Here are some pictures from the event:
On November 1, 2010, our daughter, Ellis Rose, was diagnosed with Cystic Fibrosis at 7 days old. Our family is now committed to finding a cure for this illness that 70,000 people around the world suffer from. The first words our doctor said to us during our first visit to the CF Clinic were "We are very hopeful for Ellis". Hope is a word we hear a lot in the CF community, and it is the best way to sum up how we feel as we continue our fight against CF.
Thursday, March 31, 2011
Great Strides 2011
On March 20th, Team Ellis Rose gathered at the U of A Mall in our team shirts and Ellis Island head gear to walk the 5k and celebrate our fundraising efforts for the Cystic Fibrosis Foundation. Our team raised $11,414.00 and still counting!!!
Here are some pictures from the event:
Here are some pictures from the event:
Monday, March 28, 2011
Why All The Fuss Over VX-770?!
The CF community received more great news today. Vertex has been working on several drugs that target the defective proteins that cause cystic fibrosis. An oral medicine called VX-770, geared towards the G551D mutation (which is one of Ellis' mutations!), has been in the trial phase, and Vertex released some of the amazing results. The VX-770 opens the gateway of the defective protein to allow the chloride to pass through the cell and prevent the build-up of mucus in the lungs, among other things.
In the study, 52 children (ages 6-11), with at least one copy of the G551D CFTR mutation, received VX-770 or the placebo as a single 150 mg tablet every 12 hours.
Preliminary Results
Lung Function: Results of the study showed that the difference in mean absolute improvement from baseline in lung function through 24 weeks in children treated with VX-770 was 12.5 percent. The difference in mean relative improvement from baseline was 17.4 percent compared to placebo.
Weight: Many people with CF have a hard time gaining and maintaining weight due to factors such as reduced pulmonary function, nutrition, chronic infection and inflammation. Those who received VX-770 experienced an average weight gain of approximately 8.1 lbs through 24 weeks compared to baseline while those in the placebo group gained approximately 3.9 lbs compared to baseline.
Sweat Chloride: People with CF typically have sweat chloride levels in excess of 60 mmol/L, while normal values are less than 40 mmol/L. Reduction in sweat chloride is considered to be a marker of improved CFTR function. In the study, the baseline sweat chloride level for both treatment groups was approximately 104 mmol/L (Ellis' sweat chloride levels are at 100). Through week 24, the mean sweat chloride level for individuals treated with VX-770 was below 60 mmol/L. Significant decreases in sweat chloride were not observed among those in the placebo group.
We expect to see more results in mid-July. I believe this drug will made available when Ellis is turning two years old. Keep up the good work Vertex!!!
What is Cystic Fibrosis?
According to the Cystic Fibrosis Foundation, cystic fibrosis 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.
In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.
People with CF can have a variety of symptoms, including:
very salty-tasting skin;
persistent coughing, at times with phlegm;
frequent lung infections;
wheezing or shortness of breath;
poor growth/weight gain in spite of a good appetite; and
frequent greasy, bulky stools or difficulty in bowel movements.
Those with CF are now able to identify which gene mutations they inherited. Ellis has the most common CF gene, deltaF508, and a less common gene found in only 4% of CF people, G551D. These gene mutations are ranked on a scale from 1-5, 1 being the most severe and 5 being the least. The deltaF508 is a level 2 mutation and G551D is a level 3 mutation.
Ellis takes enzymes every time she eats to help her absorb nutrients, since her pancreas is insufficient. We also give her 1/4 tsp. of salt each day since her body releases more salt than it should. Ellis also gets a multivitamin supplement each day. To prevent the build-up of mucus in her lungs, Ellis gets her chest percussions done twice a day for about 20 minutes each time to "pound" her lungs clear. The mucus can hold on to infectious bacterias, so it is important to do a lot of hand washing to stop the spread of germs and ensure that Ellis does not get sick. When those with CF do get sick, they stay sick for a longer period. If certain bacterias are detected in the lungs, it can lead to the need for hospitalization and intense treatments to take care of the problem.
So, these are the basics of CF. Each case is different, but I thought I would share a little bit about what our day to day dealings are with this illness and how we are proactive in preventing the progress of CF. Feel free to ask any questions, and I will do my best to answer them.
In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.
People with CF can have a variety of symptoms, including:
very salty-tasting skin;
persistent coughing, at times with phlegm;
frequent lung infections;
wheezing or shortness of breath;
poor growth/weight gain in spite of a good appetite; and
frequent greasy, bulky stools or difficulty in bowel movements.
Those with CF are now able to identify which gene mutations they inherited. Ellis has the most common CF gene, deltaF508, and a less common gene found in only 4% of CF people, G551D. These gene mutations are ranked on a scale from 1-5, 1 being the most severe and 5 being the least. The deltaF508 is a level 2 mutation and G551D is a level 3 mutation.
Ellis takes enzymes every time she eats to help her absorb nutrients, since her pancreas is insufficient. We also give her 1/4 tsp. of salt each day since her body releases more salt than it should. Ellis also gets a multivitamin supplement each day. To prevent the build-up of mucus in her lungs, Ellis gets her chest percussions done twice a day for about 20 minutes each time to "pound" her lungs clear. The mucus can hold on to infectious bacterias, so it is important to do a lot of hand washing to stop the spread of germs and ensure that Ellis does not get sick. When those with CF do get sick, they stay sick for a longer period. If certain bacterias are detected in the lungs, it can lead to the need for hospitalization and intense treatments to take care of the problem.
So, these are the basics of CF. Each case is different, but I thought I would share a little bit about what our day to day dealings are with this illness and how we are proactive in preventing the progress of CF. Feel free to ask any questions, and I will do my best to answer them.
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