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One of my sweet friends (Kristina) sells Scentsy. They smell AMAZING! It looks like a candle but is really just a light that warms the wax so it is safe around kids and pets. It’s paraffin wax that doesn’t get hot enough that it would burn you. You pick a light you love (they are all beautiful) and then you can get different wax to switch out your smell! She is giving 25% of every sell to the Cystic Fibrosis Foundation on sells this week until the 27th. Get your Scentsy now

https://burtonk.scentsy.us/Home

I have to say I have been doing MUCH better. I’ve felt very positive and we have had many memories and fun times this week.

Tomorrow is clinic. Clinic my friends will turn your world upside down and dump you on your ass. I noticed when I mentioned it was clinic week to a mom with a healthy child I got that look of, yeah so. I decided that tonight I would write why every CF mom HATES and DREADS clinic.

It is a realization. You’re child is sick with a life threatening disease and that is the only reason you are there.

You’re going knowing that this could be the month you get bad news. The anxiety sets in…

We go on Derek’s day off. Luckily twice a month Derek has a weekday off because he works Saturdays. I wait outside with Ginger and Derek goes and checks in. This is because we are so scared of cross contamination with other CF kids. Our angels can catch bugs other kids can not and are not aloud to be around each other. I walk in a straight line back and forth in fount of the building trying to be brave and hold back the tears.

When we get inside Derek wipes down the room (anywhere we might have to touch) the table and chairs mostly. We replace the paper on the bed with new paper and pretend we are not OCD when the nurse calls us in to weigh Ginger. I go in with her, take her clothes off while holding her with my knee. I have them re-clean the scale and where they measure her height in front of me. We wait while it drys. I try hard to make small talk. I hope they will like me enough that the extra work wont piss them off. I then ask as nice as I can for them to wear gloves before they touch my child. This sometimes gets the eye role. I smile and thank them (cussing in my head.) Then you start to pray for good news.

The RN comes in first asking a million questions and about the list of meds your child is on. It’s funny how I can’t remember who I need to call back or what bills are due, but I can name off meds, doses and times like it’s nobody’s business. We talk about growth and poop and questions Derek and I might have. To be honest I try to spread the questions out through the doctors otherwise they get annoyed I always have so many. Most of the time I’m fishing to hear them say Ginger will live a long life.

The dietitian comes in and we talk enzymes and vitamins, poop, acid reflex, and heath. They adjust doses.

Then comes the Pulmonologist  (I start to sweat)  they listen to her chest as she breaths. The room is silent and all you can do is pray that her lungs are clear.Then they check her O2 stats and look in her ears, nose and throat. This is the most knowledgeable person we will see and so we ask many questions. We talk about growth and development and any blood work, cultures, or Xrays that need to be done that week. Sometimes meds change or increase.

Depending on if you got any bad news or if you’re a crying mess after some news you got you might get to see the social worker. She helps you not kill yourself. Ha! Really though I know how stable I come off by if she “comes by for a visit” or not. She also figures out how you will pay for all the new meds too.

You might also see the Genetic Counselor, phlebotomist, and students. This appointment can last from an hour to 3 or 5!

Here is why CF families have such a hard time with clinic. It’s so uncertain. You NEVER know what you’re going to hear. You could hear all is good and normal or you could hear go home and pack your bags you’re getting admitted to the hospital. All of it is enough to make the strongest mom or dad crack. The smallest change in meds or the smallest bit of bad news set you back at square one. You never get too far from that square with CF. You think your on top and then BAM you’re where you were the day you found out. Helpless and begging for answers.

Please pray for Ginger tomorrow. That she is healthy and strong through it all and that the week to come waiting on the culture results don’t kill me. Love you all!

Update on my search for comfort and hope…

I told you I emailed the pharmaceutical co developing the drug Ataluren/PTC124, well they actually wrote me back! Here is their email!!

Dear Sarah and Derek,

Thank you for your interest in ataluren. I’m happy to answer your questions where I can but, as you suggested, I can’t answer some of them. We hope to find answers for your first three questions once we have the results of our current trial. As for the others:

Timing: the current trial is still recruiting. It will run for approximately one year after the last patient enrolled receives the last dose. Then it will take several months to analyze the data and submit it to the FDA for approval, which can take up to six months. So probably the earliest is late 2012 or early 2013.

Dosage: Dosing is by body weight so adults will take more drug than children but it is because they weigh more, not because they are older. In the current trial, the dosage is 10 milligrams of drug per kilogram (2.2 pounds) of body weight in the morning, 10 mg/kg at midday and 20 mg/kg at night. Ataluren is a mild vanilla-flavored powder that is mixed with milk or water.

Age: Right now it is only approved for use in a trial for children six years or older, so if the trial is successful and the drug is approved it will be only for that age group. If the trial is successful we will consider doing trials in younger children. Age six was specified because the trial’s primary endpoint – the measure we are using to determine if the drug shows a significant improvement when compared to placebo – is FEV1 as measured by spirometry, and children under age six cannot perform spirometry with reliability. A different endpoint would have to be chosen if we opened a trial for younger children.

Long term: theoretically the drug should remain effective but we will not have the answer to your question until the drug is approved and there is experience with long-term use.

Safety: ataluren has been studied in a variety of clinical trials in healthy volunteers and in patients.  It was given to 38 patients with nonsense mutation Duchenne muscular dystrophy and 77 patients with nonsense mutation CF who participated in the Phase 2a studies and was generally well tolerated.  Adverse events were infrequent and usually mild to moderate in severity.  A few patients with CF sometimes had mild burning with urination but this did not result in therapy being interrupted or discontinued.  There were no safety concerns based on physical examinations, vital sign measurements, electrocardiograms, or laboratory test results.  A Phase 2b pivotal study of 174 boys and young men with nonsense mutation Duchenne/Becker muscular dystrophy was completed late last year. Safety data from the study showed that ataluren was well tolerated and no participants discontinued treatment due to an adverse event.

If you would like to receive reports about ataluren in CF I invite you to join our email update list by following this link to the “contact us” section of our website and signing up: http://www.ptcbio.com/5.0_contact_us.aspx

I hope this information is helpful. Please feel free to contact us again should you have additional questions.

Best,

Diane

Diane M. Goetz | Director, Patient and Professional Advocacy | PTC Therapeutics

So I wrote her this:

Thank you so much for all the information Diane. It really means the world to me that you would write me back so quickly and with so much information. I just feel like the more I can find out the better I might feel. Our little girl , Ginger Blue is 16 months and my life. CF is such a terrible disease. We are so grateful for the work PCT Therapeutics is doing. This drug could possibly save my daughters life. Thank you ALL for what you’re doing. You’re our heroes.
Much Love, Sarah

and she wrote back!

Oh, Sarah – Ginger Blue is precious! I have shared this with my colleagues and a copy of the photo now decorates my office. Thank you so much for sending it and for your kind words. Seeing how our work might help makes us all want to work even harder.

Best,

Diane

This was good… Although I wish they would tell me, “Oh yeah, this will make her all better, forever.” I guess I’m not going to get that answer. I was really surprised and disappointed to hear she will not even be able to take it until she is 6. This was news to me. On the up side it is neat to know that Ginger’s face is in the building that a medication that could save her is being worked on. Makes me smile……

KeEp PrAyInG fOr A cUrE!!

This week’s flash, your favorite piece of art in your home! This was hard to do because Derek is such an amazing artist. How could I pick one?! I narrowed it down to two by Derek and one by a dear friend that touched my heart with her art. Here they are!!! (sorry about the glare)

The Virgin Mary

Our dog Tobey

My Birthday gift from Robin

Every single day I pray over Ginger’s body. I pray for healing, for strength and safety. I give thanks for her health today. I pray that God will never let us feel the pain other CF families have felt and continue to feel. I feel guilty to ask God not to put through that, because I feel like no family should go through that kind of  heart ache. Being a mom is hard enough on the good days. Just trying to make sure they grow into a well rounded, honest, loving person should be enough. Add health problems on to that and it’s all a bit much to take.

I knew even as a little girl that my biggest dream when I grew up was to be a mommy. That was really my hearts desire. To finally be blessed with a child is my biggest gift. Ginger is my reason for everything I do. I NEVER imagined it would be this great or that I could love so much. I just pray I will never lose that. I would switch her places in a second. I would take every test, every hospitalization, every treatment, every fear to never let her know what it was like. I wish with all my heart I could do this. I know that she is so young and know no difference but that also breaks my heart. She should get up and eat without meds and then play not worry about starting treatments in time to have her down for a nap. This is all she knows though and I can see strength in her because of what she has gone through. She is an old soul that knows a secret the world hopes she will tell them someday. Everyone wants to be close to her to hold her hand and steal her heart. How did I get so lucky to know her best. To have her love. To be the one she reaches for. I know God has a plan for her and it is to touch lives. She does that everyday. I pray Gods plan for me is to keep her. To outlive her. To never miss her. To never see her suffer. I have so much hope in Gods plans and I believe her long life is part of that plan. We continue to watch the news on the drugs that have the potential to save Ginger. Ginger has a type of mutation they call a nonsense mutation. This is when the body has a premature stop sign when making the protein. The protein can not work correctly because it was not formed completely.  This drug would tell the body to read through that stop sign and finish making the protein. Some how it does not mess with the other stop signals in the body which to me to a miracle. If this can make enough correct working protein in the body then it would be our CURE. Oh how sweet the words.

Here is a picture of how it works.

Today a guy wrote on the Cystic Fibrosis Foundation’s Facebook wall that he started this drug on trial. This is what he said…

“Good news everyone, I started the PTC 124 study for anyone with the X mutation (stoppage gene). This drug is absolutely amazing, I don’t think I’ve really coughed in 3-4 days since I’ve started it (I haven’t done any of my normal meds either, tho I should be). I feel amazing, and have the most energy I’ve ever had. Tonight I’ll re-start my normal meds in addition to this phenomenal drug. This is it!!! Good News for everyone with X genes!!!”

This could save Ginger!!! Please pray that we can get our hands on it before her lungs are damaged and that there are no bad side effects and that we can find drugs like this for all the different mutations that cause CF. Vertex is another company to watch they are working on 2 different drugs that could save lives too.

This is Ginger in her new blue vest. She LOVES it!

New vest happy dance.

We went on vacation last week and it was wonderful!! We went up and visited my oldest and bestest friend. This small town has such a special place in my heart. I went to college there for 2 years with Jess and loved it so much I would have stayed for good if I didn’t miss my family so desperately. This trip was full of fun and much needed rest. Derek and I took turns getting up early with Ginger and doing treatments. I really needed the break. It’s all so much more work than people would think. Jess and her mom and sister all went in for my Birthday and got my hair done. I got highlights and a much needed trim. I had confided in Jess that I was really having a hard time. I’ve put EVERYTHING I have into Ginger and I started to feel like I gave up on me. I just really don’t have time to worry about what I look or feel like. There are bigger things to tackle. I sat and looked at pictures and videos of the past couple months and it really shows. I’ve gained weight, my hair was stringy and out grown and I look tired ALL the time. I hear it from everybody. “Are you tired today?” Get use to it. This is me now. I do have to say I will look tired BUT with way cuter hair now! Ha! It made me want to try again. I don’t want to give up on me. So I started eating better on Monday and I’m determined to lose the 14lbs I gained over the past few months. I really do know, more than anyone that the only thing that matters is that I’m healthy but it is so hard to feel uncomfortable in your own skin, to not want to try on clothes without crying, not not want your picture taken. So I’m taking a stand. I will stop eating off the VERY high calorie plate of Ginger. I will not think I can eat the same things as my bottomless pit husband. I will take pride in me again. I’m telling you all because I think I will hold myself to it if I share that I’m going to. So I got the new hair and I working to feel better about myself again.
Here are some pictures of our trip. You can see why I would love to live there again someday.

Thank you Rosie for telling me about this song. It’s beautiful.

http://www.cff.org/Great_Strides/SarahSampson6603