DNR Stands for "Do Not Resuscitate" not for "Do Not Relay"

Recently, I was interviewed by a local news station about school policies concerning children with DNR's. Macayla had a DNR while she was going to school. From my understanding at the time of interview, the school district wanted a policy in place that did not allow them to enforce or enact the DNR during a crisis requiring life-saving measures. In other words, they would perform life saving measures until EMS or other medical professional arrived. I later learned that the real story was the school district wanted a policy that they were not responsible if the DNR was not passed on to medical personnel such as EMS. This is another issue entirely.

Sibling's Grief

Jacob is seven and Macayla's little brother. She died on May 22 and it is now August 10th. He has not cried or had any emotional outburst about Macayla yet. We have been concerned that he may be repressing his emotions. But a hospice counselor who has been working with Jacob gave us some encouragement and some signs to look for:

If Jacob were to react strongly and negatively every time Macayla was mentioned (like run out of the room screaming) then we might have a problem.

If Jacob just shut down and would not get out of bed or have difficulty functioning for no apparent reason, we might have a problem.

If Jacob simply acted as though he was not hearing us when we speak about Macayla, then we might have a problem. (At times, I think he has mildly displayed this, but nothing troubling yet. Usually, he speaks very matter-of-fact about Macayla)

Further, we have to be on the look out for regression in skills and behavior. He may be unable to perform tasks that were non-issues before Macayla passed. He may not perform well academically. He may start having obedience problems at home and school.

Fortunately, we have witnessed none of this to speak of yet. School starts in a week for him. But the counselor reminded us that he has been preparing for this time just like us. He has grieved along the way also. We cannot stress enough to parents how important it is to allow a counselor into your life and your child's life when they are facing the loss of a loved one, especially a sibling. We, of course, recommend a counselor who has a Christ-centered worldview, but not everyone shares that stance. Seeking counsel is wise and healthy because there are just somethings we are not meant to do alone. Hospice organizations, such as the one that has served us, often have counselors on staff and some specialize in children.

We have also learned to be straightforward and honest with Jacob's questions. Often adults try to shield the children when they ask about death, dying, caskets, etc. Children seem to do better when we are up front about it all. Granted, they don't necessarily need every detail of the death process, but speaking clearly and using consistent words will help. We typically use the word "died" instead of "passed away" for instance. We have explained the disease to him and the death process when it was happening. We also spoke about what happens after death and the funeral, casket, burial, etc. Be clear, simple and open. That is the advice we have been given.

Hospice - Symptoms of Death

This is not what we ever wanted to post. Our daughter, Macayla, finished her fight with Battens and died on May 22, 2010, her 9th birthday. She has been steadily digressing since the fall. But over the last two weeks, we found it difficult to get any information on signs and symptoms for people who had reached the end of life. Fortunately, for us, we had several people around the last few days who had nursing experience with dying patients. They walked us through and prepared us for some of the coming symptoms and bodily responses we might see. We also had hospice nurses who came in from time to time to give guidance and offer support.

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.

Grand Mal Seizures - Need Help and Information

Macayla had three, maybe four, grand mal seizures recently. It was the first she has ever had. They all happened over a two hour period and we gave her Diastat (valium medication given rectally) and it stopped it. Macayla stopped breathing and turned gray. It was scary. But the Diastat worked exactly the way it should. In less than three minutes she was asleep and the seizures stopped. We have no experience with grand mals and Macayla is immobile so she doesn't seem to jerk as violently as I expected her too. Her eyes just started rolling in circles and her head slowly and rhythmically shook back and forth in the "no" gesture. Her chin moved up and down rhythmically and she stopped breathing. Her arms and legs twitched slightly. I want to learn more from those families that deal with grand mals all the time. I feel very unprepared for these types of seizures and it may be something you cannot prepare for. If anyone has anything to offer, please let us know. Thank you.

Doctors prepared us for the day that Macayla may have big seizures and die or that her airway would become too floppy to remain open and she would go into respiratory arrest. We have talked a lot about that, but nothing prepares you for the real thing. Macayla's grand mals could have been much more severe, but just the 45 seconds she stopped breathing each time was tough enough to watch. We have a DNR (Do Not Resuscitate order) on Macayla because our decision was not to repeatedly intubate and extubate when the end comes because once you start you still have to decide when to stop. Intubating does not change the progression of the disease or its outcome, so we decided we did not want to put Macayla through that repeatedly. However, watching her not being able to breathe was the hardest thing I've had to do and your first response is to call 911. End of life decisions are tough and following through with those decisions are even tougher, now matter which way a family decides to go. 

Funeral

The end of life decisions are difficult on every level. It is difficult to even think about planning for a loved one's death. As a parent, it is paralyzing to consider planning the funeral of my child. We have started the process, nonetheless. It is easy to avoid this process while you're in the midst of caring for your child or loved one, but at some point it is a process we must face. I would rather not face all of the decisions and planning the day our daughter passes. It will be a most difficult day as it is and the last thing I want to have to deal with is where, how, who and what will be done at her funeral during the 24-48 hours after her death. So, we decided to begin preplanning and that process started yesterday. As we gain more information that might be helpful, we will share it here. This blog tends to be more informational and technical in nature whereas our other blog share a bit more than that. Hopefully both will be helpful and bit more holistic. As parents we cannot live in denial of the prognosis of our daughter. It will not help her if we do not acknowledge her needs; the needs of today and the needs to come. We see her funeral as another step in the journey she is on. It is not the end. It is the next step. 

We actually experienced relief to some level to start the process. We also felt relief as we discovered that funerals do not have to be as expensive as we thought. There are many options to lower costs, depending on one's viewpoint of life and funerals. We will share all we can and we invite others to share as well.