My Dad's Story

[Debra]

Hello all. I'm not a new member, but haven't contributed until today! My Dad's story is a long one - and he's been a trooper through it all! In June 07 he had a reoccurance of polyarteritis nardosa (vasculitis). His rheumatologist decided to treat the vasculitis with Cytoxan, which had a better chance of putting it into remission. Unfortunately, Dad was the one in a million for whom the Cytoxan destroyed his bone marrow. Of course, we did not know this was even possible! I took Dad to the local hospital on August 4, 2007 because he was feeling extremely weak. After a CBC, things got a bit crazy! A hem/oc was called in, and dad was given blood transfusions. A BMB was advised, at which point we were informed it looked like he had aplastic anemia - though the local hospital didn't treat it. We transferred Dad to Zale Lipshy, part of the UT Southwestern medical center. Unfortunately, Dad also had acquired a fungal infection in his blood. Dad spent 5 months in the hospital - battling infections, pneumonia, CDIFF, AFIB, fluid overload that damaged his heart and caused him to end up in the ICU on a ventilator - there's more, but I think you have the picture!! We've also had a terrible time with insurance - Medicare has stated that Neupogen and Procrit "are not indicated" for aplastic anemia!
ATG (horse) was administered in Sept. 07. He came home on Jan. 16, 2008 - totally dependent on Mom and myself. We had to move him using a "hoyer" lift, as his muscles were so deconditioned he was unable to even sit up! We purchased a handicapp accessible van to transport him to and from the doctor and the hospital. He has been transfusion dependent - platelets weekly and blood every 14 days. He had the rabbit ATG in May 08, and so far we have not had any results, his transfusions have remained the same.
After many months of physical and occupational therapy, and "Deb therapy" Dad is finally able to take several steps with the use of a walker!!! He continues to gain in strength and endurance, though his doctors did not believe he would ever walk again due to the high doses of medications he received. They believed his muscles had been permanently damaged. We believed in Dad, and never gave up.

We know what caused Dad's aplastic anemia, however, no one seems to know what his chances are for recovery. I would be thankful if his transfusions were further apart - as his quality of life would be so much better. We spend so much time in doctors offices and the hospital! Dad is having side effects from the Exjade - he is nauseous and has diahrrea. The neupogen injections also make him feel like he has the flu.

I have so many questions, I don't know where to begin. It is very helpful to read through this website, you have gotten me through some very long and sleepless nights. Sorry this is sooooo long!

My name is Debra - not Dera - but I don't know how to change it!!! Thanks for letting me participate. Keep the good information coming.....

Debra - daughter of Paul, diagnosed with SAA in Aug. 07

[ccartbmw]

Hi Debra,
My name is Carol I got diagnosed with AA in January of 2005.I had ATG in March of 2005,after being hospialized for 5 months. My AA went into remission until March 2007. My doctor from UCLA put my on 250 MG of cyclosporin with no results, I had another ATG on April 1 st of this year. My experience the second time was totally different than the first. I had the treatment done with a pic line. As of last week my levels had gone up to 11.1 hb. and my platlets had reached 121 thousand. Tell your Dad to keep the faith and try to stay positive. It will come up, they say it can take 6 months to a year to see any results. I also have still been on the cyclosporine 250 mg. This is the medication to go along with the ATG.
Thanks for listening
Hope your Dad gets better.

[Neil Cuadra]

Debra,

We fixed your user name for you. It's now Debra instead of Dera. If only we could help your dad as easily.

[Marlene]

Hi Debra,

John would get nauseated from Exjade also. How high is your Dad's FE level? You may want to either take a break from it for three weeks and start it up at a lower dose to see if that helps. Or, if he still has a central line in or a port, switch to desferral to see if he tolerates that better.

I hope they are monitoring his kidney and liver function very closely. John's kidneys did not like Exjade. It would cause a rise in his creatinine even at low doses.

You dad has been through a lot and it makes sense that his recovery will take longer. Continue with the "Deb Therapy" . With John, we focused on restoring overall health to strengthen him. We did a lot with nutrition and energy work like acupuncture, chiropractic. We have done and continue with some herbal support and therapeutic essential oils.

Marlene

[Debra]

The last FE reading was 3,000!!! Dad is currently on 2,000 mg of Exjade daily, but I've had him stop it for the last couple of days due to the stomach cramping, diahrrea and nauseau. I'm thinking he might need to be on a lower dosage and have contacted his hemotologist at Southwestern Med. Ctr. What is difficult for us is that the Hemotologist in McKinney doesn't know very much about AA. He is simply taking labs, monitoring cyclosporine levels, and ordering transfusions. The good part is that his office and the hospital are only about 6 minutes from my parent's home, versus going to Dallas.
Also, Dad's last creatinine level was 4.0 and his BUN level is 60 - both high. Our PCP has recommended Dad see a Nephrologist, and we have an appt. the end of this month. She also wants us to see a Gastroenterologist!!
I don't know about you, but it sure gets old spending so much time in doctor offices and hospitals! I am desperately trying to get the Hem/oc in McKinney to streamline our appointments - as it takes 4 to 5 hours by the time we do labs, see the doctor, and then go to the hospital to get T&C. Then the next day are the transfusions. We have a few days off, and the whole process starts again! Not a great quality of life.
I know our life is operating under a "new normal", but I long for the day when Dad can participate in life again. With his stomach issues, he is afraid to go anywhere. Due to the wheelchair and his lack of mobility - bathroom tasks are challenging outside of his home. He won't even come to my house!
I guess you can tell I'm in one of my low points right now.....

[Marlene]

Hi Debra,

A couple of things to keep in mind with FE. FE is an accute phase reacant meaning anything can cause it spike higher. An infection, bad blood draw, and inflammation can cause it to react. There were times we saw John's jump 1000 points from one draw to the next. So once your FE is high, say 1000 +, it's not very reliable as to how much iron you really have. But it is safe to say that your dad's iron is high. How many red cell transfusions has he had?

With a creatine that high, your doc should stop the Exjade. I'm glad you did it for him. Exjade can effect it big time and can be serious. Read the insert on it or go to www.rxlist.com If the Exjade is the cause, it can take about one month for it to normalize. Again, desferral may be a better option. 2000 is the max dose and may be too much for your dad. Do you know what his creatine and bun were prior to starting? His doc should have run a base line on all his labs. I would not start up the Exjade until his kidney function tests normalize and then it would have to be a reduced dose. Then I would monitor it weekly.....Just my 2 cents.

Since John was never on Cyclo, I'm not very familiar with it but it too can be rough on the Kidneys. It depletes magnesiums. Hopefully others with experience can chime in here. You may need to reduce that also.

Have you considered adding in a probiotic for the digestion? Exjade messes with the environment and since he's had/has C-Diff, his bacteria in his gut is out balance.

IMO, your dad would better off staying off the Exjade until things settle down. The iron is an issue but it's not the critical issue right now.

Lastly, I would consult with a doc who's experienced with AA. Find someone at MD Anderson or contact NIH Dr. Neil Young. At very least, your doctor should be talking to someone.

Hang in there. You're a good advocate for your Dad.

Marlene

[Debra]

Your information is wonderful. We have switched hem/oc doctors - remaining at the same practice. Managing all the aspects of this disorder is mind-boggling.
Dad has been off the Exjade (Dr. W. agreed!)and will be considering adjusting the dosage. I was not aware that the FE level could be affected in the ways you pointed out! We have an appt. this week with a Nephrologist, which I am hoping will shed more light on the situation.
I have been supplementing with Vit. B., potassium, magnesium, and folic acid. Dad receives lasix with his transfusions, which I know also affects the kidney and mineral levels.
Dad also takes Prilosec, as well as a probiotic! I try hard to cover all the bases, more input from our new doctor will be great. Dr. W. seems to be much more pro-active, and was not happy that dad hadn't already seen a nephrologist. He also wants us to see a gastroenterologist - YIKES! Try fitting in all these appointments, and the weekly and sometimes twice weekly transfusions. Doesn't do much for the quality of life.
Additionally, we go to the VA hospital for help with meds. We are so thankful for this assistance, but despise the long waits and long drive.
By the way, Dr. W. feels a consult at MD Anderson would be a good idea. Even though Dad is 76, he thinks a stem cell transplant may be possible.
We continue our quest!

[Debra]

Dad had his appt. with the nephrologist today. I find it so frustrating that so few doctors know anything about AA, the treatments, the drugs, etc. I'm just not sure what to do at this point. This doctor had not even heard of Exjade. I explained it was an iron chelator, and that one of the side effects was high serum creatinine levels. Well, Dad's ferritin level is over 4,000 - so what do we do??? Even the doctors don't know what to tell me.

Does anyone have any suggestions?? I'm really concerned about Dad's kidneys - this nephrologist is concerned that Dad is heading toward dialysis! Is despherol a better option??

[Ruth Cuadra]

Debra,

You might want to post your question in our Transfusions and Iron Overload forum where people who know about these issues may be more likely to see it.

You should also contact Leigh Clark, Patient Educator at the AA&MDSIF, for information about iron overload. She can provide a balanced review of the pros and cons of the different treatment options. You can reach her by phone at 800/747-2820 or by email to clark@aamds.org.

Hope this helps.

Ruth Cuadra

[Marlene]

Hi Debra,

I assume you don't have results yet from the visit. So a couple of things to think about.

1 - I would ask for your dad's chem panel to be done weekly to see if over the next month his creatinine starts to drop over the next 4 - 6 weeks after stopping the Exjade.
2 - Is your dad on procrit? The kidneys produce the red cell growth hormone called EPO. They should check his EPO levels. It's pretty much accepted that if your natural EPO levels are less than 500, then there's good chance that Procrit could help those with SAA. Procrit was first used for those in kidney failure because they no longer produce EPO. Your doc should be able to make a case to try procrit.
3 - Are they checking his absolute retic count and percentage. This is good to track to see if there's an increase. An upward trend usually means your body is starting to produce more of its own red cells. Reticulocytes are immature red cells. As this increases, you should see a spacing out of red cell transfusions.
4 - I hope they did a sonogram of your dad's kidneys. They did John's when his creatinine was elevated to look for fluid build-up around the kidneys.
5 - Does your dad have edema? If fluid retention is not problem, then I would discuss with the doc to stop the lasix since it's hard on the kidneys.
6- People with iron overload are ususally deficient in Vitamin E and Vitamin C. You may want to look into adding that in.
7 - Finally, its been a while since John was on deseferal but i would ask your doc about it. Unfortunately, the kidnesy do the work when it comes to excreting the iron with desferal. While Exjade excretes via the feces. But....I do believe that desferal is easier on the body. If your dad has a central line, then he can do it via the port. Go to www.rxlist.com and you see lots of data on it.
8 - What are they saying about the impact the cyclo is having on his kidneys? There may be more kidney friendly immunosuppressant out there. You may get a more comprehensive evaluation if you go to MD Anderson. It really does make a difference when dealing with docs who know this disease.

IMO, I don't think the spike from 3000 to 4000 in the FE correlates to additional iron build up. It won't jump that much in two weeks from a transfusion.

It is very frustrating to have to deal with doctors who know nothing about what you are going through. As I type this, our doc is going head to head with the medical director at the blood donor services. They are refusing to do therapeutic phelbotomies on John. Of course they waited until we showed up for our appointment, kept us waiting for 45 minutes and then the doctor, who's never even looked at John's file, decides she doesn't want to do it. Mind you, he's had them done there in the past when his counts were not as good as they are now. Since she doesn't understand all of this, and chooses not to find out, she just says no.

Hang in there and take it a day at a time.

Marlene

[Debra]

Hi Marlene, and thank you for this info. Changing docs has had it's challenges. Though we remain in the same practice, the new doc needs to get up to speed on dad's history. It's like starting all over!!
* Dad does receive Procrit, but not regularly. I am wondering if it would make a difference in his rbc if he were to receive it on a more regular basis?
* To my knowledge, they are not checking his absolute retic count, and I will ask about that. Is that part of the CBC?
*He had a sonogram yesterday. The nephrologist did mention changing the cyclosporine to something less toxic on the kidneys.
*Dad had 2 units of RBC yesterday, with no lasix. Since Dad has not had edema in quite some time, we will be much more stingy with its use! Thankfully, the nurses at the outpatient clinic explained more about transfusions, fluid overload, urine output, etc. They are confident he is doing fine without the lasix!
*Dad is scheduled for more blood work and a 24 hr. urine test, but we were waiting until after his transfusion. The lab told us they need about 7 vials of blood to run all the tests ordered!! Since he got the trans. yesterday, we will do labs early next week.
*Dad's off the Exjade until we run the tests, and I'm very curious to see the results!

Interesting input regarding the FE count. The nephrologist is wondering if Dad's vasculitis might be active again and causing some of the kidney issues. The blood tests should tell the story on that.

Sorry you are battling with the phlebotomies. I am totally unfamiliar with this procedure as it relates to someone with bone marrow failure disease. As if the stress level isn't enough, we also have to fight constant battles for treatment. I pray that someday this will change.

One day at a time - that's my mantra (most of the time!). Being the control freak that I am, it is most difficult to handle that so much about this is totally out of my control. The kidney issues have been a major cause of concern and I am very upset that the previous doctor did not address it! It is the main reason we have switched docs. The other reason is that dad's labs were always a big secret! He only told me the platelet, WBC and RBC numbers! The rest was a mystery!

Again, thanks for the excellent info. Deb

[Marlene]

It sounds like your are making progress in the right direction.

Keep us posted. We learn so much from everyone's experience.

Take care,
Marlene

[Debra]

It's been a while, but I wanted to give a brief update. As of yesterday, Dad went 4 weeks between blood transfusions!!! Previously, he went 3 weeks on 3 units, this time it was 2 units. He goes in this morning for a CBC. Could something be going on in his bone marrow??? We hope so!
Dad's biggest issues right now are pain in his back, right side, and an upset stomach/lack of appetite. We are going to a gastroenterologist next week to see if we can determine the cause of these issues. I know his creatinine and BUN levels have been elevated. Dad is so sensitive to medications, I'm wondering if the cyclosporine is the problem with both issues.
He continues to make progress with his overall strength, taking short walks with the use of a walker. A big improvement from January, when we had to move him with a Hoyer lift!
I continue to pray for my Dad and for all those challenged by bone marrow failure diseases.
Have a wonderful weekend, Deb
dauther of Paul, dx 8/07 with SAA, ATG 9/07, ALG 5/08. Dad is a wonderful and gracious person, I'm so proud of him!

[Debra]

Hello all and a belated Happy Thanksgiving. It's been a while since I've posted, and we have faced some new challenges.

Dad blacked out in the bathroom on Nov. 15th, fell and hit his head. He had a sub arachnoil bleed in his brain, and gave us a terrible scare. Fortunately, he had received platelets on the 11th, and the bleed stopped before becoming a very serious issue. The CAT scan taken at the hospital upon arrival, and the MRI taken at about 8:30 that evening showed very little change in the amount of blood in the brain.

In the meantime, Dad's been complaining about back pain that has progressively been getting worse. They thought it may be his gall bladder, but an upper GI series concluded that was unlikely.

Because of the fall, the doctors did an MRI of his back, found some irregularities and followed with a bone scan. Still unsure, they performed a biopsy (on 11/25) in the T9 and T10 area of his spine. They have diagnosed Dad with osteomylitis, which is an infection in his bones! He is on a wide spectrum antibiotic, and will need to continue IV antibiotics daily for 42 days. Dad's no sissy, but the pain in his back became so bad that he could barely move. Needless to say, this has been a setback for him, as he has already been struggling with regaining his strength after his previous 5 month stay in the hospital.

My Mom is having a very difficult time with all of this, and I must admit, I have had many days where I get very discouraged and very tired. The complexities of dealing with severe AA are frightening and daunting. I know infections are a constant concern, as we are dealing with compromised immune systems.

In a way, Dad's fall was a blessing, as I don't know how long it would have taken to discover this bone infection! By the way, he never ran a fever - which is common with osteomylitis - however, the infection is quite extensive and the radiologist said it's no wonder he's been in such terrible pain!

Dad is also experiencing pretty bad esopageal spasms, which is making it hard to eat. Has anyone else had this difficulty? I wonder if it is a side effect of the cyclosporine, since it is such a potent medication with gastrointestinal side effects.

Additionally, Dad's kidneys were damaged 4 years ago due to the length of time it took to diagnose the vasculitis. The original antibiotic they were using for the infection was toxic to his kidneys, so we've changed meds. Of course, the cyclosporine is also tough on the kidneys - so it's been a real balancing act with all the meds.

I am so thankful for this site! Though I don't post often, I do check in frequently. I sincerely appreciate all the information and support you all offer.

Deb, daughter of Paul Wenz, diagnosed with SAA 8/07, 2 ATG treatments, last one in May 08, beginning to experience longer intervals between RBC transfusions. Dad is incredible, the kindest person I've ever known.

[Neil Cuadra]

Originally Posted by Debra

In a way, Dad's fall was a blessing, as I don't know how long it would have taken to discover this bone infection! By the way, he never ran a fever - which is common with osteomylitis - however, the infection is quite extensive and the radiologist said it's no wonder he's been in such terrible pain!

His fall must have been very frightening. It's lucky that you now know about the osteomyelitis; if only there had been an easier way to find it then it could have been dealt with sooner. I hope the antibiotics take care of the pain quickly, even though he'll need to stay on the antibiotics for weeks.

My Mom is having a very difficult time with all of this, and I must admit, I have had many days where I get very discouraged and very tired.

For almost a year and a half you've been helping your dad, helping your mom, learning the medical side of things, dealing with doctors, and managing your own life, so it's no wonder you can't always keep your energy up. And still you've taken the time to help other patients in these forums! You must have inherited your dad's kindness and strong spirit.

Are there others (relatives, friends, neighbors, coworkers) who can help you from time to time? It's hard to be superdaughter around the clock for month after month, and you need a break now and then. Your mom must spend a lot of time worrying about your dad too. Does she have others to talk to about it? In this thread a few Marrowforums members were talking about finding local support in the Dallas area. Although they said they were looking for an MDS support group, it's the people more than the diagnosis that count. You could post in that thread if you or your mom might want to connect with them.

[Debra]

Thanks for your kind words, Neil. We don't have any relatives here in Texas, and my sister lives in Kansas City, so it's difficult for her to get down this way very often - though she came to visit for Thanksgiving and it was a welcome little break for me.
Dad came home from the hospital today. He's a bit weak, and his back is still very painful - but I think he will make good progress at home. We have arranged for his IV antibiotics to be administered at home, and he'll get PT and OT also. The infectious disease dr. wants blood work twice a week, so I'm we'll keep track of his counts with those draws, and get him to the hospital as needed for transfusions.
I am so thankful the osteomyelitis was discovered, but hope that we will be able to keep it under control, as I understand it can be a chronic condition. What a bummer! I just wish there was a way to be more prepared for all the curve balls that get thrown at those of us battling these bone marrow failure disorders. This infection really caught me off guard!
Dad is amazing, despite it all. He's my hero.
Deb, daughter of Paul, dx. 8/07 SAA, 2 ATG treatments, beginning to go longer between blood transfusions.....

[Marlene]

Hi Debra,

Hopefully they are monitoring your Dad's magnesium level. Lack of magnesium will contribute to spasms and cramps. And if he's still on cyclo, it can lower mag. levels. Supplementing with magnesium citrate should be helpful. I know of others on cyclo who did. Maybe they'll post the dosage they took. You may want to start another thread specifically on the side effects of cylco.

Also, make sure they really watch for C-diff while on antibiotics since he had a bout of it earlier. A good probiotic could help here. You may want to ask them about prescribing one or getting one on your own.

Marlene

[Debra]

Update on Dad. The osteomyelitis is under control. Dad completed both of the IV antibiotics and is now on oral which he will take for another 3 months. He still has back pain, but it is controlled well with pain meds. The infectious disease dr. runs blood work every 3 weeks to be sure there is not a flare up of the infection and to check dad's kidney function and general metabolic condition. He's taking probiotics, and so far, he has not had any additional stomach issues. The cyclosporine is hard on his stomach, but I guess we have to live with it!
Dad went 7 weeks between blood transfusions - a new record! He still gets platelets about every 10 to 11 days; it has become part of our routine now. He is still very weak and is only able to walk very short distances. The back infection really slowed down his physical progress, however, we are thankful the he is feeling so much better and working very hard at physical therapy.
Dad's WBC have been hovering in the "normal" range! Yippee!
Our next hemo/oc appt. we will find out his ferritin level. Dad had a terrible time with the Exjade last year, and with everything else that has gone on, the dr. really has not been following the iron issue. I am concerned since Dad has received so many blood transfusions.
Thank you all for your informative posts. Though I don't post often, I try to read new posts and keep up on what everyone is experiencing. Thank you!
Debra, daughter of Paul, dx. 8/07 with SAA, ATG 9/07 & 5/08 - we are beginnning to see improvements in HGB and WBC. Still waiting on those sluggish platelets!!!!! Dad is incredible!

[Birgitta-A]

Hi Debra,
Perhaps your father could try Desferal for iron overload? Desferal has been used for 40 years. You have to get it iv or sc - there are no pills.

I have got 80 units of packed red blood cells and had a port-a-cath implanted for Desferal that I now get iv for 4 days in connection with transfusions. I have not felt any adverse reactions.
Kind regards
Birgitta-A
70 yo, dx MDS Interm-1 May 2006, transfusion dependent from dx, Desferal, Neupogen 2 injections/week for low white blood cells