Tonsillectomy for IgA Nephropathy (Part 3)
A patient-centered Q&A guide
Blog Summary:
This extension of the main blog Parts 1-2 is for patients and care givers of those with IgAN. It addresses important questions for patients with IgA Nephropathy regarding:
End-stage renal failure
Effectiveness of tonsillectomy
Dialysis
Kidney transplant
Safety and availability of tonsillectomy
Guidance on navigating discussions with healthcare providers about tonsillectomy as a treatment option
A word cloud of outbound links to important online resources
*Podcast* - versions of selected articles - coming soon
The blog emphasizes the need for additional research, particularly in Western populations, and encourages patients to advocate for themselves while considering the potential benefits and risks of tonsillectomy.
This is a “living blog” with iterative updates as we go integrating feedback, new information, additional research, citizen-science project updates, clinical trials, and any necessary corrections.
1) What is the likelihood of progressing to end-stage renal failure for individuals with IgA Nephropathy?"
“The renal outcomes of IgAN vary significantly between individuals ranging from minimal proteinuria and stable renal function to development of end-stage renal disease (ESRD) in up to 50% of the cases. Markers of poor prognosis include impaired kidney function at presentation, hypertension, and persistent proteinuria >1 g/day in adults, and 0.5 g/day in children.” (Zand, 2014)
“Approximately 20–50% of IgAN patients would develop end-stage renal disease (ESRD) over the course of roughly 20 years.” (Yang, 2016)
“The natural course of IgAN may be indolent and benign; however, some 30–50% of patients may progress to end-stage renal disease when follow-up is extended to ≥20 years.” (Ponticelli, 2012)
“Progression to kidney failure may in the range of 20-40% within 5 to 25 years of diagnosis.” (Rychlik I, 1999)
2) What potential benefits might tonsillectomy with standard treatments offer for my IgA Nephropathy symptoms and disease progression?"
The efficacy of tonsillectomy in conjunction with standard treatments is a matter of ongoing discourse. It is not settled science and is certain to change over time. From the research we reviewed in the main blog – representing over 3,000 case of tonsillectomy in IgAN, we observed a wide range of improved outcomes across individuals were significant numbers of patients realized benefit shortly after tonsillectomy in reduction of proteinuria, hematuria, creatinine clearance, and improvement in eGFR potentially delaying the progression of the disease. As adjunct (supplemental) therapy and in some cases a standalone therapy – a significant fraction of the patients enjoyed complete clinical remission in the years that follow tonsillectomy.
While research is evolving clinical practices in western populations appear hesitant to adopt front line therapy finding success in Asia, personal advocacy and thorough understanding are key to your navigating the course of this disease. I am optimistic that well informed advocacy (citing information presented in this blog) empowers individuals to make informed decisions in close collaboration with your healthcare professionals.
Please consider sharing your journey with the supportive community forming around this blog. Ultimately, personal choice is paramount, and professional medical advice should always be sought. This blog serves as a discussion platform and should not replace medical expertise.
3) Understanding Dialysis and Its Relation to Kidney Transplant Necessity
Dialysis is a medical procedure used to perform the functions of the kidneys when they are unable to do so adequately. The kidneys are responsible for filtering waste products, excess fluids, and electrolytes from the blood. When they fail due to IgAN, dialysis becomes necessary. The type of dialysis for IgA nephropathy, typically depends on various factors, including the patient’s overall health, preferences, and the stage of the disease. Both hemodialysis and peritoneal dialysis can be used for individuals with IgA nephropathy, but the specific choice may vary. Hemodialysis is more commonly used for individuals with advanced kidney disease, as it is performed in a dialysis center. It is usually done three times a week, with each session lasting about 3-4 hours. Peritoneal dialysis, uses the patient’s own peritoneal membrane (inside the abdomen) as a natural filter. A fluid called dialysate is introduced into the abdominal cavity through a catheter. Waste products and excess fluids pass from the blood vessels in the peritoneal membrane into the dialysate, which is later drained out. Peritoneal dialysis can be done at home and is usually performed daily, with multiple exchanges throughout the day.
Not everyone on dialysis will eventually need a kidney transplant. Kidney transplantation is an option for some, but not all. Whether or not a person is considered a candidate for transplantation depends on several factors, including overall health, finding a suitable kidney donor match (either from a living donor or a deceased donor), each patient’s medical history, age, and other factors are considered when determining eligibility for transplantation.
4) Assessing Kidney Transplant Prospects: Wait Times, Recurrence of IgA Nephropathy, and Long-Term Immunosuppressive Medication Considerations
In 2021, there were 786,000 patients living with kidney failure, yet less than 25,000 received a transplant that year. “In 2022, the U.S. finally reached 25,000 kidney transplants in one year, but there are still an estimated twelve people dying each day without the opportunity to receive a life-saving transplant. Once you are added to the national organ transplant waiting list, you may receive an organ fairly quickly or you may wait many years. In general, the average time frame for waiting can be 3-5 years at most centers, but it is longer in some parts of the country.”
Probability of a candidate receiving a deceased donor kidney within 5 years of registration (28.7%), within 3 years (21.7%), within 1 year (11.6%). (Lewis, 2023)
IgAN recurrence after transplant has been reported in the range of 20-60% and a transplanted organ may on average remain healthy for 10-15 years in IgAN recipients. In a “multicenter international cohort of kidney transplant recipients, because of IgA nephropathy, cumulative incidence of recurrent IgA nephropathy was 19% at 10 years and 23% at 15 years after kidney transplantation. If only patients who had undergone a post-transplant kidney biopsy are analyzed, the recurrence rate was 42% after 10 years.” (Nephrol., 2021) Fortunately, there are several clinical studies demonstrating efficacy of tonsillectomy after kidney transplant. 11 tonsillectomies after transplant with 72% remission rate. (Zhu, 2018) 4 tonsillectomies average 52 months after transplant 100% remission 4-13 months later. (Ushigome, 2009)
kidney transplant recipients must remain on immunosuppressants (medications to suppress the immune system) for as long as the new kidney is working to prevent their body from rejecting it. There are significant side effects and potential complications of long-term use. Immunosuppressants weaken the immune system’s ability to fight off infections. This can lead to an increased susceptibility to various types of infections, including bacterial, viral, and fungal infections. Recall throughout the Covid Pandemic organ transplant patients of all types endured extreme risk of Covid complications. It was a very bad time to have a weakened immune system. If the immunosuppressive drug regimen is not carefully managed or if a recipient does not take their medications as prescribed, there is a risk of acute or chronic rejection of the transplanted kidney. Long-term use of immunosuppressants may have adverse effects on other organs and systems in the body. This can include damage to the liver, heart, and bones, among others. There are also potential gastrointestinal side effects like nausea, vomiting, diarrhea, and abdominal discomfort.
5) Is tonsillectomy relatively safe?
In electrodissection Tonsillectomy (Total Tonsillectomy using Electrocautery) the entire tonsil is removed using electrocautery or other techniques like cold steel or coblation. It is effective at completely removing the tonsils but can be associated with more postoperative pain and a longer recovery period compared to intracapsular tonsillectomy. The risk of bleeding may be higher due to the complete removal of the tonsils. Tonsillectomy is one of the most common surgical procedures in the United States, with more than 530,000 procedures performed annually in children younger than 15 years.” (Baugh, 2011)
The complication rates of tonsillectomy in adults can vary depending on several factors, including the patient’s overall health, the surgical technique used, and the experience of the surgeon. Potential complications are anesthesia risks, bleeding, infection, pain and discomfort, voice changes, difficulty swallowing and eating.
I encountered complications post-surgery, most likely due to my not diligently following the aftercare guidelines. Unexpectedly, a significant hemorrhage occurred, necessitating quick, makeshift first aid while preparing for a potential emergency room visit. Such rare risks may be rare and statistically small, but they become an acute reality when personally experiencing one, underscoring the importance of considering and preparing for all potential post-operative outcomes.
(Moriyama T, 2020) 8% of the patients in this study experienced complications as a consequence of tonsillectomy
(Hirano, 2019) The rate of complications following tonsillectomy in our study (2.8%), all of which were reversible with intervention, was comparable to those reported in the studies from Japan and the United States.
(Kondo, 2019) Post-tonsillectomy hemorrhage occurs in 10–20% of the patients who have undergone this surgery and 0–5% of the patients are required to undergo a hemostatic procedure (to stop the flow of blood in the throat) which involves general anesthesia.
6) What are the considerations and processes for obtaining a tonsillectomy based on my IgA Nephropathy diagnosis, and how is it financed
The availability and coverage of a tonsillectomy will depend on your healthcare team’s interpretation of the research and advocacy, the board of Nephrology guidelines in your country, and your health insurance policy terms. Coverage denial is possible even likely in Western countries where such procedures for IgA Nephropathy are uncommon or systemically prohibited. You may need robust advocacy from your healthcare provider to consider this controversial option. Navigating insurance and medical protocols for approval will require substantial support and possibly second and third opinions.
Prepare your case by informing your medical team with relevant research well in advance. Kindly give them time to consider the recent body of knowledge on tonsillectomy for IgAN before discussion in your next office visit. Your physician has standard care objectives they need to review with you on each visit. Discussion of this topic likely requires a dedicated separate office visit.
Effective advocacy requires mindfully respecting the magnitude of your inquiry on this topic. Healthcare professionals are working within a complex system, and understanding their perspective can facilitate more effective advocacy. I am hopeful the community forum will enable evolution of a detailed plan for advocating within these systems.
7) What are my options if medical professionals do not approve a tonsillectomy for my condition?
In the evolving debate on tonsillectomy’s role in IgA Nephropathy treatment, particularly among non-Asian populations, you may find differing medical opinions. Research may eventually clarify whether its benefits are ethnically specific or relate to other factors. While your healthcare providers might consider broader therapeutic options, they must balance potential benefits against the oath to avoid harm.
Currently, no clinical trials specific to tonsillectomy for IgA Nephropathy are listed on clinicaltrials.gov., indicating a need for further research.
Physicians are deliberately given leeway to explore off-label therapies provided they properly review the research, risks, and benefits. Medical breakthroughs sometimes emerge from clinical intuition and experience with patients. This may be the route through which we resolve this.
If a tonsillectomy isn’t supported, seeking additional opinions is your prerogative, though it may incur personal costs.
Can one personally finance a tonsillectomy for IgA Nephropathy if insurance does not cover it?
While personally financing a tonsillectomy is possible, it’s not advised here in this blog due to potential risks and costs. This option should only be considered when all insurance avenues have been exhausted, and with thorough understanding of the financial and medical implications. Local procedures can be costly, and seeking treatment abroad carries its own set of risks, especially if complications arise that require correction back home. Such decisions should involve careful deliberation and consultation with healthcare professionals to weigh all possible outcomes and costs.
Maximizing Health and Longevity Through Proactive IgA Nephropathy Management
Navigating IgA Nephropathy effectively may not only improve current health but also extend one’s healthspan significantly. Proactive management, especially from mid-life onwards, combined with recent and pending biotechnological advances make it possible to envision a future potentially adding decades to one’s life. Breakthroughs like xenotransplants and gene editing show promise in extending organ longevity, offering a glimpse into future treatments that could benefit those with chronic conditions.
In the coming decade intriguing examples of biomedical innovation will become available to those healthy and wealthy enough to take advantage of them.
In March 2024 the first transplant of a pig kidney into a living person after successful trials in brain dead recipients and monkeys were successful hints at a transformative organ transplant future. “The kidney was taken from a miniature pig carrying a record 69 genomic edits, which were aimed at preventing rejection of the donated organ and reducing the risk that a virus lurking in the organ could infect the recipient.” (Nature 2024) Monkeys have survived 1-2 years after gene-edited miniature pig-kidney transplants, (Shi En Kim, 2023 SCIAM).
A porcine-to-human heart transplant was also completed in the last year, surviving 49 days before xenograft failure. (Griffith, 2022)
Stem cell derived 3D printed kidneys (Maharjan, 2022) and (Garreta, 2021), regenerative therapies, immunomodulatory drugs, and gene therapy may be in reach for some.
Adhering to therapeutic best practices and making lifestyle changes can ameliorate the progression of IgA Nephropathy, with significant research suggesting adjunct therapies like tonsillectomy could offer further benefits. As biomedical innovations progress, they hold the potential to transform treatment paradigms, making it crucial not to let organ deterioration limit future health prospects.
Word Cloud outbound links
The substack platform understandably won’t allow the embedded code required for the word cloud but you can access it here.
Current treatment for IgAN overlap nicely with efforts to promote “Healthspan Extension”
Managing IgA Nephropathy effectively aligns well with the broader goal of extending one’s healthspan. Healthspan is the duration of life spent in good health, free from chronic diseases, and maintaining high physical, mental, and social well-being. It is a time marked by active engagement in life, with minimal disease-induced limitations. The treatments and lifestyle modifications suggested for IgA Nephropathy, such as dietary adjustments, good sleep hygiene, regular physical activity, stress reduction, and proactive medical care, are also foundational strategies for enhancing one’s healthspan. These interventions not only aid in the management of IgA Nephropathy but also contribute to the prevention of other age-related conditions, thereby supporting a life of wellness and vitality.
A call to action: mobilizing the community for IgA Nephropathy research
Engaging the broader community can accelerate research into IgA Nephropathy, particularly within non-Asian populations in the US and Europe. By leveraging data from healthcare organizations, we can drive citizen-science initiatives, gather global patient experiences, and incentivize researchers at the start of their careers. Collaborative efforts to analyze existing medical records and encourage retrospective studies can lay the foundation for more extensive, controlled research, all while ensuring that the quest for knowledge is a shared mission supported by crowd-funding and grants.
If this blog gains enough worldwide momentum a citizen-science project roadmap might look something like this.
Join us in the Substack discussion threads below. At crowd-scale we can accelerate research toward solutions.
For the most engaged on the topic I have created a community forum where we can gather, share, learn and organize crowd-scale projects that advance patient outcomes.
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Let’s get the conversation started. In this last blog I'm hoping patients will briefly share their stories, any experiences with or attempting to explore tonsillectomy for IgAN, what's working for them, open questions we need to contemplate, and suggestions for how we can advance the topic toward next steps. Please email me in Substack if any links fail.