As you know, many with Huntington Disease will in time, have trouble swallowing, followed by choking and aspiration. The tongue loses muscle control and can’t initiate the swallow reflex, and even if it can, it becomes very difficult to swallow. As the parent (or caregiver), you become concerned. In so many cases the patient with Huntington's is unable to eat as much, or they hold food in their mouth because they can’t swallow. Inevitably, they begin to lose weight at a rapid pace.
In this article, I am reporting my experience, which can be very different from others. The decisions we made followed much research. Again please understand this is a personal choice that only you or your loved one can make.
There are many adults with HD in later stages, and even young adults with Juvenile Huntington’s Disease that choose to forgo a feeding tube; they also have a DNR (Do Not Resuscitate) order. I completely respect and understand that this is their wish.
There are many different variations, stages, and symptoms of HD and JHD. Many HD patients can no longer communicate their wishes and depend on a POA (Power of Attorney) to grant their wishes, which have been discussed in advance.
In my case, My daughter just turned twenty-five. She has JHD (Juvenile Huntington’s Disease). I have chosen to write this article due to the need for many to understand the pros and, yes, some cons. I will only speak from personal experience.
My daughter Erin has had a hard time eating for the last year. Even with soft foods or puree, sometimes it was fine, but other times she just could not swallow. In result, she would spit out her food. The tongue has to be able to bring the food back to initiate the swallow, and in HD and JHD this does not always happen. Erin would choke on certain foods, especially vitamins and medications. Some of these pills can be too large to swallow. She would tell me she was full, after a few bites of food, although I knew she was not. I took the time to talk to her to find out why she was not eating. Of course, her answer was that she is scared of choking!
It is a horrible feeling for the patient to choke and not being able to breath. As the caregiver, it is frightening to do the Heimlich Maneuver to release what they are choking on, and get air into their airway.
So I had to make adjustments. I thickened her drinks, and I make sure she is upright when eating. Erin has a tendency to slide down in her chair. This happens with many patients with HD and JHD. A recommendation I have is to put pillows behind them to keep them upright while eating. In my opinion, if coughing starts, raise their arms, and encourage coughing. I changed her diet to a soft diet: scrambled eggs, mashed potatoes, mashed food, milkshakes, ensure, applesauce, and ice cream. Some tips: Be careful of Peas, as skins can make them choke. Be careful of mixing liquids with food, such as cereal. Always eat and then drink!
The biggest concern is the high caloric intake needed for HD and JHD patients, to sustain their weight. This is especially important for patients who have chorea. As the progression of the disease happens, it is very difficult for the patient to eat. This directly affects the amount of calories they intake, and weight loss begins.
If your loved one loses weight, you need to think about sickness. The patient's immune system can be compromised and they may not be able to fight off colds and flu. The more choking that occurs, the more likely that aspiration and pneumonia will follow. In many cases individuals that suffer from JHD and HD die from pneumonia. As the patient loses weight they can also develop pressure sores that are very hard to heal.
Many in the community know my daughter Erin. She was having all of these problems and it was becoming increasingly concerning for me and the entire family. I booked a swallow study for her. A swallow study can take place at a hospital and they provided different types of food for the patient. The patient is placed in front of an x-ray machine which shows their swallow reflex. Erin’s appointment was scheduled for October 16, 2017, with a follow up with the doctor the next Wednesday. This follow up appointment would have given us information about her ability to swallow, and to learn everything I could about a possible G-tube.
In a blink of an eye, everything changed. Erin had headaches everyday and would take an Advil. On Sunday October 15, 2017, she choked on the Advil. We called 911 as the Heimlich was not working to clear her airway. We were able to get enough air into her lungs before the ambulance arrived so she stayed conscious. They would not allow her to have anything by mouth until the swallow study could be done, which was the next morning. My concern was that she was unable to take her seizure meds, and I knew she could have a seizure without them. She hadn't had a seizure in a year.
It took seven hours for the Advil to dissolve in her throat, and while it was dissolving, it burned her throat. The hospital gave her something for pain and kept her until a swallow study could be done the next morning. The following day came, and the test was done. There were concerns. I was advised that a G-tube (gastrostomy tube) should be placed to give her the quality of life she needs. The doctor said she could still have thickened drinks, and puree food by mouth, but it could be a risk.
I have always tried to be proactive, just because this is happening to her, she can still be well and fight for many more years. Erin has many abilities and her mind is still sharp! She can text. She is smart. She knows what is going on around her, so why would we not decide on a feeding tube, which would provide her the vital nutrients she needs? This would keep her well, avoid choking, aspiration, and pneumonia. I asked Erin what she wanted. She told me she wanted to live and she did not want to choke anymore. This procedure will allow me to provide her with a diet that is packed full of nutrients, and help her gain weight. Why would I not do this for my daughter?
Erin had the G-tube surgery on the Wednesday, October 17, 2017. Now, I had to learn how to do the feeds, what was required, and how to take care of her. I spoke with a dietician. They started Erin off slowly, to see what she could tolerate. The rate of feed and volume is started slowly. When they know the patient is tolerating the feeds, they slowly increase the intake.
By the time I brought Erin home from the hospital, I was well versed and trained regarding the process. Erin had several follow up appointments from nurses, occupational therapists, and physical therapists. They came to our home to check to see that everything is going well. They also answered any questions we had.
Below is the WeHaveAVoice radio show where I talked about Erin's experience while at the hospital.
The patient is usually given a few options - times of feeding based on what works for the patient, and for everyone in the home. In some cases, patients require a feed continually - all night, at a slow rate. This is what they can handle. Others can have feeds three times a day at a higher rate. This is based on the patient and the amount of time the food is pumped into the stomach. The doctor calculates the patient's body mass index, and amount of calories they require within a day, to maintain an optimum healthy weight.
When the patient arrives home, they are given a pump that delivers the feed into the tube. You set the volume to be given as well as the rate it is to be delivered. An IV pole hangs the bag for the feed, and the pump attaches to the IV. The pump has to be at least six inches lower than the feeding bag. The feeds start slowly and based on the individual patient, you work your way up to a point that they can tolerate the amount of feed which will maintain a healthy weight. The patient must be in an upright position (at least a 45 degree angle) when feeding, to prevent aspiration. In cases where the patient needs to be fed throughout the night, they should maintain a 30 degree angle. When the feeding is complete, the patient should stay at that the specific angle, preferably for an hour. This eliminates that chance of vomiting and aspiration.
I would like to quote Kevin Jess on how his wife is fed. She is in end stages, but continues to do very well since the G-tube was inserted.
"Sheila has a high risk of aspiration and could not tolerate three feeds per day, and if she were elevated more for the entire day and night it would not be good for sleep, whereas she is very comfortable at the 30 degree angle. Hiccups have not been a problem. Also, if we raise her any further she tends to slump to the side and her head becomes jammed between the mattress and the rail of the hospital bed. The things you learn over a one year period of trial and error are something else. We now appear to have achieved a perfect combination and it is obvious from her coloring as well as her disposition."
Erin is on three feeds per day: morning, noon and night. Her feeds last about an hour and a half. She is upright all day, except morning feeds, where she is elevated to a 45 degree angle.
Many people come and go in our house, and I have several friends and care providers that feel bad if they eat in front of Erin. Well, this is a myth as I once felt that way myself. But now that I live with this, I can truly explain in a way I hope you understand.
I do a lot of baking and cooking, especially this time of year. Through the holidays I bake for a lot of people. Yes, Erin loved her sweets and she loves food. But, not once has this bothered her since the feeding tube procedure. She loves to watch me cook and enjoys family and friends who come by to enjoy the treats. The reason is that Erin feels full after her feeds. When you are full, like after a big turkey dinner, many cannot eat dessert because they are too full. Simply, when you are full, you do not want anything.
Quote from Annette Delbosque – JHD MOM and Caregiver to her Daughter.
"My 11 year old daughter was literally starving to death for over 2 years. She just did not want to eat whether it was because she was scared, whether it was too difficult or she just had no appetite, I don't know, but she was starving. At 11, her heaviest weight was 58 lbs. Prior to the G-tube placement, she was wearing size 7-8 clothes, 6 months later she is wearing size 10-12. She is able to handle being ill and bounces back quite quickly. We were very fortunate that although Phoebe was frail she was still quite strong physically and was able to handle the surgery. It was a textbook placement. She was so very relieved that she no longer had to eat and especially no longer had to take her medication by mouth. The pressure of trying to get her to eat and the constant worrying about her was finally relieved. It's now replaced with spending a lot of time relaxing during feeds, scheduling everything around her feeds, but overall I am so very pleased with Phoebe having the G-tube placement."
With the feed, you also do a pre and post flush with water, as well as after medication. This gives the patient the water they require as well, so they are not thirsty. Erin does have sips of water periodically throughout the day, but not very often. She is getting everything she needs through her tube. She no longer stresses about her medications or vitamins, as they are crushed and given through the G-tube. She no longer complains of headaches, which she did every day.
It is important that the area of insertion (of the tube) is clean and dry. Once a year the tube is removed and changed. As with medications, things build up in the tube which could get blocked over time. So doctors recommend changing the tube once a year. Putting in a new tube is very easy. Erin was concerned about this. The procedure consists of going to the hospital. They give a local freezing, and deflate the balloon that holds the tube in place. They simply replace it with a new one. It is a very quick, and noninvasive procedure.
It is also important to understand that feeding tubes can be reversed. They can be taken out and the patient can continue to eat as normal if their condition improves. Feeding tubes do not have to be permanent for every patient. I believe feeding tubes are truly amazing, especially if they can provide quality of life, and keep the patient healthy.
It was Erin’s choice to accept the feeding tube, and I supported her decision. It is always a choice, and thankfully Erin was able to verbally tell me this is what she wanted. It is all about hope and belief in what tomorrow will bring. For some, it is about giving up and going peacefully. For Erin, who is only twenty five, I hope she will continue to have that hope within her. There are people young and old, living with HD, who choose to hope and do whatever it takes to live. I believe your life is worth it! However, it is always a choice.
Below is a video of how I prepare Erin's G-tube bag and machine.
I believe a cure will come in 2018, and my choice is to keep Erin healthy and happy. She no longer worries about taking meds, nor does she have nightmares about choking. She has had her G-tube for a little over a month and has been gaining weight. She feels great, has no pain, or any complications from this. She is only getting better!
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