Madeline's Journey

It's been quite a while since I have updated this blog with much news from Madeline. Usually that means that Madeline's health has been going well. This is certainly true!  We have been staying healthy through the summer and, we couldn't be more thankful for that. Sometimes CF takes unexpected turns but, we have learned to celebrate the healthy stretches. Our quarterly, CF Clinic visit at Hopkins is coming up next Friday, August 9th and, we will hope for good news then too.

 

 

We are continually grateful for those that have supported us through the years by donating and walking at our annual Great Strides Walk for Cystic Fibrosis. This year we will be walking in Manassas, VA on September 28th  for Madeline and others that have CF. The money that is raised for this event goes directly to research for a cure for CF. It's not too late to sign up to walk or donate online. You can do this by clicking below.

 

 

 

                       Please consider donating to Madeline and others that have Cystic Fibrosis.

You can donate by visiting this link:

 

http://www.cff.org/Great_Strides/SandyAndJackLong8806

 

We thank you all from our hearts.

 

 

 

 

 

And oh boy...has she grown this summer.....

 

 

 

 

 

 

 

 

 

 

April CF Clinic

We left for CF clinic this morning. It was a pretty rainy morning driving through DC and onto Baltimore to see Madeline's doctors at Hopkins. After a few traffic snarls, we arrived and waited to be checked in. Madeline chose to do the ipad while she waited to be called back. It's a plus during clinic day.

(Thankfully, we got through the winter months with only 2 colds. Both colds ended with a lingering cough, which we used antibiotics to treat. Luckily those coughs disappeared  after antibiotics. We felt pretty good about going into this clinic visit knowing that she had a pretty good winter.  )

Once we were called back to CF Clinic, we met with the pharmacist (to review and change if needed) her medication list, the nutritionist (review what she was eating and how see she was getting those extra calories and fat that's required for CF patients), her CF doctor, CF nurse (use of the Sidestream nebulizer), and finally the Respiratory Tech to go over breathing techniques.
We were very interested in the meeting with our Respiratory Tech, Ms. Karen. She was able to go over some breathing techniques that would be beneficial for Madeline to learn that would help her clear mucus in her airways. Many adult CF patients use this technique called "huffing." It requires a patient to understand how to follow directions and  Madeline is at an age when she can begin to do this.

Huffing is described as breathing in using your diaphragm, holding your breath, and forcefully blowing through a wide open mouth. Ms. Karen allowed us to practice this technique with Madeline so we could help her practice this at home. Usually CF patients do this during their VEST treatments but because Madeline is so young she recommended doing a few huffs after her treatment is over. Eventually as she gets older, huffing will become part of her daily breathing techniques while she does the vest. Madeline also practiced breathing games with Ms. Karen such as using a straw to move a balled up paper towel.

Ms. Karen and Madeline doing a breathing game.

We also met with our CF Clinic nurse, Ms. Donna and discussed trying the Sidestream Nebulizer. Usually this neb is used when children are around 5 or 6 years of age because it requires them to breathe through their mouth in order to get all inhaled medications adequately. We will begin working with Madeline on using this so eventually she can get away from using the pediatric masks and use other nebulizer with mouthpieces. It was recommended that we find a nose clip to help teach Madeline to breathe through her mouth until she is old enough to fully understand. It also takes some practice getting use to holding the Sidestream upright and resting it in your mouth. We hope by practicing with it Madeline will get use to it and other nebulizers that have mouthpieces.

sidestream nebulizer

 Overall, we had a good clinic visit. Madeline's doctor mentioned that her lungs sounded clear and her BMI was great at 87%.  We will wait for her culture results to come in early next week. Hopefully the dreaded Pseudo won't be there but, our intuition tells us that it will most likely make its appearance.

Regardless, we are very happy that Madeline is thriving despite Cystic Fibrosis and pseudo.

We are just thankful for our healthy, happy little girl!



After a long day with countless nurses and doctors, we gave Madeline a "Pinkalicious" doll. She is in love with the pictures books in the Pickalicious series. She didn't let her doll out of her arms and took it to bed.



The FASTER life....

After weeks on the phone with the the Cystic Fibrosis Pharmacy and battling insurance issues, our eRapid nebulizer arrived today.


Arrived!

 Not only can this machine nebulize inhaled medication fast but, it is capable of handling MANY of Madeline's inhaled medications. We have used the TRIO and Altera neb machines in the past but, those are aimed at delievering only a specific inhaled medication (like TOBI or Cayston). The previous machines are very similar to the eRapid, but I love that we can do any of MAdeline's medicines in this one little, FAST, and VERY quiet machine. (All three thingsI love.) It's SUPER FAST! ( 6 minutes for TOBI which takes 20-25 on a regular nebulizer) There is also NO sound coming from this little handy machine which made me realize just how LOUD our regular compressor is all the time, and how loud all the ipads and tv shows really are while we are doing treatments. After her six minute dose of inhaled TOBI, I was left thinking...."IS this REALLY effective? That was just too easy!"

The truth is that the effort lies in cleaning and disinfecting this thing. It isn't terribly hard, and if you have used the other similar machines ( as mentioned above- TRIO or Altera) then it pretty much is the same step by step lengthy process. Really, that is what makes it more time consuming. Each part of the mouthpiece has to be soaked in soap and water, rinsed, boiled (or microwave sterilized). This is typical for most nebs but, the aerosol head is what separates the lengthy care.

This is what an aerosol head looks like.


aerosol head

 It is the most essential part of the machine and because of that it requires a lot of babysitting. It requires distilled water to rinse off the soap and water, touching it only by its metal prongs. Afterwards, it must be soaked in alcohol before letting it completely air dry on a paper towel. Then it is ready for its next use.

The e-rapid also requires a bit of an assembly process but the great part is that it easily can accommodate a child's mask with connector from PARI. It's great since Madeline hasn't quite learned how to breathe using the regular adult mouthpiece.



All the pieces for the mouthpiece






eRapid Mouthpiece assembled with child mask



Madeline was easily able to hold this on her own while doing the ipad. Once it's done nebulizing the medication, the machine beeps twice and a check mark icon appears on the machine's window.

Base (shows light up screen with prompts)

The whole machine costs around $1000. There was no way that our insurance would have paid for that nor were we going to fork out that amount! But after speaking directly to CF Services Pharmacy, we realized that we already had the bulk of the machine. Apparently, the Altera machine base is the same (which we already had from doing Cayston last year) but, the mouthpiece is different. For a little over a hundred dollars, we were able to just purchase the the mouthpiece in order to officially have an
 "eRapid." I have noticed the medicine reservoir is deeper (allowing it to hold more medicine) and the head is designed to nebulize many types of medications (unlike the other machines.). So, if any other cf families have an Altera machine....guard it with your life. You have a very prized possession.

Entire eRapid System (includes mouthpiece with mask and base)

After Madeline tested negative for Pseudo last culture (the first time since her hospitalization after getting IVS), our clinic gave us the choice of stopping the TOBI (antibiotic to treat Pseudo) or to continue it for a few more months in case it could come back. We chose to continue with the TOBI in the hopes that the next month with keep the Pseudo away and give it less chance of it ever coming back. We hope that once we get another negative culture we will stop the TOBI altogether. I cannot wait to give all these machines a rest. But until then,we are enjoying our 6 minute, quiet E-Rapid!

My champion using her eRapid for the first time!

You can read more about the machine here. As of now, it is only sold through CF Services Pharmacy.
It is manufactured by PARI.

Happy Birthday Madeline!

It's hard to believe that Madeline turns 4 today. Madeline was due on March 19, 2009 but, instead I was induced due to hypertension. Madeline graced our world on February 19, 2009 (exactly one  month before her original due date)  just as Jack was leaving on deployment for 6 months with the US Navy.

Daddy got to hold his daughter before deploying that day.

24 hours later, Madeline was rushed by ambulance to the NICU at the local childrens hospital.

She had an abdominal obstruction which required emergency surgery. Jack was flown home and a few weeks later we learned that Madeline had Cystic Fibrois

Madeline stayed in the NICU for a month. We finally got to take our baby home.

Madeline left the NICU and came home for the first time ever on her orginal due date

March 19, 2009 (one month from when she was born.) This was our last day at the NICU.

                                                            
                                                       1st Birthday- Thriving and chubby!

                                                         2nd Birthday- Stronger and bigger!

                                                            3rd Birthday- So happy!

                                                                    4th Birthday! -So grown up!

                                                      


And today we are in awe of our strong little girl. It's hard to believe that 4 years ago she was so tiny (3rd percentile) and went through so much. She was become such a happy, healthy, and sweet little girl.
We are so grateful for each birthday celebration and the many more to come....

Happy Birthday Madeline!                                                 

 

Clinic Day

We stopped our endless cycle of one month on, one month off of TOBI a few days ago. This cycle will continue indefinitely like it has for the past year or so due to the effects of Pseudo never wanting to actually leave. It's a constant frustration but clinic days like these prove that Pseudo has nothing on us and Madeline's well being right now....

Registration and waiting to be called to nurses station for stats check

  Friday was Cf Clinic day. We started with the usual nurses station. They get a few numbers. Madeline weighs in at the scale, gets her height measured, and her oxygen levels. She is a true trooper doing everything that that was asked of her and acted much older than 3.



height check at nurses station

We then were ushered into CF clinic and a room to wait for the specialists, head nurse, doctor, and pharmacist to all talk about Madeline during her clinic visit. It always tends to be a fairly lengthy appointment since we do end up talking and discussing cf with many different  specialists.



Doing ipad with Daddy while waiting for more specialist to talk with at clinic

 We were able to talk to the head nurse and pharmacist about a new nebulizer system that will help minimize the amount of time it takes to  do all of Madeline's inhaled medicines. Our 2 hour treatment window of inhaled meds twice per day could be greatly reduced. (And after explaining that we were starting Madeline in school this fall, we were thinking this would be a great device to have to simply get us out the door quicker in the mornings.


We were very excited to ask about the eRapid Nebulizer System that allows CF patients to do all the same inhaled medicines we are currently doing in about half the time frame. We realized at our CF Clinics Family Education Day (after talking to the CF Services Pharmacy Rep), that we already own part of this machine that was given to us years ago for a different use. The only part of the eRapid that we needed was the handset/mouthpiece which is relatively inexpensive to purchase if our insurance does not cover it. Our clinic was very supportive and is in the process of working to get this nebulizer system in our hands. You can read all about the eRapid here.

Madeline talking to Dr P while she is listening to her lungs.

Dr P was able to listen to Madeline's lungs, check her ears, check her gut, and go over a few questions with us while Madeline showed her  SUPER brave side. She pretty much took over her own appointment asking if the doctor wanted to look in her ears, get a throat swab/culture, etc. Madeline asked if she could sit on the exam table. We were blown away with how incredibly brave and confident she was being only 3 and having gone through the scary sides of hospitalizations, clinic, and unpredictable medical staff. We were so proud!

We finished off the clinic appointment with a BMI of 87 and a weight for age percentage of 92%. The doctor was very pleased with Madeline's growth, health, and BMI. We know that every great clinic visit is a celebration and,  we felt very fortunate that Madeline seems to be doing so well right now. There have been times where this wasn't the case with CF Clinic and we often remind ourselves that we have to enjoy these moments.

We headed out to a celebratory lunch at Arrundel Mills Mall



so HAPPY!




Hot dogs at the BW3's and a crown that says "Wild Winger!"

 Finally home. Daddy assembled her new toy while my little fighter pressed on with the same nebs to keep Cf under control.( Cause CF doesn't ever just stop and my fighter never stops fighting!)

And now we wait for the CF Clinics call next week about Madeline's throat culture.... It's more than likely that Pseudo will be on the culture -since it's been there for nearly 2 full years with no luck of getting rid of it (after a hospitalization, a variety of inhaled antibiotics, IVs,, and oral antibiotics. We also hope that no new bacteria has presented itself on her culture.)  It seems apparent that Pseudo has so little to do with Madeline's health right now. She is thriving, she is healthy, she is energetic, she has no cough, and she is happy. We are beyond happy  about the outcome of  this day and our brave, strong, and vibrant little girl! We are truly grateful. Thank you everyone who continues to keep up with Madeline's journey, her cf fight, and holds us in your thoughts We are so thankful.....

"Unskinny" Toddler Pepperoni Rolls- "The CF Way"

Fellow CF mamas and friends-

So I stumbled across a few recipes on Pinterest that was very similar to the one I am going to share. I tweaked the original recipe to make this one more "fatty" and toddler friendly. These pack a BIG punch of fat in a very small roll. Perfect for those picky cf toddlers-especially those that LOVE pepperoni like my little girl.  Plus, you get added protein and cheese. We are always looking for small way to pack lots of fat/calories. These pass the test of being small, fatty, and easyt make!

"Unskinny" Toddler Pepperoni Rolls
(These come out super flaky and easy to chew. They practically melt in your mouth.)


You will need:
1 can of Pillsbury Crescent Rolls -includes 8 dinner rolls
1 package of pre-sliced pepperoni
2 cups (8 oz) bag of pre-shredded Italian blend cheese (or any cheese you prefer)

Directions:

First, unroll crescents into 8 triangles on  a cookie sheet.

Place 3-4 pieces of pepperoni slices on each crescent triangle (you may have to cut pepperoni in half to fit)

Measure out 1/4 cup of cheese per triangle and dump the cheese into your hand and squeeze.

Measure

Squeeze

Creates a long compact cheese roll to put on crescent.

The cheese will stick together making a skinny log.

Place each cheese log on each triangle/crescent roll.

Roll up crescent roll from widest edge to smallest to make a roll.

Then, fold up any edges that are left open on the ends so the pepperoni roll is completely sealed.
(This will ensure that most of the cheese stays inside as it cooks- some will leak out.)

*** You can also use string cheese sticks cut in half but those don't have as much fat which is why I opt for shredded full fat cheese***

cooking- YUM!

Cook at 375 degrees for 10 minutes.

Let cool for 5-7 minutes. Then serve.

Each roll measure about 3.5 by 2.5 inches- They are small and perfect for little hands.

(I plan to refrigerate the rest of the rolls and reheat them  in the microwave for 10 seconds as needed this week. I am sure you could also freeze these too.)




She was "posing". I cut hers in half. She ate it all! -All 17 grams of fat!- ONLY 1 tiny roll :)

(YES, I tried one. It was soft, flaky, greasy, and amazing!)

Nutritional Facts per serving (1 roll)
Calories: 225 calories
Fat: 17 grams of fat

Pre- Christmas and Being Thankful...

It's been awhile since I have used blogger to say anything about CF and Madeline. The only way I can justify my lack of posting is that Madeline's health has been amazing. I have to say that it's been such a blessing to enjoy a long stretch (Since February of this year was her last hospital admission) of great sounding lungs, clear blood work, check-ups, and weight gain. She has only had a few colds to speak of during the rest of 2012 and a coughed that recently cleared with a heavy dose of Augmentin. I certainly hope I don't jinx what is still left of her good health this year. It's been a nearly solid 10 months of feeling as though your life can become somewhat normal and managed in a way that makes us all often forget that CF is still there(despite our usually meds, nebulizers, etc). I always tell myself to enjoy these lulls since CF has a way of rearing its ugly head when you least expect it.

We have been doing just that- enjoying life. Madeline started a ballet/tap class in the summer as well as a music class for preschoolers. It's been so much fun to see her become independent and involved in activities like these. We hope that preschool next year offers similar excitement. She has been asking regularly if she is old enough for school....

And now, we are in the Christmas mode. We have spread out Christmas this year with many family members starting first with Jack's parents, soon with my parents, and then on to an after Christmas celebration with my brother and his wife. It will be a almost 2 week spread of opening presents with Madeline. She has already taken to continually opening presents- whether they are for her or not. It will be a VERY exciting holiday for her and all of us.Again, Thanks for continuing to follow our journey, asking about Madeline, and keeping our family in your thoughts. We feel very blessed with all that 2012 brought us. And we look forward to welcoming more good health and happiness in the new year.

Grandpa with Madeline

Grandma and Madeline