It’s about 10 p.m. on a Thursday night when you’re dispatched for a patient who is feeling weak and dizzy and is afraid his Flolan pump may be malfunctioning. You have seen patients with equipment such as insulin pumps and left ventricular assist devices (LVAD), but have never heard of a Flolan pump, and since Flolan is not a common prehospital drug, you don’t know that much about it. While your partner drives, you take out your phone and search information on Flolan. Obviously, having a basic understanding of Flolan and the Flolan pump ahead of time would make this call less stressful, and potentially allow you to provide better care. This is the goal of this article.
Pulmonary artery hypertension (PAH) patients can vary greatly; some may not appear sick at all, while others may need a wheelchair, require oxygen, or have medication being continuously delivered by a pump. These patients may call 911 because of complications associated with their illness, or an interruption of their medication delivery due to a problem with their equipment.
Pulmonary artery hypertension is a disorder of the pulmonary arteries and occurs when pulmonary artery pressure (PAP) rises above the accepted range due to a cause other than normal aging or changes in altitude. The pulmonary arteries become narrowed causing pressure in the arteries to rise which can cause strain on the heart and potentially become life threatening.
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According to the American Heart Association, “pulmonary blood pressure is normally a lot lower than systemic blood pressure. Normal pulmonary artery pressure is 8-20 mm Hg at rest. If the pressure in the pulmonary artery is greater than 25 mm Hg at rest or 30 mmHg during physical activity, it is abnormally high and is called pulmonary hypertension.”1
Epoprostenol is used to treat PAH in patients who have already been treated with other medicines that did not work well. It belongs to a group of drugs called prostaglandins which occur naturally in the body and are involved in many biological functions. Epoprostenol works by relaxing blood vessels and increasing the supply of blood to the lungs, reducing the workload of the heart and decreasing the work of breathing. It can be inhaled in the form of Veletri or given IV as Flolan. Inhaled Veletri will normally be seen only in the hospital, while patients may be sent home on IV Flolan and is thus the focus of the remainder of this article.
Before the approval of epoprostenol, PAH was treated mainly using a combination of non-specific treatments including oxygen, calcium-channel blockers, digoxin, diuretics and warfarin. Epoprostenol was developed in the 1970s, followed by the evolution of the portable pump throughout the 1980s. Currently it is estimated that about 2,000 patients in the United States have been discharged home with a Flolan pump. A good follow-up question for the present is whether PAH has been affected by the COVID pandemic. While PAH could be identified as a comorbidity and potential risk factor for COVID, the incidence of PAH patients with COVID seems to mirror the incidence in the general public, although the mortality does appear to be higher.2
The major actions of epoprostenol are vasodilatation of the pulmonary and systemic vascular beds and inhibition of platelet aggregation.3 Unfortunately, the drug has a very brief half-life and is not stable at room temperature. It requires refrigeration for storage and may need to be kept cold while being infused. Epoprostenol is usually initiated at 2ng/kg/min, this dose is then gradually increased at increments of 1-2 ng/kg/min to achieve symptomatic relief.4
It is not uncommon for some patients to be on a dose of 40-70 ng/kg/min, or higher over time. Like many other medications, the goal of dosing is to achieve optimal benefit while staying at a safe level. It can be given in two ways: short-term through a peripheral IV and long-term through a surgically placed central venous catheter using a small battery-powered pump outside the body. It is this pump that EMS providers may see but be not yet be familiar with.
The Flolan Pump
As with the insulin pump, mechanical ventilator or ventricular assist device (VAD), people can now be sent home with a Flolan pump, which was previously only seen in medical facilities. This means that there is a higher potential for EMS providers to respond to a call that involves one of these devices.
IV Flolan can be administered using a small, lightweight infusion pump using standard disposable 9 volt or AA alkaline batteries.
The pump allows patients to receive their dose of medication uninterrupted. While using Flolan, patients are normally instructed to maintain two working pumps at all times because the medication needs to be administered without interruption and the pumps need to be alternated every 24 hours. Typically, pumps are also replaced every six months throughout the therapy for testing and maintenance. This process is mandated by the FDA and is designed to make sure the pumps are always working properly.
When patients are discharged with a Flolan pump to take home, they will receive education about their illness and the pump. Their infusion pumps require minimal care, but the patient will generally be told that: 1) the pumps are not waterproof so care must be taken when showering or swimming, 2) the medication reservoir cassette must be securely fastened before starting each infusion cycle, and 3) batteries may be an issue because the pumps use them up quickly. Battery life will depend on the amount and rate of medication being delivered and the temperature, but most patients are instructed to change their batteries weekly.
The pump must infuse Flolan continuously because the therapeutic effect of Flolan dissipates quickly. Interruptions in the delivery of Flolan for as little as 2.5 to six minutes may result in rapid symptomatic deterioration. This requires the pump’s reservoir to be replenished at regular intervals so in addition to learning how to operate the pump patients are required to learn sterile technique and care of the intravenous catheter. Patients must also change the extension lines at about the same intervals and must return to their doctor or hospital approximately every five weeks to replace the catheter needle. Careful cleaning of the catheter is crucial.
In some cases, the Flolan is infused at lower temperatures and the use of ice packs and a special pump pouch are needed to maintain the medication at around 45 degrees. The ice packs which fit next to the medication cassette need to be changed every six to eight hours to keep the temperature constant. It could be helpful for EMS providers to be aware of this information in case a patient has forgotten or become confused.
Flolan pumps, like insulin pumps are compact and may be worn in a harness or on a belt so a patient can remain ambulatory during treatment. They are designed to deliver a continuous dose of Flolan through a catheter into the bloodstream. Some reasons for a patient to call EMS could be the catheter becoming dislodged or damaged, or if the pump stops working. Other potential reasons for EMS to be called are listed below.
In this type of continuous infusion, 911 could further be called for problems such as sepsis, thromboembolism, or syncope related to the use of an implanted central vein catheter. Obviously, any patient with these complaints would be treated according to local protocol, but providers should keep in mind that the underlying problem could be regulation of the medication. A patient wearing a Flolan pump may need expedient transport to an appropriate facility, ideally the facility where the pump was placed.
The reason is that the presentation of PAH problems can be vague and non-specific, including complaints of dyspnea and fatigue, and there is no prehospital procedure that can help identify PAH, as the EKG can provide evidence of a STEMI. Standard tests performed in an ED will include EKG, chest x-ray, lab values, and echocardiogram, looking for evidence of strain on the right heart.
Another question that could arise is what if a person wearing a Flolan pump calls 911 for a completely unrelated reason, how would this impact treatment? The best answer is to treat the patient based on the presenting complaint and the provider’s assessment, while possibly making adjustments based on the patient’s history. Similar examples would be withholding nitroglycerin to a patient who has recently taken sildenafil or decreasing the dose of midazolam in an elderly patient. Some guidelines that are used in the ED that could provide some guidance are:
- Prioritize oxygenation since hypoxemia and hypercapnea cause vasoconstriction of the lungs,
- Give fluids cautiously. Remember the right ventricle is already under additional strain, and
- Right ventricle support is extremely important. The drugs of choice are normally dobutamine and milrinone. However, it is important to note that these agents may cause hypotension and vasopressors such as epinephrine and vasopressin may consequently be needed.
The following are offered as pearls for EMS providers to remember:
- Never turn the pump off – this could be fatal. A Flolan infusion should not be interrupted for any reason, including cardiac arrest.
- Only the treating physician can calibrate and reprogram the pump.
- If there is a problem with the existing line, a peripheral IV will be needed and the pump tubing will need to be connected directly to this IV. Pump tubing should be connected to as little IV tubing as possible.
- Do not infuse any other medication in the line where the Flolan is infusing.
- Do not give a bolus of IV fluid (this may worsen heart failure).
- Do not change to a different infusion pump without first talking to the patient’s PAH specialist. Like an LVAD, the specialist’s contact information should be on the pump.
- If the patient is transported to the hospital, their spare infusion pump must go with them.
- Note that fever and/or drainage from the catheter in patients on IV therapies may indicate a sudden and serious onset of a line infection.
- Whatever other actions are taken, always notify the PAH specialist of the patient’s problem and condition.