Your Smile for the Day

When Holly was a kiddo living at home, she used inhaled antibiotics on a fairly regular basis.This time each aerosol required two vials of the antibiotic, which had to be drawn up with a syringe then squirted into the nebulizer. Now, keep in mind that each vial cost about $25 even with insurance. Well, I drew up the first vial and squirted it in, then I drew up the second, and as I squirted it into the neb, I knocked the neb over spilling the contents of both vials. Fifty bucks down the drain. So, back I went to get two more vials. Again I drew up the contents of the first vial and squirted it into the nebulizer. Then I drew up the second vial, and as I started to squirt it in the nebulizer, the syringe slipped out of my hand. Then, time slowed, and everything moved in slow motion as the syringe went tumbling . . . tumbling. . . tumbling . . . stuck in my thigh and just hung there. I stood there staring at it, and my then husband stood there staring at it. "Shit," I said in a matter of fact way just as it fell to the floor. My husband collapsed. I mean it. He collapsed right on the floor laughing. I never swore. I truly never swore, so when I did, he found it absolutely hilarious. Forget the fact that I had now wasted $75. It was worth the comedy relief. I think I let him handle filling the nebulizer after that.

So, the next time you think you've screwed something, anything, up. Just think of me with that syringe dangling from my thigh and reassure yourself that not much can be any dumber than that.

Quality Versus Quantity: CFF's New Regs

The recent changes in regulations set by the CF Foundation regarding contact between people with CF have caused tremendous controversy(http://www.cff.org/UploadedFiles/LivingWithCF/StayingHealthy/Germs/WhatYouShouldKnow/Lung-Health-What-You-Should-Know-About-Germs.pdf). Most of the parents and adult children, who grew up before these rules were instituted, vehemently oppose them. Our children grew up going to CF summer camps where they did group treatments with the kids doing aerosols and CPT (pre-vest) together. They coughed all over each other. Bowls of various types of enzymes were set out on the tables where they ate and the kids grabbed the brand and number they needed and swallowed them down.

At CF clinic, the kids played, talked and shared together as the parents discussed various CF topics and how they handled them. Every summer, my husband and I hosted a party for the CF families in our area. We had a picnic, and the kids, parents and doctors all swam together, using the huge home I.V. syringes as squirt guns. The doctors perfected the art of the two-shooter, surfacing from underwater with both hands gripping syringes loaded and aiming at whomever was closest. We all bonded during those times. The kids felt closer to the other kids and learned to see their docs as real people. The parents took advantage of the opportunity to talk over questions with one another and compare strategies on getting kiddos to do treatments or take their enzymes. And we laughed. Perhaps the laughing was the best and most healing aspect of those camps and summer gatherings. The kids laughed, and moms and dads laughed. Science has proven the healing value of laughter, and these get-togethers created opportunities for ample giggles, chuckles and guffaws. Those times served as a way to lighten the heaviness of thoughts and feelings associated with CF. We all needed that. We still do.

When Holly was diagnosed at seven-and-a-half months, one of the first pieces of advice her CF specialist gave us was to always choose quality over quantity. This was in the late 70s when the average life expectancy was only about 18, and he said, “I’d rather see her live 18 really good, fulfilling years than 21 sheltered, lonely years.” Those words guided our care of her throughout her life, and she has lived well because of it. Yes, she has limitations, but she has really lived and loved without regret. She has lost loved friends who had CF, but she has also celebrated the friendship and kinship she has felt honored to share with them.

She can tell you many colorful stories about her time with her CF friends in the hospital: like rollerblading down the hallways with a friend. When they were reprimanded by a security guard, they simply moved to the parking lot. Not to mention the time she snuck a buddy out to Denny’s. That was the night we got a call in the middle of the night being questioned by the nurses who wondered if we knew where our daughter was. Ahhhhh. I’ve always said that, “she may survive CF, but I’m pretty sure it’s going to kill me!”

She has lived life her way and been better for it. She believes, as most of us whose children grew up in the era before the strict new regulations, do that the value of the kinship and camaraderie that results from time spent with others with CF far outweighs any risks that their time together might bring. Every parent and adult with CF has to make his or her own decisions about contact with others with CF, and the Foundation has imposed certain rules that must be followed at CF events that, I believe, are to the detriment of the people who should benefit the most from those events.

Some people, like Holly, will continue to meet for lunch and visit, wearing masks, in each other’s hospital rooms. They value their time together and the encouragement they gain from it too much to be overly concerned about cross-contamination.

As I said, everyone must make his or her own decision on the subject. And, as Holly would say, “Thus endeth the rant.”

The Event: CF Bathroom Observations

Just a little CF bathroom humor this morning. I was thinking of what a friend of mine said the other day when he returned after an extended period in the bathroom. Not long after, I headed for the same destination. He hung his head, chuckled and advised me to avoid the area for as long as possible. Apparently, as he reported, had just suffered "An Event." I chose to use their other potty!

It reminded me of the issues that every CF family experiences. When Holly was diagnosed and in the hospital, even the nurses held their noses when they came in our room as they insisted on examining each diaper. I advocate hazardous duty pay for anyone who's assigned that job.

When we started a parents' support group, we all laughed at the gross topics that became common in our discussions. We were surprised when subjects normally considered inappropriate in polite company became common in our midst. We never expected to discuss our children's poop: floaters, sinkers or just a film of fat coating the surface of the water. But we learned that poop provided a great deal of important information we needed to pay attention to.

Thinking about the unique digestive issues that people with CF experienced caused my, admittedly, a little off-kilter mind to consider solutions to these frequent bathroom Events. I came up with, what I think, is a brilliant solution.

The ideal CF bathroom would open with an airlock door and contain a television right in front of the toilet, complete with surround sound and a stereo for the occupant's entertainment. Of course, a library filled with favorite books would be close by as well.

Now I got the next idea from observing the precautions hospitals take for extremely contagious diseases. The bathroom would be vented outside as fresh air is pumped in. An automatic fragrance dispenser would spritz a gentle fresh scent as needed.

Of course, rather than the usual toilet, this one would have a padded seat and back, complete with head and arm rests for the occupant's comfort. It would be fitted with jets that that would very gently spritz warm water at the touch of a button, similar to a bidet. A discreet container matching the room's decor would hold soothing creams or gels to use after the completion of An Event.

The door to the room would boast a carefully calligraphed sign saying: "Enter" or "Enter at your own risk," and CF families would live happily and odor-free ever after.

5 Ways to Maintain a Healthy Relationship With Your Adult Child

When our children are babies, we learn to cope with one set of difficulties, such as how to get them to take their enzymes without burning their mouths, ensuring they swallow the applesauce, or whatever medium you use before the enzymes digest it. And then there’s the challenge of finding a way to entertain those little ones, so they cooperate with treatments when all they want is to get down to crawl and play or toddle around.

Then they grow, and as they become adolescents, we plead, we cajole, we fight, we do everything we can to entice them to sit down and do their aerosols and chest physiotherapy (or pounding, as we called it) or the Vest. We beg them to “just say no” to cigarettes and so many of the other things that teenagers often experiment with. We do all this, because we love them, and we know the work, the commitment, the sacrifices made are worth it to help them grow up to live out their dreams, while all they want to do is just be “normal,” which, of course, to them means no pills, no treatments.

Then, for those of us parents who are blessed to have our children reach adulthood, we face a whole new set of problems that many of us find ourselves unprepared for. Now they’re independent. They answer to us no more. We’re thrilled as we watch them do what we’ve prepared them for. Perhaps they go to college, maybe they get jobs and live out of reach of our daily supervision. At this point, their independence dictates that they make the choices about their health care. We discipline ourselves to avoid calling every day to check up on them. Then, some of them marry. When that happens, their relationship with us changes for good. Now, someone else replaces us as caretaker. That’s right and healthy and good. But it isn’t necessarily easy for those of us who have, until then, been their primary caregivers. We restrain ourselves from jumping in when we’re sure we know best. We resist the urge to “check in” frequently.

Knowing that we must make that adjustment in our thinking and our behavior and actually doing it are two very different things. Everyone who has children experiences this to a certain point when their nest empties. However, those of us who have committed our lives to caring for our children who cannot be healthy and, quite honestly, might not survive without our time and attention, undergo a more significant loss and experience greater anxiety.

So how do we deal with this? I have been forced into a serious change of behavior by being told in no uncertain terms that I am not allowed to ask about Holly’s health. Ever. At all. That’s hard for any loving mother, but if I am to love her the way she needs to be loved, that’s what I do. Not easy, but worth it to maintain the relationship.

Perhaps if you, as moms of children/adults with CF, realize what independence means to them and take appropriate action from the beginning, you can avoid the extreme, painful and anxiety-provoking consequenses I’ve undergone. How do you do that? Well, obviously, I’m not exactly an expert at it, but here are my suggestions:

1.     When your child leaves home, be sure to talk about her activities, her interests, her friends, whether she’s enjoying her life and other questions that parents of healthy children would ask them. Avoid questions about her health.

2.     Let your child take the lead in discussions about health. If he wants to talk about it, he will. And, he will appreciate the trust you’re demonstrating in his ability to be an adult and take care of himself.

3.     Don’t automatically assume that you’ll go to clinic visits with your child just because you always have. Wait to be invited.

4.     When your child needs you, be there unconditionally. Don’t shotgun her with questions about what she’s been doing to care for herself and immediately tell her what needs to change. Ask questions only occasionally and offer suggestions only if asked. Let your intimate knowledge of your child and your intuition guide your behavior.

5.     Let his CF specialist be the advice-giver. It will go down much easier from an objective party than from mom. Resist the urge to intervene.

This major change in your relationship to your child will not be easily navigated. You will make mistakes. However, mistakes present an opportunity for your relationship with your adult child to grow and improve as you demonstrate trust and open a dialog about your fears and your child’s need for independence.

And last, develop a tough hide and hang in there. It’s worth it in order to maintain a close relationship with your adult child.

The Starting Gun

Today is December 12, 2012,  the thirty-third anniversary of Holly's diagnosis. In 1979, it was the day before her seven month birthday. Every year since then I have grieved as I remembered the devastation of learning that Holly had cystic fibrosis. This year, however, I have decided to focus on the positive ramifications of that diagnosis.

There's no denying that learning my daughter had a chronic and fatal disease was unspeakably painful, the news was also, in a way, a relief. Now we had a name for the cause of the awful problems she'd experienced, and, with that name, came the knowledge of how to help her.

For seven months, my baby girl had struggled to gain weight and suffered with excruciating stomach cramps. She could only sleep at night if I lay on my back on the sofa with a hot towel between our tummies to ease the pain.

Holly was a beautiful baby from the time she was born. At the nursery window in the hospital, her daddy's friend had to make a way for him through the group of people crowded around in front of her bassinet to admire this lovely little girl. However, some days her healthy pink color would change abruptly to a deathly gray, which would snatch my breath away.

Most babies have a sweet scent that adults love. I noticed very early on that Holly didn't smell sweet at all even immediately after a bath. For some odd reason, that little detail made me very sad. Perhaps, it forced me to face the fact that something really was wrong with my precious baby girl. I also observed that she tasted salty when I kissed her, and, when she cried, her tears left tracks of salt behind on her cheeks. Unfortunately, I was unaware of the significance of that one fact, so I told family members but never mentioned it to the pediatrician.

At seven months old, she had only recently begun to eat more than rice cereal. However, because her body didn't produce the enzymes necessary to produce her food, no matter how much she ate, her little tummy never felt full. The amount of food she consumed was astonishing considering her age. No matter how much I fed her, she always cried for more. It had gotten to the point that her plate of food equaled mine, and she still didn't gain weight. When I fed her eight ounces of formula, she would scream for more. But, I quickly learned that if I gave her more, she threw it up, because it was too much for her little tummy. She was malnourished and weak.

Finally, on Dec. 12, my big sister called and forced herself to overcome her fears of offending me by telling me that I must call Holly's pediatrician and insist on a sweat test. God's perfect timing made me open to what she had to say; I had read an article the day before mentioning that the taste of salt on babies often indicated a disease I knew little about called cystic fibrosis. She instructed me to hang up from talking to her and immediately call my doctor. As we talked, he started in with his usual arguments until I interrupted him, saying, "But she tastes salty." He responded, "Oh, my god. I'll call Children's Medical Center immediately to set up a sweat test." I wrote about that day in my previous blog post.

Though the diagnosis was devastating, the good news was: now we knew there were treatments to help her. The lung disease, which had not caused problems yet, could be slowed. What a relief we experienced when we learned that, by using pre-digested formula and artificial enzymes to digest her food, the awful stomach cramps would stop immediately! She would gain weight!

Our guilt and frustration over our inability to help our daughter were instantly relieved with the knowledge that we could help her. Yes, we were forced to face the long-term realities of CF, but part of the reality was that the treatments available were going to make her better.

And they did. At that time, when children were diagnosed with CF, they were immediately admitted to the hospital. Actually, we were told that the whole family was being admitted in order to learn about CF and how to care for her. The nurse brought Holly's first bottle of pre-digested formula and told me that it smelled and tasted awful. I smelled it and decided to take her word on the taste. She said that Holly probably would refuse it until she figured out she wasn't getting anything else. She was wrong. I took Holly in my arms, offered her the bottle and, instead of refusing it, she sucked it down, hardly breathing, because she was so hungry. When she finished the bottle and I set it down on the counter, she smiled! The nurse was as incredulous as I. Holly smiled at the bottle, because, for the first time her seven-month-old life, her tummy was full. My heart burst with happiness and relief.

In the first month after her diagnosis, Holly gained two pounds. Two pounds! She gained energy. Our nights on the sofa with the hot towel ended. Now, we could put her down to sleep in her crib with a full tummy.

Of course, we struggled with the realities that the average life expectancy was only 18 and the problems that would inevitably be part of her life. We had no way of knowing that, at 33, she would be married with an amazing daughter and still doing well.

So with that perspective, today on the thirty-third anniversary of her diagnosis, I've decided to celebrate. As Holly put it, "Instead of  a death knell it was a starting gun." What could I possibly add to that?

Dreams Come True

The 33rd anniversary of Holly's diagnosis is coming up on December 12. She was diagnosed one day before her seven-month birthday. This is always a hard time for me. I can't held but go back to that day.

Actually, I must begin a couple of days before the diagnosis with a phone call from my sister. who bravely called to insist that I call our pediatrician and demand a sweat test. She had just spent time with us at Thanksgiving and had seen how much Holly ate, experienced the awful digestive problems she had and realized that in spite of the huge volume of food (Holly would consume a plate of food equal to what the adults ate.), she was still gaining no weight. So, Robyn, was willing to offend me for the sake of the life of my child, and I love her for that. Fortunately, God's timing is always perfect, and the day before Robyn called, I had read an article about CF and how kids with the disease often taste salty. That was something I had commented on for months but had never relayed to the doctor, not realizing it was of any significance.

Thus, when my sister called about the sweat test, I knew she was right. I knew I had to call Holly's doctor yet again and tell him that something was wrong with my child. Robyn was willing to pay for a flight to her home where her pediatrician had already agreed to do a sweat test if ours was not.

When Holly's pediatrician called me back, he started in with the same old song and dance of "normal is a range," etc., etc., until I said the magic words, "But she tastes salty." Then, he was momentarily silent before quietly declaring, "Oh, my God. I'll call Children's Medical Center immediately to schedule a sweat test as soon as possible. They'll call you back with an appointment."

Two days later my mother's cousin picked us up to take us to Children's Medical Center in Dallas for the sweat test, so I wouldn't have to endure the anxiety of the day and the bad news, if it came to that, alone. We went to Children's and the tech patiently explained the procedure, and I was relieved that Holly didn't have to be stuck and wouldn't feel pain. The tech cleaned Holly's wrists, stimulated them with the electrodes, put on the gauze (which had first been weighed), and then wrapped her arms with plastic wrap to encourage perspiration.

We spent about half an hour in the hallway trying to keep my little one occupied. She was an active baby who had bursts of energy in which she was busy sitting up, crawling, trying to stand and playing. Because of malnutrition, her energy usually didn't last long before she just wanted to be held or take a nap. At that particular time, wouldn't you know it, she wanted to play on the floor, of course. But all I could think of is: "This is a hospital, for goodness' sake. There couldn't be a worse place for her to be on the floor. I'm not putting my baby down there!" So, we passed her back and forth and took walks up and down the hallways, trying to engage her in anything that looked even remotely interesting. Fortunately, she was a beautiful and winsome child to whom people were naturally drawn, so we were able to occupy her with the help of other people who found her almost as delightful as I did.

Finally the time passed, the plastic wrap was unwrapped and the gauze was weighed to determine whether it contained enough sweat to test. Fortunately the answer was yes, and we were allowed to go.

My cousin decided to treat me to lunch at a lovely tea room, which I could never have visited on my budget. She wanted to take my mind off what was going on, so we went to this swanky eatery, which seriously frowned on the presence of babies. The maitre d protested, but was no match for my cousin who always managed to drop names, threaten, cajole or intimidate until she got her way, so we were seated in the calm, darkened interior and served wonderful entrees that I didn't taste or enjoy, because my mind was focused on the results of the test. We talked and talked. Anything to take our minds of the waiting, the endless waiting, for the test results.

The somber tone of the day was punctuated with humor, however, when I took Holly with me to the Ladies' Room. It was only then that I discovered that my face and neck were covered in orange splotches from the puréed peaches I'd fed her as I held her on my lap. (Since the tea room didn't normally allow babies, there were no high chairs for her, so she sat with me as we ate.) Naturally, I was horrified as I tried to remove the dried peaches from my face with one hand as I held Holly with the other while sophisticated ladies in their designer dresses and de rigeuer haughty looks came and went as I remained trying desperately to scrub away the last of my daughter's meal. Though mortifying at the time, it gave my cousin and I something to laugh about as we returned to the car.

I have no idea how we spent the rest of the day. I just remember the mental argument that ran over and over through my mind: "It can't possibly be something as bad as cystic fibrosis. I mean this is me. This is us -- our baby. It couldn't happen to us, could it? But the symptoms all fit. It would explain everything. But, surely, it's something simpler, not something quite so serious," though at the time I had no idea just how serious CF was.

Finally we were home. Holly was exhausted, so I put her down in her crib to sleep. I thought I would lose my mind as I waited for the phone call. Thirty minutes, 45 minutes, an hour, an hour and a half passed as we waited past the time we were promised an answer. Finally, I called the hospital and was told I'd have to wait until the morning to get the answer from Holly's pediatrician. I didn't think I could survive the night without an answer. Turns out I should have forced myself to wait.

I called our doctor's colleague, who agreed to get the results and call us. I waited by the phone as my cousin sat close by. The phone rang and I grabbed it to hear the doctor's voice inform me that the test was positive. Holly had cystic fibrosis. Instead of being kind and leaving me in my blissful ignorance until we saw Holly's doctor in the morning, he felt compelled to fill in all the details. He had never met me, had no idea of my mental or emotional state and didn't ask if I was alone or had someone with me. This cruel and uneducated professional proceeded to tell me that my 7-month-old daughter sleeping in the next room would probably die before her second birthday. I slumped against the stove as the life literally drained out of me. I remember that I was standing upright, and, then when he told me that, I felt as if a tidal wave of grief washed over me. The pain was so intense and overwhelming that I knew I could not survive it, and then I felt the life force drain out of me. I felt it leave my head and wash down through my body and out my feet. All that remained was my mental capacity to deal rationally with what to do next. All emotion had vanished.

As I hung up the phone, I told my cousin, "Holly has cystic fibrosis, and the doctor says she'll probably die before she's two. It will be time for dinner soon. I'd better get started on it." And that was that. I was in shock -- emotional shock -- a gracious coping mechanism God built in so we can cope with just such situations.

Somehow, we got through that awful night and went to visit the pediatrician the next morning. He was pale and somber, and the guilt he felt was palpable. He shook his head as he apologized and said, "I can't believe I missed it. I'm so sorry."

The good news was he educated us about the true facts of CF. It seemed strange to be relieved to learn that the average life expectancy was 18 years old. But, after the news of the night before, it felt like a reprieve from a death sentence. There was much more to learn and more to live. But here we are, happily, 33 years later. Holly's feeling the effects of CF, but she's achieved the dreams she once thought might be out of reach. She's married to a man whom she loves and who adores her. They have a precious 10-year-old daughter who is the light of both their lives. What was only a dream that I was afraid to hope for 33 years ago, has become reality, and I thank God for dreams that sometimes do come true.

Listening

As adults, we like schedules. We get up at the same time every day, eat at the same time, go to work or school at the same time, and we expect our children to live on our schedules. And, to a certain extent, that's reasonable and necessary.

However, when it comes to talking and sharing about what's on their hearts and minds, kids, whether or not they have CF, work on their own timetables, and, frequently they don't jibe with ours. Just about the time we're settling down to crawl into bed, they decide to open up. Though it's tempting to put them off until toothpicks aren't a necessary accoutrement to keep our eyes open, with kids, we have to listen when they're ready to talk. If we don't seize those opportunities, we lose them. They cannot and will not be vulnerable on our timetables.

Holly and I had a lot of 2 a.m. chat sessions sitting on her bed, sometimes high in the air in her loft. These were rare and precious times when she granted me access into her very private world of hopes, dreams and fears. These were the times she voiced the concerns that I had no idea she carried.

When we're entrusted with the gift of our children's open hearts, we must tread carefully. We must take their thoughts, feelings and fears seriously. At times, that means we sit quietly as we let it all sink in and weigh our answers thoughtfully and carefully. It's critical that our children know, without a doubt, that they have been heard and are being taken seriously.

Sometimes the feelings and thoughts they express will surprise or shock or even hurt us. I remember biting the inside of my cheek to keep from bursting into tears when, as a young teenager, Holly shared that she thought she probably shouldn't consider becoming a mother when she grew up. A friend of ours had recently died, and his death affected her deeply. She also observed and felt keenly the pain his high school and college-age children experienced. Holly said that, knowing she might not live to share the experiences of graduation, marriage and the birth of her children's children, she didn't think it was fair to them to bring them into the world only to "abandon" them too soon. As I bit my cheek and blinked back tears, I prayed for wisdom of how to answer this honest, realistic question. Finally, I told her that I believed with every fiber of my being that she was such a precious gift to those she touched that any child would be privileged to call her mom for as long as they had her, and that she should not deprive them of that. I explained that whatever time she shared with them, she would leave her imprint on them, and they would be better for it. Thank God for His insight on that one.

As it has turned out, she is now 33 and has a 10-year-old daughter who adores her mama, and her mama adores her right back. How deprived they both would have been had she chosen not to have a child.

There were many other discussions. Some were not quite so heavy and future-focused. There were the typical teenage angst talks, as well as those that dealt primarily with CF.

The point is that, as parents, we must be prepared, and we must be focused on the needs of our children ahead of our own needs for sleep. Along my journey as a parent, I have been fortunate to learn from people wiser and more experienced than myself. They have passed on valuable tips on communication with children that are especially appropriate during those intense times of sharing. These tips help us let our children know they have our attention and are our priority at that moment. It's not just important that we give our attention, they must know and believe they have our attention. How do we prove that?

1. Look them in the eye, and when they look back, don't look away, especially when they are the ones doing the talking.
2. Don't interrupt! Hear them out, even if you disagree with what they're saying.
3. Don't discount their thoughts and feelings. They're real and important to them, no matter how they may seem to us.
4. Do ask questions to show your interest and to get clarification.
5. Do take time to consider what they've said to show that you value and give importance to the information.
6. If you don't have the answers, be brave and honest enough to tell them. Or say, "I need some time to think about that. I don't have an answer right now. I'll get back to you." Then do it. Do NOT let it drop. They'll respect your honesty whether you have an answer or not. Believe it or not, they figure out sooner than we think that we don't have all the answers. They just choose to love us anyway.
7. Tell the truth, even it it's tough to say and tough for them to hear. They'll spot a lie before it gets past your lips. Children are intuitive creatures. They know us much better than we realize, and they will catch us in a lie, even if we think it's for their benefit. Once we've lied, we've lost their trust and may never get it back. (That's a topic for another post.) Just tell them what they need to know, taking their age and level of understanding into consideration.

Give your child(ren) the time and attention they need, when they need it, you'll not regret it. You'll catch up on your sleep, but you can never get back the opportunity to connect with your children if you miss it.