Pulling

Zachary came to me after school complaining that something felt like it was pulling in his legs. I gave him Benadryl to relax his muscles and put the TENS unit on him. 

A Trip to Vanderbilt

Over the past week or so Z has had minimal pain he occasionally has had to use his TENS unit I would say maybe twice in a week or so. Today when I went to pick him up for Physical Therapy he was in some pretty bad pain and wanted the TENS unit put on I picked Z up from school around 2:45 and his PT starts at 3:30. Once we got to PT his therapist noticed that he was looking tired, showed concern and then asked him if his eyes were hurting him and he said that his left eye was bothering him. She went to test his neurological function by having him track her finger, she noticed that one eye wasn’t tracking properly and went to contact Dr. Jordan to see if it was something we should be concerned about. While we were waiting Z started complaining of his stomach and his head hurting. His therapist then checked for fever, there was none. She was able to get a hold of the answering service for Dr. Jordan, they said they would call back at my number, as we were leaving Z experienced a sharp pain down the front of his left leg (from his knee to his foot) we went back to see what his therapist thought and she felt that with this disorder being one that is very rare and that Z is the only child known with it that it would be wise to go on to the emergency room for observation, just to be sure everything is okay. So away we went to Vanderbilt Children’s hospital while in route Zachary's muscles started to tighten up in his arms and legs 

he was able to speak and let us know that he was in some pretty intense pain. This lasted about 10 minutes including the time it took for his hands and feet to relax and go back to normal, we arrived at about 7:00 checked in and were taken back to a room around 8:00. Zachary seemed to be relaxing and only complained about his eye, head and stomach. Then a pediatrician came in and I explained to her all that had occurred through the day, then she did some basic exams and went to consult with the attending. She came back and informed us that they wanted a neurologist to come in and check Z, then another doctor came in and did another neurological exam, she says Z seems to be tracking just fine. We continued to wait for the neurologist. At about 9:30 one of Dr. Jordan’s colleagues came in to check on Z he also checked Z’s neurological functions and said that everything looked fine and said that if there were cause he would push for the angiogram tonight but Z looks good. He also said that if Z was having trouble tracking he would still be having some trouble with it. Something like that doesn’t just go away it usually takes weeks or even months to return to normal. I drove our son to Vanderbilt because I was scared, my biggest fears are that his sight or hearing will be jeopardized because of this disorder. I will do everything in my power to be sure Z gets the proper care that he needs. I’m aware that this disorder is very rare and that Zachary is the literature being written, and there's no comfort in this at all.

Understanding the Disorder

12:15 Robert, Zachary and myself went down to Vanderbilt Children’s Hospital today by request of Dr. Jordan to meet and talk with Dr. Singer who is a neurosurgeon who specializes in blood vessels to the head and spine. We met with Dr. Jordan first and she examined Zachary and talk to us a little bit about Z’s condition. From what I understand she said that the brain and the spinal cord are surrounded by the Dura Mater, which is the tough fibrous membrane that envelops the brain and spinal cord. Within this space there are traces of 

old and new blood. The goal is to find out where the new blood is coming from and try to stop whatever it is that is causing the leak. Once that is done the body should be able to reabsorb the old and the new blood and hopefully the blood surrounding the nerves which seems to be causing the nerves to “stick together”. The blood surrounding the nerves is what’s affecting Z’s mobility to his back and his legs. So? we didn’t really get any new news, just a little better understanding of what’s going on within Z’s little body. While we were at Vanderbilt Robert and I decided we would get copies of all of Z’s medical records so that we could add it to his medical journal. My hope is to be able to help someone else, maybe another child who has this disorder, maybe an adult who has it, or even a doctor. We didn’t get to meet with Dr. Singer at all today, unfortunately there was a scheduling muff up at his office, they’re rescheduling the appointment 2 weeks from today. Seriously? Can I just hang my bucket of nerves and concerns out to dry. I’m tired of carrying them.

Z attends Physical Therapy three times a week, as far as mobility he has had very little, to no change. Measurements are about the same, however things are stable and not getting worse.

Physical Therapy

Courtney and Zachary stretching it out on the ball

Courtney helping Zachary with planks.

Patrick and Zachary sitting against the wall with silly faces

First Day of School

First Day of School. 

Zachary was in a lot of pain, but didn't want to miss his first day and was insistent on going. I called the school to check on him and he was feeling much better after the Aleve and Benadryl kicked in.

Notes from Phone Call with Dr. Jordan

Steroids/prednisone for the pain and to help reduce inflammation take with food it will make him hungry

This sort of problem is well reported ten or fifteen years after the accident or right after an accident. 

Is there an ongoing problem? Inflammation from blood. We would like to check if there is a tear or leak.

We want to treat his symptoms and discomfort

Look at blood vessels in his head

Any issues or leakages

Spinal tap

There is Inflammation in spinal fluid

Done all at once (the scan of his head and spinal tap)

If we don’t find answers and he still isn’t well we will do more tests but I feel these tests should be enough.

MRI/contrast and Spinal Tap
No aneurisms detected, there was some old bleeding in the head and it was located between the lining of the brain and the skull.  His Spinal tap showed normal white blood cell counts but the red blood cells were higher than they should be, which means there is bleeding occurring somewhere. We believe the source of the bleeding to be very small and believe it is coming from a small blood vessel but aren’t sure where.

Dr. Jordan would like for Robert, Zachary and myself to come in and meet Dr. Singer who is a neurosurgeon who specializes in vessels going to the head and spine he will talk to us in more detail about a more invasive test that might be able to detect where this bleeding is occurring. This test is called a spinal angiogram and it will give us a more detailed look at Z's blood vessels

Dr. Jordan wants to give Zachary some time ... a few weeks to see if there are any changes in his mobility or pain and then have us all come in. The information that was gathered is good news Z has no aneurisms and there is no infection, the bleeding that was detected is very small and not of emergent concern. 

Questions for Dr. Lori Jordan

-Superficial Siderosis is a disorder, has “Z” contracted this disorder/disease because of the trauma he experienced? No,not contracted, but has this disorder from a bleed that he experienced in the accident. 

-Does everyone who has this disorder start with a traumatic accident? No, but for other reasons for bleeding in the brain. She does know of one teen who has this disorder he lives in South Africa and Dr. Levy works with him.

-Did the diagnosis change at all upon discovering the severity of the accident? No,

very sure of SS and arachnoid ?bleeding?  - she’s looking for any other infections or causes of the pain and wants to be meticulous and doesn’t want to miss any possible infection or aneurysm 

-Will therapy help Z to regain his mobility? Yes, depends on helping the inflammation in his back, if we can decrease his inflammation then mobility should return.

-This “dry blood”, has it attached itself to Z’s spinal cord? Yes, and will stay there , goal is to decrease the inflammation. Something is making this active and we want to be sure of what that is

-Is there someway to break this pocket of fluid up? No, only decreasing it

-Is surgery out of the question because of how he has developed around this clot?

-I’m confused by this diagnosis I found that it is a disorder where hemosiderin (free iron) is deposited in parts of the central nervous system (brain and spinal cord tissue). It is often caused by repeated periods of bleeding in the brain (subarachnoid space). Did Z have repeated periods of bleeding in the brain? Yes, at some point he has or he could have a leak we want to do a scan of his head we only scanned from the base of his neck to the base of his spine. Or did he bleed from somewhere else? As far as we can tell he bled from his head. How does this get missed?

-The symptoms of SS: progressive ataxia (lack of muscle coordination), Spasticity, hearing loss, dementia, depression, impaired vision, pyramidal signs, fine motor control impairment, facial palsy, numbness in legs, abnormal sensation in legs. These things do not match up with Z’s symptoms. Why? Superficial siderosis (SS) of the CNS is caused by repeated slow hemorrhage into the subarachnoid space with resultant hemosiderin deposition in the subpial layers of the brain and spinal cord. Despite extensive investigations, the cause of bleeding is frequently undetermined. He has pain in the backs of his legs with occasional spasms, stiffness in his back and stiffness in his legs, and he is losing mobility in these areas. These are caused from the inflammation that is occurring at the base of his spine.

-Is there a support group? No, I don’t think there is one I will look into that. This only occurs mostly in adults.

-Are there other children with this and where are they? No, only the teen in Africa that we are aware of.