This email was written January 5 after Kenneth King's first appointment with Dr. Ledbetter. Mama had a lot to learn at this point!!!
Hi Family!
Sorry to get this out so late, but we didn't get home until 7:00. Kenneth King's appointment lasted from 1:30-5:00. Mama and I met with Dr. Ledbetter, head of the CF clinic, Charity, CF nurse, and Melissa Moser, FNP in the CF clinic. Alex was able to be on speakerphone and listen to the conversation, but the clinic gets horrible reception:( He was there in spirit!
Kenneth King has maintained his weight at 6 pounds 13 ounces. He was weighed by the genetics counselor two weeks ago and was the same weight. Since he has pancreatic insufficient CF this is not surprising. Dr. Ledbetter stated that he was glad that we could give Kenneth King a nutritional boost now because it will make a tremendous difference in his outcome. He is still 19 1/2 inches...his fingers, toes and feet must be 50% of that..longest I've ever seen!
The two mutations that Alex and I passed to Kenneth King mimic one another which means that he will present with "classic" CF symptoms. This also means that if we can successfully treat one of the mutations then we are that much closer to golden!! Delta F508 is the most common form of CF and is the most studied. Clinical trials are getting very close on this mutation. There are studies currently being conducted in Israel for W1282x seeing as it is the most common in the Ashkanazi Jewish population. I asked if a study is done in another country if it is accessible for US citizens. Dr. Ledbetter answered that the CF community shares and shares equally.
We learned that the Chattanooga CF clinic is part of the Vanderbilt CF clinic, but will be affiliating with the UT medical center soon in Knoxville. There is a loose group that works together in this region. Dr. Ledbetter has close connections with doctors at the Memphis CF clinic, Birmingham CF clinic, and Knoxville CF clinic. He knows all about Mississippi and worked in Jackson for a time. It was nice to make a connection there. He is even knows my childhood pediatrician's son!
We will still continue to see our regular pediatrician especially when sick seeing as sick children are NOT allowed at the CF clinic. Dr. Ledbetter also advises against CF patients convening for fear of superbugs become monsters! Dr. Meredith, pediatrician, will work with Dr. Ledbetter in treating Kenneth King. We will return for visits at the CF clinic every 2 weeks gradually reducing to every 3 months. This course of treatment depends on Kenneth King's reaction to the treatments. Our first follow up visit will be January 24th at 9:00 AM. Baseline blood testing will be done at this appointment. We are also waiting on approval from BCBS to administer a once monthly RSV-neutralizing antibody until RSV season is over. The link is as follows: http://www.rchsd.org/ourcare/programsservices/g-h/homecare/rsv-synagis/rsvfaqsforparents/index.htm.
He will have a repeat Sweat Test on February 6th at 9:00 AM. Dr. Ledbetter sees this as further clarification that we do have CF. Emma Virginia will have a Sweat Test on Monday at 9:00 AM to determine if she requires further testing. SOP states that she should be tested since her sibling has tested positive. She is NOT exhibiting symptoms.
I asked about phase III clinical trials for which Kenneth King might be eligible. There are none currently. Clinical trials include adults initially and then filter down to young children. There are not any trials for Kenneth King's mutations that will be marketable in the near future. However, Dr. Ledbetter does believe that Delta F508 may have a treatment by the time Kenneth King would be eligible to take it (in 7 years). http://www.cff.org/research/ClinicalResearch/FAQs/VX-770/ This is a link to some information about a company, Vertex, and VX 770, the treatment that it is researching.
I asked if there are positive or negative things to look for and what to do if negative things are noted. A serious cough that lasts more than 2 weeks or a puffy belly are two examples I was given. CF patients are expected to cough because that is how they release the secretions. Eventually, Alex and I will need to administer respiratory therapy by clapping our hands on Kenneth King's chest or thumping his chest. There are also other therapies (such as a vest) that can help at a later date. It has not been suggested that we start either of these things currently. One very positive sign would be weight gain within two weeks. He is expected to gain an ounce a day with the enzymes we started today. More about that in a second...
Dr. Ledbetter suggested some tips to maintain health which is 75% of the battle. Limit exposure to crowds as much as possible especially during cold and flu season. Have all family vaccinated for the flu especially anyone who visits the home for extended periods. If you are coming to visit please get vaccinated. Wash hands as often as possible and use hand sanitizer as much as possible. Do NOT allow him to be around anyone that he does not have to be around that is visibly ill, sneezing, coughing, etc. One thing to remember is that a cold to us can be deadly to him. He is not allowed to be around any smokers because we want to be sure he heads into the eye of the storm with the healthiest lungs possible. Dr. Ledbetter is not concerned about pets. The bacteria that Dr. Ledbetter is most concerned about is explained at this link http://en.wikipedia.org/wiki/Pseudomonas_aeruginosa.
Today we were given Creon which is a medicine used to treat people who can not digest their food normally due to pancreatic insufficiency (the pancreas does not make enough enzymes). Kenneth King did an awesome job of taking his first round of medication!!! He will take the enzyme before he nurses every time. The capsule is opened and the beads are sprinkled on applesauce (just enough to "deliver" the beads). We can use a wide mouth syringe or a very small baby spoon. Tonight Nonie gave him his applesauce by spoon and he did great! He will also be given a Vitamin A, B, D, E, and K plus zinc once daily by dropper. We'll wait until tomorrow for that one...it has been a long day:)
Each person we spoke with stressed how available they were to their CF family. Calls are returned promptly and we will have access to any of the staff at the CF clinic. The staff includes a social worker, dietitian, Nurse Practitioner, Pulminolgist, Nurse as well as support staff. I felt very confident about our meeting today!
I was told time and time again that CFF.org is the best place to go for research. I was also given a CF "manual" of sorts.
Alex, I hope I can speak for both of us and I think I can. We are doing our very best to control the things we can and pray that God takes care of the rest. We are going to celebrate this beautiful child the same way we celebrate Emma Virginia-wholeheartedly! We will do everything we can to stay healthy so that we are around when a life saving treatment is made available. Every life faces obstacles at some point...we know Kenneth King's ahead of time...and will fight like hell to overcome it! We hope that you will join us:)
So, raise your glass to research and good health---lots of love,
Ginger
No comments:
Post a Comment