Standard of Care
(The following opinions and information is provided by the mother of a child with GAN who is not a healthcare professional. For medical advice, please seek the help of a medical professional.)
As of May, 2012, there are no treatments for GAN. With the guidance of the FDA, we hope to sponsor a GAN gene therapy clinical trial at UNC at Chapel Hill, beginning Dec. of 2012, or Q1 of 2013. Refer to Doctors & Families tab for updates on the GAN gene delivery investigational new drug (IND) product.
It is thought that exercise is the best thing for these kids. The two older, slower progressing patients emphasize that exercise helps them keep function longer. A 19 year old in New Zealand said, “Do not sit still for a minute. Once you lose function, it’s nearly impossible to regain it”.
Do not perform a tracheotomy, unless absolutely necessary. There’s a new technique where the vocal cord(s) can be tacked back, freeing the airway. Search for an Ear Nose and Throat (ENT) specialist who does this procedure.
Choking & Aspiration Pneumonia
Both the muscle reflex in the back of the throat, and the mechanism that prevents food from going into the lungs when swallowing, is impacted by GAN. A feeding tube (Gastrointestingal tube, or “g-tube”) is eventually needed. Due to the fact that we all have different genes, and some gene can be compensatory, the age of g-tube placement varies greately, even for subjects I would classify as “typical GAN”. A 13 year old with typical GAN has a g-tube, and a 20.5 year old typically progressing GAN patient still does not have a g-tube. A swallow study is recommended for patients who start coughing consistently when drinking fluids or eating.
Patients participating in the GAN Natural History Study Hannah’s Hope Fund is funding will have a pulmonary function study. For those not participating in this study, it’s wise to get a pulmonary function baseline as soon as you suspect GAN. Ventilators are needed in the later stage of the disease. Most patients start with using the ventilator at night before becoming ventilator dependent.
Many (not all) GAN subjects have a dairy sensitivity and projectile vomit milk and heavy cream. Whey, casein and caseinate are also mike proteins they should also completely avoid if they have a dairy sensitivity. When you have a food sensitivity, the whole GI tract becomes inflamed and nutrients are not absorbed properly. Also, many neurotransmitters are made in the gut. These patients are already neurologically compromised, so if you sense your child has a dairy sensitivity, completely remove it from their diet. It will take several weeks of being completely diary-free before you will likely notice a difference in your child. We even observed our child began sleeping through to the alarm clock, vs waking up during the night, and/or awakening very early in the morning.
Great food substitutions:
Milk: Almond Breeze Original (you never know it’s not cow’s milk). The chocolate Almond Breeze is amazing and is flavored with cocoa, not milk chocolate. Doesn’t have the sweet, funky taste like soy milk. Another option is coconut milk.
Chocolate: Anything flavored with cocoa is fine. Most dark chocolates are fine, just read the ingredients.
Butter: Earth’s Balance is amazing! You can get it at WalMart and most grocery stores, in both stick and in containers. It’s also great to cook with.
Dinner rolls: Crescent roles are dairy-free.
Traveling with Disabilities
The federal government’s airport security agency is offering a toll-free help line to assist commercial air travelers with disabilities and medical conditions.
Travelers who hope to expedite their passage through the security webs at most airports are advised to call (855) 787-2227 at least 72 hours before their flight. The TSA Cares system operates Monday through Friday, 9 a.m. to 9 p.m. Eastern time, except on federal holidays. Callers can ask questions about screening policies and procedures, and what they can expect at security checkpoints.
The TSA Contact Center at (866) 289-9673 and TSA-ContactCenter@dhs.gov (email) also are avenues through which travelers can ask questions, offer suggestions and file complaints about their experiences with the agency and its staff.
It is recommended you check their back frequently for alignment and take them to the Chiropractor every 6 weeks. They will appear "crooked" which indicates they are out of alignment. The alignment process is simple and not painful. When you notice they are out of alignment, you may need to take them once or twice a week (about 3 or 4 visits total), then wait another month before we go again. Your Chiropractor will advise best. This is recommended good maintenance of helping to prevent scoliosis for as long as we can. Our "unscientific" estimate is, about 70% of GAN patients develop scoliosis and the sooner they lose ambulation (no longer wait baring much), the earlier they develop it. Even in the healthy population, we are told that they do not know what causes it. But, the chiropractor said the pelvis plays a huge role in it. After first examining one patient, one leg was shorter, due to pelvis being off. The body starts compensating if left uncorrected, resulting in eventual scoliosis. Again, a simple recommendation from a parent is to take your child to a chiropractor who treats children, every 6 weeks.
All website content is the opinion and observation of parents, not medical professionals. Please consult your medical doctor for medical advise.