What is cystic fibrosis (in my limited opinion)

Someone asked me the following:

"Hey Karri I've been researching CF because I'm not too familiar with it. Most sites aren't clear about long term effects and give vague prognosis. I know Max has been in and out of the hospital a lot. Does it get better as he's older? Does it mainly cause problems with the lungs? Sorry for so many questions I just want to understand it better." 

And the poor girl got a novel in response. My reply is below. I apologize in advance if the information is not accurate or there are parts missing. I'm not a science person and this is how I understand CF as it was explained to me. I hope to learn more and be able to explain it better as time goes on but it's alot to take in. Especially since it's not something I'm learning about in a book as an unemotional topic. I'm not only learning the science but how to live with it, how to deal with the emotions of it, and how to make it a normal part of life.

"Thanks for asking. Cf is a progressive lung and digestive system disease. His body has a defective gene that effects the way salt works in his body. Salt in the body helps to break up mucus. Since his body has an issue with processing enough salt, his body has a lot of mucus in it. What this means is that his digestive system and lungs fill up with mucus. 

Lungs: the problem with mucus in the lungs is that bacteria sticks in his lungs. He is easily susceptible to get pneumonia, rsv, and other bacteria that doesn't effect you and I such as 1 called pseudomonas. Every time he gets a lung sickness, it leaves scars on his lungs. Eventually, this will be detrimental and he will need a lung transplant. Also, they always have to check his oxygen levels because if the mucus gets too thick in his lungs he will have difficulty breathing. We have to do breathing treatments everyday without fail (2x a day for now but more when he's sick and as he gets older). As he gets older, his breathing will get harder and harder bc cf is progressive. He will get worse. 

Digestive: he cannot eat anything with out taking medication called enzymes. Our digestive is mostly mucus and his is thick and sticky mucus. He is unable to digest fat soluble vitamins (ADEK) and any fats or proteins. Feeding him a bottle without his enzymes is the same as pouring it down the sink. It will go right thru him and he would get no nutrition. Before enzymes, kids would die before they were 5 because they would starve to death. So he takes his enzymes before all feedings and he takes a daily vitamin called aquADEKs. Additionally, he takes Zantac (ant acid) daily to help his stomach break down food. People with cf also get a special type of cf diabetes that is a combination if type 1 and 2. It's because their pancreas doesn't work right. Finally, because his body works so hard to function, he has to eat 2-3x more calories than the average person. Most people with cf are under weight. They've found that the better their weight, the better their lungs function so the drs monitor his weight closely. Once he starts eating foods, we will have to give him high fat & high protein foods and shakes. He will have to eat every 2 hours. All of his foods will have added cream, butter, and other fatty stuff. And salt. He has to have a lot of added salt to his diet. Currently he gets 1/8tsp of salt a day but will probably be bumped up to 1/4tsp since he's over 6 months. Doesn't sound like a lot but measure it out and pour it on a plate. It's a lot for a tiny guy.

To be perfectly blunt his prognosis is this: it's permanent, there's no cure, life expectancy is in the 30's but it's a tough road to get even that far. It gets worse as he gets old and it's highly likely I will see my son die. I rarely say this because I try and keep a positive attitude. But the truth is I see parents on my cf board tell stories about their elementary school kids dying. There was a 10 yr old girl in the news recently that was in the final stages of her disease. No one knows.

An average day in our house consists of an hour of breathing treatments now with the vest (which rocks bc we were manually beating his chest). Adults with cf spend up to 6 hours a day doing breathing treatments and vest therapy. He eats every 2-3 hours and about 40oz of milk. I have to supplement with formula bc I don't produce that much. I'm sure I would if I were a sahm but I have to work. He has to have enzymes before every meal. So I can't just make a bottle or breast feed. We pour the pill capsule beads into a spoon full of apple sauce. When he gets older, he will need to exercise. We have to clean like crazy and sanitize like crazy bc if he gets sick with a normal cold, it's a hospital stay. He has to have strong antibiotics when he gets sick to kill whatever is stuck in the mucus in his body. Kids with cf often throw up for no reason either because of excessive coughing or because of getting too much mucus in his stomach. When he gets older, I've read, he will need to keep a trash can by his desk for random puking. Their poop is SO NASTY! Stinky! I used to cloth diaper but I couldn't get the smell out. I clothed my first son so it wasn't a lack of trying or knowledge. He snores, he struggles to breath, he always has a runny nose. He has 1 medication that is 4k for 1 month. That's just 1 medication. He's on about 6 right now. Eventually he will be on about 20 a day and 4k will be a cheap one. There is one that is considered a cure but it's 30k a month and insurance doesn't cover it. There is also proper care of nebulizers. He can't be around standing water bc it carries pseudomonas which can severely harm him. Standing water includes things one wouldn't think of like my older sons bath toys that squirt water, shower heads, etc. Every move I make with him requires thought and planning to avoid him getting sick.

Because it's rare disease, the govt does not find research. All research and medication creating is from donations to the foundation. Hoping for a cure in his lifetime. A cure that we can afford. I'm grateful the life expectancy went from 5 years to 37 years. I'm grateful for enzymes and berthing treatments and this new vest that just came in the mail today. But I fucking hate cf!" 

I want to add to this that there are people who live into their 60's and 70's. There are plenty of kids who lead normal, healthy, happy lives with CF. There's even some people who don't even find out they have CF until they are adults and do gene testing to get pregnant. So, it doesn't have to be bleak. This was a moment in which I wasn't feeling too positive because I think this was the first time I wrote it all down in one explanation and it was a rough moment. 

So, what is CF? Its a pain the in ASS!! 

Support

What is support for us?

          There is a big difference between support and a rescue. A rescue is 1. doing something for someone that they can do for themselves 2. doing something for someone that they haven't asked you to do for them 3. doing something for someone that is a detriment to yourself in some way. When people rescue someone else, they often end up with negative thoughts and feelings about the "help" they have provided. Maybe you gave your last dollar to the homeless guy and he bought a beer. You think "that was my LAST dollar. I needed that. And he bought a beer!! WTF!?!" Maybe you do something for your friend that you would have liked someone to do for you and they didn't appreciate it in the way you would have liked. One definition of support said "to hold up; to keep from weakening; to strengthen." That definition gave an example of a tripod supporting a camera. The tripod isn't there to take the pictures but is instead there to help the camera take better pictures. If the tripod was rescuing the camera, it would say "here, camera. You go put your feet up and I will take the pictures." Except, a tripod can't take pictures.

I thought we were talking about CF and support. Why are you talking about a camera and a tripod?

          Individuals and families dealing with CF do need support. During this first "tune up", many people have asked us how they can help. Often times people want to help but don't know how. Or, others may feel like they should help but don't want to, don't have the time to, or don't have the resources to and may feel guilty. And others may think they know exactly what to do but are afraid it may backfire. So, where does support start? The tripod was made to provide a specific kind of support to the camera. It stands there. If the camera doesn't use the tripod, the tripod doesn't say "but i was standing there ready to be all still and the stupid camera didn't even acknowledge me." It doesn't say "all i do is stand here while the camera gets all the glory." The tripod knows exactly what it can do to make sure that the final picture is perfect.

Ok, seriously. Enough with the camera. How can I help? 

          The first step is finding out what you can do to help.  Even if it sounds stupid or simple, it is what is being asked for so respect that. That starts with the CFer or their care givers opening their mouths and asking for it. "Closed mouths don't get fed." Now is not the time for pride or stubbornness, caregivers and CFers.          
 Next, the support people have the right to say no or to say that they cannot do it. I would rather have someone decline to help (for me, honestly and directly) then to have someone help me and then resent me later. And, I personally don't need you to even come up with an excuse. "Uh, see.. what had happened was..." I don't honestly have time for your reasons and I won't remember them later. A simple "sorry, I can't" is just fine for me.I won't hold it against you, I wont hate you, and I will find someone else to help. But, if you are telling me you can help and then back out or do a half ass job because you didn't want to in the first place, that will make it harder for me. So, commit to a yes or no.

You say a rescue is doing something for someone that they haven't asked you to do but I would really like to surprise my friend by... 

          I have had many awesome friends go above and beyond what I asked for because I could not see that I needed something done. I love it. They are awesome.  The trick here is if you choose to do something that someone hasn't asked for, you assume the responsibility of your feelings about the result. Maybe someone wants to surprise the baby with balloons at the hospital but balloons aren't allowed at the hospital. Thank you for the gesture but we can't accept them. It's not a personal attack on you. Don't get mad at me because I have to ask you to take them home or to my house instead. At this point in my life, I haven't slept for 3 days, I have barely eaten, my 6 month old is strapped to machines, my 2 year old is all outta whack, and I am an emotional wreck. I don't have it in me to stroke your feelings about being disappointed that your surprise didn't go over well. I appreciate the gesture though and don't give up trying to help. 

I think that it would be easier if you guys... So, I will help you by telling you what I think you should do

          Uh, no. Do you have a baby with CF? Have I asked for you opinion? Are you a medical doctor... better yet are you OUR medical doctor? No? Then please don't try to do the things for me that I can do for myself such as make decisions about my child's health. I love getting feedback and hearing about things that I didn't know. Maybe you've been in this hospital before and know Nurse Susie Jane. You know that Nurse will do anything for a Starbucks. Tell me about it! Thanks! I love it. But, if I tell you that I already know that Nurse Susie Jane has had a Starbucks today and is still PMSing please don't come to the hospital with a Starbucks trying to bribe her for me.

You look like a mess; take a shower and a nap. Your house is gross. Your 2 yr old is being a brat. You are being irrational. You're bitchy. Your husband, your mom, your .... 

          Zip it. I know. Reserve your judgments please. I am probably going to fly off the handle a few times. I will probably look like hell. My house has a funny smell to it that I can't place because this is the first time I've been home since Thursday (its Sunday). My 2 year old is off schedule. My husband is doing the job of 2 parents. And, we're both still working because we have to afford this outrageous hospital bills. If you aren't coming to clean, watch my kid, give me a massage then zip it. Actually, even if you are helping, still zip it. I will not look like a super model. I am on edge. And all the people in my life who are helping me are probably tired too so if my mom isn't super nice or my husband doesn't say hi when you walk in, please know that we are all doing the best we can. A little understanding and patience is greatly appreciated.

I pity you. I feel so sorry for you.  

          Thanks for thinking of me. There is a difference between empathy and sympathy. Sympathy is feeling sorry for someone; pity. Pity often leads to wanting to rescue (save) someone. I don't need saving and I don't want pity. Empathy is "identification with and understanding of another's situation, feelings, and motives." When you empathize with someone, you are able to put yourself in their shoes a little bit and provide more genuine support and understanding.  It's not really about what you say but what you are thinking. You can still say "I'm so sorry you are going through this" without pitying me. I want you to know that we will be ok, though. I am sorry I am going through this too but it's apart of CF life. If you know me, you know that I am a trooper. My husband is a trooper. Jaxon (my 2 yr old) is a holy terror who is independent as hell and smarter than all of us put together so he will be just fine. And, Maddox (the CFer, 6 months old) is the happiest baby ever known to man. Seriously. Hooked up to machines, this kid is playing with the cords and laughing about it. We will all be fine, I promise.

You didn't even say thank you!

          If I forgot to say thank you to you personally, I am very sorry. Please know that in this moment I want to write thank you cards, send gifts, and sing my praises to each and every one of you. But, when and if I get back to real life, I will be playing catch up in every aspect of my day (work, cleaning, my health, the health of my kids.) I struggle with expressing gratitude enough. I want to "repay" people for everything that they have done for me because I have a hard time accepting support. Sometimes, I just cant repay everything everyone has done. Just know that if you need something from me and I can do it, I will (but you may have to ask because my mind reading ability was lost when I got pregnancy brain 2 years ago). Thank you all for everything you have done, will do, thought about doing, and everything in between.

If you are a CF family, please ask for the support you need in clear and direct terms. Don't expect people to be mind readers. Don't think "if they loved me, they would just do it without me asking. They should know how to help." They don't know how to help. Don't get mad when someone can't help you. Everyone has their own struggles and you may not what their struggles are. Maybe this is too hard for them to be involved in because they fear their child will die and they can't come see a kid strapped to machines. Be understanding of everyone else's situations and life. Be grateful for those that do come out to help. Be grateful for whatever support you are given, even if its not exactly what you pictured. And, when your life is a little calmer, return the favor. I believe in karma. Bank your karma when your kids and family are doing well. Give back to others every chance you get, but don't give with the expectation what that person will "owe" you later.