Adult with CF - when/what/how to tell my kids?

[SooB]

Hi. I was diagnosed before starting a family and my partner was screened negative for cf. That means my two kids are carriers. I am generally well (no hospitalisation, no meds for years) and have never talked about having cf with my kids. They are now 15 and 11 and for a while I've been feeling a bit of a fraud for not having told them, also as they get older the need to tell them about what being a carrier means gets more important.

I wondered if anyone had already gone through this and had any wisdom to share about how this might make my kids feel. It has come to light as my daughter (15) is studying genetics at school, and it feels wrong to not raise it. That said, I don't want to terrify them about my health or make them worry overly about their future parenting prospects. Hoping someone will tell me I'm overthinking this and I shouldn't worry. They are both sensible kids who I can explain things to.

 

[Melissa75]

Wow, I'm guessing you don't cough all the time? When my kids were little, that was how they found me in the house.

It doesn't sound like you have been lying to them e.g. "I am just going to the hospital for a routine procedure" or "I take this med for asthma." Even if you did, I don't think you should feel bad or like a fraud. Parenting decisions are hard!

If you give them the context for why you haven't said anything, that should help them not get overly scared. Something along the lines of:

I don't have a typical presentation of CF. If I did, I would have a lot of medications, hospitalizations and more. This is why I didn't share the situation with you, because its impact on my life has been minimal. There are people in their 70s with CF and I anticipate being one of them. I am telling you because you are carriers and someday this will be relevant when you have kids. Your partner will need to be tested. But don't be worried. Not that many people are carriers. Here are the stats for various races/ethnic groups. https://www.ucsfhealth.org/education/carrier_testing_for_cystic_fibrosis/

Good luck! Let us know how it goes.

 

[jricci]

Great question SooB and great answer by Melissa : )
I actually wrote an article for my clinic's newsletter some years back concerning this issue. SooB, this wouldn’t be as relevant to you since you and your family aren’t as impacted by CF. But I think it’s an important issue that needs to be addressed and often it's overlooked until it's too late (as I learned the hard way)

Here’s the article I wrote back in 2013:

My CF has shaped my children's lives for as long as they can remember. As toddlers, they developed patience. After many temper tantrums, they eventually learned that I had to finish my treatments before we embarked on our day's adventures. As preschoolers they learned empathy. They would run to get me tissues and water if I was having a coughing attack. Their teachers would comment on the level of empathy they showed their classmates and how unusual it was at such a young age. During their school years, they are learning to become more independent. Just last week, my older daughter surprised me by making their lunches and helping her sister with her homework because she knew I was not feeling well.

Since CF had been woven into their everyday lives, I took their comfort level with my CF for granted. They knew that CF meant coughing, treatments, and IVs. What they did not know was that it is a life-threatening disease. I regret having not broached this subject with them as soon as I felt they were mature enough to handle this information. Last year my daughter's teacher called to tell me that he was concerned about her. She was not herself at school. She was very distracted and seemed depressed. I knew immediately what was bothering her. I was on IVs because of an especially difficult exacerbation. The side effects of the antibiotics left me on the couch most of the day. It was the sickest she had ever seen me. We had a long discussion after the phone call and she opened up to me and told me how she had read something at the CF walk that stated that the life expectancy of someone with CF was 37 years old. I was 40. It truly broke my heart to know that I was not there to answer her questions and ease her fears when she read this critical piece of information. She deserved to hear this from me. As I spoke to her, I tried to give her realistic but hopeful answers. I told her that I wanted nothing more than to watch her and her sister grow up and that I hoped to be there for her college graduation, her wedding, and the birth of her children. I explained that there are so many new medicines being discovered that would help to make this a possibility, but I also told her that there are no guarantees, and that is why I try so hard to keep myself as healthy as possible.

Just as my children have always incorporated important life lessons from my illness into their lives, I believe that this discussion will only further strengthen their character. It will teach them the value of treating every day as a gift. I hope my experience encourages other parents with CF to be prepared to have this pivotal discussion with heir children. No matter how difficult it may seem, it will be worth the peace of mind knowing that you will be the one delivering this information, ready to help them cope with their fears.

Here are some guidelines from Lisa C. Greene, a mom of two children with CF and co-author with Foster Cline,M.D. of the book Parenting Children with Health Issues (www.PCWHI.com)
• Pivotal parenting moments can take us by surprise, so be prepared ahead of time. Our answers should be honest, calm, matter-of-fact, and hopeful. We shouldn't use terms like "fatal" or "life-shortening" nor should we make empty promises. Use terms like “healthier” rather than “healthy,” “more likely to live a long time” rather than “will live a long time.”
• We need to try our best not to let our own fears and worries show, both in our words and in our body language. Children pick up on (and tend to mirror) their parents' emotional cues, especially when they are young. If you are having trouble controlling your own emotions about these tough issues, counseling might be helpful.
• At some point we do need to address the issue of life expectancy. Hopefully this will be clarified by around the age of eight (around 3rd grade) depending on the maturity of your child. One way to address this issue is to askyour child questions to open up dialogue. Some examples are:
“How much do you know about CF?”
“How are you handling it?”
"Is there anything about CF that worries you?"
With a little awareness and preparation, you can make talking about these difficult issues a positive experience. Relationships can grow closer when people go through tough times together.

 

[jricci]

Forgot to mention that CF was brought up in my daughter's science class in 7th grade. The teacher painted a pretty bleak picture of CF (really out of date information- people diagnosed usually not living into adulthood, etc.) Although, she knew that the information on CF was out of date, she again wasn't prepared for information she was given as far as the science of it all and was surprised and very upset by the fact that she was definitely a carrier. When we had the "CF talk" as I described in my article, I didn't go into details about the genetics of it. Again, I felt like an awful mother : (
This parenting thing is a learning process, that's for sure! Unfortuanely for our children, it's a "learning as we go" process.
That's the great thing about this forum. We can learn from other's mistakes.

 

[SooB]

Thank you so much Melissa75 and jricci for your replies - you both give great information. Melissa75: that's exactly the kind of line I was going to take, but my husband is worried about how this will affect their view of themselves, as they become adults. I think we are a long way off (if I can be blunt) accidental or planned pregnancies, but I want to be able to prepare them for something that will - at the right point in time - be something they have to consider. I feel huge guilt about this - the burden I'm saddling them with. I am so lucky: this condition barely affects my life, (I used to cough all the time, but asthma medication and a move to a warmer climate put me at just-about-not-affected) but it could seriously impact their lives in the future. jricci: it is exactly this sort of old-fashioned doom-mongering that I want to nip in the bud. My kids aren't daft - they know I have something more than asthma, it's just a question of managing their fear and setting them up to deal with this with future partners.

I need to reflect further. More views from this forum will be hugely welcome.

 

[LittleLab4CF]

SooB,

I don't think I can add anything to the when and how you might tell your kids. The previous contributors have answered well. On another topic a contributor recently asked why there were so many old CFers on the site. This is a question I have pondered and at some level, researched scientific literature for an answer. A lot comes from the old CFers stories on this forum, many of them diagnosed late in relative terms. I have a personal interest in the progression of their CF and it might be a consideration in telling your children.

An unusual CFTR mutation or possibly the mutation and a T polymorphism known to work very much like a mutation has passed from my grandmother to my father to me. I'm making some suppositions, but bear with me, my point is coming.

My grandmother died at age 45, from pancreatic/liver cancer in 1944. This was years before my birth, and there's a lot I don't know in terms of her health in the years leading up to her death. The lack of stories leads me to believe that she wasn't ill for a long period before her death.

My father was a healthy vibrant man. He was a force of nature, self made man or whatever quality that genuinely separates the ordinary from the extraordinary of out lot. His energy and enthusiasm for personal and professional growth was a hard example to emulate. In 1962 he was hospitalized with a hemorrhaging ulcer. I was 12, he was 41 and the first hint that I had that he was anything other than fit. After his recovery, certain changes to his diet and such were taken in stride. He treated his ulcer as an annoyance he had to comply with.

In truth, much more was going on. His pancreas was beginning to atrophy, he had Crone's disease, IBS, what we call GERD in addition to the diagnosed ulcers. For some reason it all turned on hard when he was ~45-46 years old. He died of pneumonia complicated by malnutrition at 48.

I was the medical miniature of my father beginning around the time when his ulcer incident. I too treated my GI issues as annoyances. It wasn't fun, in fact I was going through the process many CFers share. I was also wired to a higher voltage, much like my father and powered through nausea and the undiagnosed malabsorption/malnutrition. I was blown on life and nothing was going to deny me from full engagement. Like my father and his mother, CF wasn't even considered until a Boston pancreatologist suggested that my pancreatitis might be from undiagnosed CF

Nonetheless I have to be hyperactive, there's no other explanation. I was forever burning the candle at both ends. I had virtually endless energy and I put it to good use. From the accomplishments I've read about other CFers, I wonder if this is a kind of side effect. Stress is a funny thing. It helps us fight the problems that CF inflicts on us but if an excess is produced, we not only fight the disease better, our brains and unaffected systems are gnashing at the bit. The collective burnout of the endocrine system is a possibility of why we seem to hit a wall, or several, marking the decline from CF.

The post operative report on my father didn't make any conclusions, the cause of death didn't change. My sister was able to get a copy of hospital records and the autopsy report was very helpful once I had a CF diagnosis. My doctor surmised that the actual disease at play was CF, total atrophy of his pancreas, characteristic scarring in his lungs and pseudocysts throughout the kidneys added to failure to thrive was adequate evidence. My late diagnosis and a grandmother dying from a cancer common in CF, all put out of commission at the same time, more or less. I don't know about my grandmother but my father and I appeared to have a sudden and unexpected deterioration, his was fatal and mine easily could have been.

For me the idea that there's a kind of genetic timer is an interesting thought. What I do know is the process and progress of CF marches inexorably within us, stealing from our total lifetime count of heart beats. I easily could have described my health as you have. In fact you have been able to keep your condition private and apparently from your kids.

Whether it's gene mutations turning on hard at a certain time or the natural progression of CF but I am saying that at some time in the future, your CF may suddenly and unexpectedly get much worse. We speak of flares and tune ups, and rather amazingly most CFers sound like you describe it. Not everyone is as sick and many are going to in the group awaiting DLTs or liver transplants and really might die before 37.

I believe that for whatever reason, CF waxes and wanes, we tend to get sick and rebound quickly. At least for a period of time. Just like an exciting event like a pregnancy or a death in the family, can activate the genetics of type I diabetes, or bipolar disorder and even schizophrenia, something is telling the CF mutation set to become active in more cells or something like that.

This is scientifically sketchy at best but as a geneticist I do thought experiments. I consider why some CFers are very sick at birth and others won't have any problems until years later, or never. Does this mean that genes turn on at a certain time. Siblings, even twins with identical CFTR mutation sets range from the expected similar presentation to one sibling becoming very ill and the other remains asymptomatic.

I believe the answer is found in something called disease penetration. Some mutations are famous for causing GI dominant CF, attacking the pancreas, bile/liver ducts and everything below the diaphragm. Likewise some of the most common mutation sets attack the pulmonary system. And most all mutation sets can penetrate all the wet surfaces, anywhere mucus cells are to be found, the worst case. So what makes this difference? More genetics is in play than just CFTR mutations. It has to be.

The unknown source of genes that determine how severe and when CF changes from annoying to disabling are probably the genes that determine the amount and location of penetration. As you think about the ongoing discussion after you tell your children of your diagnosis, keep in mind that someday CF could or likely will catch up with you. If you suffered maldigestion/malabsorption the consequences of it will show up someday in the form of osteopenia, arthritis, spontaneous compression fractures in the spine and such.

As carriers they might have mild CF issues, mild for CF but drastic for an otherwise healthy person. Sinusitis, maldigestion due to intestinal mucus and the sinusitis can raise the frequency and intensity of chest infections. For example, I don't have CF lungs but somebody unaware of my CF could accuse me of being a closet smoker from my X-rays.

I'm sure everyone will be fine with the new information but it is just the beginning. The questions and possibly fears will crop up in a day or a month or year so be sure to check on their heads and hearts from time to time.

LL