Tuesday, July 30, 2013

Layers of Infiltration

In the previous post, Mom spent two weeks tethered to a portable wound vacuum (affectionately known as Kirby) to help a surgical incision on her abdomen heal. This post will cover the following week, the week of July 22. So what is on Mom's schedule for this week? Other than dialysis on Monday, Wednesday and Friday, she has Ed's funeral on Tuesday near Austin, an appointment with Dr. K on Wednesday to examine the surgery site, and Mohs surgery for a spot of skin cancer on her right leg on Thursday. It's a busy week, so let's hope everything goes well.

Nope, that didn't happen. The week began poorly at dialysis on Monday, July 22. They infiltrated Mom's fistula today, which is when a needle enters a blood vessel like it is supposed to, but it continues in too far and creates another hole. When a fistula is infiltrated it causes blood to flow out of the blood vessel and pool under the skin. Mom ended up having just a little more than one hour of dialysis that day (instead of her usual four hours), and they told her she still needs to come in for three more sessions. She can't come in Tuesday because she will be out of town for the funeral, and she can't come in Thursday because she has Mohs surgery scheduled, so that leaves Saturday.

As if that wasn't bad enough, she also weighed more after this short dialysis session than she did before, which means they did not remove any fluid and she probably ended up retaining some of the saline solution they periodically flush the lines with. They told her not to drink anything and to keep her ankles propped up to reduce swelling due to edema. They also told her to get to an emergency room as soon as possible if she experienced any shortness of breath. She also needs to keep ice on her arm to reduce the swelling and bruising. This is just what she needs to deal with the day before going to Ed's funeral.

On Tuesday ten of us, including Mom, went to Ed's funeral in Taylor, TX. It was a long day and we were gone from Mom's house a little more than 12 hours. Mom did fine, although she was standing up on her feet more than usual. When we arrived back to her house she realized her ankles were very swollen, so she took it easy and kept her feet propped up as much as possible the rest of the night. She obviously still had a fluid issue due to the shortened dialysis session on Monday, but after dialysis the next day she said she could see her ankles again, so the fluid appeared to be under control.

On Wednesday after dialysis she saw Dr. K for a follow-up visit on her surgery site. There is still a small amount of discharge, but he said it is coming along fine. Regarding the small knot Mom noticed below the wound, he said that was normal and should go away over time.

On Thursday, July 25, she had Mohs surgery for the skin cancer on her right leg. The surgeon only had to remove one more layer since most of it had already been removed when the biopsy was done in June, so that is some good news that the skin cancer had not gone very deep at all.

Friday, July 26 was a good news/bad news day at dialysis. The good news is that Dr. N said she did not need another dialysis session on Saturday, since things seemed to be under control. He also lowered her dry weight again. The bad news is that Mom's blood clotting issue seems to be getting worse, meaning the machine gets clogged up. She is now taking aspirin and fish oil to address that situation. Hopefully that gets under control so we don't have to seriously consider putting her on Coumadin.

That gets us caught up with the Mom updates for now, so maybe I can get back to some article reviews while things are relatively calm.

Thursday, July 25, 2013

Living with Kirby

In the previous post, Mom had been discharged from the hospital on Friday, July 5, after yet another abdominal surgery to take care of an infection at the site where her peritoneal dialysis catheter had been inserted (June of 2012) and later removed (October of 2012). The surgeon left the wound open this time so a wound vacuum could be used to promote healing and prevent infection. Now Mom is facing two to three weeks of being attached to a small pump by a length of plastic tubing coming out of the dressing over this surgery site, which is to the right of and slightly higher than her belly button. The pump is in a black nylon carrying case with a matching black shoulder strap, so at least she can be somewhat stylish while she's out and about.

When I was at her house the day after she got out of the hospital, I referred to the pump as "Kirby" at one point (hence the title of this post). I was thinking about Kirby vacuums when I said that (we had one the whole time I was growing up), but the Nintendo video game character named Kirby is also a decent match, since that Kirby is small and cute just like the pump, and one of the main things he does is inhale objects (although a bit more dramatically than the pump does.) So use whichever reference works best for you.

The first visit from the home health care nurse to change Mom's dressing and check on Kirby was the morning of Monday, July 8. It did hurt some when the old dressing was being removed, but I suppose that is to be expected since there must be some sort of adhesive used in order to create a good seal between the outermost dressing and Mom's skin. And I suppose removing gauze that has been in an open wound for two or three days might not feel good either. But at least Mom did get some good news from the nurse. On the mornings the nurse comes to change the dressing, Mom can disconnect the hose from the pump and take a shower before the nurse arrives. (Mom was not too excited about possibly going at least two weeks without a real shower.)

Mom's nephrologist, Dr. N, happened to come to the dialysis clinic on Monday while she was there. He said it looks like she has lost some weight and he ended up lowering her dry weight. (A dialysis patient's "dry weight," which I initially talked about in the February 9, 2013 post, is what the patient would weigh without any excess fluid.) I do not know how long it has been since her dry weight was adjusted, but given how much weight she has lost this past year it probably should have been adjusted down several times. I know Mom has had to specifically request they remove more fluid than they were planning to remove on more than one occasion, especially while traveling. Dr. N thinks what happened on the cruise ship was they did not remove enough fluid during the first two dialysis sessions because they were basing it off of the paperwork Fresenius sent to Dialysis at Sea, which is sent quite a bit in advance of the cruise. So this is another reason why a dialysis patient really needs to be aware of everything that happens during dialysis and be their own advocate.

As I mentioned before, Kirby can be unplugged and operate on battery power for up to four hours, so it is not a problem for Mom to take short trips in the car to run errands. But longer car trips would be less practical, because she would either have to charge Kirby soon after reaching her destination, or stop along the way to plug in Kirby for awhile to recharge the battery. I knew Mom was going to a memorial service on Thursday for her aunt who recently passed away at the age of 97, and that was about a 90 minute drive from her house. Fortunately I remembered I have a device for the car that converts the power from the car's 12 VDC power socket to 120 VAC. It happens to be shaped like a coffee mug so it fits in a cup holder. I put my electrical engineering degree to good use and confirmed that Kirby's power supply does not require more power than the power converter is designed to supply. Then on Tuesday she made a test run with Kirby plugged into it when Cathy took her to the airport to pick up her cousin who was also coming in for the Thursday memorial service. Everything worked just fine, so on Thursday she used it on the way to and from the memorial service, which meant she did not have to keep looking for a place to plug Kirby in while they were down there.

On Friday, July 12, the home health care nurse said Mom's wound is healing very well, so she may be done with Kirby sooner than was initially estimated. Some more good news came on Friday regarding dialysis. They have started trying to do the buttonhole technique for the access on Mom's arm. (See the February 9. 2013 post for a description of the buttonhole technique.) Hopefully that will go well and significantly reduce the pain when the needles are inserted.

During the next week, Kirby and the power converter were put to use on short notice, and this trip probably would not have happened without the power converter for the car. Mom's travel buddy Ed (most prominently mentioned in the post describing their 2011 trip to Russia) was in a hospital in Houston and not doing well at all, and due to Mom's surgeries and travels she had not been able to see him since before his surgery in June. At the urging of my sister Laura we moved or cancelled a few appointments and created a window of opportunity such that my wife Cathy could drive Mom to Houston after dialysis on Wednesday, July 17, then drive back on Thursday. That's about a four hour drive to Houston, but she was able to keep Kirby plugged in during the entire drive there and back. Unfortunately Ed did pass away late Thursday afternoon while Cathy and Mom were driving back home. He lived life to the fullest up until the very end, choosing to continue traveling extensively despite the wishes of his doctors and his family. He will be missed.

But before Mom and Cathy went to Houston on Wednesday, the home health care nurse came by to change Mom's dressing. Mom told her she had been feeling a small knot below the wound, beneath the skin, and it was a little tender. The nurse checked it and said it does feel like there is something there that may need to be drained. Mom was understandably upset over that bit of news. Laura called Dr. K's office to get an appointment, but the earliest they could see her was the following Wednesday, one week away. So Mom will keep a close eye on it, and if it starts oozing or if she starts running a fever, we won't wait to take her to an emergency room.

I went over to Mom's house Friday morning to take a look at her malfunctioning garage door opener, and when she greeted me at the door I did not see Kirby. I asked her where Kirby was and she said when the nurse changed the dressing this morning she said Mom did not need the wound vacuum any more, and she just put a normal dressing back on. So that was a nice birthday present for Mom, especially considering she was in the hospital with a kidney infection on her birthday in 2011, then last year she was diagnosed with bronchitis on her birthday.

So Kirby the wound vacuum was a part of Mom's life for two weeks, from July 5 to July 19. Let's hope this time the wound continues to heal without getting infected.

Saturday, July 20, 2013

Post-cruise ooze

Our last post ended with my sister Laura picking Mom up from dialysis around noon on Wednesday, July 3. Before Mom came out, the nurse Laura had spoken to that morning told Laura that Mom was running a slight fever (around 100) and the surgery site was oozing a little and definitely infected. She said they would definitely not do the heart catheterization procedure scheduled for Friday if she was running a fever due to infection. She told Laura that if Mom could not see a doctor about it today she needed to go to an emergency room to have it looked at and probably have the abscess drained.

Laura took Mom home and they called Dr. K's office from there. They had to leave a message with his answering service since his office was closed until 2:00 PM. Things got more interesting around 1:30 PM when Mom's temperature went up to 103. Laura called Dr. K's office shortly after 2:00 PM and told them Mom's temperature was now 103 and she was taking her to an emergency room, and asked them which hospital they wanted Mom to go to. The nurse put Laura on hold while she talked to Dr. K, and then told Laura to bring her to Dr. K's office.

Due to Laura's schedule my sister Amy had to take Mom to Dr. K's office. She called me from there around 3:30 PM and said Mom was going to be admitted to Baylor Heart Hospital, which is just two buildings over from Dr. K's office. Dr. K spoke to me briefly and said he would try to get her scheduled for surgery today, and he would probably leave the wound open so she would be in the hospital for one or two days. So Friday's heart catheterization will need to be cancelled.

They got Mom prepped for surgery and took her to the operating room around 5:00 PM. Amy called me at 5:35 PM and said the surgery was done, with no complications. They did leave the wound open for healing purposes. Dr. K said he was going to arrange a home health care nurse to come to Mom's house to change the dressing and do some sort of vacuum procedure on the wound. We had no idea what that was all about, but we learned later.

I got to the hospital around 6:30 PM, and Mom was still groggy and coming in and out of consciousness. She gradually woke up and was able to eat some dinner before 8:00 PM, and I stayed until about 10 PM.

Mom got to spend the Fourth of July in the hospital, and I know she got at least nine visitors that day (not counting doctors and nurses). Laura was there in the morning when the dressing on Mom's wound was changed. Mom and Laura were surprised at how large the hole was once the nurse pulled out all the gauze. It's about 1.5 inches deep (3.8 cm) and at least 1 inch by 2 inches across (2.5 cm x 5 cm). No, I don't have a picture of it.

Some good news is that Mom's hemoglobin is still over 11 even though she did not get any Epogen while she was on the cruise. So maybe her anemia is getting under control now and she will start feeling better once this infection is behind us.

Mom had dialysis in the hospital the morning of Friday, July 5, and after that they installed the vacuum thing. I still did not know what the vacuum was all about, so I hit the internets and discovered it is called negative pressure wound therapy. The wound is stuffed with a sponge-like material, then a clear dressing is applied to create a seal around the wound. A tube is attached to the seal and to a vacuum pump that the patient has to keep on their person or nearby at all times. This constant vacuum on the wound promotes healing and reduces infection by increasing the blood flow to the wound and drawing out any discharge. The pump needs to be plugged into a power source as much as possible to keep the battery charged, but it can be unplugged for up to four hours at a time. Speaking of discharge, Mom was discharged from the hospital late Friday afternoon.

I went over to Mom's house on Saturday to see how she was doing and to check out the pump. She has about ten feet (3 meters) of clear plastic tubing, with one end attached to her abdomen and the other end attached to the pump. Her wound is packed with gauze, with a black sponge-like material on top covering that. The sponge is maybe 3 inches (7.6 cm) in diameter, so the wound is smaller than that. On top of the black sponge is what looks like a clear rubber suction cup. The plastic tubing is attached to this suction cup, at a right angle so the tubing is against her body instead of protruding straight out. Covering the suction cup and black sponge is what looks like shipping tape, but it is actually the dressing used to create a seal so the vacuum will work. The area with this clear dressing is at least 6 inches x 6 inches (15 cm x 15 cm). Yes, I took a picture. No, I'm not going to put it on the blog, even though it isn't very gross.

The pump itself is about the size of a small purse or a large pocketbook, and weighs 2.4 lbs. (1.1 kg). It has a carrying case with a shoulder strap so the patient can carry it around. The manufacturer is KCI, and it is the KCI ActiV.A.C. Here is the link to the product web page if you would like to learn more about it, including specifications and the full manual: http://www.kci1.com/KCI1/activactherapyunit. It makes a quiet "clunk" sound every few seconds, and occasionally it makes a gurgling sound, which I suppose is when some fluid reaches the pump. I only saw a few spots of fluid in the tubing, so it's not like the wound is constantly oozing at this point.

As if Mom was not tied down enough with dialysis three times each week, now she has to carry this pump everywhere with her and keep it plugged in most of the time. Just going from room to room inside her house becomes a bit of a hassle now. What makes it even better is that she cannot take a shower with this wound dressing. They told her she would probably need the pump two to three weeks. Wonderful.

With this wound vac the hospital arranged for a home health care nurse to come to Mom's house three times each week to change the dressing and make sure everything is still working properly. That will happen on Monday, Wednesday and Friday mornings, before Mom goes to dialysis. The next blog post will start with this first visit from the home health care nurse.

Monday, July 15, 2013

Post-cruise news

The last blog post ended with our return home from the cruise on Sunday, June 30. After the emergency on the ship Friday night, when Mom was essentially drowning due to the fluid buildup in her lungs, she was watching her fluid intake and diet very carefully in order to avoid another episode like that. We were hoping the dialysis sessions on the ship Friday night and Saturday morning took care of the fluid issue, such that her local nephrologist could now evaluate things and get her back to normal. Ha! 

My sister Laura spent the night at Mom's house Sunday night and she called me early on Monday morning, July 1. She said Mom was starting to have symptoms similar to what she experienced on the cruise ship Friday night, but they were not yet as bad. Mom was calling the dialysis clinic to see if they could take her immediately instead of waiting for her 11:00 AM appointment, since she knew she needed fluid pulled off. Fortunately they could get her in, so Laura took her to dialysis. Mom also took all of her dialysis flow sheets from the cruise and her records from the emergency room visit for the people at the dialysis clinic to review. The nephrologist, Dr. N, is typically at the clinic on Tuesday to see the Tuesday-Thursday-Saturday dialysis patients, and on Friday to see the Monday-Wednesday-Friday dialysis patients. So he was not there today, but I suggested that Mom have them make copies of everything so he could review it on Tuesday when he came in. I called his office and asked if they would have him call me about what happened on the cruise. I did not get a return call back, but I found out later that he talked to someone at the dialysis clinic about Mom's situation, so at least he is now aware of what happened.

Now that the dialysis is under control Monday morning, Mom needs to contact three different doctors about three different things. Using the phone during dialysis is one of Mom's favorite things to do, for two reasons. First, she cannot move her right arm much at all because that is where the needles are inserted into her fistula. And second, the nurses and techs in the clinic don't exactly speak as if they are in a library, so there is often a lot of loud talking or yelling across the room in the background. But in spite of all that, Mom was able to take care of the following items:

  • She contacted the office of her cardiologist and set up an appointment for tomorrow afternoon (Tuesday). You may recall the nephrologist on the cruise ship recommended she see a cardiologist due to how much distress she was in Friday night.
  • She contacted the office of her dermatologist regarding the biopsy results from the spot on her leg. As expected it is indeed a slowly progressing form of skin cancer, so she has scheduled Mohs surgery to take care of that on July 25.
  • The site of the June 11 surgery on her abdomen is getting red and appears to be infected. Mom called the office of the surgeon, Dr. K, and he called in a prescription for an antibiotic.

Laura took Mom home after dialysis, and then went to pick up the new prescription for the antibiotic (Bactrim). The pharmacist told her to be sure Mom drinks plenty of water when taking these pills, which is just what a person who is limiting her fluid intake wants to hear. Laura talked to the pharmacist and they decided that one full glass of water with each pill would be ok. (Since then I looked up Bactrim on the internet and the first two sites I looked at said to drink plenty of fluids to reduce the risk of kidney stones. Since Mom is still producing urine, I suppose kidney stones are still possible. Wouldn't that be wonderful?)

I took Mom to her cardiologist appointment the afternoon of Tuesday, July 2. If you have a really good memory, you may remember Mom last saw her cardiologist (Dr. R) when she had a chemical stress test in November of 2012. Everything was fine then, so I was not too concerned about this visit. We told him the story about what happened on the ship and what happened Monday morning. We gave him a copy of the EKG readings that were taken on the ship, and he said they looked fine. He examined her heart and lungs with a stethoscope and did not indicate that he found anything unusual there. He said the only way to really know for sure was to take a look at the heart, and he recommended she have a heart catheterization which is where a catheter is inserted up through the femoral artery and a dye is injected so the arteries can be clearly seen on an x-ray. We scheduled that procedure for Friday, July 5. If all goes well and they do not have to put in a stent, she will go home that same day. If they do have to put in a stent, she will stay overnight.

Mom also told Dr. R about the spot on her abdomen that she thought might be infected. She said it was now warm to the touch. He examined it and said it looks like there may be an abscess there that needs to be drained. He said the antibiotics she is taking would take care of the symptoms, but as soon as the antibiotics are discontinued the symptoms will come right back. That was not what we wanted to hear, so now Mom gets to call Dr. K's office tomorrow.

Given how Mom felt when she woke up on Monday before dialysis, we were all wondering how she was going to feel when she woke up Wednesday morning. My sister Laura called me in the morning and said Mom woke up feeling fine, she slept great, and she had no chest pain or shortness of breath. The dialysis clinic called and asked if she could come in early, so Laura took her to dialysis and they got started around 8:00 AM. So things were looking good Wednesday morning, but then Laura went to pick up Mom from dialysis around noon. Before Mom came out, the nurse Laura had spoken to that morning when she dropped Mom off came out and told Laura . . . I should probably end this blog post here. It seems long enough.

[2013 August 1: Minor edit. Inserted "care" in second bullet item.]

Wednesday, July 10, 2013

Cruising along on dialysis

In the last update on Mom she was recovering nicely from her June 11 surgery to take care of an infection in her abdominal wall muscle and remove a piece of surgical felt that had been purposely left in place when her peritoneal dialysis catheter was removed in October of 2012. Thank goodness that infection and surgery occurred when it did, because that gave Mom some time to heal before going on a 7-night Caribbean cruise.

"A week-long cruise? But isn't she on dialysis?" Yes, normally a cruise longer than a couple of days would be out of the question for a dialysis patient unless they arranged to have dialysis at ports where the ship docks. But fortunately last year we found out about a company called Dialysis at Sea (http://dialysisatsea.com/) which selects specific cruises and staffs them with medical personnel and dialysis equipment, allowing patients on hemodialysis to book a room through them so they can go on a cruise. Earlier this year we booked a cruise on Royal Caribbean to the eastern Caribbean, and we sailed out of Ft. Lauderdale, FL on Sunday, June 23.

Mom had previously requested wheelchair assistance to board the ship, since it is a pretty steep walk up the gangway to board. Once we were on board she was able to walk around, although she did get out of breath fairly easily, especially when walking long distances. Since this ship (Allure of the Seas) is the largest passenger cruise ship in the world (until next year), there were plenty of opportunities to walk long distances.

We met the Dialysis at Sea staff at a mandatory meeting the evening we set sail. The staff consisted of a nephrologist (Dr. R), a nurse, and a tech. They went over some logistics on where to go for dialysis (ship's medical facility on deck 2), made sure everyone knew when they were supposed to go, and answered any questions. There were only a total of six dialysis patients on this cruise, which is fewer than they usually have. Mom's dialysis was scheduled for 6:00 AM on Tuesday, Thursday, and Saturday.

At the beginning of the trip Mom said she was getting winded with minimal exertion, such as walking long distances. She did a lot of walking on the ship Sunday and Monday, and at first she was feeling ok and thought the walking was doing her some good because her blood pressure was staying down. But at some point she noticed her legs were very weak, so maybe she walked a little too much. We did get a wheelchair in her room on Monday, and I started pushing her around in it when she had long distances to go on the ship. She was able to walk off the ship and take tours when we docked on Monday (Bahamas) and Wednesday (St. Thomas). There were no issues with dialysis on Tuesday or Thursday, but she did ask them to remove more fluid on Thursday because she was noticing some swelling in her ankles (edema), which has never been an issue with her before. And then Friday happened.

June 28, 2013: Mom and Cathy both had appointments in the salon/spa Friday at 6:30 PM. After taking Mom there in her wheelchair I went back to our room and waited until they were done so we could go eat dinner. Cathy called me around 7:45 PM and asked if I could come get Mom and take her to the medical facility because she was having trouble catching her breath. The salon was on the same level as our room, but at the opposite end of the ship. I arrived and found Mom sitting in one of the chairs where she had been getting a shampoo. Her hair was wet and she was leaning forward because it hurt her chest to lean back. We got her in the wheelchair and headed for the medical facility, which was on deck 2 at the opposite end of the ship from the salon. One of the salon workers went with us so we could take the elevator down to deck 2 and then use the employee corridor to reach the medical facility. I told Mom that was our behind the scenes tour of the ship, which normally costs $150 per person.

The employee corridor is wide enough for a car to easily drive through, and it even has a yellow striped line down the middle like a two-lane highway. (I knew from a TV show Cathy and I had watched several months ago that it's referred to as I-95.) As we were going down the corridor Mom got progressively worse, saying her chest was hurting more and more. I wanted to go faster with the wheelchair but since the floor did have an occasional bump or rough spot, I was afraid I might dump her out, especially since she was leaning forward due to the pain. It seemed to take forever to get to the medical facility, but we finally arrived. It was after hours, so the salon worker used the phone there to contact the nurse on duty. There was a sink in the waiting area for hand washing, and I wheeled Mom over there when she said she felt like she was going to throw up. She spit up a little bit and put some wet paper towels on her face and forehead for a little relief. I knew the situation was getting serious at this point.

We went inside and got her on a bed in the examination room. The nurse hooked her up to an oxygen sensor and a blood pressure cuff, put an oxygen mask on her, and then drew some blood. Mom was in a lot of pain at this point, rating her chest pain as 10 on a scale of 1 to 10. She was almost constantly moving trying to get comfortable. I was feeling helpless and more than a little concerned at this point, since I thought she might be having a heart attack. I knew the best thing for me to do was answer questions and stay out of the way.

The nurse gave me some paperwork to fill out, and when I got to the part about medications and allergies I asked the nurse if I could go to my room to get a copy of Mom's medication and allergies list. So I did that, and the doctor arrived shortly after I got back. I believe one of the first things he did after assessing the situation was to give her a nitroglycerin tablet. He asked me lots of questions about her medical history (when did the chest pain start, how long on dialysis, is she diabetic, what caused the kidney failure, is she producing urine, etc.). They put a CPAP mask on her to assist with her breathing, and slowly her blood pressure and pulse rate came down. It seemed like the doctor asked her about every 60 seconds if the pain was going down.

At some point they started trying to contact the Dialysis at Sea staff, and the nurse was the first to arrive. The ship’s doctor realized that the problem was fluid on the lungs, and just giving her Lasix (furosemide) would not be the appropriate treatment since it would take too long. So it was looking like they would get her started on dialysis as soon as possible. The nurse spoke to the dialysis tech on the phone, and she found the nephrologist, Dr. R. He arrived around 8:45 PM, checked Mom out, and consulted with the ship’s doctor. Dr. R suggested to me that Mom see her cardiologist when she gets home because she should not have been in that much distress given the amount of fluid on her lungs. The dialysis nurse and the tech were getting the dialysis machines set up while all this was going on.

They did a chest x-ray to make sure there was not something more serious to cause the fluid on the lungs, and then they got Mom started on dialysis around 9:30 PM. The doctor said they would probably do two hours, depending on how things went. Cathy and I went to the 24-our cafe to get some food, and I went back to the medical facility around 11 PM. The nurse said they pulled three liters of fluid off of her. They had to stop the dialysis after about 90 minutes instead of two hours because Mom’s legs started cramping very badly. They said they wanted to delay Mom’s Saturday dialysis session from 6:00 AM to 9:30 AM, to give her body a little time to stabilize.

I wheeled Mom back to her room and she immediately crawled in the bed, put on her CPAP machine, and went to sleep.

I took Mom to dialysis Saturday morning and came back a couple of hours later to see how it was going. The nurse said there was still some fluid in her lungs, but nowhere near as much as last night. I asked if there were any concerns about Mom flying back home tomorrow, and she recommended that Mom wear her compression stockings during the flight to help with the edema in her legs. I also asked her how often they have emergency dialysis sessions like this on the Dialysis at Sea cruises. She said she has been doing these types of cruises for 20-something years (I think she said 27), and this was the third time for her. So once again, Mom is very special.

That afternoon I took Mom back to the salon so they could finish her shampoo and hair styling. The salon workers were very happy to see her looking much better than when she left there Friday night. The cruise ship docked in Ft. Lauderdale Sunday morning, and we flew home Sunday afternoon without any issues. (Even though Mom had wheelchair assistance at the airport, she still got very tired just walking from the front of the plane back to her seat in row 26.) Hopefully this fluid episode is behind us and things will get more stable after Mom starts dialysis at home again. Stay tuned . . .

Friday, July 5, 2013

All In The Family

As I mentioned in the previous post, we now know which of Mom's parents she inherited fibrinogen amyloidosis from. We recently learned that a cousin on her father's side was diagnosed with fibrinogen amyloidosis. Here is the preliminary information I have. This cousin, who I will refer to as RM for now, is 60 years old, has been on dialysis for about two years, and was diagnosed with fibrinogen amyloidosis when a kidney biopsy was analyzed at the Mayo Clinic. Here is how we go through the family tree to get from Mom to RM:

My mother's father, Marshall, died at the age of 76 due to complications after a spinal cord injury. He had diabetes, but no significant kidney issues as far as we know. Marshall's father died at the age of 89 and his mother died at the age of 95, with neither of them having any known kidney issues. We do not know which of them had the mutation.

Marshall's brother Charlie died at the age of 60, again with no known kidney issues. Charlie had three children, one of whom is his son RM who has been diagnosed with fibrinogen amyloidosis. Charlie also had two daughters, and they have both tested negative for the mutation. RM has two children who have not been tested.

So in this kindred we see at least two individuals (Marshall and one of his parents) who had the mutation but lived into their 70's or later without developing any symptoms associated with fibrinogen amyloidosis. That is one of the main reasons why genetic testing is so important. Lack of symptoms in an individual does nothing to indicate whether or not that individual has the mutation. The symptoms can and often do skip one or more generations, but the mutation cannot.

Marshall and Charlie came from a family of 11 children, 9 of whom lived to adulthood, with 8 of them having children. Two sisters are still living today at the ages of 94 and 79. Those 8 siblings had 20 children among themselves. Based on the information I have on that side of the family, which I know does not include all of the descendants, there are now 7 people living who have a 50% chance of having inherited the mutation and have not been tested, 17 with a 25% chance, and at least 22 with a 12.5% or lower chance.

So it looks like I have a new mission now, to let Mom's cousins on that side of the family know that an ancestor of theirs definitely had the mutation. With that information, and the fact that the symptoms can skip generations, hopefully some more family members will get tested.

Another important piece of information from RM's diagnosis is the age of onset. Mom started dialysis at the age of 71, whereas RM started dialysis around the age of 58. As we will see in some future articles, the age of onset does have a fairly wide range with fibrinogen amyloidosis. So just because Mom's symptoms stared appearing in her mid 60's does not mean I can sit back and rest easy until I reach that age.

Next up: Dialysis on a cruise ship? Yep.

=====Monthly Blog Status Update=====

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Here is my favorite spam comment from June:

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