Special Needs Bike Trailer

Recently, we went to the beach and wanted to take Macayla on a bike ride, but the only way that would be possible is with a special needs bike trailer. They cost thousands of dollars and we could not spend that. Fortunately, Macayla's wheelchair is more like a stroller where the seat comes off of the base and is held on by two rails that it slides onto. I was able to get two additional rails from Palmetto Seating who supplied the chair for us. I picked up a regular bike trailer meant for carrying small kids with a capacity of 100 lbs. I took all of the fabric off of it and with some scrap lumber I had around the house, I made a platform that Macayla's seat could latch onto. I spent $85 on the bike trailer, $10 on a trifold lounge chair (I used the middle section of it for the platform) and another $15 in hardware. Her chair fit right on it and it worked great. It was not as hard to pull as I thought it might be. She enjoyed the ride and we have a piece of equipment that we can keep for just over $100 that would have cost us thousands otherwise. I think I can also make a platform for her seating system from her Zippie wheelchair that we keep as back-up. The great thing about the kid carrier trailers is that they have a ready made attachment to convert them into a stroller. So when we got somewhere, the trailer quickly disconnected from the bike and we simply attached a front dolly wheel to the trailer tongue. The trailer was made by In-Step which is a Schwinn product. This has been a pretty good success so far and with a few modifications, it could be even better.

Wheelchair Lift

We have a wheelchair lift and the process of shopping and deciding on a lift can be frustrating. We spoke with several providers and many were difficult to deal with. We finally went with Classic Coach in Spartanburg, SC because Joe there was great to work with and was a great listener. He is very familiar with different equipment options and is a good educator. When searching for a provider, it is good to find someone who has a hands-on familiarity with the different equipment and what is possible in different vehicle types.

In our case we found a 2000 Dodge Ram conversion van with the high roof. We wanted the ability to have Macayla sit in the middle section of the van behind the driver seat and still leave room to sit in the captains chair in the middle section on the passenger side. This meant we had to have a rear-entry lift. We have found this to be very helpful in parking as handicap parking is usually hard to find. With the rear entry we can park in a normal space and still unload. The drawback is packing for trips. The only open space in the van for packing is the rear section, but that impedes loading and unloading Macayla on long trips. We removed the original bench seat in the rear of the van, but Joe at Classic Coach installed a special, two-person fold-away seat in the rear section. It gives extra seating when necessary, but folds out of the way the rest of the time. 

We have a Braun Millennium Series lift that has a solid platform and folds up into the back door of the van (they are barn-door style). This is a fairly simple lift and has worked well. We have only had one problem with it when a hydraulic hose leaked. Other than that it has held up well for the last two years. A friend of ours has a side-entry lift that folds out of the way of the door after it folds up into position. It is nice because others can still access the van through the side door when the lift is stowed. Our lift blocks the entire rear door in the stowed position. However, our friend's lift is a bit more complicated and has had a few frustrating glitches with all of its safety features. They also must park somewhere that has enough space on the side of the van for loading and unloading. There are obvious trade-offs for different lifts and designs, but when you have a good provider who can help educate and brainstorm through the decision-making process. The Classic Coach website has some great pictures of different options and it has links to three major lift companies that are worth checking out. 

Apnea Monitor

Since Macayla started having grand mal seizures that stopped her breathing, we started using an apnea monitor. It measures heart and respiratory rates through two (sometimes three) leads that attach to Macayla's torso. If her rates go too high or low, it will alarm. So far, we have found that most of the alarms are false, but they are not too frequent as long as we hook her up correctly. The most frequent false alarm has been a "loose connection" when she manages to rub one of the leads off. There is a soft, velcro belt that goes around her chest where the leads are and that helps keep them in place. Macayla is still squirmy enough to rub the leads off. It has alarmed a couple of times for apnea and low heart rate, but nothing was wrong. Her heart rate drops during deep sleep sometimes and this morning a yawn set off the apnea alarm. 

Another option we considered was a pulse/ox monitor where the lead is attached to a finger, toe or even around the foot. It measures their pulse and oxygen saturation. If either gets out of range, it will alarm. Macayla moves her feet and hands too much for one of those to work at night, and we question if we could get a good reading on her considering the poor circulation in her hands and feet. Overall, we have been pleased and even relieved by the apnea monitor. It allows us to leave Macayla in her room knowing that if a seizure starts we will be aware of it. So far it has provided peace of mind and only a few false alarms.

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. 

Decompression

We recently got a phone call from a family who was struggling with decompressing their child through the feeding tube. I would imagine that most families find little tricks that work best for their child, but in hopes it will help more people, I can share what works for us. We have found that most pediatric surgery offices do little to train their patients and their families with their new feeding tubes. So, with Macayla, we have worked with a Genie, a Bard Button, and now a Mic-Key button. Each has its own advantages. We love the Genie's design, but changing it is painful. The Bard Button was a leaking disaster for us but it is very flush to the skin. The Mic-Key is great because it can be changed out at home with little discomfort and the connectors lock on. There is also an extra port on the connector to push meds through without having to disconnect the feeding pump. It does stick up above the skin more than a Genie or Bard Button.

As far as decompression, we hook a longer connector to her (24 inch) with a 60 cc slip-tip syringe connected to it without its plunger. We lean her back in her chair or lay her back in the bed near the edge. Then we hold the syringe down below her to let gravity take over and draw everything out of the stomach. I do tilt the syringe over enough to help flow but not so much that fluids would spill out of it. Sometimes it helps to have a cup to pour excess in if necessary. However, we have always been advised by our doctors and nutritionist to never dump the fluids that come out because they are much needed electrolytes. We put all the fluids back in. The only exception to this is if we get a bunch of blood out. Typically, if the syringe is getting full and there seems to be more air, I will sit Macayla up and then let the fluid run back in. Then I will tilt her back again and once again lower the syringe down below her to decompress again. Letting the fluids run back in while she is sitting up seems to help reposition the air closer to the tube. We sometimes push on Macayla's stomach gently to assist the air out. We point the open end of the syringe away from us as Macayla has sneezed before during decompression and it turns the syringe into a mini-canon that fires stomach contents! Yeah, it's gross. We have found that Macayla needs to be decompressed before every feeding and sometimes between feedings.

Wheelchair

The only drawback we have over Macayla's New Bug (see the previous post) wheelchair is cleaning it. Recently, her feeding tube connector came undone from the feedbag port and it leaked all in her chair. It saturated the cushions and fabric to the point that it was dripping. The manual that came with the chair is not very clear about how to wash the cushions so we washed them in the machine on gentle and then tried drying them with air and no heat in the dryer. That did not work. It took most of the day to get the cushions dry because they held so much water. To make matters worse, there was still a faint oder of the spill in the cushions. We will experiment with other ways to handle this, but for now we basically must allow for an entire day for the chair to be out of commission while the pads dry. We are thankful we have a backup chair to put her in.

Cleaning Tubes

On the Parent-2-Parent forum, there are tons of helpful hints from parents on feeding tube stuff. One of the things we struggle with is keeping the feeding tube connectors clean so that they last. By feeding tube connector I mean the tube you connect to the feeding tube button. They are usually 12 or 24 inches long and are designed for the specific button you have (Mic-Key, Genie, Wizard, Bard Button, etc.) One of the suggestions on the forum was to pour some liquid soap in the connector tube and then use the back of a knife and your thumb to pinch the tube. Then you pull the tube through, running it between the knife and thumb much like the way one would curl ribbons on a present. This works but I always worry I may pull the end of the tube off. I have found that I can simply pour the soap in the tube and let it slowly run its course through the tube. Then I turn the tube over and let it drain the other way and repeat it and rinse it out well. It seems to get out all the gunk. 

Foam Pillow

We were exposed to a product called Versa Form and it is a great little invention. It is used in a few different applications, but we have been trying out the pillow. The pillow is full of foam pellets and it has a valve on the end that you attach a small hand pump to. Then you form the pillow into whatever shape you want and hold it there while you pump the air out of the pillow. Once the air is out, it stays in the shape you put it in. It is great for forming around Macayla's legs at night to keep them in a position that helps her hips. Unfortunately, she doesn't like it and has trouble sleeping with it. It can feel rigid and I think it makes her feel strange. But it certainly has many helpful uses and they make other products using the same concept. You can make a custom fit form for abduction or support and change it as needed. It hasn't been the best match for Macayla, but it is certainly a product worth checking out.

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.

Cortical Vision Impairment - CVI

Our speech therapist suggested that Macayla could be possibly suffering from Cortical Vision Impairment. We cannot actually diagnose this in Macayla due to her inability to communicate, but it certainly makes sense. CVI is caused by damage to the visual pathways in the brain and not to the eye itself. Macayla's occipital lobe and pathways are significantly deteriorated while her eyes are fairly healthy looking. Macayla seems to pick up on moving objects better than stationary and she tends to gaze at light sources. She responds to certain colors better than others such as yellow and green. From what I understand, green may be an unusual color to respond to in CVI but it may be due to the fact that she was not born with CVI but developed it after she knew colors. Typically, CVI sufferers respond to black, yellow and red. Our therapist suggested that we put simple objects on a black field so that it stands out better. Macayla responds to it and tends to focus better. I also noticed that she zeros in on white letters on a black screen such as the "This movie has been formatted to fit your screen" message at the beginning of some movies. She's not reading of course, but the pattern catches her eye. I wish we had known more about this sooner so that we could have looked for indicators and stimulated Macayla's vision more effectively.

Diapers

We have found that changing diapers when we are out and about can be challenging with a 52 inch tall child. She doesn't exactly fit on a changing table in a public restroom. One advantage to having a wheelchair that reclines is it can give you a good place to change diapers. My wife figured out that it also helps if we double up Macayla's diapers. That way when we are out, if she has a wet or dirty diaper, we simply take off the inner (dirty) diaper and simply pull the outer (clean) diaper up in its place. It saves on having to completely remove the pants and shoes. Not to mention that having two diapers on has saved us from major blowouts several times.

Some Bath Supplies

Due to Macayla's progressive condition, she continues to loose trunk and head control. She can no longer sit up on her own and this makes baths impossible in the traditional way. We have started giving her baths in the bed and found some helpful suggestions from some parents and nurses on the pediatric floors. We started using a rinse-free shampoo called Aloe Vesta. It is a cleansing foam in a pump bottle made by ConvaTec. It can be used as a body wash as well. I normally use a spray bottle with warm water in it and dampen Macayla's hair first. Then, following the directions on the bottle, I lather her hair up and then dry the shampoo out of her hair with a washcloth. I have found it helps to use the water bottle to spray water into her hair to really rinse it and prevent any build up of shampoo residue. 

We also use an inflatable basin to wash her hair in the bed. The basin holds the water and has a notched side to fit the neck in while the head rests on a "pillow" in the basin. There is a drain hose attached to drain the water. This basin helps if we want to wash her hair very well and rinse it well.

For quick washing in the bed, we have found a great washcloth made by Medline called Ready Bath. They come in packs of eight and you simply microwave the pack for about 15 seconds depending on the microwave, and you have instant, warm, soapy, rinse-free washcloths. These have been a huge help and there is no dripping water to contend with. It makes bathing less of an ordeal.

Zegerid - Reflux Medication

Zegerid is a prescription medication described as a proton-pump inhibitor/antacid combination. It is a lot like Prevacid. Doctors tend to prescribe Prevacid for children with reflux issues and Prevacid has created a "solutab" that they claim is useful for feeding tubes. However, we have a Mic-Key button on our feeding tube and before that we had a Bard Button and a Genie prior to that. The Prevacid Solutabs would dissolve in the water but only down to granules that were too big for the button. They would clog the buttons and keep the internal valves open causing them to leak. There was almost an art to getting the medication to flow out of the syringe. If the plunger of the syringe pressed any of the medication against the bottom of the syringe, it would create a rubber-like mat. Some families we know use Prevacid successfully, but we prefer Zegerid. 

Zegerid is powder that comes in packets. You simply poor the powder in water (we use about 20 ml) and with minimal mixing it makes a suspension with a consistency thinner than Motrin. It is a recognized drug that our insurance would cover, but doctors tend to be a bit hesitant with it because there are not any pediatric studies on it like Prevacid. Our daughter weighs 53 lbs and takes 40 mg per day (two 20 mg packets - one in the morning; one at night). It has to be taken at least 2 hours after a feeding and 30-45 minutes before food. For those struggling with reflux and/or Prevacid, it might be worth checking with your doctor to see if you can try Zegerid. If there are any others reflux medications that have been helpful and tube friendly, we would love to learn about them as well.

Melatonin

We have to give Macayla melatonin to help her sleep. Since she has a degenerative neurological condition, she does not produce enough melatonin naturally. Our neurologist suggested that we start melatonin and that some of the recent research suggest that the starting dose be 12 mg. The normal adult dose is 3 mg. We have found that we usually have to give Macayla 18 mg. The problem is that melatonin normally comes in tablets and when we crush them they do not mix with water and will clog her feeding tube. We have found a capsule form of melatonin made by Life Time and the capsules contain a fine powder form of melatonin. We pull the capsules apart and mix the powder with equal parts water and formula. The formula (Elecare) is the normal formula we feed Macayla. For 18 mg (6 capsules worth) we have to mix it with 30 ml of water and 30 ml of formula. We blend it all together with a fork (for several minutes) and then shoot it in with a syringe through the feeding tube. It must go quickly and be flushed quickly or the mixture will clot in the tube and cause the feeding tube to leak. We have found the particular brand we use at a local health store, but it is hard to find online. The company's website does not seem to have a direct link to it. However, they reference iherb.com and there are some melatonin capsules for sale there.

Macayla's Wheelchair

We have a new wheelchair for Macayla by Ormesa. It is a company in Italy and we have been very pleased with it. We have their New Bug system and it has many options that Macayla's Zippie does not. There are definite pros and cons to both chairs, but we have been more satisfied with the Ormesa overall. It has options that are helpful for everyday life. It has a visor, a full rain cover that protects the chair and rider. It has a thermal cover for Macayla that goes in the seat first and then when she sits in the chair it can zip up around her. The chair tilts and reclines, is easy to break down, is crash tested and transportable. 

The Zippie by Quickie offers great support for Macayla as she looses trunk support and control. But our model would only tilt and not recline. For those new to the wheelchair market, tilt is when the whole seat (bottom and back) tilt back. Recline is when only the back of the seat leans back. Having tilt and recline is very helpful for comfort, decompressing feeding tubes, and diaper changing. 

The Zippie can be broken down and transported in a car. It is also crash tested and can be used for transport in vans and busses. The Zippie has more parts to disassemble for transport in a car than the Ormesa. But we have successfully fit both chairs (in their disassembled state) in the back of our Camary station wagon. The Ormesa is easier and quicker to assemble and disassemble in this case. Both chairs transport easily in our van and both have places to hook tie downs. The Zippie has the option for the EZ lock system and the Ormesa does not to my knowledge. The EZ lock is nice, expensive, but nice. You simply roll the chair into the floor mounted lock and the chair is in place and ready for transport. 

Granulation

We are fighting the granulation battle with Macayla's feeding tube. For those unfamiliar with granulation, it is tissue that builds around the feeding tube that is caused by the body trying to heal the tube site closed. It is red, meaty, and oozes and bleeds. It can be mistaken for an infection. The red tissue grows out in all directions and forms a rough ring around the site. It is prone to bleeding. For almost six months last year we lived with constant blood in Macayla's stomach, low feeding tolerance, and much discomfort. She had granulation on the inside of her stomach at the site as well as on the outside. We often must use silver nitrate sticks to cauterize the granulation tissue. We have used a nystatin cream around the site and it helps to control it, but we never get rid of it completely. Macayla's site should be similar to an ear piercing that has healed. It should be clear skin around the site. There are some recommendations by parents on Parent-2-Parent forums about different mixtures of creams and ointments that have helped. But every child is different and will respond differently to a given treatment. Doctors should be consulted about anything used around the site, but often a doctor's office can become ineffective. Parents have found many things that work that doctors would never suggest. We are fortunate that most of our doctors and nurses are willing to try many things. The surgeons want us to stay away from a wound-care specialist because wound-care can create granulation that is out control. We have not tried every suggestion yet on Macayla, but the things we have tried have had minimal or no success so far.

Syringes

We use 20 ml syringes (lure-lock) made by BD to administer meds through Macayla's feeding tube. The problem is that insurance and Medicaid only pay for a few per month and these were designed for one-time use. To make them last I have tried many things like putting clear tape over the numbers so they don't rub and wash off. On the Parent-2-Parent forum, I found a comment on using clear nail polish to protect the numbers and it works great. The polish dries very quickly and one coat will last long past the usable life of the syringe. 

The other issue is the syringes become rigid and do not want to function or slide very easily. They get rough spots inside and you can get quite a finger workout trying to draw up medications. I have tried cooking spray, cooking oil, mineral oil (our daughter struggles with constipation so it can't hurt!) and it only helps for one time use. Then the syringe gets stuck even more. The only thing that has worked for us is to use a bottle cleaning brush that has a sponge on the tip that can clean the inside of the syringe. Using soap has not been helpful here. Keeping buildup to a minimum has been the most helpful so far, but we are open to any suggestions.