JoAnn's Story
My mom' s medical history prior to July 1997:
Blood transfusion after a miscarriage in 1958, Balboa Naval Hospital, San Diego, CA.
Two nerve blocks, one in the early 1990's and one in the mid 1990's, Methodist North Hospital, Memphis, TN.
Paradonal surgery, January 23, 1995, Memphis, TN.
During my mom's illness she only became hostile when we would try to brush her teeth. In the early part of the fall of 1998 she would actually laugh a lot for no reason. Later in the fall she would cry a lot. The only times she appeared to be in pain was after she was confined to the bed and we would attempt to either straighten her legs out or exercise them. She did not like us to touch her legs. She never showed signs of her hands shaking or being sick to her stomach. None of us can remember her commenting on food tasting bad, however one of my sister-in-laws does recall her saying that Diet Cokes, which she drank regularly, did taste odd.
In July of 1997 my mom was experiencing fatigue and dizziness. She was first diagnosed with low B12; her doctor felt that this could be the cause of her feeling poorly. She had a MRI done on July 24, 1997; it did not show anything unusual. The rest of that summer she continued to grow more fatigued and to have problems with dizziness. She went to her eye doctor several times to have her eyes checked and her glasses adjusted.
Christmas came and went and 1997 rolled into 1998. In January my mom went to the doctor and they did some blood work to see if they cou ld determine the cause of her fatigue. They discovered that her B12 level was still low and put her on B12 shots and pills. She had another MRI done on January 23, 1998; once again it showed nothing out of the ordinary. Her health continued to decline the rest of the spring of 1998. She was still bowling until April 1998 and going to water aerobics three days a week until early September 1998. She had given up driving some time prior to April and my dad had to help her to the car that she was a passenger in going to bowling or water aerobics.
One of her hobbies was cake decorating, in May of 1998 she made her last wedding cake. Another of her hobbies was sewing, by July she could not remember how to thread her sewing machine. She was an avid book reader, however she lost the ability to understand the books she read.
In June of 1998 my mom went to an ENT to see if there may be some inner ear problem; results negative. June 25th, 1998 she underwent a three and half-hour memory test; results were severe memory loss.
July 4th, 1998 my sister had a cookout at her house. By this time my mom could not get up from a seated position unless she had someone or something to pull herself up with. She went inside to use the restroom while everyone else was outside. After a while my sister went to look for her and found her in the bathroom, unable to get up from the toilet and crying. By this time we realized there was something VERY wrong.
July 9th, 1998 my dad, sister, and I took mom to her family doctor, he was on vacation so we saw his nurse practitioner. She suggested we take her to the emergency room and perhaps they would admit her. We had planned to do that anyway, we did not want to bring her home until we had some answers. We had borrowed a cane to help her walk but she did not have the coordination to use it.
My dad and I took her straight to the emergency room from the doctor's office. They did a CAT scan that showed a hemorrhage on her brain. They wanted to do a MRI so they admitted her in the hospital. My mom began to cry when they mentioned MRI; she was very uncomfortable in the MRI machine. They agreed to give her a sedative to help calm her down. I can remember how glad we were that they had found something on the CAT scan and we felt sure her problems were being caused by the bruise on her brain.
When they brought her up from the MRI, she was very "out of it" due to the sedative they had given her. My dad went home and I stayed with her at the hospital. She was very restless, apparently the medicine caused some hallucinations, and she wanted to get out of bed and kept mumbling about things coming after her. I did not sleep that night because she kept trying to get out of bed. I would look over and she would have both of her legs thrown over the rails of the bed.
The next evening when her doctor came in he said the MRI showed the same thing that the CAT scan showed, nothing more. He asked my mom if she had bumped her head lately and she and my dad both said no. I asked if she might have bumped it getting into the car, they both said no. Finally after several minutes of prodding she said that just a few days earlier she had been standing on the bed turning the ceiling fan off and had fallen and hit her head on the nightstand. We all sort of laughed and said yep that would do it. At that moment I knew things were bad, the fall and the hemorrhage were a result of her lack of balance not the other way around. We were basically back where we had started. The doctor was saying that her fatigue and lack of balance was permanent damage caused by her B12 level being low.
While mom was in the hospital I would ask her to name all of her grandchildren, there are eight of them; she could give me first and middle names, and their birthdays. I kept telling myself that there was nothing wrong with her mind if she could keep all of those dates straight. My oldest sister was sure that mom had had a stroke. The doctor kept telling us that is was not a stroke. I thought it was some muscle disease like Lou Gehrig's disease. Both of those possibilities and many others were slowly being ruled out.
Monday July 13th my mom was released from the hospital. She started physical therapy on July 21st. She was originally scheduled to go 5 days a week for three weeks. After her first day of therapy they changed it to 3 days a week because she tired so easily and after the 3 weeks were up they extended it for a few more weeks. I do not really remember it helping all that much, I think at the time we all wanted to believe it was helping.
On August 1st she had another CAT scan done to check the hemorrhage on her brain. August 6th my dad & I took her back to the doctors to get the results of the CAT scan. It showed that the hemorrhage had gone away. However the doctor stressed that the low B12 levels had done permanent damage. She was not talking very well but she was able to say that she wanted to drive again. I will never forget the look the doctor gave me, it was a "It ain't ever gonna happen" look. My dad and I asked about an experimental test they were doing for Alzheimer's disease, an APOE test. The doctor also put her on a drug, Aricept, which had shown some promise in Alzheimer's patients. He said if she had Alzheimer's it would slow it down and if she did not have it then it would not hurt her. When the test came back and showed that she did not carry the Alzheimer's gene we were so happy.
The rest of the summer and early fall my mom continued to do "odd" things. One morning after brushing her teeth instead of turning the water off she turned it on full force and walked away. She was still able to feed herself; however, we had to keep a close eye on her because you never knew what she would do. She would ask for more of something that she had plenty of on her plate or she might take a bite of a dinner roll one time and the next time she picked it up she might dunk it in her drink. In late August/early September she began to be afraid while watching television. Her favorite shows were, "Touched By An Angel," "Diagnosis Murder," and "Dr. Quinn Medicine Woman." She would get out of her chair and go to the back of the house and say, "the bad guys are after me." Around this same time she started saying, "I am not crazy," she would say this over and over.
My dad took her to Kansas, where she was from, the last part of October for my grandmother's 91st birthday. November 6th my mom and dad returned from Kansas. Dad drove the 12-hour drive without stopping for the night. He said my mom did not sleep well when she wasn't at home. She did not sleep well at home either. They had stopped in Little Rock on the way to Kansas and stayed at my brother's house. She was afraid to go to sleep unless my dad was in the room with her. She had stayed at their house, in that room, numerous times and was never afraid before. The day my mom was diagnosed with Creutzfeldt-Jakob disease I called my mom's sister Margaret to tell her. She said that she had a feeling the last time my mom was back in Kansas that it would be her last visit back "home."
Things began to fall apart very quickly after they returned from Kansas. On Saturday November 14th my dad had gone to get groceries and while he was gone someone had called the house. It scared her, so she somehow walked over to our next door neighbors. By this point she was unable to turn the TV on or off and she was unable to lower the footrest on her recliner. It still surprises us that she was able to get out of the chair and walk to the front door, unlock the security door and walk over to their house. They were very surprised to see my mom as well. My mom began to cry and it took some coaxing to get her to tell them that my dad was not home and that the phone had rung and scared her . From that day until the day she died my mom was never left alone again. She continued to keep saying that she was not crazy. On November 16, 1998 her doctor put her on Risperdal, a medication for Schizophrenia, we did not tell her what it was. This calmed her down some but it also made her drool all the time.
We had our pictures taken for the church directory on November 21st. In the pictures you cannot tell there is anything wrong but in actuality my dad had to hold my mom up because she would fall over. Since my dad already had her dressed and out he went ahead and took her to Saturday evening Mass. She missed Mass the previous week and to this point we had tried to keep things as normal as we could. We had been taking her to Mass each Sunday; it really upset her when she did not go. November 22 was the last day my mom was in St. William Catholic Church alive.
The next day my dad was very upset, he was really down about what we were going to do with my mom. This was the first time that we discussed putting her in a home. Dad called the doctor and they just increased the Risperdal, it had really slowed everything down. She was slobbering all the time, we had to keep a towel around her neck and change it several times a day. She was also unable to go the restroom by herself and my dad had started having her wear undergarments because she had lost some control of her bladder. By this point she was unable to talk in sentences. She could say a few words but most of what she said was incoherent. Talk of putting my mom in a home was a very difficult topic. Are people going to think we are putting her in a home because we don't want to be bothered with her? Is it the "easy" thing to do? If something happened to one of us would mom just "give us away?" There are no easy answers to this; my heart goes out to the hundreds of people who have to make this decision every day.
Monday November 30: we took my mom back to her neurologist. He was very surprised at how rapidly she had declined. He said that he wanted to run another MRI and asked if we wanted to schedule an appointment for one or admit her to the hospital, it would be done quicker if she were admitted. We decided to have her admitted. While in the hospital her doctor was concerned with her Potassium level being low so he had them give her some through an IV. Apparently this burns very badly. My mom was very uncomfortable and it took several hours for the entire bag of Potassium to empty. The nurse had me keep a warm cloth on her arm in an attempt to open her blood vessels some and allow the Potassium to flow with a little less discomfort. I felt so helpless as my mom tried to tell us how bad her arm was burning. While the Potassium was dripping I tried to pray the Rosary with her . She got very upset and the only reason I can think was that she was unable to recite the prayers and that upset her. After the IV was through we finally got her to settle down some only to have the lady come in to draw blood. This once again got her stirred up. By this time she was very agitated and it was a chore to get her to calm down.
Tuesday the doctor did a spinal tap, an EEG, and a CAT scan. When I spoke with him he said the next steps would be an arteriogram. If that did not give us any an swers they would run another MRI and if that did not reveal anything he mentioned doing a brain biopsy. I asked him what they were looking for in a brain biopsy and he danced around an answer. I spoke with a friend of mine whose sister is an infectious agent nurse and she mentioned Mad Cow disease. I started looking on the internet and I found that there was a human form known as Creutzfeldt-Jakob disease. I had my dad ask the doctor Thursday when he made his rounds about this disease and the doctor said that I was too smart for my own good. The next day when I spoke with him he said that he did not want to mention that until he was pretty sure everything else was ruled out but that since I had brought it up we would discuss CJD. I already knew from reading on the internet that it was an incurable, non-treatable disease. Friday, I talked to my dad and we decided that none of us would stay with my mom that night. The nurses had told us that they had a restraining vest that they could put on her. Friday we tried it, I went up early Saturday morning so my dad could sleep in a little. When I talked to my sister she told me that one of the orderlies had called my dad late Friday night because my mom was calling for him. The young lady thought maybe my dad talking to my mom would calm her down. Of course it did not and the orderly ended up staying with my mom for several hours.
By Monday all the tests had been performed except the brain biopsy and so far there was no news, which in this case no news was not good news. Late Tuesday evening her doctor showed up and said that the brain biopsy would not be done until Friday and we could take her home. The next few days were very hectic; there were nights when my dad had my mom sleep in her chair because s he was so restless in bed. There were phone calls to my brothers and sister who live out of town and phone calls to my mom's three brothers and two sisters who also live out of town. We asked one of my mom's brothers to tell my grandmother (my mom's mom) so they could tell her in person instead of over the phone. We wanted to be sure she understood what was going on.
Her brain biopsy was scheduled for December 11th. They said they might have the results of the tests by the following Monday, so it was a very long weekend which turned into a very long week. My mom's neurologist had told us that there was a chance there might be some kind of blood vessel problem in her brain and that could be what was causing all of this. The doctor's office called Monday and said that they would not have the results until Friday. They had to have a solution sent in and then the sample had to sit in the solution for 24 hours.
On Friday, December 18th we received the news, my mom was diagnosed with Creutzfeldt-Jakob Disease. We never told my mom what she had, we just told her that she was not crazy and soon she would be okay. We left it at that.
So now we were faced with some questions. Did we want to put her in the hospital, in a nursing home, or keep her at the house and have Hospice Care come out? After she passed away did we want to donate her brain to someplace studying the disease? I called my sister at work then she called my other sister and my brothers.
The weekend was spent making calls and discussing what we were going to do. By this time my sister and I had a routine where she would stop by in the mornings on her way to work and help my dad get mom changed, out of bed, and into her rocking chair. Then we would both go over about 8:30pm and get mom in bed, give her a sponge bath and get her strapped into bed. Some days we would wheel her into the bathroom and wash her hair. We tried to make her as comfortable as possible. I hope we succeeded.
On Wednesday December 23, the Hospice people came by. They wanted to get a look at my mom and see which room would be best for them to put the bed in. After going over some formalities the nurse ordered a hospital bed and a wheel chair. We were going to use the wheelchair to take her out in the spring an d get her some fresh air. The last time she had been outside was the night we brought her home from her brain biopsy. By this time she was unable to eat solid food, we were feeding her Ensure three times a day and trying to get her to drink several glass es of water.
The hospital bed was delivered the afternoon of the 23. Having the hospital bed helped a lot, it was much easier to give her a bath, feed her and change her. Throughout the entire 21 days of my mom being bedridden the Hospice people were G REAT; even coming out on Christmas day. Monday, January 11, 1999 I was suppose to attend an all day workshop at work. My dad had some things to do so I ended up not going to the workshop and stayed with my mom. I am so grateful that I did. The lady who gave my mom her baths had come by about 4:30 PM. I remember walking down the hall and I looked in and my mom was on her side with her eyes opened just looking. While my mom was getting her bath she fell asleep, which was not unusual, the baths always seemed to relax her. That night when I got ready to go to work I went over to help my dad get her ready for bed. He told me that she had not woken up since her bath. We just thought she was sleeping. The next morning my phone rang about 8:30. I let the machine pick up and I heard my dad, I picked up the phone and he started to cry, he said that there was something wrong with my mom, they could not wake her up. This happened a couple of times before and it just took some prodding to get her to wake up. I went over and as soon as I felt her skin I knew something was wrong. Her skin was very cold and clammy and her breathing was very labored. My sister had already called the nurse and our priest, both were on their way over. Our parish priest arrived a n d administered Last Rites. The nurse had also arrived and removed the catheter and said that it would be just a matter of hours. The nurse called in a prescription for some morphine and we gave my mom medicine every thirty minutes. We alternated between a liquid and a topical cream, the morphine helped her breath easier. The nurse stayed all morning and afternoon and sat in the room with my mom. Because we were unaware that my mom had gone into a coma until Tuesday morning and her condition declined so rapidly throughout the day my sister who lives in Florida was unable to make it to TN before my mom passed away. My two brothers came over from Little Rock, AR about 8:00pm. We all sat in the room with my mom; we were timing her breathing. At 9:04 her breathing stopped. We all looked and waited for her to take another breath, she did not.
The funeral home that took care of the burial did an outstanding job. We did not experience any problems unlike some other CJD victim's families I have heard about. At the time of my mom's death we had very limited knowledge of CJD and any organizations studying the disease. Because of this we were at the mercy of the doctors in this area to find someplace to donate any tissue or her brain. Unfortunately we were unable to find research facilities.
Thanks to my dad, my brothers and sisters, and their spouses for their help in compiling the information used in this story.