- A Father's Letter to the PHFriends Listserv
- A Flower For PH
- A Life of Lessons
- A Life on the Move
- A Walking and Talking Miracle
- Backpacking Through Life
- Choosing the Right Durable Medical Equipment (DME) Supplier
- Contingency Planning for C-Pap / BiPap Users
- Dark, Disturbing, but Deeply Insightful
- Do I Have To Ask?
- Exercise and Pulmonary Hypertension
- Flu Season Strategies for the PH Patient
- I Have PH. Can I take any of the PH Medications?
- In the Wee Hours
- Iron Deficiency and Pulmonary Hypertension
- Israeli PH Association Conference
- Life with Flo: The Series
- LIFE WITH FLO: The Series
- Living Life While You’ve Got It
- Living Wills: One Patient's Experience
- Living With PH and Studying Cranes
- Loose Lips Sink Studies
- Memory Loss and Pulmonary Hypertension
- Mr. Spock Speaks
- Mutterings and Musings on Being a Patient
- My best friend, Jean
- My Nightmare and PH
- My Story
- Myriam's Story
- Navigating the Benefits Maze
- Navigating The Health Care Super-Highway:
The anxieties of a consumer-oriented hospital system
- New Dietary Guidelines for 2010 Released - Changes that may affect you
- Pumpless in Colorado
- Random Thoughts
- Sarah of the Moment
- Single Parenting with PH
- Sinus Problems? Here's One Solution (no pun intended).
- The Canadian Medicare System - An Overview
- The Courage to Change the Things I Can
- The Emotional Side of PH
- The Hill, a poem
- The Lighter Side of PH
- The Way It Was, The Way It Is
- When the Insurance Company Says “NO”
A Father's Letter to the PHFriends Listserv
By Rich Bottini
Hello All !!!!
Today, January 12, is a very special date in the Bottini household. It was on this date in 1999 that our son Bryson was put on Flolan.
Bryson is a 7-year-old boy, diagnosed at age three with PPH. His diagnosis was a miracle in itself. Our Pediatrician heard what he thought was a heart murmur at Bryson's 3-year-old check-up and referred us to a Pediatric Cardiologist here in Charlotte to follow up. Two weeks later, after meeting and spending only one hour with this cardiologist, we were given the worst news that we could ever expect to hear. He diagnosed our son with Primary Pulmonary Hypertension. The prognosis at that point was not good. Although Bryce was showing no symptoms, there really was not a lot of hope for long-term survival.
We spent a few days in a complete and utter daze. But with the support of our family, friends and some special doctors including the two mentioned above, we decided we would fight this disease with all that we had. Nothing would prevent us from helping our son. With the aid of our Cardiologist here, who researched and gave us a list of five doctors who specialized in this disease, we decided to seek treatment from Dr. Nancy Bridges at Children's Hospital of Philadelphia. I would never be so bold as to say that one doctor is better than the next, but Dr. Bridges and C.H.O.P. are the best doctor and hospital for our son.
Initially she put Bryson on C.C.B.'s. He was too small to swallow the pills, so for the first eight months we had to extract the medicine from the gelcap with a needle and mix it in applesauce for him to eat. He seemed fine, constantly running around, and we were able from time to time to forget what was in front of us.
At this time we did not know any of you great people. In fact, we knew no one else who had the disease. We felt we should somehow get involved in the PH community so we attended the conference in Stone Mountain, Georgia. It was there that we saw what the disease had the potential of doing. It was there that we met so many strong-willed, beautiful people who were fighting the same battles we were. It was there that we met our PH family.
At the conference, someone -- and I am not sure if it was a
patient or not -- was dressed like the Eveready Bunny. At different times during the conference they would pop up and walk around the crowd. They had a pink suit on and a drum with the word Flolan printed on it, the premise being: with Flolan, you'll keep going, and going, and going!!! It was very funny but we had no idea how accurate and relevant it was at that stage of Bryson's illness.
We were really uncertain about what Flolan was or did. We thought the "Eveready" Flolan Bunny was a trip but we could not understand what everyone was so excited about. We did not really appreciate all that this medicine had given back to those of you who were on it at the time. We enjoyed ourselves but were not sure if we belonged, if that makes any sense.
Well, reality started to set in. Bryson started to get more and more winded. He was now six and playing T-ball and basketball for the second year, but he needed more breaks. He is a great athlete, but was unable to keep up with his friends endurance-wise. We flew up to Philly in the summer of 1998 and Dr. Bridges told us that Bryson was ready to take the next step in his treatment. We needed to consider Flolan.
Beth and I thought his life would change so dramatically. He would no longer be the same as the others his age. How do you tell a 6-year-old he is going to have a tube coming out of his chest, and a medicine pack attached to it. That he would ALWAYS have to have this with him. He would no longer be able to swim, to play organized sports, to just horseplay with his baby brother. How do we explain this to him????
It was then that I turned to this list. Thank God!!!! I asked for advice and suggestions, and you all so freely offered up ideas. We asked that we delay the procedure until we were able to completely explain to Bryson what was going to happen. We also needed to accept our situation as parents. We again cried and cried, but with the help of all of you on this list we accepted and were ready to battle again.
We then saw our son really start to fail. He could not walk 10 feet without having to rest. He had no life in him. Wherever we went I carried him on my shoulders. This disease was starting to take our son's soul. He would try to fight through it but his little body would let him down. He never complained and never stopped smiling, he just could not defeat this disease without some help. We prayed and prayed to God that the Flolan would help him, that he would be ok with the tube and it would not affect his courage and self worth. All I could think of -- all that kept flashing in my mind -- was that Eveready Flolan Bunny !!!!
We flew up to Philly on January 10. Our families all flew in from all over the country to help us emotionally. I had bought one of those wrestling figures, the 18-inch-high ones, and ran a tube out of Hulk Hogan's chest into a little battery that we made believe was a pump, so Bryson had a visualization of what it would look like on him. We were ready now to have the procedure done.
As they took him away from us into surgery, my heart broke as I watched this beautiful little boy be taken away, not knowing if we would get him back. If we did, would he be any better? I will never be able to explain the pain I felt as a father. I was unable to help him myself. I just wanted to take him into my arms and tell him he would be ok, that I would protect him and make him better, but I could not. I felt I had betrayed him. I was wallowing in self pity and then my Mom and I went down to the chapel to pray. It was then that I asked God to watch over Bryson and Dr. Bridges and her team, to please help our son. I felt a calmness set over me.
Beth and I talked alone. We supported each other as parents and vowed that we would be strong for Bryson. A nurse kept us informed every 45 minutes. Finally, after four hours, Dr. Bridges stood before us. The procedure went well. He is fine and we will wait and see what happens. We waited 15 minutes and then they allowed us to see him in CICU.
I looked at his little body sleeping so peacefully. His site was covered. We were fearful of what he would think when he woke up. After an hour his eyes opened. He smiled at Beth and I, took his mom's hand and asked her if she was ok. This brave little boy. He then asked to see his tube and pump. His CICU nurse showed him everything and he said that he wanted to name his pump "Pal". He chose Pal because since the pump was always next to him, they would become best pals.
We were allowed to sleep with him at the hospital that night and when he woke in the morning he was so...... alive!!!! Our little guy had color in his face, his face was fuller. We heard that precious little laugh of his. He was so full of energy. The disease that had been sucking the life out of our son was now being displaced by this amazing medicine.
Now we realized why everyone was so excited at the conference. His eyes sparkled, his voice was stronger. He had his "Pal" and he was ready to move on with his life. He was in the hospital for about a week. Beth and I learned all about mixing, changing dressings and all that other stuff. We were overwhelmed... he was not!!!! The only thing that mattered to him was that he FELT BETTER. If it took this medicine and this tube to make him better, he would deal with it!!!
I am a huge sports nut. I always looked up to athletes and felt that they were role models, to an extent. I idolized the Palmers, the Jordans, the Alis, Yankees, Knicks, so many others. Now I idolize just one person. That is my 7-year-old son. He is the strongest, bravest, most courageous beautiful person I will ever meet. He just wants to live, and Flolan is allowing him to do so.
He has never complained about his pump, never felt slighted. How could I ever possibly say one word about mixing meds or any other responsibility as a caregiver?? He has the tough job!!! He is the hero!!! He has energy now that he forgot he ever had. We have to warn him to slow down. It truly is a miracle. He is a gift to us from God, he truly is, as is his little brother Bailey. We have learned so much about life from our son. He has allowed me to step back and realize how important every single minute is that you spend with the people you love. Through the eyes of our son we now see life as it should be seen, as a special gift from God which we should never take for granted.
So on this January 12, we have a lot to be thankful for. First and foremost it is for our son's health. We are thankful to our families and friends who are with us every step of the way; thankful to all of you in our PH family whose thoughts and prayers have sustained and strengthened us; thankful to our doctors and their staff, the Children's Hospital of Philadelphia, all the docs and researchers who have put so much time and effort into finding a way to help us. We are thankful to God for giving us the courage to place our son in his capable hands.
Most importantly though, Beth, Bryson, Bailey and I would like to thank those of you whose footsteps we currently are walking in, those of you who cried the tears ahead of us, who tested these drugs for us, who made this whole process easier for us by your efforts. You are the true saviors to us. If it were not for you allowing the tests to be done on you, for offering your feedback and in some cases your lives, we would not be standing here right now with great hope for our son's future. You all have given him a better chance to live out his dreams. We will never be able to find the right words to express our gratitude. So we offer you these two little words, but many times over. Thank you .... thank you ... thank you!!!!!!!!
We are having a one-year birthday party tonight for PAL!!!!! We will put a piece of cake aside for all of you who are in our PH family. It is a great day for us as parents. Let us all hope and pray that a cure will be found soon. We love you all very much. HAPPY BIRTHDAY PAL !!!!!!
January 12, 2000
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