NTM Treatment Update We are nearing the end of week 11 of Leah's NTM treatment. Here is where we stand.... Promising news - As of Friday, her sputum culture from March 14th has shown no growth of the NTM (fifteen days reporting). We are pleased to see this, as usually we see the NTM show up within 5-7 days. However, results will not be finalized for another couple of weeks. If it continues to be negative, this would count as her first negative culture. Culture conversion is defined as three consecutive negative cultures. One of the IV medications that Leah was on is known to cause hearing loss. Every two weeks, Leah was having her hearing checked. Her hearing was super good when we started and we had been seeing a gradual decline (not in the language range, but in really high pitch sounds) each time. Doctor was hoping it would level off, but since it continued to decline significantly in those ranges, we could assume that it would trickle down into the language sounds if we continued on the medication. After being on the IV Amikacin for 9 weeks, we needed to switch her to the nebulized form of the medication. We will continue to do auditory testing regularly as it can still cause hearing loss, but it shouldn't effect the hearing to the same degree as the extremely high dose she was on with the IV. Leah is still on the other IV medication and will finish out the full 12 weeks on it. But when we switched her to the nebulized form of the Amikacin, we added in two more oral antibiotics, which we will continue throughout the duration of the continuation phase of her treatment. In total, she is on 5 different antibiotics right now. We had a bit of a scare as we entered Spring Break. The day after we switched her to the nebulized Amikacin and added the two heavy duty antibiotics, Leah got sick with a high fever. Doctor thought it was most likely viral, however, since it was persisting, we had to consider if it might be an infection in her central line or a drug fever from the medications she was on. Thankfully after 5 days, her fever went away, but she was completely worn out, had increased cough, congestion, and was barely eating. After a couple more days, she started to bounce back and we are being really careful not to push her too much. Doctor said it can take up to 2 weeks to completely recover from the virus that hit her. Needless to say, spring break was not exactly fun for Leah. But we are glad that she is starting to feel better and able to do a bit more now! Joliet Great Strides - May 6, 2017We are also getting ready for the Joliet Great Strides walk on Saturday, May 6th! This will be our 8th year walking to support the amazing work that the Cystic Fibrosis Foundation is doing to improve and extend the lives of those living with CF.
We are thankful for the amazing breakthrough treatments that are now available, becoming available, and in the pipeline for the future! The CFF is committed to Leah, our family, and the thousands of others impacted by CF. They will not stop until a cure is found for everyone with CF! And we want to be part of this. Please consider joining us!! Your support means the world to us! To donate or join our team, please click here.
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Treatment UpdateAs we complete week 8 of the intensive phase of Leah's NTM treatment, we wanted to give you an update on how she is doing... Overall, Leah is doing great! Her body continues to handle the medication well. Thankfully, she has not had any significant side effects thus far. The treatments are not slowing her down at all, in fact, she seems to be doing better than when we began. Her cough is minimum, she has been fever free, her pulmonary function is a bit higher than it had been Oct-Dec, and overall she is feeling better. At her last visit with doctor, he didn't hear any crackles in her lungs!! That was super exciting to hear. Crackles in the lungs indicate infection, and we haven't had a clear sounding lung report from doctor in a long, long time. Even though we are seeing good results clinically, her first culture taken at 4 weeks continued to test positive for the mycobacterium abscessus. So the stubborn infection is still hanging on. Her next culture will be taken this Tuesday. We are hoping and praying to see a culture conversion, which would be a negative culture not showing any of the m. abscessus growing in it. She is receiving home bound instruction through a teacher from her school. This has been helpful in keeping her caught up with her school work while giving us time to take care of Leah's treatments, doctor visits, lab work, and tests. Plus, it has helped keep her healthy and away from all those icky sickness bugs that have been very prevalent around here lately. BIG Thanks......Leah is our strong girl and she is a fighter! We have been blown away by the amazing support she has received as she fights this infection. So many have reached out and showered Leah with their love and support! Here is a sampling of the support that she has received... Leah's friends from Awana sent this picture to cheer her on, along with a big manila envelope filled with valentines for her. Leah's aunt made this amazing piece of art with Fight Song written out... Love it! This February, Leah was completely showered with gifts and cards from the amazing ladies of Epsilon Sigma Alpha, a service and leadership sorority at Illinois State University. This kind and generous group of 300 incredible women gathered three overflowing bags of goodies (coloring books, games, stuffed animals, and more) for Leah. They also sent words of encouragement and support through a huge pile of notes/cards letting Leah know that she is loved and supported by this awesome group of women. Reading over the cards made our hearts full of joy and happiness! Thank you, ladies! You made our day better through your extravagant kindness. Our church has loved on us in so many ways - prayers, hugs, gift cards/money, and even brand new lofts for our girls made by one incredibly generous and talented family! We are so very thankful to be part of this loving and supportive community!
Thank You! We appreciate all the prayers and support everyone has shown Leah and our family! Thank you! Leah came home from the hospital late Monday. It is nice to have us all under the same roof! Please pray for us as we transition home. We met with home health to get Leah's IV medicine set up at home. We are working on getting home bound instruction started with Leah's school. We appreciate all your prayers and cheers as Leah continues her NTM treatment at home!
We wanted to say a BIG thank you for all the support you have shown Leah! She has felt great love and encouragement from all the notes, videos, pictures, and visits she has received! On her hospital room door she has an encouragement sign from her CF Care Center Team. They are the best! We love them. Leah has been taking her medications like a champ. She seems to be adjusting and handling the medication well. Her port site is still a bit sore, but is starting to heal. We expect to be discharged on Monday. We will continue her IV/oral medication regimen at home. Leah got to go outside for a little bit this afternoon and enjoy the spring like weather while visiting with her cousins. We took a walk by the Ronald McDonald House across the street from the hospital. Thank you so much for all your prayers! We are blessed to have so many praying for Leah and our family as she tackles this NTM infection.
Leah's been singing Fight Song this weekend, thinking about making up her own fight song, knowing she is getting ready for a BIG fight! This is my fight song, take back my life song, prove I'm alright song My power's turned on, starting right now I'll be strong... Cause I've still got a lot of fight left in me Here we are... ready to tackle the NTM infection that has no business being in our little girl. To do this, Leah has to be strong and courageous and ready to FIGHT. It is going to be intense and very hard on her body, but it has become clear that it is time to begin treatment. Although Leah has cultured the NTM (Nontuberculous Mycobacteria, M Abscessus) since April 2014, up to this point there was not enough evidence indicating an active infection to begin treatment, as the treatment is very intense and taxing on the body. But since her pulmonary function dropped significantly in October and did not improve with treatment of the typical CF pathogens, which included three weeks of IV antibiotics, along with other clinical symptoms (increased cough, fevers, not feeling well), doctor strongly recommends beginning treatment at this time.
Recommended treatment for M. abscessus pulmonary disease in CF involves two phases:
We begin treatment this week. On Tuesday, January 17, Leah will have a central line port placed and be admitted into the hospital to begin the initial intensive phase of treatment. We expect to be in the hospital for probably about a week as she begins her IV antibiotics to monitor how she handles the different medications. We will continue the intensive phase of IV medications at home, with close monitoring/testing for culture response and drug toxicity. We are still not sure what this means for her schooling during the initiation phase. We are considering homebound instruction or partial days, depending on how the medications affect her and what the treatment schedule allows. As a show of support, would you take a few minutes this week to let Leah know she is not alone in this fight. Maybe....
And please pray!! Bottom line is we are desperate to see God's hand of healing move and restore Leah's health. Our family has witnessed first hand God's miraculous healing this year... and we are on our knees praying for a another miracle of healing for Leah. This is a very serious infection and is known to be highly resistant to treatment. Please join us in praying for the medicine to be effective and eradicate this infection from Leah's lungs. Please pray for our faith to be strong and our trust and confidence to be firmly in God as we go through this time! Below is a picture of Leah dressed up as Sarah Hale for the third grade wax museum at her school. She researched and wrote a speech that she recited as the "museum guests" came around to visit the different "wax figures." I was not familiar with Sarah Hale until Leah began researching her. After learning about her, I am not surprised that Leah chose her. Sarah Hale is known for making Thanksgiving a national holiday. This was not an easy task. At a time when our country was divided, she became passionate about the necessity of Thanksgiving becoming a national holiday - a day for the country to look beyond trouble or hardship and remember all that we have to be thankful for.
Sarah was persistent. She wrote articles and letters to governors and the president pleading her case for Thanksgiving. The fifth president she wrote to finally said yes. After pursuing this dream for 36 years, President Abraham Lincoln issued a proclamation declaring a national day for Thanksgiving. Leah is most definitely persistent and passionate. When she gets an idea in her head, she goes after it. She does not stop until the possibility of her idea has been fully considered. I love that about her. We can learn so much from our children. Just like Leah, we want to be persistent in pursuit of our dream to find a cure for cystic fibrosis. We want Leah to have a full life ahead of her to pursue her dreams. We won't give up our fight until a cure is found. Will you join us? Please consider supporting us in our pursuit. Here is how you can help:
Thank you for your support! An event that has become so special to us, a tradition for our family, is coming up quickly. We will once again be walking in the Joliet Great Strides walk to help raise awareness and crucial funds to support finding a cure for cystic fibrosis and our little lady, Leah! We want to invite you to support our team. Here is how you can help:
When You don't move mountains I'm needing You to move When You don't part the waters I wish I could walk through When You don't give the answers as I cry out to you I will trust, I will trust, I will trust in You! Truth is, You know what tomorrow brings There's not a day ahead You have not seen So, in all things be my life and breath I want what You want Lord and nothing less You are my strength and comfort You are my steady hand You are my firm foundation; the rock on which I stand Your ways are always higher Your plans are always good There's not a place where I'll go, You've not already stood I will trust in You! This life can have intense ups and downs. I imagine pretty much all of you can relate. When there is nothing else you can do, cry out to the one who is worthy of our trust. Let God be your strength and comfort!
Thank you for standing by us through it all. You mean the world to us! I know it has been a while since our last update... so lots to say. But let's get started with talking about our cars. I'm sure your cars have been working hard this summer, taking you here and there, and it's time to freshen them up and get them looking sparkling clean. Let us help you out with that!! This Saturday, August 15th, the Minooka Cheerleaders are doing a CAR WASH to raise money for the Cystic Fibrosis Foundation in honor of Leah and Ben, one of our brave CF warrior friends. Please come and support this great cause. If you are unable to actually make it to the car wash, you can still support the efforts of the Minooka Cheerleaders. Maybe consider how many cars you have and if you were to get them washed, how much you would like to donate to support their efforts. Then, simply click here and make a donation on the event page. How is Leah doing???Leah has had a great summer! We started off the summer with a trip to Cedar Campus in the Upper Peninsula of Michigan. Phil was staffing a week of camp for the InterVarsity college students and we were able to join him. It was a wonderfully blessed time of fun as a family and with some new friends. Leah spent the week playing at the beach, kayaking, canoeing, hiking, and playing. It was a blast! The rest of the summer has been busy with swimming, playing with sisters and friends, and spending days at Six Flags Great America, enjoying all the spinny, dizzy-making rides and most of the roller coasters (Leah probably would have tried them all, but wasn't quite tall enough to ride some of the big ones)! FUN TIMES!! And our sweet girl decided to donate her hair this summer. So, she is sporting a super cute, super short hairdo and loving it! Health Update and some BIG newsHealth-wise, Leah has had a good, uneventful summer. Clinically, she has been doing really well and doctor has been pleased. We even had a month off from clinic visits this summer. Her PFT (pulmonary function test) has increased, her cough has been minimal, her appetite has been good and she has been able to gain weight. It seems that her hospital stay back in March really helped us see an overall increase in her health. She is still culturing the NTM, but since she has been doing so well clinically, our plan continues to be to keep a close watch on her. We would need to see strong evidence of the NTM infection causing disease before starting treatment.
AND... We also have GREAT NEWS to share that is making waves not only in the CF community, but in the medical world. On July 2nd, the FDA approved Orkambi, the first drug developed to treat the underlying cause of CF in those who have two copies of the delta F 508 mutation, which is the most frequent disease-causing mutation for CF (and the mutations that Leah has)! Right now, it is approved for use in those who are 12 and older. We expect that within a year, it will be approved for ages 6 to 12. If you would like to read more about this exciting new CF treatment, please click here. While this is not a cure, words cannot express how excited we are about the potential this has for Leah's future. We eagerly look forward to her benefiting from this new treatment when it becomes available for her age. We are so thankful for all of you and your support as we continue to fight this disease. Thank You!! A big thanks for all the prayers and support you all have given to Leah and our family. Doctor is really pleased with Leah's progress. She is feeling much better. Her appetite is back and she has been gaining weight. Her cough is minimal and her pulmonary function has steadily increased from where it was when she was first admitted. Our plan is to stay in the hospital for the duration of her IV antibiotic treatment, which should mean we will be able to leave late Monday. So the end of her first hospital stay is in sight.
Her room is cheerfully decorated by the many cards she has received from her sisters, cousins, classmates, and church friends. Earlier this week, she got the chance to skype with her class. She really enjoyed seeing all her classmates and teachers. She even did a magic trick for them. Her days are so very busy with different people coming in, but this past week, we have been able to get some school work done each day. (Although, perhaps needless to say...this is not her favorite part of her day.) You may often hear how getting a cold or flu is more serious for someone with CF. Now, I understand what that means. Leah tested positive for the entero/rhinovirus, which is also known as the common cold. The doctors explained that the viral infection can stir up the bacteria in her lungs and cause the infection to become inflamed, leading to an acute exacerbation.
While we are so thankful that Leah is doing better, we also are realizing that when you go through a significant pulmonary exacerbation, rarely will you come out the other side unscathed. This leaves a mark on her lungs. While her pulmonary function is back almost to her most recent baseline, it is still not where we had been hoping it would be. Unfortunately, this is the reality of this relentless disease. And I don't like it! On top of that, we are so very aware of the NTM infection that Leah has. She will continue to be watched closely - Culture, PFT, and Doctor visits monthly, X-rays every 3 months, and CT scans every 6 months. At her last CT scan in December, three nodules were present in her lungs. This would indicate that the NTM infection is not just present in her lungs, but also somewhat active. Her next CT scan will help us determine how quickly the infection is growing. As we move forward, please pray for discernment and wisdom as we consider what the best course of treatment it would be. And pray for this particularly resilient infection to be miraculously taken away from her. Please continue to pray for Leah. Pray for protection of her lungs from further damage. We invite you to come along side of us as we pray and hope for a cure to be found! There's a First Time for EverythingBUT, this is a first that we didn't want... but knew to expect at some point. Please pray for Leah. She is having a significant pulmonary exacerbation and was hospitalized on Tuesday. This is her first hospitalization. So everything feels a little traumatic, but she is such a trooper!
She had been home from school all last week with increased cough and congestion. We thought she was probably fighting some type of virus. But rather than getting better over the weekend, her cough worsened and was deep and persistent. She also ran a high fever and had very little appetite, so she barely ate anything. We brought her in to see her CF doctor on Tuesday. He wanted to examine her, get a chest x-ray, and run some tests. Noticing the changes in her chest x-ray, he decided to admit her to the hospital to recieve two antibiotics intravenously. She started both IV antibiotics on Tuesday and will need to be on them for 14 days. We expect her to be in the hospital for at least one week, or possibly the full 14 days, depending on how she does. Leah had a PICC (peripherally inserted central catheter) line placed on Wednesday, which will stay in for the duration of her antibiotic treatment. This allows her to move around more freely, as it is in her arm, compared to the IV that was orginially placed in her hand. As you may know, we have been concerned about the NTM infection that Leah has. Doctor does not think this is from the NTM infection. Rather, this is most likely due to a pseudomonas aeruginosa infection, a typical CF pathogen. Even though Leah has not cultured pseudo in her throat culture, he suspects that it is present in her lungs. He expects her to feel much better as we treat the infection. She's being well taken care of and is mostly in good spirits. She especially cheered up when her sisters came to visit last night. Between her different treatments and our regular CF clinic team visiting throughout the day, there seems to be a constant flow of people coming in and out of her room checking on her. She even had a magician visit her today, who taught her how to do a magic trick. We will keep you updated as we are able. We appreciate your prayers! |
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We are truly blessed. We want this to be a place we can share updates on how Leah is doing with her CF. As well as a place to share our hopes, dreams, and prayer requests. Thank you for loving and supporting Leah as she fights everyday to add more tomorrows. SubscribeArchives
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