What has been going on...

I have hesitated posting about this since so many people I know are not doing very well or have suffered a loss recently due to CF, Cancer or some other malady. I need to get this out there so other will see the wonderful power of the Lord!! I know some people reading this may not believe it, I cant help that...I can only tell you what has happened!!


Some of you know some of what has been going on..some of you know more, but none of you know everything. I am going to give you a glimpse into the life of Amber Cheyenne Long.


Cheyenne was born at 32 weeks, perfectly healthy. She required no oxygen nor a stay in the NICU. She came home with us at 2.5 days old.




When Cheyenne started having respitory problems, digestive troubles and was failure to thrive…the doctors contributed it to being a preemie. By the time she was diagnosed with Cystic Fibrosis at age 5, she had had pneumonia about 20 times.




Cheyenne has had many hospital stays over the years, which means, she has had many tests to find the many problems she has developed that are directly related to C.F.




In January of 2009, during a very severe exacerbation (lung infection) her doctor decided to do a bronchoscopy (bronch). At that time, they found out Chey has left main bronchial stenosis. Which is a narrowing of the bronchial tube leading to the lung. She was also diagnosed with liver disease and placed on medicine to help. She was also diagnosed with CFRA (cf related arthritis), she is pre diabetic. At that time it was decided Cheyenne would start being admitted every three months for intensive treatment of her CF. Her lung functions we declining. She had a port a cath implanted in her chest for all of the IV treatments she would be receiving in the future.




It was decided she would see a surgeon in STL to see what he could do to help. We finally made our first trip in July of 2009. By that time, Chey had had pneumonia almost 40 times. With documented scarring to the lungs. The surgeon decided it was best to just try and use dilatation on the bronchus to open it up. The hopes were…that it would stay open on its own and wouldn’t require any further treatment.




For the past year, we have been going to into St. Johns every three months for 2 weeks and then doing home IV’s for 2-4 after being discharged. We have been going every three months to STL immediately following her discharge from St Johns.




In January of this year, the STL doctor said he thought he may be damaging Cheys airway more than it already was and may not be able to do the dilatation any more.




In March, Chey was admitted for her 3 months stint in St. Johns and it was discovered that she had high blood pressure and was placed on medicine to help control it, bringing her daily medicine count to 18 different medicines to be taken at least once per day. It was also discovered that she had a minimal amount of air in her left lung due to the fact that the bronchus was closed off again. She had a PFT ( pulmonary function test) done and it showed her lung function to be 47%. When that number reaches the 30’s, they start the transplant evaluations.




April 7th, I took Cheyenne back to STL for the dilatation. Alan couldn’t go because he was just discharged from st johns after having his gall bladder out. The doctor did the dilatation and then came to speak to me. He was very somber. He said it was the worst he had seen it and said he couldn’t go in there anymore because he was making things worse. He said we have two choices.


1) do nothing more and let the bronchus close off and kill the lung, then remove it…or


2) place a stint, which is not recommended because it will cause problems of its own and especially since she has CF, and when it closes off and kills the lung, then we will remove it.


I didn’t have to decide right then. We are to tell him our decision at our July visit.




Chey just couldn’t shake a lung infection, continued to have fever….increased cough and sputum, dispite being on antibiotics…orals and IV’s for all of this year.. So, on May 12th, her local CF doctor decided he would go in and see what was going on in her lungs. He said it was terrible in there and we needed to get her to Cincinnati Children’s Hospital ASAP. He said there is a doctor there that does airway reconstruction and he may be able to help Cheyenne. He continued to say, if this doctor couldn’t do anything to help…in the near future we would have to remove her left lung and list her for a unilateral lung transplant.




So, off to Ohio we go. We get to the children’s hospital on Wednesday..check in and find all the places we need to be on Thursday. Thursday morning Chey has a CT scan. Followed by a PFT. After that, we met with the surgeon to go over the tests results and get a game plan for Fridays surgery.




The doctor came in and said he spoke with the STL doctor at length. He said STL doc told him he wished he had never gone in there because it was a mess and he was making it worse. He also spoke with the local cf doctor and he told him the same thing.




We then discussed the tests from that day. The CT scan was absolutely perfect. Her PFT showed her lung funtion to be 80%. He examined her and said he didn’t think he would find anything on Friday when he went in there. He said he wasn’t 100% sure or he would send us home. He said he would go in there and see what was up and the other surgeon would take a look right after him to see if he could help. And they would decide if they were just placing a stint or doing reconstructive surgery.




On Friday, we went to the surgery unit..checked her in, got situated and waited on the festivities to begin. They said it would take about one hour and 20 minutes. Forty minutes later, they paged us, she was in recovery and ready for visitors. The doctor came in to see us. He said he didn’t know what the other two doctors saw, but he didn’t see anything out of the ordinary. He also said if Chey was his usual patient, he would redo her CF testing because he didn’t see any evidence of it. Lungs are perfect, no scarring whatsoever. The bronchial stenosis is gone. He did say she has some bronchial malacia but it wouldn’t cause her any trouble.




Last Wednesday, I got a call from OH…her culture results we totally normal. She has only had one other clear culture result in her life and that was in May. We had a regular CF clinic appointment and was to be admitted to St Johns right after the appointment. Her doctor came in asking if he wasted our time sending us to OH. We told him what the doctor said, I showed him the pictures. He asked what he said about continuing her medicine and cpt therapy. I told him he said it wouldn’t hurt her to continue..he said he didn’t want to stop it cold turkey, we would wait and when she doesn’t get sick, we will start backing it off. He grabbed my hand and Cheyenne’s hand and said LET’S PRAY AND THANK THE LORD FOR HIS HEALING TOUCH!!!!!


This blog has been all about CF and sickness...I will continue to update on here from time to time but I am starting a new blog and I want it to be my main blog. I will try and post on it on a more regular basis.
You can find it by clicking here. Please follow me as our journey continues...you never know what might happen.

The Life of a parent to a child with CF (Cystic Fibrosis)

Every day is a different day - you hold your breath and curl your toes each time they cough, hoping and praying it's not a precursor to something worse. Like any parent, you sit up at night when they're sick with fevers - but amplify this by holding the nebulizer mouthpiece of a snoozing or coughing child while you stare sleepily into the dark night. While the rest of the world sleeps, some tiptoe quietly into their child's bedroom to infuse a 12:00 a.m. IV treatment. Some parents kiss their children good night after reading a storybook and supervising teeth brushing - while some parents of PWCF also hook up a nightly tube feeding. This of course is after the nightly breathing treatment and favored method of airway clearance, e.g. clapping, flutter, vest, pep mask, etc. During these times, you learn how to operate on less sleep - and with an eye on the medical equipment surrounding you, wonder if it's possible to really take coffee intravenously... You learn to analyze the minute details of the monochromatic contents in a spit cup - and nervously watch for flecks of blood. You grit your teeth if you hear them cough up what sounds like a ton of gunk - to learn they spit into the sink and washed it down before you could check it. Changing the diapers of a baby with CF can sometimes be an exercise in gag control... Your toilets might plug up on a regular basis and you consider buying stock in Lysol products and whomever makes Cottonelle and Charmin... Parents of CWCF rejoice at each inch attained in height and every pound gained in weight - and cry at each ounce lost. School-age children take tests in school - our kids do too, but they also take blood tests, sputum tests and pulmonary function tests - some have bone density tests. School children have photos made each year - so do our kids, but they also get a photo taken of their lungs - and sometimes their sinuses and intestines too. Some endure finger sticks if diabetes is present. We aren't lucky enough to have a grading system of A-F - we have to figure out what numbers best apply to our child and hope human error hasn't cost us an extra stay in the hospital or the dreaded news the child is now resistant to a certain antibiotic. You learn how to hide enzymes in the foods of recalcitrant infants and tots. You learn to read labels for the highest calorie content. You learn the meaning of a well-balanced diet and your expertise in food preparation begins to rival that of Martha Stewart - presentation is 90% of the art of feeding any child. Anytime a Certified Freight truck goes by - you get a lump in your throat. Darn those big red and green CF initials anyway... Aren't we allowed to forget for just one minute? But on the other hand - you become a more compassionate, empathetic, and patient person. Despite trials, you have the opportunity to attain the golden awareness that everything you say - everything you do or don't do - really makes a difference in the life of your children. Every moment is precious. Even the smelly moments... Being the parent of a child with CF enables you to develop an inquisitive and scientific mind - scoping out information about genes, cells, organisms, herbs and treatments. Even if we weren't good at science when we were kids - just look at what we can do now! You also become adept at handling insurance and hospitalization forms... You learn to be gracious and tactful even when you don't want to be, like when a stranger or your child's new barber comments on your child's cold, or a clothing salesperson comments upon sizing difficulties because your child is too thin for his sleeve length... You learn how to be assertive so doctors and staff will listen - really listen to what it is you and your child need, because they are not gods nor mind readers... And even though you try not to push the envelope on the words hope and cure - they are always in your heart. Most every parent sleeps with one ear attuned to the littlest sound - but some parents of a CWCF listen for absence of sound, Is the oxygen tank working? Why did s/he suddenly stop coughing? Why isn't the air purifier humming? Sometimes you find yourself doing a load of laundry in the middle of the night - after your child has coughed so much they've finally vomited on their sheets and blankets - or fevers have soaked everything. In the beginning, you wonder, Is this something I should call the doctor to report? Then you learn to trust your instincts - you know when to call the doctor - and you want to be able to go straight to the top instead of dealing with a revolving door of telephone receptionists or approval from an HMO. As a parent of a CWCF your spelling and vocabulary improves. While your friend's minds are turning to mush from Barney and Mr. Rogers, you're learning to pronounce and spell words like: pseudomonas, aspergillus, and cephalosporins. You get a crash course in physiology and biology. You learn about clubbed fingers, oxygen and carbon dioxide exchange, and the role of the pancreas... ...And Part II of the sex ed talk you have with your teen takes on the added vocabulary word: vas deferens - along with the reminder, Just because you might not be able to procreate, doesn't mean you can go out and test the theory... your risk of contracting a sexually transmitted disease is the same as others in your peer group. After Christmas, while some are taking advantage of sales, you hit the pharmacy to pre-buy meds to take advantage of year-end tax deductions. You ignore the jibes of your friends who think you're obsessive-compulsive because you wash your hands frequently. You begin to resent the fact you have to remind health practitioners to wash their hands before touching your child or the things in his hospital room. You seethe when other parents send their kids to school sniffling, their noses caked with green crud. You buddy-up to the school secretary so when your child wakes up sick, she doesn't get tired of you calling and asking, So... just what is going around the schools right now? You teach your child to share - but stand aghast when your son's girlfriend shares her pre-chewed gum with him. At ticker-tape speed, your mind reels off the cost of antibiotics and vitamin c... You understand that when friends and relatives ask about the well-being of the CWCF, they don't mean to exclude the existence of your child w/o CF. Sometimes you feel like you're in a 3-ring circus. You juggle and balance home, work, hospital, and siblings. You often walk a tight-rope of emotions. You're in the lion's cage facing home-health and insurance issues (who else would hire so many Bozos?). You never hear about people running away to join a home - you wonder why people would run away to join a circus... You worry about your CWCF on their first sleepover. You count your blessings when the parent of your child's friend says, Show me how to do cpt so you can get away this weekend. In some parts of the world, there are certain rites of passage. Having a CWCF is also being aware of rates of passage: height/weight rates, O2 rates, sterility rates, insurance rates, mortality rates, divorce rates... And when people quote, It takes a village to raise a child, you realize just how big your village is, and how very grateful you are to have so many caring and competent villagers on your side.

Surgery update

OMGoodness...We had a major crisis yesterday (grandbaby mama drama) and we also had a death in the family, so that delayed my update. It has been so crazy around here I havent had a chance to update till now.

Cheyenne's surgery went great. It was closed again, so he had to re-open it. Dr. Huddleston says it will need to be re-opened every three months and he doesnt think it will ever be any longer in between and may become a shorter length of time in between. He doesnt want to do the stint right now because the disadvantages outway the advantages, so we will continue to go to St. Louis every three months for about a year and then place the stint at that time. That is what the doctor has said will happen....well, he isnt THE PHYSICIAN, so we will see what HE wants to do and follow that plan.

We are home now and Cheyenne is doing great and is in school as I type this.We will follow up with the local CF doctor the end of the week and by then they should have the culture results, so we will see if she will require I.V. antibiotics.

Please keep my sister, her husband, daughter and mother n law in your prayers.

Love and hugs to all and a great big thank you for all the prayers.

Time Flies

I am so sorry it has taken me this long to post an update...let me fast track to today..lol...

Cheyenne and I did go to church camp..Ciara was able to go with us...I bet Satan wasnt happy about that. Ciara was chosen as Camper of the Year and gets free tuition for next year. Cheyenne had an awesome time.

We went for Cheyenne's monthly CF clinic visit on the 10th. She gained two kilos, PFT's went up 20% and he said she sounded beautiful. He also said she had a remarkable improvement and we dont have to see him until September. We have never been able to go two months between appointments and usually dont make it one month without some sort of contact with him or his office. We do still have to go back to St. Louis Children's the end of September for another surgery on her left main bronchus. The two week followup with her CF doc will coincide with her October clinic visit.

I am pleased to announce the birth of my newest nephew, Greyson Quinn...born 8/21/09. Both mom and baby are doing well. Big brother, Nathan, is very pleased too.Of course school has started and is going great.

Please be in prayer for my granddaugher, Xoey. I will update more about that later.