During our family diagnostic trip to find answers, we were prescribed various drugs. After discovering that our daughter, Rylae-Ann, had aromatic l-amino acid decarboxylase (AADC) deficiency, we were hoping for a new set of medications alleviate his symptoms.
But administering his medication has never been easy. Hypotonia and autonomic dysfunction meant taking oral medication was nearly impossible for Rylae. However, we discovered several strategies through trial and error and discussing the issue with other parents.
When we gave Rylae medications or supplements by mouth, she would spit them out. Sometimes the pill would break or dissolve during her efforts to eject it from her mouth, delivering a bitter flavor causing retching. This would result in screaming, coughing and usually vomiting. Giving medication has become a dangerous process as we worry about the complications of aspiration.
Fortunately, we had more success and the process became much more manageable. The following tips are worth discussing with your GP if your child has been prescribed oral medication or supplements.
The psychological approach
If your child already knows a medicine bottle or colored pill, keep it hidden. Prepare it stealthily and try to camouflage it. The mere sight of the bottle can trigger anxiety or negative feelings that make your job difficult. With drugs, we try to be honest and happy throughout the process and offer rewards afterward. Do your best to make it a positive experience.
You can take it a step further and slip it into their food or drink, but our doctor warned us to be very careful with this approach. Children deficient in AADC are likely to be underweight due to feeding problems. If they suspect the food contains drugs or begin to associate the same negative feelings with meals, the drugs may become counterproductive. Also, mixing a drug with food or liquid can affect its effectiveness. Finally, your child will need to finish the whole meal to make sure he has taken all the medicine. You may not give a full dose.
That said, we sneak it all the time. We tried various foods, experimenting to see which darkened it the most.
For example, we give Rylae pyridoxal-5′-phosphate, an activated form of vitamin B6. I tasted a dab of the content to see what we were up against, and immediately gagged. We’ve tried adding it to chocolate milk, applesauce, and oatmeal. We recently added it to the ketchup of all things, and it was the most successful food yet.
However, it is important to experiment. I think the ketchup was helpful as there was no noticeable color difference. The B6 gave her food a noticeable orange tint, and she quickly realized what was happening.
The liquid approach
Some medications are available in liquid form. Ask your doctor about your options. For some medications, we might use a syringe or graduated dropper to drip the mixture down the back of her cheek, doing our best to bypass the taste buds. Usually, liquid medications taste sweet.
Unfortunately for us, the taste still did not satisfy our daughter’s palate. For example, we tried to give her liquid paracetamol when she had a high fever, but she immediately threw up the syrup. We tried unsuccessfully to clean up a miserable red, sticky mess while trying to make sure she wasn’t vacuuming.
Try to start the process positively. Over time, it will get easier. Direct the liquid towards the cheek and as far down the throat as possible to reduce the risk of it being spat out. Some parents of children who are more toned than our daughter hold their cheeks together to prevent their child from spitting out the medicine.
If a liquid form of the medication is not available, talk to your doctor about dissolving the medication in water. You can then use a syringe to administer it. The pharmacist can help dose a crushed pill in water. We noticed that hot water not only dissolved better but also caused less sensory overload. Also, the more water we added, the less taste was detectable in the diluted mixture.
During hospitalizations, we explained to the nurses that our daughter had problems with oral medication. They were able to give it in the form of a suppository. Many medications absorb very well this way and their effects occur as quickly, if not faster, than when taken orally.
Other drugs were given intravenously or through a feeding tube during hospitalizations. After being released, however, we ended up with the approaches mentioned above. However, our daughter received better care thanks to our requests not to give oral medication if possible.
We are also exploring gummy versions vitamins and supplements that will hopefully be made available to pharmaceutical companies. It looks like candy and it tastes like it too. Giving medicine gummies has been no problem since Rylae developed her feeding skills. If your child is still developing this skill, you can cut the chewing gum and make it a little moist so that it is slippery. It flows almost like water.
In closing, talk to your doctor about the forms of medication available. Find the form that works best for you and your child at home, and advocate for it during hospitalizations. Crushing the pills and dissolving them in a bitter liquid requires time and basic chemistry skills, but it makes them easier to administer. In contrast, coated tablets have no taste but are not the easiest for children to swallow.
Regardless of the approach, always give medications when your child is in an upright position in case there is a problem. Then watch them and keep them upright for at least 30 minutes. Make sure you give your child the correct dose and understand what the steps are if your child vomits or spits up the medicine. The protocol may be to give the drug again if you see it in the vomit or to wait for the next dose, depending on how long it has passed.
To note: AADC News is strictly a disease news and information site. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis or treatment. Always seek the advice of your physician or other qualified health care provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay seeking it because of anything you read on this website. The opinions expressed in this column are not those of AADC News or its parent company, BioNews, and are intended to spark discussion on issues relating to aromatic l-amino acid decarboxylase deficiency.