My hope is to share our experience so that others may benefit. You may not want to read this and that is completely understandable. But for those who are in the midst of this and are looking for info, we hope and pray they can find something helpful here.
Macayla's autonomic nervous system was being impacted by the disease. That is the part of the nervous system that controls all the automatic functions like breathing, bowels, heart, etc. This indicated that the disease was deteriorating the brain stem. The brain stem is quite resilient compared to the other parts of the brain. But even it cannot withstand Battens. We did not determine this with an MRI but through our genetics doctor who sized up Macayla's symptoms and put it together. This caused Macayla to have sporadic vomiting over the last couple of months as her stomach randomly contracted. Her bladder functioned but had erratic patterns and had to be cathed frequently. This symptom actually showed up over a year ago. At times it was worse than others. We also noticed over the last 6-8 months that her face would go from being red and flushed to pale without any fever or discernible reason. We learned that the autonomic nervous system also controlled how much the blood vessels dilated or constricted. This meant that the blood vessels would randomly open more in her face and make it appear red and flush. At other times they would constrict and there would be less blood flow in her face and she appeared very pale. So, over the last couple of months, we were able to recognize symptoms not previously seen. When our genetics doctor put all of it together, he guessed it was the autonomic system and brain stem being impacted. He was right.
To the tough part. Macayla's experience is, of course, not necessarily what others will endure. But there may be some common symptoms. We are thankful for those who helped us evaluate them and it helped us make Macayla more comfortable. The last week was up and down. Kids who reach the end of life really keep you guessing. Adults can be more steady according to our friends at hospice.
Macayla had a week of frequent vomiting. Then I noticed an unusual breathing pattern on Friday night. She took two quick, deep breaths, followed by a shorter breath and then she would not breathe for about 8-10 seconds. She did not seem in distress. We learned this pattern is called Cheyne-Stokes (pronounced "chain-stokes"). Saturday morning she ran a fever of about 102 and her breathing was a normal pattern, but raspy. We took her in for a chest xray and they said it showed no pneumonia. Sunday night she began vomiting profusely and it was difficult to get her to recover. She had four bouts of vomiting through the night. Between those times, she was asleep, but her breathing was much more intense and started the Cheyne-Stokes pattern again. She seemed to be straining to breathe more than usual and the breaths were raspy. Suctioning did not help.
Her fever returned and got up to 103 and then went back down again without any medication. This indicated her hypothalamus in the brain stem was not functioning properly as it controls body temp. After a touch-and-go all nighter, Macayla recovered and slept most of the next day and night. On Tuesday afternoon, I finally was able to get some responses from her. Her last smile was on Tuesday afternoon. She fell asleep that evening and remained asleep the rest of the time.
The last three days of life we saw the following symptoms. I thought a list might make it easier for you. If you have any questions or want more detail, email or comment.
- Macayla's fever went up and down on its own without medicine over the course of a couple of hours.
- She had Cheyne-Stokes breathing patterns. She may have two or three breaths followed by a apnea spell that would last 10 seconds and as long as a minute (especially toward the end).
- Circulation centralized and blood flow to the limbs slowed. We had a hard time finding a pulse in her feet and legs and even the pulse in her wrist was weak at times.
- Her lungs got "wet" and began to fill with fluids. She sounded raspy on every breath and it got worse over time. She would cough some at first, but most of the time she was unable. In the hospital, they can stick a tube down into the lungs and keep them clearer, but at some point we would face the decision to remove the tube and in Macayla's case she would just start the process again. Intubation (as it is called) would not change what was happening to Macayla or the ultimate outcome for her. For others, it might buy some time if needed. We chose for Macayla to be at home and not attached to machines in the end. For our situation, the machines would not change anything. Each family must decide for themselves what is best.
- Her heart rate stayed elevated. She normally stays between 70-100 bpm, but then she stayed up between 130 - 170 bpm.
- Her kidneys also began slowing down earlier in the week. We had to cath her most of the week and during those last three days, we barely got anything with the catheter. What we did get was very dark and concentrated in smell.
- Her skin also became mottled on the bottom sides of her arms and legs first. This was difficult to notice for me, but the nurses noticed it right away. It looks like splotchy gray and blue discoloration on the skin. It's where the blood seems to not move as much and fluids are settling.
- The intestines and stomach stopped. This we were not prepared for. She started drastically leaking around her feeding tube. Everything just sat and since she was unable to urinate, all of her fluids were finding their way out around the feeding tube. We had to remove the feeding tube and use the suction machine to quickly remove the fluids gushing from her stomach. This helped, but it continued to build up and even had stool in it eventually. Stool will start to back up and can cause a patient to start vomiting stool. We removed the feeding tube multiple times and irrigated her stomach with water and the suction machine. It prevented her from vomiting more. The leaking was so bad we actually wrapped her torso with a diaper to help absorb it.
- Because her stomach did not work, her medicines were not being absorbed. She was not getting her seizure meds and she had multiple grand mal seizures during the last three days. We had started morphine and Ativan to help with nausea and spasms and pain. We had to go to morphine injections and an Ativan cream that absorbed through the skin. We also started a pain patch. There are multiple medications that can be compounded into cream that is absorbed through the skin. Even suppositories may loose their effectiveness if circulation slows down in the lower regions of the body. So, compounded creams can be helpful and applied to the wrists or chest.
- She had a couple of spells where she stopped breathing for several minutes. In fact, the first time she did this it lasted four minutes and her heart stopped beating. We thought that was it and then she started breathing again. As her apnea spells got longer, her heart would slow down, get irregular, and then she would start breathing and the heart rate would jump back up.
- The last few hours of life, her heart rate finally slowed down and stayed close to 100 bpm. She slowed her breathing down to 4 to 6 breaths per minute. Normal is 16-20.
- The nurses told us that often people will stop breathing, but the heart will keep going for several more minutes (as much as 10). But Macayla actually did the opposite. Her heart stopped and she took a few more breaths before it was over.
- One other thing of note. Macayla seemed a bit more responsive on the last day. She opened her eyes with stimulus. It may have been one of the ways she said goodbye. We also tried to reassure her the whole time that she would be o.k. and that we would be o.k. We told her that she could go home anytime she was ready. Dying people need to know this and need reassurance that those left behind are ready. Like with anything our children would do, we wanted to be supportive, but it was the hardest thing to support.
Again, I only wanted to share this because it's hard to find info out there. Our experience was not exactly like others, but from what we gather from hospice and others, many of these symptoms are seen during the end of life.
