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First, you must understand that our mother was always extremely independent—both physically and by personality. She was divorced from our father in 1977 and lived alone in Midland, Texas. She was a public school teacher, retiring in 1987. She loved to travel, play bridge, do yard work, read and sew. She loved people and kept a busy social calendar, especially after her retirement. She always had beautiful handwriting and it was an envelope addressed to me in the fall of 2001 that provided the first evidence that something was not quite right. She told me over the phone one day, “There is something wrong with my right hand.” (She was right-handed.) She went to her primary care physician. He told her it was a neurological issue and sent her to a neurologist in Midland. Timeline Fall 2001—Handwriting was a little difficult and slowed; not as pretty as before. She had some difficulty using her right hand, mostly with writing. She stopped signing her name on her checks but used only her initials to reduce required writing. She also frequently used a typewriter for the next two years, as it was easier than handwriting. June 2002—Executed a Power of Attorney—writing is beginning to look a little wobbly. This Power of Attorney was an extremely valuable piece of paper for us. Armed with it, I was able to easily handle her business, financial and medical affairs as the disease progressed and she became much more dependent. Summer 2002—Dortie and I made a bunch of blue jean quilts together (I think 8)—where I put the pieces together and she did the sewing. She could still use both hands then, although she was slower than normal. Thanksgiving 2002—Dortie made several calculation errors regarding Thanksgiving dinner—very unlike her. She had been hosting her family for Thanksgiving for more than 30 years. Her right hand was becoming more difficult to use but she could still use it. She had received a diagnosis of Dystonia from Dr. B. in Midland. She went to a Physical/Rehabilitation doctor and received Occupational Therapy, which did not help. He also prescribed a drug (I don’t have the name of it) which did not help her use her hand but instead, globally slowed her reactions and gave her some strange behaviors. January 2003—Waiting on results from some tests made by Dr. B in Midland. When these results were in, Dr. B. didn’t know anything more to do for her hand and would not refer her to anyone. I asked her to let me find a doctor in Dallas and she agreed. We got an appointment with Dr. Padraig O’Suilleabhain at UT Southwest Medical Center. If we hadn’t sought another doctor, we probably would have never found out what was the matter with our mother. CBD is very rare. There were several times that we were very glad we had Dr. O as a resource, as no one, I mean NO ONE, had ever heard of CBD, much less knew how to cope with it. February 2003—First visit to Dr. O in Dallas. Preliminary diagnosis of CBD. Dr. O was thorough but wouldn’t be definitive regarding the diagnosis. He said he would need to see her again in 6 months to get a better idea. He prescribed Sinemet to try and help rigidity and slowness of right hand. (The Sinemet didn’t do anything for her. Dr. O later doubled the prescription and it still didn’t help her so we stopped it.) May 1, 2003—She drove to Odessa to have lunch with our cousin, Janet. Her driving was beginning to worry Jodie and me. She was slow to get in and out of the car and slow to grasp and manipulate things with her hands. We worried about reaction time, too. Dr. O had told us that he could be the “bad guy” if we needed him to be, and prohibit her driving. We later took him up on the offer. Late June 2003—in Albuquerque NM for wedding. Slow to get ready, walking is slower, eating with her right hand is becoming difficult, slight oddities in speech observed by family members. She was beginning to have a somewhat harder time pronouncing words, and a few problems with word recall. July 4, 2003—Last time at the Cabin in Ruidoso for Family Reunion August 3, 2003—Second appointment with Dr. O in Dallas. He confirmed the diagnosis of CBD and prescribed Zoloft. Dortie was pretty depressed with the outcome of the visit, having read on the internet about CBD and its course. The Zoloft helped her “keep truckin’” and lifted her spirits somewhat. I noticed about this time that she didn’t call me as often as she used to, but when I called her, she always told me that she was “Fine!” She and I made our last blue jean quilt together around this time and the stitching looked less professional than her earlier ones showed. Thanksgiving 2003—Jodie and I did the meal in Midland with Dortie’s verbal assistance. There was a lot of evidence that daily living for her was becoming more difficult and time-consuming, with the right hand working slowly and often assisting the left hand. She quit playing bridge this fall because she could no longer hold the cards. She was still driving and it really scared Jodie and me. Her sister in Lubbock was sick and she drove up there (a 2-hour drive) to help her. Privately, I couldn’t imagine how she could be helpful but our cousin was most appreciative of Mom being there. December 2003-February 2004—Several falls occured in her home. One of her church friends secretly called Jodie to report a particularly bad one, thinking we did not know about it (and we didn’t). Another visit with Dr. O in February showed continued decline in motor skills, motor planning (things like imitating the way you would throw a baseball) and “thinking skills”. He prescribed Aricept to help with “thinking”. It probably helped her a little, although we didn’t see a huge difference with this drug. It is important to note that cognitively, Dortie was always okay. She had some difficulty with speech in pronunciation of words and often specific word recall but she was never “demented”. She experienced no pain or “alien limb” phenomenon, which is often associated with CBD. March 15, 2004—Moved out of her home in Midland to an apartment in Clifton, TX. These apartments do not have assistance but she has a button that is wired into the local EMS service. If she pushes the button, an ambulance comes and she automatically goes to the emergency room. It provides a sense of security that we all have been seeking. Residents of Type A facilities must be able to get themselves out of the building in a fire drill, without assistance. When she moved in, Dortie was walking, getting up and down from a chair, using the microwave and stovetop for cooking her own meals, and generally independent. Early June 2004—Flew to Bethesda, MD for CBD Study with NIH…all but 2 tests completed; would need to return later in summer. While Dortie and her granddaughter, Elaine, were in Bethesda, Ronald Reagan died and the government, including NIH, shut down for several days. That was the reason why her last tests had to be rescheduled. It was a difficult trip for both Dortie and Elaine, and we were all sorry that NIH hadn’t been able to finish at that time. It was also a beneficial trip, however, in that Dortie received some exercises for both large and small muscles and those kept her busy for the next several weeks. The professionals at NIH were, of course, very familiar with CBD and were able to provide resources that we really needed and hadn’t found anywhere else. Early July 2004—Dortie fell, resulting in a broken nose and hospitalization to stop bleeding. About the time that Dortie was moving from Midland, she reported that she thought the CBD was in her feet. Motor planning got harder and harder for her and she began having balance problems, too. We had lots of trouble trying to help her walk. She didn’t like the way we tried to help, insisting that we were “pulling her up” too much. It was such a struggle, trying to be helpful and allowing her to continue being as independent as possible. We were able to start home health care after her discharge from the hospital. The agency sent someone to help her bathe and a nurse came once a week to check on her. She also received physical therapy for a month through the home health agency. These people were wonderful because they provided essential services for her and also broke up the monotony in her day. Later July 2004—Another bad fall, requiring stitches (staples) in her head. It seemed that Dortie had bruises of varying colors everywhere—her head, arms, legs, and backside—due to falls. She was just not able to balance well enough or react quickly enough to stop herself from falling. She was unable to use a cane or walker, although we tried many different types. She couldn’t grasp with her right hand well enough to use these items. Sometime during that summer she said, “I don’t know what I’ll do when I can’t use my left hand anymore.” Despite being right-handed for some 78 years, she had been slowly forced to use only her left hand. Summer 2004—Jodie bought her a lift chair, which was in the “nick of time” because Dortie could no longer get herself out of a chair, even one that had a huge cushion added to give it additional height. As for walking, NIH had recommended a special “platform” walker to assist her. We never got the platform walker. By the time we went through the right doctor and then found the company and representative that could make it, she was bedridden. My sister and I lamented over and over that we seemed to be “chasing” the CBD—it was leading and we had no idea where to or how fast we were going. It was almost impossible for us to successfully anticipate her needs---we seemed to be always in a “catch up” mode. I really believe that the rarity of this disease held us at a tremendous disadvantage in trying to take care of her, because there was little in the way of “roadmaps” for us, especially early on. Also, she seemed to progress more rapidly downhill than anyone anticipated. Late July 2004—The “Type A” Facility no longer could accommodate her—we had to find more and daily assistance for her. She had a fall one morning and didn’t want to push the help button because she “wasn’t hurt and didn’t want to go to the hospital”. She stayed on the floor all day that day, until Jodie found her late that afternoon. Dortie was so persistent and wanted so badly to remain independent that most people would call her stubborn. Jodie and I were both in awe of her strength, determination and high pain threshold….if anyone ever asked her how she was, she would say, with energy and authority, “I’m fine!” Jodie and I began a search for a facility near one of us that could take care of her increasing needs and uphold her dignity for as long as possible. August 2-5, 2004—Returned to Bethesda to complete tests for CBD study. Jodie went with her this time. They came home with several pieces of specialized equipment and resources, including some special dishes and eating utensils, a brace for her arm, catalogs for other special equipment, like elastic shoelaces that remained in her shoes. Dortie was very satisfied that she had contributed to a study that could someday help others with this terrible disease. She also agreed to an autopsy of her brain after her death, also to be used in the NIH study. August 6-13, 2004—Visited Autumn Oaks Assisted Living in Corinth; made arrangements to move. The people at Autumn Oaks were very warm and friendly and assured us that they could take care of Dortie without her having to move again (which translated into “No Nursing Home”---a real plus!). She would have a one-bedroom apartment that also had an efficiency kitchen in the living room. She could have meals in the dining room with the other residents or in her room. Autumn Oaks was a brand new facility and had only about six other residents. The best thing about Autumn Oaks was that it was a 6- minute drive through the neighborhood from my house. I could be there quickly and often and I know it made all three of us feel better. August 14, 2004—First night at Autumn Oaks. Having her own furniture and decorations made the transition from Clifton to Autumn Oaks pretty smooth. We had already culled her furniture and possessions in the first move from her home to Clifton and only a little more “sifting” was required for this move. At Autumn Oaks, there was a string that when pulled, activated a “call button” for the caregivers. Dortie could pull the string with her left hand whenever she needed to get up. At first, she didn’t want to pull the string but after several falls there, we (A.O. staff, Jodie and I) convinced her to always pull the string when she was getting up. I spent the most of many days with her and could even come help get her ready for bed when the one male caregiver worked the evening shift. Having assistance for her 24/7 brought some peace of mind for both Jodie and me. The staff at Autumn Oaks knew nothing about CBD but they were willing to do whatever they needed to do for Dortie and it was just what we needed. It is really a wonder they agreed to let her stay there because on August 17, her third day there, she fell three different times in her room. We also began home health care again, using the agency that Autumn Oaks staff recommended. August 26, 2004—Appointment with Dr. O in Dallas (asked for referral to Physical/Rehabilitation Dr. for help with wheelchair and therapies) This visit turned out to be Dr. O’s last real examination of Dortie. As Jodie had learned from NIH, Dortie needed a Physical/Rehabilitation doctor to coordinate her therapies (PT, OT and Speech) and assist us with things like wheelchair selection and the platform walker. We asked Dr. O for a referral to such a physician and he obliged. When we were there, Dortie could still speak and stand, with lots of assistance getting up and remaining standing. We had driven to the doctor in our car, with her seated in the front seat. We had rented a transport wheelchair that was light enough that I could lift it into the trunk of the car. It gave us freedom to take her places in town and kept Autumn Oaks from being too confining. August 27, 2004—Highland Village Balloon fest—We used a transport wheelchair and took Dortie to see the hot air balloons in a nearby town. She loved being out and seeing the balloons. She had fallen so many times that her face was black, blue, green and yellow and I was aware of people staring at us. Jodie had obtained a handicapped parking permit from the doctor in Clifton and we used it to reduce our walk to the balloons and I marveled at how helpful such a small thing can be for a person with a disability. August 31, 2004—Appointment with Dr. Simms in Denton (He became her primary care physician.) Dr. Simms and his staff were so thoughtful, caring, respectful and professional when we took Dortie to him. He made arrangements so that he could order refills for all of her prescriptions, other than the ones that Dr. O had given her. (Those were for blood pressure and they kept it at a perfect level.) He checked her over and took her history. Although he knew very little about it, he had heard of CBD. Taking her to him was a most helpful move for us, as we needed someone close by that would serve as her primary care physician. He was excellent in this important role. September 16, 2004—Trip to JC Penney with transport wheelchair—turned out to be her last “social” outing. About this time, Dortie began to have what we decided must have been little “panic attacks” sometimes, usually at night. She would start to sort of hyperventilate and sometimes had trouble sleeping, which she had NEVER had during her life. We discussed these symptoms with Dr. O and he prescribed Ativan for anxiety and sleeplessness. She used it fairly often for a couple of months. September 26, 2004—Fell forward out of wheelchair onto forehead. Walking became almost impossible during September, even with the “Gait belt” that NIH had given her. She just could not make her legs and feet do what she wanted them to do and balance was almost nil. She was using the little transport wheelchair to go down to the dining room that day, with her feet “walking along” as she sat in the chair. Her shoe must have hung on the carpet and being unseatbelted, she fell forward out of the chair onto her forehead, giving her a huge “goose egg” and bruising that covered half her face. By this time Dortie was having trouble feeding herself. It took her a long time and much of the food spilled, even with her special utensils. The staff at Autumn Oaks was feeding her every meal by the end of the month. September 29, 2004—Appointment with Dr. Susan Murphy in Dallas. Dr. Murphy was a wonderfully empathetic and sensitive physician. She spent 2 ½ hours with Dortie, carefully evaluating her level of physical function. She recommended someone who could fit her with a good wheelchair and the platform walker and prescribed speech, occupational and physical therapies with “good people”. I made appointments right away to restart home health care for the therapies and with the wheelchair man for a fitting. The other thing that Dr. Murphy suggested was Botox injections to help relax the muscles in Dortie’s arms and hands. We had read about them but did not know the best time for their administration. She suggested that now was a good time so we made an appointment with Dr. O for those injections the next month. October 1, 2004—A.M.—Phone call from Dr. Grafman regarding results of visits to Bethesda. He gave us nothing new, really, but just a summary of her visits to the NIH. October 1, 2004—P.M.—Dortie decided she could get herself ready for bed and elevated lift chair to the max, falling out onto the floor. She insisted she was fine. The caregiver noted no physical problems and put her to bed. October 2, 2004—early morning—She appeared to be having difficulty breathing and our concerns over possible injuries from the fall the previous evening prompted a call for an ambulance. The evaluation by the EMT’s suggested that a trip to emergency room was in order. October 2-3, 2004—Stayed in hospital for observation, tests. They did several tests on her heart and did a CT scan on her head. Her heart was fine and there was no concussion. A technician took her for an MRI and when she returned, about a half-hour later, she was agitated and on the verge of tears, telling me, “Electricity is running throughout my body!” She remained agitated for quite a while, until I asked for them to give her some kind of tranquilizer or relaxant. No one I asked at the hospital had ever heard of such a response to an MRI, nor has anyone since then. I have read somewhere that CBD patients sometimes have “peculiar or outrageous-sounding symptoms”. This was the strangest and most unexplainable symptom up to that point in time, for sure. What she did have, though, was a huge bladder infection. The hospital staff was helpful and kind but they, too, had no idea about CBD and it’s peculiarity. I had told them that Dortie couldn’t feed herself and yet one morning, when I came in, her breakfast was sitting on a shelf, uneaten. When I asked her about it, she said the girl had just set it down on the table, assuming, I imagine, that Dortie would get it if she wanted. It was then that I realized that Dortie had reached a position of being totally dependent on others for her care and sustenance. October 3, 2004—We returned to Autumn Oaks in a private ambulance. The doctor had ordered it, as he could see that we had no way of transporting her by car. She could no longer be of any assistance in moving in and out of the wheelchair, having no command of her legs at all. She was unable to stand or walk from that point on. When we arrived back at Autumn Oaks, we saw pretty quickly that we would need a hospital bed and obtained one that week. October 2004—We changed home health care providers and began with new therapy programs. The therapists did their best to keep range of motion and reduce tightness and rigidity in Dortie’s arms and legs but they also showed me what to do so exercises could be done every day. Since she was not able to even move herself to make “comfort adjustments” as we all do thousands of times each day, these exercises did a lot to increase her comfort level…not that she was in pain but she was frequently uncomfortable, just with posture or position, and needed moving. The staff was also turning her every two hours in an effort to try and prevent the breakdown of skin tissue and hence the formation of bedsores. She had a poor appetite, however, and her food and drink intake decreased slowly but steadily through the fall. October 7, 2004—Wheelchair fitting with man from company recommended by Dr. Murphy. The chair was not ready before she died—we called and cancelled the order after she passed away. October 12, 2004—Botox injections from Dr. O. We made arrangements with a private ambulance to transport us to Dallas for the appointment for the injections. There was no way we could have transported Dortie in our car. She couldn’t transfer from wheelchair to car and couldn’t even sit upright anymore. The injections were given in her arms and in her hands and fingers. We saw great benefit in loosening her fingers and hands but her arms remained stiff and contracted (bent into an “x” across her chest) until her death. It was during this trip that we noticed that her leg was having a strong contraction that bent her knee and brought her foot up about 12 inches on the bed. It was very painful for Dortie and except for postural discomfort and injuries from falls, was the first pain that she had felt with the disease. We brought it to Dr. O’s attention during the Botox injections and he agreed to prescribe a medication to try and stop the contractions. This medication was Carbamazepine 100 mg. (This is a generic form of Tegretol.) He started her with one tablet twice a day and over the next two weeks it would double to 2 tablets four times a day. It was trial and error with medications and their dosages, with me talking to Dr. O through his nurse, trying to describe various effects and side effects. (Working with him, awkward as it was, seemed essential, as Dr. Simms knew nothing of CBD and its management.) The Carbamazepine had little effect at first. But then, as the dosage increased, it seemed to put Dortie into a “fog” and she was unable to eat, speak and think very well at all. With the full dosage, it did decrease the leg contractions but never stopped it completely on its own. Dr. O added Mirapex a little over a month later, and Parcopa the first part of December. Together these medications finally did stop the contractions altogether. October 2004—We found some specialized equipment very helpful about this time. We obtained a Hoyer lift that the caregivers used to move Dortie from the bed to the chair and back. We searched for a portable tub of some kind, thinking that soaking in hot water would help her contracting leg and general comfort level. It took about six weeks to finally adjust the Carbamazepine, Mirapex and Parcopa and their effective dosages for the contracting leg (Actually, both legs took turns doing the contracting. If it wasn’t one, it was the other.) She had rubbed blisters on the bottoms of her heels, when the contraction would drag her foot upward in the bed. We ordered some heavily padded footpads from one of our specialty catalogs but still the blisters were hard to heal. A circulating air mattress was ordered to try and prevent bedsores. Even with turning her every 2 hours and the special mattress, she still developed an awful-looking bedsore right at the bottom of her back. We treated it with “wound care” but because she was taking in so little nutrition, it never healed, although it did improve. November 10, 2004—We were to have had a second appointment with Dr. Murphy in Dallas on this day but I called and cancelled it. By this time, we were about out of therapy visits (Medicare doesn’t keep paying unless the patient is making progress and Dortie was going downhill fast.) and we would have had to order the private ambulance again to make the trip. (Medicare would pay only one half of that trip and it was very expensive to make the trip to Dallas.) Dortie seemed so frail and it did not seem like a smart or productive use of time not to mention a tremendous aggravation to her to take her out for that appointment. (Moving her, even to turn her, was painful since she was so stiff.) She had also developed another giant urinary tract infection and now had an in-dwelling catheter, which also complicated a trip to Dallas. November 25, 2004—Thanksgiving—Most of Dortie’s extended family (mostly my cousins) visited her on Thanksgiving Day. They were surprised at how thin, frail, and unable to move that she was at that time. She could still speak but sometimes some of her words didn’t make sense. It was like the syllables got all mixed up. End of November 2004—Autumn Oaks, being a new facility, was still seeking its license as a Type B facility (residents were not required to get themselves out in a fire drill.) They were having difficulties getting the Type B license and so decided to complete requirements for the Type A license immediately and then go for the Type B. Dortie, however, didn’t fit the Type A profile and so we were going to have to move her somewhere or make some other arrangement besides the current one. We decided to move her to one of their “independent apartments” (these surround the assisted living facility). Because of their commitment to us when we moved in, Autumn Oaks continued providing a caregiver for her round the clock, for the first three weeks of December. Her intake continued to dwindle and she seemed more and more to be “out of it”, often babbling “Corticobasal, the Corticobasal….”. The medications finally had pretty much controlled the leg contractions, which was a great relief. It was becoming more and more difficult to try and feed her or give her medications, as she would bite the spoon, most likely as a reflex. The caregivers were crushing her medications and mixing them with applesauce by this time. Early-mid December, 2004—With Autumn Oaks unable to continue devoting one caregiver strictly to Dortie in her “independent apartment”, Jodie and I began to look at nursing homes in my area and in Whitney, near Jodie. Dortie continued to spiral downward, taking in and putting out less and less every day. She began to seem really far away and her eyes seemed to look through us during the second half of December. December 21, 2004—It seemed that we didn’t know “where we were” in the course of the disease and with the pressure of needing to make new arrangements for Dortie’s full-time care that might include a move, I called Dr. Simms to discuss our dilemma and he offered to make a home visit, which I eagerly accepted. When he saw her and the records we had been keeping of intake/output, he recommended hospice begin immediately and felt that she had a month or less to live. With such a short time involved, we made a decision to hire a full-time, live-in, round the clock caregiver through an agency (Heaven at Home) and leave Dortie at the apartment in Autumn Oaks. We felt that moving her to Whitney or even Denton to a nursing home would probably be fatal. It was going to be expensive to contract with this company for a live-in caregiver but we felt that it would be such a short time that we could afford to do it. December 25, 2004—Wanda English, the caregiver from Heaven at Home, moved into Dortie’s apartment to be her 24-hour caregiver. She was wonderfully sympathetic, caring, experienced and efficient. She took excellent care of Dortie and was a wonderful support for Jodie and me for the week that she was there. December 27, 2004—Hospice care began with Ann’s Haven. We had to change equipment, including the hospital bed and air mattress, because we were no longer with the home health agency. The change was done fairly easily, though, with the hospice nurse, Wanda, my husband, and me. We eliminated any medications that were not necessary for comfort. We also used butter and her essential medications to make suppositories, so that she would not have to take them orally anymore. December 31, 2004—I visited Dortie and Wanda during the afternoon. She didn’t know I was there and already seemed “gone” to me then. While at a New Year’s Eve party, I received a call about 9:00 that Dortie had just passed away. We hurried over to her apartment to start the process for the autopsy that she had agreed to for the NIH CBD study. The autopsy was completed on January 1, 2005 in Dallas at UT Southwestern Medical Center. The report was mailed to me in October 2005, after several phone calls I made inquiring about it. As of this writing, December 2005, I still have received nothing from NIH regarding the research autopsy results. Looking back over our experience with our mother and CBD, it seems like we had the rug suddenly pulled out from under us and we spent about a year trying to regain our balance. We seemed forced into reacting to situations and needs rather than being able to be proactive. Part of that might have been a subconscious reluctance on the part of Jodie, Dortie and me to “take away” any of Dortie’s independence. We let her live independently as long as possible, in every situation. If I were giving advice to another family regarding this disease, I would suggest trying to anticipate needs as best you can and seek the services of the most experienced physicians, therapists and caregivers that you can find. Also, getting financial matters in order ahead of time proved extremely valuable to our family as we took care of our mom. Judy Graham, Daughter of Dottie Powell |
