Whitney's PageMy daughter Whitney was diagnosed with Chronic Inflammatory Demyelinating Polyneuropathy in February 2002. She is only one in about two million people in the United States with this condition. This is a neurological problem so rare that most neurologists never see a patient with it, ever.
Click here for the latest health update. It starts slowly, almost imperceptibly. Imagine yourself as a fit young athlete, an experienced competitive swimmer, a dancer and a musician. One day your hands and toes start to tingle after a shower or a bath. One Saturday morning you find you can barely complete your first lap at a swim meet even though you've been doing five miles per night at practice for months. You subsequently discover you can't stand on your toes. You can't lift your legs high enough to get on the school bus. Your clarinet keys are so difficult to press down you can no longer play it. You fall on the stairs and land on your head. You can't even squeeze your tube of toothpaste. That's what happened to Whitney, in just a couple of days. This is her story, in diary form as events unfolded.
Whitney turned 15 on December 31, 2001. Around the middle of November of 2001 she got very tired. Extra sleep didn't seem to help. I thought it was just from too many activities, or maybe a virus. She started walking slowly and didn't seem to lift her feet right. She fell a couple times at school. The family doctor ran some blood tests, suspecting anemia, mononucleosis, thyroid, the standard stuff. Went back the following week to review the results. Everything was normal but she still felt terrible. A quick exam revealed she had no knee reflexes. She couldn't stand on her toes or heels. The doctor ordered an MRI and planned on forwarding the results to a neurologist. Although the doctor was obviously perplexed, there was no indication of an emergency. That night I sent an e-mail to a retired neurologist I had met at an investing club in Cincinnati. He wrote back immediately but I didn't check my mail until morning. He said there was no time to wait for an MRI. She needed a complete neurological exam immediately, because he feared this might be leading to a partial or even total paralysis. He also posted my description of Whitney's symptoms on a neurology bulletin board. Two other doctors, one in Houston and another in Kuwait, agreed with him. The likely diagnosis was Guillain-Barre Syndrome. Using my friend's pull we got in to one of the top neurologists in Dayton within one hour. Good thing, too because if the three doctors were right Whitney might have needed a respirator to stay alive. Fortunately it did not come to that. December 15: First neurologist visit. We brought the letters from the other neurologists with us. Our doctor agreed with the internet diagnosis, and the tentative diagnosis is indeed Guillain-Barre Syndrome. This is quite rare (only about one in a hundred thousand people get it). If it is GBS, she will almost certainly recover, possibly within months, maybe longer. It could turn out to be something even more unusual called CIDP, only time will tell. If it's CIDP she will slowly get worse. If that happens they will either treat her with high dose immunoglobulin therapy or plasmapheresis. Recovery from CIDP is also likely, however it can reoccur again later in life. Initially the doctor thought it might be a good idea for her to spend the night in the hospital but after consulting with a few other doctors he let her come home. Her mother started sleeping with her so she could make sure her breathing was all right. We left his office at 7PM Friday night after an exhaustive four hour examination. He will do a spinal tap on Monday. Tests Whitney has had: Nerve conduction velocity test (NCV) Electromyography test (EMG) Spinal Tap December 17: Her doctor performed the spinal tap in his office. Lots of people have trouble with these, mostly severe headaches. Whitney tolerated it quite well. It will be a few days before we get the results. If we have a high protein count without an elevated cell count it will support the diagnosis. December 19: I met with Whitney's teachers today. She is not strong enough to go to school. She has had to give up dancing, competitive swimming, cheerleading and all of her other activities. She had trouble squeezing a half-full toothpaste tube last night so I gave her a full one which she could handle better. I provided a handout for the teachers about GBS so they might get a better understanding of what she is going through. I brought a clothespin with me just to illustrate the fact that she can't squeeze it closed without using both hands. They are a great bunch of people and promised to help her any way they can. The school has an elevator that would get her to classes, but she is mortified about having anybody seeing her in a wheelchair. I don't think she has the energy to sit in it all day anyway. Most days she doesn't wake up until about 11 AM. School starts around here at seven. I guess we will be looking for a tutor. We went to the Christmas dance recital tonight. Whitney watched, but of course couldn't be in the program. She was totally exhausted from just sitting up for so long. Boy, I hope this starts getting better soon. Whitney can't go up or down the stairs without help. She is being a good sport, cheered up by one of the neurologists who explained that her brain is working just fine, it's only the muscles that are having trouble. A couple of nights ago she thought the computer keys were getting "sticky." I hope that's not an indication of more weakness. No complaints for the last two days. I am watching. December 23: Whitney and I went shopping for a Christmas gift using a wheelchair. Yuck. You should see the people stare. December 26: We went to the neurologist to review the final results from the spinal tap. As suspected she has an elevated protein count and a normal cell count. Whitney seems to be stable, neither getting worse or better. I think she is doing better because she is sleeping less, acting more alert and cheerful and getting around more under her own power. However her knee reflexes are still non-existent. The doctor has decided that we will wait another month and see how she does. Her body might be able to heal itself. One of the test results suggests that she may respond well to plasmapheresis if she needs it. That's good news because there only three treatment options. He also said that while exercise will help keep her muscles in tone, it will not help her nerves heal any more quickly. We've been advised to expect this to continue for six to nine months, and even that is a pure guess. He signed a letter encouraging her school to provide a tutor and set us up for a handicapped parking tag good for a year. January 12: Whitney's high school has graciously provided a tutor. We are very grateful for everyone's help. She continues to sleep until around 11 o'clock most days, and needs help coming down the stairs. She fell down on her head once, and that was enough that we have made a new rule: No stairs without help. On good days her energy level slowly builds and once in a while she will start homework around eight PM and continue on for several hours. On bad days, she lies on the sofa, rests and watches TV. Her occasional bursts of energy are followed by periods of exceptional weariness. In the early fifties my sister had Polio. I was too young to realize its seriousness. I now have a pretty good understanding of what my parents must have gone through. It was bad. January 25: We ventured out to Wal-Mart for a shopping trip. Midway into the store Whitney was having trouble with her knees. I went back up front and got a wheelchair for her. We have one at home too, but she has been unwilling to even consider using it since the Christmas outing. January 26: We Whitney went to the YMCA to try a little workout on some exercise equipment. She did pretty well with the machines that exercise upper body strength. Two of their machines exercise lower leg muscles. In one you sit facing forward, hook your legs under a bar and lift up. On the other you lie on your stomach and lift the legs. She could do neither, even at the lowest settings. These two outings have left her extremely tired. It is amazing to see in such a previously fit young athlete. Next week her neurologist will perform another to test her progress in a more objective way. We think there will be a small improvement from the December tests. He has already told us to anticipate 9 to 12 months before this is over. From what I gather he really can't be sure. There is not a lot of research to guide him. January 29: I did a little research on the prospects for a full recovery. I have copied them here because the author chose such a noisy background that you would have trouble reading it. It is not cheerful reading. February 12: The neurologist performed another nerve conduction velocity test today. Her body is not healing itself; it is actually getting worse. (Specifically: Motor nerves normally send signals through the lower legs at above 40 Meters/second. In December she was 20, today 15.) Her arms have worsened too, although they are still better than the legs. The doctor has subsequently revised his diagnosis to Chronic Inflammatory Demyelinating Polyradiculoneuropathy. About two people in a million have it. CIDP is officially listed as a rare disease with the National Association of Rare Disorders. People with Guillain-Barre Syndrome usually recover and don't get it again. CIDP patients might not be so lucky. We are going to begin IVIG therapy as soon as arrangements can be worked out. She will have the first treatment in a hospital because there can be complications from a possible allergic reaction. Later ones will be done at home by a company that knows how to administer these things. Whitney does not like getting stabbed with needles and shocked by the stun gun they use in these tests. She secretly arranged for the EMG technician to test me with the stun gun on my hands. After a few zaps the lady said, "Are your fingers numb?" I said, "Sometimes, can you see that?" Of course she could. Then she tested herself for comparison. What a difference! I must be at the point where I'm starting to fall apart. I don't really care if my fingers get numb. Whitney is the one who needs a break. Whitney needs the immune globulin treatments soon. We are working with the insurance company to make sure this is covered because it is very expensive. So far they are not willing to pay. Immune globulin is considered to be a blood product and not a drug. If she had needed a drug they would cover it. It is a white powder. It does not look like a blood product to me, and it says Rx on the box. Rest assured Whitney will get what she needs regardless. It is very sad that the insurance company wants to draw such a fine line between what is covered and what is not, especially when IVIG is considered the "standard of care" for CIDP by the American Medical Association. The other alternatives cost even more. Shame on them. I can recall some slogans. "Like a good neighbor" is one. Another is "Solid partners, flexible solutions." Right, show me. Whitney will receive the first of five daily immune globulin infusions tomorrow, only at home instead of at the hospital because of some communication problem between the doctor and the insurance people. We don't like this one bit. Complications can range from a rash to difficulty breathing to worse. Whitney can't wait any longer. She could be looking at permanent and irreversible nerve damage. Wish her luck. A prayer wouldn't hurt either. February 21: The first infusion was difficult for Whitney and traumatic for us. About fifteen minutes into the infusion Whitney's heart rate became irregular and her blood pressure rose quite a bit. We had a shot of epinephrine standing buy and the medical squad was on call to take her to the hospital if need be. The nurse slowed the rate way down, and Whitney tolerated the rest quite well. A one and a half hour procedure took four, but at least she did not have a severe reaction and the squad was able to stay at the firehouse. Whitney was OK, Denise and I were exhausted by the stress. A few month later the nurses fessed up. They had been freaked out too. What the heck was I doing subjecting my kid to a procedure at home which the doctor said should be done in a hospital? I'll tell you. We had battled with the insurance company for over a week. The possibility of irreversible damage was staring us in the face. We felt like reckless gunslingers. At least the remaining infusions should be relatively easy because the risk of an allergic reaction has passed. Whitney has had two ivig treatments with another scheduled for this evening. This morning for the first time since November she can:
We never expected such a rapid change! According to my internet neurologist friend, results like this are often reported after ivig therapy. Nerves are beginning to talk to muscles again. This indicates the nerves are probably not irreparably damaged. The neurologist is very pleased, as are we. Her drugs cost $66,000 a pound (including postage). Fortunately Whitney did not need that much; only about half as much as a McDonald's hamburger. Is it worth it? You bet. Two more treatments to go. Finally, really good news! A day after the immune globulin infusions ended Whitney can:
We actually took a short stroll through a mall. We are stunned at the speed of her recovery. The doctors advise taking it easy so as not to injure the muscles because they have been resting for a long, long time. My little girl is rapidly coming back to us! What do they put in that immune globulin anyway? I think I need some too. I hear the sound of a clarinet playing. It's Whitney. Her fingers can press the keys! Today marks exactly four months to the date when she crashed at a swim meet (the first sign that anything was wrong). She does not quite sound like Benny Goodman or Woody Herman, but she sounds pretty darned good to me! Whitney's neurologist has pronounced her 70% recovered. Her reflexes are still only partial in her arms and totally lacking in her legs. That seems surprising since she is getting around much better. But tests are tests. We have to do exercises for the next month to measure progress or the lack thereof. She could continue to make progress on her own or she could stabilize or even decline. Unless she keeps getting stronger he wants to give her steroids for a month to keep the healing process alive. You never know with CIDP. Whitney has already refused these because she searched some web sites that showed her the side effects of taking these over the long term. I tend to agree. These make your head get fat, make your eyes squinty and thicken your waist. Not quite what a beautiful teenage girl might look forward to. We will be looking for alternatives. There are only two: More IVIG or plasmapheresis, discussed above. Whitney was able to get back in school before it was over for the summer. She made the honor roll every grading period, including during the time when she was sick. She was just elected to student council for her upcoming Sophomore year. This summer she will take a break from competitive swimming and just be with her friends again. I'll be teaching her how to drive. I have a van. She hates them because they are not cool. She doesn't think I have the patience to be a 'cool' driving instructor either. Who, me? She must not realize that I am the world's best driver. No sweat. Today she marched with her high school band in our Memorial Day parade, just like old times. And so at this point the updates will have to pause for a while. Maybe a month, maybe six. CIDP can be a relapsing disease; there may not be an end. But we hope so and we're praying for it to be over. Whitney seems to be relapsing. Her walk is not quite right, her grip is weak and some fingers are numb. We saw the neurologist today, one month ahead of schedule. She will probably need another round of immune globulin. He is going to wait ten days. In case she has a 'bug' that is draining her limited resources, this should give it time to go away. If not, then we are set for another round of ivig, 15 grams per day for five days. It worked really well last time, no reason to change tactics yet. That's another $13,000, my friends. That's ok. She needs it and it worked well last time. I'm sorry it did not last forever, but we can try again. And we can do it before she gets as weak as before. So we are encouraged. The insurance company is still unwilling to pay for the first cycle. Hello? This is the primary approved treatment for CIDP according to the American Medical Association. I think the problem the insurance company has is that it costs a lot of money. I say tough. Isn't that why you buy insurance, to protect your own family from being wiped out? These companies have two objectives: 1) Bring in money. 2) Keep it. At this rate if Whitney lives until she is 75 her drug bills will be over $2.5 million. Our policy won't run out until we hit $6 million. Then again it will never run out if these cheap folks won't pay the first dime. We are going to launch an all-out effort to make these people honor their obligations. So far I have not named the parties. Once we start official actions I will. And it will be soon. You mess with my kid and I mess with you. With a vengeance! July 9: Whitney needed more infusions of immune globulin. This cycle is finished. This stuff works. She was weak, now she is strong again. The speed of the transition is really amazing. I just wish it would last forever rather than just four months. Our neurologist is still not sure if we can stop this stuff dead in its tracks or not. Most likely, we cannot. (That's why they call it 'chronic'.) He is planning on a maintenance dose of ivig once per month for the rest of 2002. Then he will stop and see if we've been successful. If not he is considering chemotheropathy. That can mess up your ovaries and create damaged babies. We might be able to harvest some eggs before that starts so Whitney can have a chance to become a mother of a perfect baby. Time will tell. October 21: Whitney had one immune globulin infusion per month in July, August and September and will continue for the rest of the year. They seem to last about 25 days before she starts complaining about feeling 'weak.' It is October 21 and she is sliding backwards, having a little trouble walking, climbing stairs and gripping things with her hands. We are going to do three more, once a month for the rest of the year. If we can pull everything together we'll do the next one in two days. Darn. The neuro had hoped she might be able to shake it. Not so far. People do get dependent on this stuff. Looks like she might be one of them. We got a letter from a parent whose 15 year old boy has the same thing. He's had a much tougher time of it and can't tolerate ivig. He spent 15 days in the hospital, and has to wear a leg brace to hold his toes up so he can walk. This is nasty business. Our neurologist is consulting with Dr. Jerry Mendell, who is one of the top guys in the field. He is chairman of the neurology department at Ohio State. We're confident she's getting the best care there is. This link will take you to a web site that the neurologist use. Type in chronic inflammatory demyelinating polyneuropathy and it will show you the latest 300 or so research papers on the subject. I've read most of them. They will pretty much show we are doing the right stuff. There are a few experimental procedures, but there is little if any follow-up. It's a very limited field, thankfully with few victims.
On December 12 the neurologist decided to move up the next immune globulin infusion about a week earlier than planned. Whitney was complaining about feeling week again, and we could tell she was not walking quite right. She decided she was too weak to participate in her dance class Thursday night and the swim meet last night. Still, she wanted to go to the meet so I took her over just to watch. It is always warm and humid at the pool, and after half an hour she felt faint, dizzy. I brought her home. She had a bit to eat and slept for fourteen hours.
Tonight she has the high school Christmas dance. Two hours after the infusion ended she had another friend over to help with her hair. Then two dates showed up for a quick dinner. They are about ready to go out and party. It is amazing to see your kid all curled up shivering like a nearly-frozen animal and then three hours later be ready to party. That's what Immune Globulin can do for you when you need it. One of the boys commented how she was walking funny going upstairs. Of course he has never even contemplated the realities that Whitney has faced. I was pretty upset over his remark but tried to remind myself that the boys have a total lack of understanding, and deserve some slack. I think she is pushing pretty hard, but it is hard to say no. I did, well, sort of. There will be no after dance party; she will come straight home. Tomorrow will be a day of rest. Maybe we'll get a Christmas tree. Last year Whitney picked one out while watching from the car because she couldn't walk. This year she should be able to get out and choose one by herself. Small steps, but important ones. The kind of improvement that sends tingles through my very soul. I'm getting the feeling that this ivig will become a way of life every three weeks or so.
June 9, 2003 We've continued the Immune Globulin infusions every month for about a year and a half. Sometimes they have a very good effect, sometimes only a small one. By the end of 30 days Whitney has noticeably weakened and looks forward to the next round.
Going forward we expect Solumedrol and Immune Globulin infusions to be done monthly, probably at the same time, followed up by more Prednisone. Next week Whitney will take her first real college class at Wright State University. She was one of only twenty five high school kids accepted into this special summer program. She will be taking a ten week sociology class along with older college students, cramming it into five weeks. Some of these days she will have a catheter in her arm. How will she carry her books? I'll have to think about that. Immune globulin infusion. Now we are using a metering pump. This controls the flow more precisely than the nurses can do it. It's also programmable and it increases the flow in several steps. That makes it easier to take and minimizes the potential for a bad reaction. This one took five hours and Whitney needed four more hours to recuperate. She is at the grocery store with a friend. Doing quite well.
Whitney has been doing amazingly well for months, doing everything a normal kid wants to do. This morning started off like any other infusion day. Two nurses showed up at eight am and mixed up the immune globulin. The infusion began around nine. Connie was able to get the line in with only one stab, which Whitney appreciated. We are using a metering pump. This is very helpful because we need to start slow, then speed up the flow in steps. Going too fast can cause shock or heart failure; serious business. Although we've done lots of these without a problem, today Whitney developed a fever of 101 and started shaking from the cold she was feeling, even before the pump was set to ramp up from slow to medium. We stopped the pump and covered her with six blankets. She asked for something hot to drink and for a hot water bottle. The nurses paged the doctor. He thought she might be having a reaction to this particular batch of ivig and told them to quit and throw the rest out. A mere eighteen hundred dollars down the drain. Better that than to keep pumping in more stuff that she could not handle. The problem is, she has to get this stuff to stay alive. There are few other choices. We will see the doctor three days from now for an already-planned visit. Then we'll see what he wants to do next. God bless him, her life is in his hands. He knows that, too. He is a good guy and we trust him. Nobody ever said coping with CIDP would be easy, and it is not. Whitney has had CIDP for two years now. Today her neurologist once again conducted a series of tests to electronically measure her nerve responses. There was, unfortunately, no improvement over last year. He was obviously concerned with the results, and made a point of saying that he's been treating her 'very aggressively.' (I've studied this a great deal and that is absolutely true.) Whitney's body is still attacking itself. Her immune system is confused and thinks there is an invader that has to be eradicated. The effect is that the fatty tissue that surrounds the nerves begins to dissolve. Left un-checked, raw exposed nerves could be destroyed permanently. We are not at that point. Our doctor wants us to see Dr. John Kissell at Ohio State University. He is a well-known specialist in this field. Other neurologists have also recommended him. OSU is only an hour from home, which will be good if we have to go there often. Here are a couple of links about Dr. Mendell. If you read the links I think you will be impressed. We are going hi-tech and pulling out all the stops. Thank you all for your prayers and for your support. We saw Dr. Kissell at Ohio State today. He was so impressed with Whitney's strength he actually asked why she was here. We said, 'because she still has no reflexes and her nerve conduction velocities are only 30% of normal' (about what they were two years ago). Kissell sees a lot of CIDP patients, and in his opinion neither is likely to change much. That is not what I've found in the literature, but you have to remember he is one of the guys on the cutting edge of the research. I respect his viewpoint. His most important measure of a treatment's success is her ability to function well and live a full life. She is doing that, albeit with two infusions per month. (Infusions are not like shots. They involve a lot of fluid and must be dripped into the bloodstream over a matter of hours.) He decided to try eliminating the steroid infusion and increase the amount of Immune Globulin. That's good because there are more problems associated with long term steroid use than there is with IVIG. He also told us there is only a five to fifteen percent of ever getting off of it. Oh well, so long as it works we'll be happy. Whitney has a six million dollar cap on her insurance policy, so she should be ok financially even if IVIG costs almost a thousand dollars per week (which it does). Whitney has recovered quite well and is now in college. Here she is in the center surrounded by dorm friends. She is once again able to do just about anything a normal teenager can. She remains dependent on IVIG infusions every three weeks.
December 2006 Starting to do some professional modeling.
Here she is (on the left) working as a FAN girl in 2008. That's the radio station that covers Ohio State football. Go Bucks!
Phi Beta Kappa -- May 20, 2008.
Graduation June 2008.
Next stop: Moritz College of Law, Ohio State University, August 2008. July 2009: Whitney has not needed an infusion since January. Dr. Mendell thinks her CIDP may be in remission. I can tell you this: She now has the power to actually hurt me when she squeezes my hand. That's a long ways from a few years ago when she could barely hold a pencil. Since she is doing so well, how about a trip to Europe? Here she is with her law school roommate getting ready to head over to Oxford, England for some summer study.
------------------------------- If you made it to the end of my journal, I strongly encourage you to check your medical insurance policy. Take a look at your prescription drug section. Our policy has a deductible for prescription drugs of $500. In reality because of the exclusion, IVIG falls under major medical. In our case this means that what I thought would cost us $500/year actually costs very serious money, each and every year. Does YOUR policy exclude coverage for blood products? If so, start shopping for a new policy immediately! "Blood products" sounds pretty unimportant; that is, until you or a family suddenly needs them. That seemingly minor exclusion could put you on the hook for literally thousands of dollars out of pocket per month, forever. Don't let the insurance companies do this to you. Find better health coverage and after you've secured a new policy, fire your present company for selling such a worthless policy. I don't know all the companies you should avoid, but here are three. I've disguised them a bit so the web indexing machines don't find out. Read the capital letters. These are bad guys. They have exclusions nobody would ever pay attention to (except me). They take your money and do not protect you. F*7^O$%R*#T@!I*S^ T**I*()M&_E S4T3A#T@E_F*A^@R#M
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The nurses started the infusion at 11 today and it took five hours, about 1
1/2 hours longer than normal. Her blood pressure was a bit lower than usual at
the start. This particular nurse is pretty good about running it slow, but about
1 1/2 hours into the procedure Whitney got very cold, sleepy and started shivering
violently. Even though she has tolerated this reasonably well in the past, today
was an exception. IVIG can make you go into shock and it's not something
to fool around with. The nurses stopped the flow, put a call into our
neurologist and gave her something warm to drink. He
prescribed some ibuprofen and more benadryl and told them to resume when she was
stable. (Once ivig is prepared with distilled water it has to be used quickly or
tossed out. At $2,500 a pop you don't want to throw it away,
either!) They kept the flow rate very slow
and the rest went in ok. It was pretty scary for non-medical people like me. 
IVIG
