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.