The need for Support

I say the most important thing someone with Cystic Fibrosis needs is the support of Family and Friends. Not only when times are good, but most of all when things are tough. Thank you to all of you who have stood by us and Sarah through the painful road to the diagnosis and beyond. Thanks to those who have given support even through the tough treatments. All of your thoughts and prayers have meant the world to me. 

Information

Life span

CF was once always fatal in childhood. Better treatment methods developed over the past 20 years have increased the average lifespan of CF patients to mid-late 30's.

How do you get CF

CF is genetic, and that means people with CF are born with it. Even if parents do not have CF, their child might have it. That’s because people can carry the CF gene and pass it on, but do not necessarily have the disease themselves. When both parents carry the gene, their child has a 25% chance of having CF.

Sunday Factoid

More than 10 million Americans are carriers of 1 mutation of the CF gene.  About 1 in 29 Caucasians, 1 in 46 Hispanics Americans, 1 in 65 African Americans, and 1 in 90 Asian Americans carry a CF mutation.

Saturday Factoid

People living with cystic fibrosis must follow a regular treatment routine to stay healthy and maintain optimal lung function.

65 roses

"65 Roses" is what some children with cystic fibrosis (CF) call their disease because the words are much easier for them to pronounce. Mary G. Weiss became a volunteer for the Cystic Fibrosis Foundation in 1965 after learning that her three little boys had CF. Her duty was to call every civic club, social and service organization seeking financial support for CF research. Mary's 4-year-old son, Richard, listened closely to his mother as she made each call. After several calls, Richard came into the room and told his Mom, "I know what you are working for." Mary was dumbstruck because Richard did not know what she was doing, nor did he know that he had cystic fibrosis. With some trepidation, Mary asked, "What am I working for, Richard?" He answered, "You are working for 65 Roses." Mary was speechless. He could not see the tears running down Mary's cheeks as she stammered, "Yes Richard, I'm working for 65 Roses." Since 1965, the term "65 Roses" has been used by children of all ages to describe their disease. But, making it easier to say does not make CF any easier to live with. The "65 Roses" story has captured the hearts and emotions of all who have heard it. The rose, appropriately the ancient symbol of love, has become a symbol of the Cystic Fibrosis Foundation. 65 Roses® is a registered trademark of the Cystic Fibrosis Foundation.

Thursday factoid- the CF diet

CF Diet – In CF, a high-calorie, high-fat diet is vital for normal growth and development in children, and offers adults a way to maintain optimal health. The dietitians at CF Foundation-accredited care centers work with patients and their families to map out the best diet for each person. Nutrition and general lung health are closely linked. People with cystic fibrosis may need extra calories to compensate for the malabsorption of nutrients. These extra calories also help to meet the greater energy needed for breathing. In fact, for children with CF, extra fat calories are good for fueling normal growth and development. 

Wednesday Factoid

There are Over 1800 different CF mutations have been discovered so far.

Tuesday factoid

The Cystic Fibrosis Foundation supports and accredits 115 care centers nationwide where people with CF can receive expert care and access resources. Most care centers have a pediatric and adult program or clinic. Some centers also include affiliate programs. All programs, however, are staffed by dedicated healthcare professionals who specialize in the treatment of cystic fibrosis.

Monday Factoid

There is no cure for CF; however, new drugs such as Kalydeco are the closest things to a cure that the CF community has seen to date.

Saturday Factoid

People with CF should practice the 4 foot rule to minimize the likelihood of transmitting various bacteria and other germs to each other.

Friday factoid

The Sweat Test
Overview
The sweat test has been the “gold standard” for diagnosing cystic fibrosis (CF) for more than 50 years. It is recommended that the sweat test be performed at a Cystic Fibrosis Foundation-accredited care center where strict guidelines are followed and monitored by the center director to make sure the results are accurate. The sweat test can be done on people of any age. However, some infants may not make enough sweat to do the test. If an infant does not produce enough sweat the first time, the test should be repeated.  

What happens during a sweat test?
The sweat test measures the amount of chloride in the sweat. There are no needles involved in this test. The test takes about an hour, but it may take longer.  

What is a positive result?
For infants up to and including 6 months of age, a chloride level of:
Equal to or less than 29 mmol/L = CF is very unlikely
30 - 59 mmol/L = intermediate means that CF is possible
Greater than or equal to 60 mmol/L =

CF is likely to be diagnosed For people older than 6 months of age, a chloride level of:
Equal to or less than 39 mmol/L = CF is very unlikely
40 - 59 mmol/L = intermediate means that CF is possible
Greater than or equal to 60 mmol/L = CF is likely to be diagnosed

Sarah was 32 on one arm/not enough sweat on the other arm for her first test and she was 32/29 on her second test. It was decided then to do further testing and it was her blood work that came back positive.

Thursday Factoid

CF patients have daily airway clearance therapy to help move the thick, sticky mucous out of their lungs. This can be done manually, either by cupping the hands or with the aid of a percussor cup, or a machine such as The Vest can be used.

 Sarah recieves airway clearance twice a day for 30mins as normal care. When she gets sick we do it 3-4 times a day. Right now we use percussor cups and when she is big enough she will receive a vest to do it for her.

Another Factoid

CF doesn't only affect the respiratory system.  It also affects the digestive system - the abnormally thick mucus a CF patient's body produces clogs the pancreas and disrupts the flow of natural enzymes which help break down and absorb food.  Artificial enzymes help replace those that the body fails to make.

This is Sarah's biggest problem. She takes enzymes before she eats anything or drinks her bottles. Because her body doesn't know to absorb food with out the enzymes she has trouble putting on weight. She is quite a bit smaller than her sisters were at this age. She is now 10months old and at her last weigh in at 9.5 mths old she wasn't yet 16lbs. 

CF Factiod

CF patients have daily airway clearance therapy to help move the thick, sticky mucous out of their lungs.  This can be done manually, either by cupping the hands or with the aid of a percussor cup, or a machine such as The Vest can be used.

More than 45% of the CF patient population is age 18 or older.

New fact

The gene responsible for cystic fibrosis was only discovered in 1989

May is Cystic Fibrosis Awareness Month

In honor of May being Cystic Fibrosis awareness month I am going to do my best to post a fact a day. I k ow I have already missed a bunch ,but I will start now. About 30,000 people in the US and about 70,000 people worldwide have cystic fibrosis.