The Update - Life for us with CF

Like most diseases Cystic Fibrosis does not manifest itself the same in any two cases. Not only does CF have many varied gene mutations but even those with the same two gene mutations will show with seemingly different severities. There is no way to predict CF progression. But progress it will as there still is no cure or control. For some of our friends and family CF is still a mystery. Without over simplifying, our bodies produce thin slippery mucus that coats and protects our organs. In CF the body produces thick sticky mucus that clogs the lungs and digestive tracts and creates a breeding ground for nasty germs. This disease literally slowly starves and suffocates its victims. It is because of the germs that grow in Mike's lungs that he needs repeated "tune-ups" (IV antibiotics). Mike is never without infection in his lungs. The IVs just minimize the level of germs and then they progressively get worse until the next "tune-up" yep, kind of like an oil change - thus the term. As time and progression continues the need for IV antibiotics gets more frequent. While we are all still hoping, praying and raising money for a cure, the damage done inside the lungs from repeated infections is irreversible.

Mike has been hospitalized more times than we could possibly count spending years of his life in the hospital. When you use antibiotics that much the germs that you are trying to kill can mutate and become resistant to the antibiotics. There is research that also shows that sometimes without repeated IV use the germs can still mutate and become resistant - like I said each case is different. We don't know how Mike's have gotten to where they are (really not important at this point) but they are very resistant. Mike grows some germs that are resistant to all drugs currently available. They do seem to respond to a mix of a couple antibiotics right now, thankfully. As damage to the lungs increase pieces of his lungs no longer function.

This brings us to where we are today. Mike is currently living on about half of his lung capacity if I understand everything correctly. And the half that still works gets infections and goes down sometimes as low as only 26% before coming back to his now baseline of around 45-50%. Obviously, when so little of his lungs are working his heart has to work extra hard to get oxygen to his body. His organs are strained from the lung infection and harsh antibiotics. The doctors are always searching for the balance that causes him the least distress. Oxygen at night helps him get better sleep and night feedings through a g-tube helps replace the calories that are lost fighting infection and trying to breathe with reduced lung capacity. Right now Mike seems to need antibiotics about every 6-8 weeks. When he gets out of the hospital he has a couple of pretty good weeks before the infection starts to gain momentum. An oral antibiotic will usually stop the progression for a few weeks and when that is finished it is just a few more weeks until his lung function drops to a point where he needs IVs once again. The cycle repeats.

The most important things to know:
Mike isn't doing anything wrong.
This is CF progression in Mike - not all CF patients.
The doctors are on top of things and trying what works.
This could go on for a very long time.

So why do we feel that now was a good time to share this information? A few reasons but mostly because we know how much you all love Mike. This large extended family is hard to keep up with and we know you all have lots of questions and are concerned. We just thought it would be easier to put it all out in one spot so everyone will have the same info.

While we are hopeful that Mike will have many more years with the lungs he currently has, there is no way to predict an outcome. We have talked about transplant. This could be many years away or it could be just a few months or a few years away. It just depends on the damage these infections do to his lungs. Again, simplified, the general rule right now is that when the infections get to the point where the damage to the lungs cause his lungs to no longer get above 30% he would be listed for a transplant - meaning that his life expectancy with this set of lungs would be around two years from that point. CF patients with lung transplants are living for years and the life expectancy is improving all the time. All good, promising stuff.

Mike is living his life. Not recklessly but realistically. There will be times when he chooses to do things that may seem to others to be detrimental to his health. Only Mike will be able to decide when it is time to be hospitalized and take a break and when it is time to push things.

It is hard to plan. It helps when our family and friends are understanding. It helps when everyone understands that Mike needs to take all the meds and do all the treatments. It helps when people understand that sometimes for us, things just happen at the last minute. We don't expect that others can always accommodate us, we just want you to understand we are trying. Mike wants everyone to understand that he cannot predict when he will need to be hospitalized. As CF progresses in him it is harder and harder for him to "plan" future functions. While he can sometimes wait a few days or a week to be admitted, there are other times when the infection gets bad and things seem to go downhill quickly. He tries hard not to let people down but he can't always do that. He has spent every holiday in the hospital at some point. Yes, even Christmas and his birthday. Didn't plan for that.

As Mike's infections get more frequent his immune system is getting weaker. He is at greater risk for infections of all kinds. A simple cold or sinus infection to you can mean another course of IV antibiotics (usually 14 days) for him and the risk of further damage. This doesn't mean Mike will exist in a bubble but it will help us if you let him/us know if you are sick. Hand sanitizer and masks etc., can do a lot. We respectfully ask that if you have been invited to our home or have invited us to your home and someone turns up sick that you would just let us know so we can take the appropriate precautions and make decisions from there. Depending on where Mike is in his cycle he may or may not need to change his plans.

One of the harder things for me as his mom is when people ask me how Mike is doing. I don't always know what to say. I may say, "Things are hard." Sometimes to that response I will hear people bewildered say, "Well, I just saw Mike out last week??" Without explaining all that has just been typed above how do I respond? Do I just tell people he is fine? Not only would I feel like I was lying I would also feel like I was denying his pain and struggle. So i don't say too much. But yes, Mike is out and about when he feels well. A week or even a few days later the infection can flare and he finds it hard to climb the stairs to his room. He may not be coughing much (or is able to suppress it) today and a few days from now he may not be able to control it and he coughs until he almost collapses. This is CF. This is our reality. It is more difficult at times than at others. And if you have read this far we thank you for trying to understand. We thank you for your love and support!

Mike is living with CF. Things will get harder as CF progresses. Mike is living each day to the fullest with what he has been given that day. He is strong and brave, frustrated and human. Mike understands that his times are in God's hand and he is confident in God's plan for him. Could Mike pop back up and have a miraculous recovery - with God all things are possible but given the level of damage and resistance, it isn't too likely that that is part of God's plan. We are so thankful for these weeks when Mike is able to dance and workout and be out and about with all of you . . . We have faith in Romans 8:28 And we know that in all things God works for the good of those who love him, who have been called according to his purpose.

God is in control and he is helping us do the best we can! I will continue to update the blog with the ups and downs (not quite this lengthy ;)). From the bottom of my heart thank you for your support and understanding, for your encouragement and love!
Blessings!
P.S. If you have any questions don't be afraid to ask :)