The Healthy Child

One of the primary challenges in a family, with a child who has a chronic illness and another who does not, is how to make sure the healthy child gets enough of the right kind of attention. It's so easy to focus on the child who requires a daily, time-consuming regimen and so easy to take the healthy child for granted. At one point, Vanessa, who does not have CF, expressed to her aunt (my sister) that she didn't think we loved her as much as Holly, because we didn't spend as much time with her. She was too young to understand that most of the time we spent keeping up with Holly's regimen was not fun time -- not for Holly and not for us.

I decided to do a reality check to see if we really were neglecting Vanessa in terms of the amount of attention we were giving her. My sister reassured me that from her observation it was not reality. However, I realized that what was important was Vanessa's perception, and we needed to make some changes to reassure her that we loved her just as much as Holly.

So, we decided to spend more one-on-one time with her, playing her favorite games, reading her favorite books. Personally, I also made sure that during my time with her, I was really connecting -- looking her in the eyes -- making sure she knew she had my undivided attention.

When Holly was hospitalized, we faced different challenges. As much as possible, we tried to keep the family together. Naturally, Holly avoided hospital food as much as possible, so I would bring their favorite casseroles, pizza or hamburgers to her room. We would all eat together, then, if homework was complete, we would watch a movie together until bedtime. Occasionally, my husband, my sister or I would take Vanessa by herself out for dinner and a movie while the other stayed with Holly. The balance was always precarious. Because we didn't want to swing too far in the other direction so that Holly would become jealous of the time spent with Vanessa outside the hospital.

Another challenge centered around food. When Holly was growing up, the CF Foundation had a program called 100 Percent Plus. It focused on ensuring that the children with CF got 100% of the nutrition that they needed, plus as many extra calories as possible in any form we could provide them. They recommended things like soda and candy bars, because they provided a lot of dense calories. In other words, Holly didn't have to eat a lot to get a lot of calories.

Well, try explaining to a six-year old why her sister can have two or three candy bars a day, as well as an equal number of sodas, and she can't. I pointed out to Vanessa that Holly needed to eat those things to stay healthy but that those same things were not good for her. Yeah, right. She didn't get it. Favoritism again, or so it seemed to her. So we compromised. Vanessa could have half a candy bar and one small glass of soda. That seemed to satisfy, not only her sweet tooth, but her desire to be treated the same as her sister.

It always seemed to be a tight wire, because Holly would see the unfairness in the fact that she had to spend between 45 minutes and 1 1/2 hours a day plugged into a nebulizer and getting CPT (We called it pounding.), while Vanessa was free to play. That required explanations that because of that time spent doing treatments, she could then be healthy enough to play. Naturally, that resulted in protestations of, "It's not fair!" My only response to that was, "You're right. It's not fair, and I hate that it's this way. But it is, so let's try to make the best of it." That seemed to help, though some time of pouting usually followed.

I finally realized that I had to make sure I treated each of the girls as individuals, meeting their own unique needs and hearing their thoughts and feelings about their lives. We did the best we could to be fair, and then I had to just let it go. Total fairness in this type of situation is impossible, and each child is, at one time or another, going to believe that they're getting the short end of the stick. It was up to me to make sure each child knew beyond a shadow of a doubt that she was loved. And, at the end of the day, that was the most I could do. I learned a lot about love and about loving each girl in the way she needed to be loved, because they each perceived it in different ways. They still do. So in teaching them, I learned valuable lessons that I hope I never forget. My children, and now my grandchildren, are still my best teachers!

Holly Shares

I thought I'd take some time to share, from my perspective, a few things that I thought might be helpful.

First, I am very compliant with my therapies (my regimen was written about in an earlier blog), but was not always. As I've told many parents, what made the difference was a change in perspective from feeling (despite my parents' urgings to the contrary) that I was a slave to the CF master to realizing that the various therapies served my purposes. I loved swimming throughout my school years and college and realized that I swam better after a treatment. I lived in a dorm with a community bathroom and realized that I didn't have to be embarrassed when I was faithful with my enzymes. And now, as the mother of a 7-year-old basketball player, I can cheer louder and enjoy the concessions at games, when I've done what it takes. Looking back it seems that I really became compliant when I went away to school (contrary to popular wisdom) because of my pride. I had a lot invested in showing my parents that I could make my own decisions and to me that meant my decisions would not be the ones they would have made for me. But, when they weren't there to see it, I guess I felt I wouldn't be losing anything. Then I became convinced of the good it was doing me toward my goals and I kept it up even after I came home. So, I guess the nugget of wisdom I'd want you to gain from me is that it's okay to nag, because you're going to anyway - you love us, you can't help it, even now, my daughter gets onto me if I'm not getting to things fast enough for her - and to encourage strongly the participation in anything your child is passionate about, because at some point that will become it's own motivation and consequence which will be much more powerful to your child than your emotional distress. (And resist any temptation to mitigate the natural consequences!)


Second, I cannot strongly enough impress upon you the need to encourage and assist your child(ren) in dreaming big. Reality has little to do with dreaming. Little boys everywhere dream of being astronauts and little girls of being princesses and not because there's any real chance of that happening. Encourage them to dream of universities and scholarships and marriage and children, all the things you'd encourage any other child to dream of. Then, do everything within your power to help them find ways to realize those dreams - get creative. I have much of what I dreamt of, but little of it came in the conventional way and this is true for most of my adult friends with CF. I don't know of any circumstance in which your child would need you to tell him that his dream is out of reach. If it truly is, he will discover it on his own. If you think he's giving up too easily, push a little or find someone he will listen to that can push a little. Don't give in without a fight. This point goes hand-in-hand with the previous point, because if there are no goals to be attained there is no reason to be compliant. Treatments and therapies for their own sake are worthless.
I will give an example from my own life. I started out wanting to be a doctor. Nobody told me I couldn't be or shouldn't be, but somewhere around age 15 I realized that the only kind of doctor I wanted to be was a CF doc and that it would be very emotional for me. I wasn't sure I could handle all of it without taking it personally. I also realized that I really wanted a family and I wasn't sure I was prepared to make the kind of sacrifices it would take to have a family and be a really good doctor; I didn't want to give anybody less than my all. So, I started thinking about other careers. At this point, my health was really poor and I was out of school and in the hospital a lot. I was out so much, in fact, that I was in danger of being failed (my school district was not in compliance with Americans with Disabilities Act at that time, but we weren't in a position to challenge it). Instead of my parents despairing and concluding that all these dreams were foolishness, they looked for a way to make it work out. That way came in taking my GED. Then I started taking a few classes at the local community college, majoring in music education with a focus on violin, which I'd studied for years. I could tailor my load to what I could handle and they were much more flexible. After a few semesters, I was able to get a scholarship to a state university only 3 hours away and near the clinic that had diagnosed me. We worked with the Office of Disabilities to get a few accommodations to give me the best chance at success. One was lowering the amount of credit hours required for full-time status and another was getting permission to turn in assignments or take tests late if I were sick or hospitalized at the time they were due. After two semesters, my health was too poor to return. After a year of floundering about thinking the future wasn't anything to look forward to, I met my husband and married him in short order (7 months).
Now that I was more hopeful about my future, I began reevaluating my goals. I still wanted to teach music, but I knew now that my body wasn't built for a 7-4 M-F schedule. That was a freeing realization, because that meant that I didn't need a degree to get into the classroom. I started by taking a few private students. Then, I began taking "teaching lessons" from a master teacher, who opened the door for me to work with a Conservatory. Soon I was teaching a full studio of private students as well as group lessons and ensembles. I also got involved performing with the local orchestra.
It was rather a round-about route to success and it took some real and discouraging setbacks. But, it ended up much better because the kind of work I got allowed me freedom to rearrange my schedule and to take on more or fewer responsibilities as needed. Then when I became pregnant and had an infant, I had the flexibility I needed to care for her in the way I wanted to and continue to work some as well. there are more examples, but that serves to illustrate my point that my dream was in fact attainable, even though it took some unconventional means.

"Here endeth the lesson"
- Sean Connery The Untouchables

Guest Blogger

Hi, long lost friends! Forgive my long absence from posting. The freelance work I started in July has kept me busy, and I haven't kept up with my blogging as I'd hoped to.

Fortunately, my wonderful daughter Holly has offered to write a post or two. I think, as moms of children/adults with CF, you'll be most interested in her thoughts on things, which may range from ideas on giving your kids hope, motivation to do treatments (yes!), nutrition, and, in general, what it was like to grow up with CF.

She has my permission to share what we, as parents, did right and where we missed the mark. Holly is gracious to understand that we didn't have anyone who'd gone before us to give us the insight I hope she's able to give you. Enjoy, and please comment. We'd love to hear your thoughts.

The Diagnosis

Please forgive me for taking so long to post again. I've been avoiding it, since this is a painful topic to think about, and writing of it truly takes a lot out of me.

My cousin, Bettilu, joined me on December 12, 1979 to take Holly to Children's Medical Center in Dallas for a sweat test, still the definitive diagnosis for cystic fibrosis. The techs took each of Holly's little forearms, scrubbed them clean with alcohol, then attached electric probes that would emit a small electric shock -- just enough to tickle a bit -- to stimulate the sweat glands. Next they used plastic wrap to attach cotton pads that had each been previously weighed. They wrapped the plastic wrap tightly around her arms several times, again to stimulate sweat. Next, we spent 30 minutes entertaining my little seven-month-old, waiting for her to sweat enough onto the pads so they could be tested for their chloride content. When the timer signaled the end of the 30 minutes, they weighed the pads to determine whether they contained enough chloride for the test. They did.

Leaving the hospital, my fears began to mount. Just in the past few days, I had learned about cystic fibrosis and found it difficult to believe that my precious daughter could have something so awful -- a disease that could threaten and take her life. Surely not. Surely, it was something else, a much more palatable explanation. An explanation we could live with, literally. But then, I would recount each of the symptoms and how they all added up to one diagnosis -- cystic fibrosis. Before I had read the article earlier in the week, written by a mom whose daughter had died of the disease, I had said something like, "Isn't that the disease Jerry Lewis raises money for?" Clearly, I knew nothing. But, thankfully, Bettilu took my mind off my fears and turned my attention toward a more practical and mundane matter, such as lunch. She decided to treat me to one of the finer tea rooms in Dallas. The maitre d' was not thrilled to admit a little one to his swanky place, but not many people argued with Bettilu and won. We were shown to a table. I have no idea what I had for lunch, but I remember clearly that Holly had strained peaches. Since this esteemed establishment kept no high chairs on hand. I had to feed her while holding her squirmy little self on my lap. Never an easy task with little one who'd been cooped up too long and who just wanted to get on the floor and crawl around. When we were finished, I went to the Ladies' Room. Finally, washing my hands, I looked into the mirror and realized that my face was covered with little, hand-sized smears of strained peaches. I laughed out loud in the privacy of the Ladies' Room and felt a sense of relief from the strain of the morning.

Bettilu took us home and was determined to stay with us until Holly's daddy came home from work. She was not about to leave us alone. Late in the afternoon, I got antsy. I told her that there was no way I could sleep that night without knowing whether the test was positive or negative. So, I began making phone calls to my pediatrician's office. He was out for the day, so I left messages for his colleague who was covering. I was about to give up on his returning my calls when the phone rang. (My heart is beating faster even now as I write this and remember.)I answered the only phone in our tiny house, which was in what my son-in-law would call our "one-butt kitchen." I faced Bettilu as the physician introduced himself and told me that he had the results of the test. He asked what I knew about cystic fibrosis. I said something like, "Well, I know it affects digestion and they have to take some kind of pills to digest their food." He replied, "That's true, but there's so much more than that." Without asking if there was someone with me or checking on my emotional state, he informed me that children with cystic fibrosis die -- always. He said it was possible she might live to go to school, but it was much more likely that she would die before she was two. I collapsed against the stove. I heard in the phone, "Mrs. Shute, Mrs. Shute? Are you there?" I had lost the ability to speak as I went into a kind of emotional shock. I remember thinking, "This is what a tidal wave must be like," as I was hit with overwhelming grief that washed over my body. The grief was so intense that I really thought I would die. Instead, I felt myself shutting down. It started at the top of my head and worked its way down through my body, removing all emotion so that I could survive. I finally found my voice and responded to him, asking what we should do next. He instructed me to bring Holly to the office the next morning to see our own pediatrician.

I didn't cry. I was too much in shock to feel. I simply looked at Bettilu and told her that it was time to fix dinner. I couldn't think of the reality of the doctor's words as my beautiful Holly lay sleeping unaware in her crib.

My husband came home from work, blissfully ignorant of what I'd just been told. I reported the news to him just as it had been reported to me. He looked at me, said, "We dedicated her to God, we can't take her back now." Then he walked quietly out of the kitchen into our bedroom and slammed the door. He stayed there alone for hours.

If I had thought that I could not sleep without news of the diagnosis, I certainly could not sleep with news of the diagnosis. I stood over Holly's crib for a long time, just watching her breathe, wondering how this largely unknown -- to me, at least -- disease could be stealing her away one breath at a time, even as I watched.

The next morning, we visited our pediatrician, who was pale, subdued and apologetic. He'd missed it. For those many months that he'd told us there was nothing wrong, CF had been doing its thing -- causing her to scream at night from stomach cramps, endure constant hunger because her food was not digested and feel the weakness from a lack of nutrition -- and he could have prevented it.

He asked what his colleague had told me and was appalled. He apologized again, and said, "Cystic fibrosis is bad, but there is some hope. Let me tell you the truth about it," and he proceeded to do that. We breathed a tiny sigh of relief to learn that the average life expectancy was 18 and that she would surely live to go to school.

He also told us that Children's Medical Center right here in Dallas had an excellent CF center where she could be treated and that their protocol was to admit those recently diagnosed. He promised to make the arrangements and called later in the day to tell us to take her in on Saturday for that was the first day they would have an open bed. We thanked him and set about making the painful calls to family and friends.

Our perfect child

Shortly after I learned I was pregnant with Holly, I began to be uncomfortable praying for a healthy child as I had for all my friends. I had a niece with a disability and my husband was close to a couple, each of whom had significant disabilities. I realized that in this broken world, not everyone could have a healthy child. With reticence, I began to share my thoughts with my husband only to learn that he'd been thinking the same things. So we prayed for the perfect child God had for us, whatever that meant. We also prayed that He would give us the grace and strength to deal with the problems or disabilities this "perfect" child would come with. So, it was a happy surprise that our tiny daughter, born on Mother’s Day, 1979, seemed so healthy.

We took her home, then brought her for her first visit to the pediatrician when she was 10 days old. Instead of losing the usual few ounces as we expected, she had lost a full pound. I was alarmed, but the doctor said it was really not a problem. I was breastfeeding and began to try everything I knew to make sure I had enough milk for her and to help her feed. Nothing seemed to help. She was two months old before she regained her birth weight of 7 lbs. 10 1/2 oz.

I'd never had a baby before, so I wasn't entirely sure what to expect, but her stools didn't seem normal to me. They were thin, runny, yellow or green, and sometimes full of mucous. And the smell never failed to run visitors out of the house. Again, I consulted the pediatrician, showing him one of her "dirty" diapers. Again, he was full of reassurances, telling me that she was fine, though I had a nagging feeling he was wrong.

Though it was all supposedly normal, he had me bring her to his office every week for a weight check. When I voiced concerns that her weight gain or stools didn’t seem normal, he would simply say that “normal was a range.”

Finally, when her weight didn't improve, he diagnosed it as "failure to thrive." I was cut to the heart – wounded. If he could only have seen how I held Holly and played with her. How I nursed her for 45 minutes every two hours to try to fill her little tummy. If he could have been there at night when I would heat two towels; one to go in between my stomach and hers as I lay flat on my back, holding her close, trying to ease the cramps that kept her awake and made her cry. When one towel cooled, I'd switch it with the one in the oven and repeat this over and over until 2:30 or 3:00 in the morning, when she was finally exhausted enough to be put her in crib to sleep.

At four months, we stopped breastfeeding because I “obviously” didn’t have enough milk. We switched to soy formula, which did nothing to help her, and still he insisted there was no physical problem.

At six months, we started her on solids. I ground her food from what I cooked, leaving out the seasoning. During the Thanksgiving holiday, we gathered with my family to celebrate. My sister, Robyn, observed with disbelief that I would fill Holly’s plate fuller than my own and that often within 30 minutes the food would reappear, undigested, in her diaper. Being a mom herself, she was shocked at the amount of food Holly was eating, while still gaining little weight.

About three weeks after Christmas, Robyn called me to ask if Holly was still eating so much. When I told her she was, Robyn asked how much weight she had gained. I told her that I thought maybe two or three ounces. Robyn was alarmed and told me it was imperative that we have a sweat test done. I confirmed her fears when I told her that Holly tasted salty. I had just read an article about cystic fibrosis, and the symptoms were too similar to ignore. That was when I learned the reason for the saltiness I tasted on Holly’s skin. Robyn made me promise to hang up the phone and immediately call our pediatrician and insist on a sweat test. I called him and received all the old reassurances – “normal is a range.” “But she tastes salty,” I said. He responded, “Oh, my God. I’ll call Children’s Medical Center and set up a sweat test right now.” He called back with an appointment for the next day.


Next: The Diagnosis

Remembering

Holly's 30 now! That is a big deal. When she was diagnosed, the average life expectancy was 18, and for so long, I was afraid to hope for more. Now here she is at 30 and doing well. She has her problems and flare-ups, but overall, she is really healthy. Praise God!

When I called Holly to wish her a happy birthday (she's in Alabama, I'm in Texas), I could tell by her voice that she was jazzed to be 30, so unlike many women who hate the thought of moving past their 20's. It's just so different with CF and all its statistics staring us in the face.

Every time one of my girls has a birthday, I remember back to the day of her birth. It was no different this year. I remembered when I realized I was in labor -- shortly after my husband and my cousin's husband had decided to go to stock car races. I had urged him to go, saying something like, "You might as well go and have fun. I'm never going into labor." Ha! In those days before cell phones, we had no way of reaching them, so five hours later at around midnight, my cousin and I were just about to leave for the hospital, 60 miles away, when the guys rolled in. They were covered in dirt, mud and grease, but my husband and I headed on out the door for the hospital. I'll never forget the sound of that little 4-cylinder engine whining as we flew along the highway back to Dallas.

Holly finally arrived, after an easy labor, at 7:06 a.m. on May 13th 1979: Mother's Day. Once again this year, I thought back to my first glimpse of her when a nurse put the tiny person all swaddled in pink in my arms. I remembered how good it felt to hold her -- so soft and warm. She was absolutely beautiful with perfect features even though she was scrunching up her little nose and mouth. I treasure the memory-video that replays itself in my mind when I think of that day, and I pray it never fades with age.

Thanks for the memories, Holly. This one and all the others that follow after. You will always be my special girl.

Holly's daily health care regimen

After viewing the blog, Holly suggested you might be interested in what she does to stay healthy every day. So here it is:

Albuterol twice a day
Pulmozyme once a day
7% Hypertonic Saline twice a day
Vest twice a day
Prevacid twice a day
Ultrase MT20 3 with meals
Actigall twice a day
Zithromax MWF
Celexa once a day
Metformin (not for CF-related purposes) 3 times a day
Reliv supplements twice a day
As much exercise as I can handle

She's found the Hypertonic Saline (HS) to be very helpful. After six months of using it, her lung function, which had gradually declined over several years, improved 30 percent! Her coughing had gotten bad enough that she coughed or cleared her throat almost constantly. The HS has relieved that, allowing her to get better rest at night.

For information on how the Reliv supplements have made a difference, visit her Web site at: www.geocities.com/MurrensNatureMama, and click on her Reliv Story. To summarize, I'll tell you that improving her nutrition has drastically reduced her need for hospitalizations.

Holly has a port on her right side, under her arm. This is her third, the first two being removed when she experienced two different bouts of blood infections. She prefers it under her arm, because it doesn't show when she wears sundresses or swimsuits. It's also easy when she's "hooked up" to just let the tube come out from under her shirt, so it's more comfortable and not in the way.

If you have any questions about Holly's routine or why she does things the way she does, please ask, and I'll get the questions to her. You know, if you're wondering, someone else probably is too.