by Anniel 7/8/15
The night before her scheduled Lumbar Puncture (LP), Cate went to a medical blog site and found a headache forum. She exchanged posts with a woman there, then came upstairs and asked if I had ever heard of Pseudotumor Cerebri. Of course not, who has? She told me that the people who have it somehow produce too much cerebrospinal fluid (CSF) which causes high intracranial (inside the brain) and central nervous system pressure. People who have it suffer terrible headaches, and can go both blind and deaf. They are also prone to develop another painful condition called Syringomyelia. Then she said, “I’m sure glad I don’t have that.” I agreed and put it in the back of my mind.
Many of the symptoms of our daughter’s illness had been going on for most of her life but she thought she was normal. She had not indicated any big problems and seemed fine right up until the headache and thyroid storm.
We had just then begun the long journey we have now been on for a very long time. I did try to talk to the doctors about Cate’s thyroid storm, but no one wanted to listen and I was accused of Munchhausen’s Syndrome By Proxy. I knew it wasn’t about me, it was about what I could see based on what I knew. My father, a couple of aunts, my brother and I had all been hyperthyroid. No one wanted to hear what I thought. I wonder now if they had removed Cate’s thyroid then if she could have been, not cured exactly, but saved from some of what would follow. But for now she would have an LP.
Like other people of my generation I had been told how painful and dangerous a “spinal tap”, or LP was, so I waited anxiously while Cate had the “benign” lumbar puncture the neurologist had recommended. She had seemed unconcerned when they took her away, but I was almost in tears.
When they finally finished the LP and took me back to her I was astonished to see her smiling and her eyes relaxed in her face. “Has anyone talked to you?”, she asked. I shook my head no. “Well, the radiologist did the LP and cerebrospinal fluid came gushing out all over the room. He had to send someone for an extension to the measuring device. He put my pressure down as 40, but it could have been higher. I have Pseudotumor Cerebri. He also said he hopes his ‘brothers’ treat me better than he thinks they will.” The radiologist also drained her pressure down below 20.
We learned that normal CSF pressure should be somewhere around 10cc’s of water, certainly not over 20, so at 40 she was very high. I was so grateful we had at least some idea of what was wrong. I still thought, too, that the doctors could “fix” the problem. The neurologist had made a follow-up appointment for a week away. We went home and for the next two days our girl was like her old self, happy, going out to walk and play with the dog and I knew things would be fine. Now that her pressure was down, her father and brother could finally see how different her eyes looked.
The third morning after the LP, she got up, slipped on her running shoes and leaned over to tie them. We all heard her scream. When we got to the entryway she was on the floor crying, vomiting nonstop and writhing in pain. I called the neurologist’s office right away to see where we should take her. His nurse seemed unconcerned and told me Cate had an appointment in four days anyway so not to worry about it. The vomiting and pain continued unabated. Every time I called the neurologists’s office I was put off with no further explanation.
We were frantic with worry and finally called the Internist, who agreed to see her. We had to take Cate into his office in a wheelchair because she could no longer walk. The doctor could find no reason for her constant vomiting and pain. Finally an ophthalmologist came in to check her eyes and said she had a low pressure headache and needed a ” blood patch.”
She continued vomiting and shaking as we wheeled her over to the hospital. For some reason I was allowed to stay in the OR while the doctor did the blood patch. To do that a small tube is put in the spine next to the LP site, blood is drawn from a vein, allowed to coagulate slightly and then put into the spine where it seals the hole from the LP that failed to close and has been leaking fluid. The patch works like magic. Immediately the pain and vomiting stopped. We learned that low pressure headaches are more painful and, in ways, more dangerous than high pressure.
When we kept our follow up appointment with the “best” neurologist in town, he informed us that our daughter did NOT have Pseudotumor, that the Radiologist was not capable of making that diagnosis and had no business doing so. He also said that the radiologist had done the test incorrectly. I asked him when they would repeat the test. He looked at me as though I were a beast, and said, “This is a far from benign test. What kind of a mother are you who would subject her child to it?” Last week it was a benign procedure and suddenly this week it isn’t. How does one keep up with this?
Over the next several months we went to doctors all over Anchorage and Seattle, to no avail. PTC is rare, only about 1 in 100,000 people have it. The condition and the related illnesses that often go with it are very poorly understood. We finally contacted the University of California at San Francisco and the neurosurgeon there agreed to see Cate. “Yes,” they said at her first appointment on Parnassus Heights, “she does have PTC.”
We spent many months and a lot of money going to UCSF. The neurosurgeon there installed her first ventricular brain shunt and tried a few medicines before it finally occurred to Cate that in reality he knew very little about the condition.
She did a lot of studying on her own, even tapping into the medical library at the University of Alaska, and began to suspect she also had an Arnold Chiari I Malformation of the brain. We’ll catch up with that one later. Unfortunately the doctors at UCSF would not or could not answer many questions about her condition. Cate went on the net and found the only doctor who seemed to be doing any research into either of those conditions. She called to see if he would look at her scans and charts and, after he had done so, made an appointment to see him. Then she informed us we would be going to Chicago. Her father and I were surprised at what she had done all on her own.
It has been a long and emotional medical journey. I think at one point we knew, or had heard of, every person in the United States who suffers from PTC and/or Chiari, including a small subset of people who, like our daughter, also have severe autoimmune disorders.
We have now been dealing with this condition for almost sixteen years. It has not been easy, but at the same time I think we are all better people because of what we have been through. We would not choose this path, but we have learned to love and respect so many people we would never have known had we not been challenged the way we were. Even our daughter said she would not change what has happened. Our experiences in Chicago have been interesting, putting it mildly.
A person usually approaches the medical profession almost like a new-born babe. You have no idea how uncaring some doctors and nurses are, nor how inefficient and careless some departments can be.
I once, and only once, wrote a letter of complaint to a hospital board of directors about a doctor who had treated our daughter and us in an abominable manner. The Board’s reply was that they were “. . .shocked, shocked and appalled at the way we had been treated . . .” HOWEVER, “After reviewing the matter we have concluded that the doctor acted in a medically appropriate manner.” The only way to read their answer is that it is entirely medically appropriate for a doctor to act in a shocking and appalling manner toward his patients and their families. A friend later showed me three letters she had received saying exactly the same thing in answer to her complaints.
We have also met some wonderful, caring doctors and nurses who have moved heaven and earth in helping the ill. And we have made friends from all over the world. We were so insulated from other people before this all happened it still shocks me.
The next thing we need to talk about is how diagnoses of a disease is actually done. It’s not the way you might think.
MORE TO FOLLOW in Episode 3. • (912 views)