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.