Looking for some answers

My Dad has been in the hospital for eleven days with platelet counts bouncing between 1 and 3. The doctors don't seem to know what's going on, so I'm trying to find some answers. Here's a brief rundown of what's happened:

• Turned 60 in January. About 20 lbs overweight. No prior history of platelet trouble.
• Most of March: Fought some kind of viral infection, seemed to be getting well.
• March 22: Went to ER with petechia all over his hands, forearms, feet, legs to the knees, scalp and forehead. At least ten blood blisters on tongue and lips. Platelets measured at 3. Admitted to hospital and put on Prednisone.
• March 23: Platelets down to 1. Cancer ruled out. Diagnosed with diabetes and high blood pressure (likely due to hereditary factors more than life habits), but said to be "unbelievably healthy and strong" otherwise.
• March 24 - 27: Platelets alternate daily between 1 and 3. No obvious response to Prednisone. Blood blisters completely gone from mouth. Petechia still visible but old-looking; no fresh-looking spots. Blood pressure and blood sugar responding excellently to medication.
• March 28: Platelets at 1. Two-day IVGA treatment started.
• March 29: Platelets at 3. Second day of IVGA.
• March 30: Platelets at 3. No additional treatment.
• March 31: Platelets at 2. One new blood blister on lip, no new petechia. Transfusion started around 11:30pm.
• April 1: Despite transfusion, platelets still only 3 as of 4:30am.

The hematologist still expects Dad to have a turnaround within a few days. Other doctors disagree and there is talk of transferring to another hospital with more access to hematology specialists. I've done enough research to know there may not be a medical answer to this.

I don't have a specific question, but am interested in thoughts as to what we might be dealing with here and what some good things to do or ask about might be.

Thanks!

Matthew

Matthew, you didn't say whether your dad has type I or type II diabetes. Type I diabetes is often an autoimmune reaction, often triggered by a viral infection, but with a genetic predisposition. It is not uncommon for multiple autoimmune diseases to affect the same person.

ITP is diagnosed by eliminating other diagnoses. Is your dad's bloodwork normal except for low platelets and high blood sugar? What else have they tested for? Are they planning a bone marrow biopsy?

This is a lot for you to take in at the same time. There are many very knowledgeable and experienced people on this website, so you have come to the right place.

Nobody seems to know if he has type I or II diabetes, but we're trying to find out and I'll update here when we know.

They say his blood work is outstanding except for the low platelets and high blood pressure. All other counts are excellent. I don't know what all tests have been performed, but I do know they have done some analysis of vitamins and sodium and it all came back great. I'll be over there again tonight and will see if I can get complete records of all the tests and results.

A bone marrow biopsy is on the table, but they're reluctant to do it because of how low his platelets are.

It's interesting to me that his count was clear down at a 3 within four hours of a transfusion. That sounds like something in him is really efficient at killing platelets right now.

Matthew:

Hello - it's good that you found us. There are a lot of knowledgeable people here.

ITP is a disorder in which there tends to be platelet destruction and problems with platelet production. A bone marrow biopsy will not prove if production is adequate regardless of the fact that a doctor may tell you that it does. Many Hematologists are not as up to date as you'd think. A bone marrow biopsy is a good idea if the patient is 60 or older because MDS is more common at that age and low platelets can be a symptom of that.

Platelet transfusions usually do not work for people with ITP because as you said, the platelets are destroyed quickly. They are usually reserved for emergency situations because people can become refractory to them.

There are still many treatment options available at this point. Most people eventually do respond to something, but it can take time to find the right one. ITP is often triggered by viral infections and only time will tell if it is acute or chronic. He could spontaneously remit on his own or after he responds to a treatment.

A count of 1 and 3 are pretty much the same thing. Platelets fluctuate constantly due to constant production and destruction. Some people here have had two counts run from the same blood sample and gotten two different results. Counting is not an exact science, but it's close.

You're right, there are no clear answers to ITP. A good Hematologist is a good place to start because right now, your Dad has only been given two of the oldest ITP treatments around. They are both first line treatments, but it is time to move onto a newer, different option.

Thanks Sandi. What sorts of treatments should we be asking the hematologist about, or perhaps pursuing on our own?

Also, in case it's relevant, Dad has Type II diabetes. I'm working on getting a full report of what blood tests he's had done and the results, and will post here when I have them.

Thanks so much for the replies so far - it means a lot just to talk to people who know what it's like!

Hi Matthew,

Ditto to what Sandi said. It's time for a second opinion. If you tell us what city you are in, maybe we can steer you in the direction of an expert. You could have your dad's current doctors contact one of the experts, too, and maybe save the inconvenience of transferring to a different hospital.

Is your dad still on Prednisone? and at what dose?

Than he received IVIG right? You said transfusion at some point were you referring to IVIG or a platelet transfusion?

Matthew:

Some treatments are known to work quickly and some take time. I'd imagine that you want one that works quickly so your Dad can get out of the hospital and out of the danger zone. The treatments that I would look at right now would be N-Plate or Promacta. They are only a few years old, but many refractory people have had luck with them. Both of them stimulate platelet production and they are considered maintenance drugs, meaning that you have to keep using them to maintain the counts. When using either of those drugs, the goal is keep the platelet count around 50k, not to normalize the count. 50k is considered to be a safe count for daily living. People have gone into remission while using those two drugs, but you can worry about remission later...get the counts up first.

Win-Rho is another treatment you could consider, but it doesn't always work. If it doesn't, then you've wasted a few days waiting. There are other options, but those would be your best bet for the moment. If it were me, that's what I'd want to do. Steroids can raise blood sugar, so I'd try to stay away from those right now.

If he's never been previously diagnosed with diabetes, and if he has had no signs or symptoms of diabetes other than elevated blood sugar, it's entirely possible the elevation is due to the steroids and will abate once the steroids are out of his system. This is pretty early after that viral illness. He may be an acute case that will clear up soon, but those are pretty low counts, so I'd say an experienced hematologist is the way to go. If you are near a teaching hospital/medical university, they usually have hematologists who are up to date on treatment options. (They're also great at managing patients who have multiple medical issues, since they have all the specialists under, or near, one roof, and usually all can access each others' records on the patient to better coordinate care.) By the way, platelet transfusions are rarely used in ITP except for bleeding emergencies, because, as our hema once said, ITP patients' systems "chop up" donor platelets just as they destroy their own platelets. Hope he rebounds soon.
Norma

Thanks for the input everyone. I'm still trying to get printouts of all the blood tests. There seems to be a fair bit of red tape before they'll let me get that; hopefully it will be today.

We're near Peoria, Illinois. If anyone can recommend some specialists anywhere near here, we'd love to check them out.

Winnifred, I don't know Dad's Prednisone dose except that it's allegedly the "maximum dose" for him. I can find out the amount today.

Dad's platelets were up to 5 yesterday and 6 this morning, so the trend is up, but very slowly. We're happy with any improvement at this point, but recognize there's a long way to go. From what I've read, I'd feel more comfortable if he could get above 10 and stay there, but who knows about that.

On the other hand, the hematologist he's seeing (who has seemed disinterested from the beginning) says there's no reason to stay in the hospital if Dad won't agree to a splenectomy, so he's supposedly going home tomorrow, regardless of what his count is. This is a bit concerning to my parents because all along they've been told he can't go home until he gets to at least 20. We're looking into trying to get additional opinions, as well as the drugs Sandi mentioned the other day.

I think Michael Tarantino is in Peoria. He's a pediatric hematologist, but perhaps he is associated with a clinic that also sees adult patients. He has been to ITP conferences and has been a medical advisor for PDSA.

Matthew:

Actually, patients with low counts do not have to be hospitalized. That decision is usually based on symptoms and the opinion of the doctor. At 3k, I was still working. However, any doctor who wants to kick him out because he doesn't choose splenectomy is not worth keeping in my opinion. There are other options and a patient has a right to choose. The patient and the doctor should work together to find the best option and those options should be presented. Splenctomy is as much a gamble as any other treatment and should never be forced on anyone.

Here is a video presented by a top ITP Specialist. He has a very different view regarding splenectomy.

fhs.mcmaster.ca/medicine/hematology/ITP-2010/player.html

Matthew,

How is your father?I've been thinking of him and hoping he found a great doctor, and also that he's improved.

Norma