For parents of children with Duchenne Muscular Dystrophy (DMD), every new symptom or health issue can be a source of concern. DMD, a genetic disorder characterized by progressive muscle degeneration and weakness, requires careful, nuanced management. Episodes of fever and convulsions are particularly alarming and demand effective management strategies that emphasize safety and tailored care.
DMD results from mutations in the gene that encodes dystrophin, a protein essential for muscle fibre stability. The absence of dystrophin leads to muscle weakness, cardiac issues, and respiratory complications. As this disease affects various body systems, even common ailments like fever can become more complicated. Convulsions, which can be particularly distressing, may occur for the first time due to several triggers, including fever. Parents and caregivers must understand how to manage these incidents and why a cautious approach is vital. In this write-up, I will try to offer some suggestions on managing these issues safely and effectively, drawing on current medical practices and research. However, please note that I am only a researcher in training, trying to better understand and support patients with musculoskeletal and neuromuscular diseases, and this write-up is intended as a rough guideline or general advice. Always consult with a specialist clinician before making any medical decisions.
Immediate Steps for Managing Fever and Convulsions
Airway Management
It is of utmost importance to ensure a clear airway during a convulsive episode to avoid any possibility of aspiration. Aspiration can lead to severe complications, including pneumonia-like symptoms. To ensure an open airway, it is advisable to position the individual on their side in the recovery position, which can help prevent any obstruction and lessen the likelihood of aspiration.
Temperature Control
Fever can exacerbate muscle breakdown. Managing body temperature with light clothing and sponging with lukewarm water helps mitigate this risk. Avoid drastic cooling measures, e.g., sponging with cold water or rubbing alcohol, as these can cause shivering and increased muscle activity, potentially leading to further muscle damage.
Pharmacological Considerations
Use of Antipyretics
Liver function in DMD patients can often be compromised. Medications like paracetamol/acetaminophen (Tylenol, Ace, Napa, Pyrex, etc.), which are metabolized in the liver, need careful dosing to avoid toxicity. Though paracetamol/acetaminophen are widely available over-the-counter drugs used to manage fever and pain, they may not be as harmless for those with DMD. It is thus essential to consult with a specialist clinician to ensure appropriate use of these antipyretic agents.
NSAIDs and Cardiac Risks
Nonsteroidal anti-inflammatory drugs (NSAIDs), e.g., ibuprofen (Advel, Profen, Rebufen, Reumafen, etc.), can impact renal function and fluid balance, affecting the heart. Due to the high occurrence of cardiomyopathy among DMD patients, these drugs should only be used cautiously under medical supervision. Also, the dosages prescribed for DMD patients may differ from those for other children. It's crucial to seek specialist advice before administering a standard dose to avoid any potential complications.
Anti-Seizure Medications
Diazepam and other benzodiazepines (Valium, Sedil, Easium, Azepam, etc.) are used for acute seizure management but can cause sedation and respiratory depression. The risk is particularly heightened in kids with DMD due to pre-existing muscle weakness that can compromise respiratory function. Therefore, it is recommended that these medications are administered in a controlled environment with continuous monitoring.
Combinatorial Drug Formulations
Nowadays, many over-the-counter drug combinations, e.g., paracetamol/acetaminophen with caffeine (Tylenol Ultra, Ace Plus, Napa Extra, Pyrex Plus, Pyrinol, etc.) or tramadol (Ultracet, Acetram, Napadol, Pyrex T, Xcel Max, etc.), are readily available. Using these in children with DMD requires extra caution. For instance, caffeine can increase alertness, disrupt sleep, and complicate symptom management. More critically, it can elevate heart rate and blood pressure, posing significant risks for DMD patients with potential cardiac issues. Conversely, tramadol, an opioid pain reliever, can lead to respiratory depression and increased sedation—risks that are particularly significant given the respiratory challenges associated with DMD and may also exacerbate muscle weakness. It’s hence crucial to carefully review the specific formulations of these medications due to varying brand names and compositions. Consulting with a specialist is always and always recommended to ensure safe and appropriate management of your child's condition.
Close Monitoring and Support
Vital Signs Monitoring
Monitoring vital signs, e.g., heart rate, respiratory rate, and, if possible, oxygen saturation, is crucial. These can provide essential data to guide emergency responses and hospitalization decisions. I personally recommend all parents and caregivers of children with DMD, especially those living in countries like Bangladesh, to acquire a pulse oximeter and learn how to use it. This simple and easy-to-use device can provide crucial information that can help guide further actions.
Emergency Protocols
I often advise parents and caregivers of children with DMD to develop a comprehensive emergency care strategy and ensure that all caregivers are familiar with it. This plan should always be readily accessible, both at the workplace and at home. Knowing how to contact your child's doctor immediately is crucial, and never hesitate to do so if the situation deteriorates. Always be prepared to take your child to the hospital if necessary. Maintaining a medical information sheet that lists your child's medical background, current medications, medical history, and any allergies is also incredibly beneficial.
I wish to reiterate that for children with DMD, even a simple cold or fever can present significant challenges due to their overall health vulnerabilities. Quick action and thorough preparedness can ensure timely and effective treatment, which helps alleviate the stress of such situations.
Ending Remarks
Handling a child with DMD who is experiencing a fever and convulsions can be challenging—I totally understand that. However, knowing the right steps can ease some of the stress and ensure your child receives the best possible care. I always encourage parents and caregivers to remain in close contact with patient groups and social media communities for more information or personal stories from others navigating similar challenges. For those in Bangladesh, you may consider joining our online communities on Facebook. These groups and communities can provide support, share experiences, and help from others who truly understand the journey with DMD. However, we must acknowledge that each child with DMD may experience symptoms differently, and what works for one child may not work for another. Continuous communication with a specialist doctor, staying informed about the latest management strategies, and connecting with support groups can make a significant difference in managing this condition.
P.S.: the above guideline is specifically intended for children with Duchenne muscular dystrophy and may not apply to those with any other conditions, even if related to other types of muscular dystrophy.
16 April 2024
Saeed Anwar
Edmonton, Canada