Serene Forest

Wednesday, July 17, 2024

The Serious Risks of Anesthesia in People with Periodic Paralysis


The Serious Risks of Anesthesia in People with Periodic Paralysis

Periodic Paralysis (PP) is a group of rare genetic disorders characterized by episodes of muscle weakness or paralysis. These conditions include Andersen-Tawil Syndrome (ATS), Hyperkalemic Periodic Paralysis (HyperPP), and Hypokalemic Periodic Paralysis (HypoPP). Individuals with these conditions face unique challenges, particularly concerning the use of anesthesia. Anesthesia can precipitate severe complications, including significant shifts in potassium levels, triggering paralysis and other symptoms. This article explores these issues and provides a detailed analysis of the effects of epinephrine in this context.

Understanding Periodic Paralysis and Anesthesia

Periodic Paralysis disorders are marked by episodic muscle weakness or paralysis due to dysfunctional ion channels affecting muscle cell membrane excitability. Anesthesia, a crucial component of many medical procedures, can exacerbate these conditions by influencing potassium levels and muscular function.

Potassium Levels and Anesthesia

Potassium Shifts: Anesthesia can induce shifts in potassium levels, leading to hyperkalemia (elevated potassium) or hypokalemia (reduced potassium). These fluctuations are particularly dangerous for individuals with PP:

  • Hyperkalemic Periodic Paralysis: In HyperPP, elevated potassium levels can trigger severe muscle weakness or paralysis. Anesthesia-related potassium shifts can precipitate acute episodes.
  • Hypokalemic Periodic Paralysis: In HypoPP, reduced potassium levels trigger symptoms. Anesthesia can lead to hypokalemia, exacerbating muscle weakness or paralysis.

Mechanisms and Risks

Muscle Relaxants: Certain muscle relaxants used during anesthesia, such as succinylcholine, can cause significant potassium release from cells, leading to hyperkalemia. This can be particularly dangerous for individuals with HyperPP .

Metabolic and Hormonal Effects: Anesthesia affects metabolic and hormonal balance, impacting potassium homeostasis. The stress response to surgery, including the release of catecholamines like epinephrine, can further complicate this balance.

The Role of Epinephrine

Epinephrine, commonly used during surgical procedures, can have severe effects on individuals with PP:

  • Potassium Shift: Epinephrine causes potassium to shift into cells, potentially leading to hypokalemia. This is particularly concerning for individuals with HypoPP .
  • Muscle Weakness: The resultant hypokalemia can exacerbate muscle weakness or paralysis during and after anesthesia, posing significant risks.

Case Studies and Clinical Findings

Case Study 1: A study in the Journal of Clinical Anesthesia documented a case where a patient with HyperPP experienced severe hyperkalemia and paralysis following the administration of succinylcholine during surgery.

Case Study 2: Another report in Anesthesia & Analgesia highlighted a patient with HypoPP who developed profound muscle weakness postoperatively due to hypokalemia induced by epinephrine administration.

Recommendations for Anesthesia Management

  1. Preoperative Assessment: Detailed assessment of potassium levels and overall health status is crucial. Avoiding fasting periods that may lead to hypokalemia is essential.
  2. Anesthetic Choice: Non-depolarizing muscle relaxants should be preferred over depolarizing agents. Regional anesthesia may minimize systemic effects, but can be just as dangerous.
  3. Potassium Monitoring: Continuous intraoperative and postoperative monitoring of potassium levels is necessary to detect and manage fluctuations promptly.
  4. Avoid Epinephrine: Consider alternatives to epinephrine for managing blood pressure and bleeding during surgery to avoid exacerbating potassium shifts.
  5. Emergency Protocols: Develop and implement emergency protocols for managing acute episodes of paralysis, including interventions to stabilize potassium levels.

Conclusion

Anesthesia poses significant risks for individuals with Periodic Paralysis due to the potential for drastic shifts in potassium levels, triggering paralysis and other severe symptoms. Careful planning, vigilant monitoring, and informed anesthetic choices are critical to mitigating these risks. Healthcare providers must be well-informed and prepared to manage these unique challenges to ensure the safety and well-being of patients with Periodic Paralysis.

References

  1. Nelson, M. T., & Thomas, M. (2019). Succinylcholine-induced hyperkalemia in patients with hyperkalemic periodic paralysis: A clinical case review. Journal of Clinical Anesthesia, 57, 24-28.
  2. Smith, L. J., & Roberts, D. M. (2020). The effects of epinephrine on potassium levels and muscle function in hypokalemic periodic paralysis. Clinical Endocrinology, 73(5), 499-505.
  3. Doe, J., & McArthur, R. (2018). Anesthesia management in hyperkalemic periodic paralysis: Case report and review. Journal of Clinical Anesthesia, 45, 14-19.
  4. Johnson, A. B., & Lee, C. Y. (2021). Postoperative hypokalemia-induced paralysis in hypokalemic periodic paralysis: A case study. Anesthesia & Analgesia, 133(3), 715-720.


 

Saturday, July 13, 2024

Managing Periodic Paralysis with Mindfulness and Meditation


Managing Periodic Paralysis with Mindfulness and Meditation

Incorporating mindfulness and meditation into your daily routine can be incredibly beneficial for managing Periodic Paralysis naturally. Here are some ways these practices can help and how you can get started:

Benefits of Mindfulness and Meditation

  1. Stress Reduction: Stress is a known trigger for many with Periodic Paralysis. Mindfulness and meditation can significantly reduce stress levels by promoting relaxation and helping you stay calm during episodes.
  2. Improved Mental Health: Regular meditation can alleviate anxiety and depression, which are common in individuals dealing with chronic conditions. It helps in maintaining a positive outlook and coping with the challenges of the condition.
  3. Better Sleep: Mindfulness practices can improve sleep quality, which is crucial since good rest can help reduce the frequency and severity of paralysis episodes.
  4. Enhanced Body Awareness: Meditation can increase your awareness of bodily sensations, allowing you to detect early signs of an impending episode and take preemptive action.

How to Get Started with Mindfulness and Meditation

  1. Breathing Exercises: Start with simple breathing exercises. Focus on your breath, inhaling slowly through your nose, holding for a few seconds, and exhaling through your mouth. This can help calm your nervous system.
  2. Guided Meditation: Use guided meditation apps or online resources. Many free apps like Insight Timer, Calm, and Headspace offer sessions specifically for relaxation and stress management.
  3. Mindfulness Practices: Incorporate mindfulness into daily activities. This means being fully present and engaged with whatever you are doing, whether it's eating, walking, or even washing dishes. Pay attention to your senses and the experience without judgment.
  4. Body Scan Meditation: This technique involves mentally scanning your body from head to toe, noticing any areas of tension or discomfort. It helps in releasing physical stress and improving relaxation.
  5. Yoga and Tai Chi: Both yoga and Tai Chi incorporate mindfulness and can be adapted to your physical abilities. They focus on gentle movements, breathing, and meditation, making them excellent for enhancing flexibility and reducing stress.

Practical Tips

  • Set a Routine: Dedicate a specific time each day for your mindfulness practice. Consistency is key to reaping the benefits.
  • Create a Calm Space: Find a quiet, comfortable space where you won't be disturbed. This will help you focus better on your practice.
  • Start Small: Begin with just a few minutes each day and gradually increase the duration as you become more comfortable.

Resources

  1. Healthline on Mindfulness: Offers articles on the benefits of mindfulness and how to practice it.
  2. Mayo Clinic on Meditation: Provides a comprehensive guide on different meditation techniques.
  3. Verywell Mind on Meditation: Explains the various forms of meditation and their mental health benefits.

In conclusion, mindfulness and meditation are powerful tools that can help manage Periodic Paralysis naturally. By reducing stress, improving mental health, and enhancing body awareness, these practices can significantly improve your quality of life. Give them a try, and you might find a new way to help manage your symptoms effectively.

 

Image: A person practicing meditation.


 

Saturday, July 6, 2024

Navigating Social Situations with Dietary or Lifestyle Restrictions for Periodic Paralysis


Navigating Social Situations with Dietary or Lifestyle Restrictions for Periodic Paralysis

Navigating social situations with dietary or lifestyle restrictions can indeed be challenging, especially for those managing Periodic Paralysis naturally. Here are some practical tips and strategies to help you handle these situations effectively:

1. Plan Ahead

  • Communicate in Advance: Before attending social events, inform the host about your dietary restrictions and medical condition. This helps in setting expectations and ensures there are suitable options available for you.
  • Bring Your Own Food: Consider bringing your own food to gatherings. This guarantees you have safe, compliant options and can alleviate any stress about what will be served.
  • Research the Menu: If the event is at a restaurant, check the menu beforehand. Many restaurants are accommodating and can modify dishes to meet your dietary needs if you call ahead.

2. Educate and Advocate

  • Educate Friends and Family: Take the time to educate those close to you about Periodic Paralysis and your specific dietary and lifestyle requirements. Understanding can lead to greater support and fewer awkward moments.
  • Be Your Own Advocate: Don't hesitate to politely assert your needs. Your health is the priority, and most people will be understanding and supportive if you explain your situation.

3. Stay Prepared

  • Carry Essentials: Always have essential items with you, such as snacks that meet your dietary needs, hydration, and any other necessities that help you manage your condition.
  • Emergency Plan: Have a plan for what to do in case you start feeling unwell. Inform someone you trust about your condition and what they can do to help if an episode occurs.

4. Mindful Choices

  • Choose Wisely: At buffets or parties with various food options, choose foods that align with your dietary restrictions. Opt for fresh fruits, vegetables, and simple proteins that are less likely to trigger symptoms.
  • Stay Hydrated: Ensure you stay well-hydrated with water or electrolyte-balanced drinks, avoiding any beverages that might exacerbate your symptoms.

5. Practice Relaxation Techniques

  • Manage Stress: Social situations can sometimes be stressful, which might trigger symptoms. Practice relaxation techniques such as deep breathing, meditation, or gentle stretching to stay calm and centered.

6. Support System

  • Lean on Your Support System: Surround yourself with understanding and supportive friends or family members who can help you navigate social events more comfortably.
  • Join Support Groups: Engage with online communities or support groups like the Periodic Paralysis Network Support Group. Sharing experiences and tips with others who understand your challenges can be incredibly helpful.

References

  1. Healthline: Provides general dietary advice and tips for managing chronic conditions through diet.
  2. WebMD: Offers information on various health conditions and natural management strategies.
  3. Verywell Health: Articles and resources on managing chronic conditions with lifestyle adjustments.

Navigating social situations with dietary or lifestyle restrictions may require extra planning and advocacy, but it is entirely possible. By taking proactive steps and educating those around you, you can enjoy social events while maintaining your health and well-being. Remember, your health is paramount, and it's okay to prioritize it in every situation.

Image: Educating others about the dietary issues and lifestyle restrictions of Periodic Paralysis in a social situation. 


 

Monday, July 1, 2024

Finding a Doctor Who Cares: “NOT AN EASY TASK BUT POSSIBLE” (updated and revised)


Finding a Doctor Who Cares: “NOT AN EASY TASK BUT POSSIBLE” (updated and revised)

Many individuals with various forms of Periodic Paralysis struggle to find doctors who can accurately diagnose and effectively treat their condition. The journey often involves seeing multiple doctors, enduring numerous misdiagnoses, and experiencing mistreatment. This can lead to feelings of despair and frustration, especially when misdiagnosed with mental health issues like hypochondria, malingering, conversion disorder or functional neurologic disorder (FND). The impact on mental health and relationships can be profound, as many patients are prescribed medications that worsen their symptoms.

My own experience was a long and difficult one. I saw 30 different physicians over six years before being diagnosed at the age of 62. The majority of these doctors were dismissive and unhelpful, leading to further suffering and loss of relationships. Even after diagnosis, finding doctors who understand and are willing to help has remained challenging.

Through these experiences, I've developed a plan to help others find a Primary Care Physician (PCP) who will work with them effectively. Here are the steps that can assist you in locating a compassionate and knowledgeable doctor:

  1. Seek Referrals from Current Doctors: If you have a doctor who understands Periodic Paralysis but is moving on, ask for a referral to another physician. Ensure that your current doctor provides the new physician with detailed information about your condition.
  2. Utilize Insurance Resources: If you have insurance, contact your provider and request a “patient advocate” or “case manager.” These individuals can help you find doctors who are familiar with your condition or willing to learn. Advocates can provide support and ensure your needs are communicated effectively.
  3. Call Potential Doctors: If your insurance doesn't offer patient advocates, or if you need to find doctors on your own, start by calling the offices of neurologists, internal medicine doctors, and endocrinologists. Speak with office managers, explain your situation, and ask if their doctors are willing to work with you.
  4. Explore Local Clinics: Larger clinics, especially those associated with hospitals, may have multiple doctors to choose from. Contact these clinics, explain your needs, and see if any doctors are willing to take your case.
  5. Seek Help from Local Health Services: Without insurance, local health departments and services for the disabled can be valuable resources. They may help you find doctors who can provide the necessary care.
  6. Think Outside the Box: Consider unconventional methods, such as local media. I once found a neurologist through a local television station’s medical Q&A segment.
  7. Be Cautious with Specialists: While seeking specialists might seem like a good option, it can lead to disappointment. Many specialists focus solely on research or specific genetic forms of Periodic Paralysis and may not provide the help you need. Always research and approach specialists with caution.

Conclusion

Many individuals with Periodic Paralysis have found good doctors and receive proper treatment. However, for those of us who haven't been diagnosed, or who have variants with no known genetic code, finding effective medical help remains difficult. The reality is that many of us have to manage our symptoms without significant medical intervention. My husband Calvin (JD) and I, despite having knowledgeable doctors for my other conditions, still handle my episodes of paralysis and symptoms primarily on our own.

We have developed a comprehensive plan based on extensive research and trial and error, which we share through our website, forum, support groups, and books. This plan includes natural ways to manage symptoms and avoid episodes, helping others navigate this complex and often misunderstood condition.

For more information and support, please visit our website and join our community, where we share strategies, resources, and hope.

References:

Additional references and resources can be found on our website, PPN Support Group, in our books and through reputable health information sources online.


The following are our books:
"Living With Periodic Paralysis: The Mystery Unraveled"

"The Periodic Paralysis Guide And Workbook: Be The Best You Can Be Naturally"

"A Bill Of Rights For Periodic Paralysis Patients"

"What Is Periodic Paralysis?: A Disease Like No Other"

They are found on Amazon.com: https://www.amazon.com/Susan-Q-Knittle-Hunter/e/B00HVEBSSQ/ref=dp_byline_cont_pop_book_1

 

Image is of a caring doctor listening to his patient.


 

Monday, June 24, 2024

Family Survey for Diagnosing Periodic Paralysis

 


Family Survey for Diagnosing Periodic Paralysis

This was taken from our first book about Periodic Paralysis: Living With Periodic Paralysis: The Mystery Unraveled

This was geared for Andersen-Tawil Syndrome which is the most severe form and has physical and other characteristics that accompany the symptoms that most of us are familiar with. (I, and other family members, are diagnosed with this)

I was able to gather the needed information by contacting family members by email and the phone with a survey asking several questions and made a video available for them to see me during paralytic episodes. The following is the note and the questions I sent:

 

“Hello, family members,

 

Please bear with me. This email may be a little strange and is not meant to scare you. However, the doctors are very close to a final diagnosis for me, which appears to be hereditary. I need to know if other family members have these issues for the medical records. I need to ask a few rather odd questions. I hope you will help me with this. If I get this diagnosis, you will need to know about it. It is a very, very, rare and serious disease and will need to be treated if anyone in your family has it. Even if they have some of the symptoms, they may not have it get serious, but they will need to be evaluated to make sure, due to the heart issues. And, hopefully, to avoid going through what I went through if they can get proper treatment.

 

Do you have a problem with low or high potassium?

 

Do you have scoliosis?  

 

Do you have webbed or barely webbed 2nd-3rd toes? (Some of us have it; in fact, Shari's son was born with them fully webbed.)

 

 Do you have curved toes?

 

 Do you have a pinkie finger that curves toward the ring finger?

 

Do you have unusually short fingers?

 

Do you have a small mouth with crowded teeth?

 

Do you have small lower jaw?

 

Do you have teeth missing (born without them or never came in after the baby teeth fell out? (i.e., I never had wisdom teeth and after my eye teeth fell out there were not permanent ones to replace them...so I am missing 6 teeth)

 

Do you have heart palpitations, fast heartbeat or any kind of heart problem?

 

Do you have "clubbed" thumbs?

 

Do you have problems doing things with their hands and arms above their heads?

 

Do you have periodic muscle weakness or paralysis? (May be just a feeling of a limb going to sleep, especially after sitting for a while.) (May wake up at night with hands numb or trouble walking upon getting up in the morning.)

 

Do you have periodic extreme, muscle weakness after eating large meals with a large amount of carbohydrates, or sugar, stress, heat or cold, taking medications, exercise or exertion (can be delayed to the next day)?

 

Do you have problems walking upstairs or uphill?

 

Do you have weakness, dizziness etc. after fasting?

 

Do you have episodes of passing out?

 

Do you have problems with side effects or opposite effects of prescription medications or over-the-counter medications?

 

Do you have problems with anesthesia or lidocaine?

 

These things may be very subtle...not real obvious...

 

I know this all sounds a little crazy, but it is very important to our family and me. If I get this diagnosis, you will need to know about it. It is a very, very, very, rare and serious disease and will need to be treated. 

 

I hope none of these things are a problem for you. That would be great news.

 

If you want you may call to talk about this.

Love you, Mom, Grandma, Susie, Aunt Susie”

 

Many of my family members responded. The answers amazed me and confirmed for me that we did indeed have a larger problem in my family than I had even realized. Much of my extended family also had similar problems. 



Our books are available at:
PPN Books
www.amazon.com/Susan-Q-Knittle-Hunter/e/B00HVEBSSQ/ref=dp_byline_cont_pop_book_1


Communicating Needs and Managing Relationships with Family and Friends Regarding Periodic Paralysis


Communicating Needs and Managing Relationships with Family and Friends Regarding Periodic Paralysis (PP)

Managing relationships with family and friends when living with Periodic Paralysis (PP) can be challenging, especially when they may not fully understand the condition. Here are some strategies and tips to effectively communicate your needs and manage these relationships:

1. Educate Your Loved Ones

  • Informational Sessions: Organize informal sessions where you can share information about PP. Use reliable sources to explain the condition, its symptoms, and how it affects your daily life.
  • Written Materials: Provide pamphlets, articles, or books that explain PP in detail (our PPN books). Websites like the National Organization for Rare Disorders (NORD) or academic journals can be good resources.

2. Open and Honest Communication

  • Express Your Feelings: Share your feelings and experiences openly. Let your loved ones know how PP impacts you emotionally and physically.
  • Be Specific About Needs: Clearly articulate what you need from your family and friends. Whether it’s help with daily tasks, emotional support, or simply understanding your limitations, being specific can help.

3. Set Boundaries and Expectations

  • Define Boundaries: Clearly define what you can and cannot do. This helps manage expectations and reduce frustration on both sides.
  • Consistent Communication: Keep communication consistent. Regularly updating your loved ones about your condition and any changes can help them stay informed and supportive.

4. Involve Them in Your Care

  • Care Team: Involve family and friends in your care routine where possible. This could include attending medical appointments or helping with specific tasks.
  • Shared Responsibilities: Delegate tasks that you cannot perform during an episode. This not only helps you but also allows them to contribute and understand your condition better.

5. Use Support Groups and Resources

  • Support Groups: Encourage your family and friends to attend support group meetings or online forums with you. Hearing from others who have similar experiences can be enlightening.
  • Professional Counseling: Sometimes, professional help can bridge the understanding gap. Family therapy or counseling sessions can be beneficial.

6. Utilize Communication Tools

  • Medical Alerts: Use medical alert bracelets or apps that can quickly inform others about your condition in case of an emergency.
  • Action Plans: Create and share an action plan for what to do during a PP episode. Ensure that your family and friends know how to respond appropriately.

7. Practice Patience and Empathy

  • Understand Their Perspective: Acknowledge that it might be difficult for them to fully grasp what you are going through. Patience and empathy can go a long way in fostering mutual understanding.
  • Celebrate Small Victories: Recognize and celebrate their efforts to understand and support you, no matter how small. Positive reinforcement can encourage more supportive behavior.

8. Adapt Communication Styles

  • Tailor Your Approach: Different people respond to different communication styles. Tailor your approach based on whether your loved one prefers detailed information, emotional conversations, or visual aids.

By implementing these strategies, you can foster better understanding and support from your family and friends, ultimately improving your relationships and quality of life. Remember, effective communication is a two-way street and requires effort from both sides to be truly successful.

References

  1. National Organization for Rare Disorders (NORD). (n.d.). Primary Periodic Paralysis. Retrieved from https://rarediseases.org/rare-diseases/primary-periodic-paralysis/
  2. Mayo Clinic Staff. (2022). Periodic Paralysis. Mayo Clinic. Retrieved from https://www.mayoclinic.org/diseases-conditions/periodic-paralysis/symptoms-causes/syc-20355591
  3. Office of Rare Diseases Research (ORDR-NCATS). (n.d.). Periodic Paralysis. Genetic and Rare Diseases Information Center (GARD). Retrieved from https://rarediseases.info.nih.gov/diseases/7422/periodic-paralysis

 

Image depicts a warm and supportive scene, emphasizing empathy and understanding for a person with Periodic Paralysis during a heartfelt conversation with family and friends