My short bio: Hi. I'm a journalist and blogger at Hi, this is Eli

My Proof: This photo of us today An article I wrote about his diagnosis An article I wrote about reading about the history of his disease, with audio of me finding out my kid has a terminal illness (in video form)

Update: Sunday 11 a.m. - Gotta go live in the real world for a while, but I'll check in later tonight before closing this bad boy down. Thanks.

Update 7:30 pm: I'll be back to wrap this up in about an hour. if you take anything at all away from this convo, just wash your hands a lot, cover your mouth w/ crux of elbow when you cough and vaccinate yourself and your kids. Those simple things help people w/ chronic illness/ medically vulnerable a lot.

Lastly, my petition that seeks to halt runaway train drug pricing has 90,000 signatures+. I'm trying to get 100k, one for every person in the world with CF. This is it

Comments: 457 • Responses: 57  • Date: 

DarthKoax152 karma

I'm sorry :( My cousin's wife just died of CF. She made it to 34.

I hope transplants are available when they're needed.

Where can we donate if we want to help?

Edit: cousin's

MEGAPUPIL117 karma

a little explanation behind my downvoted to hell comment below - "I am lashing out a bit, and I apologize. Truth of the matter is that as a CF patient we walk around all day with people who know having a slight 'sympathy' for us. Then people who you tell always say "oh god, I'm so sorry" and it sucks. I know it's a kind gesture and with positive intent, however it makes me (any patient) feel weak. It makes us feel different, and we are not. Our lifes are as rich and lovely as yours, our reality just includes CF. It's been something most of us have never lived without."

Best thing you can say to someone who tells you they have CF (or any disease) is "Damn! That blows, you look great!" and then leave it. "Sorry", or "thats horrible!" or "My friends kid made it to 28" is well intended but is difficult to hear.

hithisiseli2 karma

Yeah, I feel ya. I don't want us to be perceived as victims either. We're not. Eli's tough.

mdmarra73 karma

Hey! Don't have a question, but I wanted to say that I went to high school and played sports with a friend who has CF. We are in our 30s now, and although I didn't really keep in touch I know he is still alive and well.

30+ years is a long time for medicine to advance, so hopefully you and your child have a lot to look forward to.

hithisiseli40 karma

Thank you so much for your supportive words!

QuestionableVote7 karma

This, a good friend also in 30s problem free and I hope in we have advancements in a cure soon

hithisiseli3 karma

That is amazing and I love hearing stories like that. Thanks.

BBZZZZTT105 karma

Hey CF nurse here! I mainly work with adults, but is there anything a nurse/ anyone else in the medical team has done that stood out to you?

hithisiseli65 karma

I would say the nurses who stood out were just super detail oriented plus caring. We had good and bad nurses during Eli's first few months of life. He had two surgeries as an infant to unplug his guts - a CF-related condition called meconium ileus plugged him up. So w/ about 30 days in the hospital, I saw a lot.

gummylick11 karma

wow. i've had a best friend pass away at 25 w/ cf and a niece now in her 20's w/ cf... and just you're concern on how to handle it is commendable. i'll let you know that NURSES hold an amazing mark in their lives (not so much.. doctors).. thank you for the work you do!

the_silent_redditor29 karma

not so much.. doctors

Why? As a doctor just finished work on a respiratory unit, what can I do to make you say I've had an 'amazing mark' on a patient's life?

Reddit always seems pretty hard on my profession; I have never seen any comment disparaging the work of a nurse (believe me, there are pretty poor nurses out there, as with any profession), but doctors always seem quick to be criticised on this site.

hithisiseli3 karma

The whole care team makes an impact. Check out these awesome docs who have beomce friends of mine. This is editorial they published today about runaway drug prices. They helped me make a petition on the same topic

juttep110 karma

I'm A CF specific nurse in Ohio. I knew there were other CF specific facilities around but don't know all of them. Where are you located? Our unit is small (11 beds) but we get to see a lot of kids!

hithisiseli3 karma

We are in Oklahoma City. The state has one CF clininc and then an affiliate in Tulsa. Ohio has amazing care for CF b/t Cleveland and Cincy.

RedactedTact73 karma

Do you regret having a child, given his short life and reduced quality as well as the effect on yourself and your family?

hithisiseli125 karma

What a juicy - and good - question. I don't regret having Eli at all. He is a light in our lives. I'm not religious. We didn't know about CF beforehand. Still, he has changed me for the better in every way possible. I believe we will cure this disease in his lifetime. And I'm a jaded-ass journalist.

gosox267381 karma

If I'm not mistaken, only ten to fifteen years ago life expectancy with CF was just 18. Huge strides have been taken in this area, and I 100% agree with you that a cure could be found within your son's lifetime.

hithisiseli57 karma

Thanks, and yes, HUGE strides. We are very hopeful. Parents got together about five decades ago, sick of their children living such short tragic lives. The Cystic Fibrosis Foundation was formed to privately fund science for a cure. We are getting close, though we are billions and years away. Still, the prognosis is rapidly impoving.

littlebirdytoldme2 karma This was how I learned about CF. I'm so glad it's gotten better!

hithisiseli5 karma


unobstructed_views5 karma

Thank you for this, and your insight. My best friend recently found out she and her husband are both carriers of the gene, though they do not have CF. They've been struggling with the decision to have children, (previously had really wanted kids). Definitely sending your links her way.

hithisiseli3 karma

You might forward her the link to my blog. We got a different life than what we expected, but it is an excellent life. We are really happy day to day.

jc_cr54 karma

I found out recently that I carry half of a genetic mutation that would lead to CF. Have you thought about getting yourself and your partner, even your daughter, tested for genetic mutations for CF? This is likely going to be extremely important if you or your children decide to have kids. Might also be worth suggesting to any siblings of yours, as they could carry the genes for it too

hithisiseli52 karma

Absolutely! My husband (Mark) and I know we carry the gene. My sister got tested b/f she had a baby last year and didn't carry it. My daughter will be tested. Absolutely worth bringing up, thank you.

MG8724 karma

Have you thought about how you will bring up the subject of his mortality? Obviously he is too young to understand now, but I am sure that topic is gonna come up sooner rather than later.

hithisiseli77 karma

Great question. Absolutely. Because we talk openly about cystic fibrosis, what it means and staying healthy, I don't envision any kind of "sit down" where we tell him he's got a deadly disease. For now, that is of more concern for me with his sister, Laila, who is 5. She is super in tune with her surroundings, observant, smart and...obsessed with death. Every time she spins a tale, the lead character dies. is "deaded," as she used to say. She likes to talk about my mom being "dead." (My mom died five years ago of cancer when Laila was 6 months old). She wants to know if my mom is in heaven. She wants to know more about the gravemarkers we see on the side of the road. "Are they in heaven? Are they dead? Did someone die there?" She loves the idea of heaven. I'm not even sure if I believe in heaven, but for now, I tell her, "Yes, they are in heaven. My mom's in heaven. One day, that's where we'll all be."

Do I know if it's true? No. Do I know that it's not? No.

There's a lot we don't understand or know.

She's not asked directly about death and Eli's disease, but if she did, we'd be straightforward with her.

The same goes for Eli. I think it will naturally come up as he gets older, and we won't hide anything.

At the same time, our attitude has always been: "Everybody is born. Everybody dies. Life is a terminal condition."

Are you guranteed tomorrow? Am I?


RunFromLife6 karma

Growing up with my brother having cf I was never sat down and straight forwardly told my brother has a deadly disease. I was told cf affected his health, his lungs, and that a lot of people with CF live normal lives.

My brother never wanted to be treated as "the sick kid", so until he left for college he always hid that he had cf and a tube. He doesn't openly tell people he has a tube now, but he doesn't hide it.

Slipdickgreg5 karma

I'm not very familiar with the disease other than I played hockey growing up with a kid who had CF. what is a tube?

hithisiseli6 karma

Hey - no problem- CF kids have problems absorbing fat. SO , many of them have a stomach tube inserted so they can have extra calories. The calorie intake for a teen is so high it's just really hard to meet.

hithisiseli5 karma

That's really interesting. Probably in the next year or two I'll need to talk to Eli's sister, Laila, who is 5, and let her know the specific risks with cystic fibrosis. If she knows that she knows what's at stake when we tell her to wash her hands, cover her mouth, etc. She will know his body is different. But, already, she is protective over him, watching out for him. She gets it. But, we'll emphasize it, too, b/c there's nothing wrong with that. Kids are smart.

Perhapples5 karma

I have gotten into this heavy article up late and on the internet! I'm sure I'm not the only one reading this that doesn't know what to say. It's shockingly hard what you must be feeling. I am so sorry for your struggle. May peace be with you.

hithisiseli9 karma

That's super sweet. Don't feel bad for me! We all have our struggles. If we don't now, we will. That's life! You can't change what you get but you can control how you react. That's all you can do. xo

woodlandLSG235 karma

I honestly wish more people had this mentality. Hiding things like this can cause problems, so you're a wonderful person for being straight forward. I wish you and your family the best of luck.

hithisiseli6 karma

Ha, thanks. I don't know about "wonderful," but I guess I just kinda deal in reality, cuz I'm a journo by trade. I like up-front honesty. I dislike that disease carries a stigma. So, one of the best things we can do in the face of that stigma is just be honest and open and act like it's no big deal, because everyone's 'normal' is different. I agree, though, that hiding things like disease just contributes to feelings like shame, etc., and I don't want that to mess with my little boy. I want him to be proud of who he is, to not be ashamed to take care of himself. #$% whispering about medical issues. We all have our issues!

HIGHclassbroad16 karma

What does his medical treatment consist of?

hithisiseli52 karma

Hi! Thanks for your question. Cystic fibrosis requires a lot of families. That being said, when written out, it looks intimidating. In real life, it is manageable. We do one hour of chest physical therapy a day, which is automated with the help of a shaking vest. (The mucus in his lungs is thick and sticky and must be rattled so he can clear it and avoid infection). Right now he is on his third round of antibiotics for a cough he's had since August. He appears to have kicked it, but colds flare up bacteria that the sticky mucus in his lung traps, which is why even a small cold can cause an infection that leads to the need for antibiotics. He has not had to do inhaled antibiotics yet. Every day, because he is coughing, I have him do a breathing treatment of Albuterol (a bronchiodilator asthmatics also use); then Pulmozyme (a CF-specific medication that breaks up a certain aspect of his mucus issue). Since he's had this cough, we also tried to follow with hypertonic saline - essentially, inhaled salt water. Like, vaped, man. But he hated the taste and it made him thirsty. So for now, it's just albuterol then Pulmozyme. He also takes an ADK vitamin as he has nutrient absorption problems, and additional dose of vitamin D to help w/ lung development. Lastly, before he eats anything, he has an enzyme in applesauce to help him absorb fat. If he didn't take the enzyme, he would starve to death or succumb to a threat to his health stemming from small size. CF kids don't absorb nutrients properly due to the thick sticky mucus throughout their bodies. It blocks the pancreatic ducts from secreting the enzymes that help the rest of us absorb fat. Also, he needs more than 1600 calories a day. AND, we hand wash a lot and try to pay attention to wellness, generally. Healthy tribe = healthy Eli.

HIGHclassbroad19 karma

I knew about the mucus in the lungs but I didn't know about the inability to absorb nutrients. Does the shaking vest take the place of the pounding on the back?

hithisiseli19 karma

Yes - however, there is debate about whether chest physical therapy administered manually is better. Not every country's insurance/government plan will cover the shaking vest. It's worth something like 16K.

HIGHclassbroad6 karma

Holy cow that price. What is your opinion on it with your own child?

hithisiseli57 karma

Insurance covers it and it makes our lives easier. We love it. There is even a cordless version that has come out in recent years. Those in the CF community are raving about it. By "raving," I mean, a friend of mine who got the cordless version was over the moon her daugher coughed up some weird, brown shit at 9 years old. She wondered - 'wow, was that crap just pooled in the bottom of her lungs for the better part of a decade?' This new vest just moved it on out of there! And I'm over here like 'I want my son to cough up weird brown shit! Gotta look into that cordless shaking vest STAT!" We get excited about strange things in the CF community.

jc_cr7 karma

Cystic fibrosis causes a thickening of mucus from a variety of the mucus membranes in the body, not just the lungs

hithisiseli3 karma

True. We are having Eli seen by an ENT for sinus issues tomorrow.

bobcat778112 karma

What is his prognosis in terms of lifespan and physical and cognitive abilities?

hithisiseli28 karma

No impact whatsoever on brain. Lifespan right now is 42 in US (that's the median). 50 in Canada. The disease still kills children and people in their 20s. Sadly, often, US parents don't do the care routine. It's an invisible disease, and not everybody gets that. Education levels in US are low. There are a lot of poor and stressed out families out there, and treating this disease =part-time job. In other parts of the world, like Latin America, the outcomes are MUCH worse, due to both awareness, prioritization by governments and lack of really expensive specialized medicines. Throughout the Latin world, kids die b/t 10 and 15. This disease skews white but we are a melting pot, so it appears everywere, though much less common in minorities.

alters_ego27 karma

I am amazed that the lifespan is now 42. My older sister had CF. This was in the 70's before so much progress had been made. She was the Arkansas poster child for CF. We lost her at age 8 (I was 5).

Good luck to all of you! I have friends in their 40's with CF. I have no doubt that your son will live a long life.

lumpy_grandma18 karma

Every few years it seems to get better and better for CF patients. The disease took my brother at 19, and this was in 2000. Only 15 years later, and he could've had a family of his own :\

hithisiseli10 karma

I'm so sorry for your loss. Sweet guy. Care is changing really fast.

hithisiseli9 karma

Thank you so much. It's hard to look back on the suffering. I hope your sister had a beautiful life. Sweet girl.

cloud_watcher5 karma

I should have known you would already know that, but I was just about to mention it. My best friend is a respiratory therapist that works exclusively with CF patients. She sees patients from the rural part of our state a lot and some of them really bring that median age down.

They don't do the treatments, don't give the medicine, many of them smoke, and will even smoke in the house or car with their CF child right there. It's really unbelievable. It's one thing, I guess, to be too poor and uneducated to do the treatments as much as you should, it's another one to refuse to even go outside to smoke when your kid can't breathe.

Also, my friend goes and sits with a couple of kids in the hospital when she gets off work because their parents don't come! They may stop by a couple of times a week, but they don't stay. And the one in particular I'm thinking about doesn't have a job or anything she just says she doesn't like to. It's heartbreaking.

I wish every child with CF had a parent as involved and invested as you.

hithisiseli3 karma

Indeed - it's really, really sad. Especially the smoking in the car (?!?)

Ilovebooksandcheese12 karma

Im thinking this is a stupid question but is sports out of the question? Or can a panic attack be deadly?

hithisiseli21 karma

Hey! Not a silly question at all! Exercise is vital for every person with cystic fibrosis. I believe it can bump lung function by something like 9 percent (I need to verify that). Right now, Eli gets a lot of exercise through play. When we're stuck indoors, I try to do, which has lots of guided dance and the like. I'm about to sign him up for swimming again. You are supposed to avoid contact sports but I do know of older CFers who have played soccer and football, etc., and been fine. Exercise is one of the bes things a person with CF can do .

MuffinzPlox9 karma

Two of my best friends are twins (one boy, one girl) who both had cf. Ive definitely noticed the intensity can vary. The sister had to go to the hospital frequently throughout her life while the brother only went once or twice a year and he has to have a treatment breather for a couple mins every day. But he has a full life and since middle school he has been able to do everything our group of friends can do from airsoft, atv riding, swimming, shooting, video games. Heck, sometimes he smokes a cigar and looks badass. I really hope he lives a long life with us because he is a great guy. Sadly his sister passed away last week due to unrelated circumstances. My heart goes out to them.

Just know that it can work out. Hopefully we can get rid of cf some day.

hithisiseli8 karma

I'm so so sorry for your loss. Yes, we need to kick this disease's ass. We will.

Slipdickgreg1 karma

I played hockey and soccer with a kid with cf. he played just like everyone else. I assumed at the time the inhaler was for asthma.

hithisiseli2 karma

Those are both great sports for keeping up your lung strength! Great hockey program where we are for littles. Looking into it.

LedZeppelin16028 karma

I have CF. turned 30 this year (thanks to a transplant) not sure what to ask but feel compelled to...

At what age do you think you'll let your Son decide on treatments for himself?

hithisiseli6 karma

That's such a good question. I just have no idea. I guess I'm really interested to hear your insights, being someone who has gone through so much. What age do you think is appropriate? What do you wish your parents did for you / when were you ready to think /decide for yourself on these things. It seems like the 20s would be that era. Even if you messed it up, it would be your body with which to do so, and you may have to mess up to learn. What do you think?

LedZeppelin16026 karma

Well with my situation my mother was very overprotective having lost a son to CF previously. In general I'm very trusting of doctors as I wouldn't be here without them but one thing that affected my life for the worst was having a feeding tube inserted when I was around 12. I needed it for my health but it was at the worst possible time as body image is at its highest through those teen years and as a result my internal illness now had an external sign that I wasn't normal, and everyone especially teens want to fit in so it made me withdraw from friends and activities for fear of pulling it. I think I was probably too young to say no at that point as I did need it but I wish it wasn't at that point and there was more discussion and help for dealing with it

I don't regret my transplant but it came at a time when I had accepted I wasn't going to live much longer and I was depressed a lot around that time so I only really did it for my family's sake which wasn't the best idea as it's taken me quite a few years to adjust to having a future.

So I guess taking charge of treatment isn't really the question more I think it would've been better if everyone was more open like the doctors having psychologists on-hand for dealing with the illness and me and my family being more open with each other

hithisiseli5 karma

Cool - what do you mean by family being more open? But I'm really with you on the mental health aspect. CF impacts more than just the lungs. Mental health is just important as physical health.

Dennisraphael6 karma

Hi! Apart from your current supports (and a cure), would there be something else you wished that doctors/the healthcare system would be able to provide for your child or your family?

P.S. I am very touched by your determination and outlook on Eli's condition. Disease may be an impediment but it can take nothing away from who the person is or the love he/she deserves. As a physician, it is heartening to see families give their all for a loved one.

hithisiseli7 karma

HI! Thanks for the question and the note.

Except for one medicine, Eli has gotten everything he needs. That medicine is called Synagis, and it is a medicine meant to prevent a severe type of cold. Now that he's a little bigger, it's not such a concern, but RSV can kill babies. The American Academy of Pediatrics hasn't approved it for CF. Beyond that, premature infants in Oklahoma are regularly denied this.

Anyway - the biggest struggle we've had, as a a family with two working parents, has been childcare. I wish there was more support for working parents, regarding paid leave after the birth of a child and/or for medical issues. I wish there was more support for middle class people as far as help paying for childcare when a child has special needs. This has been our biggest challenge besides my son's disease as both of us work. Thanks for asking!

OSUCOWBOY11294 karma

Have you looked at Orkambi?

hithisiseli5 karma

Yeah! Right now it's approved for kids 12 and up. It bumps lung function a small percent for people w/ Eli's mutation. However, I still need to look into side effects.

jacksleepshere6 karma

Cystic fibrosis isn't terminal is it? I thought it was just a genetic disorder meaning you generate much more mucus that gets into your lungs.

ImAnAlbatross12 karma

it's not incorrect to say terminal as most people with cystic fibrosis do succumb to the disease at some point, but it is misleading as we typically associate terminal illness with stage 4 cancer diagnoses and the like

hithisiseli13 karma

There is no cure. All people with CF succumb to the disease unless something else kills them first. It's jarring to say and worse to think, especially about a child. If you're going by straight definition, "terminal" is accurate. But I see your point, because the nuance to the term does bring something like terminal cancer to mind. It's a fatal illness, though. There is no getting around it. Kids still die. My hope is that Eli, and every child, will get to grow up.

hithisiseli9 karma

It is deadly, terminal, fatal and every other synonym. People are living longer, but 70 years ago, when it first got a name, it killed by 2. Forty years ago, 15. Today, the median age of US survival is 42, though it kills kids elsewhere in the world much faster. It also often kills people in their 20s, which makes in particularly tragic. Of course, we hope for the best. We hope for a cure. "Life-threatening" is the polite term, but I stopped being polite long ago!

Call_Me_Carl5 karma

What can your child be expected to endure or experience more towards the end of his life? Will he be able to "prepare" for the inevitable? Or is he expected to meet a more sudden end?

hithisiseli12 karma

To your first question: I don't know. Second: No idea.

As far as endings go, I've read and/or discussed the end of CFers life in "Alex: Life of a Child" (Frank Deford, his daughter died in1980); "65 Red Roses," a 2009 film about a woman with CF awaiting a lung transplant; And in my own essay about a Chilean schoolgirl w/ CF who begged to die. For the nitty gritty, you can seek those out on your own.

Since Eli could outlive me, I don't think that far ahead right now.

Im_mrs_do_you_right5 karma

I watched 65 Red Roses and bawled my eyes out.

hithisiseli6 karma

I rented it (from the library) and returned it w/o watching it. But, at a fundraiser, the end was played. That's when my husband and I got sloppy sad. This isn't just someone else's random suffering to us. It's hard to witness. We can't detatch.

Call_Me_Carl5 karma

What a wonderfully worded answer! Thanks for the response!

hithisiseli10 karma

I'd go into more detail but it honestly makes me too sad. The book and movie made me cry the deepest, darkest, ugliest cries you ever did see. I actively avoid those scenarios. But, I also don't know how medicine will look, even next year, so I can't apply those to my own son!

sicMagOtt154 karma

Hi there! Reading these comments is extremely inspirational and I cant thank you enough for writing about this. You say that Eli and dealing with CF has brought positivity to your life, could you elaborate on this? Again, thank you for what you've been writing!

hithisiseli14 karma

Thanks for reading, asking a question, and thanks for being kind. Basically, Eli has a disease, and though we try not to dwell on it, it is a fatal illness. Day to day, it's not something we focus on, the fatal part. Because, Eli is so alive, happy and well. He has a huge smile, a cheeky personality, a love of trucks and choo choos. He is not his disease. So those happy moments are what I try to dwell on, not the sad moments, when I worry that I'm not doing enough for him, or I learn about something new that might hurt my son because of the way his body works. I sing to Eli at night.Eli demands a concert - every night. His favorite song is "Favorite Things" from 'Sound of Music.' I give him a full concert. (hey, he's the only person who likes to hear me sing!). It's one of the favorite parts of my day. When I drive my daughter to school, I ask her what she thinks she'll like most about her day. When she's home at night, I want to know what the best part of her day was. We play Nappy Roots "Good Day" to and from school. I mean, I'm quite cheesy about all this positivity. I think I've realized happiness is a muscle you have to work out. I've become protective of it, too, my family's happiness. I want Eli - all of us - to have a good life.

Chronically_cute3 karma

Whoa! I have Cystic Fibrosis too!

How does it make you feel knowing were so close to a cure? Also, what do you struggle with most as a mom with a kid who has CF? Having CF myself, I try not to think of what my mom is going through. It honestly makes me too sad.

hithisiseli14 karma

I am incredibly happy knowing that we are so close to a cure.

As far as what I struggle with - To be honest, sometimes there is a sadness that settles into my chest. I can feel it. It's hard to describe. It's not depression, because it doesn't stay. To borrow descriptions from other people who feel sadness and/or depression: It's like a big gray bean bag that sits on my head. It's like feeling homesick, but you are home. I dunno. It's hearbreak - a pit in my chest. Please, be assured, I, I visit, but I don't linger there. I visit when I see the struggles of others. But - I want to understand the struggles of others, so I can be prepared/aware for what my son faces. It's such a double-edged sword. I don't linger there- in sadness - b/c my son is wonderful. Life is beautiful. And friends, family, happiness, etc., pull me out of that sadness. I would be knocking down a doctor/therapist's door if I lingered there for more than - I dunno - three days?! I'm really in tune w/ my mental health. Don't feel sad to think about your mom's feelings. It's just - love is such a deep, deep emotion, and pain is part of love. It just is. Nobody's fault. When Eli needed surgery right after he was born it opened up parts of my heart that I didn't even know existed. Those feelings - the capacity to feel - it makes us stronger, better, somehow. All the best to you!

_Thom203 karma

How did you react when your child was diagnosed?

hithisiseli22 karma

Dissolved into pile of tears and snot. Grassy green field I'd pictured just got burned. But real-life Eli replaced it, and a different life has proven to be just as good. Maybe even better. Yes, better.

heresyourfuture3 karma

Do you know what specific mutation Eli carries on his CFTR gene? Different mutations require different strategies to help CFTR do its job. There's some great research going on and therapeutics in later phase clinical trials that may be really helpful to large subsets of CF patients.

hithisiseli6 karma

Hi! Yes, he carries two mutations of the most common type of cf, the Delta F508. In a way, it's great, because so much research is directed there. We are so hopeful and thankful that the reserach is ongoing and could help Eli.

heresyourfuture3 karma

Thanks for your reply - you're probably aware of this already, but Vertex has a combination therapeutic in phase III trials right now that's showing early promise in dF508 patients. It's pretty exciting to see how the field has progressed over the last few decades. I'm hoping we see CF become long-term manageable for most cases (if not curable) in our lifetimes.

hithisiseli4 karma

Me too. Thanks!

RunFromLife3 karma

I'm 18, and my older brother is 26 with CF. I've grown up not understanding what the disease does. Until about 2 years ago I was clueless the life expectancy (at the time) was 28-30. I was heart broken, and torn to pieces. My brother is my life, and if I lost him I'd be shattered. The only thing that has pushed me through is Jesus Christ. My brother has been out of the hospital for a while now, and is extremely healthy. God is doing work in our lives, and with out him my brothers life would have been extremely difficult. Even now it is difficult, but it's better then we could have hoped.

CF is one of the worst things. The thought the my brothers lungs are killing him makes me not want to leave his side, but knowing God is fighting the battle for him makes everything okay.

How are you accepting everything? And couping?

hithisiseli6 karma

I think, first of all, that your faith is beautiful.

Now is the part I admit I'm not a "God's Plan" type of gal.

This isn't a knock to your faith - it's just who I am.

I'm open to the mysteries of the universe/life - don't get me wrong.

Anyway - the initial shock of my son's diagnosis did a number on me. But me, my husband - we've come back a lot stronger than we were before. I've become a big advocate for Eli, my daughter, and everyone w/ CF and rare disease.

I'm growing a lot b/c of my son.

scarfinati2 karma

What are some things you wish were available or invented for kids with CF within reason obviously?

hithisiseli5 karma

Hi! Thanks for your question. OK - besides a cure (obvs) - actually, the best thing for me over the last three years would have been more support for family leave and childcare. There's just nothing for middle class people. My son's childcare situation has stressed me out almost as much as his disease these last three years. We're finally in a good situation - it's been tough. The toughest! I wish it were easier for working-parent families in the U.S., especially those who have a child w/ special needs.

cats_lie2 karma

I have CF 24M and can say that medicine has come a long ways. Just be proactive with your treatments and put your kid into sports to stay active. also what mutation does he have?

hithisiseli2 karma

Delta F508. And roger that on the sports. Right now his participation is limited due to age, but I plan to re-enroll him in swimming on the double.

unsavoryginger2 karma

I'm sorry to hear about your son's diagnosis, I wish the best for you both.

I just have one question, was CF passed down to him? If so, were you aware of the possibilities of passing it down to a child? How does one get CF? Just looking to get educated.

hithisiseli5 karma

No problem. Neither my husband nor I were aware we carried a gene for cystic fibrosis. Neither of our families had history of anyone dying from CF or having CF. Apparently, it is a recessive trait. We each have it. With every child we have, there is a 1/4 change the child will have CF. Eli has an older sister Laila who does not have CF. We haven't had her tested for the gene yet, but we will.

unsavoryginger2 karma

Last question. I've also heard that it isn't recommended that couples who both carry the CF gene to have children.

Is this at all true? I think it's ridiculous, especially if there's only 25% chance of having a child with CF.

hithisiseli2 karma

I haven't heard that. Reproductive choice when there is a genetic disease in the mix is way personal. I'd think it's up to any couple. We happened not to know we were both carriers before we had Eli. Had I known I would have still had Eli. However, people seeking genetic counseling sometimes receive outdated information. Also, doctors sometimes give bad information to parents of a newly diagnosed baby. A couple I know was told information about 20 years old right after the diagnosis of their baby! Twenty years ago, outcomes were tougher, so as you can imagine, that's devastating. Not everyone in the medical profession keeps up to speed, so it's important to research the disease, (NOT ON INTERNET MESSAGE BOARDS). Talk to other parents, etc., with your concerns.

Porridgeandpeas2 karma

My brother was diagnosed with CF when he was 16 (rare mutation where it doesn't affect pancreatic function too much, so he is okay for now). My mother, of course is supportive and does everything she can, she has said though that she wishes she never found out what it was because now she knows he is faced with a life limiting illness where before he was 'just sick'. Do you think it would be better not knowing?

Side note, not a question but it bothers me that we live in a country with the highest rate of CF patients and he was diagnosed so late despite presenting with symptoms.

hithisiseli2 karma

Hey! Thanks for your thoughtful response and for the question. I don't think it would be better not knowing. With understanding, you can be the best advocate for your kid that you can be. However - I can feel your ma's pain, because I'm not emotionally detatched from facts when it comes to my son's disease. Learning new stuff is actually painful for me sometimes. I'm connected to a lot of CFers on Instagram, blogs, etc. There have been Instagram pals that have died young -20s- in the three years since I've had Eli. It makes me sad, but at the same time, understanding what others face is useful as you know what to look for/ask/etc. I find it really helpful to communicate with other moms&dads of CF tots, since those parents' experiences / challenges are more closely related to ours right now.

2booshie1012 karma

Does having a child who is so dependent on you make you worry about your own mortality?

hithisiseli2 karma

Yes. I do my best on basic wellness-exercise, fruits and veggies, drinking water, keeping up on mental and physical health.

sinni8002 karma

Hey, I have Cystic Fibrosis too and I am 24 by now.

Something important but you might not know yet: How "strong" is the condition for him, can they say anything for now? I have been hit pretty mild because I can still breathe normally (except when running, I run out easily)

For us we were never emotional about the whole ordeal. I do my meds and I never really were sad about it.

Please, one important thing: Be happy. It really changes the future way of dealing with it. Deal with it in a good mood, because that's all you can do.

A good mood can and will give the body power to fight back pseudomonads and all that shitty crap better.

Pseudomonads are gonna give his body the most issues in my experience. Fight them back, fight them back early, fight them back with VARYING medicine (there are four different ones i've been treated with... one is tobramycin, the others... I forgot, they had similarly weird names). Vary, because pseudomonads will become immune to one thing in a matter of time.

Pancreas medicine... My parents used the trick of mixing it into my food... They are usually little pills filled with pellets, the pellets can be ingested directly. Mix them into food :)

hithisiseli2 karma

Great advice. We are happy every day. Definitely no time to sit around crying.

Ptomb1 karma

The only things I know about cystic fibrosis I learned from the 1997 movie "Sick: The Life and Death of Bob Flanagan, Supermasochist." Have you seen this movie and if so, would you say that it is representative of the CF community in how CF patients deal with pain/pleasure?

hithisiseli1 karma

I can't comment b/c I've not seen it. I only know stuff has changed a lot in recent years, so it's probably not representative of the state of medicine now. But I'll be sure to check it out.