TOPIC:

Hello everyone,

I’m the son of a newly diagnosed ITP patient. My father, 68, developed multiple pulmonary emboli three years ago caused by the presence of lupus anticoagulants. He was summarily diagnosed with anti phospholipid syndrome and prescribed blood thinners (Warfarin) Indefinitely.

Flash forward to August of 2019 when he was diagnosed with ITP after routine bloodwork at his hematologist’s office revealed a platelet count of 69 x 10^3. His hematologist ordered a bone marrow biopsy which revealed an indolent form of non-Hodgkins lymphoma which precipitated the ITP. As his platelet count dropped, his hematologist ceased his blood thinners. One week later he was in the ER presenting severe dyspnea and exhaustion.

Before his hematologist and the team of doctors treating him could start him on a course for Retuximab/Retuxin, he began throwing clots in his legs and kidneys. Unfortunately the medical team chose to delay administration of heparin and within three days of clotting, my father was forced to undergo a bilateral below knee amputation.

He underwent four rounds of Retuxin infusions before his hematologist began weekly injections of Nplate which my father has thankfully been responding to achieving a platelet count of 132 x 10^3 as of this week.

He’s been hospitalized since late August and transferred to a long tern acute care hospital (LTACH) on November 4th. The LTACH is presently pushing for a discharge soon (days not weeks).; however, no skilled nursing facilities will take my father as it appears to be policy here for them to supply all of his medications. But they refuse to supply Nplate as it is prohibitively expensive.

We’ve spoken with the Nplate ambassadors as well as the pharmacists at CVS Specialty, and after two frustrating weeks of confusion and miscommunication with the hematologist misunderstanding my father’s overall health and mobility, we have had them send a prescription to CVS Specialty. The case manager at his LTACH also found a skilled nursing facility (SNF) that will accept my father under the provision that we furnish their facility with Nplate at our expense.

The loss of my father’s legs and change in his quality of life has been devastating to us all. Physically and emotionally. But the frustration and patience required to sort through Medicare and a healthcare system where we often receive more shrugs and balks from sticker shock has been monumental. It seems ITP and unwillingness to procure Nplate makes my father too large a liability for most medical facilities to take him. I’m afraid of administration shuffling him through the cracks in the US medical system because it is the path of least resistance. He’s still too weak and unsteady transferring in and out of bed and we have yet to get guidance on how to modify his house such that it is ADA compliant for him to regain as much autonomy and independence as possible. Everything just feels so rushed.

Is this fear and uncertainty amidst the general healthcare system typical for ITP patients? And if anyone has bravely blazed this trail before, would they have any advice for a family under pressure and with so many time constraints given staff at these facilities are focused on the holiday rush?

Thank you kindly in advance,
R

R, I can't tell you how sorry I am to hear of your father's and your predicament. I'm afraid I can't begin to give you much practical advice about navigating the system or much medical advice for a case as complicated as this one.

Just about the only thing I can offer is that it's entirely possible that the Rituxan is the agent that's helping most. When was the fourth infusion? Average response time is around 6 weeks, but it isn't unheard of to work faster or slower, either. That's the drawback to administering two treatments at once for ITP. You can't ever be sure which is working. If this is the case... That it's actually Rituxan and not NPlate, that problem is solved. But ... You don't know for sure unless it's withdrawn.

Be advised that NPlate is NOT designed to normalize platelet counts. The optimal treatment goal is maintenance of a steady count in the 50 range, a so-called "safe but not normal" count. A count in the normal range while taking NPlate puts the patient at a higher risk of clots. There's a strict protocol for doctors to follow for titrating and skipping weekly doses dependent on the count. We've seen examples here of doctors who don't understand that.

This isn't much help to you, I know. But the board can be slow on holidays, and I hated to leave you hanging alone there. Take care. I hope things start looking up for you very soon.

As Midwest said, Nplate can caused increased clotting because it stimulates the body to produce new larger "stickier" platelets. So even for people who do not have clotting issues, it is recommended to keep counts below 100, around 50 is safer. I have been on Nplate for 5 years and my counts are safe at around 30-40. I would say from how I understand your story that your father has not had any bleeding events , brain bleeds or uncontrolled bleeding? People can live with low platelets, mine have been down to 1 and I've been fine. Just my opinion from experience, your father's counts on Nplate are dangerously high.

There have been other people on the forum with clotting issues along with ITP. Some research (don't have link) it is believed that the body actually lowers the platelet count to compensate for the APA (antiphospholipid antibodies), so in that the body is doing an amazing job protecting itself from clots by thinning the blood naturally. Now if a doctor uses a drug to boost counts up to normal, then it can get pretty risky.

There are several articles that discuss thrombocytopenia and APA. They say the low platelet condition is usually mild and doesn't cause problems. So here is the question: Does the low platelet count really need to be treated? Have to work that out with the doctors- but read and learn all you can. Good luck. Sorry your father has suffered so much, thanks for sharing your story.
www.ncbi.nlm.nih.gov/pubmed/8952756
rarediseases.org/rare-diseases/antiphospholipid-syndrome/

Sounds really tough.

How low did his platelets drop to? Did he have bleeding symptoms? If all is in the "safe range" you may not even need to consider ITP therapies at this time.

It is really unfortunate about the cost of medications. There are some grant programs that can help with Nplate cost in the US. However, getting the vials sent to you via CVS specialty will use up the grant money quickly. It is best if he can go to a nearby infusion center and then have the grant cover the expense of the drug. I think through CVS it would be about $4-500 per vial. At an infusion center it's perhaps $70. PAN and Healthwell are two I know of. You can ask either if there are others.

As, you may know, there are options other than Nplate. He could try Promacta, Tavalisse, perhaps the Rituxan will kick in more (or has already as midwest noted). Those are the most common, but they aren't the only options.

Best ~

I too am annoyed by the brief period my father spent on Retuxan. For some context, that was administered to him while he was in the acute hospital after his amputation from 8-27 to 11-4. Below is a graph to help illustrate his platelet count vs date with annotations for both his Retuxan and Nplate treatments.

Rituxan to Nplate transition (Imgur)

Unfortunately the LTACH hasn't been as good as furnishing his records and my energy has been focused on navigating the healthcare system for his next steps as opposed to collecting data.

It's difficult to determine the efficacy of Rituxan v. Nplate during this period, but given his recent platelet count has been fluctuating in the 90s to 100s; I think it is reasonable to assume that Nplate is at least keeping his count relatively high.

That said, thank you very much midwest and poseymint for the advice. My father's APS has been described as extremely severe by his hematologist. Within one week he was forming catastrophic clots not just in his legs but organs. So I am -extremely- concerned about clotting and not so much his risk of bleeding. (Frustrating since most of the general physicians and RNs are trained to have the opposite mentality due to the immediate complications of bleeding versus clotting.)

These conditions are all very new to me but I have read about the paradoxical nature of clotting and ITP. To answer your question, b2h, my father hasn't had any major bleeding symptoms. Platelet count aside, the only other symptom of ITP my father was presenting with was petechia in his lower extremities prior to his amputation.

For a bit more context, we have prioritized his conditions for the next two months (from highest to lowest):

1) amputation wounds
2) ITP
3) lymphoma

Because his mobility is severely limited and he still needs to be bladder trained from having a catheter inserted for weeks, maintaining his hygiene is our top concern. His wounds have already recovered from one infection and another one could lead to further operations and restarting the recovery process. (A process already impeded from an altered metabolism due to diabetes.)

To maintain good hygiene, I am negotiating a transfer to a skilled nursing facility (SNF) with an attached nursing home. His Medicare will cover 20 days of SNF care during which I will discuss with their management how to transition him to CNA level nursing home care covered privately. After 60 days of private care, his Medicare should reset and -hopefully- my father will be in a better position to perform more rigorous physical therapy required to regain his independence. (He hasn't been able to do much with a catheter, wound vac hoses and while on morphine every other day following debridement and wound care.)

He can transfer from a hospital bed to a wheelchair and vice versa using a transfer board, but just barely as a fall risk. (And with the bleeding risks associated with low platelets, the staff is reluctant to give him vigorous practice.) So he needs work on that, building upper body strength and core muscles so that he can maneuver better.

While he is receiving care, we can furnish Nplate via CVS Specialty. I am not enamored by this at all due to the high cost (we have yet to officially here what Medicare will and will not cover) and the lack of feedback from his absent hematologist.

But once my father regains mobility and independence we can transition him to receiving injections at his hematologist's infusion center. Fortunately this center is also an oncology clinic and his hematologist is also an oncologist. Unfortunately the hematologist also appears to be stretched very thin. She has been under the impression my father would rebound from the amputation quickly and regain his independence almost instantly and be able to transport himself to her infusion center.

But thank you all for your help and consideration. I appreciate your advice greatly since the medical professionals out here seem to be at a loss for words and almost adversarial due to the perceived liability of these conditions.

Rituxan isn't an ongoing treatment like NPlate. It's a four-week course of infusions, then stops. So that's one small thing you can relax about; it was done correctly. It is unusual for it to have been your father's initial treatment for ITP, though. It's not a first-line treatment for it.

You have one of the most complicated and overwhelming situations I've seen in my time in this group. I haven't ever had to navigate the system as you're having to do. You're father is lucky to have you on his side. Does he have any sort of case worker involved in all this? Medicare supplemental insurance plans generally can provide that kind of assistance.
I have no other suggestions to offer but do wish you both the best.

I just thought of one more thing that may matter, or it may not.
Medicare views my NPlate as a "treatment" vs. a "drug". It's covered under my Medicare part B, not part D. So you shouldn't have to obtain a supply from CVS as a specialty drug. My provider obtains it from the manufacturer and administers it accordingly.
Now... Whether a nursing facility is equipped to administer "treatments" is another question. My injection has to be given in a medical office, clinic, or hospital under the direction of an MD on site.

There is a "drug" option, a once daily pill called Promacta. It's billed under Part D. It's still dearly expensive, though, so it seems a facility still might not be open to obtaining it for your father's use.

We have had terrible luck with social workers and case managers, particularly in the acute hospital. His current case manager at the LTACH has been more helpful but she too is stumped as to how to work around SNF policies here that are prohibiting my father to be transferred to facilities able to provide him with the wound care and CNA care he requires.

That said, we secured the services of an elderly attorney who has provided advice regarding transfers between institutions. But there is only so much help they can provide given the general lack of knowledge of Nplate and my father's hematologist's lack of engagement in his wellbeing at large.

As for Medicare coverage, I'm not sure how Nplate will be covered with CVS Specialty. I'm aware Nplate is not covered by part D and since he isn't receiving this as a procedure in a clinic I don't know how part B would come into play if at all. We're simultaneously applying for a Drug Replacement Program designed to get him financial assistance with outpatient infusions at his clinic, but we were told two days ago that this process would take at least four weeks. This is hardly an ideal position to be in, but given his acute care coverage through Medicare is quickly being exhausted, we're simply buying more time by the week.

It's incredibly frustrating. But this is why I came here hoping to find helpful folks such as yourself. My hope is once we have some breathing room we can determine a more sustainable course of action.

In the meantime, the staff at the SNF have agreed to administer the Nplate injection. (Which seems suspicious, but I won't argue at this point.) I am under the impression it's a simple subcutaneous injection which can be administered fairly easily by family. And we have a nurse practitioner in the family who is willing to administer the injection should the facility's staff be unable to.

It's a messy situation. Like I said, I'm just trying to buy some time until his wounds heal enough that he's not at such a high risk for infection and another amputation. I couldn't foresee hitting the reset button on his healing process at this point given it has been 81 days since his surgery. But thank you so much for the advice, midwest. I really appreciate the thoughts and concern.

The injection is an easy sub q one, but not quite as simple as say, insulin. The preparation of the solution is an involved process. The med is shipped in powder form and must be precisely measured and mixed with sterile water. A good med tech could probably easily do it, but it may be more challenging for the typical, non-pro patient or family member. The manufacturer offers training and a prep kit for those with a need to self-inject. There's a member here who self injects and lives in the UK where it's more common to do so, but it's still unusual in the US.

You're on top of your game, R. You came here for advice, but it's clear you've already accomplished far more than many could ever do. Hang in there.

Sounds more like TTP than ITP. Have they considered that? The treatment is plasma exchange and immunosuppressant drugs. Here's a website about it.
Coincidentally my haematologist is on the little video there talking about it.

www.understandingttp.com/patient/about-ttp/