(United States) U.S.A.C.F.A.

“This is a global observance,” said Peter L. Saltonstall, president and CEO of NORD.  “Individuals and organizations around the world will all be sharing stories of how rare diseases affect their lives.”

The challenges of living with a rare disease, he said, include:

• Difficulty getting a timely, accurate diagnosis
• Too little research
• Too few treatments
• Reimbursement or other issues affecting access to treatments
• A sense of isolation
• Difficulty finding medical experts

In the U.S., any disease affecting fewer than 200,000 Americans is considered rare.  According to the National Institutes of Health (NIH), there are nearly 7,000 such diseases affecting nearly 30 million Americans.

Studies have shown that it often takes five years or longer to get an accurate diagnosis of a rare disease.  In addition, only about 200 of the diseases classified as rare have approved treatments.

Rare Disease Day started in Europe in 2008.  It was launched by EURORDIS (Rare Diseases Europe).  Last year, it was observed in more than 60 countries, with a national sponsor in each country.  NORD is the sponsor in the U.S.

More than 500 patient organizations, government entities, research institutions, and companies developing treatments have signed up as Rare Disease Day Partners on the national website hosted by NORD (www.rarediseaseday.us).  USACFA is one of those partners.

Incorporated in 1990, USACFA is an independent 501(c)(3) non-profit, tax-exempt organization which is operated entirely by adult volunteers who have cystic fibrosis (CF). USACFA is not affiliated with other CF organizations. The purpose of USACFA is to provide a source of information for CF adults regarding the basis, nature and progression of the disease, as well as the latest treatments and research to fight it.  It also offers a forum for CF adults to communicate with each other.

Each year, a global planning team selects a theme for Rare Disease Day.  The theme for 2012 is “Rare but strong together.”

Rare Disease Day activities in the U.S. will include a “Handprints Across America” campaign to create a gallery of photos on the Rare Disease Day website; educational materials for classroom teachers; and a nationwide blitz of patient photos, stories and videos to increase awareness of specific rare diseases and the challenges of living with a rare disease.

Several special events are planned, including a scientific symposium at the National Institutes of Health (NIH) and a Rare Disease Patient Advocacy Day at the Food and Drug Administration (FDA).

“Since many of these diseases are genetic, more than half of the people who have rare diseases are children” Saltonstall said.  “The problems encountered by families are enormous.  It’s important for these families to know they are not alone.”

NORD was established in 1983.  It provides advocacy, education, research and patient services on behalf of rare disease patients, families, and patient organizations.

I did not have to worry about being assisted at the airport. Overall, Delta did a great job of having my wheelchair ready when I needed it and they did everything possible to make going through security easy for me. The flight attendants were both helpful and my seat mates were nice. Despite having a little bit of turbulence I remained calm and relaxed on both flights.

I did really well managing my energy in the days leading up to my trip. I did all my treatments, got lots of rest and I maintained my workout regimen. I even worked out on the day I was leaving and then when I got there my friend and I went to the YMCA two of the three days. I probably should have worked out one more day. A girl’s gotta have some fun though!

On my last night there I some very mild (that term is relative!) hemoptysis and then the last day I felt more short of breath than normal. That continued for the whole week I was home and when I started to feel a little feverish I thought I may even need IV’s. Luckily the fevers didn’t stay and my shortness of breath is getting back to my baseline. However, I do see the reality of how long it has taken me to recover from the trip. It has been 9 days and I would say I am just now back to my baseline. That has been difficult. I know I have limitations. I deal with it everyday. But I secretly hoped I could push my limits without any repercussions.

I know I could still do it again if I needed to. I also know that I travelled when I was pretty healthy by my standards. I did all the right things. And still it was hard on my body. I have to respect that and embrace it for now. I can still travel. It is just easier to do it by car, not too far from home.

All that being said I do not regret going AT ALL. Not one bit. I had such a great time with my best friend, hanging out and laughing and feeding my soul which is just as important as doing my treatments and exercising.

Me and my best friend Pam. It was all worth it to laugh with her in person!

So my advice is, every once in a while, plan as much as you can but then take that small (or big!) step out of your comfort zone. You never know what you might be missing out on!

While the income limit under the SSA statute can be complicated by the types of income an individual or family is receiving, the resource limits are standardized: an individual is allowed a maximum total of $2000 in resources/ assets and a couple is allowed $3000.  A family which has more than 2 people will have a resource/asset limit of $3000.

One countable resource that surprises many potential or current beneficiaries is the ownership of a vehicle. Under the SSI rules, an individual or family is allowed to own one car regardless of its value. If the individual or family owns a second car, it will be counted as an asset and its value will count toward a beneficiary’s resource limit. There are only a few exceptions to this rule.  Therefore, potential applicants must be sure they meet the car ownership rules when determining if their assets meet the SSI requirements. A typical problem arises if a family’s second car is valued at more than $3000. Once the car is valued at more than $3000 the family resource and asset amount has exceeded the SSI limit and the individual who was applying for SSI or who was receiving SSI benefits is no longer eligible for SSI.

The Social Security defines an “automobile” as
“any registered or unregistered vehicle used for transportation.” Even a temporarily broken down car that is normally used for transportation will meet the SSA definition of an automobile.  It is important to understand how Social Security will determine the value of your vehicle.

If a person or family owns 2 cars a determination as to the value of the car should be made before filing an application for SSI.  The Social Security Administration uses the N.A.D.A Guide to determine vehicle value.  To read more about the SSA rules and for information on how SSA values second cars go to the SSA Digital Library Electronic Resources website at secure.ssa.gov/poms.nsf/lnx/0501130200.

More information on how to appeal the SSA evaluation of your second car will be discussed in the next issue of the CF Roundtable. If you have questions about Social Security benefits please call the CF Legal Information Hotline at 1-800-622-0385. The Hotline is sponsored by a grant from the CF Foundation. All calls are free and confidential.

People with CF and other medical conditions may call the “TSA Cares” toll-free number at 1-855-787-2227. Travelers are encouraged to call at least 72 hours before their flight. The helpline is available Monday through Friday 9am – 9pm (Eastern Standard Time) and is closed on all federal holidays. Individuals may also find information on traveling with special medical needs on the TSA website at: http://www.tsa.gov/travelers/airtravel/disabilityandmedicalneeds/tsa_cares.shtm

Traveling alone for me at this point is scary. I won’t have anyone with me who knows all my health history. Once I get to my destination, visiting my BFF, I will feel very comfortable. It is just the airport and flying time I am struggling with. I have realized though that I have to face this fear. Because the benefits definitely outweigh my fears.

It has been easy for me to simply say I can’t do some things now because of my health. I know I am missing out on some great things. There are things I do not do, not because I can’t per se, but because the effort to either get ready for it or the recovery time afterwards make it not worth it. I am always having to pace myself and my energy on a daily basis so I never get too run down and increase my chance for an exacerbation.

The more practical issue is that traveling is quite exhausting for me whether I have someone with me or not so I have to plan many things out ahead of time. I have had to schedule travel oxygen, fill out paperwork, get medical information to take with me in case of an emergency, etc. I will need airport personnel to push me in a wheelchair and carry my luggage. I have a checklist a mile long.

I am ready to do it though.

Here are some words that I will keep repeating to myself.

Source

Source

A little fear is ok. The joy I will have during my visit will be worth the 4-5 hours of total travel time. I will report back on how it went in a couple of weeks!

Happy Travels….

Unlike SSI, SSDI is not an income based program. A person can have a large amount of money in his savings account and still receive SSDI. Most individuals who stop work and are approved to receive SSDI benefits have stopped work because their health has declined and they need to spend more time taking care of themselves. A small number of individuals work part time for additional income. SSDI allows a person to work part time as long as their income from part time work is less than $1000 a month. A person on SSDI can work and make more than $1000 for only 9 months. The months are called a 9 month trial work period. A person cannot use his 9 month trial work period while his application is pending. If an application for SSDI benefits I pending the person can only make under $1000 from part time work. Once a person begins receiving SSDI benefits he should be sure he understands the risk of using his 9 month trial work period. Be warned, if a person makes more than $1000 a month even if it is part of the 9 month trial work period there is a strong chance the person will lose his benefits because SSA will determine that if he can work making more than $1000a month he is able to work full time and therefore is ineligible for SSDI benefits. SSA is being much more careful when reviewing people who have used their 9 month trial work period or a portion of their 9 month trial work period. So act with caution. As always if you have questions please contact the CF Legal Information Hotline at 1-800-622-0385 or cflegal@cff.org.

Our live free patient webinars are designed to help educate and empower people who suffer from CF and other lung diseases.  On January 19^th PM EST, we will be conducting a webinar entitled “Latest Trends in Spirometry”.  All are welcome to attend!  Here is the link to register to our webinars.  http://spiropd.com/get-more-information/

We are dedicated to creating innovative, easy-to-use, portable, and affordable personal medical devices, and to empowering people worldwide to improve their healthcare and quality of life.  Here is a link to our website where we feature Spiro PD, the world’s first truly personal spirometer. www.spiropd.com

I am not sure why I accept the death so readily when it happens. Of course, I am sad. Mainly, I feel for that person’s family and I know I will miss them and their friendship. It is possible that when I was terribly sick while waiting for a second chance with a transplant, I came to accept that I might die soon and may not make it to receive a transplant. There were days I just felt I could not keep up the rigorous schedule of inhalations, CPT, IV antibiotics, using the vest, the flutter, and the constant coughing–all for hours. Plus the constant hemoptosis. Never feeling any better and sometimes worse. Trying to navigate the razor’s edge of being healthy enough for a transplant and not becoming too sick to be disqualified. Being afraid of the common cold as it could be my last. I think I came to accept that my life was what it was and I was only pushing through for the transplant, if it ever came, for my family and especially my mom. Who, I felt horrible about leaving alone.

When I finally accepted I would die, as we all eventually do, some sooner than others,  and it was only after therapy and crying a lot about it did this occur. For me the fear of dying was more intense because I didn’t want to think about it or readily accept it. Once I started to contemplate it all, and process my fear ad anger, I gained peace and my fear was gone.

So, I guess when CF becomes so unbearable and someone is suffering, I have felt in the past that maybe it is just too much for them to keep trying. The daily suffering to great to maintain this state of living. Usually it is only because they have tried everything and nothing was working or they were not a suitable candidate for a transplant. But, recently, a good friend of mine died from CF and it was a total shock. She fought the good fight every day with every breath.

She had always bounced back. It got so that I had to tell myself to stop worrying, she always pulled through her hospitalizations, surprising her doctors. It was when I stopped worrying whether she would make it, that she surprised me, and everyone else, and passed away in her sleep at home. Although shocking, everyone who knew her felt this was the best way to go. Not being kept alive in a hospital setting, she was able to enjoy every last minute of her life with her family.

But I feel she didn’t live long enough and I miss our shared bond. Some people impact you r life more than others and I just wanted to always her to be a part of my life. I feel for her husband, daughters, parents and brother. It made me realize that even when we know someone is sick with CF, we believe they will be ok because we have to. We don’t want to miss them. One thing that gives me peace was that my friend was very religious and believed once she died, she would be reunited with her sister and her family who died before her. She told people that for the person who dies, it is not sad or scary but peaceful and that their spirit rises. But she knew it was harder for those left behind. I am hopeful that there is no more suffering for her and that she is smiling her beautiful smile and laughing with her sister, running free without a cough audible.

Titled, YELLOW CAR I WIN! A story of Beans and Roses. Is about Christine “Beans” Padasak lived with Cystic Fibrosis until her 22^nd year. For all 22 of those years, she was an incredible inspiration to all who knew and loved her. This book is not only in honor of her, it was inspired by her and is being co-authored posthumously by her.

Reason for doing this: To create something that people will read and find inspiration, hope, something to ponder and think about, something to relate to. To raise funds for CF research.

The plan is to secure sponsorship for the printing and marketing of the book. This will enable 100% of the proceeds to go directly to Cystic Fibrosis.

Authors are Rick Manzone and Christine Padasak with submissions by others. Approximately 50-60 pages.

100% of the suggested donation for the book ($10) will be given to the Cystic Fibrosis foundation. Contact Info: Rick Manzone, Rickmanzone56@gmail.com 716-725-2990

Original works may be submitted by email to: rickmanzone56@gmail.com or by U.S.P.S. mail to:

Rick Manzone
11507 Snyder Road
Springville, NY 14141