Sunday, January 29, 2012

Bestie's



Zachary loves spending time with his friend. They don't get to spend as much time together as they would like, but when they do get together they are hard to pull away from each other. Thank you, Alex for being to Z what your mom is to me. You Rock!! ;)

Friday, January 27, 2012

A Mother's Letter to the Man in Charge

Dear Father,

I know of your great love for your children ... I know that prayers are answered and blessings are given. I just wished sometimes that I could understand why things that hurt us and our loved ones have to happen. I know your plan is bigger than I can ever comprehend. I truly want to understand, I really do. I love thee with all my being and I will do my best to trust in you and not question, why me? or why us? Lord, could you please have compassion for our son Zachary and give him the strength and the courage to get through the trials that tho hast for him? I love him so much and it hurts my heart to see him suffer, please Lord, if there is a mission for him to teach others under the circumstances thrown his way, please give him strength to endure. Thank you Lord, for all the many blessings that thou hast given me and thank you Lord, for my wonderful husband who understands me more than I understand myself, thank you, for sharing your beautiful little spirits with me, I am honored to be in their presence. Please Lord, would you let my parents know that I think of them often and I can feel their presence when I need them most, I didn't know that even still ... they are my rock. Thank you Lord, for my friends who pick me up when I fall and explain your love to me when I do not understand or I forget. Thank you Lord, for all that you do for our family I love thee and cherish thee.

With Love Your Daughter
Michelle

Zachary had a PAIN FREE day!! Thank you, Father!!! :D 

Thursday, January 26, 2012

An Update on Pain

I haven't been entering blogs because everyday is much the same. Z experiences pain during the evenings and on occasion has had to wear his TENS to school, I've even had to go to the school to put his unit on for him. We treat the pain with 2 Benadryl most of the time only every once in awhile we lean toward that Nyquil. He did take Nyquil once before school because his pain was pretty bad, he said he almost fell asleep at school, but I honestly don't know what else we can do, he can't keep missing school. We take it one day at a time and we hope and pray that the doctors will get to the bottom of whatever it is that is causing Z's mobility issues. As far as pain we are just hoping that once the mobility issues are resolved that either the pain will also be resolved or that we will be able to manage the pain well enough that it doesn't affect him so much.

Friday, January 13, 2012

Break from School .... but not from Pain

Z made it through most of the day without pain, he came to me at about 6:00 p.m. with pain in his legs. I gave him 2 Benadryl, then around 9:00 I gave him a Tablespoon of NyQuil so he could rest from the pain. He does his PT everyday at 6:45. I'm planning to have him do that twice a day to see if it makes a difference.

Thursday, January 12, 2012

Zachary's Pain is Changing

Dr. Jordan
Zachary had one of his really bad episodes where the Benadryl did not work at all on Saturday. Monday and Tuesday he complained of back pain so I gave him a Benadryl and sent him on to school. Today he complained of his legs and back pain I gave him a Benadryl and sent him to school with his TENS on.

For more in depth/detailed information go to http://hug-z.blogspot.com

Dear Mrs. Huguley, I have spent hours looking at Zachary's spine MRI looking for other explanations for his spine stiffness. His vertebra (bones) look fine to me and to radiology. This is one reason that I am asking several specialists to review Zachary's case with me. I would like someone with spine and/or spinal rehabilation expertise to see him. I spoke to both Dr. Levy in Neurology and Dr. Sudhir Kathuria in neuroradiology/interventional radiology at Johns Hopkins. They feel that rather than an arachnoid cyst along his thoracic spine that he most likely has an old subdural fluid collection, perhaps this occured at the time of the MVA years ago. The subdural fluid can be seen on MRI and appears to be clear fluid, not blood but the way that fluid gets into the subdural space is usually tearing of very small veins in the subdural space at the time of trauma. Over time, the blood is removed by the body and clear fluid is left. This fluid collection stretches from about T2-T10. Arachnoid cysts and subdural fluid collections are very similar in appearance, but you are usually born with an arachnoid cyst and a subdural fluid collection may appear after trauma. There is not a way to "repair" or remove this fluid from along the spine according to several neurosurgeons and neuroradiologists and his spinal cord looks "healthy." Superficial siderosis is still the diagnosis. I can email you some slides that show the adhesion/fluid collection in the thoracic spinal cord (I will remove Zachary's name and medical record number as they will be sent via regular email). If they do not make sense, I can review them with you at our next appointment.

Wednesday, January 11, 2012

The Back Pain Continues

I gave him a Benadryl and sent him on to school. After school he told me he still had back pain, I asked if he had the pain through out the day and he said, "Yes, but not really bad enough for the TENS". I told him that if it gets that bad while at school to try the TENS and if it doesn't help to have the school call me.

A Plea for Mercy

Dr. Jordan
For two days in a row Zachary has endured some pretty intense pain. We aren't going to physical therapy anymore as the therapist and myself felt that his pain could be managed by just doing the same exercises at home as he does there, which he has been doing since before Christmas, and has done very well, however, his pain here the last two days has been pretty bad. I've done everything on my list. I've given him benadryl he's had 3 of them between 3:00 and 7:00, it doesn't seem to be doing it's magic anymore. I've put his TENS on, sometimes he's not even sure if it's on because the pain is so bad. I've put ice packs on his back and on the backs of his legs, I've rubbed his legs with his pain cream. Dr. Jordan? Please, there has got to be something else we can do to help my son through this pain. Please, I am begging for him. I've done all I can think of to do to help him, I don't know what else to do. I don't want him to have to endure this pain anymore.

Tuesday, January 10, 2012

Zachary Turns 10 ... and Now the Pain Moves to the Back

Z is showing signs that to much walking or to much activity can and will cause him pain. I wonder, and keep in mind I'm no doctor ... but I wonder if there were a way to immobilize Z's back so that the pulling and tugging are kept at a minimum. It seems that when he's active and there's a lot of running or jumping, it's followed with the pain. I don't know if it's even possible to do something like that and Z still be able to run and jump and be a kid, you know?



Z woke up this morning with back pain ... this is a "new" pain .... not sure if he just slept wrong or if there is something else going on. Keeping a close watch on that today. I gave him a Benadryl and sent him to school. He did fine the rest of the day.



Monday, January 9, 2012

Starting the Day with a Headache

Zachary woke up with a headache this morning, I gave him a regular strength Tylenol and encouraged him to drink some water, hopefully he will make it through the day.


Update on Z ....
Zachary stayed the night at his best friends house Friday night, he has been wanting to go over there and spend time with his friend since the beginning of Christmas vacation. He was so excited! At about 11:45p.m Zachary requested to have some of his pain cream rubbed on his legs, he laid on the floor on his side with his knees bent and his TENS on. He had a total of two Benadryl, an ice pack on his legs by his request and he finally settled in ... at about 12:30 or so.

Zachary realized today the reason for his pain last night ... he remembered that he played dodgeball at school during his P.E. class. Today we went out to the mall for a few items and Z tapped me on my arm and said that he was starting to feel pain again so I opted to pick him up and carry him as opposed to making him walk. For some reason walking can cause the pain to increase. When we arrived at home his pain was pretty bad so I gave him two benadryl, and his dad put his TENS unit on. He was still in pain even after the 2 benadryl, when we got to the place where we would get our photos taken Z's pain increased dramatically, he was tensing up (this is to include his hands and feet) and screaming, I gave him another benadryl in hopes that it would slow the pain down, but unfortunately it didn't touch the pain at all. So, no family portraits for us. Instead we went over to our friends house who lives fairly close, I ran Zachary in, laid him on the floor where he could position himself comfortably and I pulled out his pain cream and started to massage his leg, after a good while he said that it was getting a little bit better, then after a while longer he said that it was getting bad again. I just wanted to get him home where he could be comfortable, when I lifted him to an almost sitting position the pain got intense again. We loaded him into the back seat, let him lay down, as that seems to be a more comfortable position for him, and then the pain was getting to where he was moaning (after moaning comes the screaming). When we got home I ran him upstairs, and while I was carrying him the screaming kicked in. He was screaming for about half an hour to an hour, then the moaning came back again. I asked him how he was and he said he felt like he needed to throw up, that his eyes were hurting and that the light made them hurt more and his legs were still tensing up.The moaning lasted almost three hours .... after that I decided that was enough and gave him a tablespoon of NyQuil. He fell asleep about 15min later and is finally resting now.
I don't know what to do, I know the doctors are trying to find ways to help Zachary, but they just don't seem to be working fast enough. No child should have to suffer this kind of pain for this amount of time. My hope is that they will be able to find some kind of pain medication that will work when he has episodes like this one. The benedryl seems to work okay for the little starting pain and usually keeps the harsh pain at bay, but the intense pain? It doesn't seem to touch that pain at all. :( I hate it when it gets to where I have no more options to help stop the pain, it's very frustrating, because all a mom wants is for her child is to be happy and healthy. This pain is terrible and is now, officially the enemy!! I don't want this enemy around my son anymore! He's had enough of this torture!!

Sunday, January 8, 2012

Staying Home

We kept Z home from church today, Robert and I didn't want to risk him going through another intense night and the past few days has worn the poor little guy out. He kept his TENS on all day, it automatically shuts off after 45 minutes, so he took a few breaks from it.

Saturday, January 7, 2012

Seriously Intense Pain

Zachary realized today the reason for his pain last night ... he remembered that he played dodgeball at school during his P.E. class. Today we went out to the mall for a few items and Z tapped me on my arm and said that he was starting to feel pain again so I opted to pick him up and carry him as opposed to making him walk. For some reason walking can cause the pain to increase. When we arrived at home his pain was pretty bad so I gave him two benadryl, and his dad put his TENS unit on. He was still in pain even after the 2 benadryl, when we got to the place where we would get our photos taken Z's pain increased dramatically, he was tensing up (this is to include his hands and feet) and screaming, I gave him another benadryl in hopes that it would slow the pain down, but unfortunately it didn't touch the pain at all. So, no family portraits for us. Instead we went over to our friends house who lives fairly close, I ran Zachary in, laid him on the floor where he could position himself comfortably and I pulled out his pain cream and started to massage his leg, after a good while he said that it was getting a little bit better, then after a while longer he said that it was getting bad again. I just wanted to get him home where he could be comfortable, when I lifted him to an almost sitting position the pain got intense again. We loaded him into the back seat, let him lay down, as that seems to be a more comfortable position for him, and then the pain was getting to where he was moaning (after moaning comes the screaming). When we got home I ran him upstairs, and while I was carrying him the screaming kicked in. He was screaming for about half an hour to an hour, then the moaning came back again. I asked him how he was and he said he felt like he needed to throw up, that his eyes were hurting and that the light made them hurt more and his legs were still tensing up.


The moaning lasted almost three hours .... after that I decided that was enough and gave him a tablespoon of NyQuil. He fell asleep about 15min later and is finally resting now.
I don't know what to do, I know the doctors are trying to find ways to help Zachary, but they just don't seem to be working fast enough. No child should have to suffer this kind of pain for this amount of time. My hope is that they will be able to find some kind of pain medication that will work when he has episodes like this one. The benedryl seems to work okay for the little starting pain and usually keeps the harsh pain at bay, but the intense pain? It doesn't seem to touch that pain at all. :( I hate it when it gets to where I have no more options to help stop the pain left, it's very frustrating, because all a mom wants is for her child to be happy and healthy. This pain is terrible and is now, officially the enemy!! I don't want this enemy around my son anymore! He's had enough of this torture!!

More Pain!

Zachary stayed the night at his best friends house, he has been wanting to go over there and spend time with his friend since the beginning of Christmas vacation. At about 11:45p.m Zachary requested to have some of his pain cream rubbed on his legs, he laid on the floor on his side with his knees bent and his TENS on. He had a total of two Benadryl, an ice pack on his legs by his request and he finally settled down in ... at about 12:30 or so.

Wednesday, January 4, 2012

Questions for Dr. Jordan & Her Responses

Dr. Jordan,

I was wondering if you would be able to look at Zachary's other MRI's/Xrays to see if there is anything there that can help with the diagnosis of the immobility of his back?

I also wanted to be sure I understood that the findings from Z's Meylogram shows that the leak is in a place that can not be repaired and that SS is still the diagnosis for that.

I also wanted to find out if there is a way for my husband and I to see the pictures which show the adhesion of the lining of Z's spinal cord. We feel that the education and the viewing of these pictures will help us to better understand how and exactly where this is taking place in our son's body.

Thank you so ... much for all you do!
Michelle Huguley

Dear Mrs. Huguley, 


I have spent hours looking at Zachary's spine MRI looking for other explanations for his spine stiffness. His vertebra (bones) look fine to me and to radiology. This is one reason that I am asking several specialists to review Zachary's case with me. I would like someone with spine and/or spinal rehabilation expertise to see him. I spoke to both Dr. Levy in Neurology and Dr. Sudhir Kathuria in neuroradiology/interventional radiology at Johns Hopkins. They feel that rather than an arachnoid cyst along his thoracic spine that he most likely has an old subdural fluid collection, perhaps this occured at the time of the MVA years ago. The subdural fluid can be seen on MRI and appears to be clear fluid, not blood but the way that fluid gets into the subdural space is usually tearing of very small veins in the subdural space at the time of trauma. Over time, the blood is removed by the body and clear fluid is left. This fluid collection stretches from about T2-T10. Arachnoid cysts and subdural fluid collections are very similar in appearance, but you are usually born with an arachnoid cyst and a subdural fluid collection may appear after trauma. There is not a way to "repair" or remove this fluid from along the spine according to several neurosurgeons and neuroradiologists and his spinal cord looks "healthy." Superficial siderosis is still the diagnosis. I can email you some slides that show the adhesion/fluid collection in the thoracic spinal cord (I will remove Zachary's name and medical record number as they will be sent via regular email). If they do not make sense, I can review them with you at our next appointment.


Radiology Report


- Large intradural filling defect along the dorsal aspect of the thoracic spinal cord.
- This is the most suggestive for an arachnoid cyst, a component of fibrosis however is not excludable.
- It is not possible to determine whether this filling defect is the cause or the result of the patient's extensive hemosiderosis.



Filling defect. Looks like a crescent instead of a ring. Extensive in t-spine


Many axial images show this crescent (filling defect)


Harder to see on sagittal images, but posteriorly, contrast does not move as well. Should be white contrast material on either side of the gray, tube-like spinal cord.

Zachary has been doing his PT here at home since before Christmas and it seems to be to keeping the pain to a minimum, however, today after school he came home in some pretty serious pain, it took two benadryl between the hours of 3:00 and 6:30, and his TENS on a much higher setting to get him even close to being able to join the boys at our church for Cub Scouts, he played some dodgeball and is paying the price for that now. :(  



Tuesday, January 3, 2012

A Letter to Dr. Levy, His Thoughts, Dr. Jordan's Thoughts & Then There's Pain


Dr. Levy
I was wondering if we could have a copy of the reports you and your  
team made when you reviewed Zachary's Myelogram. We think we  
understand your findings but would like to read them ourselves. It  
would be really nice if we could see pictures of the area that is  
causing the SS in Zachary. Not sure why powerpoint presentations  
aren't made for parents who want to see the details of their child's  
disorder, :) . It would be a perk, that's for sure, lol.

I also wanted to ask, if the leak is in a place where it can not be  
repaired, what else is there that we can do to slow the progress of  
the SS and it's symptoms? We aren't sure what else to do. We are
concerned about his immobility  and Dr. Jordan is working on a
plan for Zachary to figure out the issues with that.

Thank you, for all you do to help those who suffer with SS. We are in  
hopes that there will be a cure for it soon and that Zachary and those  
who suffer will finally be free from it's wrath.

Michelle Huguley

Michelle,
I think chelation therapy is Zachary's best option at this point. Even if the leak cannot be surgically corrected, you can use a medication to remove the iron that is depositing on the spinal cord. You just have to remove more iron than is being deposited. We previously demonstrated the efficacy of Ferriprox, an iron chelator that can reach the spinal cord, in a trial of 10 adults and it showed some improvement by MRI in as little as 3 months. We have expanded that trial to 20 adults for 2 years, but since the drug is now FDA approved in the US, you can consider a personal trial for Zachary. I've discussed this possibility with Dr Jordan.

Michael Levy


Dr. Jordan's thoughts about the Ferriprox ... (11/16/11)
What about Ferriprox? Is Z able to get that yet? If Z were getting worse then this would be something we would look into but because he's pretty stable right now and the drug has just been approved by the FDA I think we should just sit tight for now. I worry that with this drug being new and not widely tested that there may be side effects that would do more harm than good for Zachary, and we really don't want to put Zachary through that if we don't need to. I feel that this drug could help Zachary, but I think we need to make it a last resort, because it is new and we don't know what kind of side effects will come 
with it


PAIN
3:00 a.m. Zachary comes up to have his TENS put on ... 

Monday, January 2, 2012

Some Pain ...

Zachary came upstairs 30 minutes or so after going to bed needing a benadryl and his TENS unit put on. :(

Sunday, January 1, 2012

Coming Full Circle

We saw a specialist (orthopedist) to start because of the excruciating pain that Zachary was experiencing she diagnosed Z with a bone infection then after treating the "so called" infection we are told that no, it is not a bone infection but seems to be hamstring contractures. Then we start going to physical therapy to help stretch the muscles causing the contractures. Then his therapist notices that there is more going on than contractures, there's something causing immobility in his back and referred us to a sports doctor who does an MRI and finds that in fact there is inflammation at the base of Z's spine and then he refers us to a neurologist who diagnoses him with Superficial Sideosis and our goal is to find the leak that is causing the inflammation which in turn is causing Z serious pain. Then when what we think is the culprit and cause to all of Z's troubles we find that there is no way to repair it and that the immobility is being caused not from the superficial siderosis but from something else and whatever it is it mimicks another disease or disorder and now we are being referred back to the same doctor we started with who misdiagnosed Z twice. We've come full circle and now .... Sit and wonder about whether or not we should grab all our previous tests and get some other opinions or not. Rolling eyes! It's sooo .... Flipping' aggravating!


And let's not forget that this morning our son was in pain and had to have his TENS put on and a Benedryl to get him through church. I think I'm coming to the end of my "patient rope" nothing upsets me more than to see my son in pain and I have no power to cease/stop it.