December's CF clinic

Today was CF clinic day. They weighed her at 3.65 kg (approx. 8 lbs) and height of 55 cm (approx 21.5 inches). Little girl is getting a little too long for her weight - they measured her length twice to make sure she had grown that much. Oxygen was at 97.

They do a throat culture every month to see if she is growing any bad bacteria in her lungs. Usually those results are posted within a week to our online link and I'm sure they would call us if it came back abnormal.

Her Creon enzymes have been upped to match the amount of calories she is eating. From 1 pill before each feed to 1.5 pills and we are to fortify the breast milk with 1tsp of formula (was 1/2 tsp) to catch her weight up to her height.


Good news that I don't think I've posted here. She was approved for TennCare. The hope is that whatever our other insurance doesn't cover will be covered by this. We have the card in but the info hasn't been sent yet. I can now go to Child Special Services at the Health Department and finish filling out their paperwork.

Also another good thing -- the CF Care Foward finally got all our info and sent vitamins for her. To explain the need for vitamins - The fat-soluble group has vitamins A, D, E, and K, which are found in fatty foods and oils. After we digest them, these vitamins are stored in fatty tissue inside our bodies, ready for future use. Because people with CF have trouble digesting fatty foods, they also have trouble getting the right amount of
fat-soluble vitamins.

For the next year our visits will be monthly then after that hopefully it will go to quarterly. So right now our next visit should be in January. We have decided at that time to go ahead and have the genetic testing done along with the annual labs (hemoglobin, etc).

Random Musings

Just a few random musings that don't fit into other posts or make for very exciting FB statuses.

We went today to pay off the main maternity hospital bill. Our insurance doesn't cover maternity so we had some slotted aside specifically for this. We had talked to them previously about paying in full and getting10% off of the bill. Well apparently there is a new bill that says if you have no insurance then you get a discount similar to what insurance would get. So instead we were able to get 27% off the bill. Yay! Now I might actually be able to do a decent Christmas for the big girls. Plus we ordered my Christmas gift from Lowe's today - a standup freezer. The bigger one was on sale for only $50 more than the small one so we got the big one.

The reason I want a freezer is store frozen breast milk in. With the older two, I only made it to 6 weeks with a combination of breastfeeding and pumping. With Olivia I did about 2 weeks of breastfeeding, then about a week of combo pumping/breastfeeding, and now all pumping. We are up to 10 weeks, plus I have about 50 bags frozen in the refrigerator freezer. It is so much more important for her to have breastmilk as it is easier for her to digest. Pumping is easier on us than the actual breastfeeding, because this way I can add extra calories to the bottles from formula and add salt. Also I was told that the enzymes could irritate skin and I prefer not to have that part of my body irritated. I'm making small goals as far as pumping. My current goal is to make it to the end of December. I'll set a new goal if I make it that far.

We had to move Olivia's Creon prescription to WalMart because Walgreens was having trouble getting it in and WalMart already carries the Creon6 for some other folks. This is her digestive enzyme so she HAS to have it in order to digest her food. Mike (hubby) didn't think to transfer the vitamin scrip over so we are still waiting on it. I need to get him to check and see if WalMart can get it in quicker or already has it unless Walgreens has gotten it in. He knows a couple of pharmacists at both places so I've been letting him take care of the scripts most of the time. Still waiting for the CFCare to send vitamins too. Starting to get frustrated with the whole vitamin deal.

We have a postal scale here at the house that goes up to 13lbs that we have been weighing Olivia on. It is pretty close to the one at Vandy. All the doctors' offices usually weigh with diaper only. We've discovered that clothes add approximately 3-4 oz. I weighed her last week and she weighed 7 lbs 9.4 oz. So I'm guessing that she is around 7 lbs 6 oz now. They want her to gain 1 oz a day but she is gaining closer to 1/2 to 3/4 oz per day. As long as she is gaining, I'm ok with a slower gain.

2 month immunizations and other stuff

Yesterday was immunization day. Poor baby got two shots in one leg and one shot in the other plus oral drops. She was miserable last night, but is feeling much better today.

I also had Alexis and Eliana get their flu shots while we were at the health department. They recommend anyone who is consistently around Olivia get the flu shot. I got mine the day we went to the health department to talk to Child Special Services (CSS) and to sign up for WIC. I feel weird being on the WIC with us being self-employed but it has helped tremendously with the groceries since we are now starting to get all the doctor and hospital bills.

We are still waiting for her vitamins and next level of CREON to come in to the pharmacy. The CF Careforward people called a few days ago and asked for some corrected information so hopefully they will get her on that then we will be mailed her vitamins for free.

I also had to get some more information together for DHS for the TennCare paperwork. I have to take that over today, but at least that means they haven't automatically denied her. I still don't think we will be able to get her on it, because we have too many "resources", but I have to get the denial or approval letter before CSS can do anything for us at all.

Two month visit at the regular pediatrician

Did our regular pediatrician visit this morning. So far she is on track for all her baby milestones - hearing, sight, pushing up, etc. Updated them with what meds she is taking so they are kept in the loop and will be able to check for conflicts if they ever have to prescribe something to her. Don't have to do another visit with them until January for her 4 month well-baby checkup. I do need to take her to the health department some time next week to get her 2 month shots though.

Grow Baby Grow!!

Good visit at the CF clinic today. Olivia is now at 6.83 lbs (3.1 kg) and 19.5 in (48.5 cm) which are both under 3% on the growth charts. However, her BMI is 12.7 which basically means her weight versus her height is at 32% now instead of 9%. So she is creeping up to that 50% that they want her to be.

The company that was supposed to send us special infant vitamins for her hasn't so far so they sent in a prescription to the pharmacy for those. If we get in the others, then that will just be extra that we have. Besides the vitamins, we are adding Zantac. She has a tiny bit of reflux, plus it will help the enzymes work better and gives the vitamins a better absorption rate. Since she is eating a bit more they upped the enzymes from Creon 3's to Creon 6's. We only have to use one pill per feeding of those. With the 3's we were using 1.5 pills per feeding.

We don't have to go back for another month. Hopefully by then we will know if the insurance will cover the genetic blood tests that way we can get them done while we have used up all the deductible.

About the added salt to Olivia's diet

I was asked about why the added salt to Olivia's diet. I found this text which explains better than I can. http://www.aboutcysticfibrosis.com/cystic-fibrosis-salt.htm

Salt Loss in Cystic Fibrosis

CF affects the body’s ability to move salt and water on the cellular level. The protein created by the mutated CFTR gene is attached to outer cell membranes of exocrine glands and organs. This protein acts as a channel that connects the inner cell material or cytoplasm with the surrounding fluid environment. In sweat ducts, these proteins facilitate the flow of chloride from the sweat to the cytoplasm.

Defective CFTR proteins result to chloride ions being trapped inside the cells of respiratory airways and outside the skin. Negatively charged chloride ions tend to attract positively charged ions outside the cell and this includes sodium, the most common ion. Chloride and sodium combined produces salt, which is lost in high amounts in CF patients. Salt levels in cystic fibrosis also upset the blood’s mineral balance and often leads to fever and dehydration especially in hot weather.


Cystic Fibrosis and Salt Level Management

Managing symptoms and salt levels in patients with cystic fibrosis include medications, nutritional supplements and pancreatic enzyme replacements. CF patients need additional salt and fluid intake to make up for excessive loss of it. Any food that tastes salty like chips, fast food, frozen dinners are good sources of the mineral for CF patients.

Salt is essential for patients with cystic fibrosis for muscle function, nerve cell health and to improve the flow of fluid in cells. Since patients are losing higher than normal levels of it when they sweat, replacing this loss is very vital. Infants with cystic fibrosis must have salt added to their formula or baby food. In children, teens and adults, sodium chloride levels can be maintained by adding liberal amounts of the mineral to their diet, especially during hot weather. The doctor or health care provider can help patients determine the right amount of salt for them.

Infant CF clinic visit

Today she weighed 2.8 kg or 6.16 lbs and was 48.5 cm / just over 19 in long. Not sure what the ratio of weight to length was, but she's not gained as much as they want. She averaged 23 grams per day gain and they wanted 30 grams per day. The reason they want her in the 50th % for BMI is that research shows better lung development and although the lung keeps making new cells most are made during the first two years.

They upped the amount of Creon (her digestive enzyme med) to 1.5 pills per feeding. We are fortifying the breast milk with 1/4 tsp of a specialty formula called Pregestimil which stayed the same this visit. We are now having to add salt to her feedings. In a 24 hour period we add 1/8 tsp of salt. Basically spreading one small packet to 6-7 feedings.

We go back in two weeks. If she increases how much she eats at one time, how much Creon she gets at each feeding will be increased. This will probably happen at every visit for a while.

Two crazy days ahead of me

The next couple of days will be a bit crazy. Tomorrow, we have to be in Nashville early for the Infant CF clinic. Yay, we get to drive through morning rush hour traffic, but I would rather go early and get it over with than drive through the evening rush hour traffic.

Olivia has been fussy the past few days, not sure if it is just normal baby fussiness, her having gas, or if it is acid reflux (which they told us at the clinic that CF patients tend to have more often than norm). They told us we would be constantly wondering if things were normal kid stuff or caused by the CF now I'm beginning to see what they mean. I weighed her earlier and she was at 6 lbs 3 oz. They want her to be in the 50% for weight vs height (BMI). Last check she was at 11%, we'll see how much closer this gets her tomorrow (and how accurate our little scale is.

Thursday, I have an appointment at DHS to try and get Olivia on TennCare to help offset some of her future expenses. Even if she/we don't qualify I have to do this before the Child Special Services at the Health Department can finish they're paperwork. We should qualify for CSS services even if not for the TennCare. It would be nice if she qualifies for both. Even nicer if I didn't have to jump through all the hoops.

Paperwork, paperwork, and more paperwork

Spent most of the day yesterday at the Health Department filling out paperwork to get Olivia on any assistance that we can. I now feel like the government knows more about me than I know about myself.


She had her normal pediatrician visit last Thursday, the day after her Vandy visit. She'll go to the ped for checkups for regular baby stuff; i.e. is she rolling over/crawling/walking, etc. at the appropriate age-range. The cool thing is even though we had just gone to Vandy the day before, they had already faxed all the info from that appointment to our ped. So I don't have to worry about their office being on the same page as far as her treatments go. They'll already know everything that Vandy has told us.

Unless she gets sick we don't go back to the ped until Nov. Around the same time we'll have to do her 2 month shots at the Health Department.

Next week we go for an infant CF clinic visit at Vandy. Plus I get to visit DHS to see if Olivia qualifies for TennCare which we have to try for in order to get the Child Special Services (CSS) at the Health Department. She doesn't have to get approved for TennCare, although it would be nice, we just have to have a denied or approved status to give to CSS before they can process our application.

Vanderbilt Doctor Visit for October 12th

Today we had our second visit to the Vanderbilt Cystic Fibrosis pediatric clinic.

One of the things with CF patients is that 95% of them have insufficient pancreatic function. Olivia is one of these. So her pancreas is clogged and the enzymes needed for her to correctly digest her food are not released to the small intestine. So last week she was started on a small dose of digestive enzymes to help her digest and gain weight. They would like her weight to be in the 50th percentile as this will help her lung function in the future. Currently she is close to the 11th percentile which is up from around the 6th percentile from which she started. We are to increase her dosage to a full enzyme pill with every feeding. Since she is unable to swallow a pill at this age, we have to open the pills to get the small beads out and get them far enough in her mouth that she will swallow them.

We will also be adding some vitamins to her feedings soon. Certain vitamins are not well absorbed by CF'ers. Along with a tiny bit of specialty formula in with her expressed breast milk. This will increase her calorie intake and hopefully she will have a quicker weight gain.

Today we are also adding 'breathing' treatments. At least two times a day we have to help her jar loose the thick mucus secretions that are in her lungs.

As she gets older, we may have to add many more types of treatments. Hopefully by being pro-active and preventive, we can lessen what all she has to have done.

My original post on October 7th

This is my original post on Facebook on October 7th after we had learned about Olivia having Cystic Fibrosis.

OK. An update on Olivia. One of her original newborn screenings came back with an abnormal reading so last week I took her to the local Health Dept. and had the screening re-done. So this Wed. we got a call that the screening still came back abnormal. So we spent all day yesterday at Vanderbilt with the specialists for that particular screening to check for possible problems.

The good news - we got a same-day diagnosis. No waiting.
The bad news - she has cystic fibrosis

Good - It's treatable
Bad - It's NOT curable 

I would post more on it but I'm still trying to get my head wrapped around all the info.