- Idiopathic pulmonary arterial hypertension – the cause of PH is unknown.
- Heritable PH – the disease is inherited.
- Associated pulmonary arterial hypertension – PH is associated with another disease or condition (e.g. connective tissue diseases such as lupus, congenital heart disease, HIV, chronic liver disease, etc. Scleroderma patients may also develop PH. There may be, for example, significant medical issues from dealing with scleroderma that complicate the challenges of PH.
- PH resulting from heart disease.
- PH resulting from lung disorders.
- Chronic thromboembolic pulmonary hypertension (“CTEPH”) is the only form of PH that may be curable other than lung transplant. 1 in 25 of those living with PH develops this condition.
- Chest pain
- Loss of energy
- Swelling of the arms, legs, ankles or abdomen
- Dry cough
- Raynaud’s phenomenon (discoloration and pain in the finger tips and perhaps toes that may be triggered by the cold)
- Brain damage can be a risk resulting from oxygen deprivation resulting in forgetfulness or worse but there is little research on this.
- If two or more family members have PH, or if a family member carries the PH-causing gene mutation, the risk of PH is more likely.
- Obesity and obstructive sleep apnea.
- Women are 2.5 times more likely than men to contract PH. Females of childbearing age are also more susceptible.
- Women after the age of puberty but before the age of menopause are the cohort most likely to be diagnosed with PH but there is not significant data on other forms of PH.
- Pregnancy is a risk factor. Also, women who already have PH and become pregnant have a much higher risk of mortality.
- Living at a high altitude for years can increase the likelihood of PH.
- Other health challenges, such as congenital heart disease, lung disease, liver disease and connective tissue disorders like scleroderma and lupus, are risk factors for developing PH.
- Organize and simplify finances and recordkeeping to make it easier to deal with all financial and other matters as your disease progresses.
- Obtain and sign key legal documents. These include:
- Durable power of attorney (authorizes someone to make financial and legal decisions).
- Health care proxy (designates someone to make health care decisions if the patient is hospitalized).
- Living will (statement of health care wishes, e.g. religious considerations, end of life decisions generally, decisions about mechanical ventilation, and DNR status)
- HIPAA release (authorizes someone to have access to patient’s medical records).
- Will (distributes assets and appoints a guardian).
- Revocable trust. In some instances a revocable trust can address many of the functions of a power of attorney and will providing a more robust mechanism to protect the PH patient as the disease progresses.
- Automate as many financial functions as possible to minimize the physical demands of handling routine financial matters (automatic deposits, bills automatically paid from checking account or charged to a credit card), recording a checkbook and all financial transactions on a computerized bookkeeping program like Quicken.
- Scanning as many financial and legal records as appropriate to be as paperless as possible.
- Review life, disability, health and long term care coverage to assess what options and benefits may exist and what steps may be advisable to take to secure those benefits.
- PH often results in short life expectancy. It is imperative that anyone diagnosed with PH begin to put their affairs in order. Many people living with a chronic illness may have no significant shortening of life expectancy. The reality of PH is that steps must be taken quickly.
- If there is a family history of PH or the gene has been identified purchase life insurance as early as possible if feasible. Also, advise children to do the same. In many cases it may be most economical to purchase a long term policy, e.g., 20-30 years depending on age that has conversion features. This will permit the patient to have insurance coverage for many years that may be precluded from doing so if he or she waits to a future date to purchase coverage. The term coverage might also include rights to convert portions of the coverage from term to permanent insurance without a medical examination. This may be the only way to obtain permanent coverage if in fact it is later advisable to have it.
- Example: Mary Jones is 18 and three family members have PH. But for that consideration she would likely not consider any type of insurance coverage and perhaps wait until she marries or has children. However, given the family history, she purchases a $500,000 30-year insurance policy at age 18 that has rights to convert all of it at specified future dates to permanent coverage.
- Some women with PH are advised not to become pregnant because of the increased mortality risks. Thus adoption, use of surrogates or other approaches may be more likely. If options like these are even contemplated the provisions in the patient’s will (and revocable trust if that document is used) defining “issue” should be modified to reflect the intention as to alternative methods of reproductive technology. Patients should also speak with family members, example a parent, encouraging them to modify their documents as well.
- Example: Mary Jones is living with PH and has been advised by her physicians not to bear children. As a result, Mary has explored adoption or contracting with a surrogate to be impregnated with her husband’s sperm. Mary’s parents’ wills provide for distribution on their deaths to their children (Mary and her siblings) but if any child (e.g., Mary) predeceases, her descendants will inherit. But the form will Mary’s parent uses defines “descendants” as natural born children, or children adopted by age 2. If Mary uses a surrogate or adopts a child older then age 2 that child will never inherit from Mary’s parents although other grandchildren may.
- Consider an organ donor card and including a provision authorizing organ donations in your living will and/or health care proxy so that scientific research into PH can be fostered.
- If the patient can afford a donation to organizations serving those with PH or researching possible treatments and cures, plan for this. If the patient’s estate will not be taxable for estate tax purposes there may be no tax benefit to making a bequest to such an organization. Instead make the donation now or include a clause in the patient’s durable power of attorney permitting the agent to make a donation. In that way an income tax benefit may be secured.
Pulmonary Hypertension: Estate and Financial Planning Considerations
I was recently asked by the Pulmonary Hypertension Association to present a webinar on estate and financial planning for those living with pulmonary hypertension or “PH.” This article reflects the reading and discussions on a disease I had previously no knowledge of so that I could create a power point and webinar for the organization. You can see a recording of the webinar and the power point at www.phaclassroom.org/financialplanning and visit the organization’s primary website at: www.phassociation.com . This is part of an ongoing effort to create resources for both professionals and consumers on a wide range of health challenges. The goals are to empower consumers and their loved ones facing challenges of chronic illness and other health challenges to better plan their lives so that they can have safer and more secure lives and to provide professional advisers the resources to quickly understand the planning implications and options for these chronic illnesses and other health challenges so that they can better advise clients without an undue time or economic burden on them in doing so. A wide range of materials have already been created covering Parkinson’s disease (“PD”) and young onset Parkinson’s disease (“YOPD”), multiple sclerosis (“MS”), Alzheimer’s disease, chronic obstructive pulmonary disease (“COPD”), and many more. A range of ancillary topics addressing using revocable trusts to safeguard those living with chronic illness, integrating care managers into the estate and financial planning process, the psychological and emotional aspects of PD and MS, and more have also been created. All these resources will be posted on a website www.chronicillnessplanning.org that should be launched by June 2016. An article on planning for Asperger’s will appear in a forthcoming issue of Trusts & Estates Magazine. Lectures are provided across the country each spring and summer on these topics as part of a charitable effort.
What is Pulmonary Arterial Hypertension?
Hypertension is high blood pressure caused by constriction of arteries throughout the body. Pulmonary hypertension (“PH”) is a type of high blood pressure that affects the arteries in the lungs. Pulmonary arterial hypertension (“PAH”) is a chronic disease that causes high plod pressure in the arteries that pass from the heart to the lungs. With PAH the arteries in the lungs become narrow and constricted. The result of this is that the right side of the heart has to exert greater effort to pump blood to the lungs, eventually leading to stress and damage to the heart, i.e., heart failure.
The blood vessels connected to and within the lungs narrow making it more difficult for the heart to pump blood through the lungs. A process called “fibrosis” makes the affected blood vessels become stiffer and thicker which increases the blood pressure within the lungs and further impairs blood flow. The increased demands on the heart cause a thickening and enlargement of the right ventricle hypertrophy, reducing the heart’s ability to pump blood through the lungs. The right ventricular muscle over time does not get enough oxygen to meet its needs, resulting in failure of the right side of the heart. PH was first identified by Ernst von Romberg in 1891. While there are therapies to ameliorate the impact of PH, and extend life expectancy, it remains incurable and is a chronic progressive disease.
There are different types of PH, including:
World Health Organization Group 1:
World Health Organization Group 2:
World Health Organization Group 3:
World Health Organization Group 4:
Symptoms and Treatments
PH symptoms may include:
As the disease progresses the symptoms may worsen and more moderate physical activity may trigger symptoms. For example, breathlessness that was triggered at an earlier stage from modest physical activity may occur while the patient is not exerting him or herself at all.
Estate and financial planning should contemplate and prepare the patient for these potential changes to minimize the exertion needed to maintain finances and to have a safety net in place to protect the patient. Financial planning must address the impact the disease course may have on earning capabilities.
Treatments for PH depend in part on the source or cause of the disease. If someone has PH due to liver disease, if you can treat the liver disease, the PH may improve. There are currently about 14 different PH treatments, with most for PAH. With chronic thromboembolic PH blood clots in the lungs scar and harden. Some patients can be evaluated and may be candidates for surgery that scrapes out the clots from the lungs which may resolve the problem. Some living with PH are candidates for transplant. The consequences and costs of a lung transplant are significant. Survival following a transplant may only be a few years. Some patients require both heart and lung transplant which is more complex, costly and difficult. Treatments can be incredibly costly from $50,000 to $250,000 per year. Financial planning must consider the costs of these treatments, what sources, insurance, family or other that may be available to apply to these costs. Estate planning must contemplate the time frames that may be involved and the significant risks associated with some PH treatments.
Who Gets PH
Almost anyone can contract PH. Common risk factors may include:
The risks and issues of pregnancy, and alternative options that may be pursued (e.g., adoption) should be incorporated into estate planning and documents, not only for the patient, but in many cases for his or her family as well.
Mortality and PH
While mortality is never an easy or pleasant topic to address, it must be addressed in order to plan properly. If the patient plans for too short of a life expectancy he or she may overspend, under-save and run out of financial resources. If a patient assumes too long a life expectancy he or she may put off planning for a period that precludes proper planning, or perhaps any planning. Understanding that planning, and addressing the difficult but essential issues of anticipated disease course and mortality, can empower the patient. An empowered and knowledgeable patient can have the most financially secure life for the years ahead, and best safeguard himself and herself as well as family and other love ones. Perhaps these dramatic benefits can motivate patients to tackle these emotionally difficult issues.
While the prognosis of PH has an untreated median survival of 2.8 years from time of diagnosis, with the cause of death usually being right ventricular failure, many patients can have much better results. With treatment, care, and earlier diagnosis and disease management longevity can be much greater. Some patients survive 4-5+ years and some in the PH community have been living with the disease for as long as 30 years. How well a patient reacts to medications can be an important factor. The patient’s physician can assist in evaluating a likely disease course for the particular patient, and estimating life expectancy based on a range of factors. This is critical to the type of financial and estate planning that is appropriate. It is also important that each patient communicate the discourse and life expectancy to his or her estate and financial planning team, and at periodic (ideally at least annual) meetings apprise them of any changes.
A problem is that many patients are diagnosed at a late stage because obesity and other factors may be assumed or misdiagnosed as the cause of the patient’s challenges. Improved education and awareness has and will change this.
Levels of mortality are very high in pregnant women with severe PH. Which is why, as noted above the recommendation is not to conceive.
Lung transplant may cure PH but the patient must deal with the complications of transplant and a post-surgical median survival of just over five years.
Specific Estate and Financial Planning Considerations for PH
Generally, those living with PH should take the same steps those living with any chronic illness and should consider the following steps. The goals of these steps are to minimize the physical effort that will be required to handle financial and legal matters, and to create safeguards to minimize the risk of financial abuse as the PH progresses.
Specific Estate and Financial Planning Considerations for PH
In addition to general planning that everyone with a chronic illness
If diagnosed with PH, although it will be difficult, it is imperative that quick action be taken to address a wide range of financial, legal and estate planning matters. Review and take steps to secure insurance benefits. Organize legal and financial affairs simplifying them to the extent feasible. Obtain the necessary legal documents and discuss them with the necessary people. Getting these steps under control as early as possible will make matters easier and more secure and enable the patient to focus his or her attention on the more important health challenges they face.
No related posts.