Depression on a rainy day

Depression on a rainy day is no fun.  Depression itself is no fun to live with.  Depression on a holiday where we honor the men and women who paid the ultimate sacrifice for our country is not easy either.  Reading/hearing all the stories of the fallen heroes is hard when you are not having a good mental day.  I got teary eyed (as usual) as the XM radio’s “The Highway” played “Taps” to honor those killed in action.  This weekend has been hard one for me and I don’t really know why.   Continue reading

CF Awareness Month

1938 Dorothy Andersen, M.D., writes the first comprehensive medical report on cystic fibrosis.
1953 During a heat wave in New York City, Paul di Sant’Agnese, M.D., and others connect the extra loss of salt by people with CF to the disease’s underlying cellular problem.
1955 The Cystic Fibrosis Foundation becomes incorporated as the National CF Research Foundation and awards the first research grants to Dorothy Andersen, M.D., Paul di Sant’Agnese, M.D., and Harry Shwachman, M.D.
1961 The Foundation establishes the accredited care center network by creating two centers devoted to treating CF.
1962 The CF predicted median survival age reaches 10 years.
1962 A total of 30 Foundation-accredited care centers are in operation.
1964 To investigate CF at the cellular level and find answers about this complex disease, the first basic science committee is established.
1966 Patient Registry launched to collect health information of patients seen at Foundation-accredited care centers.
1978 The number of Foundation-accredited care centers totals more than 100.
1982 The Research Development Program, a network of research centers at leading universities and medical schools nationwide, is established.
1989 A team of Foundation-supported scientists discovers the defective CF gene and its protein product (CFTR), opening the door to understanding the disease at its most basic level.
1990 CF researchers achieve “proof of concept” that gene therapy (in the laboratory) is possible.
1993 The U.S. Food and Drug Administration (FDA) approves dornase alfa (Pulmozyme®), which is proven to thin the thick mucus in the lungs and is the first drug developed specifically for CF.
1997 The Foundation establishes the Therapeutics Development Program.
1997 The FDA approves inhaled tobramycin (TOBI®), the first aerosolized antibiotic designed for CF, which is proven to reduce hospital stays and improve lung function.
1998 Specialized clinical research centers are designated as the Foundation’s Therapeutics Development Network.
2000 Cystic Fibrosis Foundation Therapeutics Inc. (CFFT), a nonprofit research affiliate of the Foundation, is established to govern drug discovery and development efforts.
2000 Foundation-supported scientists map the entire genetic structure of the most common cause of CF lung infections, the bacteria Pseudomonas aeruginosa.
2002 A CFFT-supported study shows the antibiotic azithromycin improves CF lung health.
2003 CFFT-supported scientists at Structural GenomiX Inc., determine the three-dimensional structure of a portion of the CFTR protein, opening the door to more drug discovery opportunities.
2004 CFFT-supported studies in Australia and at the University of North Carolina show that inhaled hypertonic saline helps clear CF mucus and improve lung health. It becomes a therapeutic option.
2006 Ivacaftor (formerly VX-770), an oral drug in development that targets the faulty CFTR protein, enters clinical trials. Ivacaftor is designed to open chloride channels that do not function correctly in people with the disease.
2008 Phase 2 studies of ivacaftor in people with the G551D mutation of CF show unprecedented improvements in key signs of the disease. The studies achieve “proof of concept” that it is possible to treat the root cause of CF.
2010 The FDA approves a new antibiotic, aztreonam for inhalation solution (Cayston®), to treat CF lung infections. The drug offers an alternative for people with CF who battle recurrent infections and develop resistance to existing antibiotics.
2012 The FDA approves ivacaftor (Kalydeco®) for people with the G551D mutation of CF ages 6 and older. The drug is the first to address the underlying cause of CF and opens exciting new doors to research and development that may lead to a cure for all people living with the disease.
2014 The FDA approves ivacaftor as a single therapy to treat people ages 6 and older with one of nine other rare CF mutations in addition to G551D, and later extends approval to children ages 2 to 5 with any of these 10 mutations — representing about 8 percent of the U.S. CF population.
2015 The FDA approves the lumacaftor/ivacaftor combination drug (Orkambi®) for people with CF ages 12 and older who have two copies of the most common CF mutation, F508del — representing about a third of those with CF in the United States.
2016 The FDA approves lumacaftor/ivacaftor (Orkambi®) for children with CF ages 6 to 11 who have two copies of the F508del mutation. The decision means that about 2,400 additional children in the U.S. are eligible to receive the drug, bringing the total number of those eligible for the treatment in the U.S. to nearly 11,000.
2017 Two Phase 3 clinical trials of tezacaftor (VX-661) in combination with ivacaftor (Kalydeco®) demonstrate positive results not only for people with two copies of the F508del mutation, but also for those who have one F508del mutation and a second mutation that results in residual function.
2017 The FDA expands the use of ivacaftor (Kalydeco®) to people ages 2 and older who have at least one of 23 residual function mutations in the CFTR gene. The FDA’s consideration of laboratory evidence coupled with clinical data to address the needs of people with CF who have less common mutations is an important step forward for the CF community.
2018 The FDA approves tezacaftor/ivacaftor (Symdeko™) for individuals with two copies of the most common cystic fibrosis mutation, F508del, as well as for individuals who have a single copy of one of 26 specified mutations — regardless of their other mutation. This approval paves the way for new, more effective triple combination therapies (treatments consisting of three different modulators, including tezacaftor), which are being tested in clinical trials.
2018 The Foundation maintains a robust pipeline of potential therapies that target the disease from every angle. The more drugs in the pipeline, the greater the odds of producing successful therapies and a cure for all people with CF.

Happy CF awareness to me

I have not been feeling good for about 3 or so week, maybe more.  I did the 2 weeks of IVs 2 weeks ago and wasn’t much better but that is probably due to the fact that my pseudomonas is multi drug resistant.  We are limited on what will “work” on the stubborn drug resistant bug (pseudomonas) in my lungs.  I made the dreaded call after the 4 days of 40mg of prednisone and no improvement.  I need something more therefore I had to call with all the I have coming up.  I have a busy 5 weeks ahead.  Next weekend we are going to the Zac Brown Band Concert, a bucket list item for myself.  Then I have a girls trip to the Gulf Shores the next week.  Then in 5 weeks we have our good friends wedding weekend. Continue reading

CF Facts

Cystic fibrosis (CF) is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:

  • clogs the lungs and leads to life-threatening lung infections; and
  • Obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.

Continue reading

CF Awareness Month begins

May is Cystic Fibrosis Awareness month.  This is the month many CF Foundations do their Great Strides fundraising walks.  I am very aware of CF every day obviously.  Now is the time to educate others.  If you have questions about CF or what I have to do every day feel free to comment on this post and I will answer them. Continue reading

Waiting…

When you have a chronic illness waiting is part of life.  Waiting to get better, waiting/holding steady or waiting to die.  Yep, I said it.  It’s a part of my reality and when I have friends waiting for lungs and others who are not candidates for transplant or don’t want to go that route who are basically waiting to die.  Maybe you could call it “living to die.”  Whatever it may be, it is life. Continue reading

CF Clinic/Follow up from the hospital

Today’s clinic visit was a follow up from my last admission.  Ya know, the one where I kept coughing up blood, yeah that one.  The visit was short and “sweet” because I had so many other things I had to get done today so I put them on a deadline of when I needed to be out of there.  hahaha   Continue reading