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ITP: a patient's perspective

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Crystal’s Story

Severe ITP in a 75-year-old woman who had a spontaneous remission after one year

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Crystal was diagnosed with ITP by her primary care physician when she had her annual physical examination and routine blood check on July 31, 2001. 

 

Looking back, Crystal recalls being very tired for no reason at the beginning of May 2001.  She also remembers seeing tiny red spots on her legs for the previous 3 months.  But she had had no excessive bruising and no other bleeding symptoms – no nose bleeds, no bleeding gums.

 

At the time of her annual physical examination, Crystal was volunteering for a local hospital.  She was called by her doctor’s office and asked to come in for another blood test.  Her blood platelet count was extremely low (6000) and her doctor thought there must have been an error.  However, the repeat platelet count was also extremely low - 10,000.  (A normal platelet count is over 150,000.)

 

Crystal had been in excellent health her entire life.  She was taking no medications.  She had never had high blood pressure.  After the low platelet count was confirmed in her doctor’s office, Crystal was taken immediately to the hospital and a hematologist was called to see her.  She was started on prednisone tablets and was given intravenous gamma globulin (IVIG) to help raise her platelets.  She had no reaction to the IVIG.  The hematologist did a bone aspirate and biopsy; the report was normal.  “At that point, I didn’t feel bad,” says Crystal.  In fact, her medical history, physical exam, and laboratory data were all completely normal, except for the low platelet count and the red spots (petechiae).  The next day, on August 1, she was released from the hospital and given a prescription of prednisone.

 

Crystal seemed to be responding to the IVIG with her platelet count rising to 32,000 on August 8, 2001.  But by August 16, her count fell to 6,000, then 15,000 on August 23, while she was still taking prednisone.  On August 28, Crystal received anti-D at her hematologist’s office.  This is another intravenous treatment for ITP, similar to IVIG.  The treatment went well; she had no symptoms.  However on August 31, Crystal had her count checked and her platelets had plummeted to 2,000.  It seemed that “anti-D didn’t do any good and the prednisone wasn’t working,” says Crystal.

 

Crystal then started another course of IVIG while continuing with the prednisone (60 mg/day).  Again, she responded to the IVIG and her platelet count increased to 122,000 by September 4.  Unfortunately, the response was brief and her platelet count fell to 5,000 again by September 19. 

 

Crystal received a third treatment of IVIG on September 24 to raise her platelet count in preparation for a planned splenectomy.  The following day, she continued with the IVIG treatment, received a platelet transfusion, and had her spleen removed.  The day after surgery, on September 26, Crystal was feeling good.  Her platelet count jumped to 49,000.  At this time, her prednisone was reduced to 50 mg/day.  Unfortunately, the next day, her platelet dropped to 34,000 and reached a low of 8,000 on October 5.  Crystal, her husband, her family, and her doctors were all very disappointed by the ineffectiveness of the splenectomy and all the other treatments.  What could they do now?  This chart describes the first 2 months of Crystal’s ITP.

 

Date

Platelet count

Treatment

Outcome

July 31, 2001

6,000

Prednisone (30mg/day)

IVIG #1

Diagnosis of ITP.  Responded to IVIG.

 

Aug. 8

32,000

 

 

Aug. 16

6,000

 

 

Aug. 23

15,000

 

 

Aug. 28

 

anti-D

No increase in platelets.

Aug. 31

2,000

IVIG #2 & Prednisone (60mg/day)

Responded to IVIG.

Sept. 4

122,000

 

 

Sept. 19

5,000

 

 

Sept. 24

 

IVIG #3

 

Sept. 25

 

Splenectomy

Responded to IVIG and a platelet transfusion.

Sept. 26

49,000

 

 

Sept. 27

34,000

 

 

Oct. 5

8,000

 

Platelets decreased again.

 

By October 9, Crystal had noticeable facial puffiness as well as puffiness on the back of her neck and shoulders.  These were part of the side effects of her ten weeks of prednisone treatment.  Along with the side effects of prednisone and low platelets, she had some bruises over her hands and arms.  These may have been caused by her low platelets, but this bruising could also have been caused by prednisone; steroids can make the skin become very thin.  Crystal was then referred to another hematologist, who specializes in ITP.  They saw him on October 9.  Her son came from California to be with her.  Together Crystal, her family, and her doctors coordinated Crystal’s plan of treatment.

 

The major issue in the treatment plan was whether any additional treatment would provide more benefit than harm.  A lot of different medicines have been used to treat patients with ITP who have failed to respond to splenectomy.  These are all drugs to suppress the immune system and slow down the destruction of platelets.  But these drugs are effective in only about 15-20% of patients.  And they can have severe side effects, especially an increased risk for serious infection.

 

The most important reason to treat patients with ITP is to prevent bleeding.  But Crystal had never had any significant bleeding – only tiny red spots on her skin.  So her hematologist told her that additional, stronger medicines would certainly make her worse because of their side effects, but how could they make her better?  She had no bleeding problems.  She felt well – except for the effects of prednisone.  Any additional medicine would only be given to prevent bleeding.

 

But was Crystal at risk for serious bleeding?  Her platelet count was extremely low – 9000.  But her hematologist told her he thought she was safe.  He said that if she had a nose bleed, or gum bleeding – wet bleeding – that he would be worried.  Or if her blood pressure was high, he would be worried that the low platelets would increase the risk for stroke.  Or if she had any other medical problems that could weaken her blood vessels, such as diabetes, he would be worried.  He worried more about her because she was 75 years old; he thought her risks for bleeding were greater than if she had been 17.  But at the end of a long conversation, everyone – Crystal, her husband, her son, her doctor – agreed that no treatment at all was the best plan.

 

Most importantly, Crystal’s doctors recognized that continuing prednisone was not an option, as more severe side effects would inevitably occur and these side effects could be dangerous in a 75 year-old woman.  The greatest risk is bone loss or osteoporosis.  Prolonged use of prednisone for several more months would result in significant osteoporosis with increased risk for a fracture with any fall.  Also, long-term use of steroids may lead to cataracts in a 75 year-old woman, and the decreased vision would increase the risk of falling.  An increased risk for serious infections may be the most serious problem with prolonged steroid use.  Furthermore, “Prednisone was really bothering me.  I didn’t feel good taking prednisone,” says Crystal.  As a result, the hematologist discussed with Crystal, her husband, and her son about reducing and eventually discontinuing prednisone.  This was a key point in her treatment.  Crystal, her family, and her hematologist all shared the decision to gradually take Crystal off prednisone, to avoid the unnecessary side effects and to closely observe whether she would remain asymptomatic with no treatment for her persistently low platelets.  A schedule was developed to gradually decrease the prednisone dose and stop it in 6 weeks.  Crystal was very happy with this plan.  To stop prednisone treatment had been her major goal.

 

On October 24, her platelet count was 8,000.  There was no change in her bruises, and she continued to feel well, to sleep well, and to maintain cautious normal activities.  She continued to volunteer at the local hospital.  Additionally, she received a flu shot.  A week later, she came down with a cold.  She also had a sore bump in her mouth.  The following day she went to her family doctor for the cold.  Her family doctor was very concerned about the low platelet count (2000).  She consulted Crystal’s hematologist and placed her in a hospital for another IVIG treatment.  She was released that evening.  Three days later, her platelet count was 9000.

 

On November 12, her platelet count was 7000.  Her prednisone was reduced to 5 mg every other day.  By November 26, her platelet count was 9000 with some bruises.  She had been off of prednisone for a week and feeling well.  Her hematologist said that she should start getting her blood count only every two weeks, not weekly, then every month, then every two months.  Now that she was off prednisone, she noticed some aches and pains, probably old arthritis symptoms that had been suppressed by the prednisone.

 

December 4, her platelet count was 6000.  After sending an e-mail to her hematologist, he called back and assured Crystal that “Everything was about as expected.  As long as there were no signs of bleeding, he was confident.  He was nervous, but he was still confident that this was the best approach.”  If bleeding or blood blisters in her mouth occurred, Crystal would have to start some type of chemotherapy treatment.  January 3, 2002 her count was 4000.

 

January 14, her platelet count was 6000.  Crystal told her hematologist that there was a small amount of bright red blood showing up in her stool.  Her hematologist said, “It was not from the nose, the blood had to be from lower down.  Blood coming all the way through from the nose would be digested and turn black.”  Her hematologist told her that he was considering treatment with vincristine, a type of chemotherapy used for ITP, because of the blood in her stool.

 

January 21, Crystal’s platelet count was 5000.  Her hematologist told her, “Hang in there; stay the course.”  Her doctor thought she would feel better if she didn’t get a platelet count every week.  The weekly blood count just caused everyone to worry too much!  So Crystal began to have her platelet count checked every two weeks.

 

On February 5, 2002, Crystal’s platelet count dropped to its lowest level, 1000.  Two days later, her platelet count went up to 3000.  But at these low levels, probably these platelet counts are not actually any different.  Crystal was told by her hematologist to continue her normal activities unless blood blisters in her mouth or bleeding occurred.  If that happened, she was to notify him immediately.  On February 20, her platelet count was 6,000.  Crystal felt good.  Her only problem was that she had lost some hair.  Her doctor thought that her hair loss was due to the prednisone she had taken.  Her hematologist agreed that since Crystal’s platelet had consistently remained below 10,000 for 5 months, and blood had been present in her stool previously, some attempt to increase her platelet count was now appropriate.  Everyone, including Crystal’s doctors, just seemed worn out by the nervous anticipation of checking platelet counts and watching for bleeding.

 

Finally, on February 21, Crystal was started on vincristine treatment.  She was scheduled for vincristine intravenously once a week.  After her first two vincristine treatments, her platelet did not change much, 4,000 to 8,000.  On March 11, Crystal was not feeling well – she had no energy and she had stomach and leg pain.  Her hematologist said that he would stop the vincristine if her platelet count was not up by the next week.  By March 13, after 3 treatments, Crystal’s platelet count jumped to 32,000.  Then, Crystal was given her last vincristine treatment on March 14.  A week later, her count was at 38,000.  However, throughout that week, Crystal says that she suffered quite a lot from the side effects of vincristine.  She recalls the stomach ache, constipation, and numbness and tingling in her fingers.

 

Unfortunately, Crystal’s platelet count dropped to 14,000 on March 28, then to 13,000 by April 10.  Crystal’s hematologist said that since the vincristine hadn’t been effective, they would – again – just watch carefully for any signs of bleeding.  Now, 8 months after her diagnosis with ITP, Crystal still had never had any significant problem with bleeding.  Her only problems had been from her treatments, not her ITP!

 

Through the spring and summer, Crystal and her husband gradually became more adjusted to living with her low platelet counts.  The platelet counts were spaced out to every 2 weeks, then every month, then every 2 months.  Crystal and her husband traveled to visit family and friends.  Life seemed almost normal again.  All of the symptoms from the prednisone and vincristine treatments had disappeared.  Then on August 22, her platelet count was 20,000.  Then it was 25,000 on November 27.  Was the ITP going away – on its own?  This was almost too good to be true!  Three months later, on February 25, Crystal’s platelet count increased to 37,000.  Her most recent blood check was on May 28, 2003; her platelet count was 53,000.  Now it seemed certain.  Crystal’s ITP was going away.  Or at least it was much less severe and her platelet counts were in a very safe level.

 

When asked what has been most helpful in her experience with ITP, Crystal quickly replies, “My faith in the Lord.”  Crystal says, “We would pray” and also there are “a lot of people praying for me.”  She also mentioned the continual support she receives from her husband and doctors.  Recalling her experience with ITP, Crystal says, “At the very beginning of this, I felt that everything will be OK.  I had a good state of mind about it.”  For the past six months, Crystals says, “I have been feeling pretty good.”  She claims that “I have a good quality of life,” as she has little to no restrictions with her daily activities.  Although she notes, “I am cautious about bumping into things because I still bruise easily.”  Her most recent platelet count at 53,000 makes her feel more confident in her recovery, and “I like to see my platelet count continue to go up.”  One issue that concerns Crystal and her husband is traveling.  According to Crystal’s husband, “It makes us a little nervous when we travel,” especially when Crystal’s platelet was low.

 

Given her experience with ITP, Crystal’s advice for people with a new diagnosis of ITP is, “Have a positive outlook on it, have good faith in the Lord, and be optimistic.”  For herself, she says, “I had a good mind about it from the start,” feeling that she would recover.  Crystal also had a good support group in her family, friends, doctors, and people who pray for her.  Crystal’s optimistic outlook on ITP, her faith in the Lord, and her support group have been important in her recovery.

 

Crystal’s update: January 2007

 

After her difficult experience with ITP five years ago, Crystal recovered quite dramatically.  We are happy to report that she continues to be in good health today.  She sees her hematologist and gets her platelets checked every six months.  At the time of her last update on this website, Crystal’s platelet count had increased to 53,000.  Then Crystal’s platelet count increased even further to 113,000 in June of 2004!  Since that time her platelet counts have been in the normal range, higher than 150,000.  She hasn’t had any ITP-related problems such as bruising or bleeding.  Her husband stays in touch with her hematologist by email.  He sent the following email on December 18, 2006:  “Crystal went for her semi-annual check with her doctor on 12/14/06.  Her platelet count was 338,000.  Sooo good.  She is doing well.  Her next appointment is 12/13/07.  She talked him into twelve months instead of six for the check-ups.  We wish you and your family a Merry Christmas.”

 

When asked about how often she thinks about ITP, Crystal says, “I do think about it often, and am grateful for the recovery, but it does not affect my life.”  She says that she thinks about ITP more when she hears or reads about someone who is affected by it.  Her advice to others with ITP is to be patient, have faith, and follow the advice of a hematologist that you trust. 

 

Crystal has stayed very active over the past few years.  She and her husband continue to volunteer at their local hospital two days a week, and she exercises daily.  Crystal states, “My activity has not been curtailed by having had ITP.”  In fact, she is soon traveling to California for the wedding of a granddaughter. 

 

In the summer of 2006, Crystal reached an important milestone.  She celebrated her 80th birthday!  She states that she feels good and is in apparent good health “for an 80-year-old!”  Crystal and her husband are looking forward to their 60th wedding anniversary this year.  We wish Crystal and her husband many happy, healthy years to come. 

 

Note by Mr. Doan:

 

I was very impressed by the record keeping by Crystal and her husband.  They kept a very detailed diary of the events throughout the course of Crystal’s ITP, which was used as a reference in our interview for specific dates, facts, events, and platelet counts.  The story of Crystal is the result of the information provided by the diary and a personal interview with Crystal and her husband in July 2003.

 

Comments by Dr. George

 

Crystal’s Story demonstrates as vividly as any experience I have had about the value of cautious, conservative treatment for patients with ITP.  The goal for treatment of ITP is only to treat or prevent bleeding.  There is no other goal.  The platelet count tells us something about the risk for bleeding, but even patients with very low platelet counts for a very long time may have minimal bleeding symptoms, or none at all.  The worry is that patients with very low platelet counts may be at great risk for bleeding.  Sometimes patients worry about this, but even more often their doctors worry about the risk for bleeding.  Therefore patients with ITP who have very low platelet counts are often treated with many different medicines even though they have never had any significant bleeding.  And the outcome is often that the treatment of ITP is much worse than the disease itself. 

 

When I first saw Crystal on October 9, 2001, she, her husband, and her son and I talked about all of these things.  Crystal was impressively healthy, except for the apparent toxicity from prednisone.  Her face was round, her cheeks were puffy, she had some bruising on her forearms related to steroids, she had the lump in between her shoulder blades – which is often called a “buffalo hump”.  And this was only the beginning of the prednisone problems.  As we said above in Crystal’s story, it would only get worse.  She would have accelerated osteoporosis with risk for fracture, she would have accelerated development of cataracts, she would have risk for serious infections.  To continue prednisone was not an option.  It had to be stopped.  But what about any other treatment for ITP?  Anything else had some chance for success, but a greater chance for not changing the platelet count at all.  Furthermore, any additional treatment for Crystal would have risks of more serious side effects, particularly the risk of suppressing her immune system and making her vulnerable to serious infection. 

 

Therefore as we sat and talked together, the decision evolved that we would gradually decrease the prednisone dose and stop it all together in about six weeks, and that she would receive no other treatment.  In one way, this just seemed perfectly reasonable, the common sense thing to do.  However in another way, this seemed radical and even frightening.  Crystal’s platelet count was less than 10,000, where the risk for bleeding may be high.  Crystal was 75 years old, not 17.  Certainly her blood vessels would be more brittle than those of a young person.  Older patients with ITP seem to have greater risk for bleeding than younger patients.  But, although Crystal was 75, she was healthy, bright, alert, and determined to avoid toxicity from medicines as much as she could.   She had had enough problems already!  A key point in her evaluation was her normal blood pressure.  If her blood pressure had been high, that alone would have increased the risk for stroke.  The risk for a bleeding stroke would be greatly increased if the platelet count is extremely low. 

 

At the end of our discussion, the decision just seemed the natural and right thing to do.  Therefore Crystal gradually decreased and stopped her prednisone, and gradually after that she lost the toxic effects in her appearance and began to feel better.  But then the issue was the constant focus on the weekly platelet count, and the frightening numbers that always stayed less than 10,000 and even at times approached zero.  Crystal’s hematologist in her own community was comfortable with our decision, but I suspected that her family doctor thought that we were all crazy!  I am sure that her family doctor felt extremely nervous every time Crystal had a platelet count of 6,000, or 4,000, or even 1,000.  So, after several months, it just seemed inevitable that a trial of some form of treatment was appropriate.  Crystal had had some blood in her stool, but otherwise she had no bleeding symptoms, other than the few petechiae on her legs and ankles.  But I think that everyone was worn out with the anticipation and apprehension.  We had talked about the possibility of additional treatment for months, it just could not be put off forever.  Therefore the treatment with four weekly injections of vincristine seemed reasonable.  Vincristine had a transient effect but no lasting effect.  The side effects of vincristine were substantial, with abdominal cramping, constipation, and toxicity of the nerves in Crystal’s fingers which lead to numbness and tingling.  So after four weekly injections, the vincristine was stopped.  The platelet count returned to 9,000 where it had been before.  And gradually the problems with vincristine toxicity went away as well.

 

Once having tried some additional therapy and having appreciated once again that the treatment was worse than the disease, everyone seemed to settle down and seemed better prepared to just live with the low platelet count.  Crystal’s life gradually returned to more normal activities.  She and her husband began to travel again.  This was all very good for her.  Perhaps the best thing was spacing out the platelet counts so she didn’t have the focus on the platelet count number week after week after week. 

 

Then, later that summer, a year after her diagnosis, an amazing thing happened.  Crystal’s platelet counts gradually began to increase – all on their own.  The ITP was gradually going away without any treatment.  Doctors refer to this as a “spontaneous remission”.  It is said to not happen often in ITP, but I am not sure that we know how often it happens.  I believe that many patients like Crystal gradually get frustrated with their lack of response to treatment, then gradually drift away from their physicians.  Their physicians may not aggressively search for them because they also are frustrated by their inability to provide effective treatment.  So perhaps many patients ultimately have a complete remission without any doctors knowing what has happened.  Crystal has excellent physicians in her community.  She has excellent rapport with her doctors.  She trusts them and they trust her. 

 

Now, nearly two years after her diagnosis, her experience with ITP seems to be securely in the past.  However the future remains somewhat unpredictable.  I think the chances are good that her platelet count will gradually recover to normal.  Even if her platelet count does not reach normal, she is certainly safe from any risk for serious bleeding now, with her platelet count of 53,000.  But, since we do not know why the ITP appeared two years, we cannot be certain that it will not reappear in the future.  But for now, Crystal will only have a platelet count several times a year.  Then, in another year or two, she will be back to her routine annual exams.  Back to where she was before this story began. 

 

Update, January 2007:  As noted above, Crystal’s platelet count did recover completely to normal and it has remained normal.  In the future, management of patients like Crystal may be different.  New drugs are being developed for ITP that stimulate platelet production and do not suppress the immune system.  Currently these drugs remain experimental but they may be approved for use in ITP patients in 2008.  These drugs may have been a safe and effective option for Crystal in 2001.  They would not have cured her ITP but they could have kept her platelet count at a higher, safer level until the ITP cured itself.  Crystal’s experience teaches us very important lessons:  (1) Treat the patient, not just the disease.  (2) Make decisions together: the patient, family, and the doctor.  (3) Stay in touch.  This is easy in our email era, and it provides reassurance for the patient and continuing education for the doctor.