by Anniel 7/11/15
So here I am, asking what I think are pertinent questions and getting ignored by everyone as much as possible. I could not put things together for a long time, the only thing I knew for certain is that CSF is tasked with both cleaning and carrying nutrients to the Central Nervous System.
I thought long and hard and prayed for understanding about the matter. No answers came until two young brothers came to Chicago for help. They were obviously Chiari and/or Pseudotumor patients. I thought they were about 8 and 10 years old. They were actually 12 and 14. They were wearing hats with brims and very dark wrap-around sunglasses. They did not mix with anyone and avoided all light and noise, which they could not bear. They generally behaved like typical “Chiari Kids.”
I met the mother and she told me their story. She had Lyme Disease, but did not know she did until after the boys were born. They were both afflicted with the disease in utero. Among other things their growth was stunted and they developed Pseudotumor. They are still the only people I have met who know WHY they have the condition. They had come to Chicago for the older boy to do a 72 hour External Lumbar Drain Test and, if indicated, shunt placement. The younger boy was to be tested at a later date.
I didn’t see the boys again until a couple of days had passed, then I ran into them at the hospital in a bright and sunny hallway. The younger boy was still muffled up, but the older boy was completely uncovered with no sunglasses, hat or anything, and he was pushing a pole with a bag of collected CSF. He rushed up to me with a huge grin, and said, “I feel great, and I get my shunt tomorrow. I even grew a whole 1/2 inch. It’s so wonderful!” His brother said he could hardly wait for his turn to get a shunt. I talked to them for awhile and walked away hoping this would really work for them. I think not, because as far as I know they never returned to the hospital.
Cate has had External Drainage several times due to infections and other shunt and tubing failures. I knew she felt better on External Drainage but had never really questioned her about what went on. I decided to ask her now. She said that when she was on drainage her headaches went away, she had no “brain fog”, light and noise stopped bothering her and she had more energy. She was “normal.” I had been with her and I knew she was better on External Drainage but had failed to see how profoundly it affected her.
The evening after I had seen the brothers I sat for awhile in the hospital chapel thinking and praying about the matter. I thought about it through dinner and after I went to bed. Finally I fell asleep, and when I awoke I KNEW the answers to some of my questions.
THE ONLY DIFFERENCE BETWEEN EXTERNAL DRAINAGE AND A SHUNT IS JUST THAT, IT IS EXTERNAL. WITH A SHUNT THE DRAINAGE STAYS INSIDE THE BODY TO BE PROCESSED. IF THE ARACHNOID VILLI, IN CHARGE OF DRAINAGE, ARE DAMAGED OR BLOCKED, THE FLUID AND PATHOGENS CANNOT BE REMOVED EFFICIENTLY, IF AT ALL.
I theorized that if the patients were placed on External Drainage until their CSF ran clear, perhaps they could overcome Pseudotumor. I tried to discuss this with three different doctors, all of whom rolled their eyes and barely listened, although one did say that “in theory it might work but the danger of infection is too high.” Even my daughter, who says she believes me, gets embarrassed when I try to talk about what I learned. One of my sons says I just don’t have the right letters after my name.
After long consideration of the matter, I came to the following conclusions:
My first question was why the CSF pulls inward in Pseudotumor. What I state here is MY THINKING ONLY.
If a healthy person becomes ill, a pathogen of some sort, say a flu bug, crosses the Blood Brain Barrier (BBB) into the Central Nervous System and the body accelerates production of cerebrospinal fluid in order to cleanse and remove pathogens from the CNS. The dirty CSF is then processed through the Arachnoid Villa (which is now known to be only a partially correct picture) and eventually leaves the body through the kidneys. When the pressure first rises the sick person gets a pressure headache, shivers from fever and aches from the excess fluid pressing on all the nerves in the spinal cord. When the CNS has cleaned the pathogens out, the body returns to normal fluid production as the patient recovers.
IF THE BODY IS OVERWHELMED WITH PATHOGENS IN THE CNS, OR HAS OTHER DAMAGE, THE BUILD UP OF CEREBROSPINAL FLUID MAY NOT BE ABLE TO CLEANSE THE SYSTEM EFFECTIVELY, OR DISPOSE OF WHAT IT CLEANS. Pseudotumor may result until the cleansing takes place. If the pathogens cannot be fully removed, the condition may become chronic. Chiari further exacerbates the problems.
As I mentioned before, the day finally came when Cate began to fall into what the doctors later called “mini comas.” One morning I found her sprawled across her bed with one knee up as though she was just crawling onto the bed and with the other foot still on the floor. Her bloody face was on the window sill. She had passed out, banged her mouth and put her teeth through her lip, and she also had a bloody nose. She was freezing to the touch. At this time she concurrently developed a condition called Torticullis where the side of the head pulls completely down onto the shoulder when the person is asleep or passed out. It looks awful and many people at Ronald McDonald House would go over and try to pull her head up. She would scream, tilt back over and pass out again. But she did awaken.
I didn’t know why then, but a married couple who were night managers at the Ronald McDonald House, began eating dinner with us every night. They told me later that they knew Cate was dying and they wanted to spend as much time with her as possible.
We talked to the doctors about the situation and they kind of ignored us because Cate pretty much stayed awake during appointments. She was so brave as she just kept putting one foot in front of the other and did what needed doing. Finally the morning came when she got up, showered, dressed, and staggered downstairs to the living room. As soon as she sat down her head fell over onto her shoulder and she went into a coma from which we could not rouse her.
Her dad, who happened to be in town, grabbed a wheelchair and we ran her two blocks to the ER. On the way I kept holding her arm up when it began to drag on the sidewalk. When we got to Triage I said, “She’s in a coma.” The staff grabbed her up, put her on a gurney in a room and started working on her. We had to wait on the other side of the curtain but we could hear how desperate everyone in with her was. After about half an hour someone came to get us and we were told to “hold her hand while she dies.” Her Glasgow score was approaching the death level of three and a doctor was standing by to intubate her in a last attempt to save her life.
As soon as I took her hands I knew she was deciding whether to go or stay and that it was entirely up to her. Bear took hold of her feet and we talked to her. The nurse practitioner who knew her best started to cry and her main doctor, who was in the OR, telephoned to tell the nurse to give her a blast of Narcan to see if it would make her wake. It did, but I thought for a minute that she had gone into convulsions.
Cate later told us she was up on the ceiling watching while all this was going on, and that it was my tears and those of her nurse friend that made her decide to stay.
For the next few weeks she was in and out of comas and everyone was wringing their hands over what to do. Finally I said to her main doctor, “This is the family thyroid disease.” This time he LISTENED to me. He called in a pediatric endocrinologist who was a total fool and said Cate was fine, even though her blood tests were all over the map. Finally an adult endocrinologist came in who said he thought she had a rather newly discovered (in 1965) condition called Hashimoto’s Encephalopathy. At the time Cate was going through this the medical literature said only about 45 cases of the disease had ever been confirmed.
The condition is, in part, diagnosed by checking the antithyroid antibodies in the patient’s blood. We hadn’t understood how these tests work. There are several kinds of antibodies, but the important one in Hashimoto’s Encephalopathy appears to be the Thyroid Peroxidase Antibody. Our daughter’s level had been 5,120 for several years. Every time it came back I thought, “At least it isn’t getting any worse.”
Well, this is how the titer on the antibodies works: the blood sample is diluted by 40 times and tested, if there are no antibodies in the blood the sample is called normal. If there are antibodies present they double the dilution to 80 and check it again. They keep on doubling the dilution (for a total of 9 times) until the sample is clean or reaches 5,120, where the test tops out and where Cate’s levels were. No one has any idea how high her titer actually was in all that time.
After several months of steroid treatments to control the Encephalitis Cate finally decided to have her thyroid removed. The surgeon nicked her recurrent laryngeal nerve and left her right vocal cord essentially paralyzed, but he never told us. We thought her hoarseness would go away and didn’t know for many months what had happened. Nor did the surgeon tell us how awful the condition of her thyroid was. Fortunately Cate’s main doctor let us know there had been some problems. We finally requested the OR notes on the surgery and found that her thyroid had shrunken, wrapped around and adhered to the recurrent laryngeal nerves on both sides, something no other thyroid surgeon we have talked to has seen before. There were only about 6.8 grams of the gland which could be removed. A small amount of tissue was left on the undamaged left nerve. A healthy thyroid weighs about 25 to 35 grams, so if only 6.8 grams could be removed, it was seriously shrunken.
The levels of her antibodies did not drop at all for over a year from the time her thyroid was removed. I can only wonder what her levels actually were. Would the antibodies still have been present if the dilution had been doubled from 5,120 to 10,240, or even again to 20,480? No one knows. For awhile the doctors were discussing the possibility of an extra thyroid under her armpit or someplace.
At the time of her diagnoses I asked if the thyroid antibodies had passed into the cerebrospinal fluid and were causing the Pseudotumor. I was told they could not pass the Blood Brain Barrier, and there was no test for them in the CSF anyway. A few years went by and one of the doctors told me they had tried a new test developed for testing for antibodies in the CSF, and hers was clear. Almost eight years later they tested her CSF again and we are now told the antibodies do pass the BBB and there are very high levels in Cate’s CSF. Were we lied to before? The docs think maybe the high levels explain her many autoimmune illnesses. She may have what they are calling Recurrent Hashimoto’s Encephalopathy.
One of the endocrinologists here in Anchorage told us that he and the other local endocrinologists all agree that Hashimoto’s Encephalopathy is not a “real” disease and thyroid antibodies “don’t matter anyway, so shut up about them.” (If they “don’t matter”, why bother to test them?) and yes, he did say exactly that, “Shut up.” He is no longer our doctor and I probably would snarl or spit, or maybe both, if I saw him.
I now had a working hypotheses about WHY the CSF pulls inward. My thinking about the “goop” was that the pathogens within the brain floated around in the CSF until they clumped together as the body attempted to remove them from the CNS tissues. Then two things happened to change my thinking
First, I saw a DVD about Lyme Disease called UNDER OUR SKIN. As I watched people who suffered from Lyme Disease I was stunned to see how closely they resembled Cate when she would have a shunt infection, a broken shunt, or go into her mini comas from Hashimoto’s Encephalopathy. At the end of the DVD a pathologist studying Lyme Disease showed one of the spirochetes that cause Lyme and said he found evidence of “biofilm” surrounding the spirochetes and that the spirochetes were protecting themselves by hiding within it. I had no idea what he was talking about. By the way, the discovery of Biofilms is an exciting story all its own.
What is Biofilm you may ask? Look all around yourself and you will see some.
The second thing was when Bear came home a month or so after we had seen UNDER OUR SKIN with a copy of DISCOVER Magazine and began telling me about a fairly new germ theory. The magazine told about the discovery of “biofilms” and how they work. It seems that germs don’t just float around in the blood and tissues as previously thought. They make a film of slime aggregate to surround and protect themselves as they stay within it, and other groups of germs and pathogens form symbiotic colonies within the same covering. In effect they become little communities living together. The biofilms can cause chronic infections of all kinds as they hide the germs within the host body. For a long time orthopedic docs and surgeons were the only ones who worried about the films because they cause terrible infections when they attach to implants. Even dentists still often don’t know that the scum and crud on teeth when you fail to brush are biofilms.
For several years Biofilms were seen only as “bad.” I thought about the films and concluded that the slime collected on the shunts in Pseudotumor was biofilm gathering together and clogging the shunts.
Online, as I was researching the matter, I found all kinds of new information about biofilms in nature and everything surrounding us in our homes. Biofilms are everywhere and very necessary in breaking down dead matter and creating compost, for instance. The rotting vegetables in your refrigerator are covered with biofilm. Many mosses are biofilm, as is the detritus in bogs and swamps. In your home, biofilms attach to water lines and taps. They are the scum on tubs and sinks. There are many forms of biofilm in the body, some beneficial, some not. I have wondered if “snot” is really biofilm that surrounds and entraps dirt and pathogens as we constantly breathe them in and out.
Biofilm is absolutely essential to all life. Decaying leaves on the lawn become “slimed” and sink into the soil as fertilizer. The films can also be threatening when “bad” germs congregate in colonies to protect themselves. The colonies are highly resistant to antibiotics once they are established within a host body.
I asked myself if biofilm colonies are the cause of PTC and are formed for protection of pathogens in the CSF. Are they the “goop” that clogs the shunts? If they are, do they also wind up clogging the Arachnoid Villi so the CSF is unable to properly drain from the Central Nervous System? If that is so, then CSF External Drainage becomes more critical in treatment of the condition.
We know that Cate makes almost half again as much CSF as she would ordinarily need, so she already has high intracranial pressure from that, but her Arachnoid Villi undoubtedly do not drain as they should.
I think now that the doctors I spoke with did not understand what I was proposing with a period of External Drainage for Cate. As a practical matter it would be almost impossible to have PERMANENT external drainage. What I had envisioned was leaving the drainage in place and checking the fluid once or twice a day for as long as it took until the CSF ran clear. If the buildup of slime at the Arachnoid Villi can be reversed and suctioned out along with the CSF, the fluid might even show an increase in slime until that source is cleared up. I have no idea how long such external drainage might take, but I was hoping for at least a 7 to 10 day trial. A lot could be learned in that time.
None of the doctors would consider it. I wondered then what else they could do to see if I was correct. I watched one boy suffer from very serious Encephalitis, just screaming in agony. Meningitis does the same thing. What would happen if people fighting these diseases were treated with External Drainage? At least a large portion of the infection in their CSF might be removed externally, leaving their own resources to fight what infection remains in other parts of the body.
Think about the people who contracted Fungal Meningitis in 2013. Would External Drainage have effectively removed enough of the tainted CSF to give them more of a fighting chance? Near the end of the outbreak, one of the doctors involved in the case said they had done testing that indicated the presence of fungus in the CSF, and they were very surprised by the finding. How could they be surprised? The infection after all was in the meninges surrounding the spinal cord, that’s where the CSF is.
I have thought about this for a very long time now. While I don’t know everything about the condition our daughter has, common sense tells me that what I do know is right.
I wish they would try prolonged external CSF drainage, and IVIG to clear the antithyroid antibodies still in her blood. Would it be a permanent cure? Maybe not, but what if she, and patients like her, could be treated a few times a year and be more healthy in between? Or maybe a few such treatments WOULD cause a cure.
Cate has so many things going on that it’s hard to keep up. I remember one doctor who told us, “None of her conditions are related, she was just unlucky enough to get them on the same day.” Now, the doctors are finally deciding that she might just have one underlying condition, and it’s probably from the thyroid.
After all these years this is so dismaying to all of us, including the good doctors who really care.
MORE TO FOLLOW in Episode 5. Which may be the end. • (964 views)