I gave a talk last fall at a roundtable organized by the Jett Foundation for parents of boys younger than ten with muscular dystrophy. If you are unfamiliar with this disorder, it is characterized by muscle weakness which is progressive over time. It is due to a mutation in the gene encoding dystrophin, a protein which makes muscles strong. It is an x-linked disorder, meaning that only boys suffer from it (although mothers may be gene carriers and have health problems as a result).
Starting in the mid-teenaged years, these young men may develop weak coughs and difficulty breathing at night. That is primarily why I see them in clinic, and I will talk about these problems in a later post. However, I do think that parents of boys with muscular dystrophy can lay the groundwork for better respiratory health when boys are younger.
First, an important note. Boys with muscular dystrophy generally DO NOT have breathing issues before the teenage years. If you are a parent of one of these younger boys, allow yourself to put the breathing issues on the back burner. Your child has no greater risk of pneumonia than any other boy his age. There are other, more significant issues such as nutrition and maintenance of flexibility to attend to at this time.
Here is my list of recommendations, which I think would be useful for the parents of any child with chronic disease:
- Create a team for yourself: The families who are skilled at navigating the medical system know how to put together a team to help them care for their child. This team should include your pediatrician, neurologist, physical therapy specialist, nutritionist, cardiologist, and possibly other specialists as well, such as orthotists and endocrinologists. If there is a clinic nearby with experience in dealing with these disorders, they can help you coordinate this. Our clinic is here. You can find a clinic near you here. Does this list seem overwhelming? You don’t need to get all of these people at once. Your neurologist can help you build the team at the right time. Other parents can help as well. Your team also includes family members and friends who are supportive. I also encourage you to find parents who have children with muscular dystrophy. They will be your best resource. But, be aware, you may come across negative people, especially on the internet. Building a good team also means excluding people who scare you or make you feel bad.
- Vaccination is critical, just as it is for every child. That means getting all of your child’s vaccines on time, including the annual influenza vaccine. Not just for your child: everyone in the family should get vaccinated against the flu. I don’t want to hear any excuses. Just get the vaccine. Even if your child is vaccinated, he is more likely to get influenza if a household member has it. Your child needs the injectable form as the nasal form is not recommended for children with chronic illness. I also recommend the Pneumovax vaccine after age 5. It is a vaccine against a common bacteria which can cause pneumonia.
- Maintenance of a healthy weight is critical. Boys with muscular dystrophy may suffer from obesity, or, less commonly, difficulty gaining weight. Oral steroids, the treatment of choice in muscular dystrophy, causes increased appetite and weight gain. That’s why I recommend going to see a pediatric nutritionist who is familiar with the side effects of oral steroids. I care about this because obesity worsens lung function and will complicate management of breathing muscle weakness later on. This is a challenging issue for families so it is important to address it early.
- Take care of yourself and your partner. Having a child with muscular dystrophy is really stressful, but these children are resilient. Take time to relax and take care of your own health, mental and physical. Get a babysitter and go see a movie. Get outdoors. It will be good for all three of you. Most importantly:
- QUIT SMOKING. I can’t emphasize this enough. Smoking is expensive and increases the risk of your child developing breathing problems and infections. It is dangerous. Please quit.
- Be involved with research, with your eyes open. Given the severe nature of muscular dystrophy, families are desperate to enter into clinical trials. That’s great– that is how we will find a cure. However, medication trials do carry risk. Read the fine print in consent forms, and make sure that you can commit to all of the trial. Smaller scale research which involves questionnaires, etc– please participate. Anything that helps us understand the disease better may help your child and others like him in the future.
- Evaluate snoring if present. This may represent obstructive sleep apnea. The only way to rule out obstructive sleep apnea is via a polysomnogram, or sleep test. This is important because a child with mouth breathing may grow into an adult with narrow nasal passages. This will complicate management of breathing muscle weakness when your child is a young man. If you are worried about snoring:
- Get to know your friendly neighborhood pulmonologist (lung specialist). I don’t bite. I’d rather get to know you and your son before he has any medical problems. We have great therapies to keep these boys healthy heading into the future. You may have uncomfortable questions which you want to get off your chest. These will be easier for you to ask if we have a longstanding relationship. I like to see boys around age 6 or so, then every two years until they are using a wheelchair most of the time, at which point I like to see them annually.
Once again– boys with muscular dystrophy don’t generally have breathing problems related to their muscle weakness until they are teenagers. However, they can have the same problems (asthma, pneumonia, sinus infections, allergies) that any other child may have. Bring them to your pediatrician, create your team, ask lots of questions, and follow my tips above. If you have any other suggestions, please leave them in the comments below.