Wednesday, January 30, 2013

November 28 through December 7, 2012 - A rash of new issues

This post covers a little over a week, and we'll deal with one old issue while some new ones come up.

November 28, 2012 (Wednesday): At dialysis today they told Mom that her catheter may have an infection and may need to be replaced. She has an appointment next week (December 3) with Dr. K to see if her fistula is ready to use. It would be soooo nice to get rid of that catheter for good.

November 29: I went with Mom to her appointment with Dr. C to review her recent bloodwork and discuss a plan of action regarding her allergic reaction to heparin. Here are the highlights:

  • Mom's factor X is normal, but her blood is already thin. Dr. C reiterated that an anticoagulant is needed to keep the fistula open.
  • The risk of using Coumadin with someone like Mom who already has thin blood is cerebral hemorrhage. The increased risk of cerebral hemorrhage when a "normal" person is put on Coumadin is 1% to 2%, but there is no way to know what Mom’s increased risk would be.
  • The risk of not using Coumadin is the fistula closing up due to clotting.
  • Dr. C asked Mom to have her nephrologist call him from the clinic tomorrow (Friday). He said it really needs to be a group discussion on how to proceed.

At the end of this appointment Mom asked Dr. C  to look at a rash that recently developed on her torso. She first noticed it Sunday while she was in Austin. Dr. C took a quick look and said it looks like a drug reaction. They had given Mom an antiobotic at dialysis to combat infection on November 19, and supposedly they have continued giving that to her. So that's probably it. Dr. C suggested she show them the rash at dialysis tomorrow.

November 30: Mom’s rash has gotten worse and it really itches. She’s going to see a dermatologist at 2:00 PM today. Dialysis has been rescheduled to 5:45 AM tomorrow (Saturday) because she's too miserable to be still for three and a half hours of dialysis. But she did go to the dialysis clinic to talk to the nephrologist, Dr. N. He is taking her off the antibiotic (vancomycin) and putting her on an oral antibiotic. He said they gave her a larger dose of vancomycin on Wednesday of this week, so that might explain why the rash got so much worse Thursday evening.

Dr. N had already talked to Dr. C about the Coumadin situation, and they agreed not to put her on Coumadin. I guess that means she won’t be on any anti-coagulant, and they’ll hope for the best on the fistula staying open.

The dermatologist gave Mom a prescription for some pills and a cream. The bottle of 48 pills would cost $5000 without insurance. Mom paid $25. The pharmacist said he was prescribed the same pills once and his insurance company didn’t want to pay, but they changed their mind when he told them they could pay for his hospitalization to be given the drugs intravenously if he didn’t take the pills.

December 1 (Saturday): Mom had dialysis early this morning but still was unable to make it to a school band concert because she didn’t get done until 10 AM and her skin rash was really bad. She’s miserable due to this rash all over her torso.

December 3: Mom’s rash is still bad. Her Monday dialysis appointment was moved to Tuesday. She had an appointment this morning with Dr. K about the fistula. He said he needed to put in a stent to expand one part. This procedure has to be done in the hospital on an outpatient basis, and it will be scheduled this week or next.

December 4 (Tuesday): At dialysis today, Mom’s nephrologist said she needs to have the permacath replaced because he thinks it might be infected, and he wants the new one inserted on her left side instead of her right side. That will be done on an outpatient basis at Baylor Heart Hospital this Thursday. The stent procedure for the AV fistula has been scheduled for Tuesday of next week.

Mom’s rash isn’t getting any better. Dr. N said it takes about five dialysis treatments to rid the body of the antibiotic that she apparently had the allergic reaction to. Her last dose of that antibiotic was Wednesday, November 28. Today’s dialysis is only the second one since then. The nurse at Mom’s dermatologist also said it will take awhile for the rash to go away, and she’s not surprised it isn’t any better yet. Wonderful. Have I mentioned how miserable she is?

December 6: Mom’s permacath was replaced today, but the new one was not inserted in the other side. The surgeon said there wasn’t any need to do that. I hope Dr. N is ok with that. At some point Mom overheard the nurses talking about giving her Heparin, and she told them she was allergic to it. I wonder what would have happened if Mom had not heard that discussion?

December 7: Mom mentioned yesterday’s Heparin incident to Dr. N, and he said that info was in her chart. He suggested they get her a bracelet to wear that indicates she’s allergic to it, since she may not be able to communicate that in an emergency situation.

In summary:
  • Mom can't take an anticoagulant because she is allergic to one (heparin) and it's too risky to put her on the other one (Coumadin). Hopefully her blood will be thin enough such that her fistula does stay open if they ever start using it.
  • Her permacath has been replaced again, this time due to an infection.
  • The first antibiotic they gave her for the infection caused a really bad skin rash, and it's taking a long time to get rid of that.
  • Next week she'll have a procedure done to help one part of her fistula stay open.

Will we ever start using the fistula? Stay tuned . . .

[Edited 6-18-13 to replace doctor's names with initials.]

Sunday, January 27, 2013

November 20, 2012 - Ticks and Fleas

Today's post covers November 16 through 26, 2012.

November 16, 2012 (Friday): I don't have any notes on Mom's dialysis from Monday and Wednesday of this week, so things must have been going smoothly. You know what that means, don't you? Something is about to happen. Sure enough, at today's dialysis appointment Mom's nephrologist (Dr. N) told her 
that based on her recent lab work he thinks she is allergic to heparin, which is typically used as an anticoagulant in dialysis machines. He suggested changing over to Coumadin, but thankfully Mom knew that she shouldn't be on Coumadin either because of how thin her blood is and how easily she bruises. Dr. N said she needs to see a hematologist, so she has an appointment with her oncologist/hematologist Dr. C for Tuesday, November 20. (He is the same Dr. C that she saw back in early 2010 right after she was first diagnosed with amyloidosis.)

November 19, 2012: Mom had a slight fever (99) at the beginning of today’s dialysis. After about an hour she realized she was freezing cold and shuddering, so she got the attention of a tech. He brought her a blue glove filled with warm water, which helped a little but not enough. Then she mentioned she had a slight fever at the start, so they took her temperature again and it was 101.6. They believed she had an infection so they gave her some antibiotics. They kept her there for awhile after dialysis because they knew she wasn’t doing well.

November 20, 2012: I went with Mom to her appointment with Dr. C today, regarding her allergic reaction to heparin. Here are the highlights:
  • If you’re on dialysis you must be on some sort of blood thinner, otherwise the fistula can start clotting up and restricting blood flow.
  • Although Mom's bruising would be a concern if she were put on Coumadin, the main concern would be the increased risk of cerebral hemorrhage.
  • She may have something called a factor X deficiency, which has to do with blood clotting. (That's a Roman numeral 10, not the letter "X.") Whether or not she has that will give us some direction on how to proceed.
  • If she has to go on Coumadin, she would need to be hospitalized for some time period to prep her body for it. Once on Coumadin, she may be put on a small dose instead of a standard dose, since her blood is already thin.
  • She had blood drawn after today’s appointment so they can determine the level of various factors in her blood. We’ll proceed from there.
So now there is a potential complication with hemodialysis, and there may not be an easy answer as to how it should be addressed. As Dr. C put it, Mom may have ticks and fleas (two rare conditions that have to be dealt with). I felt comfortable that Dr. C knows what he's doing and is not going to rush into anything. He said that once he determines what is really going on with Mom's blood, there may need to be a group discussion between us, Dr. C and Dr. N (nephrologist) to weigh the pros and cons of the possible alternatives.

Since this was the first time we had seen Dr. C since before Mom's diagnosis from Boston, I did make a point to thank him for not prematurely treating Mom for AL amyloidosis back in 2010. I told him familial amyloidosis is frequently mistaken for AL amyloidosis, and when those patients are given chemotherapy it causes polyneuropathy.

November 21, 2012: In kidney transplant news, TUD (The Unknown Donor) is compatible. However, TUD's blood pressure is borderline so they are going to send a blood pressure measuring device to measure the blood pressure for a time period. The device logs the measurements internally and the pre-transplant group downloads the data for analysis after they receive the device back. Mom still does not know there is a potential living kidney donor being evaluated.

Mom drove to Austin to spend Thanksgiving weekend with Ed and his family, which meant she had to schedule her first out of town dialysis sessions for Friday and Monday, November 23 and 26. (There is a person at the dialysis clinic who does this scheduling for patients when they travel.) Unfortunately the closest clinic they could get her in was about 20 miles away and the times weren't very convenient. I suppose you have to take what you can get when traveling on dialysis. But both dialysis sessions went fine and there were no issues. So it's good that she has traveled away from home for a few days and we know it's possible.

Next up: Of course something else can go wrong. Why do you ask?

Thursday, January 24, 2013

November 10, 2012 - Support Group Meeting

Short post for today. First, some real time news. I recently heard from our London correspondent, Nicola. She has been participating in the CPHPC trial through the National Amyloidosis Centre in the UK for almost four months, and her latest lab results show either improving or stable kidney function. Hooray for good news! Let's hope this positive trend continues.

And in even more up to date real time news, the first reported case of fibrinogen amyloidosis in Latin America has been reported, in a 52-year-old Brazilian woman. Here's a link to the abstract of the article, which was published online yesterday:

Now back to the not so distant past . . .

On November 10, 2012, Mom and I attended the local amyloidosis support group meeting. I believe this was only the second meeting Mom had ever attended. I counted a total of 48 attendees at this meeting, including Dr. B from Mayo Rochester, Muriel Finkel, and 21 patients (4 localized, 3 familial, and the rest primary). That was the largest Dallas support group meeting I have ever attended, and I think we actually exceeded the official capacity of the room. Two new patients really stood out today.

First was a 32 year old man who had AL amyloidosis with heart, kidney and GI involvement. He was undergoing chemo treatments and was in a wheelchair. It’s always a jolt when you see or hear about someone so young getting amyloidosis.

Then the saddest case today was a man in his fifties who was very healthy and active up until earlier this year when he came home from an exercise session at the gym and could not catch his breath. He has heavy cardiac involvement, can’t walk 30 feet without resting, and has lost 70 pounds this year because he can hardly keep any food down. Watching him come back into the meeting room after lunch was like watching a very frail, much older person trying to walk a few steps after getting out of a wheelchair. It was heartbreaking to hear his daughter tell their story, and at one point she basically asked if she would ever get her father back.

Next up: Can anything else go wrong with dialysis?

Monday, January 21, 2013

October 29 - November 9, 2012 - Getting stressed

This post will cover the weeks of October 29 and November 5, 2012. Hopefully less blood will be involved than last week.

Speaking of cats, those of us with fibrinogen amyloidosis apparently have something in common with some of our feline cousins in the animal kingdom. I recently came across an article with the title "Familial Renal Amyloidosis in Abyssinian Cats."

October 29, 2012 (Monday): Mom had a previously scheduled appointment with Dr. K today so he could evaluate her fistula. He was the surgeon who inserted her original permacath as well. He had already been notified about the permacath replacement that was done on Friday, but he didn't have any details so Mom got to tell him all about that. (I don't think I mentioned it in the last post, but nobody really knows exactly what happened to cause that massive bleeding incident.) Dr. K said the fistula looked good so she might get to start using it earlier than originally planned. Mom then went to dialysis, which went well although she got really cold during the last hour.

October 31: Dialysis went well today, and Mom felt well enough to go over to my sister's house for Halloween, which is always a lot of fun. Here's a picture of me with my son and one of my nephews. I'm the tall one in the middle.

Halloween 2012
Halloween 2012

November 5, 2012 (Monday): Laura called me this afternoon and said she was going to pick up Mom from dialysis. She had called Mom to check on her and Mom sounded really bad and said they were bringing her some chicken broth. Laura asked Mom if she wanted to be picked up, and she said yes. Laura picked her up and found out her blood pressure had gotten low. Laura took her home and she went to bed and had a hard time staying warm.

November 6: TUD (The Unknown Donor) had blood drawn today. TUD is being told it’s about a 60 day process to be evaluated.

November 7Mom said today’s dialysis session was interesting. They laid her down for dialysis instead of sitting her up. Everything went fine until there was less than 30 minutes left and the machine started beeping. It seems as though it was clogged, and the nurse was concerned because that’s kind of like the blood clotting. But they got it taken care of and she felt fine when she got up, in spite of lying down for so long.

November 8: Mom had a stress test this morning, and I went with her to her cardiologist appointment this afternoon to review the results. The stress test was scheduled because her nephrologist recommended it back when she had some tightness in the chest during the October 19 dialysis session. That was when they slowed the flow rate down from 400 to 350 mil/min (milliliters per minute). She has not had any more chest tightness since then, and she always tells the tech to set it to 350.

(Side note: At some point in the past her cardiologist (Dr. R) determined that she could not do a physical stress test, but would instead have to do a chemical stress test. That’s where medication instead of exercise is used to increase the load on the heart.)

Besides being interested in the results of the stress test, I also wanted to make sure Dr. R was aware that although it is rare, fibrinogen amyloidosis can affect the heart. (See this article: Mourad, 2008)  Before today's appointment I had written to Dr. Berk in Boston asking him some questions about the proactive use of echocardiograms on patients with fibrinogen amyloidosis, both with and without renal involvement. He wrote me back and I printed out a copy of that email exchange to show Dr. R. Dr. Berk basically said periodic echocardiograms or cardiac biomarker measurements should suffice for monitoring cardiac function (about every two years unless otherwise warranted), and he also recommended having echo images reviewed by a cardiologist well versed in amyloid.

Dr. R said the stress test looked fine and there was no sign of blocked arteries. I explained that patients with fibrinogen amyloidosis can, on rare occasions, have heart involvement. He read the email from Dr. Berk and I gave him a copy of Mom’s 2010 Boston evaluation, which included an echocardiogram. We talked a little about detecting amyloids in the heart, via echocardiogram and biopsy. He was aware that the ejection fraction could be fine in a heart affected by amyloids, so I was comfortable he knew what he was talking about and I didn’t need to educate him any further or ask him to consult with the doctors in Boston. He said he didn’t think there was any need to do an echocardiogram at this point since the stress test and everything else indicated there weren’t any heart problems.

So there was some good news today. Since Mom is still being dialyzed through the catheter in her chest, we’re hoping things will improve once she switches over to being dialyzed in her arm.

November 9: Mom had a good day at dialysis today, but her hemoglobin is down to 7.9, and she doesn’t really know if they have been giving her Epogen at the dialysis clinic. That's something she'll have to stay on top of and possibly become a bit of a pest about at the dialysis clinic.

Next up - Support group meeting 

[July 12, 2013: Minor edit to remove Dr. R's name]

Friday, January 18, 2013

October 26, 2012 - "I have a bit of an emergency."

This post will cover the week of October 22, 2012, which was Mom's second week of dialysis.

October 22, 2012 (Monday): Dialysis did not go well today. The first half was ok, but later in the session Mom got cold again. Since she’s using the permacath in her chest, the dialysis machine turns off whenever she coughs or moves her shoulder a certain way. Then she has to wait for a technician to turn the machine back on. It seemed like they were short handed today so she spent a lot of time waiting for the machine to be turned back on. Her blood pressure was very low when she initially stood up after dialysis.

October 23: All of the supplies for peritoneal dialysis were picked up from Mom’s house today. (These supplies were delivered October 3, but never used.) The only thing they did not take back was the IV pole. [Insert your own joke here.]

October 24: Mom drove herself to and from dialysis today. She said it went very well. The following day (Thursday, October 25) she was feeling pretty good and drove around running some errands. So finally she has a good day at dialysis. Let's see how long that streak lasts . . .

October 26: Mom called me from the dialysis clinic at 11:28 AM saying she had a bit of an emergency. Her catheter was bleeding and she needed a ride to a clinic in Plano to have it taken care of. My sister Amy was at a job and my wife Cathy was out running errands. I called Cathy and she said she could go pick up Mom. After I sent a text message to my sister Laura to let her know what was going on, she called me back and said she could take Mom. I couldn’t get Cathy turned around soon enough, so Cathy arrived at the dialysis clinic only minutes before Laura did.

They decided Laura would take Mom to the clinic in Plano, and Cathy would get Mom a clean shirt from her house. Cathy said Mom apparently bled a lot and seemed a little disoriented. So what the heck happened?

Mom said she noticed a little bit of blood on or around the dressing of the permacath this morning before dialysis, and she told the technician about it. The technician got her hooked up to the machine and then went around to turn it on. Then she came back and looked at Mom, declared “Oh my God,” and yelled for help as she turned off the machine. Apparently Mom was bleeding profusely at the point where the permacath enters her chest. Mom said she ended up with five people around her trying to get the bleeding to stop and it took quite awhile as they would take turns applying heavy pressure to her chest.  She said she expected to start passing out due to the blood loss, but she didn't.

So now Laura is at the clinic with Mom, and Cathy is stopping by Mom's house to get some non-bloody clothes for her to wear when she leaves the clinic. Laura said that according to the physician's assistant (PA), the x-ray showed the catheter was too short, and that could explain some of the difficulty she has been having with dialysis. (According to Laura and Cathy, the PA was hilarious throughout the whole visit.) The PA also said they use a different type of catheter at that clinic (two prongs vs. one, I think). They decided to replace the catheter, which is a 20-minute procedure done with local anesthetic.

After the catheter was replaced, Laura took Mom back to the dialysis center and stayed about 30 minutes. Cathy took me there with Mom’s car key around 7 pm, and then Cathy and Cliff went on to Mom’s house to wait for me and Mom. After dialysis Mom had no trouble walking out to her car, and I took her home. She seemed just a little tired from the day’s drama, understandably so. She seemed fine when we left her house a little after 8 PM and said she would probably just go on to bed soon.

What a day. It's a little scary to imagine how this easily could have been a lot worse. Thank goodness the tech saw the bleeding as soon as it started. If Mom had already been covered up by a blanket the first sign of trouble might have been blood dripping on the floor. Incidentally, as of this writing, three months after the incident, Mom says she has not seen that same tech back in the clinic.

On a positive note, we were able to clean Mom's bloody shirt (one of her favorites) and brand new bra. The people at the dialysis clinic told Laura and Cathy that they soak bloody clothes in hydrogen peroxide to get the blood out, so Cathy bought several bottle of that while she was out. We went out to the garage and set up shop in the back of my pickup truck. I naturally had to take pictures of the whole process. The hydrogen peroxide bubbled like crazy when we poured it into the plastic tub with the clothes, and I could even feel a little warmth coming off the bubbles if I held my hand right over the top. (Hooray for exothermic reactions!) We gave them two soakings in the hydrogen peroxide, then Cathy washed them in the laundry. Here are before and after pictures of her shirt:

Bloody shirt before
Bloody Shirt
Hydrogen peroxide bubbles
+ Hydrogen Peroxide

Bloody shirt after
= Clean Shirt

October 27 (Saturday): In kidney transplant news, The Unknown Donor (TUD) has submitted the application to the pre-transplant group but has not heard back from them yet. TUD plans on following up next week. At this point Mom still does not know there is a potential donor.

Maybe we can get through the next week without this kind of drama . . .

Tuesday, January 15, 2013

October 17 through 19, 2012 - First week at the dialysis clinic

Today's post is a short one since it only covers Mom's first week of dialysis at the clinic. Her dialysis schedule is Monday-Wednesday-Friday, and she had dialysis in the hospital on Monday.

In blog news, I have added a search box to the right side of the page so you can search this blog for a specific word or phrase. For instance, if you want to find that blog post about Mom's trip to Russia, just search for "Russia" and you'll get a page with a link to that post at the top. If you want to see all the posts where cats are mentioned, just search for "cat." (Ironically, the hits for those two searches are currently the same.)

October 17, 2012 (Wednesday): Today was Mom's first visit to the dialysis clinic closest to her house. My sister Amy drove her there, and my sister Laura picked her up. Mom was pleased with everyone at the clinic, including the manager who made a point of welcoming her. The only complication today was that her blood pressure got low either during or after dialysis, and they told her that she is not supposed to take her blood pressure medication the mornings of her dialysis. I guess it would have been nice if someone had told her that before her first dialysis appointment. She got a little cold during dialysis and then colder after she got home, so she’ll need to take a heavier blanket next time. She was slightly light-headed after she got home, but not totally exhausted.

October 19: Mom started feeling a little light-headed and had some chest pain early in today’s dialysis treatment. She notified the nurse who slowed down the flow rate of the machine, and she immediately felt better. The nephrologist wants her to have a stress test as a result of that, just to make sure there aren't any cardiac issues. He’s going to contact her cardiologist, and Mom will schedule an appointment with him. She was cold again after dialysis, but she was fine the next day and felt good enough to go to the soccer game of one of my nephews.

That's about it for the first week. We were expecting some minor issues as her body adjusts to being on dialysis and as she learns how to deal with being on dialysis. So far, so good I suppose. Let's see how the second week goes . . .

Saturday, January 12, 2013

Clinical Trials Update (including CPHPC)

This post will give a little update on the CPHPC trial being done at the National Amyloidosis Centre (NAC) in London, and I'll also discuss a trial being done in the US that is relevant for fibrinogen amyloidosis.

But first, if you'd like to meet me or Mom in person, all you have to do is come to the Chicago area in October. We'll be there for the familial amyloidosis meeting October 25 - 27. Details can be found here: I think there were around 175 attendees at the 2011 meeting, and the 2013 meeting already has about 100 people planning on attending.

Back to the clinical trials. This past October, Nicola Lloyd (formerly of South Africa) informed me that she made it to London and has begun the CPHPC treatments, which consist of three daily injections. Hopefully she can report some good results to us within a year. In her email she included some information provided by the NAC about CPHPC and how it functions, some of which I have copied below. Be sure to read the final paragraph, in bold. (Warning: British spelling ahead):

Amyloidosis is due to the accumulation of abnormal protein fibres (amyloid fibrils) in vital organs and various tissues throughout the body. SAP (serum amyloid P component) is a normal blood protein of unknown function that binds to amyloid fibrils, and helps to stabilise them, contributing to progression of the disease. It has been shown that SAP helps to prevent the breakdown of amyloid fibrils, and mice that do not have SAP are relatively resistant to developing amyloidosis in the first place. No ill effects have been attributed to the life long lack of SAP in these mice.
CPHPC is a drug that is given daily by subcutaneous injection 2 or 3 times a day. It is absorbed into the bloodstream where it binds selectively to pairs of SAP molecules, which are then recognised by the liver to be abnormal and are removed from the blood. CPHPC treatment thus depletes the blood of SAP protein and, in turn, SAP that has accumulated in the amyloidotic organs gradually diminishes. The hope is that removal of SAP from amyloid will result in the body clearing away amyloid more quickly and efficiently, although this has not yet been proven in man.
In hereditary renal amyloidosis, which is inexorably progressive, the amount of healthy protein being lost into the urine fell in four out of five patients receiving CPHPC, and kidney function was prolonged compared to a historical group of such patients.

So there we have a not-too-technical description of what CPHPC does in conjuction with SAP. The initial study of CPHPC in 31 human patients can be found here. Since there were fibrinogen amyloidosis patients included in that study, it is listed on the resources page of this blog under March of 2010. (Sustained pharmacological depletion of serum amyloid P component in patients with systemic amyloidosis)


The CPHPC trial is a great opportunity for people living over there in Jolly Olde England, but are there any drug trials available over here in the good ol' US of A? As a matter of fact, there is one. Boston University is currently recruiting participants for a trial on doxycycline. Doxy who? "Doxy cycling," but change the "g" to an "e". Here is a link to the information about the trial:

Here is a link to a short abstract about doxycycline presented at the American Society of Hematology annual meeting in December of 2012:

And here is my attempt at a non-technical one-paragraph summary of what this is all about:

AL amyloidosis (primary) is often treated by having patients undergo a stem cell transplant. (The other main treatment option is chemotherapy.) Patients are given antibiotics after a stem cell transplant, with penicillin being the number one choice. But patients who are allergic to penicillin are typically given doxycycline instead. The Mayo Clinic reviewed the records of 455 patients who received stem cell transplants between 1996 and 2011, and found that those who received doxycline tended to survive longer than those who received penicillin. Couple that information with some other data indicating that doxycycline can disrupt the formation of amyloid fibrils, and now that's something worth studying further.

The description of the doxycycline clinical trial on the web site doesn't specifically mention fibrinogen amyloidosis, but it does mention ATTR. So I wrote to the contact person listed and asked if a person who was diagnosed with fibrinogen amyloidosis, confirmed by kidney biopsy, but still in the early stages of the disease would qualify. Dr. Berk at Boston wrote me back and said yes. He also said he would not recommend the drug to someone who had tested positive for the genetic mutation but did not yet have any symptoms.

As far as I know this is the first clinical trial in the US with something for fibrinogen amyloidosis patients. Up until now the only treatment options were organ transplants (liver, kidney, or both). Unfortunately Mom's kidneys have progressed to the point where she is not eligible for the trial. I am currently asymptomatic so I am not eligible either. (Maybe there will be a preventive medication for fibrinogen amyloidosis someday.)

The existence of these clinical trials does underscore the importance of genetic testing and early detection. It would be a shame for someone to be diagnosed so late in the progression of the disease that the window of opportunity for participating in a clinical trial is closed. So if you're having trouble convincing family members of the importance of genetic testing and regularly having their creatinine and proteinuria checked, let them know that there may be treatment options other than organ transplants, but that window can close up quickly.

Wednesday, January 9, 2013

October 10 through 15, 2012 - Feeling the thrill in the hospital

This post will cover the rest of Mom's hospital stay, which started the evening of October 9, 2012.

October 10, 2012 (Wednesday):  My sister Amy posted the following on Facebook this morning, and our cousin Steven shared it on Facebook shortly after that:

My mother, Linda Jennings, has a rare disease called amyloidosis that has affected her liver and has caused chronic kidney disease. WOULD YOU LIKE TO SAVE A LIFE? Here's how . . . There are significant benefits to living kidney donation. Living-donor kidneys tend to work better, and they have a longer projected functional life than deceased-donor kidneys. The procedure to remove the donor kidney is minimally invasive. The donor typically spends 2 - 3 days in the hospital and resumes normal activities in 2 - 3 weeks. Donating a kidney does not cause long-term health problems. If you are considering becoming a kidney donor, don't hesitate for one second. The blessings far outweigh any temporary discomfort! For more info, please go to

Mom had the surgery to insert the permacath around noon today. The permacath is a catheter that is inserted into a blood vessel in the chest, then threaded almost all the way to the heart. This is what they will use for hemodialysis until the fistula is ready to be used. (More on the fistula in the next paragraph.) As soon as she recovered from that surgery she had her first hemodialysis treatment, and she was back in her room before 5:00 PM.

An arteriovenous fistula (AV fistula) is a connection between an artery and a vein that is surgically created for hemodialysis treatments. They typically prefer to put these in the patient's non-dominant arm, which in Mom's case would be her left arm. However, she had a lumpectomy on her left side years ago and isn't supposed to have her blood pressure taken on that arm or have any blood drawn from that arm. The surgeon, Dr. K, came to talk to Mom about it after her dialysis treatment. He said he'd prefer to use her left arm since she's right handed, but they'll do a vein mapping tomorrow to determine which arm is better.

October 11, 2012: My sister Laura posted the same thing on Facebook that Amy had posted yesterday, regarding living kidney donation. Laura and Amy each have over 500 Facebook friends, whereas I barely break 100 (even with three cats and a fully functional R2D2 as friends), so maybe their posts will generate some interest.

The only procedure today was the vein mapping. The tech who did the procedure told Mom the veins in both arms look fine, so it shouldn't be a problem to use either arm. Let's hope the surgeon agrees. Cathy and Laura visited Mom at different times today, and they both said this is the best they've seen her in months.

October 12, 2012: While Mom was in dialysis this morning, she sent me a text message to look up Dialysis At Sea and see if Royal Caribbean has any cruises on their Allure or Oasis ships scheduled for next summer. To back up a bit, we had been talking about taking a cruise next summer (2013) to the Eastern Caribbean, but with Mom going on hemodialysis now we sort of assumed the cruise wasn’t going to happen. All we knew about was a cruise line that caters specifically to dialysis patients. I looked up Dialysis At Sea ( and discovered they have teams of doctors and nurses that go on certain scheduled cruises and do dialysis right there on the ship. Sure enough, they are scheduled to be on a trip to the Eastern Caribbean on Allure, June 23-30, 2013. I texted Mom back, and later in the day Mom told me that she learned about this from her dialysis nurse Friday morning, because she goes as the dialysis staff on some of those cruises. So our cruise next summer may happen after all.

Mom had the surgery to have the AV fistula put in and the peritoneal dialysis catheter removed this afternoon. The surgeon (Dr. K) talked to Laura and Cathy after the surgery and said everything went well. He was very pleased with the results of the fistula surgery, which he ended up doing in her right arm after all.

I went to visit Mom after work. She was initially a little groggy, but not in any pain. I stayed until about 9:45 PM. Before I left, she did ask for some Tylenol for the pain at the site of the fistula.

October 13, 2012 (Saturday): Mom was informed today that she would not be discharged from the hospital before Monday because they don't have her set up with a dialysis clinic yet. So she'll have dialysis in the hospital on Monday and then hopefully be discharged.

Cathy, Cliff and I went to see Mom this afternoon. She had taken a shower and was dressed in street clothes, including a UT football shirt since she was watching the Texas-OU game on TV. (Texas got slaughtered 63-21.) I got to see and feel the site of her fistula. It's on the inside bend of her right elbow. If you put your finger on it like you're checking for a pulse, instead of a pulse you feel what I would describe as a constant "buzz." That's called a thrill, and it's due to the high rate of blood flowing through it, since an artery is connected directly to a vein.

October 14, 2012: This was kind of a lazy Sunday for Mom, since she was just waiting for Monday to arrive so she could have dialysis and be discharged. She did walk up and down the hall in the hospital a little. I tried to tell her how lucky she was to be able to relax and just order room service all weekend. She did not agree.

October 15, 2012 (Monday): Dr. K checked Mom's fistula this morning and said it looked good. She had dialysis this afternoon, and I took her home around 7:15 PM. She was feeling a little light-headed going into her house and had to sit down quickly. I'm expecting her to be tired after dialysis, at least until her body has had time to adjust. But overall she's doing much better than she has in awhile and it's certainly a relief to all of us that she is on dialysis now.

Next up, we'll see how the first week of dialysis at the clinic goes.

Sunday, January 6, 2013

October 9, 2012 - The Decision

The last post, covering October 8, 2012, was a bit of a cliffhanger since we all went to bed wondering what Mom would decide to do the next day. Peritoneal or hemo? Peritoneal or hemo? Peritoneal or hemo? Today had less activity than yesterday, with only 8 text messages and 90 minutes of phone calls (compared to 15 and 180 yesterday.)

Tuesday, October 9, 2012

I called Mom at 8:13 AM after she sent me a text message to call her. She’s feeling slightly better, but she has decided to do hemodialysis. [And there was much rejoicing.She’ll call the PD clinic at 9:00 AM to find out what to do next. I let Laura and Amy know the good news. 

At 9:28 AM I received a phone call from the nephrologist at the PD clinic, Dr. S. He explained hospital options, checking in today vs. tomorrow, and in which hospital she could be seen by Dr. V, her original nephrologist. I then called Mom and she decided to go today to the hospital where she would be seen by Dr. V. I was hoping she would at least be admitted to a hospital today, so maybe dialysis could start tomorrow. I didn't want her to be admitted tomorrow, and then we'd possibly lose another day before dialysis starts.

At 10:07 AM I called Dr. S and told him Mom would like to go to Presbyterian Hospital in Plano today. He said he would contact the clinic and Dr. V’s office, and someone from Dr. V’s office would call me back. Mom called me at 11:26 AM and said the PD clinic had called her and told her to go to the emergency room at Presbyterian Plano.

Dr. V called me at 11:29 AM. She asked a little about the history and how Mom’s doing now. She said she would contact the ER doc at Presby Plano and let them know to expect Mom, ask them to do some initial bloodwork, etc. She explained that tomorrow morning they’ll likely insert the temporary catheter for hemodialysis in her neck and start dialysis. Hopefully before she is discharged in two to three days they’ll insert the permanent fistula for hemodialysis and remove the PD catheter from her abdomen. I then called Mom and relayed all that to her.

I then arranged to have Laura take Mom to the hospital. Laura took Mom and called me at 2:52 PM. She said Mom was being seen in the emergency room. They took a chest x-ray, and they had a hard time drawing blood because she’s so dehydrated. Laura says Mom is very out of it, and simply exhausted. Laura called me again at 4:22 PM. Dr. V had come in for awhile and Mom perked up while talking to her. Dr. V talked about inserting the catheter tomorrow morning and starting hemodialysis. She’ll talk to the surgeon about removing the PD catheter and inserting the fistula for hemodialysis, but some vein mapping has to be done first. She talked to Mom about doing hemodialysis after she gets out of the hospital. It will be three times per week, 3.5 hours each time.

As of 6:00 PM they had completed the hospital admissions process, so now they are waiting on a room to become available. On my way to the hospital a little after 7:00 PM I found out she was told she might not get a room at all tonight. Wouldn't that be wonderful?

I arrived around 7:30 PM and found Mom and Laura in the ER. A nurse came in and tried to draw blood from her right arm, but gave up on getting enough. Before that nurse left, another nurse and a woman wearing a red shirt came in. Laura told me the woman in the red shirt was the leader of the red team. She came in talking on a cell phone, with quite a sense of urgency about her. She started relaying Mom’s vital signs to whoever she was talking to, and she said “family at bed side” at one point. I didn’t know what was going on, but it was as if Mom suddenly became a critical patient and got a lot of attention. The red shirt lady was talking to the people in the lab and told them they would have to go up to Mom’s room to draw blood, because she needs this room. I also heard her say something about an ambulance waiting.

They got Mom prepped to roll very quickly and moved her upstairs to her room. She got settled in just before 8:00 PM. Laura left soon after that and I stayed there until 9:40 PM. She was watching TV when I left, but she was definitely tired.

As you can imagine, it was a huge relief to finally have Mom in a hospital to get hemodialysis started. Hopefully the next blog post can cover more than one calendar day, because at this rate we'll never catch up to real time.

Thursday, January 3, 2013

October 8, 2012 - Strike three?

The last update covered the first week of October 2012, when Mom resumed her training for peritoneal dialysis (PD) but it wasn't going well. We pick up our story on Monday morning, October 8. Amy is taking Mom to her 10:00 AM appointment at the PD clinic, where they will either try another PD exchange or run some tests that her nephrologist recommended last week. I knew fairly early in the day that this was going one of those days, so I took notes throughout the day with the help of the timestamps of all the phone calls and text messages on my cell phone. Below is a detailed account of my day, which included 15 text messages plus almost three hours worth of phone calls. And right in the middle of all that, a new twist came up. There is a summary at the end if you want to skip all the drama.

Monday, October 8, 2012

10:14 AM: Received a text message from Amy. "Mom looks awful today and could barely hold a conversation with me in the car. It's kind of scaring me." [I found this to be odd because Mom seemed ok when I was at her house on Saturday and took her to run a few errands.]

10:19 AM: Sent a text message to Amy. “I hope they see that at the clinic and take note. I think I’ll see if she can call me from there.”  [We've been wondering if the people at the clinic have really been paying attention to Mom's condition when she's been there this past month for PD training or to have blood drawn. Her tolerance for pain is pretty high, so they may not have been getting an accurate reading of how she was really feeling.]

10:26 AM: Short phone call from Amy. (1 minute) She was telling me that Mom was having some pain during the first exchange, and then the nurse came back in the room.

10:31 AM: Phone call from Amy. The nurse said Dr. S’s notes from last week say Mom’s not a good candidate for PD. (4 minutes)

10:37 AM: Phone call from Amy. The nurse can have Dr. S talk to someone. They gave me his cell phone number. (1 minute) [I had never met Dr. S, so I wanted to get his opinion about whether Mom's current condition was due to the anemia, the need for dialysis, or a combination of the two. I also wanted to know what the process would be once we decided to give up on peritoneal dialysis. If she's in dire need of dialysis right now I didn't want another long delay before she could get hemodialysis.]

10:38 AM: I called Dr. S and first asked him whether her current symptoms were due to anemia or the need for dialysis. He thought it was primarily the anemia. He explained the peritoneal equilibrium test and said they’d like to run it today, then they can rush the results and make a decision Wednesday morning. I asked him about the process if PD is ruled out. He said it would be hospital admission, inserting a temporary catheter in neck, then starting dialysis in the hospital. (10 minutes)

10:49 AM: Call to Amy to relay what Dr. S told me. (10 minutes)

11:00 AM: Sent a text to Laura, since she was planning on picking up Mom today. “Mom won’t be done until 3:30 today.

11:01 AM: Phone call from Laura. (14 minutes)

11:21 AM: Phone call from Amy. She said they couldn’t complete the test because Mom was in so much pain. They told her to take a laxative and try again tomorrow. (2 minutes) [I guess most issues with peritoneal dialysis are caused by constipation, since "take a laxative" seems to be the first thing they try.]

11:23 AM: Left Laura a voicemail.

11:24 AM: Phone call from Laura. (8 minutes)

11:34 AM: Text to Amy. “Laura is planning on being at Mom’s house around 12:30 to clean up some glass. Did Mom tell you about the glass lid she broke?” [Laura had just told me about Mom accidentally breaking a glass lid over the weekend, and Laura knew Mom didn't feel like getting down on the floor and doing a thorough job of cleaning up all the glass.]

11:36 AM: I received a phone call about a potential kidney donor. We'll refer to this potential donor as TUD ("The Unknown Donor") for now. (3 minutes)

11:37 AM: Text message from TUD, requesting some info.

11:39 AM: Text message back to TUD.

12:44 PM: Text to Amy: “How does Mom feel about going to the hospital today or tomorrow?

12:46 PM: Phone call from Mom. She was home at this point. She sounded ok, but still tired. She said she wanted to get some food, get warm, and go to bed. (16 minutes) [I mainly wanted to get her side of what happened today and see what her thoughts were at the moment about continuing with peritoneal dialysis. I didn't push too hard to get her to decide to go with hemodialysis at that point since I knew she was really tired and just needed to rest.]

1:50 PM: Text from Amy: “Call me when u can.

1:51 PM: Phone call to Amy. Discussed futility of continuing with PD training. (17 minutes) [At this point Laura, Amy and I are in complete agreement that it's time to throw in the towel on the PD training. Now it's a matter of convincing Mom, and we're each working on that in our own way.]

2:34 PM: Phone calls from Laura. (9 minutes) She's been talking to Mom about moving forward with hemodialysis.

3:01 PM: Text to Amy: “Laura made some progress with Mom, but she’d rather not go to the hospital constipated.” [As if she wasn't miserable enough . . .]

3:01 PM: Phone call to TUD about kidney donation. TUD does not want Mom to know about this until it gets further along. (18 minutes)

3:12 - 3:30 PM: Four text messages back and forth with Amy discussing how to potentially get Mom to the hospital tomorrow.

3:42 PM: Text from TUD.

3:47 PM: Text to TUD.

4:09 PM: Phone call from Laura. She had been at Mom’s house. (16 minutes)

7:52 PM: Phone call from Laura. (18 minutes)


In summary, today was a total failure in terms of progress with peritoneal dialysis. I know it was painful for Amy to be there and witness what was going on, but thank goodness she was. Mom's condition is declining and we don't know what damage is being done while we wait for some type of dialysis to begin. She wants to sleep on it and make a decision tomorrow morning since she's gone this far trying PD. And today a potential kidney donor stepped forward, but I can't tell Mom yet so that information has to be held very tightly.

Stay tuned to find out what happens tomorrow . . .

Tuesday, January 1, 2013

Happy New Year!

Welcome to 2013 everyone. Since the next post will be a long one, I've decided to dedicate this post to a little blog news and the monthly blog stats.

Resources Page

The Resources Page now has a list of all 24 articles I have in electronic form, plus a few others. (Next I'll start listing the ones I do not have in electronic form.) I have uploaded all the articles I have to two different locations in "the cloud," so you can read or download all of these articles from one place instead of following links all over the internet. Here are the links to the two file locations, which should be identical:

The resources page has the file naming convention, and I give my opinion on which articles to start with if you're only curious about treatment options and prognosis.

Monthly Blog Status Update

Months behind as of December 2012: 12
Months behind as of January 2013: 3

Total posts: 45 (12 in December)

Email subscribers: 3 (Don't worry. I can't see the email addresses used to subscribe, only the count.)

Number of countries that have viewed the blog: 19

New countries that viewed the blog in December: