Sign Language Help

Sign language is not simply for the deaf or mute. Sometimes it augments communication for those whose speech is limited.

We found that Macayla could use some signs to help communicate for a short time. She learned a version of the sign for "more" and that was helpful, especially when she wanted to play in the water. She would often sign "more" to let us know she did not wish to stop.

One helpful resource is ASLPro.com. It is free to use their website and they provide video demonstrations of the signs. They do accept donations to help keep this wonderful resource going.

Transferring and Positioning - Maxi Slide Sheets

My wife is a nurse and has been training with a product called Maxi Slide Sheets. They are a really neat product for transferring immobile patients from one bed to another or for just moving a patient up in the bed for repositioning. Here is their PDF showing the product in use, however the training video does it more justice.


For us, this would have been great on several occasions as we needed to reposition Macayla for bath (which required that we spin her completely around with her head at the foot of the bed) or to just move her up closer to the head of the bed. Macayla grew to be a very tall and was heavy to move. She was dead weight much of the time and it was not always comfortable for her when we moved her. These sheets would have been great! Unfortunately, I cannot seem to find any American suppliers carrying them on their websites. Several European companies seem to though. Obviously, it is for sale here for hospitals but not the home yet.

Research into Lysosomal Storage Disorders

This article was shared and it sounds as if this discovery my offer some direction for future medicines and therapy for diseases such as Battens. The information in the article is credited to Baylor College of Medicine. Click here to read the article.

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.

Augmentative Communication for iPad & iPhone

We really wished we had learned more about aug com (augmentative communication) much sooner with Macayla. It would have helped her put some skills in place earlier while she still could. However, it did not make sense for us to have the very expensive computerized aug com systems with touch screens that was out at the time. Amazingly, Apple's iPad and iPhone have revolutionized the availability of this technology at a fraction of the cost.

iPads have larger and more accessible screens, but iPhones or iPod Touch work for those with the fine motor skills to manipulate items on their screens. (An iPod Touch is just like the iPhone minus the phone capability.) They all have built in cameras. The iPhone and iPad with cellular connectivity have the benefit of not needing a wifi network to access the internet. In other words, you can access the internet anywhere. The iPod needs to be connected to a network wirelessly. For the non-techies out there, this may sound a bit overwhelming, but these devices were designed by Apple to be intuitive and to think like we do. People who have never spent much time on computers figure these things out very quickly. Plus, when they are not in use for aug com, they have all the functions of a laptop. Email, web, word processing, photos, movies, calendar, music, etc.

iPad for Special Needs

A friend of ours recently got an iPad as a surprise from her husband. I was jealous. However, she posted some photos of her son, Daniel, playing with it. Daniel was born with an encephalocele and is now 12 years old. He has been interacting quite well on the iPad and there seems to be several apps for the iPad geared toward special-needs.

I would recommend checking it out by going to Apple and downloading iTunes for free (if you don't have it already). Once in iTunes, you can search through the AppStore and see all the great options. I found Proloquo2Go and Tap To Talk in the augmentative communication realm. Usually, you can find these apps under the category of "education." I have included a couple of screenshots from iTunes.

The iPad starts at $500, but it has so many other uses and so much augmentative communication devices can cost outrageous amounts. Plus most of the apps are free or just a few dollars if they cost anything. I think it would be worth checking out.

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.

Van Update

The van sold! Thanks to a new friend it sold via word-of-mouth and we are thankful it helped their family and ours.

Van For Sale!

We have our handicap van for sale! Hopefully, our price will be fair and helpful to another family and helpful for ours as well. (It's amazing what others are charging for handicap vans of similar age and mileage.)

This is a 2000 Dodge Ram Van 1500. -- 113000 miles -- It has a Zephyr conversion package (high top and running boards. The higher top really was helpful when we needed to work on Macayla and stand next to her) -- Braun wheelchair lift and additional seating provided by a jump seat that folds out of the way (see pictures below) -- Power windows, Power locks, CD/Cassette, Cruise, Tilt, Rear Air, Alloy wheels, hitch receiver, power seat, wheelchair anchor system, and more!

For those who are local to our area, the van can be seen at Hayes Auto Sales on Clemson Blvd. in Anderson. Next to or really behind Office Depot. They will be glad to show you the van and let you drive it. They are selling it for us and that means you can do a trade in if necessary.

Their hours are

M-F 9 a.m. - 6:30 p.m.

Sat. 9 a.m. - 4:30 p.m.

Sunday closed.

They are located @ 3415 Clemson Blvd. Anderson, SC

864-226-2472

Asking $10, 500

This control box is connected by an expanding cord. It can be used by the person on the lift or an assistant to operate the lift while standing outside. It moves the lift up and down and folds and unfolds the lift.

There are lights on both sides of the lift and they are very bright and helpful at night.

This switch is out where the person on the wheelchair can operate it once they roll up on the lift. (If they are able. The whole system can be operated by the special friend or their assistant.)

For the lift to clear on the way down, the rear bumper was removed. However, we still have said bumper incase someone wants to convert his back to a standard van.

These help keep the doors open while operating the lift.

There are controls on the inside of the van that control the lift. They are the yellow, orange and red switches on the right side of the lift. See below.

We opted to install the lift in the rear so that our daughter and son could sit side by side. It also helped when she was not doing well and we had to take her somewhere. One of us could drive while the other or a nurse could sit next to her. It also helped with parking. It never fails that a person will park their little compact in the last handicap van spot. With the lift in the rear, we could park in normal spaces and still unload.

These are the Sure-Lok hooks for the wheelchair. There are four of them and the red switch releases it so you can pull more of the blue strap out and hook to the chair. The knob on the side ratchets the strap tighter. Once all four points are hooked on, the chair does not move. If you forget to tighten one, it will self-adjust as you ride down the road and tighten some of the way for you. There is also a seatbelt system for the person in the wheelchair if it is needed.

We had a jump seat installed and it has come in handy. Usually it stays folded up like this and out of the way. Once we pushed our daughter into place, we could lower this down and provide extra seating...

The seat folds down like this, then...

...the back folds up and two people can sit here! It has seat belts also.

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.

Alternating Air Mattress Helps Prevent Skin Breakdown

Anyone who remains in the same position in the bed runs a risk of skin breakdown and bedsores. Hospitals often have special mattresses that automatically alternate pressure zones to reduce the likelihood of these problems.

Recently, we started noticing more and more issues with skin breakdown and our daughter's OT suggested an alternating air mattress. We discovered there are mattresses that can replace an existing mattress or there are covers that set on top of an existing mattress. Both work on the same principle. They attach to a motorized pump that constantly shifts air around in the different cells in the mattress. This means pressure is moved around and you can remain in the same position longer than on a regular mattress. We rent ours and have found it to be helpful. It is not as hot as memory foam or lamb's wool and when it comes to bedsores, heat and moisture are the enemies. Lamb's wool can help reduce sheering, but these mattresses help with pressure. It is certainly worth looking into if skin breakdown becomes an issue. Our daughter is very comfortable on hers.

Video Monitor a Huge Help!

For the last six months, we have been using a video monitor to keep tabs on our precious girl. It was one we got on sale and is made by "Summer" and available at Target and other places. The great features we love is that it is a color camera and monitor, picks up on sound well and has the ability to see in the dark. It also has a built in thermometer and the monitor displays the temp in our daughter's room.

(I enhanced the picture in the monitor here for demonstration purposes.) We found, however, that we needed to have flexibility in where we mounted the camera so that as we changed our daughter's position, we could move the camera to stay in view of her face. That's the best way for us to tell if she was having a seizure. So, I utilized a $14 clamp-mounted desk lamp (sometimes called an architect's lamp) and modified it to hold the camera. The lamp arm clamps to our daughter's bed and gives us an adjustable arm to maneuver the camera position.

I had to remove the light socket and switch, shade and cord from the lamp so as to leave me just the arm and springs. Then using a scrap piece of plywood, I made a platform for the camera to mount on. It has a built in slot for a screw head on the bottom so I simply mounted a screw into the plywood and slid the camera base on. However, there is only one slot (designed for the base to hang on a wall) so I used a rubber band around the other side to keep the camera base from moving. The camera itself swivels and tilts on its base.

With the base secured to the lamp arm, we have a very positional camera. The monitor it transmits to is rechargeable and has a decent range on it. I have only seen trouble with the signal if I get to the complete opposite end of the house (1600 sqft home) or if it gets too close to my wireless router. Other than that, it has been great.

You can even add an extra camera and switch back and forth on the monitor. It has been worth the money for sure.