Tuesday, August 12, 2008

(Bump) *Not* All CFers Are Created Equal

This was originally posted on 3.21.08. I thought this would be a good post to bump back up to the top for those who missed it the first time.

So, after posting about some of our CF friends the past few weeks (I encourage you to go back and read some of the CF-related posts from early-mid March), I've had several people ask me about some of the differences between their conditions and Tricia's. I won't give you any more info about the other CFers, because I haven't asked for their permission to do so, but I will explain some of the differences that can be seen between CFers (again, realize that this info is coming from my own research and education and experience).

Every CFer is different from every other CFer for several reasons:

> There are thousands of mutations of the disease, and each mutation can show itself in different ways in different hosts. Some CFers show symptoms at birth and are diagnosed very early. Some show symptoms for years, but are either misdiagnosed or undiagnosed until later in life. Some show very few symptoms and are never diagnosed.

> CF can present itself it two big ways: Pulmonary (due to increased mucus production) and Digestive (due to a deficiency in pancreatic enzymes which help digest food), and therefore (from what I know), CFers can be divided into 4 categories:

1) Those have both major pulmonary and digestion problems (the vast majority). Tricia falls in this category. These patients have serious respiratory problems as well as serious problems with gaining and maintaining their weight. This is why most CFers (before they become incredibly sick) have to take pancreatic enzymes with every meal/snack (forgetting/refusing to take enzymes can be painful) and can eat a ton of food and always be very thin. These patients will often need both lung and liver/pancreas transplants.

2) Those who have major respiratory problems but little or no digestive problems because their pancreas can produce enzymes (about 15% of CFers). These patients tend to have little to no issues gaining and maintaining weight and are not restricted to a high calorie that most other CFers adhere to.

3) Those who have major digestive problems and minor respiratory problems. From what I know, this is very rare, as usually the respiratory issues are the most prevalent. These patients will often need liver or pancreas transplants.

4) Those who have little respiratory or digestive problems. These are usually the patients who are either mis or undiagnosed for their entire lives.

> Medical care can vary greatly. Unfortunately, some parents/guardians of CFers are either uneducated or unwilling to provide the needed care for their children. You would not believe how many stories I have heard (from children, grandparents, etc.) of parents/guardians who simply do not care about their child's CF....parents/guardians who smoke, provide unclean living conditions, ignore flares, neglect treatments, etc. And, unfortunately, even in the US, many CFers live in areas that do not have a hospital or CF Center near enough to provide the most necessary care/expertise/technology available.

> Personal decisions and attitude can vary greatly. Every CFer makes his/her own decision about his/her personal care as well as the risks he/she is willing to take. As Tricia grew older, she was given more responsibility for her own CF care. Fortunately, she has made the decision to do what her doctors advise and to live a fun but cautious life, but many do not make the same decisions.

> Finances can also have a huge affect on CF care. To provide the care needed for most CFers requires either great personal wealth, great insurance, or great help from third parties.

I'm sure there are several other factors that can affect a CFer's personal health and experiences, but these are the few that I could think of off the top of my head. As always, feel free to research CF further on your own.

Thanks!

Nate

29 comments:

Jane-Jane said...

medicine is a practice, each person is different than the next (even twins...multiples) and their body may very well react differently than it did the last time they had that illness/medicine. Even a "healthy" person will react to a cold differently from year to year. There is nothing "cut and dry" about medicine.

Thank you Nate for being so patient and explaining all of this in such detail. Tricia, again, you are a very blessed lady to have such an awesome husband....imperfections and all!

still praying for you in SC!

Jeff said...

Good evening friends,

My wife, Wendy, and I follow your blog daily and celebrate the victories and continue to pray for your sweet family. On several of you posts Wendy has noticed that you long to be with your church family. Although it would not be your own, we thought that it might bless you like it has us to check out our church, if you have the chance and the desire. Here is the address www.lifechurch.tv. We hope it will be encouraging to you, as it has us. Jeff & Wendy Warner

Blonde_Lawyer said...

Thanks for educating more people about CF--I figure, the more people know, the better!

Just wanted to add the CF can have a major effect on the liver as well...I only mention it because up until a few years ago that was my husband's doctor's primary concern in treating his disease.

Beverly said...

Thanks, Nate. Your blog is doing so much in educating people about CF.

Heidi said...

Great you explained this! I was born in Boston was dx at 6 months with CF by sweat test. Stayed in Boston Childrens for over a year on and off respirator and then in and out of hospital every year with phnumonia and resp illness until 10 years ago when more tests came back negative and that I was misdiagnosed with CF but had Bronchiactisis instead? Still unclear and learning about everything. I am nowhere near as bad off as Tricia though and I am definitely keeping her in my prayers. All of your strength is an inspiration to many I am sure.

amangoprincess said...

Good post..I was wondering if a CF patient gets a double lung transplant and a pancreas/ liver transplant, does that cure their future symptoms and flare ups for good?

Carol said...

PS I love the picture of Gwyneth's hair and also the many views of her beautiful hand and fingers. I can't wait to see the pictures of the dimples in her knees.

My2Girls said...

Jeff -
After reading your post about www.lifechurch.tv, my curiosity got the best of me, so I decided to check it out for myself. I was only going to briefly "browse" the site before going to bed. Well.....that was 45 minutes ago! Ha! I ended up getting "hooked" and I watched a whole, entire sermon. Great site! Thanks for sharing! And, yes.....I really AM GOING to BED NOW! God Bless.

twin power mommy said...

can relate to the breathing treatments (although they CANNOT compare to what tricia has endured all her life), have had asthma for my whole life and thought it went away in my early adulthood. after my second pregnancy, it became so bad that now i am having to take inhaled steriods twice a day to keep it at bay.
praying and praying...

Bobbi said...

Thank you so much for educating people about CF...knowledge is the only way to get the cure!!! My daughter was pancreatic sufficient up until about 6 months ago when she changed to pancreatic insufficiency so now she takes her enzymes with her food....her main issues are still pulmonary though, she is MRSA positive & culteres PA.

The Nolls said...

Hi there!
I have really enjoyed following your blog and try to check it as often as possible. It has been a tremendous blessing to me to watch your faith lived in a difficult time. I was especially encouraged by your recent post about how your situation is NOT SAD. It is so true that because of who your God is, your situation cannot dictate sadness.
I just wanted to comment and let you know that every time I type in your web address, I just pray pray pray that you will have breaking news that Tricia is getting her new lungs as we read. I have come to long for the day that you can share that news and we can rejoice with you all. Praying that it happens very very soon.
May God continue to bless and keep each of you and be glorified as you live for Him and share his goodness so faithfully.

Scott said...

Thanks Nate, that was very informative. I'm sure many people are more aware/educated just from reading this blog.

jayla526 said...

I had my transplant at Duke on 5/11/04. Some think I have CF because of my age – not many young people need lung transplants. I have a rare disease called lymphangiomatosis - also a childhood illness – but much rarer than CF. Before coming to Duke I didn't know anyone with CF. My only experience was when I was a child – I was in the hospital and walked by a room and saw nurse pounding another girl about my age on the back. I asked my Mom what in the world she was doing and she said - she has Cystic Fibrosis. What I didn't know then was that when I grew up – many of my closest friends would have this condition. I've met a lot of CFr's over the past 4 1/2 years and they have their differences in their case, but they are the same in their courage, perseverance, independence and liveliness. It has been an unexpected blessing in my life to get to know each one of them.

Emmie said...

You're so right Nate, CF is an unusual disease in that it can affect people so differently. So whilst one child may die as a toddler, there are (a few) people collecting their pension who have it too.

My brother and I both have exactly the same mutations (deltaF508 and 3659delC) which make us both pancreatic insufficient (so we need Creon) and are also in the "more severely affected" gene bracket. And yet whilst I struggle a lot with repeated infections,lots of IV's, bowel blockages, liver cirrhosis, etc. and need my husband at home permanently to help care for me, my brother is totally the opposite. He has 90% lung function, runs 3 miles a day, works fulltime, has completed the London marathon and is aiming to do the New York marathon this year! He has never even needed a nebuliser, let alone IV's!

So even when people have identical genes, are diagnosed at the same age, receive equal (and excellent) medical care from parents and hospitals, are equally compliant with all their treatments and grow up in the same environment...they can still show very different effects of CF!

Heather N said...

Yea, that pretty much sums it up!

Up until 6 months ago I did a great job with my weight. But, I'm 30 lbs lighter now and it's not coming back as easily.

Glad you pointed out the family issues, as well. BIG problem.

Cheryl said...

Thanks for all the info on Cf, Nate. I am learning so much. Just a little side note about the category of misdiagnosis. I went to Bowling Green State University in Ohio way back in the 60's. Two of my professors were Ernie and Dorothy Hamilton, parents of Scott Hamilton. Scottie was about 5 at the time and not growing. He had lots of respiratory problems and digestive issues. The local doctors were treating him for CF because they thought he had it. So his parents encouraged him to skate in the new ice arena to help his breathing. As it turned out, he did NOT have CF, but another enzyme disease not quite as serious. He was also adopted. So Scott Hamilton, the world famous figure skate and commentator, was also misdiagnosed, although he had many of the symptoms that you have described. Just reading your latest update made me remember way back to how Scott's parents were also visiting doctors and doing research on CF. I think that parents will go to the ends of the earth to help their kids.

Happy Easter to your family, Nate, and my prayers are with your two beautiful girls.

Hugs,
Cheryl from Fairfax

Elizabeth said...

Nate,
I have been reading your blog and would like you to know that my family is praying for yours.
I have a dear friend who fought CF for 33 years. He was an amazing man of God who greatly influenced my life. He received a living donor transplant from three different donors (a third was done after one transplant did not take). Each fighter of CF has a different battle. I am praying for your beautiful wife and daughter. I know (as I am sure you do as well) that there is an AMAZING God who holds tomorrow in His hands. I believe He has only the best for you and your family.

Lots of prayers!
In Christ Alone,

Elizabeth
http://sweetygyrl77.wordpress.com

Tracey said...

Well said! I have cerebral palsy, and I have been telling people the differences in CP my whole life.

Thanks again for letting us in to your world. I have learned so much (encouragement, the importance of being loving and loved, about CF, and the power of prayer), just to name a few things.

I am sending prayers and lots of love!!

The best part of my day is checking in to see how your family is going.

Happy Easter!!

Sara said...

Great post. I just wanted to add that CF can also affect male fertility by causing congenital bilateral absence of the vas deferens (CBAVD). Some men are only diagnosed with CF when the CBAVD is discovered.

Angela R. said...

Nate, Thank you for educating people about CF. I think you pretty much hit every angle. and the truth is, unless you live with it or know someone really close with it, you don't really know what it is. And it is so important for people to know what it is about and how severe it is and can be. Awareness is key! So as a CFer, I thank you once again!

-Angela

jellyeels said...

I come in the 3rd category. I have CF and am affected most in the digestion department, my chest isn't affected as much as my digestion and have had liver disease for 17, the last 5 years it turned in to cirrhosis of the liver. I had being listed for liver tx for 14 months and was slowly get worse. I finally got my tx 7 weeks ago. So i guess you are right those who suffer with digestion more than lungs do go on to have liver problems, as i'm a prime example.

Nate your blog is excellent reading.

Jen said...

Recently heard of a lady who was diagnosed with CF at age 50!!! How does that happen? She doesn't have a blog or I would send you there. I thought of you guys immediately when I heard.

WalkingNFaith said...

What a blessing it is to see such an education endeavor through this website. You have so many hits, it's a great opportunity to inform. Information, education, research dollars and prayer can make such a difference in so many struggling, young CF lives.
Thanks for sharing the incredible journey you all have been on. It strengthens my resolve to do all that I can to see a cure soon!!!
May your journey be overshadowed by HIS grace!

Violet said...

I believe i have left a comment once before. I'm Violet, 22 years old. I'm so blessed by your blog. It's amazing to read about your family and I find it a privledge to pray for your family. It's been sort of healing for me. When I was 13 I met a young boy with C.F. (severe, like described in the 1st type). At 9 he had a double long and heart transplant. At 12 he had 75% of his pancrease removed. He was the first person I fell in love with, my fist boyfriend, and when we were 15, my first experience with the death of someone close to me. Now, as I approach 22 and feel ready to day again, I find I'm first having to go through another grieving step and reading the blog of another who loves a CF-er is helping me.

Your blog is beautiful, your family is Beautiful. I'm brought to tears often, recently by the "bald is beautiful" blog. Phew, I'm in tears now. Thank you for sharing your, trish, and gwens stories!

Violet said...

I believe i have left a comment once before. I'm Violet, 22 years old. I'm so blessed by your blog. It's amazing to read about your family and I find it a privledge to pray for your family. It's been sort of healing for me. When I was 13 I met a young boy with C.F. (severe, like described in the 1st type). At 9 he had a double long and heart transplant. At 12 he had 75% of his pancrease removed. He was the first person I fell in love with, my fist boyfriend, and when we were 15, my first experience with the death of someone close to me. Now, as I approach 22 and feel ready to day again, I find I'm first having to go through another grieving step and reading the blog of another who loves a CF-er is helping me.

Your blog is beautiful, your family is Beautiful. I'm brought to tears often, recently by the "bald is beautiful" blog. Phew, I'm in tears now. Thank you for sharing your, trish, and gwens stories!

Melissa said...

A dear childhood friend of mine lived with CF and beat many odds.. she was an angel right here on earth. Originally, her prognosis was that she would possibly make it to age 10. Every summer she seemed to do a stint in the hospital but she never lost that freckled smile and loud laugh.

She had a double lung transplant her sophomore year of college and blossomed. It was amazing and she absolutely glowed.
We lost her in her mid 20s to cancer of all things, but 15 years later, she still inspires us.

Thank you for your beautiful blog.. you and yours are in my prayers!

Tracy Anne said...

Thanks so much Nate! I didn't see this the first time you posted it. A cousin of my cousin was just diagnosed at 54 years of age! She's always had respiratory issues but not the pancreatic stuff. It's so weird.

Ruth said...

I guess the financial part only counts in places like America where medical treatment isn't free. I live in the UK so get my treatment free and my medicines for a tiny yearly fee.

I don't know what my mutation is but I'm told it's very rare so I feel special!

JT... ^^, said...

I felt like I just have to say something...

...You're great, Nate. Thanks for inspiring us with your wonderful blogs.

God bless.