HPP Awareness Day

I sit here in the ICU at CHOP and I look at this beautiful boy, who did nothing to deserve anything that has come his way.  He has been dealt the hand of hypophosphatasia, a disease  I could hardly say at the time of his diagnosis, let alone spell. He was diagnosed here at Children's Hospital of Philadelphia in the Diagnostic Center and today - nearly 10 years later - we deal with complications related to his disease. This rash is our latest concern and we are trying to figure out what could be causing it. (btw Cannon was ok with me posting this picture because it's HPP awareness day and he wants to help spread the word)

Hypophosphatasia - or HPP - has opened up a whole new world to us - a world so complicated yet beautiful, filled with sadness and hope, fear and triumph, pain and peace, anger and joy. 

Today, I am angry at HPP. We suspect his Chiari malformation was caused by craniosynostosis, which is directly related to HPP. He had his surgery and now endures these horrific complications with his surgery including repeat surgeries, staph meningitis, and now some kind of a rash that we are trying to determine a root cause. Through it all, he somehow manages to smile, laugh and tell me he loves me out of the blue. He's a true HPP Hero. And my anger turns to hope for his future to know what great things this boy will accomplish in his life. 

I look online today at how our HPP community is celebrating. We are celebrating the fact that researchers are taking time to care about an ultra rare disease. We celebrate the week anniversary of the first-ever approval of a drug that has changed lives of patients living with HPP and has given many families so much hope after living their lives with none. We celebrate our beautiful children and patients that demonstrate courage that can't be described any other way than awesome. The smiles on children's faces as they tap into their inner superhero strength. They are the real heroes in this HPP Gotham City. I hope you will check out our  public Soft Bones Facebook page or visit our website www.SoftBones.org or even build your own superhero with this superhero builder that is a fun way to raise awareness of HPP www.EveryDayWithHPP.com

So many of you have read this blog and it makes my heart happy to think that I've been able to reach a few people and to make them aware of HPP. That little piece of knowledge can transform lives. That awareness could lead someone to diagnosis -- or inspire new research -- or could spur someone make a donation that funds a grant or a valuable program that makes a huge difference in the lives of these patients. 

A few important things I hope everyone will share:

• Early tooth loss of baby teeth is a telltale sign of HPP. Have a friend or know someone who is dealing with this - please encourage them to see an endocrinologist.
• Pay attention to your blood test. ALP (Alkaline Phosphatase) is a standard measurement on any regular blood panel. My whole life, it has come up flagged as low and no one ever mentioned or paid attention to it. Now I know it's because I'm a carrier. 
• Know someone with osteoporosis that isn't responding to their meds? Check their ALP level. It might be HPP.
• Know someone who keeps fracturing for no known reason or takes a long time to heal? Check that ALP level. It could be HPP.
• Just post that you know someone with HPP and they can learn more by checking out SoftBones.org.

Prayers work - and I hope you will say a little one for us. We would like to put this behind us and get back on the lacrosse field very soon. Happy Friday and Happy HPP Awareness Day!

No news is good... and bad news

Sorry for the delay in an update - we have been taking it day by day.

The good news is that were discharged from CHOP yesterday. The doctors were pleased with the labs and looked like we were headed in the right direction. Cannon's incision is looking like it is healing well. Praise God!

But then... no sooner did we get home that Cannon started to get a fever. So back to the ER we went today (Morristown) for more labs. Luckily things looked ok - so the doctors are puzzled about what might be causing his fevers. It has been a very big challenge for me to distinguish between meningitis symptoms and his post surgery symptoms (fever, stiff neck). They both exhibit the same kinds of clinical symptom so I have kept calling CHOP's docs on call every day to get guidance.

Looks like we are headed back to CHOP tomorrow as an outpatient so that they can keep close watch on him to ensure we aren't dealing with another infection.

In the meantime, we have a crazy schedule trying to balance kids schedules, work and then the medical schedule he is on. He still has his PICC line and he has to get his antibiotic 4 times a day - 6am, noon, 6pm and midnight. The medicine has to sit out at room temp for an hour before dosing and takes about 35-40 minutes to administer which has wreaked havoc on any kind of sleep schedule.

I felt compelled to update everyone on this news in case anyone is wondering why we dropped off the radar. Thank you again for all of your continued thoughts and prayers. We really could use them.

Angels in disguise

While there is a lot of down time at the hospital, we also have our fair share of action as well. Yesterday afternoon and evening were one of those times.

First of all we had some visits from our favorite doctors - the neurosurgery team at CHOP. I call them real life heroes and I mean it. Dr. Heuer, who performed Cannon's surgery, is amazing. He has such a tremendous gift, and is humble through and through. This man does surgery on unborn babies, repairing spinal bifida. And talk about bedside manner, the nurse told me that he french braids little girls hair after doing surgery. Here is pictured with Dr. Hoelscher, another awesome doc here at CHOP.

 World Class Brain Surgeons, Dr. Greg Heuer and Dr. Christian Hoelscher

Another angel - is this man, Ari. 

Our new friend, Ari Miller from 1732 Meats 

This is a complete stranger who heard about Cannon after our friend, Chaz Godwin Ross, frantically called around Philadelphia looking for someone to deliver bacon to the hospital. During her research she learned that Philly is arguably the bacon capital of the world. By fate, she happened to reach Ari Miller. Ari, mind you, doesn't have a restaurant, isn't even a retailer, but a wonderful person who shares a love of bacon. Turns out that he's former banker and attorney who left the rat race to pursue his passion for food, specifically making gourmet cured meats, specializing in none else but bacon! Here's a little about him on his website. http://www.1732meats.com

Now, it would have been one thing to just send over some bacon. But Ari went a step further. He went home from work, and he himself cooked three kinds of bacon - smoked paprika, roasted garlic and black pepper. His daughters even decorated the bags. He drove to the hospital and came up to our room and delivered the bacon himself. And that wasn't all, he also brought t-shirts, stickers and a cooler filled with ice and - you guessed it - pounds of bacon! He has made lifelong customers and friends. Next time we are going to go to his plant to learn more about his trade. I hope everyone will take a moment to check out his website - he ships bacon and also is available in some stores (like Forager Market in New York City, etc.)

Hoping today is a better day and one day closer to bringing home the bacon! 

"Mom, can I go home?"

Boy, how I wish the answer to that question was YES!

As many of you know, we went back into surgery yesterday for a wound "washout". Sounds a lot easier than it was. Basically they reopened his wound, cleaned the edges and removed any fluid pockets and restitched him up. Cannon was not happy about this at all, as it was another general anesthesia, intubation, etc. And another round of morphine, oxycodone, and valium. They also inserted a second drain into his skull to help remove any accumulating fluid which hopefully will help his wound to heal. It looks pretty gruesome compared to the last time but if you think that this is the 4th time his poor head has been stitched up over the past two weeks, I guess it is expected. You can see the yellow cord is the drain that helps to get rid of that extra fluid

He's still on steroids and has had his share of crabby behavior. Roid rage is real people. Geez. Of course as soon as he's done yelling at me though, he apologizes. I'm glad he realizes that he's not really being himself these days.

We had another visit from a therapy dog today - this time a golden doodle which made us both really happy. We miss our dogs, our friends and family back home. This guy, Morty, is really funny. He tried to get the remainder of Cannon's breakfast (you guessed it, bacon!) and we had to quickly snatch it from him! It really is nice to see the dogs and helps us all have a sense of normalcy for a brief second. 

PT also came by, much to Cannon's dismay. It's tough to walk the day after surgery and they were not welcome guests. But we managed to walk a bit and he's feeling a little better. I'll update a little later and hopefully show some more smiles. :) 

Grammy and Bill are visiting today - so we have some guests to entertain us which is terrific.

Thank you again for all your prayers!
XO 

Ups and downs

We haven't updated for a few days because everything is constantly changing and as soon as we post something, it is outdated. Truly ups and our fair share of downs.

The drain has been doing its job to remove extra CSF (cerebral spinal fluid) but its a delicate dance where we are trying figure out how much to drain to keep fluid from leaking out of the incision. This is important so his wound stays dries and can heal and that seal is what regulates the production of CSF. If we drain too little fluid, CSF builds up and can leak out the incision (prolonging healing) and too much drainage causes him to get a wicked headache. Aside from being a nuisance, the CSF leakage can make Cannon especially susceptible to infection, which has us all very nervous and anxious. 

Over the past weekend, we had some additional challenges. First, his incision kept leaking despite having the drain in place. Then, to complicate things even more, his lumbar drain in his lower back started to leak. Because of this leak, he had to have some additional stitches in his lower back. I think the entire PICU floor knows Cannon now after that procedure, as it was with no pain killers.

This morning (Monday), his incision still was leaking, so he had additional stitches where they cleaned out his wound and restitched the part that is having trouble healing. After the ordeal sealing up his drain, they decided to pretty heavily sedate him. Cannon is hilarious when he is on these sedatives. The nurse called it "Truth serum" so of course I took advantage and asked him if he had a girlfriend or had a crush on anyone in his class, to which he replied in a very drunken slur, "Mom, this stuff is strong but it's not that strong for me to tell you that." The entire staff got a big kick out of that. :)


The ups were plenty this weekend. He had lots of visitors! Including his Grace and Clark, Grandmat and Grandpa, Uncle Scott and Aunt Nicole and cousins Bennett and Lara, as well as Will and Tristan from school. And he got LOTS of food. :)

He even had a visitor from Dakota, a therapy dog in training. This really made him very happy and he can't wait to meet the next therapy dog making the rounds.

So now that the wound has been stitched up with what we pray is a tight seal, the drain is in place and  he is resting comfortably, we ask you to pray for healing and to protect him against infection. I can't tell you how much we appreciate the continued prayers and positive thoughts. It really keeps us going. More to come later this week. 

Drain, Drain... Go Away!

Yesterday was a great day, until we got up to take a shower and his incision started leaking again. This means that there is still too much fluid building up behind the incision which is prohibiting it from healing properly, and the stitches just aren't holding it. So last night, Cannon had a lumbar drain placed in his spine, which was very stressful for him.

Despite all of this, he is still in good spirits, eating his bacon, drinking chocolate milk and playing video games. He was really excited today when a box of Mrs. Fields Cookies arrived (which he likes microwaved with a cup of milk).

Another thing that made his day today was a note from the HTS Principal, who said the 5th-8th grades got together to do a project for him. This made him SO happy.... especially when he saw photos of his classmates making a message chain to send to him.

The "chain" is en route to CHOP. We can't wait to see it and decorate his room with it. We are also expecting some visitors! Grace and Clark are on their way with Grandma and Grandpa and he's really excited to see them tonight.

We have our ups and downs each day -- procedures, pokes, prods that don't make anyone happy -- but  then we also have our highlights -- where he is happy to watch movies and have Harry Potter read to him. AND we just found out that we are being moved to a private room (woo hoo!) so good news for now.

As far as how long we will be here, it's hard to say. The drain is there to remove some of the CSF so his incision can heal a bit -- and so they are trying to balance the removal of the fluid with the healing. Once they think the incision has healed enough, they will clamp the drain to see if the incision holds. If it doesn't, we repeat the process. It's not an exact science and it can take a while - so we are guessing at least another week. And hoping that there are no other speed bumps along the way -- so thank you again for your continued thoughts and prayers. More to come tomorrow.

Update: A ray of light!

First, Cannon asked me to thank everyone for all the prayers. They are working!

It's been a tough 24 hours. We found out yesterday that Cannon has staph bacterial meningitis. We have been extremely scared and worried, especially seeing him deteriorate so quickly since Friday. He has been in the PICU as a "no touch" patient, where every time they come in they have to put on masks, gloves and special scrubs.

After they could identify the bacteria, they have been able to adjust his antibiotics to target the strain and he has been responding. His temperature is back to normal. 

Then mid afternoon, Cannon had a PIC line inserted (basically a tube that goes from his arm up through his vein into his chest area) where they can easily administer the antibiotics, fluids and also draw labs. He was NOT happy during this as he was awake... and it was a sterile procedure done with ultrasound and was pretty painful for him. 

Then, the surgeons told us they wanted to schedule additional OR time in case they had to go back in to do a "wash out" - basically reopen his incision to ensure removal of any pockets of fluid that the antibiotic couldn't reach. None of us were happy about this. Last night they did an MRI to see where these possible pockets could be and we were told he would be operated on today (Thursday). 

As of this morning, the plan was still to go to surgery -- so we started prepping. Then about an hour ago, Dr. Heuer's team came in and gave us some good news: Dr. Heuer liked what he saw on the MRI and that additional surgery doesn't appear necessary. AND -- the syrinx (the pocket of fluid that had formed in his spinal column which is the reason he had to have surgery in the first place) appears to have already started getting smaller. 

So now Cannon is thrilled that he can eat -- and we just ordered 5 side orders of bacon. :)

A case manager will be coming shortly to talk to us about possible at home care for the remaining administration of antibiotics so hopefully we will have a plan to get him home soon. 

We are all completely overwhelmed by all of your love, prayers, offers to help the kids, and everything else. Words can't express how much they mean to all of us! This has been a very trying time for all of us and we are thrilled that he seems to be making a positive turn. We hope this means we are on the mend and will have more good news soon.  

Prayers needed

Hello - this has been a real roller coaster for all of us. The latest is this:

• Cannon has been progressively worse after getting his stitches, spiking intermittent fevers (as high as 101.7 at home) and having tenderness in his neck and incision site.
• Brought him back to CHOP Tuesday -- he had a spinal tap so they could test fluid for bacteria. 
• There's still no definite word on what is going on. His spinal fluid tested as if he has bacterial meningitis. 
• There is also a kind of chemical meningitis common with spinal/skull surgeries that presents as bacterial meningitis, however they have to take the fluid sample and see if any bacteria grows. We are in this waiting period.
• Something is growing - but we aren't sure if it's bacteria or a contaminant from the procedure or what. First they said bacterial, then they weren't sure, then suspect chemical. Just don't know yet. However, they are inclined to think that it is bacterial at this point. 
• He's in quite a bit of pain. They are trying to keep him comfortable on pain medications. Last night his fever got as high as 104 degrees. 
• Since they don't know, they are treating as if bacterial to play it safe. He's on Vancomyacin and Cefepime as well as steroids.
• We don't know if he will need more surgery - they are talking about doing a "washout" but don't know what that means yet.

Please continue to keep him - and all of the the people involved in his care in your prayers. It has been very scary for all of us. Thank you. 

Not in the clear just yet...

We have had a great week -- and Cannon seemed to be doing well until yesterday. At first, we thought he was just sweating. His shirt was damp. Cannon called me this morning since I'm traveling and I said how are you and he answered, "I'm sweating like a pig." After the sweating continued, and upon further examination, we realized his incision was leaking. Cue mom's full blown panic mode. 

Turns out he was leaking CSF. Thank God we were able to get him to CHOP into the ER and he was seen by the neurologist on call. He had to get an additional 10 stitches and some glue to hopefully seal him up. However, the doctor warned that he really needs to take it easy because there's only about a 50/50 chance that the sutures will work. If they don't work, they said he will need to be hospitalized and they will have to put in a drain to remove the excess fluid. 

I'm writing this on the plane as I was in Seattle for Soft Bones, and now I'm rushing back to Philly to be with my little guy. I can't get there soon enough. 

Thank you for those of you who continue to pray for Cannon and our family. 

Home, sweet home!

Someone is excited to go home.

Even the neurologist was impressed. The past 24 hours we saw a remarkable turnaround. He was sick, in pain, crying and immobile. Today, he was up and walking, playing games, moving his head around. It was like a different kid. 

Cannon has read (or been read) every single one of your messages, played the videos and enjoyed the photos. When he came home, he had a full envelope of handmade cards from his classmates - we went through each one. Some of them made him laugh really hard, others were done with such care and love, and others were super creative and so impressive. We can't express how much we appreciate it all. The car ride home was a little rough. Those PA/NJ highways aren't exactly the smoothest ride but he managed a smile knowing he was headed home!






The recovery continues. His incision pretty looks good but he is still on a lot of medications (there are like 10). 


Since he had a patch sewn in, he needs to really take it easy and give it time to heal. That means no running around, no roughhousing... it will be a long 6 months of letting things heal.

The hospital is a very sobering experience and being there made us realize how lucky we are. Thank you for all of your continued support and love. We felt it all along the way. 

Bacon, please!

This morning didn't start out so great. Lots of pain, complaining, and tears. But as the day has progressed we have seen a lot of progress!

First he's getting out of bed and even walking a bit... followed by a 3 hour nap.

Then... we thought food might be a possibility. I have no idea what it is about post surgery with this boy, but he could eat POUNDS of bacon. I ordered two orders and he was like, "That's IT?" Wolfed it down and then ate a good size plate of pasta with butter, a chocolate milk, and seems to be holding it down.

If we continue on this path, there's a slight chance we could break the world record and come home tomorrow! Originally the doctor said we should expect Sunday or Saturday best case scenario but today we actually heard the word FRIDAY.  Someone is doing a happy dance!

More to come later on - with pics!