Before I discuss our experience with medications, I should say that we should consider the pHD in this process. It is easy to overlook the feelings of the pHD. Ultimately, a pHD is placing their faith in the caregiver to make wise and compassionate choices. They are placing their lives in the hands of the caregiver. The illness, itself, is daunting, but to agree to take medications, visit psychiatrists, etc., and deal with possible medication side-effects requires tremendous faith and trust. Caregivers should be mindful of this and strive to be as compassionate and knowledgeable as possible.
In my opinion, the issue of medications should ideally be discussed with the pHD maybe as part of personal planning before HD symptoms occur (possibly as part of discussions as you prepare your medical directives) and certainly if a person is having early HD symptoms. I say this because the caregiver and pHD need to understand their role and how each can help themselves and help one another manage the difficult HD experience. It is good to discuss this when the caregiver and pHD can have a compassionate and rational discussion--preferably not during moments of conflict.
If a pHD emphatically says that they will never take medications or visit a psychiatrist/neurologist, etc., the caregiver and pHD need to consider that home care might be impossible as HD progresses and plan accordingly, because a caregiver and family might not have the physical or emotional strength to deal with a patient that has no self-control. To keep a patient at home is a tremendous effort even in the best of circumstances. The caregiver and patient need to understand that if a person is in a care facility and refuses to take medications, there could be problems keeping them in a care facility. If a person needs medications, the issue of taking medications is almost unavoidable. The more effort you invest in this aspect of HD, the better the outcome.
Our early experiences
First, I will provide some background to help in understanding my perspective. HD was a total shock to my wife and me. We had never heard of HD. We had been seeing various neurologists regarding her symptoms—sensitivity to sound, seizures or panic attacks, weight gain, slow walking, and periods of crying. The first neurologist ran several tests (EKG, EEG) but found nothing wrong. The neurologist said, “…since I cannot find anything wrong with your wife, no medication is indicated.” I explained how my wife had passed out on the floor of a local shopping mall checkout counter presumably from the rhythmic effects of Christmas music—and fought paramedics. I told the doctor that we could not go on living like this, that we HAD to have some help. At my insistence, the neurologist said, “…ok, I will prescribe Depakote for seizures.” My wife continued to take Depakote until we eventually visited the third neurologist, who eventually recognized the symptoms I was describing, and requested that she be tested for HD. Her test was positive.
During that fateful neurologist visit, in addition to Depakote, the neurologist prescribed Haldol, Klonopin, and Paxil, and in fairly low doses. I knew Depakote was for seizures and Paxil was for depression. Haldol and Klonopin were for emotional issues and chorea and also helped her sleep. I had heard stories about Haldol, and I felt guilty that my wife was taking it. I was concerned about how many meds my wife was taking.
Meanwhile, my wife began having various symptoms that made life nearly unbearable for both of us. She would wake me up at 2 AM, unable to sleep and say, “...I cannot sleep, what are you going to do for me now?” I had to get up at 5 AM for work. I was seriously sleep deprived. Her chorea was so bad that she could not sleep in bed. She would sleep in a recliner, and I could hear her legs beating on the recliner until she fell asleep well after midnight. She might have a panic attack or seizure—hard to distinguish the difference. Afraid, she would yell for me to help her, and I would bolt out of bed any time of night. She would periodically have intense deep muscle itching and yell for me to come and help her scratch, and I would be massaging her arms or legs to stop the insanely miserable itching until sweat was running off my forehead. Side effects of medications caused her to have constipation. One night, pressure and pain from constipation caused her to have panic attacks the entire night—while I spent the ENTIRE night trying to keep her shaking body from falling out of bed.
In those days, she was still working and driving but missing lots of work. Some days, she worked almost as if she did not have HD. Other mornings, she would have crying spells or refuse to get out of bed. If she was at home, she might call me at work telling me that she could not be alone, for me to come home to be with her. If she thought I was gone from her too long, she would get extremely anxious.
I was desperate for help. At 2 AM, I would be sitting in the upstairs hallway reading the “Physician’s Guide to Managing Huntington’s Disease,” reading the sections pertaining to medications, trying to determine which of the medications I had on hand would calm her down until the next morning. (I STRONGLY advise you to read and re-read this book.) I would analyze what I read about each medication and decide to give her extra medication—planning to call her doctor the next morning. The doctor always told me that I made the right choice and began to give me latitude to alter the dosages. I began to realize that medications are often trial and error. I read elsewhere that over 30 percent of the time the first depression medication prescribed is not the optimum one for the patient.
During these lonely hours of the night, I realized that the only way I would be able to care for my wife at home was if I could get her stabilized with medications. It simply was beyond my ability or physical strength to manage HD or keep her at home. It was beyond counseling therapy. She had no control over her symptoms. No amount of discussion on my part helped significantly. We argued with one another because I thought she was more capable than she was and could control some of her emotions, plus I could not tolerate constant demands and irrational behavior.
I began calling the neurologist’s office two or three times a week with problems, trying to describe her symptoms through his nurse to him, the nurse phoning me back. After a few weeks of my phone calls, he told me that he was going to increase her medication dosages ONE MORE TIME and insisted that I find a psychiatrist to prescribe the medications. In retrospect, his insistence that we see a psychiatrist was possibly the best advice any doctor has given us in the long battle with HD.
The first psychiatrist experience was a disaster. In this particular office, patients first met with a social worker, who interviewed the patient, and then referred the patient to the psychiatrist as needed—the social worker and psychiatrist worked as a team. From work, I called my wife at home and told her that I had a psychiatrist appointment. She had been absent from work--at home for three days-- crying, not eating, not bathing, not dressing, and not combing her hair. My wife replied that she “…was not going to the appointment.” I was emotionally wiped out and desperate. I told her on the phone that she had NO choice, that we HAD to get help, and that I was coming home and, forcibly, if needed, put her in the car and take her to the psychiatrist. Thankfully, she was dressed when I got home. During the trip to the psychiatrist, she cried nonstop and begged me not to take her to the psychiatrist.
At the psychiatrist office, the social worker decided to interview my wife privately. I explained that with HD, my wife could not accurately describe her symptoms, feelings, or behavior. The social worker ignored me. After the 30 minute interview, the social worker returned and said, “...Mr. Kendall, I have interviewed Mrs. Kendall and conclude that she does not have depression. I recommend that she work less to reduce stress and listen to some relaxation tapes.” I was incredulous. I asked her if my wife was not depressed, then why had she been crying for three consecutive days, not eating, not dressing, etc. and was already missing two to three days of work per week?” She replied, “…Mrs. Kendall must have given me incorrect information.” Grrrrrrrr!!!
A good working relationship with a psychiatrist makes the difference
I began my search for another psychiatrist. Fortunately, my sister (125 miles away) had been seeing a psychiatrist for her son. My sister asked him if he had ever worked with HD patients and if he would accept my wife as a patient. The answers were yes and yes. This began our first methodological approach to managing my wife’s medications and emotional and physical symptoms.
We initially had a 90 minute baseline psychiatrist appointment, then visited him every six weeks thereafter. It took approximately two years of regular visits to get my wife stable as she is today. She has been remarkably emotionally stable for several years. Physically, she has declined a lot, but that is beyond my control. The psychiatrist decided to begin changing some medications. He (coordinating with our Center of Excellence) began phasing down Haldol and substituting Risperdal. Since she was already on Paxil, the psychiatrist decided to increase Paxil to near the maximum dose to see if a larger dose of Paxil would work before changing to another depression medication. Increasing Paxil helped some but it was clear that Paxil was not the optimum drug for her depression.
I was keeping a daily journal, but it soon became clear that the doctor could not sit and read 45 days of journal comments during our short psychiatrist appointment. I needed an efficient way to consistently communicate her symptoms to the psychiatrist and to our Center of Excellence.
I devised a method of scoring each day on a scale of 1 to 5 (5 being the very best she could be). Then, I would plot her daily score on a graph paper 30-day chart. The first monthly chart line looked like a wild month on the stock market. I would give her a 5 score if she got up and cheerfully went to work, working a good day—even if she had some chorea or memory issues. If she struggled, but still made it to work (but with an attitude and fatigue), I would rate that day a 4. If she refused to get up and go to work but was content at home, did minor chores, I would give her a 3. If she stayed home from work, but would not get out of bed or had periods of crying, had anxiety, would not get dressed or do personal hygiene, etc., I would score her a 2. Along with the score, I would list her medications and dosages so I could refresh the doctor’s mind. When I plotted that information on her chart, it immediately depicted trends in her behavior over time as medications changed. That was invaluable. Note: As your pHD declines, you will need to redefine what a 1, 2 3, 4, or 5 means, because a 5 in early stages is not a 5 in later stages. Hopefully, if your pHD remains stable over long periods, you may not need to continue the chart. I would continue writing in the daily journal.
As my wife improved, I began to wonder how much more I could expect her to improve. When is it no longer reasonable to expect improvement and leave well-enough alone? If you do not ask this question, you might end up accepting far less improvement and quality of life than is possible. Each time we visited the psychiatrist, I asked if he thought that she was doing as well as could possibly be expected. In other words, I wanted the doctor to give me his opinion as to whether it was productive to try to further improve my wife or if she was a good as could be expected. I liked to ask the psychiatrist, ”…if she was your mom or your wife, would you conclude that her current physical/ emotional state is as good as can be expected?” In the early stages, the psychiatrist always answered that “…we could do better.” Then, we would discuss medications to add, delete, or change to improve her quality of life.
What worked for us
Keep a daily journal. What kinds of things might you evaluate and write in your journal each day? You do not need to write a book—often just a paragraph or two. Write about the following types of observations:
- What is their overall behavior?
- Mood—cheerful, apathy, irritation, anger, violence, comments about harm, death, or suicide;
- Swallowing difficulties—types of foods—major choking or minor choking? Can you hear wheezing after the patient drinks or eats?
- What types of foods are problematic?
- Balance issues—falling, stumbling, gait changes;
- Car driving skills, accidents;
- Changes in manual dexterity—dropping things, accidentally cutting themselves;
- Weight gain or loss—how much over what period of time, and why;
- Chorea—when it occurs, how bad, just annoying or causing physical discomfort and falls;
- Sleep habits—12 to 13 hours is not too bad, but more sleep than that needs to be discussed with the doctor; any nightmares?
- Mental concentration;
- Bowel or incontinence issues—frequency, consistency;
- Job issues—what kind of issues;
Know your medications as much as possible. Medications are not “…little pink pills.” Many medications, prescribed for HD, are psychotrophic drugs—serious medications. One neurologist told me that less than 10% of caregivers know what their family member’s medications are for, the dosages, and many don’t even know medication names. For future reference, you should keep nearby a copy of information sheets that come with medications.
What should you know or observe as your family member takes medications?
- Try to have one doctor (preferably doctor with experience dealing with HD issues) prescribe HD-related medications. Let your primary care doctor prescribe meds for non-HD issues.
- Unless there is an emergency reaction, do not terminate medications cold turkey without talking to your doctor. Cold turkey withdrawal can lead to seizures or other responses.
- Ask about possible side effects and which side effects are most common.
- Side effects do not always mean the drug will not work effectively. Sometimes symptoms such as dry mouth or drowsiness are temporary.
- Too small a dose of the RIGHT medication may be almost as ineffective as a less effective medication. Therefore, just because a medication does not seem to work well at first does not mean that the medication is not the best medication in the long run.
- Medications are not perfect. They might result in a tradeoff of side-effects vs. quality of life.
- If you let the warnings on the bottle freak you out or listen to someone’s horror story about a medication, it could hinder your ability to get help.
For each medication:
- Ask what the medication is for and how much time it should take to produce results. For example, depression meds can take a week to several weeks. Some meds work in 20 minutes.
- Does it interact OK with other medications or HD? For example, a medication containing dopamine could cause chorea to increase. You should at least know so you can be prepared to observe those symptoms.
- What is the maximum and lowest dose of this medication?
- What are the long-term side effects of taking this medication?
- Why did you choose this medication over alternative medications? What are the advantages of this particular medication vs. other medications available for this symptom?
- Ask the doctor if he/she thinks your family member has any room for improvement (behavior, chorea, etc.)—is this the best that he/she can possibly be?
Other medication thoughts:
- If a medication has a side-effect (such as drowsiness), it can usually be taken before bedtime. Side effects do not mean that a drug will not work. Sometimes, side effects (such as drowsiness or dry mouth) are temporary.
- In some cases, you can change the time of taking the medication dosages to provide the most benefit. Your doctor should be able to tell you how much time must lapse between dosages. For example, taking medication for chorea at 8 AM and 2 PM might work better than 8 AM and 8 PM (when the pHD goes to bed).
- The caregiver MUST carefully monitor all medications. Caregivers should put medications in a daily medication organizer and watch EVERY day that the meds were taken as instructed. If you see ANY sign of abuse or error, you should personally take responsibility for dispensing only the current day’s medications.
- Be careful to monitor the pHD’s bowel functioning. Many medications can cause constipation and, if ignored, can cause serious bowel issues. I keep a daily bowel movement checklist for my wife, recording each bowel movement size and consistency—large, medium, small, firm, normal, loose.
- Depression is very common in HD patients. Read about depression symptoms so you can recognize depression. If underlying depression is not treated while you take medications for other symptoms, your success will be limited.
What do you look for in a doctor? Ideally, any doctor you see should be familiar with HD. If the pHD has difficult issues, a general practitioner (who has little or no experience with HD) is not acceptable (my opinion). Any doctor that does not regularly follow up with you regarding the effectiveness of medications is not serving your needs (another opinion). He/she should be willing (and able) to answer your questions and try different medications if they are not working. If your pHD has particularly difficult emotional symptoms, you might need a doctor that is more aggressive in trying medications or combinations of medications. My personal experience has been that a psychiatrist will be more aggressive with medications if you have a pHD with severe issues such as violence. In any case, it is fair to ask a neurologist, neuro-psychiatrist, or psychiatrist about their philosophy regarding prescribing medications. Our psychiatrist admits that he may be more aggressive than other doctors, but he said that “…he is more concerned about helping the patient than worrying about medication dosages.” It has served us well and has given my wife her life back. She has been emotionally stable for at least five years.
To a great extent, the doctor’s success with medications depends on the caregiver and patient—your feedback and willingness of the pHD to take the medications. Eventually, a pHD will NOT be able to manage their medications or give the doctor accurate feedback. If you simply live with or tolerate difficult behavior at home and do not tell the doctor, then you are likely to have to live with it and it will not well serve the pHD, caregiver, or family. If your pHD is being treated for depression and the pHD is depressed 10 days of the month and seemingly OK for 20 days—if you do not give the doctor feedback, the doctor is not likely to treat it further—and your lives at home will be more difficult 33% of the month. For me, that was unacceptable. If your HD patient is prone to occasional violence, if you do not tell the doctor and ask for solutions, then he/she is not likely to recommend solutions. An HD patient cannot be expected to rationalize himself/herself through depression or violence. An ultimatum is very unlikely to work. This could make the difference between your being able to keep your family member at home or in a facility—or the difference between you having a very chaotic home life or a relatively peaceful one.
In summary, if I had not done these things, my wife would not have proper medications and I would not be capable of caring for her at home.