medical student. So, any comments are strictly of a layman with an interest. In reading the article, I noticed it was not a study. This article reported the findings in studying one patient with KD. It proposes the possibility of this regiment in helping improve energy (reducing muscle fatigue). The article proposes a more complete study to determine if the initial findings can be duplicated.

When there was a lot of excitement from other Facebook members wanting to give this a try, the gentleman responded with the following caution.

Please, everyone, little steps.... no one gains if you break your leg running to the store. Try a small dose in the morning with food if you like for a few days. It is expected to help with muscle fatigue only, so that means embarking on some activity as well. The aim is to feel better; not eliminate the disease. Too much too soon and you may experience side effects that remove it as a therapeutic tool.

This is sound advice. One patient doesn’t make a study. IMPORTANT: Always consult with your primary doctor and your neurologist before beginning any supplements, especially in high dosages.

Of course, anyone with SBMA wants to know more, so I looked up the definition:

Mitochondria: Structures located in the cell's cytoplasm outside the nucleus. Mitochondria are responsible for energy production. Each consists of two sets of membranes: a smooth, continuous outer coat and an inner membrane arranged in tubules or in folds that form plate-like double membranes (cristae). The mitochrondria are the principal energy source of the cell. They not only convert nutrients into energy but also perform many other specialized tasks. Each mitochondrion has a chromosome that is made of DNA but is otherwise quite different from the better-known chromosomes in the nucleus. The mitochondrial chromosome is much smaller than other chromosomes. It is round, whereas the chromosomes in the nucleus are shaped like rods. There are many copies of the mitochondrial chromosome in every cell, whereas there is normally only one set of chromosomes in the nucleus. All mitochondrial chromosomes are inherited from the mother.

Reference: NIH, US Library of Medicine, Genetics Home Reference. Mitochondrial DNA. 

 

I also looked up possible side effects for this supplement and this is what I found at WebMid.  

Acetyl-L-carnitine is LIKELY SAFE for most adults. It can cause some side effects including stomach upset, nausea, vomiting, and restlessness. It can cause a "fishy" odor of the urine, breath, and sweat.


Under-active thyroid (hypothyroidism): There is some concern that acetyl-L-carnitine might interfere with thyroid hormone. Don’t use acetyl-L-carnitine if you have an under-active thyroid.

Seizures: An increase in the number or seriousness of seizures has been reported in people with a history of seizures who have used L-carnitine by mouth or by IV (intravenously). Since L-carnitine is related to acetyl-L-carnitine, there is a concern that this might also occur with acetyl-L-carnitine. If you have ever had a seizure, don’t take acetyl-L-carnitine.

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Mitochondrial dysfunction in Kennedy’s disease: a new pharmacological target?

Chong Wang, Wei Chen, Dan Miao, Jin-Tai Yu, and Lan Tan

Abstract

Background

Mitochondrial impairment and elevated oxidative stress have been implicated in the pathogenesis of Kennedy’s disease. However, there is still no study describing the mitochondrial nutrient management in patients with Kennedy’s disease.

Methods
We assessed the clinical and electrophysiological features in a patient with Kennedy’s disease. This patient was diagnosed by genetic analysis. We also measured the plasma 8-hydroxydeoxyguanosine (8-OHdG) levels of the patient and his family members using commercial enzyme-linked immunosorbent assay (ELISA). Treatment with intravenous L-carnitine (2 g/day) for the patient was started on admission, followed by two weeks.
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Goto this link to read the entire article

Image:  http://catalog.flatworldknowledge.com/bookhub/reader/2547?e=gob-ch20_s04

Listening to your body

This month’s Costco Connection has an interesting article by Laurie Gardner titled Listening to your body’s deeper messages. The article focuses on general health issues, but it is relevant for those of us living with Kennedy’s Disease also. I have condensed the article and added my own thoughts to it focusing more on SBMA or other progressive neuromuscular disorders.

The message is, "The next time you feel as if your body is letting you down, stop and listen to any deeper messages it might be trying to give you."

“The next time you feel as if your body is letting you down, stop and listen to any deeper messages it might be trying to give you. To hear your body (which is about hearing your own gut instinct and inner wisdom) go to a quiet place with no distractions and get into a position where you can feel relaxed. 

Ask the following questions, and see what answers naturally arise. You might be surprised by how much information your body will tell you when you are open to listening.

1.  Is this just a physical issue, or something deeper going on?
2. Is this temporary or permanent, and how can I get through it?
3. What does my body need today?”

Question 1 is important for us because not everything that happens to us is related to Kennedy’s Disease or our neuromuscular condition. If you are feeling something new (different from normal), try to isolate the sensation. Just because we have this rare medical condition doesn’t mean we can’t have some other more common medical condition. Loss of energy could be a thyroid issue, for example. Short-term muscle weakness could be a dietary issue. Shortness of breath can be several things. I think you get the idea. When in doubt, ask your doctor.

Question 2 is something that we all deal with. Intermittent weakness or fatigue is common, especially if we overdo. There have been days when I can hardly exercise ‘safely’. When this happens, I tell myself, “This too will pass.” Other times, I come to the realization that this is part of the progression process and I need to adjust my routines to accommodate the latest loss of mobility or use of some part of my body.

Question 3 will get you through those moments of “OMG”, this can’t be happening to me. “Each day, your body will feel different and have different needs based upon your level of energy.” By asking what my body needs today, it is easier to come up with a viable short-term solution.  If you overdid, it might be time to take a break and rest. I remember when I abused my muscles one day and paid the price the next. 

“The key point is that introspection is an important part of finding the best way to health. Your instincts can help you determine if you need rest, a lifestyle adjustment or professional help.”

Show your support for NIH

MDA's Outreach Program sent out a message this week that needs to be passed along and supported by all those living with a neuromuscular disease (ALS, SBMA, SMA, etc.).

As many of you know, NIH is currently conducting a clinical trial for a treatment for Kennedy's Disease. If this phase goes well and the treatment is determined to be safe, NIH will move to the next phase and open the trial up to a larger group of patients.

Read the message from the MDA below and consider supporting the proposed increase to NIH spending.

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Increase Funding for the National Institutes of Health (NIH)

Increased funding for the National Institutes of Health (NIH) is critical to finding treatments and cures for the neuromuscular diseases that affect our community. Together, with our generous donors and sponsors, MDA is working to fund groundbreaking research and bring critical support to families. Collaboration-including leveraging federal funding-is vital to speed urgently needed treatments to affected individuals as quickly as possible.

Ensuring increased NIH funding is one of MDA's top policy priorities. Through targeted efforts and strategic initiatives, MDA is urging Congress to boost funding for NIH. Increased support for NIH is critical, as funding levels have failed to keep up with the rising costs of medical research-resulting in a 22% decrease in purchasing power since 2003, when adjusted for inflation. This lack of funding has resulted in a lower grant application success rate and hinders the ability of NIH to conduct and support important biomedical research.

With many voices carrying the message that medical research is imperative to finding treatments and cures, lawmakers have placed increased NIH funding on the Congressional agenda in both the Senate and the House of Representatives.

One example is the 21st Century Cures Act draft released May 13, 2015, by the House Energy &

Commerce Committee. This draft would authorize an increase in NIH funding and would create an NIH Innovation Fund that would increase NIH funding by $10 billion over five years. MDA applauds the bipartisan efforts of the Energy & Commerce Committee for making NIH funding a priority and appreciates the efforts of Chairman Fred Upton (R-MI-06), and Rep. Diana DeGette (D-CO-01), Frank Pallone (D-NJ-06), Joe Pitts (R-PA-16) and Gene Green (D-TX-29) to ensure that increased NIH funding is included in the draft legislation.

In addition to the 21st Century Cures Act, several pieces of stand-alone legislation in the House and Senate support increased NIH funding at various levels. MDA appreciates the efforts of every lawmaker who takes action to increase funding for medical research.

Please click the button below to urge your Congressional leaders to support increased NIH funding. 

Clinging to ‘What was’

Those of us living with Kennedy’s Disease often find ourselves “wishing, and hoping, and praying” for a treatment or cure for this condition. I feel this process is healthy as long as it doesn’t become an obsession. 

There is another emotion not as healthy, however. FEAR … of what is happening … of what will happen … of what I will become … of what I am doing to my loved one. Fear of the unknown is something most of us experience at some time. It can be paralyzing. It can also cause other health issues and be destructive to relationships.

Yet, when you really look at the actual fear, it is usually based on ‘worst case’ assumptions and scenarios. 

I have mentioned several times in this blog how fortunate we are to have Kennedy’s Disease (SBMA) when compared to many other incurable conditions. “Fortunate!” Are you asking if I am crazy about now? Yes, perhaps I am, but that is beside the point. What I mean about being fortunate is “the progression is slow.” The snail’s pace of the progression allows us to accept and adapt. The progression is so slow at times, it can feel like it stopped. 

No matter how hard we try, there are times that the fear of ‘what is’ and ‘what will’ penetrates our rational mind. It usually surfaces when we are experiencing a new symptom–something we had escaped until this moment. The fear drives a wedge into our comfort zone; tearing at the delicate membrane of ‘acceptance’ that we so cherish and need. 

We were comfortable not having to consider ‘what next’, but now the question explodes into our consciousness. “WHAT NEXT!”

 

The truth is that we don’t know what is going to happen next. To speculate is unproductive and unhealthy. There is only ‘what is’. Before we can move on, however, we need to accept and adapt to the current change in our condition. I don’t believe suppressing the fear works. I believe you need to face it, analyze it, and break it down into facts and truths before you can rid yourself of the speculation of ‘what is to come’. 

For me, it helps to close my eyes and take slow deep breaths (abdominal breathing). When I am relaxed, I smile. Things never seem quite as bad after I smile. When my thoughts turn toward the unknown, or what might happen tomorrow or in the future, I take another slow deep breath and ask, “What is happening right now?” There is something calming about this practice. 

That doesn’t mean you shouldn’t plan for the future. Preparedness is good. Most of us are good problem solvers, so when I ask, “If this happens, then how do I continue to be safe, loving, productive, mobile and functioning,” I address a potential need in my life. 

 

Now, before you disregard everything I wrote today, close your eyes, take a slow deep breath …and smile. I am.