Remembering

On the twelfth of December 32 years ago, Holly was diagnosed with CF. Back then, I was afraid to hope that I would have 32 years with her, yet here we are, and I am so grateful. Before Holly's diagnosis I felt a sort of elation as Christmas drew near; it was my favorite time of year. Now, however, December is hard for me. I experience an intense sadness that dampens my spirits. This year, though, I'm trying to focus on the joy of the season, the joy of my two daughters and three precious granddaughters, and, not least of all, the joy of Holly's good health. Yes, she has her problems, but she is so much healthier than we ever dared hope all those years ago. And I am grateful. Those of us whose children are threatened by CF know what it is to live with fear. It is ever present in the backs of our minds. And, if we are wise, we never take one day, one breath for granted. I have had the privilege, the joy, of Holly for 32 1/2 years now. It's still not nearly enough. So I'm wishing, praying for another 32 healthy years. Merry Christmas, Holly, and thanks for sticking around.

I'm thankful!

This Thanksgiving, I have much to be thankful for, not the least of which is Holly's good health and research that could result in truly effective treatment for her in the future. When she was diagnosed in 1979, the average life expectancy was 18, and now it's double that. Holly is much healthier at this stage than we ever dared hope. She is married to a wonderful man, and nine years ago, she gave birth to a precious little girl, Murren.

In October, Holly was hospitalized for lung and sinus infections. She had minor sinus surgery to clean her out and relieve the sinus headaches that had become daily occurrences. She was in for a week then went home on IV, oral and inhaled antibiotics. After she finished the antibiotics, it took her a couple of weeks to recover from the effects of the antibiotics, then she really started to feel better. Yea!

One of the interesting things, which I've noted here earlier, is that every time she's sick it's like the first time all over again. I always hurt and grieve for her and the normal life that she strives for, but will never really be. I try not to be over-dramatic, and I know that Holly does all the things she does, including hositalizations so that she can live a life that as close as possible to normal.

I have to also say that I'm thankful for Holly's attitude toward it all. She may feel awful, but she rarely complains. She's an amazing woman who is an exceptional mom, a great daughter, and, from what I've heard from others, a generous friend. I don't know how she has the energy to do all that she does.

I must also say that I'm thankful for Don Pfender, the young man who convinced my husband and I to have another child after Holly, despite the contrary advice of physicians. Because of him and his wise words, we had Vanessa Elizabeth Dawn. (Dawn is in honor of Don Pfendner.) Vanessa is my second personified blessing. Elizabeth means encourager, and she truly is. When she was small, we called her our cheerleader. She knows just what to say and do to encourage me just when I need it.

Thanksgiving is a truly special time for me with so much to be thankful for. I hope you'll join me this season in expressing gratitude for the many people and things we have to celebrate.

My hero!

Today was the CF Climb for Life here in Dallas, in which we climbed the stairs of the Bank of America tower and raised donations to benefit CFF. There I met up with the leader of our team, Beth, whose son, John Goldberg, died one year ago today, she showed up along with her family to support her and our team, which was called, appropriately: For the Love of John.

It was also a family affair for me. Holly was there, determined to climb, along with her husband, John, and their nine-year-old daughter Murren. Holly decided that she wanted to try to climb the whole way - 70 stories. I was hoping to make it 23, which was the first official stop. She left with a big smile on her face, accompanied by Murren. John left later with those planning to go to the 42 floor, and I was last.

When I arrived, huffing and puffing, to the 23rd floor, I was taken, via elevator, to the 70th floor to await the others and join in the after-party. I met up with John and we both kept looking for Holly and Murren. Finally, another member of our "For the Love of John" team found me (It happened to be the sister of John for whom our team is named.) and told me that she had met up with Holly and Murren on the 64th floor. They were going to make it!

When I heard that, I elbowed my way (as politely as possible) to the front of the welcoming group, so I would be the first to see her. I didn't have to wait long before she came through the door, triumphant, but emotional to be The One to accomplish it. We fell on each other and cried together. She is truly amazing. None of us, including her, ever thought she could make it all the way, yet there she was.

That determination that God gave her is what has kept her going through some very tough times in her life and will stand her in good stead in the future. May I just say, she's my hero!

Holly Post #2

At 32, and as a person who's never been content to simply do what others may think is best, I've learned a lot about how to manage my CF. I would urge you, as moms, to become your child's advocate, studying what is out there, the choices you/he/she have that may improve your child's health and longevity. What follows are tips you might find useful in your own caregiving. If you have questions, please comment, and I'll do my best to answer.

Hypertonic Saline - There are different percentages of dilution, but the key is just to use the highest salinity that your child can tolerate: the saltier, the better. In 2006 I realized that my lung function had dropped slowly but substantially over the course of years. From my high in college, I'd dropped approximately 50 points! I had to fight to be allowed to try it, but the results were phenomenal! In six month's time I had regained 40 points and no longer struggled to clear the sludge in my lungs. That is what I had noticed the most and sought to remedy. I would cough and cough and cough ‘til I could hardly catch my breath, and my head would hurt, and yet, the mucus deep in my lungs - that I could feel there - wouldn't budge... until Hypertonic Saline. You can buy this prepared in different dilutions or you can make your own at home. I've done both. I still use 7% Hypertonic.

Zantac/Prevacid/Nexium (and the like) - In 1991 I was taking 12 enzymes with every meal and still having tummy troubles all the time. (That was back in the day when they just added more & more enzymes.) Then my GI doc prescribed Zantac (That was the new big thing.), and my tummy got better. After my issues resolved, we were able to bring my enzymes down to only six. Over the years as I've gotten on better PPIs, I've been able to bring down my enzymes even further. I now take Prevacid twice a day and only three Creon 24s with meals.

The Vest (of course) - Before the Vest we had manual percussion. My family never had a percussor, and about high school I quit doing the manual treatments. I was swimming lots and competitively and relied on that exercise (which was excellent airway clearance as well as aerobic exercise) solely all through college. I got the Vest in the Summer before I got married. If you have one, you know how wonderful the Hill-Rom Vest people are at making sure you get a Vest no matter what your financial situation is. I have used my Vest faithfully for 12 years now. It is great, because now as a mother in a single-car family, getting to a place where I can swim isn't feasible. And in Texas and Alabama, the heat prevents me from outside exercise several months of the year. Though I do (try to) exercise inside, it is never a replacement for the Vest.

Nutrition - I know that CF clinics talk a lot about calories and fat and vitamins and sodium, and those are good things, but there is a lot more to nutrition than just that. Educating myself has helped me great deal. (I did notice at CFIT this year that the approach was more holistic than in the past and was very pleased). I had been taking iron, ADEKS and calcium for years. Then, I began taking Reliv supplements (a whole-food-based supplement) in 2002, just months after my daughter was born. At that time I was really low, drained physically. I noticed an improvement immediately! Since then, I have continued Reliv and added JuicePlus+ (an organic fruit & vegetable supplement) and have continued to notice improvement over time. In addition to these things I have worked with a Naturopath who has helped me through particular issues and crises of health. I have not used these to replace medications like enzymes or aerosols, I have simply added nutrition therapies. I would love to spend more time on this but maybe I'll save that for another post.

Brovana/Forodil - Beginning in about 2009, I had noticed that my albuterol didn't seem to be lasting very long. After a few hours I felt that I needed another aerosol. My lung function was still good and stable, so I didn't know what was going on. During a hospitalization in February 2011, I was put on Brovana in addition to albuterol. What a difference! I felt like my lungs stayed wide open ALL DAY! Once home I switched to Forodil. The usual way of using these is to alternate treatments with the Brovana and albuterol, but I have found that it works best for me (and the MD approved) to use the Forodil immediately before my albuterol treatment. This has brought me a freedom that I've not had for several years of skipping a treatment occasionally. For some time, I just could not skip a nighttime treatment because I would either not sleep for the coughing and shortness of breath, or I would awaken in the night and have to do the albuterol. And I couldn't skip a morning treatment because I would be so tired and short of breath in tasks and coughing constantly. But, now if I come home late or particularly tired, I just do the Forodil aerolizer and go straight to bed. Or if I have to get up and out early, occasionally I will just do the Forodil and go about my day without struggle. Now, this is only an occasional skip. I still have to be diligent to do full treatments (Forodil, albuterol, Pulmozyme, Hypertonic Saline, and Vest) most mornings and evenings. This allows me a greater freedom in my social life, which is wonderful!

Nutramagen - this is the infant formula I was placed on immediately after diagnosis. As a 7-month old, my major symptom was malabsorbtion. The doctors warned that the formula was terrible tasting and I would probably reject it at first, but I didn't. I took the first bottle, sucked it down, and smiled at it. Problem solved! I quickly began gaining weight and thriving. Of course, that was 1979 and now they have, not only the formula, but enzymes for infants so that they can drink breastmilk or other formulas.

Probiotics - I'm not just talking about acidophilus, though that is certainly better than nothing at all. I'm talking about multiple strains of beneficial bacteria in massive quantities. I like Nature's Sunshine Probiotic 11 and Solaray Multidophilus 12. Over the years antibiotics have become harder and harder on my body, and, if I don't take probiotics with and after my antibiotic rounds, I am miserable. It can take months for my body to recover from the thrush, diarrhea and upset stomach. So while on the antibiotics, I take as much as I can afford, and usually the cheap stuff since I know it's going to get killed off. I also eat live active-culture yogurt by the quart. After I'm off the antibiotics, then I bring in the higher quality stuff and continue eating the yogurt. When things seem to have returned to normal, I scale back to simply taking enteric-coated acidophilus every morning and, usually by this time, I'm sick of yogurt so I take a break.

Neti Pot - I began using a Neti Pot to rinse my sinuses about 5 years ago. After years of chronic infection, inflammation and headaches, I finally broke the cycle. My sinuses took a while to become clear and during this time I rinsed my sinuses (from the left AND right) twice a day. After they became clear, I rinsed only one way once a day and now I only rinse when things become congested. I very rarely have sinus infections now and most days are headache-free. On the rare occassion that I do get an infection, I have been able to treat it at home without antibiotics and have not needed a surgery since using the Neti Pot.

I hope you find this helpful. And, if you find something you're thinking about trying, keep in mind that your doctor may be resistant. Don't just avoid telling him/her. Do your research, think it all through carefully. As I mentioned, I've had to fight sometimes to be allowed to try things that, ultimately, have had a positive impact on my life and health. So, just because you have to be persistent doesn't mean you shouldn't try it!

Thanks, John

Nearly 31 years ago, on December 13, 1979, Holly was hospitalized at Children's Medical Center in Dallas, where she had just been diagnosed. The staff there thought that we should go to CF Clinic there, since we'd be going regularly for the rest of her life. I didn't want to go and resisted mightily. I didn't think I was ready to walk into a room full of small children who were pale and wasted with oxygen masks covering their noses and mouths. The social worker had arranged for us to meet a mom and her son who had CF. She thought they would be helpful to us, especially since we attended the same church.

We were to go Thursday morning. However, I had no plans of going. I was resolute. I was not going with my beautiful seven-month-old daughter to a depressing place until I was forced.

I was forced. I was holding Holly when they came. As I argued, they gently took me by the arms, one on each side of me, and pushed/pulled me out the door of Holly's hospital room, all the way to the CF Clinic. When I got there, I sat off by myself, not wanting to speak to or even make eye contact with anyone. I did cast a few furtive glances about the room. Enough to see that there were only a couple of children on oxygen. The others were playing or sitting on their mothers' laps. That was somewhat of a relief -- not as bad as I'd thought it might be.

You have to understand: When Holly was diagnosed and I learned exactly what CF was, I cried until I could cry no more. I thought I would never smile, and certainly I would never laugh again. I was on the brink. I didn't think I could cope with anymore sadness. The sight of a dying child would push me over the edge.

We waited a little while, and then the door to the exam room behind us opened. Out walked this adorable, blond-haired little boy followed by his mother, who was sharing a laugh with Dr. Prestidge. I remember staring. He looked healthy. She was laughing. Was there cause for hope? Might I someday smile again? It was still too early to tell.

As I stared, I was introduced to Beth and little five-year-old John. I don't remember what we talked about, just that I was amazed and hopeful. Maybe, just maybe . . .

Beth and I became friends and babysat for one another. After all, who else knew how to get our kids to take their enzymes? I learned to hope and even smile again. Laughter took a bit longer. Even after moving from Dallas, we kept up. When we moved back to Texas from New Jersey, we lived in Temple, which was two and half hours from Dallas. However, we still took Holly to Clinic at Children's. Sometimes, we would spend the night with their family, because Clinic started bright and early at 8 a.m. Clinic is set up on a first-come, first-served basis. So the earlier you get there, the earlier you get out. Plus some days, Holly had fasting blood work ordered. That meant that the earlier we got there, the earlier she could eat!

Years later, I remember calling Beth's house to give them a personal invitation to Holly's wedding. John answered. We talked about his health, his activities and his plans for the future. He had dealt with some serious scares over the years but always managed to come through.

At some point in time, he and Holly renewed their friendship, and she kept me informed of what was going on in his life. We rejoiced with him and his wife, Jennifer, at the birth of their son, Jack. And prayed for new lungs as John's health deteriorated. We celebrated when we learned of his bilateral lung transplant in September of 2007, and were thrilled to hear his reports of breathing easily for the first time in years. Holly tried to imagine what it would be like to live without daily breathing treatments.

We kept John and Jennifer informed when Holly was diagnosed with a brain tumor and then underwent surgery to remove it in September 2008. Holly corresponded with him and Jennifer as she sat alone in Birmingham for five weeks undergoing radiation every day. They assured her of their prayers.

Then, he began to have problems. His body was rejecting his lungs. Eventually, he went into chronic rejection, and his only hope was another transplant. We looked anxiously for every email update and prayed fervently. It was a special thrill for me when John received his second pair of new lungs, as well as a new kidney, on my birthday this year.

This time, though, it seemed there were complications from the start. The doctors would resolve one issue just as another presented itself. It was one thing after another. Holly and I both cried when we learned that he had a fungal blood infection. We knew the implications, because Holly had had one when she was 17. She had barely survived, and she was healthy going into it. John's body was already compromised.

Less than a week later, we got the news that John had passed away. I sent a text message to Holly's husband and asked him to tell her, because I thought she needed to hear it in person. This was just too much to share in a phone call. We have never talked about it. It's just too much and too close. John's death brings up so many emotions for us both that we can't handle over the phone.

I went to the memorial service, and it was a wonderful tribute for an amazing, courageous man who had a zest for life in spite of all his trials and difficulties. Friends shared their memories of John and what a terrific impact he had on their lives. Sitting there, I realized that John influenced more people and packed more living in his 36 years than most people do in 80. Sad as I was for Beth, Jennifer, little Jack, Holly and myself, I was happy for John knowing that he is in the arms of his loving Savior for all eternity. He's breathing easily and probably dancing for joy. What a lovely thought.

So, this post is a thank you to Beth and to her remarkable son. Goodbye, John. See you later.

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