IVs at home

This is what IVs at home look like. In times past we've had to administer the IVs up to 5 times a day. This round is only 3 times a day - every 8 hours. Here is a picture of Brett's Broviac central line (tunneled catheter). I have also added pictures of the other types to my Broviacs, PICCs, and Ports, oh my! page.

The catheter actually comes out of his skin (called the "Site") underneath the circle, which is called a "Bio-patch." It has some extra antibiotic on it to reduce further infection, mostly used while in the hospital.

This round is only one drug, and we administer every 8 hours. I have chosen 7am, 3pm, and 11pm. Basically, we  flush with saline, hook up the medicine, let it run about 30 minutes, then unhook and flush again with saline and heparin. That way we're in bed at 11:30 at the latest and don't have to get up super early, because this round is happening over Christmas Break from school.

The medicine comes in this pressure-filled ball, called an elasti-ball, or elastomeric ball. To me, this is supremely more convenient than the kind that comes in pre-filled syringes that we have to prime and put inside a very heavy pump that runs on "D" batteries. It looks as if it was made in the 70s. In fact, it probably actually was. 

Anyways, this is great because it's very light. We hook it up to Brett's broviac and put it in his backpack, and he just wears it around for the 30 minutes. At night we just lay it beside him in the bed. The backpack is for his g-tube feed, which is still going for 18 hours a day, so he must wear it around 6 waking hours. Don't feel bad for him, though. The backpack is his freedom from an IV pole. 

5th Broviac Placement Day and 14th Admission

Brett had surgery today to place a Broviac central venous catheter. All went well with the anesthesia and the procedure. As I am writing this he is sleeping off the anesthesia and we are waiting on a bed to become available. His bronchoscopy cultures from last week only grew staph, so he will soon start the appropriate IV antibiotic. We will start treatment here at the hospital then finish the course at home. After treatment he will return for the Broviac to be removed. This will probably happen after the new year.

Brett sleeping off the meds

Getting his post-placement x-ray. Doesn't he look so small in the bed!

Bronch Day

Today was  bronch day for little Brett. His most recent bronchoscopy was over a year ago. Today's bronch was successful in that everything was done safely with the procedure; however, the doctors saw plenty of secretions and inflammation in his airways that suggest infection and the need to treat with a course of IV antibiotics.

The hospital is full right now, so the plan was originally to do the bronch, take labs, and place a PICC line and send him home until a bed opened. This was acceptable because we have had plenty of experience with caring for and maintaining central lines in Brett. Unfortunately, after 2 attempts, the sedation team was unsuccessful in placing the PICC line. That means that surgery will have to get involved.

The cutest thing today was when Brett didn't  mind getting into the gown but demanded that we put back on his socks and shoes! He wore them the whole time - maybe it made him feel more secure and brave...

This is how he feels when awakening from sedation - probably like he's been hit by a ton of bricks!

Eating crackers, feeling a bit better, and saying over and over "Go home, go home!" I'm glad we were able to.

Since Brett's case is not an emergency, the surgery to place a Broviac central catheter has been scheduled for next Monday, and a bed request has also been placed so hopefully he will be able to be admitted right away after the procedure. This scenario is not such a bad thing for 2 reasons - first, not much happens at the hospital over the weekend and now we can spend it at home with the family, and also the cultures taken during the bronch will have a chance to grow and we can target the pathogens more specifically with the most effective antibiotics. Of course, not every area of the lungs can be cultured, so we pray that the cultures that were taken are indicative of what is really going on in Brett's lungs.

11/30 and 12/8 Appointments

Brett had a routine clinic visit in Chapel Hill last Thursday, and we discussed various options of treatment for his cough, recent gastrointestinal issues, failure to thrive, and general behavior suggesting that he feels bad.

After talking with Dr. Esther I felt as though we would wait until January and then do a bronchoscopy (what we call a "bronch") to see what his lungs look like. But then over the weekend his cough got worse and more frequent. Also he just acted as I he felt bad, which is often our most important indicator. He just doesn't smile very much, and basically just wants to be held, or sit and watch tv, or sleep. His favorite being all of the above!

Therefore, we decided to call Monday and see about getting him in for the bronchoscopy this week. Today in Raleigh we saw Brett's immunologist, Dr. Hernandez. This was also a scheduled visit, and it just happened that Dr. Esther was there as well. It is always helpful to have any of Brett's specialists in the same room to talk and make sure they are all on the same page as far as treatment. Because Dr. Esther was there I went ahead and signed all the consent papers for the bronch tomorrow in Chapel Hill.

The plan is to do the bronch, place a PICC line, and admit him for his 14th hospitalization. He has not had IV antibiotics in 11 months--that's quite a long time in Brett's world. If for some reason his lungs appear awesome-clear of secretions, infection, and inflammation, we will just come home and await the culture results from the bronch to decide on treatment.

Gantt and Brett playing at the Pediatrician's office

In other news, Gantt is sick as well with some sort of stomach illness. Trying to take care of him and get him better at the same time. Luckily, none of the rest of us have gotten sick yet!

13th Hospitalization - Sept 28- Oct 1

We took Brett to the emergency room per his doctor's orders when he was having severe stomach cramps and pain. He had a full work-up complete with x-ray and ultrasound to try to see if there was some physical blockage in his intestines or some scar tissue formation blocking the flow of instestinal matter. 

 Sweet baby sleeping in the emergency room

 You can certainly tell how bad he feels

First time in a big boy bed instead of a crib!

Over the course of several days, he was not allowed to eat as the doctors wanted to "rest his gut." He got IV fluids to sustain him. Nothing happened, so eventually he was given an enema, it worked, and we went home. As of December 13 (when I'm writing this) this has happened twice more at home and we just give an enema when he begins complaining about his stomach hurting. Obviously no one is sure what is going on or why this keeps happening!

He was soooooo hungry once we got the OK for him to eat!

Brett got to do a wagon ride but was very distressed that his riding partner Gantt was not in HIS seat!

June 2011 Clinic Visits

Brett has 5 visits in the last 5 weeks to the UNC Children's Speciality Clinic! The good news is that all of these visits were scheduled. We seem to be turning the corner, with 6 months now without a hospitalization, to a somewhat long run of wellness.

Brett showing the stickers he got at Infusion

Our clinic visits still average once  a week, but it's easier to deal with when they are planned "maintenance" kinds of visits and not emergency "illness" types of visits. We think this is mainly due to the fact that Brett does not have a central line in his body at this time. And it looks as if the next time he requires one we ought to be able to get away with just having a PICC line now that he's bigger and he's had several successes with PICC lines under his belt now. The main advtantage to the PICC line is that you only have to be sedated to place it. It can stay in place up to around 8 weeks, then it can be pulled out by a nurse; it does not require surgery to remove.

Brett's June 3, 2011 visit was for his IVIG (intravenous immunologlobulin) infusion. It was a little rougher than usual since the first IV did not work so he had to be messed with a lot more and poked twice. His June 7 visit was to see his immunologist, Dr. Michelle Hernandez. On June 9 he had his second set of infant PFTs, June 22 he saw his GI doc, Dr. Katherine Freeman, and July 1 he had another IVIG infusion. I'll break this information into two posts.

June 7 with Dr. Hernandez - she is the doctor that monitors the values we see in his immune function blood tests. I actually get to visit her in the Raleigh location at Rex! I'm always excited for this possibility since it's a good deal closer with much easier parking and walking in. We are still monitoring his Natural Killer cell (NK cell) function, which has been the main focus on whether or not he has an acquired autoimmune problem or a true autoimmune deficit. There are 2 values she monitors, the natural killer cell count and the NK cell function.

The strawberry band-aid that is on his hand was where we had to put the IV this time

I guess it will just take time to see, but she did say she if he had a true autoimmune deficiency, she would expect to see his function values stay down. It appears that they go up and down. (I think I'm saying this right, but don't hold me to it.) It could be that his immune system just used up all the cells that he was born with since he was so, so sick for so, so long. It may take awhile for his body to build them back up, which is why we keep supplementing his immune system with the IVIG every month. It seems to be working, as even though he doesn't have a central line anymore and shouldn't have sepsis, you might expect that he would've had another lung infection by now. Whatever the case, we'll take it! And we'll just be glad for his relative wellness and enjoy somewhat of a more "normal" life where our family is under the same roof every night and we are able to go places, spend time at the pool, and enjoy a vacation or two. This week we're traveling to Atlanta to see our extended families, who all live in the area. It will be the first time our twins have visited Georgia and the first time many of my extended family will meet them!

June 2011 PFTs

Pulmonary Function Tests (PFTs) are routinely done on children and adults with lung disease, such as CF, or respiratory problems, such as asthma.  Infant PFTs are a special type of pulmonary function test that is only done in specialized centers.
In infants, lung function testing requires sedation so the child sleeps and is passive during the test. This allows the infant PFT equipment to perform testing since the child is not old enough to cooperate with PFTs. Infant PFTs can be performed very early in life until about 2 to 4 year of age depending on the equipment and size of the child.
Once children undergoing an infant PFT are asleep, they are placed in the equipment with a mask over their mouth and nose. They breath air with oxygen if needed through the mask. Lung volumes and flows are measured while the child breathes through the mask. An inflatable jacket is also placed around the child's chest to help them blow out air.
Since a baby or toddler cannot comprehend nor perform these tests the way older children and adults can, so the baby must be sedated and the exercises must be done for them. Click here to see Mark performing a set of PFTs last year.
These were Brett's second set of PFTs. The first were done in March of 2010 - (you can read about them here), when he was very, very ill and required oxygen. Not surprisingly, he had very low scores. The chief of the division actually told us they were among the worst she had ever seen in an infant. It was unsettling, to say the least. Back then he was put on an extremely long course of steroids, and his oxygen requirement diminished,
Fast forward until June 9, 2011, and here we are again to repeat the tests. It was a good time to test him again because it is always better to go under anesthesia or sedation when you are not sick. First Brett got some Versed to relax him while they inserted an IV line and began his sedation.
Then they put him in this little box :
then put a mask on his face with some putty so no air can escape. For part of the test they inflate his lungs and then put a bladder-type thing on his stomach to force the air out. They can measure the forced expiratory volume this way. It's usually called FEV1, meaning the volume you can blow out in one second. For babies this is measured in .5 seconds as opposed to 1 second. Again, there are various values measured, but for the CF folks, FEV1 is the one most talked about. After it was over, the doctor coached in learning to read this report:

I'm sure those of you who are farther along in your CF journey can decode this easily, and probably explain all this way better than I can, but I am still fairly illiterate when it comes to reading and understanding these values. The GREAT news is that Brett's FEV.5 went up drastically - 40%. This is an incredible improvement. One we are so very happy about and grateful for. But this blessing is tempered with the knowledge that these scores do not mean that his lung disease is not severe. It is. Worse than most CF kids his age. In fact, after last years, CT scan on his lungs, our doctor told me his lungs look like what you would expect a CF teenager's to look like. Today when we were discussing the findings, I got that sick, sinking feeling you get when you talk about your child having an inspid disease like cystic fibrosis, and the description on his report reads, "severe obstructive airway
Most of the time it's easy enough to just not think about it much - just go along with life, do what we can, be extremely disciplined about medicines and treatments. And persevere. And pray. Please join us in praying for our boy's continued wellness. And most of all for a cure, which still looks promising, and quite possibly could happen in his lifetime.

New Record!

73 days! New record for days out of the hospital! The last record was 58 days, and it seems as if he's still going strong. He's doing so well, in fact, that the new record sort of snuck up on me. Very thankful, though!

 It was a beautiful day on the Hill!

Wednesday March 23 I took Brett to clinic for a routine scheduled visit with his nutritionist, pulmonologist, and gastroenterologist. It was a good visit - although he didn't gain any weight, at least he didn't lose, and that's a small victory in and of itself.

We talked about adding more salt to his diet now that the weather is warmer and he will be sweating more. The GI doc increased his Prilosec dose in hopes of making him feel better in his tummy and maybe eat more. She also prescribed a substance called a "binder" that is supposed to help his stool not burn his bottom so bad. It was complicated the way she described it - as well as gross - so I'll leave it at that. She took a culture on his bottom to test it for strep. Also his stool will be tested again for c. diff., even though there's no real reason to suspect he has that again. I guess since he's had c. diff once now, they will test almost every time for that in order to catch it and treat it before it begins causing him to lose weight. His last culture was negative.

No changes on his feed - still tube fed for 20 hours a day, while eating and drinking whatever he can on top of that. Still over 2000 calories a day for our little guy and no weight gain. Frustrating.

The good news is that he is healthy right now, with no lung infection. He is not scheduled to go back for four weeks - yay, with a weight check in 2 weeks on infusion day, April 8. 

The beautiful daytime weather turned into a hailstorm that evening!

Brett's pulmonologist decreased his Flovent dose from 110 mcg to 44 mcg. (This is the dose Mark takes for managing his mild asthma). Oh and he started on Zyrtec this week to hopefully help with his eczema that is under control only when I use the heavy steroid ointment. His eyes are all eczema-looking, so maybe it's allergies. Nobody really knows, but it's not a bad idea since the pollen season is again upon us.

No changes on his feed - still tube fed for 20 hours a day, while eating and drinking whatever he can on top of that. Still over 2000 calories a day for our little guy and no weight gain. Frustrating.

The good news is that he is healthy right now, with no lung infection. He is not scheduled to go back for four weeks - yay, with a weight check in 2 weeks on infusion day, April 8.

Brett's Update

On February 11, Brett had his monthly IVIG (Immunoglobulin infused intravenously). Although it takes most of our day, it is an outpatient procedure. I left the house at 8:30am and got home at 5:30pm.

UNC's pediatric infusion room

Since Brett no longer has a central IV access, he had to get a PIV (peripheral intravenous access) put in just for the infusion and then have it taken out as soon as the infusion was over. Not a fun thing, but still better than having a line that is inevitably going to get infected. We hated that the port had to come out - we hoped it would last him years, but now that it's over and done, we're glad he doesn't have access so his body can sort of take a rest from having so much "hardware" inside.

Brett has gotten very used to this room!

Gantt and Brett both had a cold or some sort of virus affecting them that week, runny noses and coughs - it started over the weekend of the 12th, and I was getting concerned because of the frequency and severity of Brett's coughs. At this time in his life, Brett doesn't cough at all when he is at baseline. I contacted his doctor when the weekend was over and the cough wasn't really getting much better. He called in some oral antibiotics, Omnicef, that Brett would take for 10 days just in case the virus were to kick up the bacteria that is always in his lungs. We did this in hopes that he wouldn't develop a bacterial infection in his lungs.

He thinks it is a playground!

As the week progressed, the cough (for both boys) improved. was Brett's last scheduled clinic visit was on Friday, February 18th. He saw his GI doc and his pulmonary doc. Everyone thought his lungs sounded great. His cough was gone, and he seemed to have dodged a pulmonary infection. Brett's did lose weight, but we chalked it up to fighting an illness, plus having been on antibiotics that usually create extra loose stool. He is still desperate need of weight gain and growth. He has grown a little taller, but he needs to pack on some serious pounds. Please keep praying for that - we are doing all we can to help facilitate that growth, but his body's got to do it on it's own.

Mr. Alan plays his guitar and sings for the kids

This week my "angel mother" and "darling dad" are taking care of the children while we are away. And as you well know, caring for these three can be a joy, but it is never a walk in the park! Well, When our first general session began for Chick-fil-A Seminar, I noticed that I had just missed a call from Brett's doctor. I was concerned because it seems not much good can be going on when your doctor calls you from home on a Sunday night. But, it wasn't an emergency - just that the stool samples we got in clinic on Friday the 18th came back and had shown that Brett has c. diff, and rather than explain all that, I just decided to link it to an explanation if you aren't familiar with it and want to read about it. Mom had been concerned about him having very frequent and loose stools, so that made sense because that's what happens when you have a c. diff infection. This is Brett's first one. Thankfully it is no emergency, and he called in a prescription for Flagyl, the medicine they use to treat this bacteria overgrowth. Hope it settles down soon.

*Breathe*

This is a photo of Brett when we were in the waiting room at UNC for clinic last week. He was seen by his pulmonologist, Dr. S, and got a good health report! The main thing is that we are finished with IV antibiotics!! Since December 17, either we've been IN the hospital, or on home antibiotics.We are so happy that not only does Brett not need them anymore, but we are also so tired of having to administer them every 6 hours. That's round-the-clock, even at 3 and 3:30AM.

Weight: The same. About 21 pounds and 7 oz, or 9.55  kilos. The great thing is that he was able to maintain his weight throughout the last couple months of illness! The not-so-good thing is that since he hasn't gained any, he is still sort of on the brink of a nutritional "crisis." Do you remember way back when I blogged about trying new formula? That didn't actually end up happening after we found out the extraordinary cost of the formula. Even with 2 free cases a month, it was still going to run us $800-1000 a month. We wanted to try every other option we had first. At last week's appointment, we discussed at length the different options we might have - with the doctor, social worker, nutritionist, and special infant care coordinator all in the room. 

I got some names of foundations and agencies that help people that have children with extraordinary medical needs. But the best thing was that since we switched insurance on January 1, the new insurance plan will PAY FOR THE FORMULA!!! Also, our home health company provides it, so they will bring it to our doorstep every month without me having to remember to get online and re-order, the way I've been doing. Also, it comes in pre-mixed bottles! I've been mixing formula for almost 2 years now! By the way, though, I will be finding somewhere in North Wake that will take #5 bottles for recycling - we will be using over 200 bottles a month!We are so excited to be able to try this formula and needless to say, we have high hopes for this stuff! 

Venous Access: PICC line was removed in clinic on Jan 18. Brett has no IV access now.

IV Meds: IgG (immunologlobulin infusion once a month)

Deep Pharyngeal Swab: Culture on Jan 18 -No information yet

Next Appointments: 

February 11 - IVIG infusion

February 18 - clinic (pulmonology & gastroenterology)

March 1 - immunology

How Brett's Doing

Brett has been doing extremely well since he was able to come home. He is back to his normal schedule, lasting about 2-3 hours a day including chest PT, inhalers, meds, and breathing treatments.

We have continued the IV antibiotics that were started in the hospital. We had to administer them every 6 hours since Christmas Eve up until yesterday. In the daytime it's not too bad, but we usually go to bed pretty early, so we were getting up at midnight, 12:30, 6 am and 6:30 am to do these. (Since we were on Christmas break, we weren't waking up that early for school). Whenever we get so tired of doing that, we just remind ourselves how much better it is to be able to get it done ourselves, in the comfort of our own home. 

Yesterday was the stop date because his next appointment is tomorrow, and if any live bacteria are still in his system that are just being suppressed by the antibiotic, they will have time to grow. We'll get a blood culture Wednesday (about 48 hours after stopping meds), and wait to see if any bacteria grows in the next 48 hours after that. No bacteria growth will be great, and that's exactly what we're praying for. 

Brett's 12/8/10 Clinic Visit

Brett had a good clinic visit last week on December 8th. I had taken him in on December 1st because I was concerned that he was losing weight again. He had lost weight - he was 9.025, down from 9.8 when left the hospital last. (They immediately ordered him to have IV lipids - more on that in next post). On December 8th's clinic visit, he weighed 9.27, and that meant he had gained an average of 36 grams per day since December 1st! That is remarkable growth for Brett. He still has a hard time absorbing the fats from his diet, as will probably always be the case. Our biggest obstacles are being diligent in feeding him the enzyme pills every 3 hours, which he has come to often spit back in our faces, and keeping his feed on for 20-22 hours a day. The pump in the backpack thing is great compared to having to be hooked up to an IV pole all the time, but it doesn't always work so well in reality. It gets pretty frustrating and sometimes we fail to hook it back up to him because we're just so tired of it beeping and getting stuck and having to take it off and prime it again, etc. etc. But we're trying.

On this visit, all in the same room Brett saw his pulmonologist, gastroenterologist, dietician, nurse, and surgery nurse. His pulmonologist thought his lungs sounded like a different child altogether! He thinks we are really getting Brett on the right track. Brett is doing really well with the lungs right now, and it seems as if our discipline in doing treatments is paying off. However, we are so very aware that with Brett, he could still be back needing a hospitalization next week - we just stay thankful for each day of good health.

Brett's next scheduled appointments are December 14th, December 17th, and January 5th.

Brett's Procedure

Brett's procedure was completely successfully. During his bronchoscopy, they saw that his airways are clear and there is actually less inflammation than there usually is. (I think this was his 5th bronch.) There were hardly any thick secretions! This was GREAT news. They did culture one spot on each lung to see if any pseudomonas grows. We'll know about that next week.

Additionally, Brett got his port-a-cath!! We are super excited about it. I wasn't really counting on it, but Brett's surgeon is amazing, and he got it done. This is going to be so much better for Brett.

First Outpatient Infusion

Brett's first outpatient infusion of IgG went well today. I was a little anxious since we were going to have to just sit in the infusion room for hours and try to entertain Brett. He has had 2 or 3 IgG infusions in the past, but all have been inpatient, so I wasn't sure what to expect.

Brett looks a little rough after finally getting the PIV. This was taken at the beginning of the treatment.

We arrived around 9:30am and we finally got around to inserting the peripheral IV line around 11:00. The first attempt was successful but then failed. The second attempt was also successful, and it kept. It was in his right arm, and the "no-no" (Pedi-wrap) is around his arm to cover it. After all that ordeal, Brett was pretty tired and ready for sleep. The room is full of recliners and everyone has their own personal TV, which was pretty cool. I meant to take a picture of the room, but I forgot - so maybe next time. As you can see, we are not in a recliner- we got an adult size gurney. Brett was ready for a nap when the infusion actually started around 11:30. The nurses helped make us comfortable. We slept for 2 hours, and the infusion was done! And unexpected treat for me!

I am praying (and making lots of calls) that we get on the Operating Room schedule soon and have a central line put in before his next infusion so that he will not have the trauma of the attempted venous access again. His next infusion is scheduled for October 19.

*Breathe*

Brett's vest arrived this week! Click here to read my original post describing this device. It helps with airway clearance, which must be performed 2-3 times a day, every day, on someone who has CF. Until now, we've performed manual "Chest PT" several times every day. The way it works is that the hoses you see connect the jacket to the main machine, which sends air through to inflate the jacket. The jacket then vibrates and compresses at a high frequency and at various speeds and intervals to loosen the thick mucus from the airways.

Today was the first day we used the vest twice. He doesn't seem to mind it - and this afternoon he was relaxed enough to fall asleep on his daddy's lap while he got his airway clearance. I'm thinking of continuing the manual airway clearance at night because he usually goes to sleep and I put him straight in the bed. This vest will be a nice break from manual therapy every single time. It will also be nice to be able to count on the consistency of the quality of airway clearance when other people are responsible for the treatment. Also, when Brett is older, it will be very important to him because it means that he can be independent from us and still be able to get his treatments. It means he will be able to live away from home on his own and take care of himself without having to depend on another person to give him therapy.

Although it doesn't reduce the amount of time we spend on it (still 30 minutes for each session), it shakes all the lobes of the lungs at once for 30 minutes, versus us spending just 3-5 on each single lobe during manual PT. Therefore, it's very efficient and it's been proven to reduce hospitalizations for those who use it regularly. This device is going to ease the rigors of Brett's care quite a bit, and make our lives easier. We are very happy about that! Tomorrow he gets his first IVIG infusion.

Sept 8 Clinic Visit Results

Brett saw his GI doctor, pulmonologist, and dietician this past Wednesday at UNC. He gained 60 grams in 2 weeks. This is less-than-stellar growth; however, in the CF world, sometimes just not losing any is counted as a success. We were slightly encouraged by the weight gain, and no major changes were made to his feeding regimen - other than always trying to get him to eat more.

His pulmonologist thought his lungs sounded fantastic- in fact, the best he thinks he's ever heard them in Brett's entire life. This is great news! It has been nice to enjoy a couple of weeks of wellness. I thought it was going to be a relatively non-invasive visit, but I forgot about the usual deep throat culture and the bloodwork. Always bloodwork. They especially wanted it this time since he seems well. This helps to have something on file with which to compare "sick" bloodwork. We've never really had that before since he's never really seemed completely well. Since Brett does not have a Broviac or a PICC right now, he had to have all the blood taken peripherally, which this time meant 2 sticks. The first in his arm clotted off, so we had to start all over again - restraining him, using the tourniquet, finding a vein in his hand, getting access, then seeing if the blood would flow. It did the second time, but veeeeeerrry slowly. Thirty minutes and 7 vials later (small vials), we were finished and could go home.

The clinic visit was 4 hours long, but went well since Brett is feeling so much better.  This period of wellness may be short-lived, though, since his initial bloodwork results showed that his IgG level is plummeting again. His last infusion was during his last inpatient visit - the beginning of August. The high level of IgG may even be what has caused him to be able to stay well for the last month or so. But now that number is less than half of what it was even 2 weeks ago. At that rate, he would probably get sick again soon and have another hospitalization. Right now they are looking IVIG therapy**.

Should we and the doctors decide on going forward with this, there will be logistical issues facing us - frequent use of a blood product, hospital admissions or "outpatient" hours-long visits, and probably most importantly, venous access. It can be done peripherally to start off with, but that is of course not ideal for the long-term. They are thinking without the frequent IgG infusions, he'll probably be sick and hospitalized a lot of the winter. For now we will be seeing his immunologist soon, and will be discussing access options with his surgeon. So long for now!


**(Intravenous immune globulin (IVIG) is a blood product administered intravenously. It contains the pooled IgG (immunoglobulin (antibody) G) extracted from the plasma of over one thousand blood donors. IVIG's effects last between 2 weeks and 3 months.)

A Long Day at UNC

After yesterday's unscheduled visit to UNC (an hour drive both ways), I got home in enough time to help Mark with homework then it was off to soccer practice! After soccer practice, Rob and Erica took the boys to get dinner (drive-through at Chick-fil-A!) then home and I went to 2nd grade parent orientation at school. By the time I got home, Rob had already left for his hockey game (they are in the playoffs again - woo!). It was after 10 PM before I got Mark to sleep, and then Gantt woke up! Ended up just putting him in the bed with me and hitting the sack.

In clinic yesterday

Today we had 4 scheduled appointments at UNC - physical therapy, pulmonology, nutrition and gastroenterology. After the docs looked at Brett yesterday, they decided to do a throat swab and a nasal swab - to test for bacteria and for viruses. He was so listless and lethargic, he hardly even had the energy to protest much. They also ordered some blood tests, but before they did them, they made a courtesy call to Brett's gastroenterologist (GI doc) to see if she wanted to order any tests since they were already going to be drawing blood. That is one thing I like the most about this hospital staff - they always work together to care for the patients. Today we had his pulmonologists, GI, and nutritionist all in the same room, discussing Brett and what changes to make in his care regimen. It is a very effective way of doing things in a system (American healthcare) that oftentimes is the farthest thing from effective. Today Brett is feeling a bit better, but I still wouldn't be surprised if his viral panel shows that he does have a virus. If this were the case, we wouldn't treat it unless he developed some respiratory distress.

New formula - yay!

The blood tests came back great - they mainly checked white blood cell count (if this number was elevated it might suggest infection), CRP (inflammation), and IgG (immunoglobulin). The IgG is already falling since his last supplemental infusion.  All the tests the GI doctor wanted were surprisingly good - showing that he is in fact absorbing his nutrition pretty well. Unfortunately this was the 5th visit with weight loss. However, he just finished his steroids last week, so much of the weight loss is due to losing the water weight the steroids made him retain. Since we haven't increased the feeds in quite a while (and before he started crawling and moving around so much all day, burning up calories), we decided to increase the rate of the 20 hour feed. Also, we will be switching him from Pregestimil infant formula to Peptamen Junior toddler formula. We hope he tolerates it well. For us, this means in a couple weeks we will be DONE mixing formula!!!! Yeah!!!

*Breathe*

It's been a challenging few weeks! This past week was a lot better, but it was still a challenge as Mark started his first week of school and our new nanny, Erica, began her first 40 hour week. She worked quite a bit over the summer and she's doing great!

Here is Brett's most recent PICC line in his arm. For his daytime infusions, we'd hook up the syringe and pump and put it in his backpack so he could keep playing while he got the treatment. It worked out pretty well.

Last Sunday (August 15), I e-mailed Brett's doctor to ask if we could stop the IV antibiotics. It was getting really difficult for us not to get as much sleep since you have to stay up real late and now we have to get up at 6 or 6:30 all the time. He said that would be fine if I thought Brett was not coughing as much and he was "back to baseline". It had been 17 days of antibiotics already. I thought so, so we stopped. Since he still had the PICC line, we still had to flush it everyday, though, and the next Tuesday night I couldn't get it to flush. I called that night to let the doctor on call know, and we went to clinic the next day (last Wednesday, Aug 18). The good thing was that Brett's doc got to actually see him and agree with me that he seemed well enough to come off the antibiotics. Since we came to that course of action, they just removed the PICC there in clinic instead of trying to get it to work again.

This is the wrap we put over the PICC line for bathtime. 

The bad thing for us is that now that Brett has had pseudomonas, he will be treated as if he always has psudeomonas growing in his lungs. For right now that means going back on TOBI for the rest of the month. (TOBI is the inhaled version of the antibiotic Tobramycin. When Brett gets IV antibiotics, it's usually a combination of Tobramycin and Cefepime). It's basically one month off, one month on. We are having an extremely hard time with it. I don't know what to do to make it better. It's the time commitment of one hour in the morning (20-30 minutes chest PT then 30 minutes TOBI neb right after) in addition to all the other things we have going on in the mornings, and the screaming all the day through the 30 minute inhaling. We can do the other neb (Pulmozyme) in the afternoon if we find time for that, but if we don't and we have to tack it to the night time routine, he's got to do the Pulmozyme neb for 15 minutes, chest PT for 20-30 minutes, then the TOBI neb for 30. This also falls during the time we are trying to get the other 2 kids to bed as well. We don't really have any options as far as timing goes because the TOBI doses should be 12 hours apart, ideally. He's hardly even awake 12 hours, truthfully! I'm told that 10 hours is the very least the doses should be spread apart.

Everything seemed fine until this weekend, when Saturday Brett seemed just a tiny bit under the weather. By Sunday (yesterday, August 22), he was sleepy and lethargic all day long. We do have an appointment tomorrow, but based on the way he felt I thought it was not wise to wait that long to see the doctor. Again I called the on-call doc and she made us an appointment for this morning around 10:30. Today he's actually acting a bit better. He's so complicated! The good thing is that they all realize how complicated and tricky Brett is too! I'll update when I can!

*Breathe*

Oh, TOBI. We now have a love/hate relationship! I love it that it appears to be working! I hate it that it takes so long and we have to do it twice a day. Also, the ideal time between doses is 12 hours. Therefore, we have to do it as soon as he gets up and right before he goes to bed. The TOBI is an inhaled antibiotic designed to work against pseudomonas. Using the compressor we have, he has to breathe the medicine for 30 minutes. The TOBI must be done after airway clearance (chest PT), so we're pretty much making him sit in the chair for an hour morning and night.This is in addition to Albuterol in a metered-dose inhaler, once-a-day Pulmozyme treatment which lasts 15 minutes, and Flovent also in a MDI.  Sometimes it takes 15 minutes just to prepare the medicines, not to mention all the washing and sanitizing of the equipment and syringes.  At 15 months old, and he's got his parents sticking stuff on his face all the time. He'd rather be doing something else. If not for the g-tube, he'd have us forcing all his oral meds down, too. To some others who have CF or other CF families, this regiment might seem fairly "easy." But for us it's quite an adjustment.

We took Brett in to clinic Friday afternoon because he developed a fever after his shots, which is normal, but his breathing rate was 80-90 times a minute and his sats started getting pretty low (87-89). After an Albuterol treatment and 20 minutes of airway clearance, the sats improved to 92-94. He still felt fine during all this - we were really just being cautious. Brett is tricky, and you never really know what is going on with his body. It's like playing detective. He got a chest x-ray which looked improved from 2 weeks ago! That was reassuring that the TOBI and Cipro appear to be effective working against the psuedomonas. Hopefully that is really the case! He also had a nasal swab for viruses and a throat culture. These are easy tests that are pretty much routine and can give us good information at times. Otherwise, we're just hanging out, doing the meds for 3 weeks, then off for 3 weeks, then go back for another bronchoscopy. Here are the meds he's on now.

Enzymes - 18 capsules/day

Prisolec - 2x/day

MCT oil - 2x/day

Vitamins - 1x/day

Probiotics - 1x/day

Actigall - 2x/day

Baclofen - 3x/day

Zinc - 1x/day

Cipro (oral antibiotic) 2x/day

Potatoes in g-tube 3x/day

Chest PT 2-3x/day (20-30 min each time)

Pulmozyme 1x/day (15 min)

Albuterol 2-3x/day

Flovent 2x/day

TOBI 2x day (30 min each time)

Table Salt

20 hour Tube feed - still wears the backpack most of the day

Total Time spent managing CF:  4 hours

15 Month Check ups

Twins at 15 months

Last Thursday we had Brett and Gantt's well 15 month checkups. This was the last time I'm taking them both at the same time! It was crazy, even with 2 people!! We saw Dr. Davis at Wake Forest Peds, and Gantt got all his 12 months shots. His were on hold because of Brett's natural killer cell deficiency. Brett got shots also, but none that contained live viruses.

Brett weighed in at 21 lbs 3.5 oz, which is in the 8th percentile for his age. His height was less than 5%. However, we are truly encouraged that he is even on the chart at all, because for a while he wasn't.

Gantt weighed in at 23 lbs 4 oz, which is in the 28th percentile. His height is 82%!!! Gantt will go back in a few weeks to get the 15 months shots and our next well visit will be at 18 months.

At the doctor's office