One Week and Counting...

I can't believe we have been on this journey for five and a half years... Just when I think we have it handled...things change. We are one week away from heading to Madison, Wisconsin, where Hank will have his 5th surgery. My heart breaks for this kid...having to face another operation, another hospital stay...another recovery...and just all the fear and worry that goes with it. Hank is stoic...he doesn't mention it...and neither do I. But we know it is there...looming ahead...and neither one of us is happy about it.



First day of school...Last day of school...grow much?

We waited 6 weeks to hear back from UCSF, for the referrals to the other doctors they wanted Hank to see. By the time they finally called us, we were planning the trip to Madison. After waiting so long, all they did was refer us to 2 different departments...cranial and plastics. Well I could have done that myself! "Is there a particular doctor we should see?" I asked. "No..." said the receptionist, "just call the numbers I gave you and they will set you up with some appointments." Hmm...so our child has a disease that affects less than 2,000 people worldwide, and you want us to see just anyone? That didn't even make sense to me...

Luckily, I had received a call from Hank's surgeon, Dr. Siebert, the week before. "Hank doesn't need that bone surgery," was the first thing I heard, "He is too young, and even then, I don't know if he will ever need it." I could feel the weight of the world start to lift off my shoulders. "But what about the other people who have needed bone surgery?" I asked, "Isn't Hank going to need it too? I know his cheek bone has deteriorated." Dr. Siebert explained, "Hank was better off than most, when he came to me, we caught it earlier...Yes, his bone has deteriorated, we know that...fixing the tissue is to mask it...but I don't know if he will ever need bone surgery...and certainly not now." I couldn't believe my ears! Just what I wanted to hear..."Bring him to Wisconsin," he said, "We need to lift the flap and remove some tissue, it's weighing down his eyelid." I took a deep breath, "Hank does not want another surgery and Jeff and I don't want to put him through anything unless it is absolutely necessary."

There was a pause...and then I heard the words that squeezed my heart..."Hank is going to be 13. These are the years that matter. The years that form who we are. Hank deserves to feel confident...to look his best. Hank deserves that chance. We owe it to him...I owe it to him... I owe it to this kid to give him the best chance I can. We need to do this for Hank." My heart sank. I knew he was right... He then went on to talk about Hank's eye and all the things that could go wrong if we don't do the surgery and the tissue continues to pull his eyelid down...won't be able to close his eye, resulting in eye ulcers, dryness, and many other issues. And so that was that. Surgery on June 19th...now how were we going to tell Hank?

The next week was crazy busy, with me working long hours and putting on three events that week. I only saw Hank for an hour before school and knew that I couldn't tell him then. And so we waited all week to tell him. It was painful...and worrisome...how would he react? We finally found our chance that weekend...I had to work on Saturday, so Sunday after Mass would have to be the time. As Jeff and I made our way up the stairs to Hank's room, my heart was heavy. I rehearsed in my head what I would say. Hank was sitting on his bed, playing a game on his laptop. He looked up when we came in and I saw the questions on his face. Must be serious...both mom and dad in his room...and wanting to talk!

Jeff sat down next to Hank and I sat on Charlie's bed across the room. We started off by telling him that he would not be having bone surgery. "Oh thank God!" he exclaimed as he looked up at the ceiling in relief. "but..."said Jeff, "you do need the tissue surgery in Wisconsin." Hank's face fell..."NO! NO NO NO!" He said in a voice laced with anger and disappointment. "We are so sorry," I said, "but we have to do this...you need it.", and went on to explain why. Hank sat silently and listened...and then the tears started to roll down his cheeks. He tried to wipe them away before we saw them, but they were too quick and there were too many. "No..." he said quietly. I felt terrible. This was awful. It was so much easier when he was little. He didn't quite understand and he didn't seem to remember the surgeries. But now...now he gets it...and he remembers...and I don't blame him...I wouldn't want it either.

I watched his face and could see the range of emotions...anger, disappointment, sorrow...and then resignation... "OK. Fine. I'll do it," as he wiped the tears away and sat up. And that was that. I knew he was angry and hurt...but he did not want to talk about it... he wanted to be alone. We left the room and went downstairs...and then it was my turn to cry...

I sent Dr. Siebert a text..."We just told Hank about his surgery. He is very upset. Would you be willing to talk to him about it?" The response came back right away..."Absolutely!" He called that night. "Hi Terri, It's Dr. Siebert. Is Hank available?" (I love this man) I handed Hank the phone, "Oh Hi Dr. Siebert!" Hank exclaimed when he heard the voice on the other line. They talked for a few minutes, and I heard Hank say, "OK. See you in a few weeks." Whew...he sounded OK. Hank handed the phone back to me..."I told Hank not to be mad at mom and dad," Dr. Siebert said happily, "I told him to be mad at me...it's not mom and dad's fault, but you need to have this done. He'll be fine now." Once again Dr. Siebert leaves me speechless..."See you in a few weeks!" he said, and we hung up.



Last day of 7th grade

It's been two weeks now...Hank seems OK. Summer has started, with the last day of school just a few days ago. Hank received two awards and sailed through the year with good grades. He is happy to have this week at home to just relax. Unfortunately, we will be celebrating his 13th birthday at the Ronald McDonald House, and therefore he has told me, that I we will be having his party this weekend. (What's one more thing to do before we leave?) I am trying to get him to have a slumber party...outside in our tent, but he is indignant..."You want us to sleep outside?" He clearly doesn't get the whole "camping vibe" that we are trying to create...

Today is the day of our fundraiser. "Pizza With a Purpose". It's our first one since moving back to our hometown. Wish I had time to really plan one and advertise. We put this together in a day...but with our kids' amazing school, and the radio station running a PSA about it, it should go well!

Little miracles have been happening this whole last week. A letter came from the State Controller's Office that said a local bank had lost funds of mine. I went to the bank and found that it was just enough to cover the plane fare to get us to Chicago and back...the radio station recording a PSA for Hank's fundraiser...unsolicited all about 12 year old Hank Gibbs and his fight against Parry Romberg Syndrome!...and last night...last night put me over the edge of stunned gratitude...

Hank is not the only one having surgery next week...13 year old Kristina, from Atlanta will also undergo surgery for her PRS. Mom Karen and I have become close friends over the years...and saw an opportunity to meet in real life and for the kids to have someone to go through the surgeries with. I had put out note on Facebook, asking if anyone had an "in" with the Chicago Cubs...we will be flying out of Chicago and would love to take the kids to a game...but tickets are pricey and our money needs to go towards the necessities...



Our surprise visit with the McQuillen Family in NYC 2009

Last night I got a message from another PRS mom, Flora McQuillen, "How many tickets do you need?" she asked...I responded with a cringe..."nine. 5 for our family and 4 for Kristina's family". (nine is a lot to hope for...) I told her if we had one family covered, we could split the cost of the other tickets... The next thing I know...she sends me an email with a confirmation attached...she and her family have purchased our tickets...all of them...NINE! I couldn't believe it...Here is another family who have walked this walk that we are on. We met them in New York after Hank's first surgery. Their son Pat, was 13 at the time and had already had his first surgery. I will never forget how excited we all were, to meet in person! Pat was the first person with PRS that Hank had ever met and I was so grateful to get the chance to meet them. It was a short visit...just coffee before they caught the train home...but it is a memory that I will treasure forever. I can't thank Flora enough for her generosity. I was in tears as I let Jeff know...and still in tears when I told Karen. And then she was in tears too...
I have no idea what today will bring...The fundraiser starts at 11:00 and goes all day. I will be running back and forth from work, so that we can be there at lunch and again at dinner. I have high hopes that Hank will have some friends there...we will raise some money for Hank...and raise awareness for this disease does not define who we are...but has definitely changed our priorities and opened us up to things we never imagined...good and bad. Today is all about Hank...strong and stoic Hank...facing surgery #5 with grace and courage...


* Note: I started this blog years ago, to keep our family and friends informed on Hank's illness and progress. This is a disease full of unknowns...unknown cause, unknown progression and it affects each person differently. Although we wish Hank did not have to have surgeries, the alternative is much worse and we are extremely grateful we have Dr. John Siebert in our corner. I can't imagine what Hank's life would be like if we never had the micro-vascular free flap tissue transfer. These surgeries give Hank the normal life we have prayed for and we would not change a thing!

Game Changer

Hank is almost 13...in just 2 months, he will be a teenager...and he already looks like one! Hank has grown at least 5 inches in the last year...going from little boy to young man overnight! And nothing reminds me more of this, than the fact that Hank is starting to want to know and have some say about his medical care.



L: Easter 2013, R: Easter 2014

After Hank's last surgery in October 2012, Jeff and I told Hank that "this is it...no more surgeries unless medically necessary or if you ask for it." Here we are a year and a half later and faced with the knowledge that more surgeries are in his future. Hank is scheduled for surgery this June in Madison, with Dr. Siebert. I have known for a few months, but didn't want to tell Hank until he got through all his big projects at school. But this week, push came to shove and I had to tell him.

Waiting Room with a view!

We are up in San Francisco right now...after having gone to UCSF for an appointment with an ocular plastic surgeon. Hank's eye looks like it is falling...so Dr. Siebert referred us to a doctor in SF for a consult. I don't know what I expected...but what I got...was NOT what I expected! In all honestly, I was supposed to bring Hank here a year ago! But after just having a surgery, I couldn't bear to take him to yet another doctor...so I dragged my feet until I HAD to go...but I wanted to make sure before heading to Madison for surgery, that we had all the info.



Always a smile on his face!

The good news is that Hank's eyesight is pretty normal (although, he could benefit from glasses...which makes glasses-wearing-little-brother-Charlie, quite happy!)..and Hank's left eye is just a millimeter lower than his right eye...which is just where most people are. So what we are seeing is not Hank's eye "dropping" but his lower eyelid being pulled down. What is less than happy...is the theory is that his bones have been affected...and after the doctor had me place my hands on Hank's cheekbones, I know he is right. The right cheekbone is where it should be...the left is flat...the bone has deteriorated.

After examining Hank, the doctor told us, "I don't think he has Parry Romberg Syndrome." WHAT? How is that statement even possible? I pointed out that two doctors at UCSF diagnosed him with PRS over 5 years ago, as did our pediatrician and Hank's surgeon! He pointed out that Hank does not have "en coup de saber" which is the indentation of the forehead. I told him not everyone manifests that way...and then he said that PRS only affects soft tissue and not bone, and yet clearly...Hank's bone has been affected. Again, I explained that Hank had a micro-vascular free flap tissue transfer 5 years ago and then 3 revisions, so of COURSE his tissue would look good! At that point, the doctor, turned and looked at me, "You have obviously done research, and probably know more about this disease than I do..." (which reversed the opinion of him that I was quickly forming..) 

So here's the bottom line....This doctor thinks Hank needs bone surgery....either a bone graft or a surgery to move the bones forward. He wants Hank to come back and see the cranial team....the facial plastic surgeons, to have an MRI or CAT scan, depending on what those doctors think, and then to come back and see him in 2 months. But in less than two months, we are supposed to be in Madison, Wisconsin, where Hank will be undergoing surgery to raise the tissue up around his eye and debulk his cheek. ARGH!!!

"Hank is scheduled for surgery in two months!" I exclaimed, "What do we do? Go forward or wait? I don't want to put Hank through another surgery that won't work!" The doctor said that the tissue lifting is just to mask what is really happening and would turn out much better with the bone addressed first. Now here is where my heart breaks...I KNOW what this surgery is...I have been afraid of this surgery for years, and have been PRAYING that Hank would avoid it. But the fact is, our friend, Kyle, in Connecticut had to undergo this surgery when he was 17. His family flew out to UCLA for it, and I remember his mother Patty, telling me how terrible it was. Hank was just 8 at the time...and that seemed so foreign to me...but in the back of my mind, I have never forgotten her words..."Worst surgery he had ever been through...and the recovery was horrible"... I remember the photos of the aftermath, and her telling me how angry and miserable he was....

Hank is happy....he thinks the bone surgery will be better than the tissue surgery, "I am OK with it as long as I don't swell like I do after the other surgeries..." Ahh....ignorance is bliss!

We left that appointment with Hank in good spirits and Jeff glad to have more info...but my stomach is in my feet and I feel lost. We had a plan...I liked our plan...with a doctor we know and trust. A doctor who understands the pathology of the disease. I know this new doctor is knowledgeable about his field...but this feels like it's a bit foreign to him...and I don't like it.

After a beautiful dinner at our favorite San Francisco restaurant, Scoma's...and a much needed drink or two...I once again have put this in God's hands. I can't worry about it...I know the path will unfold and we will follow it, just like we have in the past. (OK, who am I kidding....I will still worry about this 24/7)

I sat down this morning to send a note to Dr. Siebert, and to ask him to call this doctor. Halfway through the email, my cell phone rang and on the other end was the tirelessly upbeat voice of our favorite doctor, Dr. Siebert!! What a relief! We had a long conversation...he reminded me that we have known about the bone...and that we have been just doing the best we can with the tissue...(I think I have been in denial about that). He is going to call this doctor, mull it over and call me on Monday to come up with our game plan. I love that he asked what Hank thinks...which I replied, "That he never wants another surgery!" and he asked if Hank's eye hurts, or is red... I believe he is trying to determine severity of his eye...

So now we wait...to hear from Dr. Siebert...whether we go forward here at UCSF or south to UCLA, I think we have a couple of months of doctor appointments and tests. I don't know if we will end up in Madison or not...which is a killer, since we had planned to meet our friends from Atlanta in Madison, where Hank and Kristina would undergo their surgeries on the same day...giving two kids with PRS a chance to hang out...and two moms and dads a chance to connect in REAL life with people who understand EXACTLY how they feel!

Please keep Hank in your prayers...I continue to pray for a miracle and hope for a cure! In the meantime, we are heading out to have some fun in San Francisco and join some friends for tonight's Giant's game! Life continue's on...and we will continue to meet each hurdle head on...together...!