Symptom Management

Children with MLD often have difficulty with swallowing, seizures, tight muscles, nerve spasms, or reflux. All of these conditions can be addressed with medication, and you should work with your child’s doctor to develop a medication regimen for your child. Keep in mind that medications that work well for a period of time may need to be adjusted after awhile, and we work with our doctors regularly to adjust medication levels depending upon the boys’ needs. Below is a listing of the medications our boys take:

• Reglan helps the stomach to empty and can help stop reflux. Since children with MLD cannot move around, it takes longer for their stomachs to empty. If their stomachs are not working efficiently, this can lead to reflux and vomiting. If your child has issues with this, Reglan may help. Another option to consider is adjusting the child’s g-tube formula, feeding continuously or using a pre-digested formula such as Peptamen Junior PreBio.
• Prevacid also helps with reflux and reduces the amount of acid in the stomach, making the stomach less irritable.
• Baclofen is a muscle relaxant and is commonly used. Some children have baclofen pumps surgically inserted in order to help with pain management. However, children can develop a tolerance to Baclofen and higher doses may not be effective. Consider using Dantrium (see below) in conjunction with Baclofen in order to avoid this.
• Dantrium is similar to Baclofen in that it is a muscle relaxant, but it can also have the effect of enhancing the effectiveness of the Baclofen.
• Inderol is generally used for high blood pressure or headaches. It was prescribed to our boys by our neurologist, who asked if the boys would tense up, arch their backs and get very upset at times. We were amazed that he knew this! The doctor said that the hypothalamus portion of the brain, which regulates the flow of hormones, is often damaged in neurologically impaired children. The hypothalamus can trigger a release of hormones/chemicals into the body causing the child to experience extreme emotions for no apparent reason. Inderol helps to control this release of hormones.
• Robinul is used to control drooling. If children have a hard time swallowing, saliva can accumulate in their mouths, causing them to gag or drool. Robinul reduces the amount of saliva that is produced but a side effect can be dry mouth
• Klonipin or Clonazepam is an anti-seizure medication that our boys receive in small amounts three times per day.
• Elavil is a drug that helps to control random neurological impulses. We found that our boys were experiencing extreme sensitivity in their hands and feet and would quickly pull away whenever they were touched in those areas. Elavil helps to manage those impulses so they are not so startling.
• Motrin and Tylenol are used to manage pain and muscle tightness. We alternate using Motrin and Tylenol since Motrin can be hard on the stomach.
• Pulmicort is a steroid that is inhaled into the lungs through the use of a nebulizer and helps to reduce any inflammation. As MLD children cannot move and run around, they are susceptible to pneumonia and breathing issues. Pulmicort can reduce any inflammation. Our boys use Pulmicort once a day when they are healthy and 3-4 times per day when they are sick.
• Miralax is a laxative that helps maintain regular bowel movements. It is now available over-the-counter and does not require a prescription. Miralax does not have to be given every day (it may result in excessive bowel movements!), so you may want to experiment with the amount given and how often you give it. For instance, our boys used to get ½ capful every other day. Please make sure to give your child plenty of water during a 24-hour period while they are on Miralax, or it can actually gel together and block the colon even further.
• Tylenol 3 or Tylenol with Codeine can be used for muscle relaxation and pain relief. It is best to give this in the evening or other times when the child does not need to be alert.
• Botox If your child’s muscles are very tight, Botox therapy can be very helpful. Botox is now commonly used for cosmetic procedures, but has been used for cerebral palsy patients for many years. Christopher’s arms used to be very tight and clenched. His doctor gave him Botox injections in his arms, and coupled with the short-term use of arm braces, he no longer has the tightness in his arms that he used to have. A key point to note is that it is very important to go to a doctor who is skilled and experienced in administering Botox since the location of the injection and the amount given can make a difference in the success of the treatment.

Some of these medications can be made into a liquid form while others are available in pills. If only pills are available, we grind them up into a powder using a mortar and pestle (available at cooking stores). We then mix the powder with other liquid medication or water and administer it with a syringe through the G-tube. However, different drugs may require specific handling, so please consult your physician for guidance on how to administer each medication.