Saturday, August 15, 2009

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.

Tuesday, June 23, 2009

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

Wednesday, June 3, 2009

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.

Monday, May 18, 2009

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.

Thursday, May 14, 2009

Introductions, continued


Meet Vanessa Elizabeth Dawn Loften, named after her grandmother Elizabeth (Betty) Freeman, who has been quite influential on both girls, and after our friend Don Pfendner, who was crucial in our decision to have another child. Vanessa is, at 26, nearly four years younger than Holly. When we chose, after Don's wise counsel, to have another child despite the doctors' advice, we decided that this child would be our last. If she did not have CF, we would celebrate and consider our family complete. If she did have CF, we knew we did not have the resources financially, physically, mentally or emotionally to deal with any more who might have the disease. So two would be our limit barring supernatural intervention, and after Vanessa was born, I felt complete. I did not long, as I had before, for more children. I felt blessed, as I still do, by my two precious daughters.

Vanessa and Holly are nearly as different as night and day. However, in the ways that are most important, they are very much alike. Vanessa is, like her sister, very creative and extremely intelligent. I felt constantly challenged by them. I still do. I'm learning from them all the time. The best part about being their mother is that they are women I would want to know, even if they weren't my daughters. As mothers, they constantly amaze me. They know how to love their own daughters in the way they need to be loved. They are doing what I strove to do, but better, and I admire them deeply.

Elizabeth means "encourager," and Vanessa has always lived up to that. When she was small, Holly made her a sweatshirt with a cheerleader on the front that she cherished and wore until it didn't cover the essentials anymore. Vanessa continues to encourage, as she has through Holly's most recent challenges, flying from Texas to Alabama to visit Holly as soon as it was practical. Her influence was dramatic. Holly began to improve noticeably while Vanessa was there, pushing herself to do more and more.

As sisters who live far from one another and have very different personalities and, sometimes, very different views, they work hard to stay close. They both are happy to be sisters, and when it counts, they're there for one another.

Examples: Holly gave birth to her daughter, Murren, at home. Crazy as it might sound, it's really not. Check out Holly's blog, www.geocities.com/MurrensNatureMama, to learn more. So when Vanessa got pregnant, she and her husband started planning a home birth. (Brian wasn't sure at first, especially since he works in the medical field, but after he learned all the facts, he became convinced.) During Vanessa's especially difficult labor, Holly was our long-distance support. We learned later that, as encouraging and confident as Holly was over the phone, as soon as she'd hang up, she'd be on the phone with a friend, frantic, and questioning if everything was really going OK.

And when I called Vanessa to give her the news of Holly's most recent crisis, I didn't have the foresight to make sure Brian was with her. As the shock hit her, Vanessa became very distraught at this unforeseen threat to her sister. I stayed on the phone with her for a long time, talking her through it until she was calmer. She told me then that as soon as possible, she would be on a plane to Holly. She just had to see her for herself to know that Holly would be OK.

They are, as I've said before, special gifts to me. They are also precious to one another. Thank God for Holly and Vanessa.

Wednesday, May 6, 2009

Forgive me for taking so long to post again. I had a particular photo of Vanessa that I wanted to use for my introduction of her. However, due to my recent move, I can't find it!

At any rate, I realized that in my introduction to Holly, I failed to tell you about her blog. It's for anyone with CF, especially those hoping to become parents either biologically or through adoption. She put in hours of research to offer complete and accurate information. It's probably the only place you'll find it all put together so clearly and concisely. I recommend that you check it out at: www.geocities.com/MurrensNatureMama.

Friday, April 10, 2009

Introductions


Now I think it's appropriate for you to be introduced to my family, the motivation for and source of any expertise I have on the subject of mothers of children with CF.

First, I think it's appropriate to introduce Holly, whom you're probably most anxious to meet. As you can see, she is a beautiful and talented young woman. She is a violinist and violin teacher, but that role comes a distant third behind her roles as wife and mother. Though CF has made reaching her goals difficult and sometimes forced her to take alternate routes to reach them, she has met them. Holly has an indomitable spirit. The more her goals are threatened by obstacles, the more determined she is to overcome them. The past year has been the most difficult yet, and there were times she thought she could not make it through another day. But she survived, and is now fighting her way back to health. More about that later. (Sometimes I will let Holly speak for herself.)


In my profile, you can see me with both of my daughters and granddaughters. If you click on "View my complete profile" you can see a larger picture. As you would probably guess, that's me on the left (the one with the white hair). Vanessa, my younger daughter, and her daughter, Brigid, are in the middle. Holly, who has CF, and her daughter, Murren, are on the right. Just in case you can't tell from the photo, I am crazy about my girls. They are my joy (and just once in a while, my pain :) ), but that's just between you and me. I will be sharing more photos as I continue to blog, because each of my girls is important to this story. I hope you'll keep coming back.

Wednesday, April 8, 2009

For CF Moms

You and I may be very different, and yet, we have so much in common. When others ask how we are or what's up? it's "Fine," and, "Not much." Then we run into one another and suddenly the social veneer we weren't even aware of drops and we share our hearts, our concerns, our fears and sometimes even celebrate joys. The questions change to: "How are his bowel problems? Hmmm ... sinkers or floaters ... or time for GoLytely?" "What color is her mucous? Is she able to cough it up?"

And then we have advice: "You might try putting the enzymes in mashed bananas instead of applesauce. Oh, and make sure he takes a drink before he sticks his fingers in his mouth!"

We celebrate when, despite the hour-long treatments morning and night, he makes honor roll or she makes first chair in orchestra. No one else understands the phenomenal effort it took to get there. We are proud for our children.

We share one another's burdens as well. We hurt when he learns that biological children are impossible without significant intervention. And we cry together when her PFTs drop again or the oxygen tank comes home to stay.

We belong to a very exclusive club -- that of mothers of children with cystic fibrosis. It's a club we did not choose and we hope our friends never have reason to join.

Most important of all, we are proud to be moms to these incredible children. These wonderful, wise-beyond-their-years, courageous, precious children. And difficult as our lives may be, they are also filled with a joy that makes it all worthwhile. Our children make every sacrifice, every heartache worth it. No regrets.

This blog is for you. I hope you will respond to my posts, make comments and pose questions. Welcome to "Faded Genes."