Why Cystic Fibrosis Patients Need More Salt

I have had several people ask me why Bennett requires more salt than the "normal" person and why am I so anal about her sweating too much in the Summer time. I "googled" the question to help me explain it a little better to them and this is what I found. And it explains it perfectly! 




 Salt is the combination of sodium and chloride. The biochemical processes that go on in our bodies are such that only a very narrow range of salinity (concentration of salt in our body’s fluids) is acceptable. Too little or too much is literally a matter of life and death. Fortunately, our bodies are really good at ensuring that it contains the ideal concentration of salt by adjusting the volume of fluid in our blood and tissue so that the salinity is always in the ideal range. If you have too much salt you become thirsty, causing you to drink water, which dilutes the too-high concentration of salt in your body. If you have too little, you usually crave and eat something salty.

In cystic fibrosis, improper transport of chloride (a component of salt) causes all the horrible symptoms and manifestations of this disease. CF bodies don’t fully regulate the concentration of salt within the cells that line our airways, digestive tract, and skin, causing the concentrations to be a bit out of whack—either too low or too high, depending on which side of the cell you’re looking at. In the case of the cells that create sweat in a CF patient’s sweat glands, they leave too much chloride inside the “sweat reservoir,” which must in turn be balanced with an equivalent amount of sodium (wherever chloride goes, sodium follows), making the super-salty sweat characteristic of cystic fibrosis.

Every time a CF person sweats, their body loses up to 10 times the amount of salt a normal healthy person would in the same amount of sweat. That means excessive sweating (as might happen in the summer or when exercising) puts people with CF at a much higher risk of dehydration than other people. Because of this salt loss, people with CF must be very diligent to replace lost salt whenever they sweat, otherwise there is a risk of suffering from dehydration.

Generally speaking, people with CF need to include salt as a regular part of their diet to compensate for abnormal amounts of salt lost through sweat. The usual reason doctors recommend a low-salt diet for non-CF people is so that they don’t develop high blood pressure, which causes your body to retain more fluid in your blood to compensate for the excessively high concentration. If you have CF and have high blood pressure, obviously the amount of salt in your diet is something you need to discuss with your doctor.

Bottom line: it’s important for people with CF to add a little extra salt into their diet in order to avoid dehydration in warm weather and when exercising. Also, always make sure you consume something salty with plenty of fluids so your body can process it properly.

March 25, 2001

March 24, 2001 was just any other day. I was 17 years old. A senior in highschool. We just finished celebrating my Mom's birthday as family. I went upstairs and got all snazzied up. Ask my parents to borrow the car since they were going out to celebrate my Moms birthday at their favorite place to spend time with their friends. Of course, being Daddy's little girl and all he said yes that I could use the car. I waited for my bestfriend to get to my house so we could leave. We went uptown where it was "cool" to hangout in the lower parking lot of Danks. 




We were hanging out and a little after 11pm there were sounds of the sirens of an ambulance blaring down Third Street. My heart dropped into my feet. And this really weird feeling came over me and I told my friends that I would be back in a few minutes. I hopped into the car and drove to where my parents were. And as I was pulling into the parking lot, I saw the ambulance. I sat and I waited and waited and waited some more which felt like hours. Then the front door came busting open, the paramedics came running out pushing the gurney. My heart started to race as I saw a paramedic straddling the gurney and seeing that they were giving my Father CPR. I floored it across the parking lot, picked up my Mom and sped to the Emergency Department of the hospital. We beat the ambulance to the hospital. As we were standing in the waiting area, they wheeled my father past us and the same paramedic was once again straddling the gurney and really working hard on my fathers chest. 


A nurse came out to get the family of Richard Wagner and we were taken back into a conference room. We waited for what yet seemed like an eternity for the doctor to come back and speak to us. As he walked into the room and closed the door. We all knew what he was going to tell us by the look on his face. His words were "I am sorry but we were unable to save your husband, he was pronounced dead at 12:31am". 


I instantly felt ill. I couldn't believe that I had just lost my Father. March 25th is a day that I wish would vanish from the calendar. With each passing year it never seems to get any easier. It was the worst day of my life.


I was pissed off. He passed away 2 1/2 months before I was to walk across the stage and receive my highschool diploma. He never get the chance to walk me down the aisle. To enjoy my children. 


I began to spiral out of control once I turned 21. I was drinking every weekend, all weekend until the point of being so drunk that I remembered absolutely nothing the next morning. This went on until I was 23 and I found out I was pregnant. 


March 25, 2006 was the fifth anniversary of my Fathers death. It also is the day that I found out I was pregnant with my daughter Bennett. I believe that my father sent Bennett to me to save me because he was watching me from above and saw the path of destruction that his little girl was going down. He knew that I needed something positive in my life. My daughter is a splitting image of me. We talk alike, look alike, same sassy attitude, girly personality. 


June 2006 is when I found out that Bennett was going to have Cystic Fibrosis, I was 4 months pregnant. This is another thing that my father did to give me the strength I needed to carry on. He knew that his babygirl would be able to handle the test of being a Mommy to a Cystic Fibrosis Warrior Princess. He is our guardian angel. 


I miss my Father very much. I still struggle and cry! My father and I were very close. It may be 11 years that he has left his earth but it hurts the same as it did the day it happened. My daughter was named after him -- Bennett Madalynne-LEE Flenner. She received him middle name. She asks alot of questions about her "Pappy On The Wall". I take her out to visit him at his special box. One day when she is a little older she will understand just how amazing her Pappy On The Wall is. He was the best father a girl could ever ask for! 




Daddy,


I miss you more than anything in this world. Thank you so much for sending me my babygirl. She is the best blessing I have and will ever receive. She is my world. I know you are watching her from above and enjoying her. Especially, when she sasses her Mommy. If you were here on this Earth with us I can only imagine how rotten you would have her. There is not a day that goes by that I do not think of you. Mom and I talk about you often. I am sure you are upset about the huge tattoo that I have in your honor but I love you! I hope you are proud of the woman I have become. I am a Mommy. I have a college education. Richard has grown up to be young man. He is taking care of his Ninny. You would also be so proud of him. He is taking his first responder course. He is a firefighter, runs EMT calls with FAME EMS and is about to go on to college this coming fall. You also have a 2nd grandson named Bradley. He is very ornery and keeps his Ninny on his toes. We all miss and love you! Heaven has an awesome angel up above!


Love your daughter,
Emily






Cause there's holes in the floor of heaven and his tears are pourin down
that's how you know he's watchin, wishing he could be here now.
and sometimes if you're lonely just remember he can see,
there's holes in the floor of heaven, and he's watchin over you and me 

Please Be Our Friend

We get told some pretty scary things about CF from our kids doctors. Lots of it won't affect you. You don't have to worry about how to help our kids avoid diabetes or osteoporosis, you don't have to measure up the pros and cons of sending our kids to swimming lessons, you don't have to make sure that airway clearance is done properly. And we don't expect you to worry about all of those things. That's our responsibility, our privilege.


One of the scary things we are told about, is how a common cold, and other illnesses that are minor to most, can affect our child in the long term. What might be a day off school for your child, could very easily be a hospital admission for ours. A week of illness for your child could equal permanent lung damage for ours. Your child will feel better and be better. Our child may feel better, but all of these "little" illnesses shorten our childs life. That's just how it is.


Again, not your problem. Or is it? Being the mom of a Cystic Fibrosis child can be stressful and sometimes isolating. We don't want to lose friends, we need you more than ever. But we know more now, and our priorities may have changed slightly.


The good news is, it's easy for you to still be a good friend! There are just a few simple rules. These should help you out:

Accept that Cystic Fibrosis is serious.

One of the hardest things about CF is that our kids don't often look or act sick. And we don't really like reminding people. But the fact is, our kids have an incurable, life shortening, chronic illness that needs to be managed every single day. They are not the same as other children. We are not being cotton wool parents, we are keeping our children as healthy as we can for as long as we can.

Learn the basics.

We don't expect you to become an expert on the subject, but knowing the basics of the disease will help you understand why we are so anal about some things! In the smallest nutshell in the world: Cystic Fibrosis is a disease that causes the thickening of secretions in the bodies organs. This means that germs like to breed in the lungs, causing irreversible lung damage and eventually respiratory failure. If you would like to know more...ask! Your head won't get bitten off, I promise. Or you can check out the links at the bottom of the page. And no, they won't grow out of it.

Practice good hygeine.

Our kids don't need to be stuck in an antibacterial bubble. Practicing normal good hygeine will protect our kids. Wash your hands often, cover your mouth when you sneeze or cough. If you want to be a super good friend, let us know that you know that we want you to know! ie "oh give me a cuddle of your bubba! I can't wait to get my freshly washed hands on that gorgeous bundle of joy!"

And of course...

Stay away if you are sick! (or smell like smoke).

This is the most important point. Read the start of the page again. Your minor illness is our major fear. We won't get offended if you cancel a playdate, we won't hate you if you can't come to our dinner party. In fact, we will love you even more if you avoid us when you or your kids aren't well. This is the part where you can help us with managing our child's illness. Please please please let us know if your child has been sick. You don't have to decide if we should stay away or not, we will make the call. But we need to be able to make an informed choice. The smoking part is self explanatory and common sense.

Don't turn us into the bad one.

These rules will never change. Our child will always be sick, you will always need to practice good hygeine, we will always want to know if you or your kids are sick. Please don't make us bring it up all the time. We would really really really appreciate it if you remembered these points. It's no fun telling people over and over to wash their hands or cover their mouths. It's frustrating and sometimes embarrassing, but we will do it if we have to. We hope our friendship is worth the trouble.

Kiss and cuddle our kids!

I hope I haven't scared you off. The rules aren't too tricky, and our kids (like all kids) need all the love and good times that they can get. Big cuddles are awesome, tickle time is hilarious, holding hands is precious. If you are well and washed, please don't treat our kids differently.


That's pretty much it! Our lives changed dramatically when we got this diagnosis. We try to keep things as normal as possible, but it's not always possible. Our friends and family are so important to us, our child’s health more so. We need you, please be there for us.

This was an amazing blog entry that another Cystic Fibrosis Mom had written and I thought that ALL of our friends would benefit from reading it. Since ALOT of people like to say that she doesn't look sick and yada yada yada! 

Aiming To Make Treatment Time A Little Easier

This is a different blog entry for me. I am posting this for other Cystic Fibrosis Mommies or just other Mommies to try this technique. Yesterday, while we were at her CF Appointment her physical therapist and I were talking about different methods of praise for when she cooperates to take her medications & vest. I was giving monetary praise each time she would sit through her vest/nebs and take her pills. Well it was getting really expensive plus she was only doing the things to get the money to go buy stuff. We came up with several different ideas and I wanted to post them for you guys to try if you would like. 


At times things can get very stressful and trying when it comes to treatment time especially in the morning. I am very heartbroken each time that I have to hold her down and force her to do it. So I spoke with her physical therapist for ideas to help make this easier and will stop breaking the bank. 






Praise Chain


This is similar to the paper chains you would make for holidays. Cut a bunch of strips of paper that are 1 inch wide by maybe 6 inches long. You can use paper with the child's favorite colors, patterns, animal patterns, etc on them. And each time that they take their medications, do their nebs and vest without a fuss, they get to add another "ring" on the chain. Allow them to tape/glue it on. It will make it even more fun for them. And set a goal for how long the chain has to be before they get a special prize or go to their favorite place, also choose which prize they are aiming for before beginning the chain. Once the chain gets to the goal length then they get a special prize. 








Magical Fishy Bowl


This idea sounds really fun. Get a small glass jar or dish. Mark a "magical" line on it where you decide the goal height will be. Not too high up on the jar/dish because if it is too high then they may get discouraged. Once you do that then have the child choose what they want to put inside the "Magical Fishy Bowl" each time they do their meds/treatments. Some ideas can be marbles, wrapped pieces of candy, legos, their favorite dry cereal, etc. Once the "Magical Fishies" reached the goal line then they get their prize that they were aiming for. One "Magical Fishy" for each time they do the entire vest session or all of the meds without a fuss. 






Sticker Chart


This is pretty self explanatory. Help draw your child draw up the chart and color it. Then allow them to pick their favorite stickers out. And put one on each time they do their treatments/meds and once it is filled they get their goal prize.





I will be adding more ideas to this blog as I think of more fun ways to help make treatment just a little more easier for everyone!






Some of the goals prizes that Bennett has chosen to aim for are:


Uncle Bubbie gets to take her to the firehouse for a few hours in the evening
She gets to have a friend spend the night
An Itunes card for her Ipod
Stay up late and have a special movie night

ENT -- Dr. Rizzi

As you all know Bennett has been sick for a very long time again. It all began back in September and it has been a revolving door ever since. She has been having a junky, wet cough, random fevers that go as high as 103 but are broken with Tylenol, sinus congestion, crackling/wheezing and tightness in her chest/lungs, coughing episodes until she brings up tons of mucus, swelling in her cheeks/nose/eyes and ear pain. She is just all around miserable. She has been seen 8 times at CHOP (both ENT & CF doctors), 15 times at her pediatrician and has had 9 ER visits since September 4th. Yet, they keep saying that they do not want to admit her and subject her to more germs. Which I totally understand. There are far worse sick children on the CF floor that she goes too and he is protecting her from getting even worse. While he is protecting her, she remains miserable and it is making me extremely frustrated. She will be starting her 11th oral antibiotic tomorrow morning when the pharmacy gets in their shipment. They have done countless chest x-rays and only one of them has shown bad spots. She has had 9 throat cultures and all of them have been clean besides the usual stuff she cultures. They did a round of TOBI to keep that bacteria at bay so it didn't cause extra problems.

Well today she has an appointment with her ENT at CHOP. He reviewed her CATScan images and said that they looked worse than the one from 8 months ago. Her right side is worse than the left. He checked her ears and there is fluid in them which is causing hearing loss. She has had the fluid for 3 months. He wants to recheck her ears in 4 weeks and if the fluid is still there then they will do another hearing test on her. We discussed tubes and agreed that she needs them inserted again.

We also discussed endoscopic sinus surgery. He said that she definitely needs it and will benefit from it. He explained the procedure in detail to me and it caused my anxiety to get really high.

These are the questions that I asked and the answers that Dr. Rizzi had given me:

What are some of the reasons you are not wanting to do the sinus surgery on her now?

He said: The reason he isn't wanting to do the surgery right now is because he does not want to ruin her facial bones. Sinus surgery causes alot of scaring. Her facial bones are still forming.

What else can we possibly do for her besides the oral antibiotics and steroids?

He said: The ONLY option that is left is 14 days of IV antibiotics followed by endoscopic sinus surgery because we have exhausted all of our resources meaning that we have done more than enough oral antibiotics without any progress. Then another 7-14 days of IV antibiotics after the surgery is done because of how much trouble she has had. Any how many back to back sinus infections she has had.

Since she has had this nasty wet, junky, congested cough is this now a permanent thing that will we have to get accustomed to since we cannot seem to get it cleared up?

He said: No, it isn't a permanent thing. We need to get to the bottom of why it is lingering as long as it is. He said he also feels that her sinuses are causing post secondary problems for her lungs and respiratory system.

Okay, so what is the game plan now?

He said: I understand you are beyond frustrated. You are sitting here crying. But, this is what I want to do. She needs 6 consecutive weeks of oral antibiotics before coming in for the IV abx and endoscopic sinus surgery to get on top of everything. She has already done 3 straight weeks of Bactrim and is on her 7th day of Cipro. We are going to do another 14 days of Cipro and then come back in for a pre-surgery appointment. Unless, she continues to show signs of side effects from so many antibiotics. Right now she is having tummy discomfort after taking the Cipro. He said if it continues then we will take action immediately instead of following the protocol that he likes to do with his patients. He wanted to do the 6 weeks of consecutive oral abxs because of how nasty her sinuses looked. And since sinuses are nothing but bones and hollow spots it takes longer for them to absorb the abx. Cipro is an abx that gets absorbed by the bone. It will take at least 14 days for the bone to absorb enough Cipro to start clearing out the infection. So, the plan is to do another 14 days of oral Cipro then go in for a pre-surgery appointment unless the tummy discomfort continues. He is also consulting with Dr. Rubenstein her Cystic Fibrosis doctor.



This isn't part of the update... I am getting fed up with people telling me that something isn't being done properly to care for Bennett. Each CF Center treats their patients differently. Yes, I understand other clinics would have admitted by now but her center doesn't admitt patients until they feel it in their hearts that they have done everything possible to avoid exposing her to extra unneccessary germs. I am beyond frustrated but once I sit down in a quiet room and think about everything that we discussed it all makes sense.

But, you all are up to date with what is going on. I just ask that you continue to pray that things start going the way we are planning it to go. Pray the fevers stay away. The cough instantly disappears. The vomitting and choking on mucus comes to a hault. And her sinuses become clear overnight! CF is a very difficult and stressful disease. And you will never know what our life is like unless you are walking the same journey we are! I appreciate the amazing support that some of you continue to give me. I truly love you all and thank you for always being there for me. This journey is far from easy!

Uncle Bubbie

On this day 18 years ago I became an aunt for the very first time! I was mearly 10 years old when he was born. I remember the day. The night that my sister went into labor there was a blizzard. We barely made it to the hospital before he was born. I was very excited and proud to be an "aunt". Little to my knowledge that I would end up having a 75% hand in helping raise this little bundle of joy. Growing up he was extremely close to my father. He was his little sidekick. If my father was in the garage he was by his side tickering on something. He loved going out on the road with my father who was an over the road truck driver. Richard is not your typical 18 year old. He helps hold our family together. Him and his Ninny (grandmother) are very close. He became the "Man Of Our House" when he the time came. He is our repairman when something breaks, our plumber, our mechanic, he is our security guard if something bad is happening.  He has his head on his shoulders and is striving for his goal of becoming a state police trooper. Everywhere we go no one has a negative word to say about him. I get complimented all the time on how well mannered he is. I am so very proud of him.

On November 21,2006 Richard became an UNCLE for the first time. Techinically if you want to follow the silly family tree he is Bennetts cousin, but in reality he is her UNCLE. He spent every single minute of the 25 1/2 hours of my labor by my side. Never once left. He was estatic to become and UNCLE. Not many people know this but he cut Bennetts umbilical cord. Bennett is the apple of his eye. She is his number one girl.

He has had countless sleepless nights staying awake with me when she was having really bad nights, has gone to numerous doctors appointments, spent countless hours in the emergency room with her. He worries about her constantly when she is in the hospital. He is truly the worlds greatest UNCLE to her and Bradley.

He lives and breathes for these two children. He is always talking about his niece and nephew. Bennett and Bradley are very lucky to have such an amazing UNCLE in their lives that loves them unconditionally even when they are bad and make him do silly things!! January 25, 2010 he became an UNCLE for the second time to Bradley. He now has a NIECE and NEPHEW.

Basically why I am doing writing and dedicating this blog to Richard is because I want to tell the world how extremely proud of him I am. He is an amazing kid. He isn't into drugs or alcohol. He is turning 18 years old and is a member of 3 fire companies, 1 EMS Station, works hard  at his job (nearly full time hours) all while going to school and striving to be a state police trooper. He never once complains when it comes to being our "Nanny" (we jokingly call him that). He is always there for me when I need him to babysit, ride to an appointment with me or when I ask him to sit up and keep me company when Bennett is having a bad night. I do not regret one bit stepping up and helping raise him. I am proud to say I am one of the reasons he is the sweet, well-mannered young man that he is! And I wouldn't have it any other way!


Happy 18th Birthday Richard. I love you so very much! And I am extremely proud of you! You are becoming an amazing young man! Keep up the awesome work!!

My Uncle  Bubbie

My uncle is a special man, Always helping all he can.

He takes time to listen to me, Whatever my gripes might be.

Other times we just hang out, And I learn what life is all about.

My uncle’s very special, I love him a whole lot too…

My uncle’s wonderful because my uncle’s you!

With All Of Our Love,

Bennett & Bradley

Bennett IS A Blessing

The other day when I went to pick Bennett up from school her one teacher pulled me to the side and said she had something that she needed to tell me. But first let me begin clear back to the beginning so everyone understands why she said what she did to me. The director of the Pre-K program had a son with Cystic Fibrosis and he recently passed away at the age of 25 a few months ago. When I called to register Bennett for her new school, I spoke to the director and when I heard her say her name; I knew who she was because I knew her son. I was VERY hesitant to continue on with the conversation. I just about hung up on her. I did not want to bring up the subject of Cystic Fibrosis because of her son. I actually stayed on the phone and told her that I wanted to register Bennett into her pre-k program. I didn’t mention that she had CF until I went to fill out the paperwork and give Bennett a tour of the school. My heart was racing and I was sweating because I did not know how to bring it up. I did not want to bring up the subject and cause her to relive the tragedy of her son passing away. A million thoughts were racing through my mind. Do I tell her? Or do I tell one of Bennetts other teachers? I need to tell someone so they are aware of it and can administer proper care if something were to happen. Seconds felt like minutes. Minutes felt like hours. As we were sitting there talking about the prices, hours and the program details I just blurted out that Bennett has CF And immediately my head dropped. I was scared to look at her and see her reaction. Her words weer "Really? My son recently passed away with CF." I told her that I knew her son and then she began to openly talk about it and ask questions about Bennett. It was a major relief. We actually spoke nearly two hours both about her son and Bennett. When I had left the school from registering her I was still on the fence whether or not to send her because with the director seeing Bennett daily at school I didn’t want to keep CF right in her face. Make sense? I felt as if I was causing her more pain and hindering her healing process by her seeing Bennett for 8 hours a day 5 days a week. Each morning when I take Bennett to school she greets us with huge smile, gives Bennett a hug and asks how our night was and how Bennett is feeling. Then when I pick Bennett up she hugs her and tells her she will be thinking of her and she cannot wait too see her tomorrow. When she misses school from being sick and not feeling well she will call me at home to let me know that they are praying for Bennett and checking too see how she is doing. So now that you all are caught up to date this is what her teacher had to say. Bennetts teacher had said “Thank you for registering Bennett for our school. Since she has been attending here Margaret’s spirits have been lifted. She seems so much happier having her here. Bennett is a blessing to her. She talks a lot about Bennett and we pray often for her.” I then told her how I was hesitant to send Bennett to this school because of bringing up bad memories. The teacher said that the other teachers have noticed a difference in Margaret since she started attending there. It feels good too see her smiling again. I did not know how to respond to this. After having the talk with the teacher I no longer feel guilty sending Bennett there. Maybe it was a blessing in disguise for me to call that school first and make the decision to send her there before looking at other schools. Knowing that Bennett is helping her cope with the loss of her son melts my heart. I always knew my daughter was a blessing to me but it is something else when she is a true blessing to others!