Double Whammy!

It's been awhile since I've updated. Bennett WAS doing well from July up until the first week of September. She had two whole months that she was doing well. It all began with a runny nose and cough. We did oral abx and upped treatments. They didn't help! She had a CF appointment on September 24th. Her lungs sounded clear. She was still coughing. PFTs were down from July. Dr. Rubenstein put her on Augmentin again for 14 days and continue upped treatments. She started to show improvements the first few days. Over the weekend her cough had gotten worse and she was bringing up tons of junk when coughing. She was miserable. Working harder to breathe. Easily winded just walking up the stairs. I made an appointment with her Pediatrician so he could listen to her lungs. Yesterday, her lungs sounded very junky, her chest was tight, wheezing in upper and lower airways and diminished airflow on expel. Dr. Dedeoglu decided to put her on yet another 14 days of antibiotics to try to prevent her from getting a post secondary infection on top of everything else that's going on. He said she's having a severe asthma flare up on top of the CF Exacerbation. It's a double whammy and hits her twice  as hard. She's suppose to be taking it easy so she is off of gym for two weeks. I'm suppose to send her to school as long as she's physically able. Because if I refuse to allow her to go when she wants to it will cause her to resent me and CF. I am hoping that with 14 more days of upped treatments and airway clearance that she starts feeling better.  Her cough is very heartbreaking. It is the the worse feeling in the world watching your daughter coughing until she is unable to breathe, her face bright red with a blueish tint. I feel so helpless! 

Surgery Cancellation

As of you all knew Bennett WAS scheduled for two procedures on July 3, 2012 by her ENT Dr. Rizzi. But, before she would have been able to undergo the procedures she had to see her Cystic Fibrosis Doctor and be "cleared". We went to see Dr. Rubenstein on June 19, 2012 at 1pm. Her PFT's have slowly been declining over the past few months. She did PFT's on March 20th and she could only expell air for 1.33 seconds which indicated to Dr. Rubenstein that something was :brewing: so he put her on a 21 day course of Bactrim.

Yesterday her PFT's were 82% well down from her usual 116-119% when she first started doing them over a year ago. She has had a great decline in the PFT's. Well with the numbers that she blew on Tuesday and comparing them to the ones from March and they were not any better. He said that there is DEFINITELY something going on and she needs a more aggressive form of treatment. Admission is the best thing for her right now. He cancelled her procedures until he feels she is well enough to have them done. He also said he does NOT think that her sinuses are the majority of her problems as we all had thought. We were all prepared and ready for her to be admitted. I had already had the :talk: with her about her getting another PICC Line and having to remain in her room for 14 days until it was time for her procedures. Dr. Rubenstein and I both agreed to admit her but then :Father Of The Year: chimed in and said that he would feel better if they tried something else so she was able to be home for his wedding. He mentioned doing TOBI again and another oral abx for 28 days. At this point my blood was boiling. He somehow convinced Dr. Rubenstein to keep her home so she can attend his wedding. 

I spoke up and said that the best thing for her right now would be to be inpatient and get the aggressive treatment of IV abx that she has needed for a long time so she can be better for Kindergarten. Dr. Rubenstein did say to Jason that the longer she is outpatient doing orals and inhaled abx at home and if it does NOT make things improve that it will just cause her to need a longer period of IV's. As of right now, when we go back on July 24th if her PFT's do not improve at all she will be in for 21 days -- not one day less! Basically, Jason is putting his own needs before his daughter's health! Everyone with common sense can calculate 21 days from July 24th will be an approximate discharge date of August 14th and his wedding is August 11th. 

Right now, she is not having any other symptoms (IE: cough or fever) besides a decline in PFT's. They did a culture and we SHOULD know the prelim results Friday or Monday. 

She is growing well! She is 54 pounds and 3 foot 8 1/2 inches tall. 

To sum things up -- The surgical procedures are cancelled for now. She is doing 28 days of inhaled TOBI and 28 days of Bactrim, 4 vest treatments and 2 manual CPTs sessions a day, and upped nebs treatments to 4 times day with the hopes of improving her PFT's!

Surgery Update -- May 11, 2012

I sort of got answers this morning! After calling CHOP for 4 days! I called Dr. Rizzi's Secretary Ryan and FINALLY was able to talk to her. Dr. Rizzi (ENT) said he spoke with Dr. Rubenstein (CF) and Dr. Rubenstein wants to see Bennett so she can do PFT's and see if there was a change in the number and they were to call me with an appointment (which they NEVER called). Before Ryan and I hung up, I had her transfer me to Dr. Rubensteins office because I knew they would answer right away because of another doctor forwarding my call. Dr. Rubensteins Secretary told me that I need to call back at the end of next week because he schedule isn't opened up for the following week. Well she already has an appointment scheduled with Dr. Rubenstein on June 19th because it is her 3 month check-up. So, if they cannot get her in before then we have to wait until June 19th. By then it will be two months since I signed the consent form for her surgeries to be done. Depending on what her PFT's are, depends the course of IV antibiotics she will be on and how many days she will be on them leading up to the surgeries. She is now coughing and fussing that her ears are hurting again. She has already had a perforated ear drum  in April. So it is another two months of suffering with coughing, fevers, ear pain and just plain old feeling crappy.

Recap from previous appointments -- 

Her PFT's dropped 20% from December to March. They went from 108 to 88 but he wasn't concerned with the numbers in March because she was only able to expel air for 1.33 seconds. But, now he is going to be concerned with the numbers if they are lower than 88. I am hoping they are back up to where they have been for a year now (108 or around that number). Depending on what her PFT's are, depends the course of IV antibiotics she will be on and how many days she will be on them leading up to the surgeries. She is now coughing and fussing that her ears are hurting

ENT Update -- April 27, 2012

Bennett had an appointment at her ENTs office on Friday. It was to discuss our next plan of action since the oral antibiotics have not been aggressive enough to clear up the sinus infection completely. He said that it was clearing it up a little bit but not completely. She is currently having a runny nose with bloody/green drainage and a really junky cough! :0(  She is having tons of coughing fits to the point of not being able to breathe and is choking on the mucus. As soon as she is done throwing up the mucus, I hook her onto a nebulizer of Albuterol. I have also been doing her Electro Flo 5000 along with manual CPT plus her vest because she is really junky.

He said that he is going to move forward with the endoscopic sinus surgery and put another set of tubes in her ears since the last set that she had really helped minimize ear infections.

But, before the surgery can be scheduled he needs to meet with her Cystic Fibrosis Doctor first. Dr. Rubenstein will choose the action plan for before and after the surgery. He is going to chose which IV antibiotics he wants her on and for how many days. It depends on what her PFT's are like also with what he decides to do. Her PFT's in December were 108 and March 20th they were only 88 (he was not concerned with the drop in PFTs then because she was only able to expel air for 1.33 seconds and he knew she was sick with a runny nose and started her on Bactrim) Once Dr. Rubenstein and Dr. Rizzi meet and get a formal plan together, Dr. Rizzi's secretary Ryan will call me with the date/time to be in Philadelphia for admission. Also, I have to meet with ARC (Anesthesia Recovery Center) before the surgeries as well so they can decide if she is healthy enough to be sedated, so far I feel she is,  but that is up to them. Basically, right now we are going to be living day to day and not make any big plans since at any given point in time my phone is going to ring and then we are going to have to pack our bags and head to Philadelphia. I hate not knowing when things are going to happen. My main priority is keeping her happy and smiling! :0)

The ball is rolling for the surgeries. I signed all of the consent forms yesterday, which totally broke my heart. She is so innocent and does not know what is coming. She knows she has to go back to CHOP and stay, she understands she will be getting another PICC Line and tubes but does not understand the sinus procedure.

I had asked Dr. Rizzi if doing this FEES procedure will open another pathway of problems for her and he said no. I know other CF Patients that get this procedure every few months and that scared me. He said that with the way her sinuses her, he thinks she will only need this one procedure until she is older. I truly hope that he is right. This was the one reason I was afraid to sign the consent forms yesterday because if infact does open a new pathway of complications for her, I am going to feel like a horrible piece of sh*t. I want only what is best for her and what is going to eliminate complications.  Please everyone hope with me that this will NOT cause her more complications! I know we have some awesome support behind us and if I was making the wrong decision I am sure you guys would let me know. I hope?

Dr. Rizzi agreed with me when I said that the perforation could have possibly been avoided if Barb Jansma would have listened to me when I called AND e-mailed asking for an abx eardrop and oral abx. She argued with me for nearly 4 days that it was her allergies and I was overreacting.Thankfully, her pediatrician Dr. Dedeoglu is amazing and made room for her in his busy schedule and started her on abx immediately. I told Dr. Rizzi straight up that I totally trust him right now because so far he has giving Bennett excellent care BUT if he messes up and breaks my trust then he will not want to have to face me. I told him that I hope I don't cross her path because she will be getting an earful. I know they are only human but I was telling her it was NOT allergies because of her symptoms. The look on his face was priceless. I only speak the truth. 

Well, the curve ball that I had mentioned yesterday is that she has lost hearing in her left ear and her middle ear is not functioning properly anymore and that is from the perforation that she had. It closed up on its own which is what we wanted so she could go on with getting the tubes put it as soon as possible. . I was so pissed that I was seeing red when her test results came back that her left ear was abnormal. Her testing was done once the appointment with Dr. Rizzi was over so I do not know what his thoughts about the abnormal results are. Her right ear is perfectly healthy which is a plus. By the time we were done in the Audiology department Dr. Rizzi had already left for the day since we were his last patients of the day. Our appointment was at 1:40 but we did not leave his office until 4. We were done with her tests at 4:20. I am going to call his office on Monday and ask him what he thinks of her test results. 

Dr. Rizzi agreed with me when I said that the perforation could have possibly been avoided if Barb Jansma would have listened to me when I called AND e-mailed asking for an abx eardrop and oral abx. She argued with me for nearly 4 days that it was her allergies and I was overreacting.Thankfully, her pediatrician Dr. Dedeoglu is amazing and made room for her in his busy schedule and started her on abx immediately. I told Dr. Rizzi straight up that I totally trust him right now because so far he has giving Bennett excellent care BUT if he messes up and breaks my trust then he will not want to have to face me. I told him that I hope I don't cross her path because she will be getting an earful. I know they are only human but I was telling her it was NOT allergies because of her symptoms. The look on his face was priceless. I only speak the truth.

A Whirlwind Turn Of Events

Lastnight around 3:50 pm my cellphone rang! I looked over at it and it said "Dr. Rizzi -- Cell" (meaning the doctor was actually calling me from his personal cellphone). I had a feeling that this phone conversation was not going to go very well since he is calling "out of the blue" and from his personal phone call! When a doctor calls from his personal cell phone it is NEVER going to be a good conversation from my experiences! He first off asked how she was feeling -- I told him that she is still very congested yet she has bloody/green sinus drainage, her ears are still sore and her cough is junky! He said that obviously the oral antibiotics that she has been taking for the past 8 months isn't going to clear things up and get her on the road to recovery! Hallelujah! Hallelujah! FINALLY! After 8 months of Mommy not being very nice and 13 sinus/ear infections/rounds of oral abx, he FINALLY agrees with me that she definitely needs IV abx to kick whatever it is out of her system! This is then where my heart dropped into my feet! He mentioned doing the endoscopic sinus surgery on her. I have been leaning towards wanting her to have this done so she can get some relief but at the same time I am scared. I know others that have to have the sinus surgery like every two months or so! I just hope she doesn't have to go thru this every couple of months. I am praying that once she has this done it will give her relief for a long time. HIS protocol for doing endoscopic sinus surgery on CF Patients is --- 

14 days of IV antibiotics BEFORE the surgery to clear up any possible infections/bugs that are hiding

Do the surgery

Then an additional 7 days of IV antibiotics as a precautionary measure

That is a total of 21 days in the hospital!

He wants too see her right away! Right away! Okay that sounds fine! His secretary Ryan would be calling with the appointment time and date.Thinking we would have a week or a week and a half to prepare for the trip to Philly for the appointment! Nope! Not at all! We have a little over 48 hours to reschedule the appointments and prior engagements that we had scheduled the rest of the week because we would be going to Philly on Friday morning! Mind you the conversation was happening on Tuesday! We go Friday at 1:40 to schedule the admission and surgery! I am nervous as HELL! As scared as a virgin in a whore house! She will be having endoscopic sinus surgery, having her perforations surgically closed (if they are not already closed on their own) & tubes... But if he needs to close the perforations on his own then the tube procedure will be pushed back until her ears heal! If the perforations are closed then he will put tubes in at the same time he does the sinus procedure. She is having 2 possibly 3 procedures done! Well 4 including the PICC placement for the IV Abx!

After we hung up the phone I looked at the calendar to see what all she is going to miss while being in the hospital and I became madder than ever! There are so many things that she is going to miss and those things will never be able to be done! However, he did say that he does not want to interfere with her Kindergarten Registration which is May 8th! 

April 27th -- Pre-K Picture Day! She is going to miss picture day! That is a memory that you cannot recreate! So sad!!

May 5th -- Dad's Day at Pre-K (able to attend)

May 8th -- Kindergarten Registration (MUST attend)

May 17th -- Dentist Appt -- must reschedule 

May 22nd -- Pre-K Graduation -- this is her last year of Pre-K and she will NEVER have a Pre-K graduation AGAIN ... this is a big thing for children entering Kindergarten

F*ck You Cystic Fibrosis --- I HATE You

A Mother's Instinct

As many of you know that when Bennett went to her CF Clinic appointment on March 20, 2012 he put her on 21 days of Bactrim  and upped her airway clearance due to a runny nose and a drop in her PFT's. He wasn't too concerned about the drop in PFT's because she was only able to expel air for 1.33 seconds. 

She finished up her Bactrim on April 9, 2012. Approximately 4 days (4/13/2012) later she started having another runny nose which was slightly green in color, congestion and a slight cough. As the days went on the runny nose turned more of a green color, the cough had gotten junkier and she started getting low grade fevers. I started putting our sick plan in action which is upping airway clearance again trying to kick whatever it was that was causing her to not feel well. After another 4 days of it not getting any better, I e-mailed the nurse practitioner at her CF Clinic and explained to her the symptoms that Bennett was having. She told me that I was overreacting that it was just her Allergies and not to worry about it.Which REALLY upset me and pissed me off because I knew it wasn't her allergies. I then called the CF Nurse and CF Social Worker and gave them a piece of my mind. 

Lastnight at about 9:44pm she woke up screaming bloody murder about her left ear (this is when he suspects the perforations happened). I rushed downstairs and had gotten her numbing ear drops. That only caused everything to hurt more. So, I gave her a dose of Advil for pain relief. Which eventually relieved the pain and she was able to sleep for a few hours until it began wearing off. Her fever was about 102 lastnight overnight as well.

I woke up and called her ENT first thing this morning and only had gotten to speak to his nurse because he is out of town at a conference until Monday. Nurse Amy asked me to make a sick appointment with her pediatrician here in town and ask him to look at her. Thankfully I was able to get her in this afternoon. He looked into her ears, nose, throat and listened to her lungs. He said she has two perforated ear drums, two severe middle ear infections and another sinus infection. Her lungs sounded a little junky as well from all of the junk draining out of her sinuses. He told me to look into her left ear which was causing her the most pain. He showed me what the perforation looked like. It looked really nasty and painful. To me it looked like a huge blister that was leaking puss. It was yellow and around it was bright red. My poor poor girl. He prescribed Ofloxacin (antiobiotic eardrop) & another bottle of Antipyrine and Benzocaine Otic (numbs the ear canal). And told me to continue giving Advil for fever and extra pain control. Also, he gave her Augmentin which is good for this type of infection. Augmentin makes her 13th antibiotic since September.

When we left his office I had to call Nurse Amy back from Dr. Rizzi's office. I told her about the perforations and she wasn't very happy. She said if Barb Jansma the Nurse Practitioner from Dr. R's office had listened to me the day prior her ears possibly wouldn't have perforated because we could have gotten the abx eardrops started and reduced the risk of it happening. I told her what all her Pediatrician had prescribed and she was satisfied with his decision. When she spoke to Dr. Rizzi during a break at the conference he had told her he wants too see Bennett in his office in two weeks. He wants to give the medicine time to work. He said he possibly will have to close the perforations himself unless they close on their own in the next two weeks. She will be in pain on and off until they close (that is if they close on their own). She is calling me back tomorrow with the appointment date and time. He also mentioned doing the endoscopic sinus surgery and putting tubes in. He will only be able to put the tubes in if the perforations close on their own, if they don't it will postpone the tube procedure even longer. We will be once again discussing the procedures. If he decides to do the surgeries she will need to get 14 days of IV antibiotics beforehand as a precaution then once she does those 14 days she will be able to get the surgeries. :0(

She will be getting the eardrops until further notice which will be at least 21 days but no longer than that. She will only be taking Augmentin for 10 days. This isn't hard on her tummy. She cannot take anything stronger orally right now because of her Vitamin K levels. Also, going to continue giving Advil for extra pain relief and for her fever. 

Vitamin A, D and K Deficiencies

Bennett had her yearly bloodwork taken in September 2011. The final results for her vitamin levels were back in December 2011. When those initial results came back, her Vitamin D and Calcium levels were low. So, her dietitian put her on 1000UI of Vitamin D and 500mg of Calcium. A few weeks after starting these medications the dietitian had called back and upped her Calcium to 750mg a day. 

They scheduled her for a re-check of her vitamin levels for our next clinic visit which was March 20, 2012.  Dr. Rubenstein put in an order for her vitamin levels and also ran a blood test for anemia since she has been extremely fatigued lately. They told me that once the results came back that they would give me a call. A few days had passed and they never called so I e-mailed our CF Nurse Kerri and she had told me that once the final results are in she would have the dietitian give me a call. :0(

Another week had passed and my anxiety was through the roof. After 24 days of waiting for the results. I received a phone call around 10:45am on Friday the 13th of April and it was the dietitian. She had told me that the final results were in and that the reason she is having leg cramps, breathing difficulty (aside of typical CF breathing struggles), muscle pain, fatigue, low immunity (again aside from typical CF struggles) and slight hearing loss is because her Vitamin D levels are lower now than they were in December. Also, her Calcium is still low along with her Vitamin A and Vitamin K are low. She decided to up her Vitamin D dose from 1000UI to 2000UI and leave her Calcium at 750mg.

She explained to me that the reason her Vitamin K is low is because of the amount of antibiotics that she has been on. The antibiotics rid the body of both the good and bad bacteria in the body especially the intestine and that is where Vitamin K is mainly absorbed. Bennett was on 12 rounds of oral antibiotics from September 2011 and finishing the 12th one April 9th 2012. And she began to explain some symptoms of Vitamin K Deficiency. FINALLY, there is an explanation on why she is covered in bruises head to toe. The slightest little bump or fall and she bruises instantly. She wants to start Bennett out on 1/2 of a tablet of Vitamin K Monday, Wednesday and Friday. 

 For the Vitamin A Deficiency she said she needed to talk further to Dr. Rubenstein and she would be calling me first thing Monday morning.

Her iron levels were a little on the lower side so she will be starting an iron supplement once Dr. Rubenstein decides which one he wants her to take.

She will be getting her Vitamin levels checked monthly so they can monitor them and adjust her medications as needed.

Thanks to her Cystic Fibrosis she now has Vitamin A Deficiency, Vitamin D Deficiency, Vitamin K Deficiency and Calcium Deficiency. Gee Thanks A Ton CF! You Bastard!! 

I am a little upset with her GI Specialist because she has limited her milk intake to ONE EIGHT ounce glass of 2% milk A DAY! I feel that if she would allow her to have more milk a day that it would help keep her Vitamin D and Calcium levels up a little bit. 

Vitamin Deficiencies aren't anything easy to deal with because they can cause added problems that we definitely do NOT need right now! 

Vitamin D Deficiency is causing Bennett the following problems: leg cramps, breathing difficulty (aside of typical CF breathing struggles), muscle pain, fatigue, low immunity (again aside from typical CF struggles) and slight hearing loss

Vitamin K Deficiency is causing her the following problems: Thankfully it is only the bruising and they said the reason she is having bloody stool at times is because of the deficiency. Between the deficiency and intestinal tears we now have answers on the bloody stools. 

Vitamin A Deficiency isn't really causing any symptoms that I am noticing!! THANK GOD!!!!