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What information or input is important to you when starting a new PH1 treatment?

If your doctor prescribes a new-to-you primary hyperoxaluria type 1 (PH1) treatment, what’s important for you to know before you start taking it?

Maybe you receive the majority of your medical information from your doctor, and defer to their recommendations about possible treatment options. Maybe you would prefer to talk to your loved ones about your options, or perhaps you look to the experiences of other patients who have already tried the medication you are considering.

Share what’s important to you in the comments below. There’s no wrong answer!

#KidneyDisease #KidneyTransplant #LiverTransplant #Transplant #OrganTransplant #kidneyawareness #KidneyStones #KidneyPain #KidneyProblems #RareDisease #ChronicIllness #CKD #ChronicKidneyDisease #PrimaryHyperoxaluriaType1

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What’s on your health to-do list this week?

Living with a kidney disease like primary hyperoxaluria type 1 (PH1) means that taking care of your health can often feel like a full-time job. Maybe you are making phone calls to your medical team, paying bills, requesting medical records, scheduling an appointment, or picking up medication at the pharmacy.

What do you need to get done this week?

#KidneyDisease #KidneyTransplant #LiverTransplant #Transplant #OrganTransplant #kidneyawareness #KidneyStones #KidneyPain #KidneyProblems #RareDisease #ChronicIllness #CKD #ChronicKidneyDisease #PrimaryHyperoxaluriaType1

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Broken Hearted in Nevada: Julie's Story

Part 1 of 2 Congenital heart defects are the most common birth defect in humans, affecting 1% of the world’s population. In the United States alone, 40,000 babies are born with CHD every year.1. Of those babies, about 25% will have a critical CHD, requiring surgery in the first year of life. There is no cure.

In the last few decades, advances in surgical techniques and research mean that today, about 95% of those born with non-critical CHD and 69% of those born with critical CHD will live to see adulthood. Those medical advances were largely in the pediatric space and focused heavily on survival.

In recent years, estimates show that over 1.4 million adults are living with CHD in our country. But for this population, the U.S. medical system is woefully underprepared to treat them. You may have seen stories from actors, politicians, or athletes living with CHD who seem to have no issues receiving appropriate care, but what about everyone else?

Meet Julie, a 53-year-old woman living with critical congenital heart defects. She is a daughter, twin sister, and loving mother. Julie has miraculously outlived every grim prediction doctors made in her early childhood, despite the odds being overwhelmingly stacked against her.

For babies like Julie, born with congenital heart defects between 1970-1974, the biggest hurdle was surviving the first year of life. If they did reach their first birthday, the chances of surviving to adulthood were just 77-83%.

Heart surgery on infants did not become commonplace in the United States until the early 1970s. After a year of infant CHD surgery at Boston Children’s Hospital in 1972, surgeons had some data to work with. They realized that babies with critical CHD who died generally did so in the first few weeks of life. The rest of that decade saw a shift from surgery in infancy to surgery in the newborn period for these babies, with the first successful procedure coming in 1983 on an 11-day-old.

Julie had her first open-heart surgery at the age of 5 1/2 in 1976. Her childhood was filled with summer vacations, school, birthdays, and lots of visits to the pediatric cardiologist. Because there were no specialists for adults with congenital heart defects until 2015, Julie continued to see her pediatric cardiologist as an adult. Her heart remained stable until her mid-20s.

In 1995, at age 25, Julie gave birth to a beautiful, healthy baby girl. Researchers had barely begun to study adults with CHD and no one told Julie she should have additional cardiac care and monitoring during pregnancy (probably because they didn’t know yet). Standards of care for adults with CHD were not issued until 2008 with the next update coming in 2018.

After the birth of her daughter, Julie’s heart really struggled. By age 26, in 1996, she was back in the hospital for her second open-heart surgery. There was no road map for Julie’s medical team to follow and they were dealing with a long list of heart-related issues in her body. Not to mention, Julie lived in Nevada, where the healthcare system could barely serve its healthy population.

The surgery did not go as planned and took far longer than anticipated. As the procedure entered its seventh hour, extensive bleeding and the length of time on the bypass machine forced the surgeons to place Julie into a coma, with an open chest. She wouldn’t wake up for almost two weeks.

What happened during her 13-day coma is largely unknown. Medical records were not easy to access, and communication from doctors to family members was limited. Julie woke from the coma completely paralyzed on her right side, unable to hold, feed, or care for her young daughter. She didn’t know it at the time, but this would be a turning point for her health.

Facing yet another uphill battle in her young life, Julie persevered and recovered function on her right side with months of intensive therapies. She would go on living life, raising her daughter, and continuing to follow up with her pediatric cardiologist.

As she aged, additional health conditions arose and everyday tasks became more difficult. Julie could feel that something was happening in her body, but unfortunately, Nevada still did not have any adult CHD specialists that could investigate. Despite the herculean efforts of her pediatric cardiologists, Julie declined into heart failure and would require a full evaluation by a specialized adult CHD center.

The team at UCLA’s Adult Congenital Heart Disease Center confirmed Julie’s heart failure diagnosis, but she was blindsided by the severity of her disease. Without knowing they even existed, Julie never had the chance to use the ACHD care standards for her anatomy, and was now looking at evaluation for a heart transplant.

If Julie’s life were a feature film, it


Broken Hearted in Nevada: Julie's Story

Part 2 of 2 would have been at that moment when the screeching tires sound filled the room. A heart transplant?!

While processing that earth-shattering news, Julie was reminded that her home state did not perform heart transplants. In fact, with only one transplant center, Nevada could only perform kidney and liver transplants. She would have to leave her home, her family, and her support system to survive. And she is not the only one in this terrible position.

In October of last year, UCLA physicians told her there was no more time.

Read the full story:

Broken Hearted in Nevada - A+J Patient Advocacy

Julie was forced to leave home in order to receive life-saving care. She is truly fighting for her life with every heartbeat.

Why are we accepting poor infection control?

While I am thrilled that the pandemic phase of Covid 19 is over, the complete lack of infection control in medical settings is nothing for anyone vulnerable to Covid 19 to feel happy about.  I was born without a whole immune system.  What I have is a rare antibody deficiency disease.  I don’t make enough antibodies to fight infection normally.  It’s easier for me to get sick, I can become sicker from infections, and I do not always have a normal vaccine (or antibiotic) response.  Avoiding infections is a crucial part of my staying healthy.

I caught covid last July from a routine doctor’s visit.  I had 4 doses of vaccine, I wore a properly fitted mask and I did not remove it for even a second.   There were no other patients in the office.  The waiting room was empty but I sprayed my pocket-sized Lysol anyway.  I was immediately taken into an exam room by an unmasked assistant.  I sprayed the exam room a total of 3 times with Lysol as I waited for my doctor.  Any more than that would have been overpowering.   My doctor wore a mask and everything went well so my visit was brief.  When I left used hand sanitizer until I got home and washed my hands.   48 hours later my symptoms began.

My point in telling how I caught covid is not to debate vaccines, or masks but to be clear that I went above what most patients do to avoid infection during a doctor’s visit.  My mini bottle of Lysol is seen as a bit brazen by most standards.   I am unable to avoid infection all by myself.

I currently live with a handful of chronic conditions.  Each condition has its own specialist.  How am I supposed to get through all my appointments without getting sick?  Covid is still circulating all year round and the vaccines don’t do much to prevent infection.  I am up to date on my boosters and I still mask indoors, but I know it’s not enough.  And so I am delaying my appointments and routine tests and care as much or even more now than I was during the pandemic.  I have health insurance but I don’t have full access to my healthcare unless I want to be sick all the time,  risking the health I have fought so hard for my entire life.

I am sharing this today because of even worse stories I have been hearing lately.  One was by a distraught wife whose husband was in the ICU and had a lung transplant.  She and her family were wearing a mask to visit her husband but the hospital staff caring for him were not!   And this is why I am speaking up today.

It’s not just people with rare diseases like me who may be vulnerable to covid.  The elderly, organ transplant patients, cancer patients and others with existing conditions are all more likely to have problems from covid.  But let’s be honest here.  Covid is not healthy for anyone.  It’s inconvenient for everyone and so it is understandable that most people don’t want to take precautions.  However, an ICU is not a pizza parlor, mall or movie theater.    Hospitals exist to help people get well.  Anyone requiring ICU care is very sick and under no circumstances should anyone be careless with their health and safety.  Maskless medicine when working with with at-risk patients is wreckless & sloppy.   Why as patients, as human beings are we accepting sloppy medicine?

Even aside from covid what about all the other respiratory infections- RSV, Flu, and Strep throat?  So many infections can be passed to patients by healthcare workers before they have symptoms.  Better infection control protocols have been desperately needed.  Hospital-born infections have always claimed lives.  When as patients are we going to demand better care?

My condition is rare but it affects 250,000 Americans.  If you add HIV patients, cancer patients, our elderly, all our immune-compromised & Immune suppressed we are not a small group.  What we need is for our patient organizations that represent us to speak up.  We deserve patient-centered care that does all it can to avoid harming us while treating us.  All human beings can benefit from better infection control in medical settings.  The idea of preventing the spread of respiratory viruses in healthcare settings may seem radical, but if we look back to the early 1800’s expecting your doctor to wash his hands would have been controversial.  Medicine has come a long way since then, but Covid has taught me we still have so much further to go.

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A Common Guide to Hyperparathyroidism

Part 1 of 3 A Common Guide to Hyperparathyroidism:

Most people know that they have a thyroid gland, but the parathyroid glands that sit just behind the thyroid get less attention. The four parathyroid glands are about the size of a grain of rice, and they perform an essential function: releasing parathyroid hormone (PTH), which regulates calcium, magnesium, and phosphorus levels in the body.

For a snapshot of what this looks like, let’s take a look at calcium. This mineral plays a crucial role in bone strength, proper muscle contraction, nerve function, and blood clotting. Roughly 99% of the body’s calcium is stored in the bones. The other 1% is in the blood and tissues. The parathyroid hormone helps balance the amount of calcium in the blood. If the blood calcium level drops, PTH will prompt the bones to release calcium into the bloodstream.

When more calcium is needed in the bloodstream, PTH helps release the mineral from our bones so it can enter the bloodstream and travel to the tissues that need it. Additionally, PTH can use vitamin D to help the intestines absorb calcium from food and helps prevent the kidneys from flushing too much calcium in the urine.

What is Hyperparathyroidism and Who Does it Affect?

Hyperparathyroidism is when the parathyroid glands stop working as they should, producing an excess of parathyroid hormone (PTH). This overabundance of PTH plays a role in pulling too much calcium from the bones, which can cause bone fragility and osteoporosis. Excessive calcium in the bloodstream can also cause kidney stones, joint and muscle pain, fatigue, abdominal upset, depression, and a general feeling of discomfort.

For reference, a normal parathyroid hormone level is 14 to 65 picograms (pg) per milliliter (mL). A normal blood calcium level is 8.6 to 10.3 milligrams (mg) per deciliter (dL).

Hyperparathyroidism tends to affect people over the age of 60 and women in particular. Other risk factors for the condition include people who have had radiation therapy in the neck area, people with ongoing, acute Vitamin D or calcium deficiencies, and patients who take lithium. There are three main types of hyperparathyroidism: primary, secondary, and tertiary.

The Difference Between Primary, Secondary, and Tertiary Hyperparathyroidism:

Several factors can influence how much parathyroid hormone the parathyroid glands produce. Each type of hyperparathyroidism can have different symptoms and causes. Knowing the difference between the three can help patients decide which type of care to seek out. It’s important to seek out medical care if you are experiencing any of the symptoms of HPT. Left untreated, HPT only gets worse, causing bone, kidney, and even heart problems.

Primary Hyperparathyroidism:

With primary hyperparathyroidism, the cause is an enlarged parathyroid gland that produces too much PTH, causing blood calcium levels to rise. Parathyroid gland enlargement can be caused by:

Adenoma: An adenoma is a benign tumor, which is usually present only in one gland. This type of enlargement causes approximately 85% of hyperparathyroidism cases.

Hyperplasia: The presence of abnormal cells in each gland is called hyperplasia. Hyperplasia causes all four glands to enlarge and malfunction, and roughly 15% of hyperparathyroidism cases are caused by this condition.

Parathyroid Cancer: Most hyperparathyroidism patients do not have parathyroid cancer, as cancerous cells cause less than 1% of all HPT cases, but it is a potential cause of hyperparathyroidism.

The most common symptoms of primary hyperparathyroidism are frequent urination, constipation, excessive thirst, joint pain, abdominal pain, lethargy, weakness, bone pain and/or fragility fractures, nausea, vomiting, and memory loss.

Because primary hyperparathyroidism is caused by abnormal cells or growth in one or more parathyroid glands, the most effective treatment is surgery to remove the dysfunctional gland. While the idea of neck surgery can be intimidating, a skilled parathyroid surgeon can perform a minimally invasive parathyroidectomy with intraoperative parathyroid testing to ensure a high level of success. With a minimally invasive parathyroidectomy, surgeons utilize local anesthesia instead of general anesthesia, meaning patients do not need to have a breathing tube or stay in a hospital overnight. The incision on a minimally invasive parathyroidectomy is smaller and can be blended into the neck’s natural creases, and recovery time tends to be shorter than it is with a more involved surgery.

Secondary Hyperparathyroidism:

Secondary hyperparathyroidism happens when a condition outside of the parathyroid glands causes all four glands to enlarge and release an excess of PTH. The most common causes of


A Common Guide to Hyperparathyroidism

Part 2 of 3 secondary hyperparathyroidism are severe vitamin D deficiency and kidney failure.

When kidney failure occurs, the kidneys cannot make vitamin D or flush the phosphorus in the body. This is problematic because vitamin D helps the body absorb calcium, and excessive phosphorus can limit calcium absorption. With kidney failure or low vitamin D, a person is at risk of blood calcium deficiency. The low blood calcium level stimulates PTH production in the parathyroid hormones. This constant stimulation, in turn, can lead to overactive, enlarged parathyroid glands.

Patients with kidney failure and secondary hyperparathyroidism may have PTH levels in the hundreds or thousands, which is far beyond the normal upper limit of 65 pg/mL. Without treatment, these extreme PTH levels can cause calcium deposits to form in the muscles and skin (calciphylaxis). This is a serious, life-threatening condition that can lead to ulcers, gangrene, and even death.

Symptoms of secondary hyperparathyroidism are similar to those that occur with primary HPT: abdominal pain, osteoporosis, nausea, vomiting, kidney stones, appetite loss, and weakness.

With secondary hyperparathyroidism, the first line of treatment should be to take care of kidney failure or vitamin D deficiency. Kidney failure is often treated with a kidney transplant or with dialysis. Once the primary cause of secondary hyperparathyroidism has been addressed, medications to help regulate PTH and calcium levels can help restore a more normal parathyroid function. In some cases, however, surgery may be needed.

Tertiary Hyperparathyroidism:

If secondary HPT is left untreated, or if it persists even after a kidney transplant or dialysis, it becomes tertiary hyperparathyroidism. Because tertiary HPT is essentially advanced secondary HPT, the symptoms can be more severe and may include:

Uncontrollably itchy skin (pruritis)

Bone pain and fractures

Vascular or soft tissue calcifications

Inflammation in the pancreas (pancreatitis)

Kidney stones

Calciphylaxis, a life-threatening condition

Parathyroid surgery is required to treat tertiary hyperparathyroidism.

Next Steps After Being Diagnosed with Hyperparathyroidism:

Once you seek out medical care for your HPT symptoms, your doctor can order blood tests and perform scans to confirm a diagnosis. Then, you and your doctor can discuss the best treatment options.

Being diagnosed with hyperparathyroidism can be overwhelming. The good news is that a highly skilled parathyroid surgeon can help cure primary hyperparathyroidism and treat secondary and tertiary hyperparathyroidism once the underlying issue has been addressed.

Primary hyperparathyroidism caused by a parathyroid adenoma is the most common form of HPT. Minimally invasive parathyroidectomy can treat this type of HPT in most cases because the surgery has a high cure rate.

This is especially true if the parathyroidectomy is performed by a surgeon who does rapid intraoperative PTH testing, in which the surgeon tests the patient’s parathyroid hormone before the surgery to get a baseline level and then again after the parathyroid has been removed—but before the surgery is completed. Rapid intraoperative PTH testing helps the surgeon make sure that the remaining parathyroid glands are functioning normally. If they aren’t, additional steps can be taken to treat the issue while the patient is still in surgery, thus minimizing the need for additional surgeries and ensuring a higher cure rate.

At the CENTER for Advanced Parathyroid Surgery, Babak Larian, MD, FACS, uses intraoperative PTH testing and has a parathyroidectomy cure rate above 97%. He also treats primary hyperparathyroidism caused by parathyroid hyperplasia and parathyroid cancer. Dr. Larian can also perform surgery if needed for secondary or tertiary hyperparathyroidism.

Parathyroid surgery can cure your hyperparathyroidism and bring you relief from the debilitating symptoms that come with it, often within just two to four days after surgery. You’ll be able to go home the same day and return to your normal activities within one to three weeks.

To learn more about how to lessen your HPT symptoms, contact Dr. Babak Larian of the CENTER for Advanced Parathyroid Surgery. His goal is to make every patient he treats feel welcomed, listened to, and fully informed before, during, and after parathyroid treatment.


Parathyroid Surgeon Beverly Hills, Los Angeles | Dr. Babak Larian

Dr. Babak Larian is a board-certified parathyroidism surgeon in Beverly Hills, CA. He specializes in minimally invasive parathyroid surgical techniques.

I Nearly Died Last Year and Now I Have To Do The Same Thing On The Same DayA Year Later-So Anxious!

Last year on May 19, 2022 I went in for my routine Remicade infusion and bloodwork. I had been told my liver enzymes were a little elevated but nothing that they felt I needed special medication or a new specialist added to my team. The day after my infusion I felt really bad, but I thought it was because I was a having a really stressful time at work. I woke up on Saturday morning with pain at the bottom of my sternum. I thought it was heartburn so I took some medicine, it didn’t work. I started having trouble breathing so I used my inhaler which helped with my breathing. Later in the evening I had to use my heartburn meds again. I started checking myself for heart attack symptoms-none, but the pain increased. I eventually got to the point of tears I was in so much pain. My fiancé (now husband) was cooking supper when he came in the living room and saw me crying he dropped everything and took me to the hospital. I was in liver failure with enzymes near 1,000. (If you’re unfamiliar with what’s normal it’s 50-55.) The next week I was in the hospital but don’t remember a lot. Over the next couple months I had tons of blood draws, scans, and a biopsy. I was told if I didn’t have a transplant I was going to die. I was diagnosed with Autoimmune Hepatitis. It was an emotionally and physically draining summer and then I started back to teaching the best I could with enzymes that started going down for no reason. I stopped Remicade and started Rituxan in November. This coming Thursday I have my next Rituxan infusion which will exactly one year from when the nightmare started. I start crying when I think about going for my infusion. I’m so scared about getting it. I know my enzymes are ok but I can’t stop feeling anxious about it.
I know I’m going to be ok. I just needed to vent about it. Thank you for reading this far. #AutoimmuneHepatitis #RheumatoidArthritis #Lupus #ComplexRegionalPainSyndrome #chronicmigraine #remicade #rituxan #Fibromyalgia #ChronicIllness #ChronicPain #Anxiety

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I’m new here!

Hi, my name is odderprod. I'm here because I have Lupus and Vasculitis which has put me in End Stage Kidney failure. I had one failed kidney transplant last year. Hoping to hear other people’s stories.

#MightyTogether #VasculitisSyndromesOfTheCentralAndPeripheralNervousSystems #ChronicKidneyDisease #Lupus

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Painful bladder syndrome (interstitial cystitis)

Has anyone ever been diagnosed with this? I have been to the doctor many times with what I thought was a bladder infection. After my urine was tested it showed no sign of any infection. I had a kidney transplant years ago so I take no chances with that part of my anatomy. My kidneys were damaged because I developed an autoimmune disease called Granulomatosis with Polyangiitis.