ACKNOWLEDGEMENTS, COMPETING INTERESTS, and FUNDING:
The authors would like to thank our fellow BFS patients for taking part in this survey and study. Due to their participation a decent sample size was obtained for this study and subsequently brought forth pertinent statistical information about the BFS ailment.
The authors received no funding for this work and have no competing interests.
Appendix I
Methods: Study Background
This survey meets human research criteria as outlined by the “Committee on Human Experimentation” and the “Helsinki Declaration of 1975” for the following reasons: 1. The survey was anonymous; 2. The participation in the survey was voluntary; 3. The privacy and confidentiality of the participants is maintained and protected; and 4. Survey participants were notified in advance that results would be shared publicly.
A video example of chronic BFS twitching in the primary author’s lower leg can be found on his website: http://patrickbohan.elementfx.com/BFS.htm. All tabulated data in this paper is original. The survey and subsequent data was not a clinical trial of any kind. However, the survey consisted of control questions to eliminate people from the data analysis who have not met at least two of the following conditions: 1. They have been diagnosed with BFS by a physician; 2. Their symptoms were bad enough to warrant an EMG; and 3. their symptoms were bad enough to warrant a MRI. This eliminates any survey participants who may falsely input responses into the survey and or may not have BFS. Finally, the authors of this paper have no conflicts of interest and therefore, no information to disclose. In fact, the authors are independent and have no affiliation to any university, group, organization, or company what so ever and therefore, received no funding for this project.
Appendix II
Methods: User Groups
The subjects for this study included 438 individuals who have been formally diagnosed with BFS and or had to have an EMG or MRI to rule out more serious ailments. If participants in the survey answered “no” to more than one of the 3 following questions: 1. “Have they been officially diagnosed with BFS?”; 2. “Have they had an EMG?”; and 3. “Have they had an MRI?”; they were omitted from the data analysis. This is the only way to ensure the survey participants have BFS and are not fabricating some response in the survey.
People were contacted via social network forums listed below to participate in the survey:
Facebook: https://www.facebook.com/#!/groups/88467288815/
Internet: http://www.nextination.com/aboutbfs/
It is understood and highly probably people who seek to join social networking sites dealing with medical conditions generally have a chronic condition. If the symptoms were inconsequential or insignificant then why would anyone reach out for assistance? Therefore, this is a study of people with chronic BFS conditions since there are probably very few people in the survey with mild BFS. This may skew results, but treatment is needed for chronic sufferers of BFS and not necessarily for mild sufferers of the syndrome.
Appendix III
Methods: Survey and Data
Information regarding all data gathering and the survey / tools used, is listed below:
A survey was created in Google Docs and can be found at the following link: https://spreadsheets.google.com/spreadsheet/viewform?hl=en_US&authkey=CJvBgaQM&formkey=dElCQkFBRWlvY1ZSTThKTmNsbEg4d0E6MQ#gid=0
The survey will be open indefinitely with the hope to grow the sample size and therefore, better understand the disorder.
The Survey can also be reached from the Author’s BFS webpage: http://patrickbohan.elementfx.com/BFS.htm Click on the link “BFS Survey”.
The data results for the 527 participants (total participation was 527; however, 89 were considered outliers due to not meeting certain criteria for this study) can also be found on my web page: http://patrickbohan.elementfx.com/BFS.htm. Click on the “Survey Data Summary” link. This will open an excel file. The “BFS” and “BFS No Zero” tabs contain the raw survey data with outliers removed (These tabs were used to calculate the statistics within this paper). The “BFS NO” and “BFS No Zero NO” tabs contain the raw survey data with No Outliers excluded. The “Data Summary” tab contains a complete statistical analysis of each variable or question in the survey. The “Statistical Significance 500+” tab contains the statistical significant data between variables (t-statistic data). [8] The “Correlation Results” tab in the excel file contains the correlation data between variables. Only those variables that show high statistical significance are analyzed for correlation. Since the survey data is based on a rank-order system (ordinal data), the Spearman method of correlation is used. [9] This excel file is included as supplementary data with this writing.
Appendix IV
Methods: Data Analysis
The data was first analyzed to determine if outliers exist. Survey participants who had more than 3 responses (data points) outside of plus or minus 3 standard deviations were considered outliers and omitted from the calculation by placing brackets [] around the result in the raw data on the “BFS” tab in the excel file.
Most of the linear regression models generated from the BFS survey have very low adjusted R² values (the results are not linear) and are therefore, not very good models to predict future outcomes. [8] However, t-statistic measurements are a good measure of statistical significance and are also computed during linear regression calculations. T-statistic results with an absolute value greater than 2 designate strong statistical significance between variables (~95% probability). A Spearman correlation study is conducted on those parameters that show high statistical significance. Spearman results can be broken down as follows: +/- 0.5 to +/-1 for strong correlation, +/- 0.3 to+/- 0.5 for moderate correlation, +/- 0.1 to +/-0.3 for weak correlation, and 0 to +/-0.1 for no correlation. [9]
Each question in the survey, e.g., “What is your age?”, “What is your sex?”, “Are you experiencing pins and needles?”, “How well yoga works for you?”, etc., is a variable or parameter (terms used interchangeably in this paper).
When modeling variables using a linear regression model, there are two sets of variables - x and y. In the data result array (on the “Statistical Significance 500+” tab in the excel file) the horizontal axis is for y variables and the vertical axis is for x variables (this is reversed from conventional algebra, but it facilitated getting the data into the table using this reversed format, in this case). Only one variable is allowed for y in a linear regression analysis, but multiple variables can be used for x (as long as there are more equations than unknowns). The data was computed by running each variable (y) against all other 55 variables (x). Hence, the “Statistical Significant 500+” tab in the excel file is a matrix of t-statistic results that is 55 long by 55 wide. The correlation results tab in the excel file has the Spearman correlation results for any t-statistic values greater than the absolute value of 2.
The data on the “BFS” tab was used to model all results except for Remedies. When Remedy parameters were the y variable the excel file tab “BFS No Zero” data was used to model the results. It isn’t necessary to find statistical significance for remedies that people have not tried (a “0” response means people did not try the remedy). Hence, the data within the “BFS No Zero” tab is the same as the data on the “BFS” tab except “0” responses to Remedy questions were omitted from the data. The model results of Remedy parameters using the “BFS No Zero” tab will result in fewer data points (smaller sample size, n) in the model. For this reason, the results from these models, including t-statistic results, may prove to be less conclusive because the data size is in some cases significantly smaller. Hence, when evaluating the data models for Remedy correlation, sample size should be noted. When Remedies are grouped together as the x variables, the data on the “BFS” tab was used to run the models. Only a few people have tried all potential remedies, hence the sample size would only be a single digit number if the “BFS No Zero” tab data was used to model Remedy results as the x variable. Also, Potassium Channel drugs and Acupuncture remedies were removed from the survey data because they had very few responses.
Appendix V
Methods: Sample Size
What is the correct sample size for this survey study? First, we need to determine (estimate) how many people suffer from severe and chronic BFS symptoms (Population Size). Symptoms must be bad enough for a patient to see a neurologist to be officially diagnosed with BFS after possibly having an EMG and or brain MRI performed to rule out ALS and MS. According to the Center for Disease Control about 1 in 10,000 people in the U.S. have ALS and about 1 in 600 people suffer from Parkinson’s disease. At these rates, it means as many as 700,000 people around the globe can have ALS and 12 million people can have Parkinson’s disease. If the rate of chronic BFS is comparable to the rate of ALS and even Parkinson’s disease, the sample size of the survey would need to be at least 384 people to tolerate a 5% error and a 95% confidence level. There are dozens of online calculators available to compute and verify these calculations. Hence, our current sample size of 438 meets this criteria.
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I developed BFS, also know as Peripheral Nerve Hyperexciteability, Neuromyotonia, etc., when I was 37. I am 43 today. I was diagnosed by and large by Dr. Albert Tamoush in NJ. He's published on the condition so I was fortunate having him as a doctor.
ReplyDeleteBefore you get properly diagnosed going from doctor to doctor is exasperating since you get a lot of shrugged shoulders and are told it's due to 'your own anxiety'. This is confusing and upsetting because you know you're sick, but can be treated in a very patronzing way. It's also frightening since these symptoms can be due to conditions that are much more debilitating over time. Anyway, when you find a knowledgeable doctor who knows how to treat your condition it's a blessing.
I've been on Lamictal for these many years and it controls my symptoms greatly. Not to say they are gone or cured, but I feel more like a normal person. No internal vibrations, twitching, cramping, burning sensations, pinprick pains, and so on and so forth. An EKG reveals the continual activity of the nerves throughout your body, ones you don't even feel yourself. It's just a constant condition.
We are lucky in that it will not get worse and their is no real physical damage that progresses, but that doesn't make it easier to live with. Especially if you're one of the poor souls who can't even get treated for lack of health insurance or a doctor who has no idea what you have and neither do you.
From what I understand it is caused via an antibody created that inhibits the closing of the potassium and sodium ion channel which results in the constant signals being sent out to your nerves thus not allowing to muscles to rest. This can create painful cramping and a lot of fatigue. For every single person it is different. That's my simpleton layman explanation anyhow.
Anyway, I just thought I'd join in and share my story since we are a rather small group of indivituals contending with this condition. And it's true that it is life changing for us, I will never be able to do what I used to do and can't handle any stress, but others don't understand that. Overnight my body was not my own anymore and I had no control over what did what and when. It's upsetting, but with treatment over time it gets easier. It's reassuring to at least know what it is when so many people struggle for so many years trying to at least get diagnosed.
Angela, thanking for sharing your story. It will without question help others deal with PNH and or BFS. It is definitely a Potassium channel imbalance of some kind. You are one of the few people who seems to have found sort of remedy work for them. Most people have tried many things with little help. I am currently using carbamazepine - it helps a little. But a little is better than nothing.
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