This is my hero's story...

At the request of my husband, Blake himself and several family and friends, I am making a page to tell Blake's ongoing fight with his disease. This will also be where I post his check up and routine cat scan results and where I will keep you informed of any future surgeries. My husband wanted a place to point people to when they ask what it is Blake has. There is not a lot of information on the web about it, you can find some bits and pieces but since it is rare you will not find anything too great to read so I try to describe it in the best way I can. So here it is, first a short and simple version, then below the long and detailed story from beginning to now...

THE SHORT VERSION:

Blake has a rare disease, a very serious life altering/life threatening disease, bilateral cholesteatomas. Bilateral Cholesteatomas are serious, insidious tumors composed of well-differentiated keratinized epithelial cells deep in the middle ear leading upward. He had 2 major medical mastoid surgeries in March and Sept. 2008 at CHOA (Egleston) with his otolaryngologist to remove tumors and a mastoidectomy with simultaneous tympanoplasty and cavity obliteration. His inner ear of his left ear is a complete empty slot, with all bones, tissue and walls torn down and out. They predict he will continue to have these surgeries until his body is done growing (around 18,19) The surgeries themselves pose the same threat that the tumors do, the tumors sit on nerves that control vital function. If not removed, they can cause paralysis, cause him to loose control of facial movements, making it so that he cannot blink, eat, talk, ect. They could shut down his brain. They COULD cause death, though we would never let them sit there that long. The tumors lay on the nerves, so they can't be removed at the root, but as they grow they remove the amount of them that they need to to stop damage. The surgery is risky in the same exact ways the tumors are, if they clipped those tiny, vital nerves during surgery it would be BAD for all the reasons I already explained above, but he has the number one otlaryngologist in America and we trust him. Since it is such a tedious surgery and take 8-10 hours, it makes it even riskier because that is a long time for someone Blake's age to be under anesthesia, especially someone with symptomatic asthma like Blake. And while Blake does not have cancer (PRAISE GOD) he has something just as scary, just as threatening and extremely painful. He lost so much blood during his last surgery that he has to have a blood transfusion.

His last 2 surgeries were successful, no nerves were clipped and the tumors have not grown back so far, it seems the experts know they will at some point, they just do not know when. They go in through his inner ear (the big hole in your ear is the entrance point, and they the cut open the back of his ear, and basically flop his ear over and navigate all the way back, and then up- this leaves his hole in his ear about three times the size of the normal persons) and the tumors are way back there, leading up towards the brain. The surgeries are painful and LONG (8-10 hours, totally about 12 hours with recovery time and waiting for him to get back to his room) each. Those are the longest 10 hours of my life. Recovery is rough and painful and it takes about 6-8 weeks each time. He goes for routine cat scans and check ups with his otolaryngologist often to see where he stands. We take it check up to check up. So far so good, there has been no growth since Sept. 2008. It is like we are all holding our breaths waiting for them to say "OK it is back." We pray that a miracle occurs and it doesn't come back at all, but they say realistically that is not likely, however even his specialist knows God is in control,not us. GOD CAN WORK MIRACLES. Due to the trauma of the tumors and the surgeries, Blake's inner ear and beyond are totally blown out. He has no hearing in his left ear, but has has NO effects from that. His speech and motor skills are perfect, even advanced and his is in the top of his class in every way :-) Eventually when this battle is over he will need surgery to totally reconstruct his inner ear to prevent further problems and damage. His specialist and him have a very special bond. Blake is his most serious case, and he gets lots of special treatment from the specialist, his nurses, the audiologist, etc. :-) This whole experience has made Blake a different kind of child, he is mature, he is wise beyond his years and he is just very special, everyone seems to think so. I think so the most. :-)

THOSE are the basics, above, there are many details I left out. If you are a mom who has felt deep down that something more was wrong than what the doctor was saying, you should read this. Mother's intuition is not to be ignored....

Here is the detailed story, a true account from a mother's prospective of the hardest time in our lives...

THE LONG VERSION

It really began I think when he was born, I think he was born with this, though I could never prove that. Blake was like a lot of babies, he had one ear infection, after another, after another. Sometimes it was months before he had a break in between infections. He got his first set of tubes at 9 months old. It really seemed to help in his right ear, but not his left. NOTHING seemed to help in his left ear. They said this was normal. He saw the ENT doctor ALL the time and his regular pediatrician just as much. I felt like we lived at the doctor's office. They counted one time the number of ear infections he had in just one year, and it was crazy, the number averaged out that he had an ear infection pretty much every day that year. And those were the recorded ear infections. Some may have gone unnoticed.

We knew that his hearing in his left ear was failing, but his right ear did not look so great either, so it never crossed my mind for a while that this was anything more than usual childhood ear problems, why would it? The doctors said it was SO normal. The tubes seemed to improve hearing in his right ear especially. The pattern continued, at 18 months old he got his second set of tubes, again great improvement in the right ear only. I began to get concerned, but again, they assured me that was how it goes sometimes. Blake spent a lot of time at the ENT getting his left ear suctioned out, a lot of time with the audiologist. His audiologist could not figure out why he could speak so well since his hearing was so poor in one ear. Blake has always spoken VERY well!! Another reason I think this has been there since birth. I think his body knows no difference in not hearing from his left ear. He is very articulate, and he was back then at 18 months old as well.

We spent so much time at the ENT, the nurses had fallen in love with Blake. The ENT would always say that he would out grow this. Things continued this way for years. One ear infection after another. And slowly I began to think something else was wrong. It just seemed so abnormal from other kids stories about tubes, ear infections and such. But I am not a doctor and you think you should trust the man with the higher education and the expertise. Right?

Then a day I will never forget came. This is when I KNEW I was right, that something bad was wrong. Blake was almost 4 years old at this point and still always sick, but always extremely smart :-) I went in to get Blake up and I saw a HUGE pool pf blood on his pillow, I immediately ran over and noticed it was coming form his left ear. I screamed for my husband and he came running. It was not a little blood and it was not orange colored drainage like we had seen before, it was bright red blood and it was EVERYWHERE. I shook Blake to make sure he was conscious, he was :-) he SEEMED fine in every aspect except his ear was bleeding, I asked him if it hurt and he said no more than usual. His ear always hurt and it was always draining, but it had never bleed, well I take that back, maybe it had bleed a little after tubes, and after a bad infection, but never anything like this.

We took Blake straight to the doctor, and she said though she could not see down in there because of all the dried blood and gunk, she would assume that Blake's ear drum had burst from a bad infection. She told us to bring him back in 10 days and gave up us a script for a new antibiotic that he had never had. She said if that did not take care of it we were going to have to take the next step (I was thinking, if there was a next step why has it taken so long?). Needless to say the new antibiotic did nothing and his ear continued to bleed, so they drew some of the fluid out of his ear and sent it to the lab to test it for bacteria, to find out exactly which kind of bacteria so that they could treat it with the exact antibiotic it needed. My heart dropped when I got the phone call regarding the results, there was NO bacteria and there must be something bigger going on- something I think I already knew. He was sent to his regular ENT for another set of tubes and to get his opinion. When he came out of the short tube surgery he told us, "It did not look good in there, I believe he has a growth of some sort." When he would not elaborate because he did not want to scare us, my heart sank. He referred us to an otlaryngologist at Egleston, whom we saw 3 days later. They put a "STAT" rush on it.

Upon meeting Dr. Todd (the otolaryngologist) I felt a comfort from him. He is an older man but seemed very wise and he seemed to KNOW what he was talking about. Before Blake even had his first CT scan, he said he knew exactly what it was and that he had a severe case. Blake had hist first CT scan the next day, for which he had to be sedated. He also had x-rays and blood work. His blood count was off. Another bad sign. Dr. T wanted to get the results back right away, so we waited a couple hours and then he sat us down and the first thing he said was "Buckle up, this is going to be a long ride, your son is very sick and there are many details, so many so that your head is about to spin." He was right. The shorter version of what he said: He told us the name of the disease, bilateral cholesteatomas, he said it could be cancerous, but we would not know that until after surgery and biopsy's were sent to pathology. He said Blake would need major surgery and that it was VERY dangerous but not taking the tumors out was even more dangerous. He told us this would be a long, scary road and it would change us and change Blake and he was right. He said it was very dangerous and very serious and we needed to treat it accordingly. He said Blake could go into surgery a normal child and come out disabled, with paralysis or worse but leaving the tumors alone was a sure road to being those things. He also said that while this disease is rare the type of it and severity of it that Blake had was even more rare.

On March 3, 2008 Blake had his first surgery. It was 8 hours long. I am not sure I even took a breath the whole time, though I am certain I cannot hold my breath for that long. It was successful (meaning no nerves were damaged and he pulled through) but it was a hard recovery. He had bandages that had to be changed every day, and it was so painful for him. It took him about 8 weeks to recover fully, about 5 weeks to even feel better. He had to go back to his specialist every week to have more and more of the bandages deep within his ear removed, and every time Blake would lay there and patiently let his doctor do what he needed but the whole time with tears streaming down his face. It hurt him so bad, I could not feel his actual pain, but I think it hurt me even more. The good news was that while the part sent tot he lab did have some "abnormal cells and precancerous cells" it was not cancer.

Things were going good for a while and they thought it would be at least another 2 years before Blake would need another surgery, but in October 2008 we went in for a routine check up like we did every month and they sent him back for a CT scan and we got news no one expected, it was back and had tripled in size, posing even bigger threats. He needed immediate surgery and this time it would take longer and be even harder, I didn't think it could get any harder than it had been the first time. He had his second major surgery on Sept. 12, 2008 and it was 10 hours this time and recovery was harder. I knew when they wheeled him back in the room form recovery that it was worse, the first time Blake had actually been acting OK and alert and like he was feeling OK. This time he looked and acted pitiful. You knew right away it was 10 times worse. His daily care of his bandages was even more painful and harder and again, he had to see Dr. T every week to pull more and more of the inner bandages out. A painful process in itself. It took about 12 weeks for him to get back to normal this time.

Blake was a real trooper the entire time. He kept a smile on his face and an upbeat attitude the whole time and we fed off of his good attitude. I thought if he sees me crumble, it will scare him so I tried to remain upbeat and strong for him, but when we would come home form the hospital stay after surgery, I would retreat to the bathroom or my room and just cry and cry. I can tell you his story but I could never put into words just how extreme and dangerous what he has is and how hard and painful the surgeries were. You would just have to have been there. Even just to have to sit there and hurt him while changing bandages, while tears stream down his face but you know you have to do it or it could become infected, is not easy. My heart was broken for my child. He has some lasting effects that may never change, such as hearing loss in that ear (thank God, by some miracle his speech has not been affected in any way), he cannot get water in that ear and his ear hole (think were you insert the q-tip or ear plugs) remains stretched out and much bigger than a normal ear. His ear also comes off his head farther than other peoples. But Hadley anyone even notices now.

We will fight this battle for years to come and we ask for your continued prayers for Blake. One day when he is an adult and his body is done growing, this battle will be over and we can all take a deep breath. We pray that even when it is active and surgery is needed that God will watch over him and protect him from losing any vital functions and from paralysis and worse. We try not to focus on his battle while it is idle. But at other times, it has to be our main focus. Blake gets very nerved up before his routine check ups and cat scans, and honestly so do I. But he takes it all in stride. Other than this, he is a normal child. He goes to Kindergarten, where he does exceptionally well. I cannot think about all Blake has been through, whiteout streaming tears out of my eyes. What he went through was a hell on Earth, especially for a child, not child should have to hurt that much. I have heard my child cry out in pain "Mama please don't, it hurts so bad, please don't hurt me anymore." While changing out his bandages more times than I even care to count. So while Blake does not have cancer (PRAISE GOD) he has something just as scary, just as threatening and extremely painful.