Dear friends and family,

Last night we attended a birthday party for our dear friend Teresita Garrison. In lieu of gifts, her request was that her guests donate to Cullen's COTA. Her family and friends were very generous and as always, we are very grateful.

As you know, we have reached our goal so these donations help suppliment what we have been taking out to pay for current medical costs. It is an overwhelming relief to have Cullen's COTA to turn to in helping with the financial end of things so that we can focus more on caring for Cullen. Most important of course is that the funds are there for Cullen when the need for transplant becomes a reality.

Cullen is doing fine. Minor issues take place from time to time, but for the most part, he's been hanging in there and enjoying being a 12 year old boy. His big appointment at Stanford will be in August when he has his annual heart cath, and as always, we will ask for prayers during that time. You all remain in ours.

Love,

Colleen 

So the last time I blogged we had taken Cullen to the hospital for a possible site or line infection.  It was confirmed that it is a site infection and we have been treating it with Clindamycin.  We also received the results from the PH Probe. He does have acid reflux contributing to the mess his stomach is in.

Then this past Tuesday I had to take him to the ER with severe chest pains. Since it starts with a burning sensation in his throat and his EKG didn't find anything out of the ordinary for Cullen, the doctors believe the chest pain is reflux related. I agree with that diagnosis.  However, as I mentioned before, we stopped the Tracleer and started him on Latairis (PH drugs for those not familiar).  The Latairis can also cause chest pains and lower blood pressure (his is a little lower than usual).  He wore a 24 hour heart monitor to see how his heart his handling this new drug.  No results from that yet.

Wednesday we made some changes in when we give certain meds. I'm forcing Cullen to drink a lot more water with his pills, restarted another drug and we are being very careful with what Cullen eats and when.  The result, some chest discomfort on Thursday but he was well enough to return to school.  We felt hopeful that things were going to get better.

Then last night he broke out in a red, very itchy rash from head to toe. He tried to go to school this morning but with tears in his eyes said, "Mom, I'm miserable!"  I am thinking it is the Clindamycin, but he has to continue taking it until at least Sunday. As it is we were warned that the Clyndamycin might not completely clear the site infection or that it might return once it is stopped.  We have to be very careful about controlling the site infection so that it doesn't become a line infection. We are consulting with his doctors to find out their theory on the rash and what to do. In the meantime he is taking Benadryl to ease the itch.

I just pray we can get him more comfortable before Christmas. Please keep him in your prayers too.

~Colleen

Cullen was sent home with antibiotics after his site was swabbed and bloodwork was taken,  We won't have results until Friday or Monday.  Hopefully the antibiotics he is on now will fight whatever this infection is so that no other action will need to be taken.  The area hurts a lot but he is at home resting and we are monitoring him closely.

Tomorrow he stops the Zantac, but will increase the Prevacid in an attempt to control the acid reflux.  Tonight he will take his last Tracleer and start Letairis tomorrow. Hopefully his transition to this new PH pill will go smoothly without much side-effect.

Lots going on with Cullen right now. Thank you for the prayers.

~Colleen

Brian stayed home with the boys today.  He called me around 9AM at work to tell me that he was going to have to take Cullen into the ER. The  central line site is hurting, swollen and has some oozing...not a good sign. I don't have any more information to give then that.  He is at the hospital and depending on what kind of infection it is will depend on whether they can send him home or if they will need to admit him. Please pray that it's not serious enough that they would have to replace the line and that he can simply come home with some antibiotics. Pray also for Cullen to stay strong.  He is upset and worried about what the outcome of this might be.

To catch you up on some other news.  The PH Probe did show reflux so we are stopping one reflux med and increasing another in the hope of improving the problem.  Tonight Cullen is suppose to take his last Tracleer pill and start Letairis tomorrow night, but I guess that will depend on the results of today.  I don't think we want to introduce a new medication during a possible line infection.

I do have good news!  Cullen turned 12 on November 17th! 

Thank you for the love and prayers.  I will update again when I have more information about the infection.

~Colleen

Agh!  It was a rough, long day at the hospital.  Due to Cullen's PH, Long QT and Mitral Valve Prolapse, the Endoscopy and PH Probe were done in the Pediatric Cardiac OR.  The Endoscopy pictures of his stomach didn't look so great.  The doctor said, "It's not suppose to look like that."  The poor kid, does anything ever come back normal for him?  The stomach is irritated, inflamed and has a little bleeding.  The plan is after the PH Probe comes out tomorrow he will begin Carafate 4 times a day.  As you know, Cullen is on a LOT of medication throughout the day.  He can't have food, drink or meds an hour before and  a hour after taking the Carafate, keeping in mind he will be taking it 4 times a day.  We are going to have to do med schedule changes to make this work.  The good news is he will only be taking it for about a week.  It's suppose to help heal his stomach.

The Endoscopy also took cell samples that will be tested for more information about what might be going on.  The PH probe results will also hopefully give up some information too, but those results take a little longer to get back.

So, speaking of the PH Probe, which was placed while he was asleep.  Once he woke up an X-ray was taken to make sure it was placed properly.  Turns out  the probe went down, looped back around and started coming back out his throat, making Cullen gag and hurt.  While awake this time, they had to pull the probe out and place it back in which goes up his nose and down his throat.  It was horrible!  Thank God Brian was there because I was begging the nurse to stop, but Brian understood the importance of getting this done and held Cullen so they could get it back in.  My poor Cullen!  I felt so bad for him.  However, he bounced back better than I did.  Once it was in right it didn't bother him as much and he was just happy to be released from the hospital.

Tomorrow he has a check-up with his cardiologist, routine ECHO and EKG, then at 2 we head to the GI clinic to have the PH Probe removed. At least now he knows what to expect. 

We ask for prayers that Cullen's stomach will heal quickly and that we can easily deal with whatever is causing the problem.

~Colleen

Dear Friends and Family,

Since the last time I updated we were increasing Cullen's Flolan and starting Oxygen at night. He is at the desired Flolan rate now and we have seen an improvement.

The Pulmonary Hypertension and medications can cause severe acid reflux. Cullen has been on reflux medications for several years.  Due to increasing problems he will have an Endoscopy and PH Probe done tomorrow, Wednesday, November 2nd to find out how bad the reflux is and if there are any other issues going on.   He will be asleep for both the Endoscopy and Probe, however, the PH Probe is a tube that will go in his nose, down his throat and worn for 24 hours.  So when he wakes he will be coming home with it then return on Thursday to have it taken out.  It doesn't sound pleasant, but it is necessary that he have this done.  It's also a test he needs completed before transplant, so it will be one more thing out of the way. As always, especially when being put to sleep for something, we ask for prayers.

Cullen will be 12 on November 17th and just attended his first Middle School dance!  He had a blast despite being spent by the time he got home. Trick-or-Treating was hard on him as it has been for the past few years.  He got a little over a block away and had to return home.  His ankles and legs were hurting and swollen which is a side effect of his medications, and he was exhausted like he just climbed a mountain. It is for this reason that we decided to order a walker that converts into a wheelchair. Cullen was so relieved and grateful when he heard what will be arriving in a few days.  Now whenever we go somewhere that requires a lot of walking, (zoo, mall, middle school camping trip in May) he will have his walker/wheelchair to rest if needed. It's hard to be 12 years old and worried about whether you will be able to keep up with everyone else. 

Thank you for your continued support (donations have already helped with several challenging medical costs) and for your love and prayers.  Please remember to pray for Aidan too.  He worries a lot about his big brother.

Love and Prayers,

Colleen

Dear friends and family,

I am sorry for being a day late in updating but we were so exhausted upon our return yesterday.  The drive home was uneventful, thank God, except for some beautiful views of nature.  We pulled into our driveway at 6:30AM and the oxygen was delivered at 1PM.  They forgot the mask so he had to use the nose piece last night, which he doesn't care for.  It kept falling out during the night so he eventually leaned over and turned the O2 off.  Hopefully the mask will work better.  He is very willing to give it a try.  Anything to feel better!

He is also being very accepting of the added inhalers, Pulmicort twice a day and Xopenex when needed.  He's used the Xopenex a few times so far and it does seem to help.

We've started the Flolan climb and so far so good on that as well.  We have a ways to go yet before we reach the desired rate.  Only then will we stop the Tracleer and switch to Letaris, probably in a month or so.

Later I will be picking up the increased dose of Prevacid.  In case you are wondering about the reflux, a combination of his PH and his medications contribute to that problem.

Thank you for all your prayers and well wishes.  They really mean a lot!  My cell was beeping all week with kind messages which I shared with Cullen and Brian.  We received a few messages here as well.  Thank you Mrs. Batzel and Amy!  If you ever want to leave messages at this COTA site just go to the guestbook tab. Cullen reads all the messages.  I think you have to register first but the other perk to that is you will receive update notifications by e-mail.  If you have any problems doing this just let me know. 

Hello to the wonderful students at Cathedral Catholic in Johnstown, PA. The children there have been so supportive, caring and have fundraised for Cullen, a child whom they have never met!  Mrs. Batzel, I recently mailed you a DVD of Cullen's COTA journey. I hope the students will have a chance to view it.  God bless! 

Love & Prayers,

Colleen

This trip I was more focused on just getting Cullen out of the heart cath safely, which he did.  I knew in my heart that the results were going to show problems because of the increasing breathing issues.  I was recognizing a lot of the old symptoms returning, so I wasn't surprised when his PH Specialist told us he was not happy with the numbers from the cath. Cullen's ratio number is almost as high as it was when he was first seen at Stanford, so obviously that is not a good thing.  However, as I mentioned previously, Cullen has grown quite a bit.  His Flolan dose is not high enough for the weight and height Cullen is at now, so his doctor feels fairly confident that we can get his numbers back under control by aggressively increasing the Flolan. Also, Cullen's blood flow is still good, which is so important.  If that showed a decline as well then we would really have to worry. Thank God that is not the case.

His specialist said in seeing the results it makes a lot of sense why Cullen has been feeling the way he has. We're not sure now how much the asthma is effecting him and how much is the PH but we will be treating both.

In order to get Cullen back on track there will be several changes and additions.  First we need to get Cullen's Flolan to a certain rate which will help lower his pressures and therefore get his ratio number back down.  Once we do and if he is handling it ok, then we will be taking him off of Tracleer and introducing Letaris, which is a newer drug.  This he will only have to take once a day and he will only need a liver function test 2 times a year instead of every month...that will be a nice change.

Cullen was taken off of Lasix when placed on the Diltiazem. Now that his body has adjusted to the channel blocker he will have to restart the Lasix in the near future. One of his acid reflux meds will be increased right away.

The inhalers he has already started and when we return to WA a company will come to our home to set Cullen up with the Oxygen for nighttime. His PH specialist thinks the oxygen at night is really going to help him a great deal. It will be a new reality to the disease for Cullen, but if it will help him, we are all for it!

So, the results were not great, but at least there is hope that we can get him back down to a lower ratio.

Cullen is doing fine.  A bit sore from the cath but very relieved that it is over, as we all are. Check-out time tomorrow is 11AM, then we will be back on the road headed back to WA.  Can't wait to see our Aidan! 

Love,

Colleen

Cullen is in having his heart cath now.Cullen hates the way the anesthisia makes him feel as he starts to go to sleep.  It down right terrifies him.  I'm allowed to go into the surgical room as they get him settled and until he falls asleep.  He really fought it this time, started thrashing around, yelling he couldn't breath and trying to pull the mask off.  I was in tears when I left him, but he was finally asleep and resting.  My poor Cullen!

So now it's the waiting game.  We are told sometime around one he should be out of surgery.  He forgot to bring his favorite stuffed animal to this hospital with him this morning so I'm headed to the gift store now to buy a new friend to comfort him as he comes to.

So far what we do know is that we will need to increase the Flolan, but for a good reason, he had a major growth spurt since we were last here.  He's gained weight and gotten a lot taller, thus the reason for increasing the Flolan.  Also, he will start using oxygen at night.  His PH Specialist said he will feel so much more rested in the morning and probably feel a lot better  with the oxygen.

I will update later but it might not be until tonight. 

~Colleen

It was a long 7 hours at the hospital for appointments today but for the most part, they all went smoothly.  Cullen had an ECHO and EKG then 6 minute walk which he was able to complete without any problems.  Then he had his PFT (Pulmonary Function Test).  The tranplant specialist said that the PFT showed some minor asthma present which might be contributing to Cullen's recent breathing difficulties.  He's not thrilled about it but a daily Pulmicort inhaler and emergency inhaler has been prescribed.  If he continues having issues, especially at night, after using the inhalers for a while, then the specialist wants him to have an acid reflux test done.  They have been treating reflux with 2 different meds but an actual test to see how significant it is has not been done yet.

The last appointment after meeting with the clinic was a MRI.  Cullen is very use to having this done so it's not scary for him, however he had some problems today.  About half way through he started yelling for me that he couldn't breath, so they pulled him out and I had his PH nurse paged.  Due to the breathing problems he has been having the straps and the plate placed on top of his chest was too much pressure for him while being asked to hold his breath off and on.  With some adjustments he was able to complete the MRI.

We went out to dinner but Cullen wasn't able to enjoy it because his breathing was so labored. I'm thinking the CA heat is effecting him. 

Brian dealt with the vehicle issues throughout the day.  He got a rental. They sent him on his way with a Dodge Charger, which Cullen loves! He wants us to buy one...dream on kid!  He hates all the pictures I take but this time he asked me to take one, of him standing next to the car. I'll post it at a later time. The van is fixed and it was the fuel pump.  We got it back tonight and will return the rental, much to Cullen's dismay, tomorrow morning before heading to the hospital.  We have to be at check-in by 9, and the cath is at 10:30.

Thank you everyone for the prayers and well wishes!

~Colleen